Joshua M.

Abzug
Scott H. Kozin
Dan A. Zlotolow
Editors

The Pediatric
Upper Extremity

1 3Reference
The Pediatric Upper Extremity
Joshua M. Abzug Scott H. Kozin
Dan A. Zlotolow
Editors

The Pediatric Upper

Extremity

With 1116 Figures and 449 Tables

Editors
Joshua M. Abzug
University of Maryland School of Medicine
Baltimore, MD, USA

Scott H. Kozin
Shriners Hospitals for Children
Philadelphia, PA, USA

Dan A. Zlotolow
Shriners Hospitals for Children
Philadelphia, PA, USA

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To the children and adolescents all over the world who have
differences and/or injuries that involve their upper extremity
Joshua M. Abzug

To my mentors in hand surgery at the Mayo Clinic, many of whom

have become friends and are critical in my professional and
personal life. Their inuence has compelled me to provide the
highest-quality care to my patients and has guided me to achieve
success in my career as a physician and as a person
Scott H. Kozin

To my coeditors for their tireless efforts on this project and all of

the authors for their ongoing, enduring contributions to the care
of the pediatric hand
Dan A. Zlotolow
Foreword

It is paradoxical that focus on the childs hand came rather late in the specialty
of Hand Surgery. Treatment methods for the adult hand and upper limb
initially developed following surgical experience in adults during the Second
World War. The reverse was true in adult orthopedics; the children came
rst. Orthopedics as a specialty prospered when First World War soldiers
were treated by English orthopedic surgeons using methods developed
in dedicated crippled childrens hospitals before the war. Some First World
War soldiers even returned to crippled childrens units in England for
convalescence.
Furthermore, until recently in well-respected childrens orthopedic centers
treating large numbers of children with hand anomalies, there was little interest
in these cases among pediatric orthopedists. The care of childrens hand
problems in these centers was relegated to what I call hobbyists. A hand
surgeon or two in a particular community would volunteer to spend a day or
two each month to run a hand clinic in pediatric orthopedic hospitals. Even in
standard Hand Surgery texts, with few exceptions, pediatric upper limb prob-
lems in children were treated as an afterthought. In a real sense, the pediatric
hand was the last of the areas in Hand Surgery to be fully explored by surgeons
who had dedicated their practice to children.
This began to change at the end of the twentieth century as pediatric
orthopedic hospitals nally had the foresight to establish a hand surgery
service with full-time hand surgeons and hand therapists. These pioneer men
and women were willing to limit their careers exclusively to the treatment of
children. This changed the face of Pediatric Hand Surgery, and training
programs for surgeons in childrens hand surgery methods were established.
The editors of this work are among the most distinguished of this new group
of pediatric hand surgeons. They and others like them have taken the
treatment of childrens hands to a whole new level. Today, full-time pediatric
hand surgeons are spreading throughout North America and around
the world.
Kozin, Zlotolow, and Abzug have also enlisted other well-respected surgi-
cal colleagues with unique and extensive experience in specic problem areas
to contribute to this textbook. Because of this, the nal product lays out the
current state of our art in this new and specialized eld, Pediatric Hand
Surgery, a subspecialty that has nally come into its own.

vii
viii Foreword

Surgeons-in-training as well as the experienced hand surgeon will nd

The Pediatric Upper Extremity a thorough, up-to-date, and useful reference
in their management of these problems. This title is destined to assume a
leading place in Hand Surgery libraries around the world.

Dallas, Texas, USA Peter R. Carter

August 2014
Preface

Pediatric Upper Extremity Surgery is emerging as a standalone subspecialty,

that combines the anatomic and disease specic knowledge of hand surgery
with the unique physiology of the growing child. The worldwide expertise in
all facets of Pediatric Upper Extremity Surgery has grown exponentially over
the past few decades. This has been fueled in large part by the dedication,
innovation, and cooperation of the authors that contributed to this title and
those that came before them.
This book is the rst of its kindan entire work dedicated to Pediatric
Upper Extremity Surgery. The book is divided into various sections to
cover the gamut of pediatric upper extremity surgery, including embryology,
physical examination, imaging, anesthesia, therapy, outcome measurements,
congenital differences, nerve injuries, brachial plexus palsies, spinal cord
injury, neuromuscular disorders, trauma, infection, tumor, compartment syn-
dromes, burns, skin lesions, vascular disorders, rheumatologic diagnoses, and
sports injuries. Woven into each section are chapters that provide the necessary
detail for the diagnosis and treatment of the pediatric upper extremity.
Operative techniques are detailed in tabular form and highlighted by photo-
graphs to maximize the reader's benet. Pearls and pitfalls are discussed by the
experts to optimize patient care.
We hope that the accumulated wisdom of these pages will help to enhance
current practice and stimulate others to advance the eld of pediatric upper
extremity surgery.

August 2014 Joshua M. Abzug

Scott H. Kozin
Dan A. Zlotolow

ix
Acknowledgments

It would not have been possible to pursue and complete this project without the
knowledge, hard work, and wisdom of my mentor and coeditor Scott H. Kozin,
MD. I am truly indebted to him for the mentorship he has provided me during
my early career. Additionally, Dan A. Zlotolow, MD, my other coeditor, has
been a great friend and teacher during my early years of practice. His hard
work and dedication to this project has denitely been shown, and I thank him
greatly for his willingness to be our go-to illustrator. Additionally, the contri-
butions of all of our authors are greatly appreciated as their collective wisdom
is what has made this book such a great reference for those who treat the childs
upper limb. Without question, the lives of children will be changed for the
better thanks to their hard work and willingness to contribute. It is especially
important that I thank my parents, Edward and Tobie, and my Aunt Renee, all
of who helped support me and encourage me during my education and
training. Most importantly, I want to thank my family including my boys,
Noah and Benjamin, and my amazing and very understanding wife, Laura, for
understanding the time and work involved in completing this project. Daddy is
nally done doing his chapters.
Joshua M. Abzug

I want to acknowledge that behind every great book is a host of experience,

contributors, and supporters. Experience is attributed to the patients and
families that I have had the privilege to treat over the last 20 years. These
relationships have made me a better physician and person. Contributors to
this book include the cadre of talented authors and the photographic excel-
lence of Brian ODoherty. Sharing their knowledge with the readership has
resulted in an authoritative treatise on pediatric upper extremity. Supporters
include my coeditors and friends, Josh Abzug and Dan Zlotolow. Our
relationship and teamwork has bettered the care of children and challenged
dogma. Family support for such a project is without question. My wife,
Louise, and our children, Bryan and Samantha, provide ongoing joy and
gratication.
Scott H. Kozin

This work would not have been possible without the help of Brian ODoherty,
whose photographic skills are beyond compare. I want to thank my friends

xi
xii Acknowledgments

Josh Abzug for his vision and perseverance in conceiving and then pushing
this work to completion, and Scott Kozin for everything he has taught me in
the clinic, the operating room, and in life. And to my wife Marie who endured
the frequent refrain, Ill get to it after I nish the book.
Dan A. Zlotolow
About the Editors

Joshua M. Abzug is Assistant Professor

in the Department of Orthopaedics at
the University of Maryland School of
Medicine. He is the Director of Pediatric
Orthopaedics at the University of Maryland
Medical Center and the Director of the
University of Maryland Brachial Plexus
Clinic.
Dr. Abzug initially was introduced to the
eld of pediatric upper extremity surgery
during a medical mission trip, when he had
the chance to work with Howard Clarke,
MD. Subsequently, as a resident, Dr. Abzug
had the opportunity to work with
Scott Kozin, MD. After the rst week together, Dr. Abzug decided that he
wanted the pediatric upper extremity to be the focus of his practice. Upon
nishing his orthopaedic residency, Dr. Abzug went on to complete two fellow-
shipsone in adult hand and upper extremity surgery at Thomas Jefferson
Medical College and The Philadelphia Hand Center, under the tutelage of
A. Lee Osterman, MD, and the second in pediatric upper extremity surgery at
Shriners Hospital for Children in Philadelphia and St. Christophers Hospital for
Children, under the tutelage of Scott Kozin, MD and Martin Herman, MD. Upon
completion of the fellowships, Dr. Abzug moved south to Baltimore to begin his
current practice.

xiii
xiv About the Editors

Scott H. Kozin graduated from Duke

University in 1982 with a degree in
computer science. Medical School was
completed at Hahnemann University in
Philadelphia, followed by orthopaedic
residency at Albert Einstein Medical
Center. Fellowship was completed in
1992 at the Mayo Clinic, focusing on
hand and microvascular surgery.
Dr. Kozin initially cared for adults and
children until the year 2000, when he
devoted his practice and research to
children at Shriners Hospitals for Children
in Philadelphia. Since that time, he has
been an advocate for improving the lives of children via research, education,
and patient care. Dr. Kozin is currently Chief of Staff at Shriners Hospitals for
Children in Philadelphia.
Dr. Kozin has published over 100 peer-reviewed papers, mainly on the care
of children with various diagnoses including brachial plexus injury, spinal cord
injury, and congenital differences. He routinely travels to developing countries
to operate on children in need. Dr. Kozin received the Weiland Medal by the
American Society for Surgery of the Hand in 2010, which honors a hand
surgeon/scientist who has contributed a body of research that advances the
eld. He is President of the American Society for Surgery of the Hand 2014.
Dr. Kozin is also a devoted husband and father to his two children, Bryan
and Samantha. During his leisure time, he enjoys traveling and mountain
bike riding.

Dan A. Zlotolow's passion for pediatric

hand surgery began as an intern with a
syndactyly reconstruction performed by
Berish Strauch. This was strengthened
later in residency by working with the
talented Vishal Sarwahi. As a hand fellow
at the Roosevelt Hospital in New York City,
he had the opportunity to travel to the Texas
Scottish Rite Hospital for Children to
learn from Peter Carter, Scott Oishi,
and Marybeth Ezaki. After a few years
of an adult and pediatric hand practice, he
joined Scott Kozin as a full time pediatric
hand surgeon. His practice focuses
on post-traumatic reconstruction, arthrogryposis, congenital differences, and
brachial plexus injuries.
Contents

Volume 1

Part I Development . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1
1 Embryology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3
Yen Hsun Chen and Aaron Daluiski

2 Functional Development . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 25
Susan Duff

Part II Physical Examination . . . . . . . . . . . . . . . . . . . . . . . . . . . . 37

3 Physical Examination . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 39
Andrea Bauer and Michelle James

4 Outcome Measures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 57
M. J. Mulcahey and Scott H. Kozin

Part III Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 75

5 Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 77
Archana Malik, Jacqueline A. Urbine, Erica D. Poletto,
Bret Kricun, Evan Geller, Polly Kochan, Robert L. Siegle, and
Eric N. Faerber

Part IV Anesthesia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 115

6 Anesthesia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 117
Andrew J. Costandi and Vidya Chidambaran

Part V Occupational Therapy . . . . . . . . . . . . . . . . . . . . . . . . . . . . 139

7 Orthotics and Casting . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 141
Jenny M. Dorich and Carrissa Shotwell

8 Occupational Therapy Evaluation and Treatment . . . . . . . . . 171

Sarah Ashworth, Timothy Estilow, and Deborah Humpl

xv
xvi Contents

Part VI Congenital Differences . . . . . . . . . . . . . . . . . . . . . . . . . . 197

9 Introduction to Congenital Differences and Genetics . . . . . . . 199
Heather Lochner

10 Hypoplasia, Brachydactyly, and Other Failures of

Formation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 213
Scott A. Riley and Ronald Burgess

11 Radial Deciency . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 237

Nick A. van Alphen and Steven L. Moran

12 Ulnar Deciencies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 265

Mohammad M. Al-Qattan and Abdullah Al-Thunyan

13 Syndactyly . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 277
M. Claire Manske and Charles A. Goldfarb

14 Syndromes Associated with Syndactyly . . . . . . . . . . . . . . . . . . 297

Lorenzo Garagnani and Gillian D. Smith

15 Duplication . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 325
Goo Hyun Baek

16 Macrodactyly . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 369
Amit Gupta and Charity S. Burke

17 Thumb Hypoplasia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 389

Scott H. Kozin and Dan A. Zlotolow

18 Constriction Band Syndromes . . . . . . . . . . . . . . . . . . . . . . . . . 413

Gloria Gogola

19 Metabolic and Endocrine Abnormalities . . . . . . . . . . . . . . . . . 431

Krister Freese and Arabella Leet

20 Genetic Syndromes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 453

Julie D. Kaplan and Carol L. Greene

21 Skeletal Dysplasias . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 467

Alphonsus K. S. Chong, Rosalyn P. Flores, and Eng Hin Lee

22 Toe-to-Hand Transfers in Children . . . . . . . . . . . . . . . . . . . . . 483

Neil Ford Jones

Part VII Nerve . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 513

23 Nerve Anatomy and Diagnostic Evaluation . . . . . . . . . . . . . . 515
Jeffrey A. Stromberg and Jonathan Isaacs

24 Ulnar Nerve Injury . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 529

Angela Wang
Contents xvii

25 Median Nerve Injury . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 543

Allan Peljovich and Felicity Fishman
26 Radial Nerve Injury . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 563
Anjan P. Kaushik and Warren C. Hammert

Part VIII Brachial Plexus Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . 587

27 Neonatal Brachial Plexus Palsy . . . . . . . . . . . . . . . . . . . . . . . . 589
Charles T. Mehlman
28 Microsurgery for Obstetrical Brachial Plexus Palsy . . . . . . . . 607
Marc C. Swan and Howard M. Clarke
29 Glenohumeral Joint Secondary Procedures for Obstetrical
Brachial Plexus Birth Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . 633
Roger Cornwall
30 Secondary Procedures About the Elbow, Forearm,
Wrist, and Hand . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 653
Scott H. Kozin, Dan A. Zlotolow, and Joshua M. Abzug
31 Traumatic Brachial Plexus Injury in the Pediatric
Population . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 683
Harvey Chim, Allen T. Bishop, Robert J. Spinner, and
Alexander Y. Shin
32 Transverse Myelitis and Neuralgic Amyotrophy . . . . . . . . . . 711
Allan Belzberg, Glendaliz Bosques, and Kelly Pham

Volume 2

Part IX Tetraplegia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 733

33 Upper Limb Reconstruction in Persons with Tetraplegia . . . 735
Scott H. Kozin, Dan A. Zlotolow, and Joshua M. Abzug

Part X Neuromuscular Disorders . . . . . . . . . . . . . . . . . . . . . . . . . 767

34 Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 769
Nina Lightdale-Miric and Carolien P. de Roode
35 Arthrogryposis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 803
Dan A. Zlotolow and Scott H. Kozin
36 Inherited Muscle Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 839
Diana X. Bharucha-Goebel and Carsten G. Bnnemann

Part XI Trauma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 859

37 The Multiply Injured Child . . . . . . . . . . . . . . . . . . . . . . . . . . . 861
Susan Scherl
xviii Contents

38 Non-accidental Trauma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 879

Richard M. Schwend
39 Nail Bed Injuries . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 899
Ryan Katz
40 Flexor Tendon Injuries . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 909
Steve K. Lee and Joseph J. Schreiber
41 Extensor Tendon Injuries . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 929
Robert B. Carrigan
42 Amputations/Replantation . . . . . . . . . . . . . . . . . . . . . . . . . . . . 943
Daniel Calva, Harlan M. Starr, and James P. Higgins
43 Pediatric Phalanx Fractures: Evaluation and
Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 961
Ross Feller, Augusta Kluk, and Julia Katarincic
44 Metacarpal Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 981
Shannon Cassel and Apurva S. Shah
45 Hand Dislocations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1005
John Lubahn, Rey Ramirez, Raymond Metz, and
Patrick Emerson
46 Carpal Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1029
Theresa O. Wyrick
47 Distal Radius Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1047
Ahmed Bazzi, Brett Shannon, and Paul Sponseller
48 Forearm Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1069
Johnathan J. Whitaker, Brandon M. Tauberg, Michael S. Kwon,
and Martin J. Herman
49 Monteggia Fracture Dislocations . . . . . . . . . . . . . . . . . . . . . . . 1095
Lisa L. Lattanza and Sam Chen
50 Galeazzi and Essex Lopresti Injuries . . . . . . . . . . . . . . . . . . . . 1107
Kevin Little, Philip To, and Reid Draeger
51 Supracondylar Humerus Fracture . . . . . . . . . . . . . . . . . . . . . . 1121
Afamefuna Nduaguba and John Flynn
52 Distal Articular Humerus Fractures . . . . . . . . . . . . . . . . . . . . 1137
Anish G. R. Potty, Sasha Job Tharakan, and B. David Horn
53 Medial Epicondyle Fractures, Elbow Dislocations, and
Transphyseal Separations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1167
Rachel Y. Goldstein, James Lee Pace, and David L. Skaggs
54 Radial Head, Radial Neck, and Olecranon Fractures . . . . . . . 1203
Brandon S. Schwartz, Joshua M. Abzug, Charles Chan, and
Joshua E. Hyman
Contents xix

55 Proximal Humerus Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . 1227

Casey M. de Deugd and Steven L. Frick
56 Humeral Shaft Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1243
Brandon S. Schwartz and Joshua M. Abzug
57 Clavicle Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1259
Brandon S. Schwartz, Raymond Pensy, W. Andrew Eglseder, and
Joshua M. Abzug
58 AC Dislocations, SC Dislocations, and Scapula Fractures . . . 1277
Brandon S. Schwartz, Raymond Pensy, W. Andrew Eglseder
and Joshua M. Abzug

Volume 3

Part XII Infection . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1299

59 Pediatric Hand Infections . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1301
Leo Kroonen
60 Cellulitis and Necrotizing Fasciitis . . . . . . . . . . . . . . . . . . . . . . 1323
Joshua A. Ratner
61 Osteomyelitis and Septic Arthritis . . . . . . . . . . . . . . . . . . . . . . 1331
Andrew R. Tyser and Douglas T. Hutchinson

Part XIII Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1351

62 Benign Soft Tissue Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1353
Ngozi M. Akabudike
63 Malignant Soft Tissue Lesions . . . . . . . . . . . . . . . . . . . . . . . . . 1363
David S. Geller
64 Benign Bony Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1385
Brandon S. Schwartz, Scott H. Kozin, Dan A. Zlotolow, and
Joshua M. Abzug
65 Malignant Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1419
Mark E. Puhaindran and Edward A. Athanasian

Part XIV Compartment Syndrome . . . . . . . . . . . . . . . . . . . . . . . 1437

66 Compartment Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1439
Fraser J. Leversedge
67 Late Management of Compartment Syndrome . . . . . . . . . . . . 1453
Milan Stevanovic and Frances Sharpe

Part XV Skin Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1479

68 Skin Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1481
Mark A. Cappel and Katherine J. Willard
xx Contents

Part XVI Burns . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1509

69 Burns . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1511
Michael A. Baumholtz and Mark P. Solomon

Part XVII Vascular Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1543

70 Congenital Vascular Disorders . . . . . . . . . . . . . . . . . . . . . . . . . 1545
Benjamin Christian and Joseph Upton
71 Acquired Vascular Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . 1579
Anne Argenta, Lorelei Grunwaldt, and Alexander Spiess

Part XVIII Rheumatologic Disorders . . . . . . . . . . . . . . . . . . . . . . 1591

72 Rheumatologic Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1593
Donald P. Goldsmith and Scott H. Kozin

Part XIX Factitious/Somatization Disorders . . . . . . . . . . . . . . . . 1607

73 Factitious Disorders, Conversion Reaction, and Malingering
in Children . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1609
Meredith Osterman, L. Andrew Koman, and A. Lee Osterman

Part XX Sports Injuries . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1619

74 Caring for the Athlete . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1621
Christine M. Goodbody, R. Jay Lee, and Theodore J. Ganley
75 The Throwing Athlete . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1635
Gregory Pinkowsky and William Hennrikus
76 The Gymnast . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1667
Michael S. Bednar

Part XXI Arthroscopy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1673

77 Wrist Arthroscopy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1675
Min Jung Park and Jeffrey Yao
78 Elbow Arthroscopy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1689
Dan A. Zlotolow
79 Shoulder Arthroscopy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1709
Gerald R. Williams and Bryan J. Loefer

Part XXII Potpourri of Other Conditions . . . . . . . . . . . . . . . . . . 1733

80 Pediatric Trigger Thumb and Finger . . . . . . . . . . . . . . . . . . . . 1735
Ann E. Van Heest
81 Kienbocks Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1743
Abdo Bachoura, Sidney M. Jacoby, and Eon K. Shin
82 Madelungs Deformity . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1763
Scott N. Oishi, Lesley Wheeler, and Marybeth Ezaki
Contents xxi

83 Torticollis and Sprengels Deformity . . . . . . . . . . . . . . . . . . . . 1773

Neil Saran, Edward J. Harvey, and Jean Ouellet
84 Multi-ligament Laxity . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1811
Kevin J. Little
85 Thoracic Outlet Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1823
Brandon S. Schwartz, Joshua M. Abzug, Dan A. Zlotolow, and
Scott H. Kozin

86 Prostheses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1835
JoAnne L. Kanas
Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1857
Contributors

Joshua M. Abzug University of Maryland School of Medicine, Baltimore,

MD, USA

Ngozi M. Akabudike University of Maryland School of Medicine,

Baltimore, MD, USA

Nick A. van Alphen Plastic Surgery Department, Mayo Clinic, Rochester,

MN, USA

Mohammad M. Al-Qattan Divisions of Plastic Surgery, King Saud

University and King Fahad National Guard Hospital, Riyadh, Saudi Arabia

Abdullah Al-Thunyan Divisions of Plastic Surgery, King Saud University

and King Fahad National Guard Hospital, Riyadh, Saudi Arabia

Anne Argenta Department of Plastic Surgery, University of Pittsburgh

Medical Center, Pittsburgh, PA, USA

Sarah Ashworth Shriners Hospital for Children, Philadelphia, PA, USA

Edward A. Athanasian Hand Surgery Service, Hospital for Special Surgery,

New York, NY, USA

Abdo Bachoura UPMC Hamot, Department of Orthopaedic Surgery, Erie,

PA, USA

Goo Hyun Baek Department of Orthopedic Surgery, Seoul National

University College of Medicine, Seoul, South Korea

Andrea Bauer Shriners Hospitals for Children, Sacramento, CA, USA

Michael A. Baumholtz Plastic Surgery Consultant, Shriners Hospital for

Children, Philadelphia, PA, USA

Adjunct Faculty, Division of Plastic Surgery UTHSCSA, San Antonio,

TX, USA

Private Practice, San Antonio Cosmetic Surgery, San Antonio, TX, USA

Ahmed Bazzi Pediatric Orthopedic Surgery, Childrens Hospital of

Michigan, Detroit, MI, USA

xxiii
xxiv Contributors

Michael S. Bednar Department of Orthopaedic Surgery and Rehabilitation,

Stritch School of Medicine, Loyola University, Maywood, IL, USA
Allan Belzberg Department of Neurosurgery, The Johns Hopkins Hospital,
Baltimore, MD, USA
Diana X. Bharucha-Goebel Neurology, Childrens National Medical
Center & National Institutes of Health, NINDS, Bethesda, MD, USA
Allen T. Bishop Department of Orthopedic Surgery, Division of Hand
Surgery, Mayo Clinic, Rochester, MN, USA
Carsten G. Bnnemann National Institutes of Health, NINDS, Bethesda,
MD, USA
Glendaliz Bosques Childrens Memorial Hermann Hospital, The University
of Texas Health Science Center at Houston (UTHealth) Medical School,
Houston, TX, USA
Ronald Burgess Hand and Upper Extremity Surgery, Shriners Hospital for
Children-Lexington, Lexington, KY, USA
Charity S. Burke Louisville Arm and Hand, Norton Orthopaedic Care,
Louisville, KY, USA
Daniel Calva The Curtis National Hand Center, MedStar Union Memorial
Hospital, Baltimore, MD, USA
Mark A. Cappel Department of Dermatology, Mayo Clinic, Jacksonville,
FL, USA
Robert B. Carrigan Division of Orthopaedic Surgery, Childrens Hospital of
Philadelphia, Perelman School of Medicine at the University of Pennsylvania,
Philadelphia, PA, USA
Shannon Cassel Department of Orthopaedics and Rehabilitation, University
of Iowa Hospitals and Clinics, Iowa City, IA, USA
Charles Chan Department of Orthopaedic Surgery, Columbia University,
New York, NY, USA
Sam Chen University of California, San Francisco, San Francisco, CA, USA
Yen Hsun Chen Hospital for Special Surgery, New York, NY, USA
Vidya Chidambaran Department of Anesthesiology, Division of Pain
Management, Cincinnati Childrens Hospital Medical Center, University of
Cincinnati, Cincinnati, OH, USA
Harvey Chim Department of Orthopedic Surgery, Division of Hand Surgery,
Mayo Clinic, Rochester, MN, USA
Alphonsus K. S. Chong Department of Hand and Reconstructive Microsur-
gery, National University Hospital, Singapore
Department of Orthopaedic Surgery, Yong Loo Lin School of Medicine,
National University of Singapore, Singapore
Contributors xxv

Benjamin Christian Private Practice, Baltimore, MD, USA

Howard M. Clarke The Hospital for Sick Children and the Department of
Surgery, University of Toronto, Toronto, ON, Canada
Roger Cornwall Cincinnati Childrens Hospital, Cincinnati, OH, USA
Andrew J. Costandi Department of Anesthesiology, Division of Pain
Management, Cincinnati Childrens Hospital Medical Center, University of
Cincinnati, Cincinnati, OH, USA
Aaron Daluiski Hospital for Special Surgery, New York, NY, USA
Casey M. de Deugd University of Central Florida College of Medicine,
Orlando, FL, USA
Carolien P. de Roode Division of Orthopaedic Surgery Childrens Bone
and Spine Surgery, University of Nevada School of Medicine, Las Vegas,
NV, USA
Jenny M. Dorich Division of Occupational Therapy and Physical Therapy,
Cincinnati Childrens Hospital Medical Center, Cincinnati, OH, USA
Reid Draeger Hand Surgery Specialists, Inc, Cincinnati, OH, USA
Susan Duff Department of Physical Therapy, Thomas Jefferson University,
Philadelphia, PA, USA
W. Andrew Eglseder University of Maryland School of Medicine,
Baltimore, MD, USA
Patrick Emerson UPMC Hamot Medical Center, Erie, PA, USA
Timothy Estilow The Childrens Hospital of Philadelphia, Philadelphia,
PA, USA
Marybeth Ezaki Department of Hand Surgery, Texas Scottish Rite Hospital
for Children, Dallas, TX, USA
Eric N. Faerber Department of Radiology, St. Christophers Hospital for
Children, Philadelphia, PA, USA
Ross Feller Department of Orthopedic Surgery, Brown University, Rhode
Island Hospital, Providence, RI, USA
Felicity Fishman Yale Orthopaedics and Rehabilitation, New Haven, CT, USA
Rosalyn P. Flores Department of Orthopaedic Surgery, National University
Hospital, Singapore
John Flynn Department of Orthopaedic Surgery, Childrens Hospital of
Philadelphia, Philadelphia, PA, USA
Krister Freese Division of Orthopaedic Surgery, University of Hawaii,
Honolulu, HI, USA
Steven L. Frick Department of Orthopaedic Surgery, University of Central
Florida College of Medicine, Nemours Childrens Hospital, Orlando, FL, USA
xxvi Contributors

Theodore J. Ganley Orthopaedic Surgery, The Childrens Hospital of

Philadelphia, Philadelphia, PA, USA
Lorenzo Garagnani Department of Hand Surgery and Microsurgery,
University Hospital Policlinico di Modena, Modena, Italy
David S. Geller Orthopaedic Oncology Service, Monteore Medical Center,
Albert Einstein College of Medicine, Bronx, NY, USA
Evan Geller Department of Radiology, St. Christophers Hospital for
Children, Philadelphia, PA, USA
Gloria Gogola Shriners Hospital for Children, Houston, TX, USA
Charles A. Goldfarb Department of Orthopaedic Surgery, Washington
University School of Medicine, Saint Louis, MO, USA
Donald P. Goldsmith Pediatrics, Drexel University College of Medicine,
Philadelphia, PA, USA
Rachel Y. Goldstein Keck School of Medicine, Childrens Orthopaedic
Center, Childrens Hospital Los Angeles and University of Southern California,
Los Angeles, CA, USA
Christine M. Goodbody The Childrens Hospital of Philadelphia,
Philadelphia, PA, USA
Carol L. Greene University of Maryland School of Medicine, Baltimore,
MD, USA
Lorelei Grunwaldt Division of Pediatric Plastic Surgery, Childrens Hospital
of Pittsburgh of UPMC, Cleft-Craniofacial Center, Pittsburgh, PA, USA
Amit Gupta Department of Orthopaedic Surgery, University of Louisville,
Louisville, KY, USA
Warren C. Hammert Department of Orthopaedic Surgery, University of
Rochester Medical Center, Rochester, NY, USA
Edward J. Harvey Montreal General Hospital, Montreal, QC, Canada
Ann E. Van Heest Department of Orthopaedic Surgery, University of
Minnesota, Minneapolis, MN, USA
William Hennrikus Department of Orthopaedic Surgery, Bone and Joint
Institute, Penn State College of Medicine, Hershey, PA, USA
Martin J. Herman Department of Orthopaedic Surgery, Drexel University
College of Medicine, St. Christophers Hospital for Children, Philadelphia,
PA, USA
James P. Higgins The Curtis National Hand Center, MedStar Union
Memorial Hospital, Baltimore, MD, USA
B. David Horn Perelman School of Medicine, The Childrens Hospital of
Philadelphia, University of Pennsylvania, Philadelphia, PA, USA
Contributors xxvii

Deborah Humpl The Childrens Hospital of Philadelphia, Philadelphia,

PA, USA
Douglas T. Hutchinson Department of Orthopaedic Surgery, University of
Utah School of Medicine, Salt Lake City, UT, USA
Joshua E. Hyman Department of Orthopaedic Surgery, Columbia
University, College of Physicians and Surgeons, New York, NY, USA
Jonathan Isaacs Department of Orthopaedic Surgery, Division of Hand
Surgery, Virginia Commonwealth University Health Systems, Richmond,
VA, USA
Sidney M. Jacoby The Philadelphia Hand Center, Philadelphia, PA, USA
Michelle James Shriners Hospitals for Children, Sacramento, CA, USA
Neil Ford Jones University of California Irvine, Irvine and Shriners
Hospitals, Los Angels, CA, USA
JoAnne L. Kanas Shriners Hospitals for Children, Tampa, FL, USA
Julie D. Kaplan University of Maryland School of Medicine, Baltimore,
MD, USA
Julia Katarincic Department of Orthopedic Surgery, Brown University,
Rhode Island Hospital, Providence, RI, USA
Ryan Katz Union Memorial Hospital, The Curtis National Hand Center,
Baltimore, MD, USA
Anjan P. Kaushik Department of Orthopaedic Surgery, University of
Rochester Medical Center, Rochester, NY, USA
Augusta Kluk Department of Orthopedic Surgery, Brown University, Rhode
Island Hospital, Providence, RI, USA
Polly Kochan Department of Radiology, St. Christophers Hospital for
Children, Philadelphia, PA, USA
L. Andrew Koman Department of Orthopaedic Surgery, Wake Forest
School of Medicine, Winston-Salem, NC, USA
Scott H. Kozin Shriners Hospitals for Children, Philadelphia, PA, USA
Bret Kricun Lehigh Valley Diagnostic Imaging, Lehigh Valley Health
Network, Allentown, PA, USA
Leo Kroonen Department of Orthopaedic Surgery, Naval Medical Center
San Diego, San Diego, CA, USA
Michael S. Kwon Department of Orthopaedic Surgery, Drexel University
College of Medicine, St. Christophers Hospital for Children, Philadelphia,
PA, USA
Lisa L. Lattanza Department of Orthopaedic Surgery, University of
California, San Francisco, CA, USA
xxviii Contributors

Eng Hin Lee Department of Orthopaedic Surgery, Yong Loo Lin School of
Medicine, National University of Singapore, Singapore
Division of Paediatric Orthopaedics, National University Hospital, Singapore
R. Jay Lee The Childrens Hospital of Philadelphia, Philadelphia, PA, USA
Steve K. Lee Hospital for Special Surgery, New York, NY, USA
Arabella Leet Department of Orthopaedic Surgery, Shriners Hospital for
Children-Honolulu, Honolulu, HI, USA
Fraser J. Leversedge Department of Orthopaedic Surgery, Duke University
Medical Center, Durham, NC, USA
Nina Lightdale-Miric Department Orthopaedics, Childrens Hospital of
Los Angeles, Los Angeles, CA, USA
Kevin J. Little University of Cincinnati Department of Orthopaedic Surgery,
Hand and Upper Extremity Surgery, Cincinnati Childrens Hospital Medical
Center, Cincinnati, OH, USA
Heather Lochner Department of Orthopaedic Surgery, Hand Surgery
Division, Johns Hopkins University, Baltimore, MD, USA
Bryan J. Loefer OrthoCarolina, Charlotte, NC, USA
John Lubahn Hand, Microsurgery and Reconstructive Orthopedics, Erie,
PA, USA
Archana Malik Department of Radiology, St. Christophers Hospital for
Children, Philadelphia, PA, USA
M. Claire Manske Department of Orthopaedic Surgery, Washington
University School of Medicine, Saint Louis, MO, USA
Charles T. Mehlman Cincinnati Childrens Hospital Medical Center
Brachial Plexus Center, Cincinnati, OH, USA
Raymond Metz UPMC Hamot Medical Center, Erie, PA, USA
Core Orthopedics and Sports Medicine, Elk Grove Village, IL, USA
Steven L. Moran Division of Plastic Surgery and Department of Orthopedic
Surgery, Mayo Clinic, Rochester, MN, USA
Shiners Hospital for Children, Twin Cities, MN, USA
M. J. Mulcahey Jefferson School of Health Professions, Thomas Jefferson
University, Philadelphia, PA, USA
Afamefuna Nduaguba Childrens Hospital of Philadelphia, Philadelphia,
PA, USA
Scott N. Oishi Department of Hand Surgery, Texas Scottish Rite Hospital for
Children, Dallas, TX, USA
A. Lee Osterman Philadelphia Hand Center, King of Prussia, PA, USA
Contributors xxix

Meredith Osterman Orthopedic Hand Surgery, Mary S. Stern Hand

Fellowship, Cincinnati, OH, USA
Jean Ouellet Shriners Hospital for Children, Montreal, QC, Canada
James Lee Pace Keck School of Medicine, Childrens Orthopaedic Center,
Childrens Hospital Los Angeles and University of Southern California,
Los Angeles, CA, USA
Min Jung Park Southern California Permanente Medical Group, CA, USA
Allan Peljovich The Pediatric Hand and Upper Extremity, Center of Georgia,
Atlanta, GA, USA
Raymond Pensy University of Maryland School of Medicine, Baltimore,
MD, USA
Kelly Pham Johns Hopkins University, Baltimore, MD, USA
Gregory Pinkowsky Penn State College of Medicine, Hershey, PA, USA
Erica D. Poletto Department of Radiology, St. Christophers Hospital for
Children, Philadelphia, PA, USA
Anish G. R. Potty The Childrens Hospital of Philadelphia, Philadelphia, PA,
USA
Mark E. Puhaindran Department of Hand and Reconstructive Microsur-
gery, National University Hospital, Singapore
Rey Ramirez Hand, Microsurgery and Reconstructive Orthopedics, Erie,
PA, USA
Joshua A. Ratner Hand and Upper Extremity Center of Georgia, Childrens
Healthcare of Atlanta, Scottish Rite Campus, Atlanta, GA, USA
Scott A. Riley Hand and Upper Extremity Surgery, Shriners Hospital for
Children-Lexington, Lexington, KY, USA
Neil Saran Shriners Hospital for Children, Montreal, QC, Canada
Susan Scherl Orthopedic Surgery, The University of Nebraska, Omaha,
NE, USA
Joseph J. Schreiber Hospital for Special Surgery, New York, NY, USA
Brandon S. Schwartz University of Maryland School of Medicine,
Baltimore, MD, USA
Richard M. Schwend Division of Orthopaedics, Childrens Mercy Hospital,
Kansas City, MO, USA
Apurva S. Shah Department of Orthopaedics and Rehabilitation, University
of Iowa Hospitals and Clinics, Iowa City, IA, USA
Brett Shannon Johns Hopkins University School of Medicine, Baltimore,
MD, USA
xxx Contributors

Frances Sharpe Keck School of Medicine, Department of Orthopedics,

Los Angeles County Medical Center, University of Southern California,
Los Angeles, CA, USA
Alexander Y. Shin Department of Orthopedic Surgery, Division of Hand
Surgery, Mayo Clinic, Rochester, MN, USA
Eon K. Shin The Philadelphia Hand Center, Philadelphia, PA, USA
Carrissa Shotwell Division of Occupational Therapy and Physical Therapy,
Cincinnati Childrens Hospital Medical Center, Cincinnati, OH, USA
Robert L. Siegle Department of Radiology, St. Christophers Hospital for
Children, Philadelphia, PA, USA
David L. Skaggs Keck School of Medicine, Childrens Orthopaedic Center,
Childrens Hospital Los Angeles and University of Southern California,
Los Angeles, CA, USA
Gillian D. Smith Department of Plastic and Reconstructive Surgery,
Great Ormond Street Hospital, London, UK
Mark P. Solomon Plastic Surgery Consultant, Shriners Hospital for
Children, Philadelphia, PA, USA
Private Practice 191 Presidential Blvd, Suite LN24, Bala Cynwyd, PA, USA
Alexander Spiess Department of Plastic Surgery, University of Pittsburgh
Medical Center, Pittsburgh, PA, USA
Robert J. Spinner Department of Orthopedic Surgery, Division of Hand
Surgery, Mayo Clinic, Rochester, MN, USA
Paul Sponseller Kennedy Krieger Institute, Orthopedic Surgery, Johns
Hopkins Hospital, Baltimore, MD, USA
Harlan M. Starr The Curtis National Hand Center, MedStar Union
Memorial Hospital, Baltimore, MD, USA
Milan Stevanovic Keck School of Medicine, Department of Orthopedics,
Los Angeles County Medical Center, University of Southern California,
Los Angeles, CA, USA
Jeffrey A. Stromberg Department of General Surgery, Virginia Common-
wealth University Health Systems, Richmond, VA, USA
Marc C. Swan Nufeld Department of Surgical Sciences, University of
Oxford, Oxford University Hospitals NHS Trust, John Radcliffe Hospital,
Headington, Oxford, UK
Brandon M. Tauberg Drexel University College of Medicine, Philadelphia,
PA, USA
Sasha Job Tharakan Department of Surgery, Childrens Hospital of
Philadelphia, Philadelphia, PA, USA
Philip To Hand Surgery Specialists, Inc, Cincinnati, OH, USA
Contributors xxxi

Andrew R. Tyser Department of Orthopaedic Surgery, University of Utah,

Salt Lake City, UT, USA
Joseph Upton Department of Plastic Surgery, Childrens Hospital, Boston,
MA, USA
Jacqueline A. Urbine Department of Radiology, St. Christophers Hospital
for Children, Philadelphia, PA, USA
Angela Wang Department of Orthopaedic Surgery, University of Utah,
Salt Lake City, UT, USA
Lesley Wheeler Department of Hand Surgery, Texas Scottish Rite Hospital
for Children, Dallas, TX, USA
Johnathan J. Whitaker Department of Orthopaedic Surgery, Philadelphia
College of Osteopathic Medicine, Philadelphia, PA, USA
Katherine J. Willard Department of Dermatology, Mayo Clinic, Jackson-
ville, FL, USA
Gerald R. Williams Department of Orthopaedic Surgery, Thomas Jefferson
University, Rothman Institute at Jefferson, Philadelphia, PA, USA
Theresa O. Wyrick Department of Orthopaedic Surgery, Arkansas
Childrens Hospital, University of Arkansas for Medical Sciences, Little
Rock, AR, USA
Jeffrey Yao Department of Orthopaedic Surgery, Stanford University
Medical Center, Redwood City, CA, USA
Dan A. Zlotolow Shriners Hospitals for Children, Philadelphia, PA, USA
 Part I
Development
 Embryology
1
Yen Hsun Chen and Aaron Daluiski

Contents Abstract
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4 Developmental biology has greatly contributed
to the understanding of upper limb develop-
Molecular Events of the Developing
Upper Limb . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4
ment. Whereas early understanding of limb
development centered on morphological
First Phase: Early Development change during organogenesis, current empha-
and Limb Identity . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7
sis is on discovery of molecular signaling
Second Phase: Limb Patterning mechanisms that drive the remarkable transfor-
and Initial Growth . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 8
Proximodistal (PD) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 8
mation of single cells into fully functioning
Anteroposterior (AP) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 10 limbs and the human body. These discoveries
Dorsoventral (DV) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 13 have laid a foundation for fundamental
Coordination Between Axes . . . . . . . . . . . . . . . . . . . . . . . . . . 15 embryology-based concepts that have
Third Phase: Tissue Differentiation . . . . . . . . . . . . . . . . 19 reshaped the way congenital limb differences
Vascular System . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 19 are conceptualized, with the ability to trace a
Nervous System . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 19
phenotype back to single genes, and, con-
Musculoskeletal System . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 20
versely, the ability to predict developmental
Extrinsic Factors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 20 differences from single-gene mutations. Not
International Federation of Societies for Surgery only do these discoveries advance understand-
of the Hand (IFSSH) Classication System . . . . . . . . 20 ing of limb development, but clinical benets
Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 21 are also realized. Clinicians are provided with
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 21
the information they need to adequately inform
patients and their families about the nature of
limb differences, the hereditary implications,
and the downstream developmental needs and
challenges that the patient may face. Pediatric
upper limb surgeons, standing at the interface
between clinical care and genetic research,
play a unique role in this eld. Through recog-
nition of novel human variants, pediatric upper
limb surgeons act as gatekeepers by referring
patients for appropriate work-up, facilitating
Y.H. Chen (*) A. Daluiski
Hospital for Special Surgery, New York, NY, USA research that offers novel insights into human
e-mail: chenye@hss.edu; daluiskia@hss.edu limb development. The goal of the discussion
# Springer Science+Business Media New York 2015 3
J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_1
4 Y.H. Chen and A. Daluiski
that follows is to provide the pediatric upper
limb surgeon with the fundamentals of limb
embryology that have implications both clini-
cally and academically.
Introduction
Congenital birth defects affect 3% of all live births
in the United States, with upper limb differences
occurring at a rate of approximately 1 per every
3,000 live births (Parker et al. 2010). Develop-
ment of the upper limb can be described in terms
of the anatomic changes that occur during
embryonic growth or in terms of the molecular
cues that cause the developmental processes. The
basics of both are important for the upper limb
surgeon to understand to properly evaluate
congenital limb anomalies that present clinically.
It is particularly important to recognize genetic
defects as they may have wider clinical implica-
tions for the patient. Conversely, the treating
upper limb surgeon may be the rst to identify
new clinical presentations that may in turn
advance the understanding of embryonic limb
development.
Normal limb development begins with the
appearance of the upper limb bud as early as
postconception day 24 and attains all the major
structures of an adult by the end of week 8, the end
of the embryonic period (ORahilly and Gardner
1975). An overview of the milestones of human
upper limb development is described in Table 1,
with select milestones for development of the
lower limb for comparison.
The upper limb develops proximal to distal,
starting from the trunk, and begins initially as a
homogenous mass of undifferentiated mesenchy-
mal cells. During limb outgrowth, musculoskele-
tal elements generally precede development of
other elements such as nerves, vasculature, and
lymphatics. Three distinct segments are identi-
able in both the developing and mature limb: the
stylopod (upper arm), zeugopod (forearm), and
autopod (hand plate) (Fig. 1). Development of
the three segments occurs both sequentially and
concurrently. That is, the most proximal structures
tend to be at a slightly more mature developmental
stage than the more distal structures due to earlier
onset of formation.
A host of genes and molecular signaling work
harmoniously together to ensure proper develop-
ment of these structures. Much of these mecha-
nisms and pathways remain to be worked out, but
there is sufcient knowledge to be able to charac-
terize defects in the context of basic embryologi-
cal events that dene the complex, coordinated
process of limb development. Whereas early
developmental work focused on the morphology
of these embryological events, the emphasis is
now placed on understanding the molecular
basis of these changes, enabling detailed under-
standing of genotype-phenotype correlations that
may have substantial clinical implications.
In the discussion that follows, the gene name
nomenclature will be followed in which human
genes are designated by having all letters in upper-
case (e.g., SHH for Sonic Hedgehog) and their
animal counterparts have only the rst letter in
uppercase (e.g., Shh). While the two counterparts
may be considered interchangeable, this distinc-
tion serves as a reminder that not all molecular
mechanisms for limb development may be con-
served between humans and the mouse, chick, or
other model species.
Molecular Events of the Developing
Upper Limb
Molecular signaling pathways control the growth
and tissue differentiation, leading to the gross
anatomic milestones of limb development. These
molecular events can be divided into three phases.
The rst phase is early limb development, which
includes initial establishment of limb identity and
the initiation of limb bud outgrowth. The second
phase is generally considered classical limb
development, characterized by basic patterning
of the developing limb. During this phase, limb
patterning is commonly subdivided into three spa-
tial axes: proximodistal, anteroposterior
(radioulnar), and dorsoventral. The third phase is
characterized by growth to increase limb size and
cellular differentiation to form discrete tissues that
make up the individual structures of the limb.
1 Embryology 5

Table 1 Key milestones in the development of the human upper limb (ORahilly and Gardner 1975). Select milestones
for the lower limb are provided for comparison. AER apical ectodermal ridge
Carnegie
Week Day stage Upper limb Lower limb
4 24 11 Swelling appears in region of upper limb bud
28 13 Scattered blood vessels Appearance of lower limb bud
5 32 14 Upper limb AER
Early marginal vessel
Early brachial plexus development
33 15 Hand plate appears Lower limb AER
Humerus mesenchymal condensations
6 37 16 Humerus chondrication Lumbosacral plexus
Radius and ulna mesenchymal
condensations
Brachial plexus with radial, median, and
ulnar nerves to the elbow
Early muscle masses
41 17 Finger rays (webbed) Femur, tibia, bula, and tarsus
Radius, ulna, and metacarpal chondrication mesenchymal condensations
7 44 18 Interdigital apoptosis Femur, tibia, and bula chondrication
Scapula and humeral head chondrication
Carpals and proximal phalanges
chondrication
Trapezius innervated (accessory nerve)
Major muscles distinguishable
48 19 Middle phalanges chondrication
Shoulder and elbow interzones (joint cavity
formation)
8 51 20 Distal phalanges chondrication
52 21 Humerus ossication
Radius ossication
All muscles distinguishable
Wrist and carpal interzones
54 22 Ulna ossication Femur and tibia ossication
9 57 23 Scapula ossication Fibula ossication
Intramembranous ossication of distal tip of Tarsus and digits chondrication
distal phalanges

These phases are continuous and overlapping.
Early events initiate signaling processes that
trigger establishment of proper limb patterning.
Appropriately patterned groups of cells subse-
quently undergo expansion and differentiation to
form specic limb structures and tissues. While it
is more practical to consider each of these pro-
cesses as distinct steps, these processes overlap in
time and space, are dynamic, and are often
interdependent through the cross talk of different
signaling pathways. Disruption in any number of
these events produces many of the congenital
abnormalities seen clinically.
Several key tenets apply:
1. Tissues and structures that develop concurrently
may be driven by common molecular signaling
pathways, although not always. Defects in com-
mon pathways may explain constellations of
symptoms that are frequently seen together.
2. Many of the molecular mechanisms identied
are derived from experimental work performed
6 Y.H. Chen and A. Daluiski

Fig. 1 Anatomy of the

developing limb. The
development of proximal
structures precedes the
development of more distal
structures due to earlier
onset of formation (Zeller
et al. 2009)

Table 2 Terminology for etiology-based description of congenital birth defects

Terminology Denition
Sequence A set of defects in which the steps involved in pathogenesis to produce a distinctive phenotype are
known, e.g., Potter sequence
Syndrome Recurring pattern or constellation of defects, commonly due to genetic defect
Association Defects that occur together more commonly than would be expected by chance
Disruption/ External forces damages a normal developmental process
deformation
Malformation Genetic or developmental abnormality
Dysplasia Normal genetic programming, but aberrant tissue development

in nonhuman species, especially the chicken
and the mouse. These ndings may or may
not be conserved in human limb development.
3. Many molecular pathways are critical for
development of other organ systems. Severe
defects in these pathways may not be encoun-
tered clinically due to the failure of develop-
ment of major organs, resulting in a nonviable
fetus. Conversely, milder defects may be
encountered clinically, but necessitate screen-
ing for dysfunction in these other organ
systems.
4. Not all congenital limb differences are directly
caused by changes in molecular signaling path-
ways. Amniotic band syndrome, for example,
results from mechanical insults to normally
developing tissue in utero.
5. Specic terminology is used to distinguish
clinical phenotypes due to genetic defects
from nongenetic insults. This terminology is
dened in Table 2.
For the purposes of this discussion, an exhaus-
tive review of all the molecular events contributing
to the developing limb is beyond the scope of this
chapter or the need of the pediatric upper limb
surgeon. Molecular events presented in this review
will be key signals that play a major role in limb
development and/or are clinically relevant. Two
things are important for the pediatric upper limb
surgeon: (1) the recognition of associated patterns
of deformities and (2) the recognition of novel
abnormalities or unique variants that are inherit-
able. The former will help with diagnosis of comor-
bid conditions that may help our patients, whereas
the latter will help our scientist colleagues who
continue deciphering the molecular mechanisms
underlying the deformities that afict our patients.
1 Embryology
First Phase: Early Development
and Limb Identity
Limb buds initiate in the region of the developing
somites and rst appear in the region of the upper
limb on approximately day 24. This is followed
4 days later with the appearance of the lower limb
buds on day 28.
Upper and lower limb bud identities are
believed to be predetermined early on during
cranial-caudal patterning by a conserved, sequen-
tial genetic program encoded by Hox genes (Cohn
et al. 1997). Differential Hox gene expression
establishes the upper limb- and lower limb-
forming regions of the corresponding lateral
plate mesoderm and somites from which the
limb buds initiate.
These limb-forming regions dictate the mor-
phology of the limb created in this region. Ectopic
induction of the lateral plate mesoderm using
Fgf-soaked beads in either region results in for-
mation of complete upper or lower limbs
depending on where the limb was initiated
(Cohn et al. 1995). Determination of whether the
limb develops upper or lower limb structures was
correlated with proximity of bead placement to
the limb-forming regions. Beads placed close to
the lower limb-forming region produced ectopic
lower limbs, whereas beads placed close to the
upper limb-forming region induced formation of
ectopic upper limbs (Cohn et al. 1995, 1997).
Beads placed in between the two regions pro-
duced chimeric limbs with both upper and lower
limb characteristics (Ohuchi et al. 1998).
A triggering signal is required to initiate limb
bud outgrowth from the programmed limb bud
forming regions of the embryo. The exact
sequence of events leading to activation of growth
remains unclear, but at least three transcription
factors are critical for initiation of the correct
upper or lower limb bud development: Tbx4,
Tbx5, and Pitx1 (Agarwal et al. 2003; Duboc
and Logan 2011a; Rallis et al. 2003). Tbx5 is
specically expressed in the upper limb bud,
whereas Pitx1 and Tbx4 (downstream of Pitx1)
are specically expressed in the lower limb bud.
The expression of these genes is quite specic and
7
clearly identies the limb based on this pattern in
both mouse and chick embryos. Further
supporting the specicity of Tbx4, Tbx5, and
Pitx1, Fgf-soaked bead-induced ectopic upper
limbs express Tbx5 whereas ectopic lower limbs
express Tbx4 and Pitx1. Limb buds with a mixture
of upper and lower limb characteristics express all
three. In the upper limb, retinoic acid from the
trunk plays a permissive role in limb bud initiation
by permitting induction of Tbx5 expression
(Cunningham et al. 2013).
Functionally, Pitx1 is clearly a determinant of
lower limb morphology whereas the exact contri-
bution of Tbx4 and Tbx5 to the development of
the corresponding limb morphology is less clear.
Loss of Pitx1 results in loss of lower limb charac-
teristics, which can be rescued by Tbx4 since
Tbx4 is downstream of Pitx1 signaling (Ouimette
et al. 2010). Misexpression of Pitx1 in the upper
limb causes a partial forelimb-to-hind limb trans-
formation in mice and humans (Liebenberg syn-
drome, Mendelian Inheritance in Man [MIM]
number 186550) (Spielmann et al. 2012),
reecting the role of Pitx1 in directing develop-
ment of lower limb structures. Conversely, ectopic
expression of Tbx4 in the upper limb does not
produce the same effect. In fact, Tbx4
misexpression in the upper limb bud can substi-
tute for Tbx5 in a conditional knockout mouse
with Tbx5 deleted in the upper limb-forming
region to form an intact upper limb (Minguillon
et al. 2005). The Tbx5 conditional knockout
mouse also indicated that Tbx5 (or misexpressed
Tbx4) is needed for limb bud initiation, the
absence of which results in absent upper limbs
(Rallis et al. 2003).
Mutations to all three genes are associated with
clinical syndromes in humans. Tbx4 mutations
cause small patella syndrome (MIM 147891),
characterized by patellar and hip defects. Tbx5
mutations can cause Holt-Oram syndrome (MIM
142900), characterized by upper limb and cardiac
defects. Pitx1 mutations can cause clubfoot (MIM
199800) or Liebenberg syndrome (MIM 186550).
A key milestone for limb bud initiation by
Tbx5 (upper limb) and Tbx4 (lower limb) is the
induction of Fgf10 expression. Maintained Fgf10
expression is required for successful completion
8 Y.H. Chen and A. Daluiski
of limb bud initiation and subsequent limb devel-
opment (Duboc and Logan 2011b). In the upper
limb, Tbx5 is a direct activator of Fgf10 signaling
(Agarwal et al. 2003). In the lower limb, some
Fgf10 expression persists despite loss of Tbx4,
likely due to other contributors. This overlap in
signaling may explain the formed but signicantly
smaller lower limbs in mice with loss of Tbx4
(Naiche and Papaioannou 2003). In mice, com-
plete loss of Fgf10 resulted in initiated limb buds
but no limb outgrowth (Sekine et al. 1999). Once
initiated, similar Fgf-dependent signaling mecha-
nisms enable limb outgrowth for both upper and
lower limbs.
Second Phase: Limb Patterning
and Initial Growth
After limb bud initiation, outgrowth of the limb
bud begins. All limb buds consist of mesodermal
tissue originating from lateral plate mesoderm
(forms bone, cartilage, and tendons) and somitic
mesoderm (forms muscles, nerves, and vascula-
ture) and are covered by a layer of ectoderm.
Anatomically, the limb goes through a progressive
set of morphological changes starting with a thin
nubbin of tissue along the chest wall of the
embryo through the limb paddle stages. During
this process, limb development can be thought of
as occurring in three spatially distinct axes. Each
of these limb growth axes contains a signaling
center, an area or group of cells, that is responsible
for establishing the corresponding axis. The three
axes are proximodistal (PD), anteroposterior
(AP) (radioulnar or pre-/postaxial), and dorsoven-
tral (DV).
Proximodistal (PD)
At the distal tip of the developing limb buds, at the
interface between dorsal and ventral ectoderm, a
ridge of thickened ectoderm forms in response to
signals from the underlying mesoderm. This
thickening develops into the apical ectodermal
ridge (AER), the signaling center for the PD axis
(Fig. 2). Signaling from the AER is critical for
Fig. 2 Whole-mount RNA in situ hybridization visualiza-
tion the AER at the tip of a developing mouse limb bud
(Soshnikova and Birchmeier 2006)
limb bud outgrowth and elongation, directly
inducing formation of upper limb structures in a
proximal (early) to distal (late) manner. These
structures may be viewed as three distinct seg-
ments achieved by patterning along the
proximodistal axis: the stylopod (upper arm),
zeugopod (forearm), and autopod (wrist and
hand). This PD patterning appears to occur very
early when the AER is established in the initiating
limb bud and is dependent on FGF signaling from
the AER (Mariani et al. 2008; Sun et al. 2002).
The importance of the AER is demonstrated by
limb differences along the PD axis if AER func-
tion is impaired. Disruption results in limb trun-
cations at a level corresponding with the stage of
development when the disruption occurred
(Summerbell 1974). The later the removal or dis-
ruption of the apical ectodermal ridge in develop-
ment, the more distal the resulting truncation.
Structures proximal to the level of truncation
remain intact. These truncations can be rescued
by grafting an AER from a different chick
embryo, dening the AER as both necessary and
sufcient to promote outgrowth.
The molecular cue from the AER was isolated
and identied to be one of several growth factors
from the broblast growth factor (Fgf) family.
1 Embryology
In the developing limb, members of the Fgf family
exhibit varying degrees of functional redundancy.
Of the Fgfs, Fgf10 alone is both necessary and
sufcient to produce an intact limb (Duboc and
Logan 2011b) and is normally expressed by the
mesenchyme underlying the AER. The AER itself
specically expresses Fgf4, Fgf8, Fgf9, and Fgf17
(the AER-FGFs). Mice decient in Fgf4, Fgf9, or
Fgf17 retain normal limb development, likely res-
cued by the functional redundancy of the Fgfs. In
contrast, Fgf8 is critical as loss of Fgf8 caused
impaired limb outgrowth and signicantly smaller
limbs (Mariani et al. 2008).
Fgf10 from the sub-AER mesenchyme and
Fgf8 from the AER are intrinsically related in a
positive feedback loop that forms the core signal-
ing required for growth along the PD axis (Ohuchi
et al. 1997). Early expression of Tbx5 during
upper limb bud initiation rst induces expression
of Fgf10 from the sub-AER mesenchyme
(Agarwal et al. 2003). Fgf10 signals to the over-
lying AER to express Wnt3a, which in turn drives
Fgf8 expression. Fgf8 from the AER then signals
to the sub-AER mesenchyme to maintain Fgf10
expression from the underlying mesenchyme,
resulting in an Fgf8-Fgf10 positive feedback
loop located at the distal end of the developing
limb. This Fgf/Wnt signaling loop is critical for
limb development loss of Wnt3 (tetra-amelia,
MIM 273395) (Niemann et al. 2004) or Fgf10
(Sekine et al. 1999) results in amelia, or absence
of limb formation.
The importance of the distal end of the limb
bud in limb outgrowth was initially conceptual-
ized in a progress zone model to explain
proximodistal development. The progress zone
model posits that there is a zone of undiffer-
entiated mesenchymal cells within the sub-AER
mesenchyme with an intrinsic timing mechanism.
The cells in this zone are maintained by Wnt3a
and Fgf8 from the AER, which maintains a pool
of progenitor cells by stimulating proliferation
and inhibiting differentiation (ten Berge
et al. 2008). A timing mechanism would provide
progenitor cells with positional cues based on how
long the cells reside in the progress zone. Mesen-
chymal stem cells that exit relatively early
develop into proximal structures such as the
9
humerus. Cells that exit late end up in distal loca-
tions as the limb elongates and develop into distal
structures such as the forearm and hand. The time-
dependent mechanism of the progress zone model
provided a mechanistic explanation for the time-
dependent transverse decit phenotype produced
by removal of the AER. The later the AER exci-
sion, the more distal the defects due to loss of
progenitors with longer residence in the
progress zone.
Subsequent experiments, however, demon-
strated that the progress zone model is inaccurate.
Lineage tracing in X-irradiation experiments to
induce phocomelia in chick embryos demon-
strated that proximodistal patterning was unaf-
fected despite radiation-induced defects in
proximal structures (Galloway et al. 2009). Simi-
larly, fate mapping in chick limb buds demon-
strated that proximodistal cell fates were
established early, with limb truncations occurring
due to apoptosis of these fated cells rather than
defects in PD patterning (Dudley et al. 2002).
Modern attempts to understand PD patterning
emphasizes the conceptualization of limb pattern-
ing in the context of dynamic interactions between
molecular events (Tabin and Wolpert 2007). More
recently, a two-signal model was proposed to
explain proximodistal determination of limb
structures. Specically, the two opposing signals
were retinoic acid (RA) for induction of proximal
structures, with the FGFs from the AER determin-
ing formation of distal structures (Cooper
et al. 2011; Rosell-Dez et al. 2011). Supporting
this model, ectopic introduction of RA to the
distal limb bud resulted in proximalization of the
distal limb (Mercader et al. 2000). Additionally,
the AER-FGFs were demonstrated to establish
distal structures by repression of Meis1/2, homeo-
box genes that establish the proximal limb
(Mariani et al. 2008).
The role of retinoic acid appears to be permis-
sive rather than actively establishing PD pattern-
ing (Cunningham et al. 2013). Fgf8 expression by
the developing heart suppresses limb bud initia-
tion due to inhibition of Tbx5 expression.
Retinoic acid from the trunk functions to block
cardiac Fgf8, enabling limb bud expression of
Tbx5 and Meis1/2 and limb bud initiation. For
10
Fig. 3 In situ hybridization
for SHH, visualizing the
SHH-expressing ZPA
located in the posterior
margin of both the upper
and lower limb buds in a
mouse embryo (e10.5)
(Daluiski et al. 2001)
proximodistal patterning itself, RA is unneces-
sary. Interestingly, Tbx5 itself is needed for
cardiomyocyte differentiation (Holt-Oram syn-
drome, MIM 142900), the mutation of which is
characterized by upper limb and cardiac defects.
PD patterning remains incompletely under-
stood and is an evolving eld. It is also not
known the extent to which these mechanisms are
conserved in human limb development.
Despite our lack of understanding, the clinical
implications have not changed. Defects along the
proximodistal axis, such as limb truncations and
longitudinal defects, remain intrinsically related
to the AER and FGF function. Insult to the AER
during development will lead to clinically
observed truncations at variable stages depending
on the timing of the insult. Frequently, isolated
limb cases suspected to be secondary to insults to
the AER will predominantly be mechanical and
nonheritable due to the extensive role FGFs play
in other biological systems, resulting in severe
systemic defects that may render limb defects
lower in priority.
Anteroposterior (AP)
Arguably, the most studied axis of limb pattern-
ing, and perhaps the most clinically relevant, is the
AP axis. The anteroposterior axis distinguishes
Y.H. Chen and A. Daluiski
the radial (or preaxial) from the ulnar
(or postaxial) side. This axis was initially discov-
ered when sections of posterior (ulnar) limb bud
tissue were excised at varying stages of chicken
limb development resulting in limbs that devel-
oped longitudinally but that did not develop
radioulnar-based digit identities. Excision of
radial tissue did not produce the same effect. It
was discovered that a small region of posterior
cells, at the junction of the limb paddle and the
trunk, was a signaling center for AP development
termed the zone of polarizing activity (ZPA). This
zone of tissue polarized the limb along the AP
axis. When ZPA tissue was grafted onto the radial
side, a mirror image of the limb along the AP
plane was produced (Tickle 1981).
The key signaling molecule of the ZPA is
Sonic Hedgehog (SHH). This ligand was named
for its molecular resemblance to the drosophila
molecule Hedgehog, which is important for y
segmentation, thus leading to its similar name
despite different phyla. SHH is a diffusible sig-
naling molecule with expression restricted to the
posteriorly located ZPA (Fig. 3) and forms a pos-
terior (high) to anterior (low) gradient of SHH
signaling. Functionally, SHH is critical for regu-
lating AP patterning and growth of the zeugopod
(forearm) and autopod (hand).
Restriction of SHH expression to the posterior-
located ZPA is accomplished by Gli3, a
1 Embryology
Fig. 4 SHH and GLI3A:GLI3R ratio in the hand plate (Anderson et al. 2012)
transcription factor that pre-patterns the early limb
bud along the AP axis before SHH expression is
activated (te Welscher et al. 2002). Gli3 exists in
two forms: GLI3A (activator) and GLI3R (repres-
sor). By default, Gli3 is modied to form the Gli3
repressor form. On the posterior (ZPA) side, SHH
inhibits this modication to allow for production
of the GLI3A activator form, producing a gradient
of high GLI3A:GLI3R ratio posteriorly to low
GLI3A:GLI3R anteriorly. Manipulation in chick
embryos to produce high GLI3A:GLI3R ratios
throughout results in polydactyly with posterior
digit identities of all extraneous digits (Litingtung
et al. 2002), reecting the role of high SHH signal-
ing in specifying posterior structures. Of note, the
stylopod (upper arm) is patterned independently of
SHH, presumably accomplished by Gli3-mediated
pre-patterning (Niswander 2002). The elbow repre-
sents the transition from SHH-independent to
SHH-dependent limb development, which may
have clinical implications in such clinical pheno-
types as below-elbow truncations.
The interactions between SHH and Gli3 result
in a gradient of SHH signaling and GLI3A:GLI3R
ratio along the AP axis that directs specication
and development of autopod ulnar-sided to radial-
sided structures (Fig. 4). In humans, defects in the
function of either SHH or Gli3 may cause limb
defects along the AP axis, frequently manifesting
clinically with digit abnormalities such as poly-
dactyly and syndactyly (Anderson et al. 2012).
Mutations of GLI3, for example, result in various
preaxial and postaxial polydactylies in Greig
cephalopolysyndactyly syndrome (MIM 175700)
(Hui and Joyner 1993).
11
Defects in digit development are closely corre-
lated with SHH function because SHH signaling
species the number of digits as well as the iden-
tity of each digit. To achieve this, SHH is
expressed in a gradient that varies both spatially
and temporally (Harfe et al. 2004). Spatially, the
concentration gradient exposes the mesoderm to
varying concentrations of SHH, with the concen-
trations greatest on the posterior (ulnar) side and
none on the anterior (radial) side. The mesenchy-
mal progenitors of the fth digit are exposed to the
greatest SHH concentration whereas the thumb
develops in the absence of SHH signaling. Tem-
porally, the length of exposure to SHH determines
digit identity. Shorter-term exposure of the mes-
enchyme is sufcient to specify anterior digits
(second digit), whereas the fth digit requires the
longest SHH exposure for correct specication
(Scherz et al. 2007). In fact, the posterior three
digits contain SHH-expressing cells from the ZPA
while the SHH contribution to the second digit
derives from paracrine signaling (Harfe
et al. 2004). Furthermore, SHH acts as a mitogen
to produce the necessary progenitor pool for
forming ve complete digits (Malik 2014).
Digit identity is established by the SHH gradi-
ent from the ZPA, but digits initially develop as
webbed ngers. Extraneous tissue between n-
gers must undergo apoptosis to form digits with
interdigital separations. Bone morphogenetic pro-
teins (BMPs), widely known for their role in
chondrogenesis and osteogenesis, mediate apo-
ptosis of the interdigital mesenchyme to produce
separated ngers. The interdigital mesenchyme
expresses BMP2, BMP4, and BMP7 which
12
antagonizes the pro-survival effects of the Fgfs
from the overlying AER (Pajni-Underwood
et al. 2007; Suzuki 2013). In animals with webbed
limbs such as bats, BMP antagonists block BMP
signaling to produce persistent interdigital tissue
(Oberg et al. 2010). Enhanced function or signal-
ing of Fgfs can also prevent interdigital apoptosis
by overcoming BMP-mediated inhibition, as
occurs in the syndactyly observed in Apert syn-
drome (MIM 101200).
The factors governing formation of the digits
are less well understood. Digit formation is cur-
rently thought to occur from the combination of
the initial AP patterning established by SHH,
BMP signaling from the interdigital mesenchyme,
and phalangeal growth mediated by the phalanx-
forming region (PFR) found in the sub-AER mes-
enchyme. Cells from the sub-AER mesenchyme
are continuously incorporated into each of the
PFRs and subsequently into the growing digit.
Cells incorporated earlier form the proximal
digit, undergoing condensation to form the prox-
imal phalanges; cells incorporated later form the
distal phalanges. BMP signaling from the
interdigital mesenchyme promotes this process
via its stimulatory effects on the PFR, in contrast
to the inhibitory effect BMPs have on the
AER-FGFs that produces interdigital apoptosis
(Suzuki et al. 2008).
SHH is critical in various development pro-
cesses, most notably is the development of the
notochord. Mutations to the SHH gene proper,
such as large deletions that produce a defective
protein, occur but are unlikely to be encountered,
due to either developmental lethality or serious
neurologic defects that take precedence. More
relevant, however, are mutations to regulators of
SHH signaling. Several of these regulators have
clinical importance and include ZRS mutations,
GLI3 mutations, and ciliopathies.
The ZRS (ZPA regulatory sequence) is an
enhancer sequence that is necessary and sufcient
for regulating the spatiotemporal SHH activity in
the developing limb (Anderson et al. 2012). This
sequence is located at a distance (1 Mb upstream)
from SHH, in an intron in the LMBR1 gene.
Mutations to this region produces a range of phe-
notypes involving the upper limb including
Y.H. Chen and A. Daluiski
preaxial polydactyly (MIM 174500), isolated
triphalangeal thumb (MIM 174500), syndromic
triphalangeal thumb (MIM 174500), syndactyly
(MIM 186200), and acheiropody (bilateral con-
genital amputations, MIM 200500).
Triphalangeal thumbs and preaxial polydactyly
arise secondary to point mutations that result in
ectopic SHH expression, producing an ectopic
ZPA at the anterior margin of the limb bud (Lettice
et al. 2008). This ectopic SHH may respecify
anterior digits into posterior digits (triphalangeal
thumb or thumb-to-nger transformation) or
induce the formation of a mirror hand (duplication
of posterior digits in the anterior margin) (Fig. 5).
Syndactyly and polysyndactyly may occur
from overexpression of SHH, particularly in the
interdigital mesenchyme, and are associated with
mutations that increase the dosage of SHH such as
ZRS duplications (Klopocki et al. 2008) or the
adoption of a more widely expressed enhancer
by SHH (Anderson et al. 2012). Acheiropodia,
characterized by congenital upper and lower
limb amputations and aplasia of the hands
and feet, is associated with mutations to
the LMBR1 gene not involving the ZRS
sequence, raising the possibility of additional reg-
ulatory sites in addition to the ZRS (Ianakiev
et al. 2001).
GLI3 regulates SHH signaling via the GLI3A:
GLI3R ratio along the AP axis. Mutations to GLI3
are associated with Greig cephalopolysyndactyly
syndrome (GCPS, MIM 175700), Pallister-Hall
syndrome (PHS, MIM 146510), preaxial polydac-
tyly (MIM 174200), and postaxial polydactyly
(MIM 174700). GCPS and PHS yield interesting
genotype-phenotype correlations. Splitting the
GLI3 gene into thirds, GCPS is frequently asso-
ciated with mutations to the rst and last third of
GLI3 whereas PHS is frequently associated with
mutations to the middle third. Correspondingly,
GCPS exhibits pre- or postaxial polydactyly
(or crossed polydactyly) whereas PHS exhibits
central polydactyly (Biesecker 2011). PHS muta-
tions are frequently gain-of-function mutations
that generate constitutively active GLI3R. This
GLI3R disrupts the normally high GLI3A:
GLI3R ratio in the posterior limb, resulting in
anteriorization. These disruptions to GLI3A:
1 Embryology 13

Fig. 5 Development of a
mirror hand due to a ZRS
mutation causing formation
of a second, ectopic ZPA on
the anterior margin of the
hand. A mirror hand
develops due to duplication
of the posterior digits
(Zeller et al. 2009)

GLI3R ratios correlate to the pre- or postaxial
polydactyly observed in GLI3 mutations.
Ciliopathies involve mutations to cilia, which
actively mediate signaling of the Hedgehog fam-
ily of proteins including SHH (Hildebrandt
et al. 2011). GLI3 has also been shown to be
localized to the tips of cilia. Consequently, in
addition to disrupting SHH signaling, cilia defects
may also disrupt GLI3 function or GLI3
processing into GLI3A and/or GLI3R. Such
defects result in the pre- and postaxial polydactyly
seen in the preceding discussion on SHH regula-
tory and GLI3 mutations. Examples of syndromes
associated with ciliopathies that affect the limbs
include Bardet-Biedl syndrome (MIM 209900),
Joubert syndrome (MIM 213300), Meckel syn-
drome (MIM 249000), and Pallister-Hall
syndrome (MIM 146510). In accordance with
the involvement of SHH and GLI3, upper limb
differences seen in these syndromes frequently
include polydactyly.
Dorsoventral (DV)
Dorsoventral patterning is less well understood
and deformities in this plane are infrequently
encountered. Similar experiments to those done
for the PD and AP axes initially established that
the dorsal ectoderm provides the signal for DV
development. However, unlike AP development,
the entire dorsal ectoderm provides the source of
signaling rather than a single section or zone.
When the dorsal ectoderm was excised, the limb
14
Fig. 6 Dorsal dimelia, with a palmar nail on the fth digit. Dorsalized skin is apparent on the palm (Al-Qattan 2013)
lost dorsal structures (nail plates, extensor ten-
dons) and assumed a more ventral appearance
(with palmar-like exion creases, sweat glands,
and lack of hair follicles) (Loomis et al. 1996).
While it is unclear what initially establishes DV
polarity, it does appear that this mechanism may
be distinct from that of the AP and PD axis. Fgf10
null mice are characterized by initiated limb buds
without any subsequent limb growth. In these
limb buds, neither the ZPA nor the AER formed,
but expression of molecules that establish DV
patterning remained normal (i.e., PD and AP
axes disrupted, but DV axis intact) (Sekine
et al. 1999).
The causative agent for establishing DV polar-
ity is the secreted factor Wnt7a. Wnt7a from the
dorsal ectoderm induces expression of Lmbx1b, a
LIM homeobox transcription factor necessary and
sufcient for the development of dorsal limb
structures (Riddle et al. 1995). Excision of the
dorsal ectoderm results in loss of dorsalization
associated with deciency of Wnt7a, which can
be rescued by application of a Wnt7a-soaked bead
(Yang and Niswander 1995). Conversely, the ven-
tral ectoderm expresses Engrailed-1 (En-1).
Engrailed-1 inhibits Wnt7a, restricting expression
of Wnt7a to the dorsum, and allows for the devel-
opment of ventral limb structures. In mice, loss of
Engrailed-1 results in dorsalization of the ventral
surface due to uninhibited expression of Wnt7a
Y.H. Chen and A. Daluiski
ventrally (Loomis et al. 1996). This dorsal dupli-
cation can also be reproduced by ectopic
overexpression of Wnt7a on the ventral surface.
Defects in either Wnt7a or En-1 signaling are
implicated in clinical presentations of ventral
(Wnt7a-decient) or dorsal (En-1-decient)
duplication phenotypes of varying severity. In
humans, dorsalization may manifest as a circum-
ferential or palmar nail, frequently aficting the
fth digit (Fig. 6) (Al-Qattan 2013; Rider 1992).
Conversely, defects in Wnt7a or its mediator
Lmx1b result in defects in dorsal structures, fre-
quently presenting with ngernail hypoplasia
(Fig. 7). Greater degrees of palmar duplication,
for instance, involving the entire hand, may also
occur in humans (Al-Qattan 2013). Some patients
do not have identiable abnormalities in the cod-
ing regions of the Wnt7a gene, reecting our
incomplete understanding of the molecular mech-
anisms of DV patterning.
Clinically, DV defects are very rarely seen in
isolation. Mutations to Wnt7a that causes
Fuhrmann syndrome (MIM 228930) or
Al-Awadi-Raas-Rothschild/Schinzel phocomelia
(AARS) syndrome (MIM 276820) have a broad
range of defects that include decits along the AP
and PD axis (Woods et al. 2006). Defects isolated
to a single developmental axis should be consid-
ered the exception rather than the rule as the three
axes develop in combination to form the upper
1 Embryology
Fig. 7 Example of defects
in dorsalization. (a) Nail
dysplasia in nail-patella
syndrome (LMX1B
mutation). (b) Palmar
duplication with
hypoplastic nails. Note the
palmar creases localized on
the dorsum of the hand
(Reproduced with
permission of A Daluiski)
limb. Selected clinical phenotypes are provided in
Table 3, demonstrating the frequent involvement
of multiple axes as well as other organ systems.
Coordination Between Axes
While each of the three axes (AP, PD, and DV)
was discussed separately in the preceding section,
these axes are highly coordinated through com-
plex interrelated pathways. Integration of the
molecular events provides a more complete pic-
ture of events of limb development, despite the
signicant gaps in knowledge that still remain.
This integrated approach will assist in the recog-
nition of associated developmental defects that
may provide clues as to the nature of the underly-
ing genetic defect, as it places distinctive pheno-
types in the context of underlying developmental
biology. An overview of the interdependency of
the major players of each spatial axis is shown in
Fig. 8.
Anteroposterior and Proximodistal
Arguably, the AP and PD axes are the most
intricately related. From the early establishment
of the two axes during limb bud initiation, the
AER and ZPA mutually induce the other in a
positive feedback loop to sustain normal limb
development; loss of one results in loss of the
other (Niswander et al. 1994). Molecularly, this
positive feedback loop has been referred to as the
15
SHH-GREM1-FGF ectodermal-mesenchymal
feedback signaling loop (Zeller et al. 2009) that
has been proposed to explain each of the phases of
limb development (initiation, propagation, and
termination) (Bnazet et al. 2009). The default
loop involves SHH inducing expression of
GREM1 from the sub-AER mesenchyme. Mesen-
chymal GREM1 antagonizes BMP signaling to
disinhibit expression of ectodermal AER-FGFs
(recall that BMP signaling inhibits the
AER-FGFs). AER-FGFs, in turn, signal back to
the ZPA to maintain SHH expression.
At the time of limb bud initiation, high BMP
signaling initially induces formation of the AER.
Feedback upregulation of GREM1 quickly fol-
lows, blocking BMP signaling to permit
FGF-mediated limb bud outgrowth during the
propagation phase (Ahn et al. 2001; Bnazet
et al. 2009). As limb outgrowth nears completion,
the SHH-GREM1-FGF feedback loop is capable
of self-termination to restrict limb size
(Verheyden and Sun 2008). Termination occurs
once the sub-AER extends sufciently far away
from the SHH/ZPA in the growing hand plate such
that SHH is no longer able to maintain GREM1
expression from the sub-AER mesenchyme. Loss
of GREM1 inhibition allows BMP signaling to
redirect undifferentiated cells from proliferation
to tissue differentiation (digits) or apoptosis
(interdigital mesenchyme).
The interdependence between the AP and PD
axes through the SHH-GREM1-FGF feedback
 16

Table 3 Selected genes involved in limb identity or patterning along the three axes (anteroposterior, proximodistal, and dorsoventral). Limb findings as well as findings in other
organ systems are provided to illustrate the frequently pleiotropic manifestations of mutations to single genes critical for upper limb development
Limb ndings
Gene Phenotypes Inheritance Gene function (bold characteristic) Other ndings
Limb TBX4 Small patella syndrome AD Lower limb Pelvic and lower limb defects
identity (147891) development Aplasia/hypoplasia of
patellae
PITX1 Clubfoot (119800) Multifactorial Lower limb Clubfoot
development Various lower limb
malformations
Liebenberg syndrome AD with Lower limb Upper limb malformations
(186550) variable development (dysplastic elbow joints,
penetrance carpal fusion, radial deviation)
Homeotic arm-to-leg
transformation
TBX5 Holt-Oram syndrome AD Upper limb Thumb anomaly (various) Cardiac defects
(142900) development Atrial septal defect
Cardiomyocyte Various upper limb anomalies
differentiation (aplasia/hypoplasia)
PD axis FGF10 LADD Tissue growth Clinodactyly (5th digit) Puncta aplasia/hypoplasia, lacrimal duct
(FGFR3, (lacrimoauriculodentodigital) and Thumb anomalies obstruction
FGFR2) syndrome (149730) differentiation Mild syndactyly Hearing decits
Wnt3 Tetra-amelia (273395) AR Tissue growth Amelia Pulmonary hypoplasia
and Urogenital defects
differentiation Craniofacial defects
Y.H. Chen and A. Daluiski
 1

AP axis GLI3 Greig cephalopolysyndactyly AD Digit Pre-/postaxial polydactyly Cranial (frontal bossing, scaphocephaly,
(GLI3 inactivating mutation) development Variable syndactyly hypertelorism)
(175700) Craniosynostosis
Pallister-Hall syndrome AD Digit Central polydactyly Pituitary dysfunction
(GLI3 truncation) development
Embryology

Postaxial polydactyly Visceral malformations

Syndactyly/polysyndactyly Hypothalamic hamartomas
Brachydactyly
Postaxial polydactyly, types AD Digit Postaxial polydactyly (often
A1 and B (GLI3 point development functional)
mutation) (174200)
Preaxial polydactyly, type IV AD Digit Preaxial polydactyly +/
(GLI3 point mutation) development postaxial polydactyly
(174700)
DV axis WNT7A Fuhrmann syndrome AR Dorsalization Posterior (ulnar) aplasia/ Primary (major axes), secondary (heart or
(228930) hypoplasia limb primordial), and local (tibial-bular
Digit abnormalities differentiation) developmental elds
Pelvic abnormalities
Femur bowing
Absence of ulna and bula AR Dorsalization Absent ulnae and bulae
(276820) Femoral hypoplasia
Pelvic hypoplasia
Abnormal genitalia
LMX1B Nail-patella syndrome AD Dorsalization Nail dysplasia Distal neuropathy
(161200) Patellar aplasia/hypoplasia Nephropathy, renal disorders
Iliac horns
17
18
Fig. 8 A simplied view of
signaling between the major
signaling molecules of the
three (AP, PD, and DV)
spatial axes (Duboc and
Logan 2009)
loop accounts for clinical phenotypes that involve
both axes simultaneously.
Dorsoventral and Anteroposterior
Excision experiments indicated that the dorsal
non-AER ectoderm (expressing Wnt7a) plays a
role in maintaining SHH expression from the
ZPA. Removal of dorsal non-AER ectoderm
resulted in decient expression of SHH from the
ZPA. Conversely, removal of the ventral
non-AER ectoderm had only minor effects on
SHH expression (Yang and Niswander 1995).
Furthermore, loss of the dorsal ectoderm induced
ulnar defects (AP axis) as well as defects in limb
outgrowth (PD axis).
The differential effects produced by excision
of either the dorsal or ventral ectoderm can be
explained by the dorsal ectoderms expression of
Wnt7a. Conrmatory experiments in mice dem-
onstrated that Wnt7a, in addition to its role in the
DV axis, also induced and maintained expression
of SHH. Loss of Wnt7a in mice resulted in dorsal-
to-ventral transformation, as well as loss of pos-
terior digits that require SHH for formation (Parr
and McMahon 1995). The observed defects in
limb outgrowth are likely the result of defective
Y.H. Chen and A. Daluiski
AER-FGF signaling due to impaired SHH
expression.
In humans, disruption of Wnt7a results in dor-
soventral patterning defects (MIM 276820), as
well as impaired ulnarization of tissue resembling
SHH deciencies (MIM 228930).
Dorsoventral and Proximodistal
Disruption of either Wnt7a or En1 results in DV
defects, but without any impairment to PD
growth, suggesting that Wnt7a or En1 does not
regulate AER function. However, simultaneous
DV and PD defects can exist due to their common
dependence on BMP signaling for initial induc-
tion of the patterning signals. In both mice and
chicks, BMP signaling is necessary and sufcient
for establishing the DVaxis and induction of AER
formation (Ahn et al. 2001).
Due to the close relationship between the AP
and PD axes, defects of DV patterning genes such
as Wnt7a cannot only result in defects in the AP
axis, but secondarily affect the PD axis. In accor-
dance with its role in dorsalization, relatively mild
Wnt7a mutations produce dorsal defects such as
ngernail hypoplasia. Mutations resulting in mod-
erate loss of Wnt7a function result in impaired
1 Embryology
SHH expression, leading to ulnar ray deciencies
due to decient ulnarizing SHH signals. Severe
loss of Wnt7a function produces severe deciency
of SHH, resulting in phenotypes such as limb
truncations that resemble defects occurring from
AER dysfunction or acheiropodia (LMBR1 gene
defect that affects SHH regulation). The severe
loss of SHH likely results in loss of
SHH-GREM1-FGF signaling, resulting in limb
truncations due to failure of AER-mediated
proximodistal growth.
Third Phase: Tissue Differentiation
In concert with the establishment of upper limb
patterning, progenitor pools must appropriately
migrate, expand (for growth), and differentiate
(for the development of specic structures). The
lateral plate mesoderm, somitic mesoderm, and
neural crest provide all the necessary progenitors
for the formation of a complete limb. From the
initial proliferating mass of undifferentiated mes-
enchyme that characterizes the early limb bud,
limb structures develop at a rapid pace with
remarkable coordination and organization. Pro-
gression of development is a uid process with
many events occurring simultaneously. Rather
than presenting individual events chronologically,
it is useful to consider limb development by
system.
Vascular System
One of the earliest systems to appear in the early
limb bud is the vascular system. Progenitors from
the somitic mesoderm migrate into the limb bud
and undergo angiogenesis to form a rudimentary
capillary system. The AER is instrumental in
guiding the longitudinal growth of the vascular
system as the limb bud elongates and matures.
Initially, a vascular plexus forms in the
subectodermal mesenchyme of the early limb
bud, which coalesces along the peripheral border
to form a marginal sinus. Formation of the venous
system follows the capillary network, with lym-
phatics differentiating last. As the limb bud
19
elongates, the marginal sinus is lost whereas the
vascular plexus maintains continued angiogenesis
to supply blastemas of muscle progenitors that
develop along the periphery of the forming limb.
The core of the limb receives comparatively fewer
penetrating vessels, allowing for a lower oxygen
tension region that facilitates cartilage develop-
ment and subsequent formation of the skeletal
system via endochondral ossication.
Initial arterial supply of the rudimentary capil-
lary system is provided by the subclavian artery
originating from the right dorsal aorta. Formation
of the brachial artery occurs early as the limb bud
elongates. The brachial artery then branches into
the median and interosseous arteries. Formation
of the ulnar artery then follows with the radial
artery forming last.
Nervous System
Two sets of neural cells grow out from the devel-
oping spinal cord toward the limb buds to form the
brachial plexus: ventral rami (motor) and dorsal
rami (sensory). The two sets of proliferating neu-
rons coalesce during week 4 to form the brachial
plexus. From then on, nerves grow into the devel-
oping limb innervating structures proximal to dis-
tal. Motor neurons have cell bodies in the spinal
cord, yet send out a single axon that extends over
great distances to innervate a single target. This is
accomplished by the enlarged tip at the ends of the
growing axon known as the growth cone. The
growth cone contains numerous highly motile
lopodia that interact extensively with local cues
within the developing limb that progressively
guide the axon to its nal target. Inability of the
axon to reach its target muscle results in neuronal
cell death. For each individual muscle, multiple
axons compete to establish innervation with apo-
ptosis of the neurons unable to establish a connec-
tion. The number of neurons that establishes a
connection and survives correlates with the size
of the muscle, with one neuron eventually becom-
ing the dominant one.
The remarkable process of selective axonal
targeting is not completely understood. Experi-
mental studies suggest that migrating neural cells
20
provide the guiding cues. However, it appears that
once an axon reaches the brachial plexus, the
axons are able to nd their target muscle regard-
less of duplication, rotation, or amputation of the
limb (Beatty 2000).
Musculoskeletal System
In the developing limb bud, the muscular blas-
tema and the chondrogenic blastema form the
muscles and bones, respectively. The muscular
blastema is located in the periphery, where oxygen
tension is higher due to the vascular plexus. The
chondrogenic blastema is located centrally, where
the oxygen tension is comparatively lower. Mus-
cle development occurs sequentially, with proxi-
mal muscles separating from the muscular
blastema and differentiating before distal muscles
and supercial muscles differentiating before
deep muscles. The skeletal system in the upper
limb forms through endochondral ossication.
The chondrogenic blastema forms cartilage in
the central region of the limb bud in a proximal
to distal manner. The cartilage later undergoes
ossication to form bones.
Joints form in regions called the interzone at
the junction between the ends of two blastemas. A
joint capsule forms early on at the interzone. Sub-
sequently, cavitation occurs within the center of
the interzone to produce a joint space with pro-
duction of joint uid. At either end of the
interzone, articular cartilage forms to cap the two
ends of bone. The formation of a functioning joint
requires joint motion. In the absence of motion,
the joint space becomes inltrated by brous tis-
sue, resulting in an immobile joint.
Extrinsic Factors
Limb differences secondary to extrinsic factors
are deformations with a normal genetic develop-
mental program. These differences are not related
to dysfunction in molecular signaling or tissue
differentiation. Had embryo development been
allowed to proceed unhindered, no differences
would have otherwise been observed.
Y.H. Chen and A. Daluiski
The major constituent on this category is
known as the amniotic band syndrome (ABS)
(MIM 217100), also known as ADAM (amniotic
deformity, adhesion, and mutilation) sequence
(Cignini et al. 2012). ABS produces a variety of
limb differences attributed to in utero mechanical
compression or amputation of normally develop-
ing tissue. Theories for pathogenesis include rup-
ture of the amniotic sac resulting in either the
formation of brous amniotic bands that act as
tourniquets or the extrusion of fetal parts through
the amniotic sac defect with subsequent constric-
tion. These compressive forces may result in con-
striction bands with or without distal hypoplasia
secondary to impaired vascular supply, classically
seen in the digits. More severe strangulation can
result in outright amputation.
Ischemia due to either spontaneous or induced
vascular insufciency may also produce similar
phenotypes due to necrotic loss of developing
tissue.
International Federation of Societies
for Surgery of the Hand (IFSSH)
Classification System
The IFSSH classication currently in use was
adopted in 1976 (Swanson 1976). Originally pro-
posed by Alfred B. Swanson in 1964 as a modi-
cation of Frantz and ORahillys proposed system
(Frantz and ORahilly 1961), the classication
system was intended to be a practical, efcient
method to facilitate identication and diagnosis
of upper limb differences. Conception of the sys-
tem was made with comparatively limited under-
standing of limb development, resulting in a
classication system that was primarily based on
morphology (Table 4).
Over the years, several modications have
been proposed to change the IFSSH classication
system to better reect the updated view of human
upper limb development. While new classication
systems for upper limb differences will undoubt-
edly emerge, the Swanson IFSSH classication
system remains the most universally accepted
system as of the writing of this text. Furthermore,
it is a logical, easy-to-use clinical tool that
1 Embryology
Table 4 The IFSSH classification for characterizing
human congenital differences of the upper limb
I. Failure of formation
II. Failure of differentiation
III. Duplication
IV. Overgrowth (gigantism)
V. Undergrowth (hypoplasia)
VI. Congenital constriction band syndrome
VII. Generalized skeletal abnormalities
provides clinicians with a framework with which
to begin evaluating patients with upper limb
differences.
More recently, moves toward overhauling the
IFSSH classication in favor of a more
embryology-based classication system have
been made. In particular, Tonkin and colleagues
have proposed a system that integrates our current
understanding of how the upper limb develops in
humans to aid in the diagnosis, communication,
and understanding of congenital limb differences
(Tonkin et al. 2013). Underscoring this move
toward dening limb developmental differences
on a molecular level, the ability of pediatric upper
limb surgeons to recognize, dene, and character-
ize limb difference phenotypes becomes all the
more important.
Summary
Many of the key molecular players in this phase
may not have great clinical signicance as we have
not yet identied human mutations in these genes
that produce limb phenotypes presenting for clini-
cal treatment. Thus, though these may be of vital
importance to the developing limb, they may be of
little importance to the treatment of our patients.
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 Functional Development
2
Susan Duff

Contents Abstract
Key Components of Prehension . . . . . . . . . . . . . . . . . . . . 25 Much of our functional independence is
Vision . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 25 inuenced by our prehensile skill or the ability
Reaching . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 26 to use our hands and upper limbs effectively.
Grasp Patterns . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 27 Typical prehension patterns develop rapidly
Manipulation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 28
Release . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 30 over the rst year of life yet renement of
skill and dexterity continues into adolescence.
Associated Components . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 31
Feedforward and Feedback Control . . . . . . . . . . . . . . . . . . 31
Prehensile decits stemming from congenital
Postural Control . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 31 differences or acquired injury can limit object
Gross Motor Development and Prehension . . . . . . . . . . 31 and environmental exploration thus inuence
Biomechanics and Stabilization . . . . . . . . . . . . . . . . . . . . . . . 31 skill development. To appreciate the adapta-
Bimanual Coordination . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 32
Hand Preference/Dominance . . . . . . . . . . . . . . . . . . . . . . . . . . 32
tions often required to enhance independence
Handwriting . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 33 in the presence of upper limb disorders, it is
worthwhile to review key features of prehen-
Atypical Prehension . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 33
Adaptations to System Changes . . . . . . . . . . . . . . . . . . . . . . 33 sion and how skill changes from infancy to
adolescence.
Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 34
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 35
Key Components of Prehension

Prehension reects the interaction between goal-

directed movement and intrinsic/extrinsic con-
straints. Pencil writing is just one task that exem-
plies the key features of prehension: visual
regard, reach, grasp, manipulation, and release
(Table 1).

Vision
S. Duff
Vision guides prehensile acts to enhance accuracy
Department of Physical Therapy, Thomas Jefferson
University, Philadelphia, PA, USA and control. Visual perception allows us to recog-
e-mail: susan.duff@jefferson.edu nize, recall, discriminate, and understand what we
# Springer Science+Business Media New York 2015 25
J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_2
26 S. Duff

Table 1 Features of prehension

Description Example actions
Visual regard Attention on an object and associated visual perception prior to Determining the location and
an action orientation of a pencil
Reach and grip Arm transport to object location and hand pre-shaping to match Reaching for a pencil and
Formation object characteristics (i.e., size, shape) opening ngers slightly wider
than the diameter
Grasp Finger and thumb closure with or without sustained pressure to Securing and holding the pencil
stabilize an object
Manipulation Movement of an object in one hand Rotating or adjusting the ngers
toward the pencil tip in one hand
Release Manner in which an object leaves the hand or taking pressure Letting go of the pencil on the
off of an object table

see (Goodale and Milner 1992). Depth perception,
gure ground, and visuo-construction are select
perceptual constructs used in everyday tasks.
Depth perception allows us to estimate distance
and size of objects in the environment.
Figure ground helps us to selectively screen out
background stimuli to focus on foreground
details. Visuo-construction is a spatial planning
process involved in building two- and three-
dimensional objects such as a model airplane.
Experience contributes to visual memory to fur-
ther guide prehension.
We use visual-motor control for precise move-
ment guidance. Peripheral vision provides cues
about object distance and direction during
reaching (Abahnini and Proteau 1997), whereas
central vision allows xation on critical land-
marks for ne-motor control during manipulative
tasks. When vision is compromised, we rely on
somatosensory cues or visual memory to guide
prehension.
Reaching
Features
Reaching is the extent, orientation, and speed of
the hand path as it moves to a new position
(Abend et al. 1982). With reach-to-grasp tasks,
the shoulder places the hand over a wide area in
space and the elbow places it close or far from the
body. The forearm and wrist accurately position
the hand before grasping or weight bearing, and
during grip formation, the ngers and thumb
accommodate for object size and shape.
Reaching in late adolescence is characterized
by smooth velocity proles and relatively contin-
uous, straight hand paths to the target (Jeannerod
1984). Grip formation or pre-shaping of the hand
to object shape and size gradually unfolds with
reaching and can be divided into nger opening
and closure. The timing and size of peak nger
opening gradually scales and by adolescence
occurs within 7075 % of total movement time
of a reach (Jakobson and Goodale 1991;
Jeannerod 1984).
Development
Pre-reaching is described as a apping, inaccurate
movement with frequent accelerations and decel-
erations. Anticipatory, visually guided reaching
emerges between 3 and 4 months of age (Thelen
et al. 1996). von Hofsten and Lindhagen (1979)
showed that 4-month-old infants could predict or
anticipate the future position of a slowing moving
ball and catch it by directing the hand toward the
upcoming point of contact. Unimanual and
bimanual reaching behavior uctuates in the rst
year and is inuenced by postural control
(Corbetta and Thelen 1996; Corbetta and Bojczyk
2002; Rochat 1992). By 2 years of age, toddlers
display more adultlike reaching patterns
(Konczak and Dichgans 1997) with less variabil-
ity in repeated performance.
Grip formation has been documented in infants
as young as 18 weeks (von Hofsten and
2 Functional Development
Fig. 1 Pre-shaping of the hand in a young infant elicited in
preparation of toy contact
Fazel-Zandy 1984), yet scaling to object size
gradually improves throughout childhood. Before
object contact, infants and young children open
their hand wider than necessary, which allows a
higher safety margin of error to prevent missing
targets (Fig. 1). Finger opening is modied to
target size in 913-month-old infants, and by
13 months, infants initiate nger closure prior to
object contact. Six-year-olds continue to display
exaggerated nger opening during reaching,
whereas 12-year-olds scale nger posture more
closely to object size (Kuhtz-Buschbeck et al.
1998). Grip formation is not adultlike until late
childhood (Kuhtz-Buschbeck et al. 1998).
Grasp Patterns
Features
The characteristics and purpose of an object deter-
mine the grasp pattern used to secure it (Newell
et al. 1989). Power grips are thumb and nger
actions that act against the palm to apply a force to
an object (Napier 1956) and primarily include
cylindrical, spherical, and hook grips. For preci-
sion grip or pinch tasks, forces are directed
between the ngers and thumb and primarily
27
include pad-to-pad, tip-to-tip, and lateral or key
pinch. Isometric muscle contractions are used to
sustain power and precision grips. Table 2 lists the
muscles and joints associated with common pre-
hension patterns.
We typically grade the ngertip forces used
to secure and lift objects based on memories
of object weight, texture, and other features.
Fingertip force control is triggered by discrete
mechanical events that relay information from
somatosensory receptors relevant to object con-
tact and slip (Johansson and Cole 1992).
Feedforward or anticipatory control is used to
grade the grip (squeeze) and load (vertical) forces
in advance of contact, whereas feedback control is
used to make adjustments after contact. When
turning a key in a tough lock, a strong steady
grip force is used, but a light load force is suf-
cient to lift an empty soda can. Friction at the
interface between the nger and object helps to
maintain a secure grip on objects (Aoki et al.
2006). To prevent drops, feedback obtained from
tactile receptors induces an increase in grip force.
Without sufcient anticipatory control or tactile
feedback, objects may slip from grasp or be
squeezed too tight.
Development
In the rst few months of life, tactile and propri-
oceptive reexes control nger opening and clo-
sure (Twitchell 1970). Gradually hand reexes are
integrated and evolve into voluntary prehension
patterns. From birth to 5 months, an array of
patterns unfold (Wallace and Whishaw 2003).
Initial grasp patterns involve the ngers only,
with the thumb function progressively included
from 3 to 12 months. With postnatal neural devel-
opment, independent nger movements emerge.
Index isolation is often seen about 10 months of
age, and the index evolves into a dominant pointer
by 1 year of age. By 11 months, infants display
pad-to-pad opposition of the thumb to index to
grasp small objects. Further development and
experience helps to expand the repertoire of func-
tional grasp patterns which become more adult-
like by the end of the rst year.
The popular view of prehension development
is shown in Table 3. However, there may be subtle
28 S. Duff

Table 2 Classic prehension patterns

Patterns Joint motion Muscles used Function
Cylindrical Thumb opposition, nger adduction, FPL, thenar group, AdP, 4th Grasp and hold of a
grasp and exion lumbrical, select interossei, FDP cylindrically shaped
(FDS more power) object such as a can of
soda
Spherical Thumb opposition, nger exion, FPL, thenar group, AdP, FDP (FDS Grasp and hold of a
grasp and abduction more power), AbDM, 4th round object such as a
lumbrical, all interossei (except softball
2nd)
Hook grasp MCPs neutral, exion PIPs/DIPs, FDS and FDP, EPL and EPB, EDC, Grasp and hold of a
and thumb extension 4th lumbrical and 4th dorsal small luggage handle
interossei
Pad-to-pad Thumb opposition with slight thumb Thenar group, FPL, FDS of Grasp and hold over the
pinch and index exion, MCP/PIP exion, involved ngers (FDP if DIP head and tail aspects of
and slight index DIP exion exion present), 1st DI and 1st a coin
lumbrical
Tip-to-tip Features of pad-to-pad pinch, with Features of pad to pad, with greater Picking up a needle
pinch greater thumb/nger exion, with FPL and FDP force due to DIP
index DIP exion exion, index interossei
Lateral Thumb adduction with IP exion, FPL, FPB, AdP; FDS and FDP all Turning a key in a lock
(key) pinch index nger exion, and abduction ngers; less interossei and
lumbricals except rst DI
AdP adductor pollicis, AbDM abductor digiti minimi, DIP distal interphalangeal, DI dorsal interossei, EPL extensor
pollicis longus, EPB extensor pollicis brevis, EDC extensor digitorum communis, FPL exor pollicis longus, FDP exor
digitorum profundus, FDS exor digitorum supercialis, IP interphalangeal, MCP metacarpophalangeal, PIP proximal
interphalangeal (Data from Landsmeer 1962, Long et al. 1970, Napier 1956, 1980, Norkin and Levangie 1983)

variations to this viewpoint. Halverson (1931,
1932) indicated that an ulnar grasp, elicited by
tactile cues to the ulnar hand, preceded a radial
grasp. However, Forssberg et al. (Lantz
et al. 1996) found that after palm contact, young
infants may display a palmar grasp for a
dumbbell-shaped object (see Fig. 1). This sug-
gests that object characteristics may inuence
the pattern displayed more than previously
assumed (HohIstein 1982; Newell et al. 1989).
Anticipatory ngertip force control during
grasp and manipulation develops gradually over
the rst 2 years (Forssberg et al. 1995; Pare and
Dugas 1999). Initially, infants and toddlers
increase grip and load forces sequentially, using
a feedback strategy (Forssberg et al. 1995). In the
second year, grip and load forces begin to be
generated in parallel, displaying a transition to
anticipatory control as the child begins to use
cups, utensils, writing implements, and self-care
items (Fig. 2). Until sufcient ngertip force con-
trol develops, children may crush fragile objects
such as paper cups or potato chips or lift light
objects too quickly. With the improvement in
distal control, crayon and pencil grips are modi-
ed. A developmental scale of pencil grip is
shown in Fig. 3.
Although some 6- and 8-year-old children
demonstrate adult-like ngertip force coordina-
tion during object manipulation, some do not
attain this ability until 11 years of age or later
(Forssberg et al. 1995).
Manipulation
Features
Manipulation or haptic exploration requires alter-
ations in object position in space and accurate
control of thumb and nger position (Landsmeer
1962). Manipulation of an object within one hand
is entitled in-hand manipulation and can be
divided into translation, shift, and rotation
(Exner 1990). Translation involves object
2 Functional Development 29

Table 3 Prehension development from birth to 1 year

Description Age Illustration Stimulation
Recognizes hands 8 weeks Hand enters visual eld via ATNR
(2 months)
Reexive ulnar group 12 weeks Object placement onto ulnar side of hand
(3 months) elicits grasp
Retains objects placed in hand: ngering 16 weeks Object placement in hand will foster grasp
on chest in midline, nger mouthing, and (4 months) and hold; hanging toys in visual eld will
object swiping encourage swiping
Primitive squeeze grasp (wrist exed), 20 week Introduction of varied toys will foster raking
raking (5 months) and voluntary grasp
Palmar grasp (no thumb wrist moves to 24 weeks Placing toys in different positions will
neutral) (6 months) encourage eyes and hands to search prior to
reach and grasp
Radial palmar grasp (thumb adduction 28 weeks Ideal toys are those that can be picked up
begins) (7 months) and transferred easily from one hand to the
other
Scissors grasp (thumb adduction stronger) 32 weeks Introduction of toys with a thin
(8 months) circumference will strengthen thumb
adductor
Radial-digital grasp (beginning 36 weeks Toy clay or nger food that is pliable will
opposition) (9 months) foster opposition
Inferior pincer grasp (volar hold versus 3652 Small objects varied in shape will promote
pad to pad, hand supported prior to weeks exploration via poking, feeling, and
grasping) and isolated index pointing (912 manipulation
months)
Pincer grasp-pad to pad (some support 3852 Tiny objects, such as raisins, to pick up and
before grasping) weeks drop will encourage development
(1012
months)
Superior pincer grasp-tip to tip (hand 5256 Thin yet safe objects the size of a pin will
unsupported prior to grasping) weeks encourage development
(1 year)
Three-jaw chuck (wrist extended and 5256 Toys requiring a strong radial hold like
ulnarly deviated), maturing release weeks blocks with containers allowing for repeated
(1 year) actions will foster strong grasp & release
(Data from Halverson 1931, 1932, Knobloch et al. 1987, Rosenbloom and Horton 1971)

movement from palm to ngers or ngers to palm
as when one moves a coin before placing it in a
slot. Shift is the movement of an object along the
nger pads or ngers as when moving a pencil
toward the ngertips. Rotation incorporates
motion of an object around its axis within the
ngers as when turning a spoon in the hand or a
pencil to erase (Fig. 4). In-hand manipulation
skills can involve stabilization of an object or
part of an object within the hand, while another
object or part is concurrently manipulated within
the same hand (Exner 1990).
Object recognition and naming without vision
using somatosensory cues, in-hand manipulation,
and memory is entitled stereognosis. Slow and
fast adapting mechanoreceptors in the nger fat
pads and ridges supply tactile information (Vallbo
and Johansson 1984). Proprioception is relayed
via joint receptors, muscle spindles, Golgi tendon
organs, and cutaneous mechanoreceptors (Edin
and Abbs 1991). Without sensory input,
stereognosis is signicantly impaired.
Development
Stereognosis and in-hand manipulation develop
gradually from infancy to childhood. Six-month-
old infants can visually recognize a shape after
only tactile contact (Rose et al. 1978).
30
Recognition of familiar objects through haptic
exploration is fair by 23 years and mature by
5 years of age (Stilwell and Cermak 1995).
Finger-to-palm translation and simple rotation
are displayed before 2 years of age, and complex
rotation (180 ) continues to be rened in the 6- to
7-year-olds.
Fig. 2 Grading of opposing ngertip forces used during
grasp and lift of objects such as a plastic cup
Fig. 3 Developmental scale of pencil grip: (a) radial
cross-palmar grasp, (b) palmar supinate, (c) digital pronate,
(d) brush grasp, (e) grasp with extended ngers,
S. Duff
The use of implements at any age requires the
use of sustained grip or pinch force, ngertip force
control, and in-hand manipulation. As intrinsic
muscle strength develops, children usually can
demonstrate sustained pinch force on items such
as a crayon while coloring. Once ngertip force
control and in-hand manipulation improves, a
crayon can be translated, rotated, or shifted ipsi-
laterally without assistance from the
opposite hand.
Release
Features
Release is the process of taking pressure off an
object. It can be quick or graded, as when we set a
glass onto a counter. A master pianist can hold and
release pressure on the keys in a graded fashion
that skillfully alters the tones. A novice pianist
may not exhibit the same degree of nesse on
the keys, making the tones sound loud and
sustained. As control of release improves, our
repertoire of ne-motor tasks enlarges.
Development
Release develops off a point of stability. Mutual
ngering in midline at 4 months and hand-to-hand
object transfer at 56 months occur because one
(f) cross-thumb grasp, (g) static tripod grasp, (h) four-
nger grasp, (i) lateral tripod grasp, and (j) dynamic tripod
grasp (Redrawn from Schneck and Henderson 1990)
2 Functional Development
Fig. 4 Example of rotation component of in-hand manip-
ulation while holding a spoon
hand can release off the stability of the other. From
7 to 9 months, voluntary release emerges, initially
with stabilization from an external surface, such
as the tray of a high chair, and later without
external support. Object release into a small con-
tainer often occurs by 12 months, and by
15 months, pellet release into an even smaller
container occurs (Hirschel et al. 1990). Ball
throwing is an example of release that gradually
improves in control and accuracy throughout
childhood.
Associated Components
Feedforward and Feedback Control
Prehension involves feedforward and feedback
control. Under feedforward (anticipatory) control
(Patla 1995), muscle contractions are triggered in
anticipation of upcoming actions. Feedforward
control prevents undesired movement and allow
for adjustment in our center of gravity before the
upper limb moves in space. For example, to
pre-shape the hand to receive an object, the ngers
open slightly wider than the size of the object
during the reach before object contact (Jeannerod
1981). Feedback control allows us to respond to
perturbations such as slip of the hand on an object.
Postural Control
Reaching is closely linked to postural control.
Under feedforward control, stabilizing muscle
31
contractions or anticipatory postural adjustments
(APAs) are triggered in preparation for upcoming
instabilities (Patla 1995). APAs allow for an
adjustment in our center of gravity before the
arm moves in space. Under feedback control,
muscle responses are elicited after perturbations
and include arm muscle activation received from a
reach. Core stability allows control of trunk posi-
tion and motion over the pelvis to optimize
reaching and distal control used for athletics and
other tasks (Kibler et al. 2006).
Gross Motor Development
and Prehension
Advances in manual performance, visual percep-
tion, and cognition correspond with object and
environment exploration aided by gross motor
development. With gross motor transitions from
one position to another, the infant strengthens and
stretches various muscle groups that are later used
in prehensile tasks. For instance, shoulder and
trunk musculature are recruited when weight bear-
ing on extended arms, and weight shifting from
the ulnar to radial side of the hand, stretches the
intrinsics. Strength and early prehensile skills nat-
urally develop with tasks executed in prone,
supine, and quadruped, yet once sitting control
develops, prehensile skill improves considerably.
As trunk and upper limb strength expands, the
infant begins to reach to grasp with graded con-
trol. Motor planning, the ability to execute novel
motor acts, and task-specic practice play key
roles in the acquisition of new ne-motor tasks.
Through trial and error, modeling, and practice,
children expand and rene prehensile skill.
Biomechanics and Stabilization
Motor responses of proximal and distal muscula-
ture are viewed as task dependent (Case-Smith
et al. 1989; Schieppati et al. 1996). Generally,
the joint to be stabilized is determined by the
goal of the activity. Anticipatory trunk activation
stabilizes the body during reach-to-grasp move-
ments. Sustained pinch or grip can be viewed as a
32
form of distal stabilization that frees the proximal
joints to move as when we hold a toothbrush and
the wrist, forearm, and elbow are allowed to
move. Many manipulative tasks are best
performed with the wrist stabilized in about
2030 extension and 10 ulnar deviation
(ODriscoll et al. 1992). Wrist extension keeps
the nger exors within the useful range of the
length-tension curve, allowing for adequate ten-
sion during ne-motor tasks. With the wrist
extended, the ngers can ex fully and the
thumb can move into opposition.
The thumb and ngers play different roles in
grasp function. Because of its unique ability to
oppose, the thumb contributes from 40 % to 70 %
of total hand function and is incorporated into
most prehensile patterns (Flatt 1977). The oppos-
ability index (thumb length  100/index [long]
nger length) is an important factor in pulp-
to-pulp contact (Napier 1980). A low opposability
index is found in a child with a thumb distal
phalanx resection, and a high index may be
found in a child who has undergone pollicization
using the former index nger (Netscher et al.
2013). The index nger is the most important
digit after the thumb, given its mobility and inde-
pendent muscle attachments. It accounts for 20 %
of lateral pinch, 20 % of power grip from supina-
tion, and 50 % of power grip from pronation (Raj
and Marquis 1999; Tubiana 1984). The long n-
ger is the strongest and longest digit and for some
individuals replaces the index as the dominant
pointer and manipulator of small objects (Raj
and Marquis 1999). The index and long ngers
are the most stable. The small and ring ngers are
the most mobile yet weakest digits and are
recruited for power grip (Tubiana 1984). Both
the index and small ngers can produce isolated
extension via the extensor indicis proprius and
extensor digiti minimi, respectively. The loss of
any digit will limit prehensile ability to some
degree.
Bimanual Coordination
Bimanual coordination requires temporal and
spatial cooperation of both hands. Bimanual skills
S. Duff
can be separated into symmetrical tasks in which
there is a strong interlimb coupling, as when we
throw a ball with two hands, and asymmetrical
tasks as when one hand stabilizes an object and
the other manipulates it, exemplied by playing
musical instruments such as the guitar. Task goals
and constraints inuence the neural organization
associated with bimanual tasks (Kazennikov and
Wiesendanger 2009).
Bimanual skill develops gradually in infancy
and childhood. Initially, asymmetry dominates as
seen in the asymmetrical tonic neck reex
(ATNR) of a 2-month-old. Greater symmetry is
displayed in the 3-month-old exemplied by mid-
line hand play on the chest. Four-month-olds
often display a bilateral reach for objects in mid-
line. After 5 months, object characteristics deter-
mine whether a reach will be bilateral or
unilateral. The 5-month-old can also loosely
transfer objects between hands, hold a bottle
with two hands, and bang or shake toys. The 6-
to 7-month-olds display a stronger unilateral reach
and a mature hand-to-hand transfer. Despite the
reported tendencies by age, Corbetta and Thelen
(1996) found that most infants seem to move
easily between a unimanual and bimanual pattern
throughout the rst year.
At around 810 months of age, the two hands
begin to embrace different roles. For example, one
hand can hold a toy while the other reaches for a
bottle. By 1218 months of age, differentiated
bimanual movements progress so each hand can
assume an active or stabilizing role. Thus, the
stabilizing hand may hold the base of a block,
while the active hand places another block on
top of it. After 2 years of age and beyond, biman-
ual tasks increase substantially in complexity as
when holding and cutting paper with scissors,
buttoning clothing, and tying shoelaces. As chil-
dren mature into adolescence, interest and experi-
ence further guide the renement of bimanual
skill.
Hand Preference/Dominance
Hand preference is a tendency to use one hand
over the other for prehensile tasks and is well
2 Functional Development
established by 46 years of age. Hand dominance
is the consistent use of one hand over the other to
throw a ball, write with a pencil, and eat with a
fork and is often determined by 67 years of age.
Lateralization of the brain, the process by which
the hemispheres become specialized for particular
functions, is generally thought to be the driving
force behind hand dominance (Sainburg 2005).
The dominant hand displays better dexterity than
the nondominant, yet, it is possible that by altering
the speed and accuracy of a task, performance
between hands may become more similar (Lewis
et al. 2002).
Handwriting
All components of prehension contribute to hand-
writing. The visual-spatial relationships among
the desk, paper, and pencil need to be accurately
perceived for one to reach for and pick up the
pencil, hold the paper, and begin to write.
Sustained grip, in-hand manipulation, and biman-
ual coordination contribute to the task.
Pencil grip is an example of sustained pinch.
The variation in pencil grip found among children
is shown in Fig. 3 (Schneck and Henderson 1990).
With adequate strength and somatosensory feed-
back, a child can sustain a hold on a pencil without
using excess pressure. Pencil grips are considered
efcient if the thumb and index form a circle or
open web space, allowing for skillful distal
manipulation. Inefcient grips demand greater
wrist and elbow movement to control the pencil
and thus reduce speed and uidity. The dynamic
tripod is the most efcient grip in terms of speed
and dexterity since the pencil movement is con-
trolled by the ngers and thumb. The lateral tripod
is an inefcient yet functional grip because the
web space is closed. Up to 25 % of typically
developing children and up to 10 % of adults
employ the lateral tripod (Schneck and Henderson
1990).
In-hand manipulation is often used with pencil
writing. Quick writing and erasing can be
achieved by shifting the pencil in one hand and
rotating it on its axis to use the eraser. Without
sufcient ngertip force control and in-hand
33
Fig. 5 Bimanual adjustment of hold on writing utensil due
to insufcient in-hand manipulation
manipulation ability, a child will often adjust the
position of a pen held in one hand with the con-
tralateral hand (Fig. 5). Bimanual coordination is
required for writing, since one hand must stabilize
the writing surface and the other hand actively
use the pencil. Palmar and key pinch strength
contributes to pencil grip. Grip and pinch strength
increases throughout childhood and contribute to
all prehensile tasks. Normative data for children
and adolescents 619 years old for key pinch
strength can be found in Mathiowetz et al.
(1986). The demands for written work increase
by 89 years of age, necessitating skill in holding
and sustaining a pencil grip while completing the
complex task of handwriting.
Atypical Prehension
Adaptations to System Changes
Skilled prehension helps us perform many every-
day activities. Children of any age need to address
the demand for executing prehensile tasks despite
congenital limb differences or an acquired injury.
Peripheral nerve injury may lead to a reduction
in input to muscle and sensory end organs. Thus,
there may be a greater dependency on muscular
feedback to enhance proprioception. If somato-
sensory feedback is reduced, a tighter grip may
be used to provide the necessary sensory input.
34
Table 4 Atypical prehension
Sample obstacles
Visual regard Diminished visual acuity or scanning ability
Reach and Shoulder pain, upper limb weakness or
grip limited joint range of motion, and weak
formation nger extension
Grasp Finger exor and thumb weakness,
inefcient grading of ngertip forces
Manipulation Weak intrinsics or limited in-hand
coordination
Release Weak nger extensors or spasticity of nger
exors
Fig. 6 Contrast between the pinch patterns of a two-year-old: (a) left unaffected arm/hand and (b) affected arm/hand
with radial dysplasia s/p ulnar centralization and pollicization
A tighter grip may strain joints and increase mus-
cle ber recruitment due to the reduced strength
and contraction speed which is often associated
with nerve injury. If muscle contraction speed is
reduced, dexterity will also be affected. These
muscular changes along with a reduction in sen-
sibility may increase the time needed for task
completion.
Limb deciencies may ensue in response to
atypical constraints placed on a growing upper
limb (Table 4). For example, amniotic bands
may result in partial amputations or tissue damage
to a hand or arm in response to compression and
ischemia (Light and Ogden 1993). Injury to the
brachial plexus at birth contributes to weakness
(Waters 1997) but may also lead to shortness of
the arm if diminished recovery is long-standing
(Bae et al. 2008). Prehension available with con-
genital differences or after injury depends on the
available muscles and adaptations that the child
employs (Fig. 6).
S. Duff
Sample conditions
Weak eyesight, perceptual issues secondary to
neurological injury
Rotator cuff tendinitis, distal humeral or radial head
fracture, brachial plexus injury, median or radial
nerve injury, hemiparesis
Median nerve injury, hemiplegia, absent thumb
Ulnar nerve injury, hemiplegia
Radial nerve injury, wrist ligament injury
Learned nonuse ensues when voluntary limb
movement is suppressed (Taub et al. 1975). This
can develop in children with congenital limb dif-
ferences, brachial plexus injury, hemiplegia, or
other disorders. With learned nonuse, the more
affected arm does not develop efcient prehension
due to neuromuscular, musculoskeletal, or sen-
sory decits and thus is ignored, while tasks are
predominantly performed with the less affected
arm. This condition can inuence bimanual task
performance and independence.
Summary
During daily activities, the primary features of
prehension are employed: visual regard, reach,
grasp, manipulation, and release. Postural control
and bimanual coordination may be used
depending on the task goal and related constraints.
Flexible prehensile skills allow us to adapt actions
2 Functional Development
to constraints and environmental demands while
meeting task goals. The prehensile adaptations
made by children with hand or arm dysfunction
often exceed expectations. The performance of
simple daily tasks frequently become creative
solutions to functional demands. The short- and
long-term benets of these adaptive solutions
must be considered prior to the implementation
of well-intentioned therapeutic and surgical
interventions.
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 Part II
Physical Examination
 Physical Examination
3
Andrea Bauer and Michelle James

Contents Abstract
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 40 The physical examination of the young child
can be difcult, especially for practitioners
Understanding Child Development Stages . . . . . . . . 40
03 Months . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 40
who do not routinely treat children. However,
312 Months . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 40 a good physical exam is vitally important to the
15 Years . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 42 proper evaluation and diagnosis of many pedi-
512 Years . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 42 atric upper extremity conditions. As many of
1318 Years . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 43
these issues also involve other body parts and
General Examination of the Upper Extremity organ systems, a complete physical examina-
in Children . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 43 tion, along with a focused upper extremity
Specic Tests . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 45 examination, is important in all new patients
Tape Measure Test . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 45 to the pediatric hand surgeons ofce. The
Testing for Opposition . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 46
Testing for Nerve Injuries . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 47
development of childrens motor, verbal, and
Home Photos and Videos . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 48 social skills proceeds along a predictable path,
and these developmental milestones can be
Specialized Examination for Children
with Brachial Plexus Birth Palsy . . . . . . . . . . . . . . . . . . . 49 used to the examiners advantage as the exam
is tailored to the age of the child. The structure
Specialized Examination for Children
with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 51 and ow of the upper extremity examination in
children must be exible, and specic exami-
Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 54
nation techniques, such as the use of a retract-
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 54 able tape measure, can be very helpful.
In the specic cases of children with brachial
plexus birth palsy and cerebral palsy, special-
ized examinations have been created to better
standardize the evaluation of these difcult and
heterogeneous conditions. For brachial plexus
birth palsy, these include the Active Movement
Scale (AMS) exam, as well as the modied
Mallet exam. For cerebral palsy, the video-
based Shriners Hospital Upper Extremity Eval-
uation (SHUEE) provides a comprehensive tool
A. Bauer (*) M. James
Shriners Hospitals for Children, Sacramento, CA, USA for evaluating a childs functional use of their
e-mail: anbauer@shrinenet.org; mjames@shrinenet.org upper extremities.
# Springer Science+Business Media New York 2015 39
J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_3
40 A. Bauer and M. James

time period, the infant can already turn toward a

Introduction
For many problems involving the pediatric upper
extremity, a careful physical examination is the
best diagnostic test available. However, patients
who are young, in pain, or in need of a nap can be
difcult partners in this endeavor. This does not
mean that the surgeon should simply give up and
rely on veterinary medicine. Rather, patience
and exibility along with knowledge of anatomy
and child development are all combined to enable
the physician to examine the child appropriately.
This chapter will discuss the stages of child
development and apply them to the examination
of the upper extremity. The technique of the gen-
eral upper extremity exam will be detailed, along
with the specialized exams specic to children
with brachial plexus birth palsy and cerebral
palsy. The entire general physical exam will not
be discussed in detail here. However, it is impor-
tant to note that many children presenting to a
pediatric hand surgeon will have more than just
a hand problem, and so a general physical exam
should be performed on all new patients (Table 1).
Understanding Child Development
Stages
parents voice and attend to a human face. But
they still have open fontanelles, poor head control,
and poor ability to regulate their body tempera-
ture. It can be intimidating for the surgeon who
does not see infants regularly to handle and exam-
ine these fragile little humans under the watchful
eye of their anxious (and sleep-deprived) parents.
The exam should begin with the infant swaddled,
either in their parents arms or on the exam table.
The area of interest can then be exposed for the
exam, while the remainder of the infant remains
covered and warm. Provocative maneuvers and
anything that might cause pain should be reserved
for the end of the examination. Passive range of
motion testing can be a provocative maneuver in
an infant, as they generally do not like for an
extremity to be held or conned, so this testing
should be saved for the end as well.
Observing active use of the upper extremities
can be difcult in newborns. They will tend to
hold the upper extremities with the arms adducted,
elbows exed, forearms pronated, and sts
clenched. Very young infants will not yet reach
for toys and rattles. Instead, active motion can be
elicited by tickling or stroking the area of interest
(such as the back of the hand to elicit nger
extension). Infant reexes can also be used to the
examiners advantage (Table 2).

03 Months
The rst 3 months of life are sometimes referred to
as the fourth trimester, in reference to the dra-
matic transition, the newborn must make from life
inside the uterus to the outside world. During this
312 Months
Infants at this age should be able to engage much
more readily with their environment than the new-
born. Late infancy is a time of rapid development

Table 1 Components of general physical examination

General: Is behavior age-appropriate? Is gait normal and age-appropriate? What percentiles are the childs height and
weight?
Skin: Does the child have axillary freckling, caf au lait spots, or other birth marks or skin lesions?
Head and neck: Does the child have dysmorphic features? Preauricular skin tags? Antihelices present in the ears? Does
the child see and hear normally?
Spine: Is there full motion at the cervical spine? Is the spine straight? Is there a dimple or hairy patch at the base of the
spine?
Lower extremities: Are the hips reduced? Are the thigh creases and leg lengths equal? Are there any foot anomalies?
Neurologic: Are there upper motor neuron signs such as Hoffmans or clonus? Are reexes (including abdominal reex)
normal?
3 Physical Examination 41

Table 2 Infantile reflexes relevant to the upper extremity

examination
Reex
name Description Duration
Moro or Back arches and extremities 46
Startle extend to startling stimulus months
Tonic When the head is turned to 6 months
Neck or one side, the arm on that side
Fencers will extend, while the
opposite arm exes at the
elbow
Grasp Infant grasps ngers around 34
an object placed in the palm months

in both gross and ne motor skills. Although each

childs development may progress differently, Fig. 1 Instinctive palmar grasp. Note lack of thumb
there are general milestones that can be expected: involvement with grasp

Three months: Independent head control,

track objects with eyes, turn head to sound,
smiles
Six months: Roll over, sit unsupported, put
objects in mouth, transfer objects from one
hand to the other
Nine months: Crawl
Twelve months: Pull to stand, cruise (walk
holding on to furniture)

Specic to hand development, infants progress

through a standard pattern of grasp development
during this time (Case-Smith 1995). At 56 months,
an instinctive palmar grasp develops, using the
entire hand as a unit without independent use of
the digits or any meaningful use of the thumb and
without regard for visual input (Fig. 1). The infant
typically secures the object and then adjusts their
grasp accordingly. This is a transitional behavior
between the grasp reex (see Table 2) and more
mature grasp patterns. By 78 months, the infant
develops a more purposeful grasp, most commonly
a raking (or palmar) grasp, in which they can
stabilize the object using the ngers themselves,
rather than by holding the ngers against the palm
(Fig. 2). The 7-month-old typically approaches and
manipulates an object with both hands, grasping
and inspecting it before bringing it to their mouth.
At 912 months, the child begins to use the thumb
independently as the tip pinch (pincer grasp)
Fig. 2 Raking or palmar grasp. More purposeful and
independent use of the ngers and thumb is noted
develops. In the pincer grasp, the thumb and
index nger are opposed pad to pad to hold an
object (Fig. 3).
Toys can be a useful distraction device at this
age and can also assist in the examination. We
often allow a baby to warm up to the ofce
setting and the examiner by providing a few toys
for them to play with independently. This inde-
pendent play allows for careful inspection of hand
function, and the examiner can gradually join in
with the play to encourage specic hand func-
tions. Similarly, once infants begin to eat nger
foods, around 6 months of age, many parents will
have snacks with them which can be a great
42
Fig. 3 Pincer grasp. The
thumb and index nger are
opposed pad to pad to hold
the cookie (Reproduced
with permission from
Shriners Hospitals for
Children Northern
California)
adjunct to the examination. Stranger anxiety pre-
sents at different ages and to a different extent in
each child but generally peaks between 6 and
12 months of age. For this reason, more focused
or difcult portions of the exam of older infants
are often better accomplished with the child in the
parents lap.
15 Years
Growth and development continue rapidly during
this time period. By 18 months, the child should
be able to walk independently, say several intelli-
gible words, and feed themselves with a spoon.
A 2-year-old can run, speak in 23-word
sentences, manipulate large buttons and zippers,
and scribble with a crayon. A 3-year-old can
speak in full sentences, throw and catch a ball,
and hold a crayon with a tripod grip. By 5 years
old, language is more complex, as are ne motor
skills (such as stringing small beads) and the
childs hand dominance is generally established.
Toddlers become accustomed to visiting the
pediatrician for immunizations, so during this
time period they may begin to associate the doc-
tors ofce and all the white coats with getting a
shot. They may start to be intimidated by large
groups of people, such as the myriad of doctors
and trainees at a teaching institution. In addition,
toddlers are increasingly difcult to distract and
win over with a simple toy, so distraction devices
that worked in the past for the infant examination
will not always continue to work. Although some
parents may request that the doctor remove the
A. Bauer and M. James
white coat, this rarely will actually make a differ-
ence in the toddlers perception of the exam, and
appropriate dress is really at the discretion of the
examiner.
Toddlerhood is all about increasing self-
awareness and increasing independence. This
can be used to the examiners advantage by giving
the toddler choices throughout the examination. A
classic mistake is to ask a toddler Can I see your
hand? After this question, any self-respecting
2-year-old will shout No! and hide that hand
from view for the rest of the doctors visit. Instead,
the 2-year-old should be asked Do you want me
to look at this hand rst, or the other one? This
and other choices such as Do you want to sit on
your moms lap or on the table? can help the
toddler feel in control of the situation while you
can proceed with the necessary parts of the
examination.
Even with all of the above, some children of
this age will be simply too difcult to examine
properly on a given day. If this is the case, it is best
to do as much of the examination as possible,
then reassess the situation. At times, a short
break from the exam is all that is needed. At
other times, you will need to document thor-
oughly what you were or were not able to examine
and bring the child back for a repeat examination
on another day.
512 Years
In general, school-aged children are much easier
to examine than children under 5 years of age. The
3 Physical Examination
examination can begin to model a more adult
pattern. For example, children can follow instruc-
tions so that things like motor strength can now be
directly examined. However, children at this age
often require explanations of the various compo-
nents of the examination. To use motor strength
testing as an example, most adults will readily
comply if you ask them to bend your elbow and
dont let me straighten it. While school-aged
children can follow these instructions, the exam-
iner needs to slow down and explain tests more
completely. For more complex tests, demonstrat-
ing with a parent rst can help. For example, you
may say something like Now I need to see how
strong you are. Can you bend your elbow? Now
hold it bent as strong as you can and dont let me
straighten it out.
Modesty also develops during the late elemen-
tary school and middle school years. Things that
adults take for granted, such as men removing
their shirts for a shoulder exam, are often much
more sensitive concepts for children of this age. It
is common for a 10-year-old boy to not want to
take off his shirt for doctors to examine him. If the
childs chest and shoulders need to be visible for
the upper extremity exam, the child should be
given the opportunity to change into a gown
while the examiner waits outside the room. Dur-
ing the exam, only the necessary body part should
be exposed from the gown.
1318 Years
During this time period, childrens developmental
skills are much like adults, but their social and
cognitive skills are still maturing. Modesty
remains an issue and should be addressed as
above. In addition, there may be certain parts of
the examination or interview that are better
addressed without the parents in the room. For
example, a teenage boy presenting with swelling
and pain over the metacarpophalangeal joint may
be reluctant to admit in front of his parents that his
symptoms began after punching someone in the
teeth, information which is vital to the correct
diagnosis and management of his likely joint
infection.
43
The examination can generally proceed as for
adults. However, teenagers are still less accus-
tomed to the doctors ofce than are adults, so
each part of the examination should be explained,
and any tests that may elicit pain should be
discussed beforehand.
General Examination of the Upper
Extremity in Children
As discussed above, the physical examination
may be performed in different orders depending
on the age and cooperation of the child. In general,
the physical exam can be divided into either the
action of the examiner or the system being exam-
ined. See Table 3 for an example of dividing the
upper extremity examination by systems. For chil-
dren, it is best to think of the exam in terms of the
action of the examiner. Although this may make
the exam seem out of order to the surgeon, it
will generally yield the best cooperation from
young patients:
1. Observation: The child is rst observed at rest
and at play in the examination room (Fig. 4).
Age-appropriate toys should be available in the
room even before the examiner enters, so that
the child does not associate the toys with the
exam. While the reason for the visit and history
are discussed with the parents, the surgeon
should keep one eye on the child at play.
Through play, you can see whether both
upper extremities are symmetric in appearance
and whether the child uses both hands equally
or tends to favor one over the other. Consistent
hand preference (handedness) does not usually
appear until age 2 to 3 years (Murray 1995),
so strong preferential use of one hand in a
Table 3 Upper extremity examination by systems
1. Skin: Lesions, abrasions
2. Muscles: Strength testing, atrophy
3. Nerves: Sensory testing, provocative maneuvers
4. Blood vessels: Pulses, capillary rell, Allens test
5. Bones and joints: Range of motion, deformity,
tenderness to palpation
44
Fig. 4 Observing hand function in a child at play in the
ofce (Reproduced with permission from Shriners Hospi-
tals for Children Northern California)
younger child should raise suspicion for a
central nervous system condition, such as
cerebral palsy, affecting the control of the
contralateral hand.
2. Inspection: Any skin abnormalities, such as
lesions which can be associated with congeni-
tal conditions (i.e., caf au lait spots, hemangi-
omas), or signs of trauma (ecchymosis,
swelling, lacerations, abrasions) can be
detected easily by inspection. In addition, care-
ful inspection should include:
a. Number and size of digits. In general, the tip
of the thumb should be almost in line with
the index PIP joint when adducted, and the
ngertip pad of the thumb should be larger
than that of the index nger. The index and
ring ngers should be roughly the same size
and length, with the middle nger being
slightly longer. Digits should be the same
length as the corresponding digits on the
opposite hand.
b. Nail plate and eponychium. Absence of nail
elements may indicate nail-patella syn-
drome or be associated with hypodactyly.
In the case of syndactylized digits, it is
important to note whether the paronychium
is present or will need to be reconstructed
when the ngers are separated.
A. Bauer and M. James
c. Digital exion creases. Absence of digital
exion creases indicates that the affected
joint did not move during prenatal develop-
ment. An extrinsic abnormality, such as in
hypoplastic thumb or arthrogryposis, or joint
abnormality such as in symphalangism, may
cause creases to fail to form. Similarly, chil-
dren with arthrogryposis may have dimin-
ished or absent creases at the wrist and
elbow as well.
d. Muscle bulk or wasting. Absence or wasting
of thenar musculature may indicate median
nerve injury or thumb hypoplasia, while
wasting of interossei may indicate ulnar
nerve injury.
e. Angular and rotational deformities. The
nail plates of the index through small ngers
should all face in the same direction. When
exed, the index through ring ngers should
point generally toward the scaphoid, with-
out overlapping each other. The thumb
should be rotated out of the plane of
the hand.
3. Palpation/percussion: If passive range of
motion testing is not expected to be painful
for the child, it can be performed at this point.
However, in young children or those with con-
tractures, passive range of motion can be pain-
ful and should be saved for later in the
examination. The degree of opening of the
rst web space along with the range of motion
of the digits, wrist, forearm, elbow, and shoul-
der is noted. When measuring wrist range of
motion, it is important to include radial and
ulnar deviation, as these can be affected in
many childrens conditions such as radial or
ulnar deciency, arthrogryposis, and multiple
hereditary exostoses. When measuring forearm
range of motion, the examiner should stabilize
the childs elbow against their side with one
hand and rotate the distal forearm with the
other hand. This prevents compensatory
motion of the shoulder and wrist from affecting
the measured rotation. Actual goniometric
measurement of forearm rotation often requires
a second examiner to work the goniometer
while the rst examiner positions the childs
forearm.
3 Physical Examination
Fig. 5 Fist bump to
demonstrate active nger
exion (Reproduced with
permission from Shriners
Hospitals for Children
Northern California)
4. Tests requiring cooperation: Active range of
motion can be observed at play in younger
children, as discussed above. By using familiar
activities such as a high ve for nger exten-
sion or st bump for nger exion, active
range of motion can be directly tested in chil-
dren as young as 12 years (Fig. 5). Parents can
help in this regard as they will know which
activities are likely to be familiar to their child.
Children over the age of 3 years can generally
participate with testing active range of motion,
resistive motor strength, and sensation; how-
ever, sensation may not be reliable in children
under 8 years of age.
5. Tests that might hurt: Anything that might
produce pain should be saved for the end of the
examination. For the newborn, this generally
includes passive range of motion and provoc-
ative maneuvers on the hips. For older chil-
dren, this may include palpation of a painful
area, joint maneuvers such as the midcarpal
shuck or Watsons scaphoid shift test, and
nerve stimulation such as trying to elicit a
Tinels sign. In the case of fractures, we have
found that percussion of the fracture site rather
than palpation is less likely to provoke anxiety
and is therefore a quicker and less painful way
of testing for clinical union.
Specific Tests
A few specic tests are especially useful in exam-
ining childrens upper limbs:
45
Tape Measure Test
A small retractable tape measure provides a quick,
reproducible assessment of hand function in
young children (James 2005; de Roode
et al. 2010). A box of these is always available
in our hand clinic. First, the examiner shows the
tape measure to the child and demonstrates how
the tape can be pulled out and then retracted by
pushing the button. Then the tape measure is
handed to the child, and the examiner can observe
how they play with it. For reluctant examinees
who dont want to accept the tape measure, it
can be given to the parent to hand to their child,
or the examiner can pull out about a foot of tape,
hold the end of the tape, and let the child reach for
the dangling plastic casing.
As the child plays with the tape measure, it is
simple to assess different grasp and pinch
methods (the way they hold the casing shows
how they grasp larger objects; pushing the button
shows thumb extrinsic function, and grasping
the tab shows tip pinch) (Fig. 6). Children
will usually switch hands spontaneously; if not,
they can be asked to do so or the examiner can
move the tape measure to the opposite hand.
In our experience, the average developmentally
normal 15-month-old can pull out the tape and
retract it using the button. Six- to twelve-month-
old infants are usually more interested in eating
the tape measure than playing with it. Children
with hemiplegia or transverse deciency will g-
ure out how to use it by one of several different
methods; usually, theyll hold it between their
forearm and chest with the affected side and pull
46
Fig. 6 The tape measure test. A. Child demonstrating
grasp of the larger aspect of the tape measure. B. Child
demonstrating tip pinch of the tape portion of the tape
measure (Reproduced with permission from Shriners Hos-
pitals for Children Northern California)
the tab out with the unaffected hand. Two-year-
olds like to pull out the entire tape and then push
the button. A tape measure will keep a 4-year-old
occupied for at least 15 min, measuring every-
thing in the room.
Recovering the tape measure at the end of the
exam is sometimes challenging, but most children
will return it to the examiners pocket when asked
(theyre willing to put it back where it came from,
they just dont want to have it taken away from
them). Occasionally, however, it is easier to let the
child keep the tape measure than wrestle over it.
Testing for Opposition
Opposition of the thumb is a critical concept in
pediatric hand surgery. Hypoplasia of the thumb,
A. Bauer and M. James
preaxial polydactyly, and many other conditions
can affect a childs ability to oppose the thumb. As
children progress through early ne motor devel-
opment, diminished opposition can affect their
ability to form normal grasp and pinch patterns
and manipulate smaller objects. Once they begin
school, children with weakness in opposition will
complain of fatigue with writing, while parents
notice poor penmanship.
The Kapandji score was rst described in 1986
and provides a score of 110 for the amount of
opposition the child can achieve (Kapandji 1986).
A score of 1 is given for the ability to bring the pad
of the thumb to the lateral aspect of the index
middle phalanx, 2 for bringing the pad of the
thumb to the lateral aspect of the index distal
phalanx, and pad to pad opposition to the index,
long, ring, and small ngers earns scores 36.
After this, the examination continues down the
volar aspect of the small nger, such that a score
of 10 is given for bringing the pad of the thumb
to the distal palmar crease in line with the
small nger (Fig. 7). For children who have
undergone a pollicization, we modify this score
to start with a score of 4 for touching the pollicized
index nger pad to pad with the middle nger.
While the Kapandji score can be very useful in
children, the concept of pad to pad opposition is
difcult to explain. We nd that placing small
stickers on the pads of the ngers and asking
the child to squeeze the sticker can reliably
accomplish this test in children over 2 years of
age (Fig. 8). Another method is to draw dots on
each ngertip so that the child can match up the
dots by placing the ngertips in pad to pad
opposition.
In children under 2 years of age, even the use of
stickers and drawing may not be sufcient to
achieve cooperation with a complete Kapandji
scoring. In this case, it is often possible to test
opposition more grossly by holding a pen or
small toy in the center of the childs palm and
encouraging them to touch the object with their
thumb. The ability to oppose the thumb to the
middle of the palm in this manner is a reliable
indicator of sufcient opposition strength, even
when formal pad to pad opposition cannot be
elicited.
3 Physical Examination
Fig. 7 Diagrammatic representation of the Kapandji test
for opposition
Fig. 8 Using a sticker can help a younger child understand
the Kapandji test (Reproduced with permission from
Shriners Hospitals for Children Northern California)
47
Testing for Nerve Injuries
Examining a child with a suspected nerve injury
can be very challenging. Most commonly, a
suspected digital nerve injury occurs along with
recent open trauma. Therefore, the young child
may be reluctant to even have the hand looked at,
let alone participate in a sensory examination.
Three specic tests can be helpful in this scenario.
If the ngers are accessible and the child is able to
cooperate, we nd Semmes-Weinstein testing to
be the most reliable examination for detecting
sensory decits, as well as for following changes
over time. Testing kits are commercially available
which include Semmes-Weinstein monolaments
of varying thicknesses, corresponding to varying
pressures and hence varying degrees of sensory
loss. We have the child close their eyes, then
respond with yes or no whether they are able to
feel the monolament. Often, the test will need to
be repeated several times in different locations to
account for any lucky guesses.
When the child is either too young or too
scared to cooperate with this type of testing, two
other options are available. First, simple inspec-
tion and palpation of the ngertips can alert the
examiner to a nerve injury. Since normal skin
hydrosis is mediated by the sensory nerves, n-
gers in the distribution of an injured nerve will feel
drier and rougher than the other ngers, and the
skin will have a dry appearance (Fig. 9). Second,
if even simple inspection is not feasible, it is
possible to test for a nerve injury by immersing
the hand in water. The skin wrinkling that occurs
in water is mediated by afferent nerves, so the
ngers affected by the nerve injury will not wrin-
kle (Wilder-Smith 2004) (Fig. 10). This test is
especially useful in the case of recent open inju-
ries, as many children will need to soak their hand
in the ofce to allow the dressings to be removed
anyway. Following this soaking, a quick look at
the ngertips to conrm equal wrinkling of all
digits can settle the question of a nerve injury.
The skin wrinkling test is not sufcient to follow
nerve recovery after repair, however, as there is no
established timeline for how and when this phe-
nomenon returns. Hsieh and colleagues demon-
strated that this test remains abnormal more than a
48 A. Bauer and M. James

Fig. 9 Note the tapered

and dry appearance of the
thumb, index, and middle
ngertips of the left hand in
this child with a chronic
median nerve injury
(Reproduced with
permission from Shriners
Hospitals for Children
Northern California)

Fig. 10 After water immersion. Note that skin wrinkling

has taken place in the thumb, index, and middle ngers.
There is some wrinkling in the ring nger but none in the
small nger in this child with an ulnar nerve injury
(Reproduced with permission from Shriners Hospitals for
Children Northern California)

year after replantation for complete digital ampu-

tations (Hsieh et al. 2006).

Home Photos and Videos
Lastly, some children, especially between the ages
of 1 and 3 years, are simply more difcult to
examine in the ofce setting than others. If the
desired motion or activity cannot be elicited in the
ofce despite all the tips and tricks discussed
above, the childs parents or occupational therapist
may be able to record the activity in a more com-
fortable setting. There are several children in our
Fig. 11 Photo taken during an occupational therapy ses-
sion demonstrating overhead reach in a child with brachial
plexus birth palsy
practice who routinely come to ofce visits with
photographs or videos of range of motion taken in
therapy sessions (Fig. 11). In addition, old family
photographs or videos can help parents determine
when a particular problem started, such as a thumb
exion contracture related to a trigger thumb.
3 Physical Examination
Specialized Examination for Children
with Brachial Plexus Birth Palsy
Brachial plexus birth palsy (BPBP) encompasses
a heterogeneous group of birth injuries, with dif-
ferent manifestations that present in various ways
throughout childhood. For these children, the
physical exam is often the best way to evaluate
the severity of the injury and which nerve roots are
likely to be involved. General age-appropriate
examination techniques for the upper extremity,
as discussed above, are used here as well. In
addition, several specic examination techniques
and scoring systems have been developed to better
assess children with BPBP.
The Active Movement Scale (AMS) was
developed at the Hospital for Sick Children in
Toronto and rst published in 2002 (Curtis
et al. 2002). This system is best used for evaluat-
ing infants and young children, as it does not
require the child to perform any specic activities
or follow commands. Rather, through a combina-
tion of positioning the child and observing them at
play, the examiner records the status of 15 active
movements of the upper extremity, each of which
is graded on a scale of 07 (Fig. 12). Each move-
ment is rst examined with gravity eliminated,
and if full motion (within the childs available
range of motion for that joint) is achieved, that
movement is then graded against gravity. In our
experience, the most difcult movement to grade
is supination. Although not part of the original
authors description, we nd it easiest to grade
24 as the amount of motion between full prona-
tion and the neutral position, and 57 as the
amount of motion between neutral and full supi-
nation. Placing a sticker on the childs palm or
volar forearm is often the best way to test active
supination in young children, as they will almost
always try to look for the sticker by attempting
supination.
There are a few additional nuances to the appro-
priate performance of the AMS examination:
Movement is assessed within the
age-appropriate range of motion, using the
uninvolved contralateral limb as a control.
49
Wrist extension should be tested while the
child is grasping a small object such as a
crayon, so that they cannot substitute for the
movement with nger extension.
Finger extension is evaluated as extension of
the metacarpophalangeal joints.
Finger exion is evaluated as the distance at
rest between the ngertips and the palm
In our hands, this test is best performed with
two examiners, generally two physicians or a
physician and an occupational therapist. This
allows one examiner to observe and record the
motions while the other positions the child and
offers toys (Fig. 13). It also allows for exibility in
the examination, as the child may prefer one
examiner, or a certain motion might be better
observed from one angle in the room versus
another.
Several movements of the AMS have been
collected in a simplied manner to create the
Toronto Test Score (Michelow et al. 1994). In
this score, elbow exion, elbow extension, wrist
extension, nger extension, and thumb extension
are each graded on a scale of 02, for a total
possible combined score of 10. The authors
found that an infants score on this test at 3 months
of age could accurately predict their recovery at
12 months of age. A score at 3 months of age less
than 3.5 out of 10 predicts poor recovery at
12 months, while a score of greater than 3.5 pre-
dicts good recovery. The authors suggest that
although all infants should be followed closely
as their recovery progresses, those with a score
over 3.5 at 3 months of age are unlikely to
need microsurgical intervention (Michelow
et al. 1994). The cookie test is a useful adjunct to
the AMS and Toronto scores in determining an
infants need for microsurgical plexus reconstruc-
tion. It was developed in Toronto and most
recently described by Borschel and Clarke
(2009). The test consists of placing a cookie in
the childs hand while holding the humerus to
the childs side. If the child is able to bring the
cookie to the mouth without exing the neck
beyond 45 , they have passed the cookie test
and are not likely to require microsurgical
intervention.
50 A. Bauer and M. James

Fig. 12 Worksheet for performing AMS examination

For older children with brachial plexus birth on functional activities that require shoulder
palsy, the Mallet score is a commonly used test of motion (Mallet 1972). The Mallet score requires
shoulder function. This test focuses specically active cooperation of the child and measures
3 Physical Examination
Fig. 13 Two examiners
performing the AMS
examination. The child is
demonstrating active
shoulder abduction and
wrist extension against
gravity (Reproduced
with permission from
Shriners Hospitals for
Children Northern
California)
active abduction, external rotation, hand-to-neck,
hand-to-mouth, and hand-to-spine activities. The
original score has been modied to include inter-
nal rotation to the front of the body as well (Abzug
et al. 2010). The score is calculated by grading
each activity on a scale of 05 (Fig. 14).
All of the above physical exam scores are used
routinely in the evaluation of children with bra-
chial plexus birth palsy. The reliability and valid-
ity of these tests have been evaluated by Bae and
colleagues (2003). The authors found excellent
interobserver reliability for the individual compo-
nents of the Mallet score and the AMS score, and
interobserver reliability of the Toronto Test Score
was rated as good. The same authors also studied
the ability of these exam scores to predict quality
of life in children with brachial plexus birth palsy,
as measured by the Pediatric Outcomes Data Col-
lection Instrument (PODCI). They found that all
three scores (Mallet, Toronto Test Score, and
Active Movement Scale) could predict the global
function, upper extremity function, and sports/
physical function domains of the PODCI (Bae
et al. 2008).
In our clinic, the Toronto Test Score is used for
infants being considered for microsurgical bra-
chial plexus exploration. The Active Movement
Scale is also recorded for these infants, and we
continue to evaluate children using this scale until
the age of 3 years, so that the results of early
51
surgical interventions can be assessed. We have
found it difcult to record the Mallet score reliably
in children under the age of 3, so we begin using
that score at 3 years of age and continue through-
out childhood. In addition, routine assessment of
active and passive range of motion, strength, and
sensation at each ofce visit is important as chil-
dren with brachial plexus birth palsy grow. In
particular, we examine the passive range of
motion of the shoulder, elbow, and forearm at
each visit to monitor for the development of
contractures.
Specialized Examination for Children
with Cerebral Palsy
Examining a child with cerebral palsy can be
intimidating, and it can be difcult to know
where to start. In general, upper extremity inter-
ventions are not commonly done early in life for
cerebral palsy, and therefore the upper extremity
specialist will tend to see children with cerebral
palsy who are of school age and older. At these
ages, most children will be able to cooperate read-
ily with the examination. It is helpful to rst put
their upper extremity issues in the context of their
overall condition. Cerebral palsy is broadly char-
acterized by the Gross Motor Function Classica-
tion System (GMFCS), which places children into
52 A. Bauer and M. James

Fig. 14 The Mallet a MALLET EVALUATION FORM

examination. (a) Worksheet
for performing Mallet Involved side: Date:
examination. L R Therapist
Initials:
(b) Demonstration of the
external rotation component Global Abduction
of the Mallet score. The left
arm in this case would be Global External Rotation
given a score of 2, for
Hand to neck
achieving less than
0 external rotation Hand to spine
(Reproduced with
permission from Hand to mouth
Shriners Hospitals for
Children Northern
MALLET CLASSIFICATION
California)
Mallet classification (Grade I = no function, Grade V = normal function)
(Enter 0) (Enter 1) (Enter 2) (Enter 3) (Enter 4) (Enter 5)
Not Testable Grade I Grade II Grade III Grade IV Grade V

a. Global Not Testable No function Normal

Abduction <30 30 to 90 >90

b. Global External Not Testable No function Normal

<0 0 to 20 >20
Rotation

c. Hand to neck Not Testable No function Normal

Not possible Difficult Easy

d. Hand on spine Not Testable No function Normal

Not possible S1 T12

e. Hand to mouth Not Testable No function Normal

Marked trumpet Partial trumpet <40 ol
sign sign abduction

b
3 Physical Examination
categories based on their gross motor function,
primarily as it relates to ambulation (Table 4).
Although this scale does not specically apply to
the upper extremity, it is a useful start to under-
standing a childs overall level of function.
In addition, it is important to know which
limbs are affected and to what extent, as well as
the type of motor involvement. Affected limbs can
range from monoplegia or very mild hemiplegia
to quadriplegia with further involvement of the
head and trunk. The types of motor involvement
can be spastic, athetoid, dystonic, or a combina-
tion of these. Pure spastic cerebral palsy yields the
most predictable results with interventions such as
tendon transfers, while other types of motor
impairment may respond better to joint stabiliza-
tion procedures.
Table 4 GMFCS classification of cerebral palsy
GMFCS
level Description
I Walk indoors and outdoors, climb stairs,
run and jump
II Walk indoors and outdoors, climb stairs
with a railing, trouble with uneven ground
III Walk on a level surface with an assistive
device, wheelchair for long distances
IV May walk short distances with a walker but
primarily use wheelchair for ambulation
V No independent mobility, limited control of
head and trunk position
Fig. 15 Setup for
stereognosis examination
53
In the clinic setting, active and passive range of
motion of the upper extremities are examined,
taking note of which motions the child is able to
actively control. In addition to limitations in
motor control, the sensory functions of
stereognosis, two-point discrimination, and pro-
prioception are often impaired in cerebral palsy.
Two-point discrimination and proprioception are
readily tested in the clinic setting. Stereognosis is
more difcult to assess but can be accomplished
by placing small, familiar objects in a bag (such as
a coin, a key, and a button) and asking the child to
identify the objects using their affected hand only
(Fig. 15). In more severely affected children, the
skin is also evaluated carefully, as contractures
can lead to skin irritation and infections, as well
as hygiene problems for caregivers.
For children with cerebral palsy, asking them
to move their hands and arms in a specic way
through active range of motion testing is a good
start, but it is often more helpful to understand
their patterns of use in activities. Therefore, in the
evaluation of lower extremity function, standard-
ized gait analysis is now routinely performed by
many pediatric orthopedic surgeons. However,
using patterns of the upper extremity are more
complex and variable than the gait cycle. This
makes it more difcult to use motion analysis of
the upper extremity in routine clinical practice.
Instead, there are several clinician-based func-
tional performance evaluations that are used rou-
tinely (Wagner and Davids 2012). Probably the
54
Table 5 House scale of voluntary use
Class Description
0 Does not use
1 Poor passive assist
2 Fair passive assist
3 Good passive assist
4 Poor active assist
5 Fair active assist
6 Good active assist
7 Partial spontaneous use
8 Complete spontaneous use
most familiar of these is the House scale of vol-
untary use (Table 5). As initially described by
House, the scale does not require any specic
equipment or tasks, it is merely a description of
the overall functional status observed by the
patient, family, surgeon, and therapist. The com-
bined assessment of a childs sensory capabilities
and voluntary use category can reasonably predict
their expected response to surgical intervention.
The Shriners Hospital Upper Extremity Evalu-
ation (SHUEE) was developed in 1996 and rst
published in the literature in 2006 (Davids
et al. 2006). This is a videotaped activity-based
test that builds on Houses concept of voluntary
functional use. The rst section of the SHUEE is a
standardized evaluation of active and passive
range of motion and spasticity. Next, there is a
subjective assessment of the patients ability to
perform seven activities of daily living. The nal
section is the functional assessment. This consists
of an evaluation of spontaneous use during nine
common tasks using a modied House scale, a
dynamic positional analysis of the affected limb
during 16 tasks, and a grasp and release analysis
with the wrist held in exion, neutral, and exten-
sion. At our institution, all children with cerebral
palsy who are being considered for upper extrem-
ity procedures undergo a videotaped SHUEE
evaluation with an occupational therapist, which
is then reviewed by the surgeon and therapist
together before deciding on a treatment plan
(Fig. 16).
These functional tests can be performed with
and without a splint or brace in place to assess
whether the splint is helpful to the child. We do
A. Bauer and M. James
Fig. 16 A child using their affected hand to actively assist
stringing a bead during an SHUEE
this routinely when planning an operation such as
a wrist fusion, to understand preoperatively
whether we can expect the childs function to
improve after the surgery. In addition, tests can
be performed before and after a trial of Botox, to
understand whether a surgical release or length-
ening of a specic muscle will help to improve
function.
Summary
The pediatric upper extremity examination requires
knowledge of child development and anatomy,
along with a good deal of patience. It is best to let
the childs developmental stage lead the direction
and ow of the examination. Props and distractions
such as toys, stickers, and, our favorite, the tape
measure can be very helpful. In the specic cases of
brachial plexus birth palsy and cerebral palsy, stan-
dardized, validated scores are available which can
help place the childs function along the spectrum
of their diagnosis while offering guidance about
their expected response to an intervention.
References
Abzug JM, Chafetz RS, Gaughan JP, Ashworth S, Kozin
SH. Shoulder function after medial approach and
derotational humeral osteotomy in patients with bra-
chial plexus birth palsy. J Pediatr Ortho.
2010;30:46974.
3 Physical Examination
Bae DS, Waters PM, Zurakowski D. Reliability of three
classication systems measuring active motion in bra-
chial plexus birth palsy. J Bone Joint Surg
Am. 2003;85-A(9):17338.
Bae DS, Waters PM, Zurakowski D. Correlation of pediat-
ric outcomes data collection instrument with measures
of active movement in children with brachial plexus
birth palsy. J Pediatr Orthop. 2008;28:58492.
Borschel GH, Clarke HM. Obstetrical brachial plexus
palsy. Plast Reconstr Surg. 2009;124
(1 suppl):144e55e.
Case-Smith J. Grasp, release, and bimanual skills in the
rst two years of life. In: Henderson A, Pehoski C,
editors. Hand function in the child: foundations for
remediation. St. Louis MO: Mosby Year Book; 1995.
p. 11335.
Curtis C, Stephens D, Clarke HM, et al. The active move-
ment scale: an evaluative tool for infants with obstetri-
cal brachial plexus palsy. J Hand Surg.
2002;27A:4708.
Davids JR, Peace LC, Wagner LV, et al. Validation of the
Shriners hospital for children upper extremity evalua-
tion (SHUEE) for children with hemiplegic cerebral
palsy. J Bone Joint Surg Am. 2006;88(2):32633.
de Roode CP, James MA, McCarroll Jr HR. Abductor digiti
minimi opponensplasty: technique, modications, and
measurement of opposition. Tech Hand Surg.
2010;14:513.
55
Hsieh CH, Huang KF, LiLiang PC, et al. Paradoxical
response to water immersion in replanted ngers. Clin
Auton Res. 2006;16(3):2237.
James MA (2005) The retractable tape measure in pediatric
hand exams. Correspondence News of the American
Society for Surgery of the Hand. October 2005, issue
no. 127.
Kapandji A. Clinical test of apposition and counter-
apposition of the thumb. Ann Chir Main. 1986;5
(1):6773.
Mallet J. Paralysie obsttricale du plexus brachial. Traitement
des squelles. Primaut du traitment de lpaule
Mthode dexpression des rsultats. Rev Chir Orthop
Reparatric Appar Mot. 1972;58 suppl 1:1668.
Michelow BJ, Clarke HM, Curtis CG, et al. The natural
history of obstetrical brachial plexus palsy. Plast
Reconstr Surg. 1994;93(4):67580.
Murray EA. Hand preference and its development. In:
Henderson A, Pehoski C, editors. Hand function in
the child: foundations for remediation. St. Louis:
Mosby Year Book; 1995. p. 15463.
Wagner LV, Davids JR. Assessment tools and classication
systems used for the upper extremity in children with
cerebral palsy. Clin Orthop Relat Res. 2012;470
(5):125771.
Wilder-Smith EP. Water immersion wrinkling physiol-
ogy and use as an indicator of sympathetic function.
Clin Auton Res. 2004;14(2):12531.
 Outcome Measures
4
M. J. Mulcahey and Scott H. Kozin

Contents Abstract
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 57 The assessment of children with upper extrem-
ity (UE) impairments is a process that involves
Considerations in Selection of Outcome
Instruments . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 61
the use of diagnostic testing, standardized
observation, performance measures, and
Functional Performance Measures . . . . . . . . . . . . . . . . . 62 patient-reported outcome (PRO) instruments.
Patient-Reported Outcome Instruments . . . . . . . . . . . 63 Purposes for assessment include screening
Individual Patient-Reported Outcomes . . . . . . . . . . . . 65 and obtaining diagnoses, benchmarking and
reimbursement, goal setting and prioritization
Computer Adaptive Tests . . . . . . . . . . . . . . . . . . . . . . . . . . . 66
of treatment goals, longitudinal monitoring,
Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 68 building evidence in support of treatment, and
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 68 evaluating and comparing outcomes of routine
care. This chapter provides a description of
criterion and norm-referenced instruments,
describes psychometric properties associated
with outcome instruments, provides an over-
view of research literature on outcome instru-
ments that have particular relevance to the
pediatric UE and are reported on repeatedly in
research literature, and discusses computer
adaptive testing (CAT) as an emerging mea-
surement technology.

Introduction

The assessment of children with upper extremity

M.J. Mulcahey (*) (UE) impairments is a process that involves the
Jefferson School of Health Professions, Thomas Jefferson
use of diagnostic testing, standardized observa-
University, Philadelphia, PA, USA
e-mail: maryjane.mulcahey@jefferson.edu tion, performance measures, and patient-reported
outcome (PRO) instruments. Purposes for assess-
S.H. Kozin
Shriners Hospitals for Children, Philadelphia, PA, USA ment include screening and obtaining diagnoses,
e-mail: skozin@shrinenet.org benchmarking and reimbursement, goal setting
# Springer Science+Business Media New York 2015 57
J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_4
58 M.J. Mulcahey and S.H. Kozin

and prioritization of treatment goals, longitudinal
monitoring, building evidence in support of treat-
ment, and evaluating and comparing outcomes of
routine care. Although they may not be specic to
the upper limb or to children, there are excellent
resources for clinicians to aid in the selection of
outcome instruments (Dorerick et al. 2005;
Hoang-Kim 2011; Slobogean et al. 2011; Smith
et al. 2012, http://www.rehabmeasures.org; http://
www.scireproject.com; http://www.proqolid.org/
about_proqolid; http://www.neuroqol.org; http://
www.nihpromis.org). As summarized in Table 1,
there are also notable critical and systematic
reviews of outcome instruments for children
with cerebral palsy (CP) (Klingels et al. 2010;
Wagner and Davids 2012; Gilmore et al. 2010;
Harvey et al. 2008; Sakzewski et al. 2007), bra-
chial plexus birth palsy (Chang et al. 2013;
Bialocerkowski et al. 2013), limb deciency
(Burger et al. 2004; Christakou and Laiou 2013;
Buffart et al. 2007a; Lindner et al. 2010; Wright
2009), rheumatic diseases (Kleeper 2011), and
muscular dystrophy (Mazzone et al. 2012).
This chapter provides a description of criterion
and norm-referenced instruments, describes psy-
chometric properties associated with outcome
instruments, provides an overview of research
literature on outcome instruments that have par-
ticular relevance to the pediatric UE and are
reported on repeatedly in research literature, and
discusses computer adaptive testing (CAT) as an
emerging measurement technology. Although
they may be used to evaluate outcomes, classi-
cation systems such as the Manual Ability Classi-
cation System (MACS) (Eliasson et al. 2006) for
children with CP, the Mallet Classication and
Active Movement Scale (AMS) (Bae et al. 2003)
for children with BPBP, and the International

Table 1 Summary of notable studies on outcome instruments for children with upper extremity (UE) impairments
Study Design Instruments Population(s) Salient ndings
Bialocerkowski Systematic Multiple instruments Children and PODCI, PEDI, and AHA
et al. (2007) review including AHA, Arm and adolescents have the strongest
Hand Function, Brachial with BPBP psychometric properties for
Plexus Outcome Measure, BPBP
PEDI, PODCI, MUUL Additional psychometric
studies are needed for more
robust measures
Buffart et al. (a) Repeated AHA, PUFI, ABILHAND Children, age Each of the measures has
measures, 412, with good psychometric properties
psychometric radial for children with RD types
study deciency IIV
(RD), types
IIV
Unilateral and AHA and PUFI have the
bilateral strongest correlation with
type of RD, hand grip, and
global assessment of hand
function
No instrument is optimal
Burger Cross- UNB, CAPP-FSIP, CAPP- Children with No instrument is optimal
et al. (2004) sectional, FSI limb
psychometric deciency
study
Chang Systematic Multiple instruments Children with Serious void in validated
et al. (2013) review including AHA, BPBP instruments for population
ABILHAND, PEDI, PODCI with BPBP
Disparity in use of
classication systems and
instruments limiting ability to
evaluate outcomes
(continued)
4 Outcome Measures 59

Table 1 (continued)
Study Design Instruments Population(s) Salient ndings
Christakou and Critical PODCI and ASK Children with ASK has the stronger
Laiou (2013) review orthopedic psychometric properties when
impairment compared to PODCI
Further psychometric work is
needed on both instruments
Gilmore Systematic Multiple instruments Children, age The best performance
et al. (2010) review including MUUL, AHA, 516, with measure with strong
ABILHAND, QUEST, hemiplegic psychometric properties for
SHUEE cerebral palsy bimanual function is AHA
The best performance
measure with strong
psychometric properties for
unilateral hand function is
MUUL
ABILHAND has strong
psychometric properties and
is both a measure of capability
and parent report
Multiple measures may be
needed based on purpose of
use
Harvey Systematic Multiple instruments Children with ASK and GMFM have the
et al. (2008)a review including PODCI, PEDI, cerebral palsy strongest psychometric
ASK properties for measuring
activity limitation
No one instrument addresses
all aspects of the ICF; thus
multiple instruments should
be selected based on
psychometric properties and
purpose of measurement
Kleeper (2011) Critical CHA-Q, ASK, PODCI, Children with Each of the measures contains
review JAFAS rheumatic activities relevant to children
disease across broad age range
ASK is the only instrument
that is child report
PODCI is the most
comprehensive
Using two or more of these
measures is recommended to
obtain the best understanding
of childs functioning
Klingels Systematic Multiple instruments Children, age MUUL is recommended for
et al. (2010) review including QUEST, SHUEE, 218 years, capacity measure
AHA, MUUL, PEDI, with AHA is recommended for
ABILHAND hemiplegic performance measure
cerebral palsy ABILHAND is
recommended for patient-
reported outcome instrument
(continued)
60 M.J. Mulcahey and S.H. Kozin

Table 1 (continued)
Study Design Instruments Population(s) Salient ndings
Lindner Critical Multiple pediatric and adult Children and Multiple instruments are
et al. (2010) review instruments including PUFI, adults using needed to assess the
UBET, CAPP-FSI, CAPP- upper limb constructs across the ICF
PSI prostheses The majority of pediatric
instruments measure activity
and participation domains of
ICF
Assessment with children
requires measures that
evaluate social interactions
Mazzone Critical Multiple instruments Children with Many of the instruments
et al. (2012) review including ABILHAND, Self- muscular reviewed are
reported Scale of Activity dystrophy psychometrically sound,
Limitation, Muscular (MD) although not with samples of
Dystrophy Functional Rating children with MD
Scale, Jebsen Test of Hand None covers the full
Function, Upper Limb ability range of children with
Functional Ability Test MD (ceiling and oor
potential)
Sakzewski Systematic Multiple instruments Children with All instruments measure
et al. (2007)a review including COPM, GAS CP aspects of childhood
participation
No one instrument covers all
aspects of participation
Responsiveness of all
instruments is unknown
Wagner and Systematic Multiple instruments Children with The understanding of
Davids (2012) review including AHA, BB, MUUL, CP psychometric properties will
QUEST, SHUEE, PEDI- assist clinicians with selecting
PODCI, ASK, COPM, GAS the most useful instruments
based on purpose of
measurement
Wright (2009) Systematic Multiple instrument Adult and Comprehensive summary of
review including AHA, PUFI, pediatric instruments used with adults
ABILHAND, CAPP-FSI, prosthetic and children with limb
PODCI users deciency
Measures have varying
degree of psychometric
properties
Further research is needed
AHA Assisting Hand Assessment, PEDI Pediatric Evaluation of Disability Inventory, PODCI Pediatric Outcomes Data
Collection Instrument, MUUL Melbourne Assessment of Unilateral Upper Limb Function, UNB University of New
Brunswick Test of Prosthetic Function, PUFI Prosthetic Upper Extremity Functional Index, CAPP-FSIP Child Amputee
Prosthetics Project-Functional Status Inventory for Preschool Children, CAPP-FSI Child Amputee Prosthetics Project-
Functional Status Inventory, ASK Activities Scale for Kids, QUEST Quality of Upper Extremity Skills Test, SHUEE
Shriners Hospitals for Children Upper Extremity Evaluation, C-HAQ Childhood Health Assessment Questionnaire,
JAFAS Juvenile Arthritis Functional Assessment Scale, COPM Canadian Occupational Performance Measure, GAS
Goal Attainment Scaling, BB Box and Block Test
a
Studies that included outcome instruments not directly related to UE but include COPM and GAS, two instruments/
methods discussed in this chapter and have high relevance to evaluation of children with UE impairments
4 Outcome Measures
Standards for Neurological Classication of Spi-
nal Cord Injury (ISNCSCI) (Kirshblum
et al. 2011) and Classication of the Upper
Extremity in Tetraplegia (Mulcahey and Weiss
2008) for children with spinal cord injury (SCI)
are not outcome instruments per se and thus will
not be discussed in this chapter. Methods such as
imaging and electrodiagnostics as well as physical
examination measures for muscle strength, sensi-
bility, joint range of motion, pain, and spasticity
are beyond the scope of this chapter but can be
found in chapters within this text and in other
excellent resources (Platz et al. 2008; Van den
Beld et al. 2011; Gajdosik 2005; Mulcahey
et al. 2007; Koman et al. 2008).
Considerations in Selection
of Outcome Instruments
Generally, there are two types of instruments that
differ in how scores are interpreted. Criterion-
referenced tests are those for which the test score
is interpreted relative to a continuum of possible
scores that represents some level of performance
(Hinderer and Hinderer 2005; Portney and
Watkins 2009). In contrast, norm-referenced
tests measure performance that is interpretable in
terms of the individual relative to performance of
some known group (Hinderer and Hinderer 2005).
An excellent example of norm-referenced tests is
the developmental motor scales where scores are
interpreted against normal development. With
norm-referenced instruments, the mean of the dis-
tribution of the scores from the reference sample is
used as the standard and the variability is used to
determine how an individual performs relative to
the reference sample (Portney and Watkins 2009).
Norm-referenced tests are usually used for diag-
noses, while criterion-referenced tests are used to
examine prociency of performance along a con-
tinuum, for example, a continuum of cannot do
to can do, and are felt to be more useful for
developing and evaluating rehabilitation out-
comes (Portney and Watkins 2009).
In addition to understanding the distinction
between criterion- and norm-referenced tests,
informed use of an outcome instrument requires
61
the understanding of the psychometric properties
of the instrument, particularly when the scores are
used for decisions related to treatment and reim-
bursement. These properties include reliability,
validity, and responsiveness. Reliability is
concerned with the degree to which an instrument
can distinguish differences among persons,
despite measurement error. The reliability of
scores on individual test items can be determined
using the kappa and weighted kappa coefcients
for nominal and ordinal level data, respectively
(Portney and Watkiins 2009). Reliability of total
scores is usually determined by the Intraclass Cor-
relation Coefcient (ICC) (Streiner and Norman
1995). Internal consistency reects the degree to
which items within a scale measure a single con-
struct and is usually assessed using Cronbachs
alpha (Portney and Watkins 2007). Agreement,
which is a form of reliability, is concerned with
how close the scores are on repeated testing in
stable conditions (De Vet et al. 2006) and is a
fundamental property if an instrument is used to
detect change or determine treatment effective-
ness. The ICC is recommended for studies of
agreement and reproducibility. Interpretation of
reliability estimates is not standardized but rather
based on the context of the study and instrument
(Portney and Watkins 2007; Streiner and Norman
1995). While reliability estimates of 0.7 and 0.9
are recommended for outcome instruments
(Fitzpatirck et al. 1998), reliability estimates
higher than 0.9 are preferred (Portney and
Watkins 2007).
Measurement validity concerns the extent to
which an instrument measures what it is intended
to measure. Validity places an emphasis on the
objectives of the instrument and the ability to
make inferences from the test scores (Portney
and Watkins 2007). Face and content validities
are qualitative characteristics that indicate the
instrument appears to measure what it is intended
to measure (face) and that the instrument ade-
quately covers the domain of interest (validity).
Further evidence of face and content validities can
be obtained from an understanding about how the
test items were developed and eld-tested (Guyatt
and Cook 1994); development of test items should
include content experts, including people who
62
represent the intended responder (i.e., children
with upper extremity impairments), and the
items and response scale should undergo iterative
cognitive testing, as conducted by Dumas
et al. (2008) and Mulcahey et al. (2009, 2011).
Validity can be quantitatively evaluated by com-
paring scores of a new instrument to scores of a
similar, traditional, or gold standard instrument
(criterion validity); by evaluating differences in
scores among known groups (discriminant valid-
ity); or by evaluating if there are expected associ-
ations of scores with scores from instruments
measuring similar attributes (convergent validity)
or different attributes (divergent validity).
Responsiveness of an instrument addresses the
degree to which an instrument is capable of
detecting important changes in health status.
While there is not consensus on the best method
to establish responsiveness, common approaches
include calculating the effect size, standard
response mean (SEM), and minimally important
difference (MID) (Guyatt et al. 2002). Most
researchers use Cohens interpretation of effect
size whereby values of .5 reect a moderate effect
and values of .8 reect a large effect (Portney and
Watkins 2007). There is ongoing dialogue about
how best to interpret meaningful change mea-
sured by PRO instruments (McLeod et al. 2011;
Wyrwich et al. 2013).
Functional Performance Measures
Functional performance measures refer to upper
extremity assessments that require actual perfor-
mance of arm and hand tasks. Usually, these mea-
sures are administered by a trained therapist and
have procedural guidelines for scoring and inter-
pretation; they can be criterion or norm
referenced. There are many upper extremity per-
formance measures (http://www.rehabmeasures.
org; http://www.scireproject.com), and while
some may be used with children, most have been
developed and eld-tested using adult clinical
samples.
The Jebsen Test of Hand Function (Jebsen
et al. 1969) and the Box and Block Test
(Mathiowetz et al. 1985) are non-categorical or
M.J. Mulcahey and S.H. Kozin
generic (not disease specic) upper extremity per-
formance measures. The Jebsen Test of Hand
Function is a timed test of hand dexterity that
was originally established for adults (Jebsen
et al. 1969) and subsequently eld-tested in chil-
dren (Taylor et al. 1973). It requires manipulation
of objects that reect everyday tasks and one
writing task. Despite its use with children with
varying diagnoses (Noronha et al. 1989;
Mulcahey et al. 1995; Aliu et al. 2008; Klingels
et al. 2013; Netscher et al. 2013; Lee et al. 2013a;
Shingade et al. 2014), sound psychometric studies
in samples of pediatric populations with upper
extremity impairments are lacking (Gilmore
et al. 2010). One study (Hiller and Wade 1992)
established the discriminative validity of the
Jebsen Test of Hand Function in children with
Duchenne muscular dystrophy. In studies by
Brandao et al. (2013), Shingade et al. (2014),
and Lee et al. (2013b), the scores on the Jebsen
Test of Hand Function were responsive to pediat-
ric treatment, but others (Mulcahey et al. 1995;
Staines et al. 2008; Aliu et al. 2008; Netscher
et al. 2013; Noronha et al. 1989) reported limita-
tions to the Jebsen Test of Hand Function when
used with children. BovendEerdt et al. (2004)
described a modied Jebsen Test of Hand Func-
tion in which the number of items was reduced
from seven to three items; a review of the litera-
ture did not reveal widespread use of the modied
Jebsen Test of Hand Function.
The Box and Block Test (Mathiowetz
et al. 1985a) is another generic performance mea-
sure that evaluates unilateral hand function as
assessed by the number of blocks acquired, car-
ried, and released in 1 minute. Although the
majority of psychometric studies have been
conducted with adults with neurologic and ortho-
pedic impairments (Chen et al. 2009; Desrosiers
et al. 1994; Lin et al. 2010; Platz et al. 2008),
studies have also been done with children.
Jongbloed-Pereboom (2013) established norms
for children between 3 and 10 years of age;
Mulcahey (2012a) showed that the Box and
Block Test had strong discriminant validity in
children with BPBP, noting that the scores dis-
criminated among the three primary categories of
brachial plexus injuries were predictive of
4 Outcome Measures
classication of neurological decits; and Ekblom
et al. (2013) used the instrument with children
with limb deciencies.
The Assisting Hand Assessment (AHA)
(Krumlinde-Sundholm and Eliasson 2003) is an
upper extremity performance measure that evalu-
ates the use of the assisting hand while performing
bimanual play in usual environments. Based on
the work by Gordon (2007) and supported by the
International Classication of Functioning, Dis-
ability and Health (ICF) code assignment to the
AHA items (Hoare et al. 2011), the AHA reects
what the child typically does in usual environ-
ments and thus may be more responsive to change
and detecting effectiveness of treatment on typical
activities in daily life. The AHA was developed
using the Rasch model of measurement
(Krumlinde-Sundholm et al. 2003) and has strong
psychometric properties for children with spastic
hemiplegia, cerebral palsy, and other orthopedic
conditions (Krumlinde-Sundholm et al. 2007;
Gordon 2007; Holmefur et al. 2007; Chang
et al. 2013; Bialocerkowski et al. 2013). The
Mini-AHA (Greaves et al. 2013) has been
established for babies with CP between 8 and
18 months of age but has not been exposed to
rigorous psychometric testing.
The Melbourne Assessment of Unilateral
Upper Limb Function (MUUL) (Randall
et al. 1999, 2008), the Quality of Upper
Extremity Skills Test (QUEST) (DeMatteo
et al. 1993, http://www.canchild.ca/en/measures/
resources/1992_quest_manual.pdf), and the
Shriners Hospitals Upper Extremity Evaluation
(SHUEE) (http://www.greenvilleshrinershospital.
org/2012/01/what-is-a-shuee) are performance
measures that were developed to evaluate upper
extremity function of children, primarily those
with cerebral palsy. While they differ in adminis-
tration and scoring, unlike the AHA, the MUUL,
QUEST, and SHUEE are impairment or body
structure-level measures (Hoare et al. 2011). All
three instruments have strong psychometric prop-
erties when used with children with CP, provide
important information about upper limb function,
and have been used in treatment effectiveness
studies (Klingels et al. 2008; Sakzewski
et al. 2007; Bard et al. 2009; Randall et al. 2008;
63
Klingels et al. 2010; Lee et al. 2013a; Thorley
et al. 2012a; Thorley et al. 2012b; Davidson
et al. 2006; Gilmore et al. 2010). Based on a sys-
tematic review of psychometric studies (Gilmore
et al. 2010), for children with CP and upper limb
involvement, the MUUL is recommended for
assessment of unilateral performance and, when
used with the AHA, is most effective at measuring
change in unilateral and bimanual hand function
over time or following treatment.
Patient-Reported Outcome
Instruments
The use of patient-reported outcome (PRO)
instruments has increased over the last several
decades. They are now an integral element in
outcomes research, including studies under the
auspice of the US Food and Drug Administration
(FDA) (2009), and longitudinal monitoring of
usual care. Similar to the resources that are avail-
able on performance measures for the upper limb,
there are notable resources on patient-reported
outcome instruments (http://www.proqolid.org/
about_proqolid; Lai et al. 2012; http://www.
nihpromis.org; McPhail et al. 2012; Pencharz
et al. 2001, http://www.neuroqol.org). When
PRO instruments are used in pediatrics, a particu-
lar consideration in their selection involves the
use of proxy reports (Magaziner et al. 1988) by
parents. There is clear evidence that children as
young as 6 years of age can report on their own
health (Riley 2004) and that the information pro-
vided by children and parents is equally impor-
tant, albeit many times, differs on perspectives of
health outcomes (Eiser and Morse 2001;
Majnemer et al. 2008; Forrest et al. 2004; Shefer
et al. 2009). Despite the lack of instruments devel-
oped and validated for child report, there is over-
whelming agreement that child and parent
outcomes should be obtained (Varni et al. 2005;
Erhart et al. 2009; Tluczek et al. 2013).
Many PRO instruments that are designed
for children evaluate global health-related out-
comes and quality of life as opposed to outcomes
specic to the upper extremity. As examples, the
Pediatric Quality of Life Inventory(PedsQL)
64
(Varni et al. 1999), the Child Health Questionnaire
(CHQ) (Landgraf et al. 1996), and the Pediatric
Evaluation of Disability Inventory (PEDI) (Haley
et al. 1992) are child/parent PRO through
self-report or interview (PEDI) that captures func-
tional activity associated with ne motor, self-
care, school, play, and global health but do not
focus on the upper extremity. The Pediatric Out-
comes Data Collection Instrument (PODCI) and
Activities Scale for Kids (ASK) are two other
PRO instruments that also evaluate global health,
but have subscales or specic items that address to
the upper limb.
The PODCI (Daltroy et al. 1998) is a 114-item
instrument with items focused on upper extremity
(UE) function as well as physical function, activ-
ity and sports, mobility, pain, and happiness; it
also has a satisfaction (with treatment) domain
and normative values for comparison. The UE
items focus on the difculty encountered to com-
plete self-care and school activities. As an exam-
ple, the adolescent self-report version of the
PODCI contains items such as during the last
week, was it easy or hard for you to comb your
hair, use spoon or fork, and lift books. The
PODCI has undergone psychometric testing with
a variety of clinical samples including children
with chronic upper extremity conditions (Amor
et al. 2011; Matsumoto et al. 2011; Lee et al.,
2010; Nath et al. 2011; Dedini et al. 2008;
Huffman et al. 2005) and in healthy children
with isolated orthopedic injuries (Kunkel
et al. 2011).
The Activities Scale for Kids (ASK) (Young
et al. 2000; Plint et al. 2003), like the PODCI, was
developed for children with musculoskeletal con-
ditions and has items that address multiple
domains of physical functioning. There are far
fewer items on the ASK (n 30) compared to
the PODCI (n 114) suggesting less burden for
the child responder. Like the PODCI, the ASK has
been used in treatment effectiveness studies
involving chronic conditions as well as those
who are without chronic conditions but who are
being treated for isolated orthopedic impairments
(von Keyserlingk et al.; Wai et al. 2005;
Rabinovich et al. 2005; Wright et al. 2008;
Boutis et al. 2010). The UE items of the ASK
M.J. Mulcahey and S.H. Kozin
address the ability to perform self-care and play.
Both the PODCI and ASK have strengths and
limitations (Christakou and Laiou 2013) that
should be considered in the context of evaluating
outcomes associated with upper extremity
function.
Perhaps the most widely used UE PRO is the
Disabilities of the Arm, Shoulder and Hand
(DASH) Outcome Measure (www.dash.iwh.on.
ca/home). The DASH is a 30-item questionnaire
designed to measure physical function and symp-
toms in patients with any or several musculoskel-
etal disorders of the upper limb. The DASH
Outcome Measure contains two optional, four-
item modules intended to measure symptoms
and function in athletes, performing artists, and
other workers whose jobs require a high degree of
physical performance; these modules likely have
little relevance to younger children. Because they
may be having difculties only at high perfor-
mance levels which are beyond the scope of
the 30-item DASH Outcome Measure clinicians
may nd the modules, which are scored separately
from the DASH, useful in assessing these special
patients. The QuickDASH is a shortened version
of the DASH (11 items) and, despite question
about its dimensionality (Gabel et al. 2009), it
has good reliability and internal consistency in
older children adolescents (Quatman-Yates
et al. 2013). Further pediatric psychometric test-
ing of the DASH and QuickDASH is needed to
establish validity, reliability, and childrens ability
to read and understand the items for self-report.
There are also disease-specic PRO instru-
ments for children. The ABILHAND-Kids ques-
tionnaire is a 21-item measure of manual ability
developed for and eld-tested in children between
6 and 16 years of age with CP (Arnould
et al. 2004). The strengths of the ABILHAND
include its construction using the RASH measure-
ment model (Penta et al. 1998), its linkage with
the adult ABILHAND that allows for assessment
of hand function across the pediatric-adult contin-
uum using a common instrument (Vandervelde
et al. 2012), and its use in randomized clinical
trials (Aarts et al. 2010; Klingels et al. 2013;
Sgandurra et al. 2013). Although the
ABILHAND-Kids was developed for children
4 Outcome Measures
with CP and despite lack of psychometric studies
with other clinical populations, the ABILHAND-
Kids questionnaire has been used with children
with arthrogryposis (Foy et al. 2013), brachial
plexus birth palsy (Spaargaren et al. 2011), limb
deciencies (Buffart et al. 2007b), and muscular
dystrophy (Kumar and Phillips 2013).
The Prosthetic Upper Extremity Functional
Index (PUFI) (Wright et al. 2001, 2003) and the
Child Amputee Prosthetics Project-Functional
Status Inventory (CAPP-FSI) are PRO instru-
ments developed for children with limb de-
ciency. The PUFI has a version for children
between 3 and 6 years old (n 26 items) and
for children older than 6 years (38 items); there
are 14 common items (Buffart et al. 2006; van
Dijk-Koot et al. 2009), presumably for linking.
The scoring method for the PUFI is somewhat
complex. Responses are scored on three scales:
method of performance using a 6-point scale and
ease of performance and usefulness of the pros-
thesis, both using 3-point scales (Buffart
et al. 2006). Concurrent validity has been
established with other measures and with parent
response anchors (Buffart et al. 2006), but dis-
criminative validity is poor (Buffart et al. 2006)
and the validity of summed scores for
non-prosthetic users has been questioned (van
Dijk-Koot et al. 2009). Buffart et al. (2008) used
the PUFI and AHA in combination to capture
outcomes of hand\arm function (PUFI) and activ-
ity performance (AHA) and found a relationship
between outcomes of the PUFI and functional
performance as dened by the AHA.
Individual Patient-Reported Outcomes
The Canadian Occupational Performance Mea-
sure (COPM) (Law et al. 1990) and Goal Attain-
ment Scaling (BovendEeerdt et al. 2009) are
unique from other standardized PRO instruments
due to the individualized approach they use to
establish goals. The COPM capitalizes on the
semi-structured interviews that reect usual inter-
action between an occupational therapist, who
approaches practice from a client-centered
framework, and his/her clients. Through
65
semi-structured interviews, parents and children
identify performance activities that are perceived
as important by the parent, child, and/or society
(e.g., activities that a child is expected to per-
form); performance is rated on a scale between
0 (cannot do) and 10 (can do very well) and the
activities are used to establish goals for treatment.
Changed scores between baseline and
reassessment are calculated to evaluate outcomes
of treatment; although individuals may differ in
their idea of what constitutes meaningful change,
research suggests that a change of two or more
points reects meaningful change (http://www.
rehabmeasures.org). Psychometric properties of
the COPM have been well established in adult
and pediatric clinical samples (Cup et al. 2003;
Dedding et al. 2004; Eyssen et al. 2011; Cusick
et al. 2006). Although it was adapted for very
young children (Cusick et al. 2007), the COPM
focuses assessment of performance in self-care,
productivity, and leisure (Law et al. 1990). The
COPM has been used in pediatric studies
(Carswell et al. 2004; McColl et al. 2005), several
of which demonstrated its responsiveness to
change (Mulcahey et al. 1995; Davis et al. 1999;
Pollock et al. 2013; Brandao et al. 2013).
Goal Attainment Scaling (GAS) is a technique
for evaluating individual progress toward patient-
dened goals that involves a sequential process
that sets goals of treatment, assigns a weight for
each goal based on the importance or priority of
the patient, establishes a continuum of possible
outcomes, assesses baseline function, provides
intervention for a specied period of time, evalu-
ates performance on each goal using specied
possible outcomes, and evaluates the extent of
goal attainment (Kiresuk et al. 1994). Similar to
the COPM, one of the strengths of GAS is the
ability to evaluate individualized change
(Mailloux et al. 2007). However, unlike the
COPM, Kiresuk et al. (1994) have demonstrated
that GAS scores from multiple patients can be
aggregated and compared. Whereas the COPM
focuses on occupational performance within the
domains of productivity, self-care, and leisure, an
advantage of GAS is that goals can be established
across the International Classication of Func-
tioning, Disability and Health (ICF) domains.
66
Multiple upper extremity studies have used the
GAS to evaluate treatment outcomes (Ten Berge
et al. 2012; Wesdock et al. 2008; Lowe et al. 2007;
Steenbeek et al. 2007) and showed that it was
more responsive than two widely used standard-
ized pediatric instruments (Steenbeek et al. 2010).
Cusick and colleagues (2006) examined the
utility of the COPM and GAS as an outcome
measure for pediatric rehabilitation; children
with CP received occupational therapy or occupa-
tional therapy and botulinum toxin A injection.
Cusick et al. (2006) found that both the COPM
and GAS had utility as outcome measures, with
the GAS associated with more sensitivity to treat-
ment and the COPM associated with less burden.
While Steenbeek et al. (2010) showed good inter-
rater reliability of GAS, BovendEerdt
et al. (2011) found it to be poor and suggested
further work on improving reproducibility of
GAS scoring prior to use in research and clinical
trials. Table 2 provides an example of GAS for a
child with a cervical spinal cord injury who had
upper extremity reconstructive surgery for restor-
ing grasp and pinch.
Computer Adaptive Tests
Contemporary measurement technology and
computer adaptive testing (CAT) for health-care
applications are transforming approaches to eval-
uation of outcomes (McHorney 1997; Cella
et al. 2007). CAT platforms are built upon a set
of coordinated items, referred to as item banks
that dene a common domain (Bode et al. 2003),
for example, upper extremity function. The
CAT employs simple articial intelligence that
selects items from the item bank that are directly
tailored to an individual; CATs can be shortened
or lengthened to achieve a desired precision, and
each test administered can be scored on a standard
metric such that scores can be compared across
time points and across groups of patients, despite
using different test items for different patients.
The well-known PEDI instrument has been
developed into a CAT (Haley et al. 2006; Coster
et al. 2008; Allen et al. 2008; Dumas et al. 2010a)
and eld-tested in children with respiratory
M.J. Mulcahey and S.H. Kozin
disease (Dumas et al. 2010b); infantile, juvenile,
and idiopathic scoliosis (Mulcahey et al. 2008);
and mobility impairments (Haley et al. 2005). The
PEDI-CAT is available at http://pedicat.com/cate-
gory/ordering/.
Two large-scale efforts on developing item
banks, short forms, and CATs involve the work
by Neuro-QOL (http://www.neuroqol.org) and
PROMIS (http://www.nihpromis.org). Although
there are physical functioning constructs
included, Neuro-QOL focuses largely on psycho-
social constructs such as anxiety, depression,
fatigue, and social relationships for pediatric and
adult populations with neurological conditions.
PROMIS instruments focus on constructs associ-
ated with physical, mental, and social health and
are non-categorical (not disease specic) but have
been eld-tested in diverse clinical samples. Both
Neuro-QOL and PROMIS instruments are avail-
able at no cost to researchers and clinicians (http://
www.assessmentcenter.net); their use with chil-
dren has been discussed (Lai et al. 2012; DeWitt
et al. 2011; Gipson et al. 2013; Kratz et al. 2013).
The Shriners Hospitals for Children (SHC)
has also advanced CAT for application in
pediatric health care (Calhoun et al. 2009;
Haley et al. 2009; Haley et al. 2010; Montpetit
et al. 2011; Mulcahey et al. 2012a; Bent
et al. 2013). Unlike the PROMIS instruments,
SHC item banks and CATs are disease specic
and assess physical functioning in children with
CP (Haley et al. 2009, 2010 Tucker et al. 2009a, b)
and BPBP (Mulcahey et al. 2012b, 2013) and
activity performance and participation in children
with SCI (Calhoun et al. 2009; Bent et al. 2013;
Mulcahey et al. 2012a). The CP and BPBP CATs
use parent proxy reports and have been translated
into Spanish. The SHC SCI CATs are available as
both parent and child reports and have been linked
to the adult SCI functional index (SCI-FI) CAT
(Tian et al.) developed by Jette and colleagues
(Jette et al. 2012). Among several strengths of the
SHC CP, BPBP, and SCI item banks is that they
have been administered to 2,000 typically develop-
ing youth living in the United States, providing a
basis for developing not only disease-specic tra-
jectories of functioning but also a mechanism for
normative comparison (Fig. 1a and b).
 4

Table 2 Example item on GAS with a 16-year-old boy with C6 spinal cord injury who had the brachioradialis transferred to the flexor pollicis longus for pinch and the radial wrist
extensor to the finger flexors for grasp. The concern (first column) drives the development of the goal (second column). The uniqueness of GAS is the description of the scale
(columns 36) that is developed collaboratively among the physician, therapist, child, and parent. The score of 0 indicates that the goal was achieved; scores of 1 and 2
Outcome Measures

indicate that the goal was not achieved, but provides a mechanism to evaluate progress (score of 1). The scores of +1 and +2 indicate that the child exceeded the expectation and
agreed upon goal (score 0). Pre- and post-tendon transfer scores are shown in columns 8 and 9, respectively. TTtendon transfer
Before After
Concern Goal 2 1 0 1 2 TT TT
Unable to empty To be able to Unable to Able to pick up Is able to pick up Is able to pick up Is able to 2 1
bladder in school independently perform catheter that is catheter that is unwrapped catheter and remove
without full perform intermittent unwrapped and unwrapped and apply lubrication, inserts catheter from
assistance from intermittent self- catheterization prepared and prepared and insert to catheter into the bladder package,
school nurse; catheterization in after setup of attempts to insert. empty the bladder, for emptying, able to do apply
refusing to allow school after setup supplies cannot insert requires some effort on rst attempt, but lubrication
nurse to empty of supplies by fully to allow for and excessive time, requires some effort and and insert into
bladder at school school nurse emptying multiple attempts excessive time the bladder for
emptying
Cannot grasp Able to undo Able to button/ Completes
catheter to jeans but requires unbutton and task without
insert into the assistance to zip zip/unzip jeans effort and in
bladder and button jeans acceptable
time frame
Requires total Cleans up by
assistance to placing
unbutton/unzip disposables in
jeans trash
Requires
assistance to
zip and button
jeans
67
68 M.J. Mulcahey and S.H. Kozin

Fig. 1 Examples of score

reports from SHC CP CAT a
mobility scale. Reports are Upright Mobility Historical by Patient Assesment Dates
generated at the point of 100
care for the patient (blue
line and bar) with 90
comparisons to similar
children with CP (clinical
80 80 Patient
comparison, red line and 79 78
red bar) and to typically Clinical
developing children (gold 70 70 Normative
line and gold bar) 68
61
63 64
60
60

50
1/22/2010 6/1/2010 12/15/2010

b
Upright Mobility
80
80 70
64
70
60
50 Patient

40 Clinical
30 Normative
20
10
0
Patient Clinical Normative

testing approaches are transforming the landscape

Summary of outcome instruments.

Despite the plethora of outcome instruments

available to evaluate upper extremity function,
for many of them, there is a void in psychometric
studies in pediatric clinical samples. Several
instruments that have been repeatedly discussed
in the research literature and with psychometric
properties in pediatric clinical samples were
reviewed. Patient-reported outcomes have
become increasingly important in health-care out-
comes; pediatric scales have been developed with
particular commitment to having the child self-
report. Contemporary measurement practices
such as item bank building and computer adaptive
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 Part III
Imaging
 Imaging
5
Archana Malik, Jacqueline A. Urbine, Erica D. Poletto,
Bret Kricun, Evan Geller, Polly Kochan, Robert L. Siegle, and
Eric N. Faerber

Contents Abstract
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 77 There is a wide array of modalities available
for imaging evaluation of the pediatric upper
MRI . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 82
Congenital Differences . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 83
extremity. It is incumbent on radiologists in
Trauma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 85 consultation with clinical colleagues to select
Infection . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 88 the most appropriate imaging modalities that
Arthritides . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 91 are both time and cost-effective, in keeping
Neoplasia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 91
Vascular Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 99
with the ALARA (as low as reasonably achiev-
able) principle and the Image Gently
Computed Tomography . . . . . . . . . . . . . . . . . . . . . . . . . . . . 105 campaign.
Ultrasound . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 106
Applications of Upper Extremity Ultrasound . . . . . . . 106
Nuclear Medicine/PET . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 109 Introduction
Interventional Radiology . . . . . . . . . . . . . . . . . . . . . . . . . . . 109
Trauma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 110 Available imaging modalities are briey
Infection . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 113 discussed followed by selected examples of
Non-traumatic Vascular Abnormalities . . . . . . . . . . . . . . 113 upper extremity abnormalities.
Neoplasia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 113
Additional Applications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 113 Plain lm radiography: Plain lm radiography
is customarily the rst imaging modality to be
References and Suggested Readings . . . . . . . . . . . . . . 113
utilized because of the relatively low cost, low
radiation dose, ease of acquisition, and ability to
discern bones, soft tissues, joints, and some for-
eign bodies.
A. Malik J.A. Urbine E.D. Poletto E. Geller Radiographs are usually performed in at least
P. Kochan R.L. Siegle E.N. Faerber (*)
two planes, 90 tangential to one another (Brant
Department of Radiology, St. Christophers Hospital for
Children, Philadelphia, PA, USA and Helms 2007). There are many normal variants
e-mail: Archana2.malik@tenethealth.com; jacqueline. to be cognizant of, and it must be emphasized that
urbine@drexelmed.edu; erica.poletto@tenethealth.com; the appearance of the physes in the growing child
evan.geller@tenethealth.com; robert.siegle@drexelmed.
may simulate fractures (Fig. 1). This is especially
edu; eric.faerber@tenethealth.com
true in the maturing elbow, where ossication
B. Kricun
centers may be mistaken for fracture fragments
Lehigh Valley Diagnostic Imaging, Lehigh Valley Health
Network, Allentown, PA, USA (Table 1). Understanding of radiographic anatomy
e-mail: bretkricun@hotmail.com is particularly useful in the assessment of the
# Springer Science+Business Media New York 2015 77
J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_5
78 A. Malik et al.

Fig. 1 Normal appearance of the proximal humeral rotation view, however, it is clear that the physis (arrow)
physis in a 6-year-old boy. (a) On the AP internal rotation and humeral contours are intact, without discontinuity that
view, the physis has an oblique orientation, which can be would suggest fracture
easily mistaken for a fracture. (b) On the AP external

Table 1 C-R-I-T-O-E, the mneumonic for the order of ossification of the apophyses about the elbow joint, is as above
(Iyer 2012)
Age (years) of appearance on radiograph, Age (years) of appearance on radiograph,
Ossication center girls boys
C Capitellum 1 2
R Radial head 3 4
I Inner (medial) epicondyle 5 6
T Trochlea 7 8
O Olecranon 9 10
E External (lateral) 11 12
epicondyle

pediatric elbow. The radiocapitellar line is one of
the key lines used to assess alignment on an elbow
radiograph (Figs. 2 and 3).
Congenital dysplasias are best demonstrated
by plain radiography (Lachman 2008) (Fig. 4).
Plain radiographs are also the mainstay of imag-
ing to evaluate trauma. Cross-sectional modalities
are only utilized in select instances (Fig. 5).
Periosteal reaction is most commonly seen in
the setting of healing fracture; however, it also
present in a wide variety of benign and malignant
conditions (Rana et al. 2009) (Table 2 and Fig. 6).
While cross-sectional imaging and radionu-
clide imaging are the most sensitive and specic
in the evaluation of infection of the bones and soft
tissues of the extremities, plain radiography
remains the rst-line imaging modality as the
presence of ndings will prevent delays in treat-
ment. For some infectious organisms, most nota-
bly in congenital syphilis, characteristic
radiographic ndings such as destructive
metaphyseal lytic lesions may aid in the diagnosis
(Shore 2008) (Fig. 7). Plain radiography has a
limited role in early osteomyelitis (Fig. 8); how-
ever, it is useful in the evaluation of the compli-
cations of osteomyelitis, such as premature
physeal closure, joint destruction, and growth
disturbance.
With inammatory arthropathies and connec-
tive tissue disorders, plain radiographs are often
obtained at diagnosis in order to evaluate for ero-
sive changes, sclerosis, joint space narrowing,
5 Imaging
Fig. 2 Normal radiocapitellar relationship. Line drawn through neck of the radius should bisect the capitellum on
frontal (a) and lateral (b) radiographs
Fig. 3 Congenital radial head dislocation. Frontal (a)
and lateral (b) radiographs of the elbow demonstrate dis-
ruption of the radiocapitellar line. Additionally, the radial
and/or distribution of disease (Brant and Helms
2007). Soft tissue calcications may be evident in
some conditions, such as dermatomyositis.
In a child with suspicion for rickets, frontal
radiographs of the wrists and knees are obtained
(Fig. 9) (Shore 2008). These are the sites of fastest
growth and will demonstrate early ndings of
irregularity of the zone of provisional calcication
of the physis and apparent physeal widening. In
79
head is deformed with a convex superior border,
suggesting congenital etiologies
premature newborns, incidental changes from
rickets involving the humeral heads may be
detected on routine chest radiography.
Plain radiographs are also important in the
evaluation of benign and malignant bone
tumors. The benign or aggressive nature of the
lesion can be estimated by several criteria,
including the morphology, the zone of transi-
tion, the internal matrix, the appearance of
80
Fig. 4 Select congenital abnormalities of the upper
extremity in children. (a) Carpal coalition involving the
lunate and triquetrum (arrow). (b) Polydactyly, with an
accessory digit that extends from the thumb and contains
rudimentary phalanges. (c) Aperts syndrome, with soft
tissue and osseous syndactyly (mitten-hand) deformity
periosteal reaction, the presence of cortical
destruction or a soft tissue mass, the location
of the lesion, and the number of lesions (Brant
and Helms 2007). It should be noted that in
children, Langerhans cell histiocytosis and
A. Malik et al.
that is typical of this syndrome. (d) Radial ray anomaly,
with absent radius and foreshortening of the humerus. (e)
Chondrodysplasia punctata. Note stippled calcication of
the articular cartilage of the upper extremities with
rhizomelic pattern of limb shortening
osteomyelitis are two benign conditions that
may appear aggressive/malignant on plain radi-
ography. The differential diagnosis may become
quite short on the basis of radiographs alone (see
Table 2 and Figs. 10 and 11).
5 Imaging
Fig. 5 Elbow fractures in children. (a) Supracondylar
fracture (arrow) results in a large joint effusion as well as
anterior displacement of the distal humeral fracture frag-
ment, which includes the capitellum. (b) A medial
epicondylar avulsion fracture (arrow) occurs in the 715-
year age group and, due to disruption of the cartilage
81
between the epicondyle and the condyle, is either a
Salter-Harris 3 or 4 fracture (Iyer 2012). (c) Lateral condy-
lar fracture (arrow) is seen as sliver of fractured bone along
the lateral condyle of distal humerus. There is no disruption
of the anterior humeral line as seen on lateral view, unlike a
supracondylar fracture
82 A. Malik et al.

Table 2 Periosteal reaction is the bones response to many different causative etiologies. The appearance on
radiograph can be characteristic of either a benign or a malignant process
Type of periosteal reaction Radiographic example Etiologies
Benign Smooth Physiologic
Symmetric Prostaglandin-induced
Solid Thermal injury
Healing fracture
Sickle Cell Disease
Scurvy
Leukemia
Caffeys Disease

Malignant Spiculated Osteogenic sarcoma

Langerhans Cell Histiocytosis
(LCH)
Infection

Lamellated Ewings Sarcoma

LCH
Infection
Codmans Triangle Osteogenic sarcoma
LCH
Infection
Amorphous/ LCH
disorganized Infection

In the setting of a soft tissue mass, radiographs

may demonstrate secondary osseous involvement
or internal calcication. Phleboliths associated
with venous malformations may be apparent on
plain radiographs.
Bone age evaluation is traditionally performed
by plain radiography of a hand with comparison to
normal standards of age and gender. The most
widely used reference is the standard text by
Greulich and Pyle (1959); however, there are
now multiple alternatives available, most notably
by Gilsanz and Ratib (2012).
MRI
Magnetic resonance imaging (MRI) is the
modality of choice for musculoskeletal cross-
sectional imaging in the pediatric upper extremity.
Inherent advantages of MRI include excellent
spatial and contrast resolution of the soft tissues
and bones, which is accomplished without
imparting radiation to the patient. However, the
long imaging time necessary to perform a
complete MRI exam can be a signicant
5 Imaging
Fig. 6 Periosteal reaction. (a) Prostaglandin therapy can
result in solid periosteal reaction in the neonate. (b) In this
2-year-old who suffered a severe thermal injury, smooth
periosteal reaction is identied along the humeri. (c)
Dactylitis is a common nding in sickle-cell patients, the
result of extensive infarction of the marrow, medullary
trabeculae and the inner layers of the cortical bone [with]
disadvantage, resulting in the need for sedation or
general anesthesia in young patients who cannot
lay still throughout the protracted study (Strouse
2008).
83
subsequent circumferential periosteal elevation and forma-
tion of subperiosteal new bone (Babhulkar 1995, p. 312).
(d) In this patient with large cell lymphoma, amorphous
periosteal reaction is identied with an underlying moth-
eaten appearance to the bony matrix, indicating a malig-
nant process
Congenital Differences
While most congenital differences of the upper
extremity are assessed with plain radiographs,
84
Fig. 7 Characteristic ndings of congenital syphilis,
including diaphysitis as well as productive metaphysitis
(distal humerus) and destructive metaphysitis (proximal
humerus). The alternating bands of increased and
Fig. 8 Limited role of plain lms for osteomyelitis in
the acute setting. (a) Patient presenting with fever, ele-
vated white count, and refusal to move left shoulder. Plain
radiograph of the left shoulder demonstrates widened
glenohumeral joint space concerning for effusion without
MRI is a useful adjunct in some instances. MRI is
particularly useful in the pediatric population as
the skeletal structures may still be cartilaginous
and are therefore much better seen with MRI as
opposed to computed tomography (CT) or plain
x-ray (Strouse 2008).
A. Malik et al.
decreased density in the proximal and distal radial and
ulnar metaphyses are the typical sandwich congura-
tions of superimposed productive and destructive
metaphysitis (Rasool and Govender 1989, p. 753)
any osseous abnormality. (b) Follow-up plain radiograph
obtained 2 weeks later depicts periosteal reaction adjacent
to the scapula and proximal humeral diaphysis (arrows),
consistent with osteomyelitis in this patient with septic
joint
An important entity diagnosed easily on MRI
is glenohumeral dysplasia, which may occur in
isolation or in association with mucopolysac-
charidoses, mucolipidoses, or skeletal dysplasias.
Injury to the brachial plexus during birth, with
resulting paralysis of the shoulder girdle
5 Imaging
Fig. 9 Characteristic ndings in rickets. In areas of
rapid bone turnover, such as the metaphyses of the distal
radius and ulna, fraying and cupping occurs secondary to
uncalcied bony matrix. Additionally, bones can become
severely osteopenic resulting in microfractures and smooth
periosteal reaction, as demonstrated in this child with rick-
ets (Berry 1952)
musculature, can also inhibit normal development
of the glenoid and humeral head and thus lead to
glenohumeral dysplasia (Gudinchet et al. 1995).
The glenoid articular surface is decient, particu-
lar posteriorly, and the humeral head is attened
and small. Furthermore, the humeral head is often
posteriorly dislocated because of the glenoid de-
ciency (Fig. 12). With advances in imaging, MR
neurography can be used to depict normal and
abnormal peripheral nerves (Figs. 13 and 14)
(Chhabra et al. 2011).
Trauma
Osseous injury may result from a single traumatic
event or due to repetitive stresses. Although con-
ventional radiographs are the rst modality uti-
lized for evaluating osseous trauma, many acute
and chronic osseous injuries are not detectable on
plain radiographs. MRI is exquisitely sensitive for
85
detection of radiographically occult injuries. A
normal MRI essentially excludes the presence of
an osseous injury.
Radiographically occult fracture. It is well
known that MRI is able to demonstrate radio-
graphically occult fractures in a rapid, cost-
effective manner to enable optimal patient man-
agement. In such cases, in addition to demonstrat-
ing marrow edema, a fracture line is usually
identied as a linear or curvilinear focus of low
signal intensity on T1W images that may demon-
strate low or high signal on STIR images
(Fig. 15).
MRI is also useful in the evaluation of fracture
complications. For example, in the setting of a
prior scaphoid fracture, MRI will show changes
of avascular necrosis before plain radiographs
(Fig. 16).
Avulsion fracture. Avulsion fractures typi-
cally demonstrate abnormal marrow signal at the
site of the injury, but the extent of marrow abnor-
mality generally is limited and appears less robust
than with impaction injuries.
Repetitive trauma. Stress injuries to bone
typically are divided into two categories: insuf-
ciency and fatigue fractures (Daffner and Pavlov
1992). Fatigue fractures result from abnormal
stress to normal bone and, therefore, are seen
commonly in the pediatric population. Chronic
avulsive injuries such as shin splints can be dif-
cult to diagnose on plain radiographs, but can be
detected with MRI.
Physeal injury. In a skeletally immature
patient, trauma to the physes can result in prema-
ture closure of a portion of the physis, which
results in differential growth and subsequent
deformity. The diagnosis of osseous bridging can
be challenging on plain radiographs. MRI with its
gradient echo images, which are designed to
assess the cartilage, is the gold standard for the
assessment of such injuries (Carey et al. 1998).
The physes may be susceptible to chronic
repetitive trauma in athletes, most characteristi-
cally the distal radius and olecranon in gymnasts
and the proximal humerus in baseball pitchers.
The characteristic ndings on MRI include
physeal widening and T2-hyperintensity at the
junction of the physis and metaphysis.
86 A. Malik et al.

Fig. 10 Typical appearance of several benign bone associated pathologic fracture and a fallen fragment. (c)
tumors. (a) AP and lateral view of a sessile Enchondromas of the phalanges, most prominent in the
osteochondroma of the proximal humeral metaphysis. (b) fourth digit, demonstrate the typical arcs and whirls of a
Unicameral bone cyst without (i) and with (ii) an chondroid matrix
5 Imaging
Epiphyseal injury. Osteochondritis dissecans
(OCD) is fragmentation or separation of a portion
of subchondral bone along the articular surface.
Two types of OCD have been described: the
Fig. 11 Large cell lymphoma. Frontal radiograph of the
forearm shows permeative pattern of bone destruction in
the radial and ulnar diaphysis with periostitis
Fig. 12 Congenital glenohumeral dysplasia. MRI of
bilateral shoulder joints. Axial GRE images through
glenohumeral joints show (a) normal alignment in the
right shoulder and (B) posteriorly subluxed humeral head
87
juvenile form, occurring before physeal closure,
and the adult type. Common joints involved
include the knee, ankle, and elbow. In the elbow,
there is involvement of the capitellum along its
convex anterior surface. MRI is used in the eval-
uation for instability, as well as osseous or carti-
laginous loose bodies within the joint. Linear
T2-hyperintense signal between the osseous frag-
ment and the parent bone is suggestive of insta-
bility (Fig. 17). The capitellum is also susceptible
to avascular necrosis, called Panners disease.
This disease is most commonly seen in children
with repeated valgus stress to the elbow, such as
baseball players and gymnasts. The entire
capitellum is affected, becoming diffusely
T1-hypointense on MRI. Children with Panners
disease are typically younger than those with an
osteochondral injury.
Soft tissue injuries. An added advantage of
MRI is its ability to demonstrate soft tissue inju-
ries, including injuries to the ligaments, tendons,
and cartilage that may mimic a fracture clinically
(El-Khoury et al. 1996). MR arthrography refers
to distention of the joint with a solution containing
dilute gadolinium (contrast agent). It is extremely
useful in assessing intra-articular soft tissue inju-
ries in the shoulder and hip (Waldt et al. 2005).
A common labral injury seen in the setting of an
with deformed, dysplastic glenoid cavity (arrow) and
absent labrum in the left shoulder joint. Note globally
decreased muscular caliber compared to the
contralateral side
88 A. Malik et al.

Fig. 13 Normal MR
neurography appearance
of the brachial plexus.
Coronal MIP projection
from 3D STIR SPACE
image shows normal
symmetrical appearance of
bilateral brachial plexuses.
Notice dorsal nerve root
ganglions are the most
hyperintense and the signal
intensity and caliber of the
brachial plexus segments
decrease gradually along
their distal course with no
nerve kinking or irregularity
(Courtesy: Avneesh
Chhabra M.D.)

Fig. 14 MR neurography
in a patient with chronic
inammatory
demyelinating
polyneuropathy. Young
boy with multifocal motor-
sensory neuropathy, worse
on the left side. Coronal
MIP projection from 3D
STIR SPACE image shows
multifocal patchy
thickening and asymmetric
hyperintensity of the
brachial plexus, especially
on the left side (Courtesy:
Avneesh Chhabra M.D.)

anterior shoulder dislocation is the Bankart tear
(Stoller 1997) (Fig. 18).
Some other uses of MRI in the setting of
trauma include ligament tears in the elbow and
wrist. Scapholunate ligament and triangular
brocartilage (TFCC) tears are optimally evalu-
ated for on MRI (Fig. 19).
Infection
Musculoskeletal infections can affect bones, soft
tissues, and joints. Although MRI diagnosis of
osteomyelitis does not require contrast, use of
contrast does increase the condence of the radi-
ologist in providing the diagnosis and aids in the
assessment of complications such as abscesses
(Averill et al. 2009). The extent of infection and
the presence of abscess formation are best
assessed with fat-suppressed T2-weighted, STIR,
and post-gadolinium fat-suppressed T1-weighted
images (Averill et al. 2009).
Soft tissue infection. While the supercial soft
tissues can be assessed with ultrasonography,
MRI is the preferred modality for the evaluation
of deeper soft tissue infections such as cellulitis
(Fig. 20), septic tenosynovitis, septic bursitis,
infectious myositis, and necrotizing fasciitis
5 Imaging
Fig. 15 Elbow joint dislocation with radiographically
occult intra-articular fracture of ulna. Lateral (a) and
frontal (b) plain radiographs demonstrate disruption of the
radiocapitellar relationship with large joint effusion. Small
osseous fragment (arrow) is seen in the joint space without
(Strouse 2008) (Fig. 21). With myositis, MRI
depicts muscle enlargement and intermediate sig-
nal on T1 and high signal on T2 images.
Abscesses are best diagnosed with utilization of
contrast, as they demonstrate rim enhancement
(Fig. 22). Necrotizing fasciitis is a surgical emer-
gency and, therefore, early diagnosis is impera-
tive. MRI allows for the distinction between
cellulitis and necrotizing fasciitis. The typical
ndings of necrotizing fasciitis include high
89
denite donor site. The intra-articular osteochondral ulnar
fracture with bone marrow edema (arrow) is seen on sag-
ittal STIR (c) and T1 (d) images from MRI study as high
and low signal, respectively. The full thickness cartilage
defect is best seen on the GRE sequence (e)
signal within supercial and deep fasciae on
STIR images, which can demonstrate enhance-
ment on post-contrast imaging; however, contrast
is not mandatory to make the diagnosis.
Septic arthritis. In a monoarticular inamma-
tory process, septic arthritis must be high in the
differential as this is a surgical emergency. The
diagnosis often requires aspiration of joint for
culture and sensitivity. MR ndings of septic
arthritis are not specic and cannot be easily
90 A. Malik et al.

Fig. 16 Scaphoid fracture with avascular necrosis of scaphoid fracture (arrow) and additional nding of
the proximal pole. (a) Scaphoid fracture is seen on the decreased T1 signal in the proximal pole (arrowhead)
plain radiograph. (b) Coronal T1 image from MRI shows consistent with avascular necrosis

Fig. 17 Osteochondritis
dissecans. MRI of the
elbow with sagittal STIR (a)
and sagittal GRE (b) images
demonstrates osteochondral
lesion involving the anterior
articular surface of the
capitellum with high T2
signal. The full thickness
cartilage defect is better
seen on the gradient
sequence (arrow)

distinguished from any other inammatory arthri-
tis. The presence of a joint effusion and synovial
enhancement are the key imaging ndings. Adja-
cent soft tissue and bone marrow edema are more
commonly seen with a septic joint rather than
other inammatory processes.
Osteomyelitis. MRI is the modality of choice
in the evaluation of osteomyelitis as it enables
early detection, the extent of involvement, and
the activity of disease. MRI is as sensitive as
scintigraphy and more sensitive than CT in the
early detection of osteomyelitis, with superior
5 Imaging
Fig. 18 Bankart lesion with Hill Sachs deformity. (a)
Axial PD FS image at the level of the superior
glenohumeral joint depicts high T2 signal with deformity
of the posterolateral humeral head (arrow), consistent with
Hill Sachs deformity. (b) Axial PD FS image at the level
delineation of the extent of marrow, adjacent soft
tissue, and joint space involvement (Fig. 22).
Arthritides
Plain radiographs remain the initial modality in
the evaluation of most cases of arthritis. MRI has a
limited role in the evaluation of arthritis; however,
it is important to recognize arthritis-related
changes, as patients with arthritides may be
imaged for other reasons. The key imaging nd-
ings seen with inammatory arthropathies such as
91
of the inferior glenohumeral joint depicts anterior labral
tear with disruption of the periosteum (arrow). C. PD FS
image obtained with the patient in ABER (abduction and
external rotation) conrms the displaced anterior labral
tear, consistent with Bankart lesion
juvenile idiopathic arthritis include joint effusion,
synovial proliferation, pannus formation, and ero-
sion of cartilage. Chondrolysis is a very important
do not miss diagnosis in the pediatric popula-
tion and can be easily seen with the cartilage
sensitive MR sequences (Fig. 23).
Neoplasia
MRI plays a key role in evaluating patients with a
suspected musculoskeletal neoplasm. In addition
92 A. Malik et al.

Fig. 19 Tear of the

triangular brocartilage
(TFCC). MRI of the wrist
with coronal T1 (a) and
coronal GRE (b) images
demonstrates signal
abnormality within the
body of the triangular
brocartilage (TFCC),
consistent with a tear

Fig. 20 Cellulitis secondary to lymphadenitis. Gray
scale (a) and color Doppler (b) ultrasound images show
inammation of the subcutaneous fat with an enlarged
lymph node as seen on color images with typical central
vascularity. Follow-up MRI was done to assess for deeper
involvement and osteomyelitis. Coronal STIR (c, d)
images show abnormal high T2 signal in the medial soft
tissues at the elbow with enlarged lymph nodes. Pre- (e)
and post-contrast (f) coronal LAVA images show multiple
enhancing lymph nodes consistent with lymphadenitis. No
abscess or osseous involvement was noted
5 Imaging
Fig. 21 Necrotizing fasciitis with osteomyelitis. Plain
radiographs of the forearm (a) show mild soft tissue swell-
ing with normal bones. Note the rapid progression of the
soft tissue swelling on the repeat plain radiograph (b)
obtained 1 day later. No osseous abnormality is seen.
to conrming the presence of a lesion, MRI per-
mits accurate characterization of the tumor extent
and, sometimes, a specic diagnosis, which
enables optimal patient management. Therapeutic
planning requires accurate tumor staging.
Depending on which anatomic structures and
compartments are involved, MRI is the best
modality to accurately stage soft tissue and bone
tumors.
Important MRI features. The checklist for
staging a musculoskeletal tumor on MRI includes
intraosseous extent, extraosseous extent,
neurovascular involvement, joint invasion, skip
metastases in the same bone, and local
adenopathy.
Bone tumors. MRI is the most sensitive
modality for detection and delineation of osseous
tumors, especially those involving the marrow
cavity (Wyers 2010). For example, osseous
lesions with high signal on T1W images include
intraosseous lipoma, hemangioma, and bone
infarct. Similarly, osseous lesions containing low
93
Corresponding MRI shows diffuse abnormal high T2 and
low T1 signal in the bones and soft tissues on STIR- (c) and
T1-weighted (d) images, respectively. The patient subse-
quently had an emergent fasciotomy
signal on T2W images include sclerosis, calcied
masses, brous lesions, and lymphoma. Cystic
masses such as an aneurysmal bone cyst can pre-
sent as expansile masses with uid-uid levels
(Fig. 24).
MRI may also be useful in benign lesions such
as osteochondromas to assess for complications
such as neurovascular compression or malignant
transformation. Osteochondromas have a
T2-hyperintense cartilaginous cap; thickness
greater than 1 cm raises concern for malignant
degeneration into chondrosarcoma. MRI is also
useful in evaluating the relationship between the
lesion and adjacent neurovascular structures,
which may be compromised due to mass effect
(Fig. 25).
The appearance of most bone tumors on MRI is
nonspecic and requires concurrent assessment
with plain radiographs. The radiologic distinction
between benign- and malignant-appearing lesions
is based on plain radiographs, which allow for
evaluation of tumoral margin, zone of transition,
94 A. Malik et al.

Fig. 22 Osteomyelitis
with cellulitis and abscess
MRI. Coronal STIR (a)
sagittal T1 (b) images of the
hand with attention to
thumb demonstrate
abnormal low T1 and high
T2 signal within the rst
proximal and distal phalanx
consistent with
osteomyelitis. Coronal
LAVA pre- (c) and post-
contrast (d) images reveal a
rim enhancing abscess
within the adjacent soft
tissues

associated matrix, and type of periosteal reaction
(if any) (Khanna and Bennett 2012). Some benign
lesions, such as non-ossifying broma and brous
dysplasia, have typical plain radiographic appear-
ances and, therefore, do not require further imag-
ing. It is also important to remember that some
benign osseous lesions such as osteoid osteoma,
chondroblastoma, Langerhans cell histiocytosis,
and stress fractures can have a very aggressive,
potentially misleading appearance on MRI
(Wyers 2010).
Unfortunately, there is an overlap in the
appearance of benign and malignant lesions;
therefore, the indeterminate lesions may require
biopsy for accurate characterization. If a malig-
nant osseous neoplasm is suspected, particularly
osteogenic sarcoma, imaging of the entire bone
from joint to joint is recommended because of the
possibility of skip lesions (Fig. 26).
Soft tissue tumors. Ultrasonography (US) is
often utilized as an initial modality in children
presenting with supercial soft tissue masses of
5 Imaging 95

Fig. 23 Juvenile
idiopathic arthritis. Plain
radiographs (a and b) of the
hand show juxta-articular
osteopenia, soft tissue
swelling, and subtle
erosions involving multiple
bones. Follow-up MRI
conrms the ndings of
erosions seen as high T2 (c)
and low T1 (d) signal
intensity. Pre- (e) and post-
contrast (f) LAVA images
show synovial
enhancement consistent
with synovitis

the upper extremity. US can be helpful in
conrming the presence of a mass and in charac-
terizing the mass as cystic or solid. It avoids
unnecessary further imaging in cases such as
lymphadenopathy, which can present as a
palpable mass. MRI remains the modality of
choice for imaging workup of an upper extremity
mass, as it can provide exquisite detail, with
regard to the cystic, solid, fatty, calcied, or vas-
cular nature of the mass.
96
Fig. 24 Aneurysmal bone cyst. Axial (a) and sagittal (b)
STIR images show an expansile T2 bright lesion within the
ulnar metadiaphysis with uid-uid levels. There is no
cortical destruction or associated soft tissue mass. Coronal
Some soft tissue tumors can be diagnosed with
certainty based on their MR signal characteristics.
For example, fatty masses demonstrate high sig-
nal on T1W images, which suppress on fat satu-
ration (Fig. 27).
Gadolinium is utilized to aid in the differenti-
ation of cystic versus solid masses. Some
cystic-appearing lesions include ganglion cyst,
intramuscular myxoma, synovial sarcoma, and
nerve sheath tumors. A purely cystic mass
demonstrates homogenous low signal on T1W
images and high signal on T2W images. After
A. Malik et al.
pre- (c) and post-contrast (d) images show peripheral and
septal enhancement within this lesion. (e) Plain radiograph
shows increased sclerosis in the distal ulnar lesion post
curettage and grafting
intravenous gadolinium administration, there is
enhancement of the thin, peripheral wall
without central enhancement (Fig. 28). In addi-
tion, MR allows for complete evaluation of
the extent of the mass and involvement of the
adjacent bones and neurovascular structures
(Fig. 29).
As many benign and malignant lesions are
T1-hypointense and T2-hyperintense and enhance
after administration of contrast, MR may not be
able to differentiate between these lesions, thus
necessitating biopsy.
5 Imaging
Fig. 25 Multiple hereditary exostoses. Frontal (a) and
lateral (b) radiographs of the left forearm show multiple
osseous protuberances arising from proximal and distal
radius and ulna consistent with osteochondromas. No path-
ologic fracture. Follow-up MR was obtained to assess for
97
vascular compression. Coronal T1 (c) image again shows
multiple osteochondromas arising from radius and ulna
with cortical and medullary continuity. Coronal MIP
image (d) from MR angiogram shows the short segment
narrowing of the radial artery (arrow)
98 A. Malik et al.

Fig. 26 (continued)
5 Imaging
Fig. 26 Osteosarcoma of proximal humerus
multimodality evaluation. Plain radiographs (a and b)
show an aggressive destructive lesion in the proximal
humeral metadiaphysis with osteoid matrix, periosteal
reaction and Codmans triangle (arrow). Tc-99m bone
scan (c) shows uptake in the humeral lesion. Follow-up
MRI with coronal T1 (d) and sagittal STIR (e) sequences
depict the lesion as T1 low and T2 high signal intensity.
MRI is routinely used for routine post-therapy
follow-up of malignant osseous and soft tissue neo-
plasms, as well as some benign but locally aggressive
lesions, including bromatosis and lipoblas-
tomatosis. MRI can also be used to evaluate postsur-
gical complications, such as a seroma.
Vascular Lesions
Vascular malformations comprise a broad spec-
trum of lesions which can involve the arterial,
venous, or lymphatic systems. MRI is an excellent
noninvasive tool for imaging and classifying vas-
cular malformations based on the presence of
lobulated masses, signal voids, and hemodynamic
ow characteristics (Donnelly et al. 2000). The
detailed anatomic extent of the lesion, proximity
to vital structures, and involvement of multiple
tissue planes can be obtained with MRI. With
the advent of 3-dimensional time-resolved MR
99
Imaging for a malignant bone lesion is done joint to joint
to assess for any additional skip lesions. Cortical destruc-
tion and associated enhancing soft tissue component are
better seen on the axial PD FS (f) and post-contrast axial
LAVA (g) images. Posttreatment plain radiograph (h)
shows interval resection of humeral osteosarcoma with
prosthesis in place
angiography, a dynamic functional assessment of
these lesions can be obtained; thus, this is emerg-
ing as a useful noninvasive alternative to conven-
tional catheter digital subtraction angiography
(DSA) (Donnelly et al. 2000).
The vascular malformations are classied into
high-ow and low-ow type on MRI. This classi-
cation has signicant impact on the management,
as the treatment of the former is transarterial embo-
lization while the latter is treated with percutaneous
sclerotherapy (Donnelly et al. 2000).
The prototype of vascular tumors is an infantile
hemangioma with its typical involution after a
proliferative phase during infancy (Donnelly
et al. 2000). Hemangioma appears as a markedly
T2-hyperintense lesion, often referred to as light
bulb bright. This demonstrates the intensely
enhancing soft tissue mass with enlarged feeding
arteries and draining veins.
Venolymphatic malformations are inltrative
multilocular, multiseptated masses that are
100 A. Malik et al.

Fig. 27 Lipoma of the

arm. (a). Initial evaluation
by targeted US shows
circumscribed echogenic
mass in the soft tissues,
consistent with fatty mass
such as lipoma. MRI of the
right arm shows high signal
intensity on T1W images
(b) which lose signal on fat
suppression (c), conrming
fatty nature of the mass.
Pre- (d) and post-contrast
(e) LAVA images,
respectively, do not
demonstrate enhancement,
therefore excluding
sarcomatous elements

T2-hyperintense and may show uid-uid diffusely enhance during the venous phase of
levels. Lesions may have coexisting venous and contrast enhancement (Fig. 30), while the lym-
lymphatic components. The venous components phatic components only have septal enhancement.
5 Imaging 101

Fig. 28 Ganglion cyst. MRI of the left hand. (a), (b), and insinuating just deep to the exor tendons. (d) and (e)
(c) coronal STIR, T1, and axial STIR images, respec- Pre- and post-contrast LAVA images demonstrate only
tively, show lobulated uid signal intensity lesion peripheral enhancement conrming cystic nature

Phleboliths, which are hypointense on both T1- will have enlarged, high-ow feeding arteries, a
and T2-weighted images, are characteristic but nidal tangle of vessels, and early lling draining
not always present (Donnelly et al. 2000) veins without an associated soft tissue mass
(Fig. 31). Arteriovenous malformations (AVMs) (Donnelly et al. 2000).
102 A. Malik et al.

Fig. 29 Synovial
sarcoma. MRI of the left
elbow. Coronal (a, b) STIR
images demonstrate
lobulated T2-hyperintense
mass arising from the volar
musculature with
heterogeneous
enhancement on post-
contrast coronal (c) and
axial (d) images

Vascular malformations may be seen in conjunc- common than soft tissue involvement.
tion with other ndings in several syndromes, Intraosseous lymphatic malformations are
including Klippel-Trnaunay syndrome (capillary- most commonly associated with other soft
venolymphatic malformation with limb tissue and visceral lymphatic malformations,
overgrowth), Parkes Weber syndrome in the setting of lymphangiomatosis. In
(AVM-capillary-lymphatic malformations with Gorhams disease, also known as vanishing
limb overgrowth), Maffucci syndrome (multiple bone disease, the uncontrolled proliferation of
enchondromas and venous malformations), and multiple venolymphatic malformations leads to
blue rubber bleb nevus syndrome (cutaneous venous resorption and replacement of bone, so that they
malformations and internal venous malformations, are invisible on radiographs.
most commonly intestinal) (Donnelly et al. 2000). In the setting of penetrating trauma to a blood
Primary osseous involvement of vessel, pseudoaneurysm can occur and can be
venolymphatic malformations is much less assessed with MR angiography as well.
5 Imaging 103

Fig. 30 Lymphatic
malformation. US (a)
shows multiloculated cystic
mass with intervening
septations and no
appreciable color ow. MRI
of the right shoulder with
coronal (b) and axial (c)
STIR images conrm the
multilobulated high-signal-
intensity mass in the right
axilla which insinuates in
surrounding soft tissues.
Note absence of ow voids
as expected for a low-ow
vascular malformation.
There is peripheral and
septal enhancement on
post-contrast T1 FS
(d) images
104 A. Malik et al.

Fig. 31 (continued)
5 Imaging
Fig. 31 Venous malformation. Frontal (a) and lateral (b)
radiographs of the left hand show lobulated soft tissue
mass along the dorsolateral forearm and hand without
evidence of calcications. The osseous structures appear
normal. MRI/MR angiogram of the left forearm and hand.
Coronal STIR (c, d) images demonstrate high T2 signal
serpiginous mass along the soft tissues of the left forearm
Computed Tomography
Computed tomography (CT) is primarily
employed as a complementary imaging modality
in the evaluation of the upper extremity due to its
limited soft tissue and bone marrow resolution
(Brant and Helmes 2007), as well as associated
105
and hand. The mass contains internal areas of T2
hypointensity which may represent phleboliths or
thrombosed vessels. Dynamic contrast enhanced MR
angiogram (TRICKS) in arterial (e) and venous (f). Phase
reveal delayed lling of venous lakes with absence of
arterial feeders
high doses of ionizing radiation. The few
instances in which CT is preferred to other modal-
ities in the evaluation of the upper extremity will
be discussed below.
Vascular injury. In the evaluation of a vascular
injury in the setting of blunt or penetrating trauma,
CT angiography provides excellent assessment of
large and small vessels. Using various techniques,
106
the radiologist can coordinate the timing of the
injection of iodinated contrast with the scan time
so that the vascular structures are optimally
enhanced. CT can also be used to localize foreign
bodies within the soft tissues with respect to adja-
cent vascular structures.
Complex fractures. While radiographs are
often sufcient to evaluate fractures, CT can be
used as an adjunct to radiographs prior to surgical
intervention, especially in the setting of fractures
about the shoulder joint or articular fractures in
adolescents approaching skeletal maturity (Brant
and Helms 2007). Two-dimensional images can
be post-processed into three-dimensional images
of the osseous structures, providing a road map
prior to surgery (Fig. 32).
Osteomyelitis. While MRI and skeletal scintig-
raphy are superior to CT in the evaluation of
osteomyelitis, CT is occasionally used in
detecting changes of chronic osteomyelitis
(Donnelly 2008), including formation of cavities,
sequestra, involucra, and sinus tracts.
Neoplasia. Following the initial plain radio-
graphs, most osseous neoplasms are further
assessed by MRI. However, CT is optimal in the
evaluation of a suspected osteoid osteoma, as CT
shows the location of the associated nidus better
than MRI (Donnelly 2008). Percutaneous thera-
pies, including radiofrequency ablation or injec-
tion of sclerosing agents, can be performed using
CT guidance (Fig. 33).
If myositis ossicans is suspected, CT is supe-
rior to MRI for the evaluation of calcications. The
calcications may not be apparent and the lesion
may mimic a mass on MRI; if biopsied, the histol-
ogy may be confused with an osteosarcoma.
However, when radiographs and CT show the char-
acteristic peripheral calcication with central
lucency, the diagnosis of myositis ossicans can
be conrmed and this is considered a dont touch humeral head displacement due to abnormal mus-
lesion (Brant and Helms 2007).
Ultrasound
Musculoskeletal ultrasound is an extremely useful
modality in the pediatric population. Neonates
and young infants can be imaged without
A. Malik et al.
sedation. In some instances it may be the only
imaging modality required after plain radiographs
have been obtained (Fig. 27).
Neonates and young infants have thinner soft
tissues and long bones with ends that are largely
composed of cartilage; thus, they may readily be
examined by ultrasound (Keller 2005).
Furthermore, ultrasound is portable and readily
available and does not involve ionizing radiation.
It is, however, operator dependent.
Radiologists need to be cognizant of the
appearances of imaging pitfalls involving the
upper extremity such as the thickness and
echogenicity of the supraspinatus tendon which
may resemble that of the subacromial-subdeltoid
bursa (van Holsbeeck and Strouse 1993), the nor-
mal appearance of the posterior interosseous
nerve which has normal alterations in contour
about the elbow joint and not due to entrapment
(Jamadar et al. 2010), and variations in the
echogenicity of the normal extensor retinaculum
at the wrist that may simulate a complex
uid collection or tenosynovitis (Robertson
et al. 2007).
Applications of Upper Extremity
Ultrasound
Shoulder
The shoulder joint, including the cartilaginous
humeral head, glenoid, and surrounding soft tis-
sues, can be examined by ultrasound (Grissom
and Harcke 2001).
Effusions can be identied and subsequently
aspirated under ultrasound guidance.
Brachial plexus neuropathy and the signs of
Erbs palsy at birth are not infrequently encoun-
tered and can lead to postnatal posterior
cular balance. The orientation of the glenoid with
respect to the axis of the scapula and abnormal
angles or contours of the glenoid may be
ascertained by ultrasound (Keller 2005). CT,
MRI, and especially MR neurography (Chhabra
et al. 2011) are now being increasingly utilized
for neonatal brachial plexopathies (Figs. 13
and 14).
5 Imaging
Fig. 32 Shoulder fracture dislocation. (a)
Anteroposterior radiographs in internal rotation (i) and
external rotation (ii) as well as a Y view radiograph (iii)
demonstrate a right proximal humeral fracture dislocation.
A transverse fracture is seen through the surgical neck of
the humerus and the humeral head is dislocated inferopos-
teriorly within the glenohumeral joint. (b) The subsequent
107
noncontrast CT was obtained for surgical planning, with
select images in axial (i) and sagittal (ii) planes
redemonstrating similar ndings. (c) Posttreatment radio-
graph in anteroposterior projection demonstrates a near-
anatomic alignment of the humeral head and distal fracture
fragment after open reduction and internal xation
108
Fig. 33 Osteoid osteoma. (a) Coronal view of noncontrast CT shows extensive cortical thickening of the lateral humeral
diaphysis with a cortical radiolucent nidus. (b) CT-guided percutaneous radiofrequency ablation of osteoid osteoma
Trauma
Plain radiography is the modality of choice for the
evaluation of fractures; however, ultrasound may
be of assistance when the initial radiographs are
equivocal or negative (Graif et al. 1988). Injuries
involving the elbow joint are common in children,
and disruption or discontinuity of the echogenic
line that represents the periosteum is indicative of
an underlying fracture (Pai and Thapa 2013).
Soft tissue injuries of the upper extremity can
be well assessed by ultrasound (Pai and Thapa
2013). These include tears of the rotator cuff and
biceps tendon and injury to the ulnar collateral
ligament of the elbow or thumb.
Derangement about the elbow joint can also be
assessed dynamically using a linear high-frequency
transducer (Pai and Thapa 2013). Radial head dis-
locations are frequently encountered and injuries to
the annular ligament may be demonstrated as focal
areas of disruption or areas of hyperechogenicity
within the substance of the ligament when the
ligament is partially torn (Kim et al. 2004).
Physeal Separation
Slipped humeral epiphysis is an uncommon birth
injury that is not well assessed by plain radiogra-
phy, but ideally demonstrated on ultrasound
(Graif et al. 1988). Similarly, separation of the
distal humeral epiphysis may be demonstrated
with ultrasound.
A. Malik et al.
Infection
Soft Tissue Swelling (Cellulitis)
Extra-articular soft tissue swelling with oblitera-
tion of the normal fascial planes and increased
echogenicity within the soft tissues compared to
the surrounding normal tissues can be demon-
strated by ultrasound. A normal joint space with
normal deep soft tissue adjacent to the bone is
indicative of cellulitis, thereby excluding septic
arthritis, osteomyelitis, or fracture (Markowitz
et al. 1992).
Osteomyelitis
Ultrasound plays a limited role in establishing the
diagnosis of osteomyelitis. Its main value is the
demonstration of periosteal elevation of the
infected bone and associated deep soft tissue
inammatory changes (Abiri et al. 1989).
Necrotizing Fasciitis
Distortion and thickening of the fascial planes and
uid accumulation between tissue layers have
been detected on ultrasound (Chao et al. 1999).
Vascular Abnormalities
Ultrasound with Doppler is useful in the demon-
stration of normal vascular anatomy and to dem-
onstrate vascular occlusion, aneurysms, or
pseudoaneurysms.
5 Imaging 109

Soft Tissue Abnormalities

Masses including ganglion cysts, tumors, hemato-
mas, and cellulitis with uid collections may be
characterized on ultrasound (Fig. 27). Ganglion
cyst is the most common focal pediatric wrist
mass. It is easily amenable to evaluation by ultra-
sound, although the imaging appearances are vari-
able. Lesions 10 mm or less in the greatest
dimension often appear hypoechogenic (Wang
et al. 2007) although most appear complex (Teefey
et al. 2008).

Nuclear Medicine/PET

Pediatric musculoskeletal scintigraphy provides a

generalized functional evaluation of the upper
extremity in cases of suspected infectious/
noninfectious inammatory, traumatic, ischemic,
and neoplastic conditions. When combined with
SPECT or SPECT/CT imaging, precise localiza-
tion of an abnormality may be achieved and cor-
related with other cross-sectional imaging
modalities such as CT and MRI.
The most commonly used imaging agent for
bone scintigraphy is technetium-99m tagged to
methylene diphosphonate (MDP), a phosphate
analog. Once injected intravenously, it localizes
physiologically to the bony cortex of the axial and
appendicular skeleton with disproportionate
activity seen in the growth plates as a result of a
high rate of bone turnover (Fig. 34). Abnormally
increased or decreased activity on a bone scan is
usually associated with alterations in blood ow
or metabolic activity.
The major advantage of bone scintigraphy is
its high sensitivity for a lesion(s) of variable eti-
ologies. Its major disadvantages include a lack of
lesion specicity (and therefore a need for correl-
ative imaging), relatively high radiation dose,
high cost, and oftentimes the need for sedation
in younger children (Stauss et al. 2010).
Abnormal upper extremity uptake may also be
seen with iodine-123 MIBG imaging, for exam-
ple, in a child with stage IV neuroblastoma. In
these cases, as in cases of other neuroendocrine
tumors, augmented activity within the axial and
appendicular skeleton may be due to inltration of
Fig. 34 Normal bone scan in a 5-year-old. Note physi-
ologically increased uptake in the growth plates of the
extremities
the cortical bone and/or bone marrow with tumor
(Kushner 2004) (Fig. 35).
Positron emission tomography (PET) imaging
is a form of molecular imaging that offers a unique
way of assessing functional impairments in organs/
organ systems, including the musculoskeletal sys-
tem. One of the more commonly used agents is
18
F-uorodeoxyglucose (FDG), the localization of
which is determined by alterations in glucose utili-
zation. Its primary use is in oncologic imaging and,
when combined with CT or MRI, offers a precise
means of detection, localization, and follow-up of
tumor deposits (Buchbender et al. 2012).
Interventional Radiology
Interventional radiology is now being increas-
ingly utilized as it not only aids the orthopedic
surgeon in the diagnostic workup of many abnor-
malities of the upper extremity but may also be
part of the treatment (Donaldson 2008).
110 A. Malik et al.

Trauma injury. Arteriography and venography can be

performed to detect vascular extravasation, arte-
While radiography and CT are useful tools in rial dissection, and venous thrombosis. Some of
identifying osseous injury, angiography can be the treatment options for traumatic vascular inju-
performed to both diagnose and treat vascular ries include embolization and stent grafting.

Fig. 35 (continued)
5 Imaging 111

Fig. 35 Metastatic neuroblastoma. (a) Plain lms of the
chest and lower extremities demonstrate multiple lytic
lesions of the right humerus in an irritable 2-year-old.
There is a suggestion of a soft tissue mass in the left
upper quadrant of the abdomen. (b) Coronal CT shows a
large left adrenal mass, a pathologically proven neuroblas-
toma. (c) Technetium-99m MIBG study demonstrates mul-
tiple foci of abnormally increased activity in the skeleton
consistent with advanced-stage disease. (d) Normal I-123
MIBG scan for comparison

Fig. 36 Upper extremity

arteriogram. Embolus at
left brachial artery
bifurcation into radial and
ulnar arteries
112 A. Malik et al.

Fig. 37 Upper extremity pseudoaneurysm post stab pseudoaneurysm (arrow). Patient underwent an emboliza-
wound to forearm. Axial image from CT angiogram (a) tion procedure. Pre- (c) and post-embolization (d) images
and coronal volume-rendered image (b) demonstrate the depict successful coiling of the pseudoaneurysm
5 Imaging
Infection
Certain infectious processes of the upper extrem-
ity can be diagnosed and treated by the interven-
tional radiologist. Ultrasound can be used to
readily visualize soft tissue abscesses and provide
guidance for percutaneous drainage. An added
benet of ultrasound is that it obviates the use of
radiation (Chau and Grifth 2005).
Non-traumatic Vascular Abnormalities
A wide spectrum of lesions including subclavian
steal syndrome, Takayasus arteritis, Raynauds
phenomenon, hypothenar hammer syndrome,
Paget disease, and vascular malformations such
as arteriovenous malformations, arteriovenous s-
tulae, and venolymphatic malformations can also
be diagnosed utilizing angiography.
In patients with malfunctioning upper extremity
dialysis grafts or arteriovenous stulae, minimally
invasive angiography can be performed to identify
areas of stenosis, areas of pseudoaneurysm forma-
tion, or areas of blood clot, and when an abnormal-
ity is detected, treatment can be attempted during
the same procedure using a variety of techniques
such as sclerotherapy, directed thrombolysis, and
embolization (Dubois and Garel 2002, Mulliken
et al. 2000) (Figs. 36 and 37).
While MRI and magnetic resonance angiogra-
phy (MRA) and CT are used for the initial dem-
onstration of most soft tissue masses of the upper
extremity, conventional arteriography can be
performed in order to determine their precise vas-
cular supply, and certain masses like hemangi-
omas can be successfully treated with
sclerotherapy (Donnelly et al. 2000). Some
masses may be initially treated with embolization.
Neoplasia
In addition to soft tissue and vascular masses,
some bone tumors like osteoid osteomas can be
treated by the interventional radiologist utilizing
image-guided radiofrequency ablation (Donkol
et al. 2008) (Fig. 33).
113
Ultrasound guidance may be performed during
biopsy of both soft tissue and osseous masses and
ultrasound can help guide ne-needle aspiration
of lymph nodes.
Additional Applications
In addition to the diagnosis and treatment of
numerous conditions as described above, the
interventional radiologist can place peripheral
lines such as ports for chemotherapy and
peripheral inserted central catheters (PICC lines)
for long-term antibiotic therapy (Nosher
et al. 1994).
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 Part IV
Anesthesia
 Anesthesia
6
Andrew J. Costandi and Vidya Chidambaran

Contents Abstract
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 118 The use of general anesthesia in pediatric
patients has greatly increased as a result of a
Preoperative Assessment and Preparation . . . . . . . 118
Preoperative-Fasting Guidelines . . . . . . . . . . . . . . . . . . . . . 119
growing number of outpatient orthopedic sur-
Preoperative Assessment . . . . . . . . . . . . . . . . . . . . . . . . . . . . 119 gical procedures. In children, upper extremity
surgical procedures usually result from trauma,
Intraoperative Management . . . . . . . . . . . . . . . . . . . . . . . 120
Induction of Anesthesia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 120 brachial plexus injuries related to birth injuries,
Airway Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 121 congenital hand disorders, or joint contractures
Maintenance of Anesthesia . . . . . . . . . . . . . . . . . . . . . . . . . . 123 and are often conducted under general anesthe-
Emergence from Anesthesia . . . . . . . . . . . . . . . . . . . . . . . . . 123 sia. Despite its common use among children,
Postoperative Pain Management . . . . . . . . . . . . . . . . . . 124 general anesthesia-related neurotoxicity
Systemic Analgesia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 124 remains an issue of controversy. Much medical
Local Anesthetics and Adjuncts . . . . . . . . . . . . . . . . . . . . . 127
Local Anesthetics Systemic Toxicity (LAST) . . . . . . . 128
debate currently exists surrounding the
Regional Anesthesia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 129 safety and complication rates of general anes-
The Brachial Plexus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 130 thesia, and thus, there has been a recent
Intravenous Regional Anesthesia (Bier Block) . . . . . 136 increase in the awareness of the need, success
Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 136 rates, and advantages of the use of regional
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 137 anesthesia among the pediatric population dur-
ing surgical procedures. Effective, safe, and
prolonged postoperative pain control allowing
early discharge from the hospital is an added
benet of the use of regional anesthesia, espe-
cially with the introduction of continuous
peripheral nerve blocks. This chapter will
focus on the preoperative anesthetic concerns
and preparations; the several types of anesthe-
Disclosures: Both authors have no conicts of interest to sia and their applicability to various age
disclose. groups; the anesthetic precautions surrounding
A.J. Costandi (*) V. Chidambaran different surgical procedures; the different
Department of Anesthesiology, Division of Pain regional anesthesia techniques, their appropri-
Management, Cincinnati Childrens Hospital Medical
ate selection and management for blockade,
Center, University of Cincinnati, Cincinnati, OH, USA
e-mail: Andrew.costandi@cchmc.org; and their implementation into daily clinical
vidya.chidambaran@cchmc.org practice; and nally postoperative pain
# Springer Science+Business Media New York 2015 117
J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_6
118
management through both pharmacological
and non-pharmacological interventions in chil-
dren. A complete discussion of local, regional,
and general anesthesia will also be provided
along with the safety of each within the pedi-
atric population in hopes of increasing knowl-
edge of the various anesthetic techniques that
currently exist within the eld of medicine.
Introduction
Common indications for surgery on the upper
extremity in children include (1) hand and arm
fractures from falls and accidental trauma; (2) bra-
chial plexus injury related to birth injuries, which
require surgeries like upper extremity nerve, func-
tional muscle, and tendon transfers; (3) congenital
hand disorders including syndactyly, polydactyly,
symbrachtyly, and amniotic band syndrome;
(4) joint contractures in patients with cerebral
palsy and arthrogryposis; and (5) joint surgeries
for patients with arthritis, shoulder dislocation,
and sports injuries, among others. Although gen-
eral anesthesia is often used for children because
they lack the emotional maturity and understand-
ing to be awake during surgical procedures,
anesthesia-related neurotoxicity remains a highly
debated and controversial issue, especially in
infants. However, in the absence of convincing
evidence, the International Anesthesia Research
Society and the American Academy of Pediatrics
have issued a consensus statement which states
that it would be unethical to withhold sedation and
anesthesia when necessary (U.S. Food and Drug
Administration 2012). There is an increasing ten-
dency towards using regional anesthesia with
sedation or ultrasound-guided nerve blocks
under general anesthesia for this population to
allow for prolonged postoperative analgesia with
the use of pumps that are compact, are easy to
operate, and can provide safe infusion rates of
local anesthetics even at home. This chapter will
discuss the anesthetic considerations and tech-
niques commonly used in pediatric anesthesia
for upper extremity surgery under three broad
headings: preoperative preparation and assess-
ment, intraoperative management including
A.J. Costandi and V. Chidambaran
immediate postoperative care, and postoperative
pain management including use of regional anal-
gesic techniques.
Preoperative Assessment
and Preparation
Understandably, most children presenting for sur-
gery exhibit anxiety and distress preoperatively,
and up to 50 % of them may be signicantly
anxious (Kain et al. 1997; Davidson et al. 2006)
requiring preoperative measures. Besides fear of
surgery and death, facing a new environment, the
greens and whites of physicians and nurses,
procedures like blood pressure measurement,
hunger from fasting, and separation anxiety from
family are important factors feeding patients anx-
iety. This is especially compounded in children
who have had previous surgical experiences
(Rosen et al. 1993), developmental disabilities,
personality disorders, or pain and adolescents
who are often fearful of loss of control.
Ideally, preoperative preparation should begin
much earlier than when the child comes in for
surgery; instead, it should begin in the surgeons
ofce where the patient and parent(s) are informed
about the surgery. Moreover, many institutions
place a preoperative call the day or a few days
before surgery to inform the parents about the
expectations for fasting, the day of surgery, etc.
The following are suggested means of reducing
preoperative anxiety:
1. Information: Preoperative tours may be helpful
especially for older children so they can famil-
iarize themselves with their surroundings and
know what to expect when they arrive for
surgery. While information on both procedural
(what will happen) and sensory (what it will
feel like, including pain) aspects must be
included, what is important is how and by
whom it is presented. It has been recommended
that preparation should be provided at least
5 days in advance for children >6 years of
age and no more than a week in advance for
children <6 years of age (MacLaren and Kain
2007).
6 Anesthesia 119

Table 1 Commonly used preanesthetic medication

Drug Route Dose (mg/kg) Onset (min) Duration (min)
Midazolam PO 0.250.75 1530 6090
IV 0.050.15 13 6090
Nasal 0.10.2 10 60
Diazepam PO 0.050.1 3060 180240
IV 0.050.1 13 180240
Pentobarbital PO 5 30 120240
Ketamine PO 35 1020 3060
IM 23 510 3060
Clonidine PO 0.004 90 360720
Dexmedetomidine PO 0.002 3045 60120
Nasal 0.0010.002 3045 60120
This is a list of medications used for premedication before anesthesia and their recommended doses via commonly used
routes of administration, along with time to onset of anxiolytic effect and duration of effect. IV intravenous; PO per oral,
IM intramuscular

2. Teaching coping skills via a peer model: These
can be in the form of relaxation, deep breath-
ing, distraction, parent and nurse coaching of
the child, nonprocedural conversations, use of
child life services and electronic media like a
cartoon on an ipad, etc.
3. Written materials alone are not effective forms
of preparation.
4. Premedication: Preoperative anxiolysis might
be helpful before certain surgeries for children
who are delayed and not pliable with the above
methods and in children with medical condi-
tions like congenital heart disease, brain aneu-
rysms, etc., which necessitate smooth
induction and also in conditions where parental
presence is not safe for the patient (e.g., full
stomach patient for rapid sequence induction
or precarious airway). Also, parental presence
during induction might be a boon sometimes
and a bane especially if the parent is highly
anxious. Kain et al. have shown that
premedication allowed for decreased levels of
anxiety during stressful situations such as sep-
aration and induction (Kain et al. 1998). At the
same time, it is necessary to remember that
premedications like benzodiazepines are usu-
ally sedatives and can worsen airway obstruc-
tion after short surgical procedures in patients
with sleep apnea and can cause prolonged
sedation, emergence delirium, and even para-
doxical excitation. The list of commonly used
premedications and their doses and routes of
administration is provided in Table 1.
Preoperative-Fasting Guidelines
Preoperative fasting is a balance between
maintaining an empty stomach to prevent aspiration
of gastric contents on induction of anesthesia with
an unprotected airway and a dehydrated, irritable,
and hypoglycemic patient. Based on gastric empty-
ing times which is smaller for clear isotonic liquids
(35 min) < clear liquids containing protein/lipids/
glucose (10 min) < light nonfatty snack (12 h) <
full meal (8 h) (Moore et al. 1981), recommenda-
tions for preoperative fasting have been formulated
for children (Cote 1990; Strunin 1993). In general,
in a healthy child, the fasting time for clear uids is
2 h before surgery, 4 h for breast milk, 6 h for
formula feeds, and 8 h for solid meals. It is impor-
tant to remember that more conservative guidelines
may be advisable in patients with conditions like
pregnancy, morbid obesity, history of recent acute
trauma, raised intracranial pressure, or therapy with
opioids, which all slow gastric emptying.
Preoperative Assessment
Patients are assessed preoperatively for
medical history, review of systems, and pertinent
120
physical exam. Medical history especially
focuses on presenting history, allergies, medica-
tions, signicant organ system-related medical
problems, history of prematurity and related
issues, previous history of anesthesia and family
history of anesthesia, presence of syndrome,
recent infections especially upper respiratory
infections, snoring and sleep apnea, and fasting.
Physical exam and ROS include pertinent his-
tory and also an airway exam. This is done by
looking and listening for signs of airway narrowing
(e.g., suprasternal narrowing or stridorous sounds)
and examining for facial anomalies, like a retracted
mandible, adequacy of mouth opening and tempo-
romandibular joint mobility, relative size of tongue
to the oral cavity (Mallampati classication
(Mallampati 1983; Samsoon and Young 1987),
thyromental distance, and range of movement of
the neck. Based on difculty in exposure of the
vocal cords during laryngoscopy, Cormack and
Lehane have graded the difculty of tracheal intu-
bation (Cormack and Lehane 1984). Of course, the
Mallampati score is not practical in infants and
young children due to poor cooperation. Also, the
correlation with the score and laryngoscopic expo-
sure was not found to be optimal in pediatric
patients. Predictors of difcult airways include
known history of difcult airway, upper airway
obstruction including epiglottitis or airway masses,
micro- or retrognathia (as in patients with Pierre-
Robin syndrome), large tongue (as in patients with
Down syndrome or Beckwith-Weidman syn-
drome), limited mouth opening (as in patients
with rheumatoid arthritis affecting the temporo-
mandibular joint), limited cervical spine mobility
(as in patients with Klippel-Feil syndrome with
fused spines), unstable cervical spine (as in patients
with Down syndrome with atlantoaxial subluxa-
tion), and conditions like epidermolysis bullosa.
Intraoperative Management
The intraoperative care of the pediatric patient can
be described in three stages: induction, mainte-
nance, and emergence. While general anesthesia
is the technique used most commonly, regional
anesthesia and sedation or monitored anesthesia is
A.J. Costandi and V. Chidambaran
rarely employed for cooperative older children or
other patients depending on their comorbidities.
Standard American Society of Anesthesiologists
recommended monitors including electrocardio-
graph, noninvasive blood pressure, pulse oxime-
try, capnography, and temperature monitoring to
be employed during routine cases in the
operating room.
Induction of Anesthesia
Induction of anesthesia is usually via an inhala-
tional or intravenous route, though intramuscular
ketamine (Hannallah and Patel 1989) and rectal
methohexital (Forbes et al. 1989) could also be
used. Inhalation induction is the most common
method and allows for a more rapid, less stressful
induction for a younger child (Kotiniemi and
Ryhanen 1996; Kain et al. 1999). In children, the
higher minute ventilation to functional residual
capacity ratio and the increased blood ow to the
vessel-rich organs, like the brain, allow for more
rapid equilibration of volatile anesthetic in neo-
nates, infants, and small children. Halothane has
been replaced by sevourane as the agent of
choice for inhalation induction. The minimal alve-
olar concentrations, solubility, and side effects of
different volatile anesthetics are provided in
Table 2. Sevourane is less pungent than
isourane and desurane and causes less myocar-
dial depression and sensitivity to arrhythmias
compared to halothane (Navarro et al. 1994;
Holzman et al. 1996). Emergence delirium, how-
ever, has been more of a problem with sevourane
than compared with other volatile agents.
Intravenous induction is preferred in older chil-
dren and those with full stomachs, severe sleep
apnea, a propensity to obstruct airway on induc-
tion, and medical conditions such as aortic steno-
sis. Nitrous oxide in oxygen is commonly used in
older children to facilitate intravenous access. The
topical use of a eutectic mixture of local anes-
thetics like EMLA, composed of 2.5 % prilocaine
and 2.5 % lidocaine, produces anesthesia of the
skin after application with an occlusive dressing
for about 60 min. The time delay, need for prep-
aration of multiple potential sites, and discomfort
6 Anesthesia 121

Table 2 Inhalational anesthetic agents

Partition coefcient
Inhalational gas Blood/gas Oil/gas MAC (%) Major side effects
Nitrous oxidea (I) 0.47 1.4 104 Diffuses in gas-containing spaces
Emetogenic
Greenhouse gas that depletes the ozone layer
Halothaneb (I) 2.3 220 0.75 Dose-dependent myocardial depression,
arrhythmogenic
20 % of agent is metabolized leading to increased risk of
nephrotoxicity and liver toxicity
Isourane 1.4 97 1.15 Pungent, airway irritation
Sevourane (I) 0.68 53 2.5 Bradycardia especially in patients with Down syndrome
Emergence agitation
Formation of compound A which is nephrotoxic in rat
studies
5 % of agent is metabolized leading to increased risk of
nephrotoxicity and liver toxicity
Desurane 0.42 18.7 6 Pungent, airway irritation
Black box warning against using it in inhalational
induction
Carbon monoxide formation from when desiccated in
CO2 absorbents
This table lists the anesthetic gases used for induction (I ) and maintenance of anesthesia with indicators of their solubility
in blood and oil, minimum alveolar concentrations (MAC), which is the alveolar concentration of the gas that prevents
movement (motor response) in 50 % of subjects in response to surgical (pain) stimulus, and major side effects of the gases
a
Nitrous oxide can only be used as an analgesic adjunct and cannot provide complete anesthesia due to its high MAC
b
Halothane is used less commonly in the developed world due to listed adverse effects
PONV postoperative nausea and vomiting

related to an approaching needle may, neverthe- Airway Management

less, act as drawbacks with this method. Induction
agents commonly used are propofol, thiopental,
etomidate, and ketamine. Doses of these agents
and their effects on the various systems are
presented in Table 3. Lidocaine is often used
before or along with propofol and etomidate due
to pain on injection, although it is not always
successful (Mirakhur 1988). Propofol has the
advantages of more rapid awakening, less emer-
gence delirium, and less nausea but does cause
more hypotension if given in large doses.
Etomidate is more cardiac stable but causes myoc-
lonus and adrenal suppression, which may not be
a problem with induction doses (Bergen and
Smith 1997). Ketamine is a dissociative anesthetic
and an NMDA blocker, with analgesic effects that
maintain respiration but increase secretions, intra-
cranial pressure, blood pressure, heart rate, and
occurrence of emergence delirium (Reich and
Silvay 1989).
Airway management is crucial for general anes-
thesia. An infant airway is different from that of
older pediatric and adult airways in a number of
ways; the most important of which are larger
occiput, larger tongue relative to size of the oral
cavity, more cephaled larynx (C34), narrower
epiglottis, and the cricoid cartilage being the
narrowest portion of the larynx and not the vocal
cords. The practical implications are that for intu-
bation, the infant head does not require to be
extended (maintain in neutral position) for the
laryngeal/pharyngeal and oral axes to line up;
straight laryngoscope blades (Miller/Phillips)
may be preferable to curved ones (Macintosh);
and, it may be preferred to use uncuffed endotra-
cheal tubes compared to cuffed ones (Marraro
2002). Recent practice has changed the use of
cuffed endotracheal tubes with very short high-
volume low-pressure (HVLP) cuffs made from
122
Table 3 Intravenous anesthetic agents
IV dose
Drug Mechanism (mg/kg)
Propofol Nonbarbiturate anesthetic 25
GABA potentiation
Thiopental Barbiturate 35
GABA agonist
Etomidate Carboxylate imidazole 0.20.3
derivative
GABA receptor modulator
Ketamine Phencyclidine derivative 12
NMDA antagonist, dissociative
anesthesia
Listed are the commonly used intravenous agents for induction of anesthesia, their mechanism(s) of action, and actions on
various systems; the recommended intravenous (IV) induction dose is also tabulated; GABA -amino butyric acid;
CV cardiovascular; RS respiratory system; CNS central nervous system; PONV postoperative nausea, vomiting;
ICP intracranial pressure; IOP intraocular pressure
polyurethane, with improved sealing characteris-
tics (Weiss et al. 2004). There are myriads of
intubation assist devices available in the difcult
airway armamentarium (e.g., Glidescope (Sat-
urn Biomedical System Inc., Burnbaby, Canada),
intubating laryngeal mask airway, and breoptic
bronchoscopy) (Sunder et al. 2012).
Airway management is usually accomplished
either by use of mask anesthesia, use of laryngeal
mask airways, or use of endotracheal tubes. Mask
anesthesia using appropriate size mask (5), oral or
nasal airways, and chin-lift/head-tilt and
jaw-thrust maneuvers as required are usually
used for shorter procedures such as closed fracture
reduction, excision of extra digits, etc. The laryn-
geal mask (LMA) is a supraglottic airway device
that was designed by British anesthesiologist
Dr. Brain. It is placed such that the aperture in
the mask is positioned at the laryngeal inlet. It is
often used instead of the mask as it helps free the
anesthesiologists hands. While it is less invasive
than an endotracheal tube, is relatively easy to
A.J. Costandi and V. Chidambaran
Salient properties
CV: dose-dependent cardiovascular depression
RS: blunts airway reexes, respiratory depression
CNS: hypnosis, amnesia, no analgesia
Quicker recovery, less PONV, pain on injection
CV: cardiac depression
RS: bronchoconstriction in asthmatics, loss of
airway tone
CNS: cerebral protection, anti-analgesic, amnesia
CVS: cardiac stable
RS: minimal respiratory depression
Endocrine: adrenal suppression
CNS: amnesia, anesthesia, no analgesia, myoclonus
Pain on injection
CV: direct myocardial depressant, increases
HR/SVR/BP
RS: bronchodilator, increases secretions, maintains
respiratory drive
CNS: increases ICP/IOP, analgesic, hallucinations
insert, and has an important role in the difcult
airway algorithm, both mask and LMA are
contraindicated in patients with full stomachs as
they do not protect against risk of pulmonary
aspiration of gastric contents. Also, these may
not be the ideal methods for patients with low
lung compliance who require controlled ventila-
tion as gastric insufation with air may occur with
use of higher airway pressures for ventilation.
Endotracheal tubes (ETT) used today are made
of polyvinylchloride and are disposable. They are
calibrated according to internal diameter (ID) in
mm, are beveled, and usually have an aperture
opposite to the bevel and just above the distal tip
(Murphys eye), which allows for alternate path of
airow in case of distal tube occlusion. The appro-
priate size recommended is 22.5 mm ID for a
premature baby, 3.03.5 mm ID for term to
3-month-old, 3.54.0 mm ID for 39-month-old,
and 4.0 for a 918-month-old and for >2-year-old
children. The formula for the ID of uncuffed
ATT is given by ID (mm) (age (year) +16)/4
6 Anesthesia
and that for microcuffed ETT is determined by age
(year)/4 + 3.5 (mm) (Duracher et al. 2008). Com-
mon methods of ETT placement include deliber-
ate main stem intubation with subsequent
withdrawal of the ETT 2 cm above the carina
(main stem method), alignment of the double
black line marker near the ETT tip at the vocal
cords (marker method), or placement of the
ETT at a depth determined by the formula: ETT
depth (cm) 3 times ETT size (mm ID) or ETT
depth (cm) age (year)/2 + 12 (formula
method). The formula method only placed the
ETT at the appropriate depth 42 % of the time,
according to a study that compared these methods
(Mariano et al. 2005). The preferred technique is
via auscultation. Conrmation of appropriate ETT
placement is done by auscultation, conrming
chest rise, capnography, and condensation in the
ETT. Air leak at an inspiratory pressure of
2025 cm H2O is thought to prevent excessive
mucosal pressure. For microcuffed ETT, a cuffed
tracheal tube with a smaller diameter is selected,
which does not wedge within the susceptible cri-
coid, and the airway is sealed within the trachea
using a cuff (Weiss et al. 2006). In contrast to
cricoidal sealing, tracheal sealing with an HVLP
cuff allows precise estimation and adjustment of
the pressure exerted by the cuff on the tracheal
mucosa. Down syndrome patients should be
intubated with manual in-line cervical stabiliza-
tion technique until atlantoaxial instability is
ruled out.
Maintenance of Anesthesia
Maintenance of anesthesia can be done in various
ways including inhalation and intravenous anes-
thetic agents. Inhalational agents used typically
may be sevourane, isourane, or desurane
(Table 2), and common parenteral intravenous
(IV) agents are benzodiazepines, propofol, muscle
relaxants, and opioids. Balanced anesthesia is a
triad of narcosis (analgesia), amnesia (anes-
thetics), and relaxation (volatile anesthetics or
muscle relaxants). Analgesia can be achieved
with opioids, nonsteroidal anti-inammatory
agents like IV ketorolac, and IV acetaminophen
123
(doses and salient considerations are discussed
under acute postoperative pain management at
the end of the chapter). The amount of any med-
ication is typically weight based and tailored also
to patient conditions and surgery. Neuromuscular-
blocking agents (NMBA) are used to facilitate
tracheal intubation, prevent movement, and pro-
vide muscle accidity for certain procedures. In
general, nerve signals cause release of neurotrans-
mitter acetylcholine at synaptic clefts, which
binds to postjunctional acetylcholine receptors,
which then activates ion channels ultimately caus-
ing muscle contraction. NMBA act at the neuro-
muscular junction by either competing with
acetylcholine for the receptor (non-depolarizing)
or by activating both receptive sites and
maintaining a depolarized muscle membrane so
that acetylcholine cannot act on it (depolariza-
tion). The different muscle relaxants commonly
used, doses, site(s) of metabolism, and duration of
action are presented in Table 4. Importantly, neu-
romuscular transmission is immature in neonates
and infants below the age of 2 months
(Goudsouzian and Standaert 1986). Moreover,
the organs for metabolizing and eliminating
these agents (kidneys/liver) may also not be
mature. Reversal of muscle relaxation is generally
required after use of non-depolarizing NMBA,
which is done by use of acetylcholinesterases.
Typically, neostigmine and edrophonium are
used these drugs inhibit the enzyme that metab-
olizes acetylcholine (acetylcholinesterase) and
thereby increase the available concentration of ace-
tylcholine in order to overcome the competitive
inhibition at the receptor. These reversal agents
are used with anticholinergic agents to counter
unwarranted muscarinic effects of acetylcholine.
Emergence from Anesthesia
Emergence from anesthesia is akin to ight land-
ing and ideally should be smooth and safe. During
emergence, the anesthetic is discontinued, neuro-
muscular block is reversed, and when the patient
meets certain criteria (responding to commands,
acceptable minute ventilation, normoxic, and
good headlift/hand grasp), the trachea is
124
Table 4 Neuromuscular-blocking agents
Dose
Drug Route (mg/kg) Onset
Succinylcholinea IV 2 2030
s synaptic cleft
IM 35 3060
s pseudocholinesterase hydrolysis
Vecuroniumb IV 1 3 min
Rocuroniumb IV 0.61.2 3 min
Cisatracuriumb IV 0.150.2 3 min
Pancuroniumb IV 0.15 1.53
min
List of depolarizinga and non-depolarizingb muscle relaxants used during anesthesia, recommended dose to facilitate
tracheal intubation, time to onset of action, and duration of effect are given. Of the non-depolarizing muscle relaxants,
pancuronium is long acting and has vagolytic effects
extubated. In patients with easy airways and not at
risk for aspiration, the trachea may be extubated
under deep anesthesia. Postoperative care
includes continued monitoring of vital signs,
maintaining an open airway, pain management,
and dealing with possible postoperative compli-
cations including hypoxia, hypercarbia, hypo- or
hypertension, agitation, and weakness. Emer-
gence delirium (ED) also referred to as emergence
agitation (EA) is a well-documented phenomenon
with an incidence in all postoperative patients of
5.3 % with a more frequent incidence in children
(1213 %) (Mason 2004). The incidence of emer-
gence delirium after halothane, isourane,
sevourane, and desurane ranges from 2 % to
55 %, with a higher incidence noted for the newer
inhalation agents, desurane and sevourane.
Emergence delirium is dened as a dissociated
state of consciousness in which the child is incon-
solable, irritable, uncooperative, typically
thrashing, crying, moaning, or incoherent (Wells
and Rasch 1999). Characteristically these children
do not recognize or identify familiar and known
objects or people. Generally, these episodes are
self-limiting (515 min) but are unnerving to par-
ents and can result in physical harm to the child.
Ten factors were associated with ED including:
(1) younger age (4.8 vs. 5.9 years), (2) no previous
surgery, (3) poor adaptability and anxiety
A.J. Costandi and V. Chidambaran
Duration
(min) Site of metabolism
35 Effect terminated by drug diffusing away from
35 Elimination is through plasma
3545 Hepatic metabolism
Renal metabolism
3575 Biliary metabolism
Renal elimination
35 Hoffman elimination
Ester hydrolysis
90120 Hepatic metabolism
Renal elimination
(Kain et al. 1996), (4) ophthalmology and (5) oto-
rhinolaryngology procedures, (6) sevourane,
(7) isourane, (8) sevourane/isourane, (9) anal-
gesics, and (10) short time to awakening (Voepel-
Lewis et al. 2003).
Weakness from a residual neuromuscular
block is another postoperative complication,
with an incidence between 4 % and 50 %,
depending on the diagnostic criteria, the type of
NMBA, the administration of a reversal agent,
and the use of neuromuscular monitoring (Plaud
et al. 2010).
Postoperative Pain Management
Perioperative pain management begins preopera-
tively with anxiolysis and preemptive analgesia
before surgical incision intraoperatively. Pain
management modalities are discussed below
under two main headings: Systemic Analgesia
and Regional Analgesia.
Systemic Analgesia
After minor procedures, when no regional anes-
thesia is used, the use of these systemic analgesic
drugs is indicated to provide analgesia
6 Anesthesia
postoperatively or to supplement local analgesia.
Non-opioids, such as acetaminophen and
NSAIDs, play an increasing role as components
of multimodal analgesia in children.
Non-opioid Analgesics
(a) Acetaminophen is an over-the-counter analge-
sic and antipyretic drug indicated for the man-
agement of mild pain, the management of
moderate to severe pain with adjunctive opi-
oid analgesics, and the reduction of fever.
Exact mechanism of action is still unclear
but is proposed to be through the inhibition
of the cyclooxygenases. It is currently FDA
approved for patients greater than 2 years old
and contraindicated in patients with severe
hepatic impairment or with known hypersen-
sitivity to acetaminophen. Most common side
effects include nausea, vomiting, constipa-
tion, pruritus, and agitation in pediatric
patients and, in rare cases, Stevens-Johnson
syndrome. Toxicity is associated with acute
liver failure. The maximum recommended
intravenous dosage is 15 mg/kg Q 6 h.
(b) Nonsteroidal anti-inammatory drugs
(NSAIDs) are a class of OTC drugs that has
analgesic, antipyretic, and anti-inammatory
effects. It is often used for their opioid-sparing
effects; however, they display a ceiling
effect on analgesia irrespective of the dose
administered. Their mechanism of action
involves inhibition of cyclooxygenases
(mainly COX-1) and inhibition of prostaglan-
din synthesis, which also produces side
effects of decreased renal function (decreases
renal blood ow), gastric mucosal irritation
(affects protective stomach mucosal lining),
decreased platelet activity, and delayed bone
healing (Chidambaran et al. 2012).
Ketorolac is a nonsteroidal agent with potent anal-
gesic and moderate anti-inammatory activity. It
reversibly inhibits cyclooxygenase and decreases
the hypersensitization of tissue nociceptors that
occurs with surgery. It can be administered orally,
intramuscularly, and intravenously. The intrave-
nous route is preferred during the immediate post-
operative period. Intramuscular injections are not
125
recommended in children, unless the intravenous
route is unavailable. The recommended intrave-
nous dosage of ketorolac in children is 0.5 mg/kg
Q 6 h. The Food and Drug Administration (FDA)
has approved ketorolac for use in children older
than 2 years of age, although data describing
its safe use in neonates and infants exists
(Moffett et al. 2006). Neonates less than 21 days
of life have markedly delayed drug clearance
(Aldrink et al. 2011). Older children may require
somewhat lower dosages, while infants and young
children may require slightly higher dosages to
achieve the same level of pain relief. Ketorolac is
not recommended for use in infants <1 year of
age (Forrest et al. 1997). It has reversible
antiplatelet effects due to the inhibition of
thromboxane synthesis. Uncommon serious side
effects include interstitial nephritis and acute renal
failure. Unlike opioid analgesics, ketorolac does
not depress ventilation and is not associated with
nausea and vomiting, urinary retention, or
sedation.
Ibuprofen is another NSAID that is frequently
administered orally or rectally. It can cause gas-
trointestinal mucositis as well as increased bleed-
ing. Recommended intravenous dose is 510
mg/kg/dose Q 6 h. It is not recommended for
patients less than 6 months of age.
Opioid Analgesics
Opioids are the mainstay in the treatment of post-
operative pain. They provide very effective anal-
gesia with a relatively wide margin of safety. Side
effects include pruritus, nausea, vomiting, consti-
pation, urine retention, respiratory depression,
and hypotension.
(a) Morphine is the gold standard against
which all other opioids are compared. It is
commonly administered via intravenous,
oral, intramuscular, and epidural routes. Oral
morphine undergoes extensive rst-pass
metabolism in the liver, and therefore, a larger
dose is required than when given parenterally.
In the liver, morphine is metabolized to mor-
phine-3-glucuronide (M3G), which is a
neuroexcitatory metabolite and morphine-6-
glucuronide (M6G), which is a very active
126
metabolite. Morphine is excreted by the kid-
ney, and thus, these metabolites accumulate in
patients with renal failure. Infants and neo-
nates preferentially metabolize morphine to
M3G. Histamine release, redness, and local
urticaria at the IV injection site are common
side effects. Recommended IV dose is
0.050.1 mg/kg Q 24 h.
(b) Hydromorphone is a derivative of morphine
that is about ve to eight times more potent
than morphine in the IV form with decreased
occurrence of pruritus, nausea, and vomiting.
It is commonly administered via intravenous,
oral, intramuscular, and epidural routes. Oral
dose is one-fourth to one-fth the IV dose
because of hepatic metabolism.
Recommended IV dose is 0.020.04 mg/kg
Q 24 h.
(c) Fentanyl is a synthetic lipophilic opioid that is
100 times more potent than morphine. It is
commonly administered via intravenous,
oral, intramuscular, epidural, and transdermal
routes. It has diminished hepatic metabolism
in premature and term neonates, and thus,
half-life is prolonged after repeated doses or
infusion. The effect of single-dose administra-
tion is short due to rapid redistribution. It is
the most commonly used opioid in the PCA
and in the epidural space. Side effects include
bradycardia and chest wall rigidity with high
doses. It rarely causes cardiovascular instabil-
ity but is known to depress the baroreceptor
response. Recommended IV dose is 12
mcg/kg Q 1 h.
(d) Codeine is a natural opioid that can be admin-
istered orally. It is metabolized in the liver by
the enzyme CYP2D6 into morphine. Ten per-
cent of the US population has genetic varia-
tion in CYP2D6 where ultra metabolizers
produce large concentrations of morphine and
slow metabolizers do not produce enough
morphine. Severe nausea and vomiting is a
very common side effect at comparable anal-
gesic doses of other opioids. It is not routinely
used alone in children and often given in
combination with acetaminophen.
Recommended PO dose is 0.51 mg/kg/dose
Q 4 h.
A.J. Costandi and V. Chidambaran
(e) Oxycodone is a semisynthetic derivative of
codeine that is better tolerated by the gastro-
intestinal system. PO oxycodone is more
potent than PO morphine. Mental and physi-
cal dependence can occur but are unlikely
when used for short-term pain relief.
Recommended PO dose is 0.10.2 mg/kg/
dose Q 4 h.
(f) Hydrocodone is a synthetic derivative of
codeine that is frequently prescribed. It is
well tolerated by children. Mental and physi-
cal dependence can occur but are unlikely
when used for short-term pain relief. It is usu-
ally given in combination with acetamino-
phen. Recommended PO dose is 0.10.2
mg/kg/dose Q 4 h.
(g) Methadone is a potent synthetic opioid that
has a long variable duration of action
(824 h). It is not to be used in an acute
setting, but in weaning children after long-
term use of opioids. Its effect is through stim-
ulating the opioid receptors, antagonizing the
NMDA receptors, and inhibiting the serotonin
and norepinephrine reuptake. It also prolongs
the QT interval (FDA black box warning). PO
and IV doses are comparable. Recommended
dose is 0.10.2 mg/kg/dose Q 624 h.
Patient-Controlled Analgesia (PCA)
PCA is a delivery system with which patients self-
administer predetermined doses of analgesic opi-
oid medications. Pediatric patients benet from
PCA, which eliminates the need for painful intra-
muscular injections of opioids and improves the
childs sense of control. Children must be care-
fully screened for their cognitive and physical
ability to manage their pain using PCA. Family-
controlled analgesia and nurse-controlled analge-
sia may be considered in select cases as alterna-
tives to PCA in children with cognitive or physical
disabilities. PCA dosage regimens must be indi-
vidualized on the basis of age and comorbidities.
Potential adverse effects of PCA therapy include:
respiratory depression, nausea, vomiting, and pru-
ritus. Serious adverse effects can be prevented by
sedation monitoring, pulse oximetry, and individ-
ualized dosage requirements. The safety and ef-
cacy of PCA in pediatric patients have been
6 Anesthesia
established, and their role has increased beyond
postoperative pain management (Lehr and BeVier
2003). Recent recommendations by the Anesthe-
sia Patient Safety Foundation (APSF) for contin-
uous capnography in adults receiving PCA for
earlier detection of opioid hypoventilation
become relevant to pediatrics also (APSF News-
letter, Fall, 2011) as children experience a higher
risk of respiratory depression from opioids.
Local Anesthetics and Adjuncts
Local Anesthetics (LA)
Local anesthetics are used for regional or eld
blocks. Local anesthesia with or without sedation
has been increasing in use in children following
the rise in popularity of wide-awake surgery in
adults. Awake and responsive patients are able to
participate in the surgery, allowing for more pre-
cise surgical treatment in cases of joint releases,
tenolysis, spasticity, and/or tendon transfer.
Patients must be carefully screened for the emo-
tional stability needed to be awake during their
surgery.
LAs block the generation and conduction of
nerve impulses at the level of the cell membrane
by crossing the phospholipid membrane mostly in
their unionized form to block the open voltage-
gated sodium channels along the inner part of the
nerve membrane in the charged form (Mazoit
2012). They cause an allosteric change in the
receptor conguration, modifying drug afnity
for the receptor, which ultimately leads to the
inhibition of the inward surge of sodium and
nerve depolarization. In general, small nerve
bers are blocked rst, and myelinated bers are duration of analgesia. Lidocaine and ropivacaine
blocked before nonmyelinated bers of the same
diameter. Autonomic, pain, and temperature bers
are blocked rst followed by sensory and motor
bers. The potency of local anesthetics and their
onset and duration of action are primarily deter-
mined by lipid solubility, pKa of the drug, and
protein binding, respectively. The addition of
vasoconstrictors can prolong the duration of
action of local anesthetics, decrease their absorp-
tion, and enhance their blockade. LA are weak
bases made of an aromatic ring, intermediate
127
chain, and a hydrophilic residue. Depending on
the link between the aromatic ring and the inter-
mediate chain, they are categorized into esters and
amides.
Ester LA such as chloroprocaine, procaine, and
tetracaine are metabolized by plasma cholinester-
ases. Para-amino benzoic acid (PABA) resulting
from ester metabolism can induce severe allergic
reactions.
Amide LA such as lidocaine, bupivacaine,
ropivacaine, and prilocaine are metabolized in
the liver through the cytochrome P450
(CYP450) pathway to be ultimately excreted by
the kidney.
The recommended doses of most commonly
used LA are tabulated in Table 5.
Local Anesthesia Adjuncts
Local anesthesia adjuncts (Neal et al. 2009) are
medications usually admixed with local anes-
thetics to moderately prolong the analgesic effects
of single-shot blocks when a continuous catheter
placement is not warranted.
Clonidine: Clonidine, an 2 adrenoceptor ago-
nist, is used as an additive to short- and
intermediate-acting local anesthetics in single-
shot peripheral nerve blocks to prolong duration
of analgesia and motor block by 100120 min.
Opioids: When an opioid was combined with
local anesthetics in peripheral nerve block, no
increased improvement in analgesia was reported
in comparison with systemic controls except with
buprenorphine.
Epinephrine: Its use nowadays is limited to test
dose purposes. Its 1 adrenoceptor agonist effect
leads to decrease in blood ow and thus increased
can cause vasoconstriction and are synergistic
with epinephrine.
Local Anesthetics Concerns in the Pediatric Age
Group
1. Dose: With the use of any local anesthetic in
the pediatric age group, the total milligram
dosage on a per-kilogram basis must be calcu-
lated to avoid toxic blood levels.
2. Ester LA: Neonates and infants less than
6 months have less than half adult levels of
128 A.J. Costandi and V. Chidambaran

Table 5 Local anesthetics

Onset Duration Maximum recommended dose Continuous infusion
Local anesthetic (min) (min) (mg/kg) (mg/kg/h)
Esters
Procaine 510 1530 7
Chloroprocaine 510 3060 15 10
Amides
Lidocaine 1015 90120 5 1.5
Lidocaine with 1015 120240 7
epinephrine
Bupivacaine 1520 240480 3 0.4
Levobupivacaine 1520 240480 3 0.4
Ropivacaine 1520 240480 2.5 0.4
Mepivacaine 1520 120180 5
Listed is a summary of commonly used local anesthetics, their onset and duration of action, recommended doses to
prevent toxicity, and dosing for continuous infusion in continuous peripheral nerve catheters and epidurals

plasma cholinesterase, and thus, clearance may
be reduced although clinically insignicant.
3. Amide LA: In the premature infant and neo-
nate, the hepatic, microsomal enzyme system
is not fully developed, which leads to
decreased intrinsic hepatic clearance. Amide
LA binds to serum proteins in what is thought
to be a protective mechanism against local
anesthesia systemic toxicity (LAST). Alpha-1
acid glycoprotein (high afnity to LA) and
serum albumin (low afnity to LA) concentra-
tions are very low at birth and increase slowly
during the rst year of life to reach adult levels
by 912 months of age. Thus, higher concen-
trations of free, unbound local anesthetics are
present within the blood increasing the likeli-
hood of LAST. The large volume of distribu-
tion in infants offsets low clearance after a
single injection in infants. This is not the case
for repeat injections or infusions where steady-
state conditions are determined by clearance.
4. Cardiac output and local blood ow of infants
is relatively greater than in adults. Therefore,
systemic absorption of local anesthetics is rel-
atively faster in children, as are peak plasma
concentrations.
5. The perineurovascular sheaths that are located
around nerve roots and bundles are more
loosely attached to underlying structures in
children than in adults. It is for these reasons
that injected local anesthetics appear to spread
more in children and cover a greater area of
innervation. In addition, the endoneurium is
relatively loose in young children and allows
rapid exposure of the local anesthetic to the
nerve (Litman 2013).
6. Prilocaine should not be used in neonates as
they have reduced levels of methemoglobin
reductase predisposing them to
methemoglobinemia.
7. Neonates do not metabolize mepivacaine, and
most is excreted unchanged in the urine. In
general, the intermediate-acting agents, like
lidocaine, demonstrate faster onset and lower
failure rates than ropivacaine and bupivacaine
but at the expense of a shorter analgesic
duration.
Local Anesthetics Systemic Toxicity
(LAST)
Despite high cardiac output and increased local
blood ow in infants leading to rapid systemic
absorption of local anesthetics, LAST in children
is rare (Neal et al. 2012). It still occurs, however,
from inadvertently administered overdose or
intravascular injection of LA. Local anesthetics
depress the central nervous system in a dose-
dependent manner. Low serum concentrations
6 Anesthesia
are used clinically to suppress cardiac dysrhyth-
mias and status seizures, while higher concentra-
tions are used to induce seizure activity especially
in the presence of hypercarbia as it displaces local
anesthetics from their plasma protein-binding
sites. Central nervous system (CNS) toxicity
occurs when increasing amounts of local anes-
thetics cross the BBB resulting in CNS excitation
(agitation, confusion, muscle twitching, and sei-
zure), CNS depression (drowsiness, obtundation,
coma, or apnea), or nonspecic symptoms (metal-
lic taste, diplopia, circumoral numbness, dizzi-
ness, and tinnitus). Under general anesthesia,
these signs may not be evident and diagnosis
may need to be made based on indirect signs
such as muscular rigidity; hypoxemia without
other causes or signs of cardiac toxicity, such as
progressive hypotension, conduction block, bra-
dycardia, or asystole; ventricular arrhythmia (Tor-
sades de pointes, ventricular tachycardia, or
ventricular brillation); or unexplained cardiovas-
cular collapse with decreased myocardial contrac-
tility nonresponsive to resuscitation efforts.
Treatment of LAST
Once LAST is suspected, one should call for help
while initiating BLS and ACLS protocols. All
efforts are focused on maintaining the vitals
while managing the airway, ensuring adequate
oxygenation and ventilation. Benzodiazepines
are preferred over propofol to control seizures,
especially when patients are unstable. Lipid emul-
sion (20 %) therapy should be initiated as soon as
possible with a bolus dose of 1.5 mL/kg (lean
body mass) intravenously over 1 min followed
by continuous infusion of 0.25 mL/kg/min for at
least 10 min after attaining circulatory stability. In
case of persistent cardiovascular instability, repeat
the bolus once or twice and double the infusion
rate to 0.5 mL/kg/min, with a recommended upper
limit of 10 mL/kg over the rst 30 min. Lipid
emulsion is thought to act through partitioning
the local anesthetic away from the receptor. Indi-
vidual epinephrine doses should be reduced to <1
mcg/kg to avoid severe vasoconstriction, allowing
lipid emulsion to reach the affected site(s). Refrac-
tory cardiovascular collapse would necessitate a
cardiopulmonary bypass. Cardiac arrhythmias
129
should be treated medically while avoiding
administering vasopressin, calcium channel
blockers, beta-blockers, or local anesthetics.
Regional Anesthesia
There has been a recent increase in the awareness
of the importance of pediatric regional anesthesia
due to its several signicant benets (Litman
2013). It is a safe, reliable, and effective method
of providing profound analgesia to children post-
operatively provided a proper technique is
followed. Untreated pain has several deleterious
effects; effective pain relief may play a signicant
role in surgical outcome as untreated surgical
stress may produce a spectrum of autonomic, hor-
monal, metabolic, immunologic/inammatory, and
neurobehavioral consequences (Bosenberg 2012).
While the duration of analgesia provided by
single-shot blocks is limited, the development and
application of continuous peripheral nerve cathe-
ters allows for prolonged postoperative pain con-
trol with decreased opioid-related side effects and
sleep disturbances. Supplementing general anes-
thesia with regional anesthesia allows a decrease
in general anesthesia requirements leading to
faster and smoother emergence and decreased
incidence of opioid-related side effects during
the rst 24 h postsurgery, including postoperative
nausea and vomiting (PONV), and pain scores.
An added benet of regional anesthesia to an
injured extremity is the sympathetic blockade
and vasodilation that increase distal blood ow.
Serious complications have been reported anec-
dotally, while the reported morbidity related to
regional anesthesia in children based on large
retrospective and prospective studies is extremely
low. The safety of peripheral blocks has been
established, and it has been recommended by the
French-Language Society of Pediatric Anesthesi-
ologists (ADARPEF) (Ecoffey et al. 2010;
Ecoffey 2012) that peripheral blocks can be used
in place of central blocks when appropriate. This
section will review the applications and tech-
niques of the most commonly used peripheral
nerve blocks for the upper extremity in children
(Ecoffey et al. 2010; Ecoffey 2012).
130
Continuous Peripheral Nerve Blocks
Continuous peripheral nerve blocks (CPNB) are
becoming more popular nowadays as they allow
for effective, safe, and prolonged postoperative
pain management. Main indications are intense
postoperative pain surgical procedures, with or
without postoperative rehabilitation, and complex
regional pain syndrome. Contraindications to
these procedures are rather similar to those in
adults, along with parental and/or child refusal.
CPNBs are usually performed under general anes-
thesia or sedation in children and require appro-
priate equipment in order to decrease the risk of
nerve injury. The use of US guidance has
improved precision in regard to the proper needle
position of the block placement, resulting in
shorter block performance time, higher success
rates, faster onset, longer block duration, reduc-
tion in the volume of local anesthetic used, and
decreased incidence of complications. CPNB may
facilitate early ambulation by an improved pain
management or even postoperative analgesia at
home with disposable pumps (Dadure and
Capdevila 2007). The most common complica-
tion is unintentional dislodgment, while serious
infections associated with CPNB are extremely
rare. CPNB may theoretically mask a compart-
ment syndrome after trauma surgical procedures.
The peripheral nerve blocks performed for
postoperative pain relief after upper extremity
surgeries mainly involve blocking one or more
parts of the brachial plexus.
The Brachial Plexus
The brachial plexus innervates the entire upper
extremity. The roots of the brachial plexus are
the anterior divisions of C5C8 and T1 spinal
nerves. They exit out of the intervertebral foram-
ina over the transverse processes to run downward
in the neck towards the rst rib. They then join
and divide several times to form trunks, divisions,
cords, and nally the terminal branches. The bra-
chial plexus is encased by a sheath formed of
prevertebral and scalene fascia, extending from
the intervertebral foramina to the upper arm. Its
blockade in children at different levels can be used
A.J. Costandi and V. Chidambaran
for a broad spectrum of clinical indications
(Marhofer et al. 2012). The usual approaches
used to block the plexus and its terminal nerves
are presented below (Fig. 1).
Interscalene Approach
Relevant Anatomy: The interscalene groove is
bordered by the anterior scalene muscle laterally,
the middle scalene muscle medially, the rst rib
inferiorly, and the lateral border of the sternoclei-
domastoid muscle superiorly when the head is
turned in the opposite direction.
Overview: This approach anesthetizes the C5C8
roots of the brachial plexus as they pass through this
groove. As the roots of the brachial plexus are orga-
nized in a superior to inferior direction, the lower
dermatomes of the brachial plexus (C8T1) are less
effectively blocked than with a supraclavicular
approach. Therefore, there may be less effective
analgesia over the distribution of the ulnar nerve.
Indications: Typically performed with surger-
ies to the entire upper arm, especially the shoulder,
to provide excellent anesthesia or analgesia.
Technique (Fig. 2): Using US, with the patient
in the supine position and the head turned away
from the block site, the anatomy of the C5C8
nerve roots within the interscalene groove
between the anterior (ASM) and middle scalene
muscles (MSM) is identied. This can be done by
scanning the brachial plexus in the
supraclavicular position and then following it
cephalad with the US or by scanning horizontally
at the level of the cricoid moving the probe later-
ally to image the carotid artery and internal jugular
vein in the short-axis view and then sliding the
transducer laterally towards the lateral border of
the sternocleidomastoid. Once the two scalene
muscles are identied, the probe is angled slightly
caudally, and the BP roots are visualized as
hypoechoic circles surrounded with hyperechoic
outer rings. The needle is advanced using the
in-plane (IP) technique, either from the posterior
aspect (posterior approach) or the anterior aspect
(anterior approach) of the transducer. An out of
plane (OOP) approach is usually used if catheter
insertion is desired. With this approach, it is
important to manipulate the needle to avoid the
external jugular vein as it usually runs supercial
6 Anesthesia 131

Fig. 1 Schematic diagram

representing the anatomy of
the brachial plexus and its
main terminal nerves. The
black lines indicate where
the roots, trunks, divisions,
and cords of the brachial
plexus are formed and the
levels at which different
approaches for brachial
plexus block are performed.
The black arrows point to
the levels where each
peripheral nerve block is
performed

at the puncture site. For shoulder surgery, the
needle should be advanced under direct guidance
between the C5 and C6 nerve roots. 0.40.5 cm3/
kg of local anesthetic is injected in increments
forming a donut around the brachial plexus after
multiple negative aspirations of blood.
Possible Complications
(a) Accidental venipuncture.
(b) Phrenic nerve block leading to diaphragmatic
paresis.
(c) Cervical sympathetic ganglia blockade lead-
ing to Horner syndrome (ptosis, miosis, and
anhidrosis) associated with unilateral ushing
of the conjunctiva and nasal congestion.
(d) Recurrent laryngeal nerve block leading to
hoarseness.
(e) Short-term paresthesia may rarely occur, but
most cases resolve within weeks to months.
(f) Inadvertent vertebral artery, intrathecal, and
epidural injections are extremely rare.
Supraclavicular Approach
Anatomy: The roots of the brachial plexus com-
bine to form the superior, middle, and inferior
trunks in the supraclavicular region. The plexus
132
Fig. 2 US image of the
interscalene approach of the
brachial plexus block. The
roots of the brachial plexus
are seen in the interscalene
groove between the anterior
scalene muscle (ASM) and
the middle scalene muscle
(MSM) and inferior to the
lateral one-third of the
sternocleidomastoid (SCM)
is usually located lateral to the subclavian artery
and right above the rst rib.
Overview: A supraclavicular approach
involves injecting local anesthetic in close prox-
imity to the trunks where the trunks are most
compact resulting in a fast, reliable block. It is
often called the spinal of the arm as it provides
excellent anesthesia and analgesia to the entire
upper extremity.
Indication: Typically performed with all differ-
ent surgeries (orthopedic, plastic, and vascular)
below the shoulder level. It can be used for shoul-
der surgery but will require supplementation of
the supraclavicular nerve (C3C4) to ensure anes-
thesia of the cape of the shoulder.
Technique (Fig. 3): Patient is positioned supine
with the head rotated away from the block site.
The US probe is then placed parallel to the clav-
icle resting in the supraclavicular fossa. The bra-
chial plexus is then visualized as 36 hypoechoic
circles in a bundle superior and lateral to the
subclavian artery. An IP approach is used while
cautiously advancing the needle from lateral to
medial after identifying the brachial plexus, sub-
clavian artery, rst rib, and the pleura. The needle
tip should then be positioned in the corner
pocket just below the brachial plexus and above
A.J. Costandi and V. Chidambaran
the rst rib to ensure blocking of the ulnar nerve.
0.40.5 cm3/kg local anesthetic is injected in
increments forming a donut around the brachial
plexus after multiple negative aspirations of
blood.
Complications
(a) Pneumothorax, whose incidence has
decreased dramatically with the use of US
(b) Vascular injury to the subclavian artery and
formation of hematoma
(c) Intravascular injection of local anesthetics and
associated local anesthetic systemic toxicity
(LAST)
(d) The theoretical risk of nerve injury if the
needle tip contacts the nerve
Infraclavicular Approach
Anatomy: The trunks of the brachial plexus branch
to form divisions that subsequently rejoin to form
medial, lateral, and posterior cords in the
infraclavicular region. They enter the
infraclavicular region at the calvipectoral triangle
lateral to the axillary artery and vein.
Overview: An infraclavicular approach
involves injecting local anesthetic in close prox-
imity to the cords as they lie lateral to the axillary
6 Anesthesia
Fig. 3 US image of the
supraclavicular approach of
the brachial plexus block.
The trunks of the brachial
plexus are seen supercial
and lateral to the subclavian
artery. Note the intimate
location of the rst rib
artery. With this approach, visualization of the
brachial plexus could be harder because of the
muscle mass, but it has lesser incidence of com-
plications, such as pneumothorax. It is usually
used when a catheter is desired for long-term
pain relief because it is an area with little move-
ment and, therefore, presents with a decreased
chance of being displaced.
Indications: Typically performed to provide
anesthesia and analgesia to all the upper extrem-
ity. However, it works best for procedures below
the elbow. It provides good analgesia for tourni-
quet pain, but will not anesthetize the axilla or the
proximal medial arm as it misses the intercostal
nerve.
Technique: Patient is positioned supine with
the head rotated away from the block site. The
US transducer is then placed in the infraclavicular
fossa (12 cm medial to the coracoid process of
the scapula perpendicular to the clavicle along its
lateral segment) (Fig. 1). After imaging the axil-
lary artery, the brachial plexus cords appear as
three hyperechoic circles located around the axil-
lary artery (Fig. 4). The needle is then advanced
using the IP approach till the tip of the needle is
positioned between the axillary artery and the
posterior cord of the brachial plexus to ensure
133
blocking the radial nerve. 0.40.5 cm3/kg of
local anesthetic is injected in increments forming
a donut around the axillary artery after multiple
negative aspirations of blood.
Complications
(a) Vascular injury to the subclavian artery and
formation of hematoma
(b) Intravascular injection of local anesthetics
and associated LAST
(c) The theoretical risk of nerve injury if the nee-
dle tip contacts the nerve
Axillary Approach
Anatomy: The cords of the brachial plexus com-
bine to form the axillary, musculocutaneous,
median, radial, and ulnar nerves. The latter three
along with the axillary artery run in the axillary
sheath.
Overview: It was the most commonly used
method for brachial plexus anesthesia in children
in the past due to its easily identiable landmarks.
Because of the extreme supercial position of the
target structures, the axillary approach is rarely
performed in toddlers. The axillary approach
involves injecting local anesthetic around the ter-
minal branches of the brachial plexus. It is a better
134
Fig. 4 US image of the
infraclavicular approach of
the brachial plexus block.
The three cords of the
brachial plexus are seen
around the subclavian artery
at 3, 6, and 9 oclock
positions
choice for providing anesthesia of the ulnar nerve,
a branch of the inferior trunk of the brachial
plexus, which may be missed with the
interscalene approach. However, with the axillary
approach, the musculocutaneous nerve is usually
missed as it arises proximally from the lateral cord
and courses away to enter the coracobrachialis
muscle.
Technique: The technique can be performed
blindly or using the US (more common
nowadays).
With the blind technique, the patients arm is
abducted 90 from the body, and the elbow is
exed so that the hand is over the head or behind
it. The block is performed using a transarterial
two-injection technique. In this technique, the
artery is xed against the humerus, and a 22- or
25-gauge needle is inserted 12 cm away from the
axilla, at a 6090 angle to the skin, directed
towards the arterial pulsation. Constant aspiration
is maintained on the plunger of the syringe as it is
advanced into the artery. The needle is advanced
through the artery until blood is no longer aspi-
rated. One-half of the local anesthetic solution is
injected posterior to the artery. The needle is
then withdrawn back through the artery until
there is no longer blood return and the other half
A.J. Costandi and V. Chidambaran
of the local anesthetic is administered in front of
the artery. The advantage of this technique is a
successful blockade of the posterior cord of the
brachial plexus, which gives rise to the radial
nerve.
Using the US, the patients arm is abducted 90
from the body, and the elbow is exed so that the
hand is over the head or behind it. The US probe is
then placed in the axilla perpendicular to the
course of the axillary artery and at the level of
the crease formed by the pectoralis major and
biceps muscles (Fig. 1). The axillary artery and
vein are visualized in the short-axis view. The
median, ulnar, and radial nerves most likely will
surround the artery in a triangular pattern.
Using an in-plane approach, the needle is
advanced and 0.40.5 cm3/kg of local anesthetic
is injected incrementally in a circumferential pat-
tern around the axillary artery (the donut sign)
after multiple negative aspirations of blood
(Litman 2013).
Complications
Vascular injury to the axillary artery and for-
mation of hematoma
Intravascular injection of local anesthetics and
associated LAST
6 Anesthesia
The theoretical risk of nerve injury if the needle
tip contacts the nerve
Musculocutaneous Block
Anatomy: Arises proximally from the lateral cord
of the brachial plexus and courses away to enter
the coracobrachialis muscle and is, thus, com-
monly missed with the axillary block.
Nerve Supply: Supplies the coracobrachialis,
biceps brachii, and the brachialis muscles. It also
innervates the skin on the lateral surface of the
forearm through its branch, the lateral cutaneous
nerve of forearm.
Nerve Block: With the US in the axillary posi-
tion, the musculocutaneous nerve can be
visualized as a hyperechoic area between the
short head of the biceps muscle and
the coracobrachialis muscle. Blindly, through the
same needle insertion site as the axillary
approach, a musculocutaneous block may
be performed by injecting the local anesthetic
solution into the body of the coracobrachialis
muscle.
Suprascapular Nerve (C5C6) Block
Provides sensory innervation to the
posterosuperior aspect of the shoulder joint.
Used frequently with general anesthesia when
the interscalene block is not used to decrease
postoperative pain and hospital stay.
Intercostobrachial Nerve (T2) Block
T2 is not part of the brachial plexus and, thus,
requires a separate block. It supplies the medial
upper arm and axilla and, thus, helps to alleviate
tourniquet pain.
Median Nerve Block
Anatomy (Fig. 1): The median nerve is formed by
the lateral and medial cords. In the axilla, it
descends down the arm lateral to the brachial
artery then crosses over medially halfway down
the arm until it enters the cubital fossa. It enters the
forearm between the pronator teres and biceps
tendon and then continues to run between the
exor digitorum profunda (FDP) and exor
digitorum supercialis (FDS) to enter the hand
via the carpal tunnel.
135
Nerve Supply: Provides sensory innervation to
the lateral part of the palm and the lateral three and
a half ngers on the palmar surface of the hand. It
also provides motor innervation to all the muscles
responsible for pronation of the forearm, exion
of the wrist, and exion of the digits of the hand in
addition to the lateral 2 lumbricals and the thenar
muscles.
Nerve Block: Using the US, it is usually
blocked at the proximal and middle third of the
forearm (Fig. 1), using an OOP technique where it
lies between the FDS and FDP muscles. At the
level of the wrist, the median nerve is blocked by
inserting the needle between the tendons of the
palmaris longus and exor carpi radialis until it
pierces the deep fascia. Local anesthetic solution
is then injected.
Ulnar Nerve Block
Anatomy: The ulnar nerve is formed by the medial
cord. As a continuation of the medial cord, it
descends down the medial side of the arm and
passes posterior to the medial epicondyle to
enter the forearm. In the forearm, it pierces the
two heads of the exor carpi ulnaris and then
passes down the forearm alongside the ulna. At
the wrist, the ulnar nerve travels supercially to
the exor retinaculum, later giving rise to the
supercial and deep branches.
Nerve Supply: Provides motor innervation to
the muscles of the hand (apart from the thenar
muscles and two lateral lumbricals supplied by
median nerve), exor carpi ulnaris, and medial
half of FDP. It also supplies sensory innervation
to the anterior and posterior surfaces of the
medial one and half ngers and the associated
palm area.
Nerve Block (Fig. 1): Usually blocked at the
proximal third of the forearm where the
ulnar nerve is embedded between exor carpi
ulnaris, the FDS, and the FDP using an OOP
technique.
Radial Nerve Block (Wrist Block)
Anatomy (Fig. 1): The radial nerve is formed by
the posterior cord. It runs in the axilla posteriorly
and descends down the arm, moving laterally
while accompanied by the profunda brachii artery
136
as it travels within the radial groove. It then
courses anteriorly over the lateral epicondyle of
the humerus through the cubital fossa and into the
forearm, where it divides into motor and sensory
branches.
Nerve Supply: It supplies the medial and lateral
heads of the triceps muscle of the arm, as well as
all twelve muscles in the posterior compartment of
the forearm and the associated joints and
overlying skin.
Nerve Block: It is usually blocked at the wrist
level (Fig. 1) by performing a eld block just
above the radial styloid aiming medially.
Intravenous Regional Anesthesia
(Bier Block)
Intravenous regional block (Bier block) is an
alternative to peripheral nerve block for extremity
surgery, usually the hand or forearm (White and
Apfelbaum 1997). Peripheral nerve endings of the
extremities are nourished by small blood vessels.
Injection of a local anesthetic solution into a
venous system results in diffusion of the local
anesthetic into the nerve endings with the conse-
quent development of anesthesia. This holds true
for as long as the concentration of the local anes-
thetic in the venous system remains
relatively high.
Technique: The technique uses a 22-gauge
intravenous cannula placed usually in the opera-
tive hand for administration of local anesthetic in
conjunction with one double pneumatic tourni-
quet placed proximally in the arm. The arm is
then elevated, exsanguinated using an esmarch
bandage, and the proximal tourniquet is inated
to maintain xed predetermined pressure. When
the pressure in the tourniquet is stable, the
esmarch bandage is removed. Tourniquet pres-
sures that are 100 mmHg greater than brachial
systolic pressure should be adequate. For most
pediatric patients, insufation pressure of
230 mmHg is usually adequate. 34 mg/kg of
0.5 % lidocaine is injected into the intravenous
line. Anesthesia of the upper extremity develops
in 35 min followed by motor paralysis. If the
surgery is to involve the hand, the intravenous
A.J. Costandi and V. Chidambaran
cannula is removed before the hand is prepped
and draped. If tourniquet pain becomes an issue,
the distal tourniquet (within the zone of anesthe-
sia) is inated and the proximal one is deated.
Insufation times are limited to a maximum of
2 h, and the insufation time should not be less
than 30 min. If the procedure takes less time than
anticipated, the tourniquet can be briey partially
deated and then reinated to gradually release
the lidocaine for systemic breakdown. Once the
tourniquet is released, sensation usually returns
quickly with the release of the tourniquet. The
block is usually very easy to perform, but there
is a risk of local anesthetic toxicity with improper
tourniquet application or accidental tourniquet
deation. Long-lasting postoperative analgesia is
not provided with this technique.
Complications: Although the technique has a
high degree of safety, serious toxic reactions to
local anesthetics occur in about 1.6 % of patients
and include somnolence, incoherence, seizures,
and cardiac arrest. These are typically due to acci-
dental cuff deation within 20 min of local anes-
thetic injection but have also been reported during
tourniquet ination. Other complications include
self-limited skin discoloration or petechiae,
thrombophlebitis, compartment syndrome, and
nerve damage.
Summary
This chapter has elucidated perioperative anes-
thetic and pain management for children
presenting for upper extremity surgery. New anes-
thetic agents are in development and will likely
improve the safety and efcacy of anesthesia. The
gas xenon, one of the noble gases of the periodic
table, is one such agent. It has NMDA antagonist
properties, has minimal side effects, and is supe-
rior to nitrous oxide as it has lower blood-gas
solubility and MAC of 70 %, which makes it a
fast-acting anesthetic. However, its use is cur-
rently restricted by high prices of manufacturing
the gas (Jordan and Wright 2010). Sugammadex
is a selective relaxant-binding agent, which is
currently not approved by the FDA for use in the
USA. Its advantage is that it can effectively
6 Anesthesia
reverse rocuronium effects and, hence, provides
an alternative to succinylcholine for rapid
sequence intubation (Abrishami et al. 2009).
Also, the advent of a liposomal injection of
bupivacaine, for injection into surgical sites to
provide pain relief up to 72 h, has been approved
by the FDA for use in nonpregnant adults.
Another area of recent interest is the use of
pharmacogenomics for personalization of analge-
sia and anesthesia for patients (Chidambaran and
Sadhasivam 2012). After reports of multiple
deaths after the use of codeine in children post-
tonsillectomy and in a breast-fed infant of a
CYP2D6 UM mother (Koren et al. 2006;
Ciszkowski et al. 2009; Kelly et al. 2012), the
FDA has approved pharmacogenetic warnings in
the labels of over 100 drugs, including analgesics
like codeine, tramadol/acetaminophen combina-
tions, and celecoxib (Crews et al. 2012). Like all
other disciplines, this area is constantly evolving,
and the search for the ideal anesthetic that pro-
vides amnesia, analgesia, and muscle relaxation
rapidly and without side effects continues.
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 Part V
Occupational Therapy
 Orthotics and Casting
7
Jenny M. Dorich and Carrissa Shotwell

Contents Rheumatic Diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 162

Joint Hypermobility . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 163
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 142
Reasons Orthotics Are Used in the Pediatric Patient and Family Education . . . . . . . . . . . . . . . . . . . . . 164
Population . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 142 Education: Purpose of the Orthotic . . . . . . . . . . . . . . . . . . 164
Prescribing Wearing Schedules . . . . . . . . . . . . . . . . . . . . . . 164
Orthotic Design . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 143 Strategies to Achieve Adherence with Wearing
Styles of Orthotics . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 143 Orthotics . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 165
Types of Upper Extremity Orthotics and Casts . . . . . 144 Education on Weaning from an Orthotic . . . . . . . . . . . . 166
Therapy Evaluation for Orthotics . . . . . . . . . . . . . . . . 148 Education on Precautions . . . . . . . . . . . . . . . . . . . . . . . . . . . . 166
Occupational Prole . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 148 Practice Donning and Dofng Removable
Health Status . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 149 Orthotics and Casts . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 166
Level of Function . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 149 Educational Supports . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 166
Goals/Outcome Measures . . . . . . . . . . . . . . . . . . . . . . . . . . . 150 Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 167
Orthotics for Specic Conditions . . . . . . . . . . . . . . . . . . 150 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 168
Congenital Differences . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 150
Peripheral Nerve Injury . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 153
Brachial Plexus Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 154
Tetraplegia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 155
Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 155
Arthrogryposis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 157
Muscular Dystrophy and Progressive Neurologic
Conditions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 158
Trauma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 158
Flexor Tendon Injuries . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 159
Extensor Tendon Injuries . . . . . . . . . . . . . . . . . . . . . . . . . . . . 159
Amputations/Replantation . . . . . . . . . . . . . . . . . . . . . . . . . . . 160
Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 161
Dislocations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 161
Hand Infections . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 161
Compartment Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 161
Burns . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 161

J.M. Dorich (*) C. Shotwell

Division of Occupational Therapy and Physical Therapy,
Cincinnati Childrens Hospital Medical Center, Cincinnati,
OH, USA
e-mail: jenny.dorich@cchmc.org;
carrissa.shotwell@cchmc.org

# Springer Science+Business Media New York 2015 141

J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_7
142
Abstract
This chapter provides an overview of the role
of orthotics as a therapeutic intervention for
children with upper extremity conditions. The
chapter begins with an overview of why orthot-
ics are used in the pediatric population,
followed by the basic styles of upper extremity
orthotics. Then the types of orthotics and casts
used along with the functions they serve are
reviewed. The role of orthotics in the care plan
for the specic diagnoses seen in the pediatric
hand population is outlined. Finally, patient
and family education specic to pediatric
orthotics and casting is discussed.
Introduction
The use of orthotics is one of the many treatment
interventions used by therapists who provide care
in the pediatric hand therapy setting. Like other
treatment choices, clinical judgment, client/family
priorities, and medical status help to guide the
decision-making process. It is important to realize
that orthotics are rarely used exclusively, but
rather in conjunction with other interventions as
part of a treatment plan. This chapter will explore
reasons to use orthotics, commonly used orthot-
ics, common diagnoses that benet from orthot-
ics, and special considerations for the pediatric
population.
This chapter is meant to serve as an overview
of pediatric orthotics. There are many factors to
consider when evaluating and using orthotics with
children. Two of the most important aspects to
consider are the child and the family. It is essential
to consider the childs home life and use a collab-
orative approach with the family and clinicians
overseeing care (Chen et al. 1999). Likewise,
caregiver education is necessary for successful
orthotic intervention. Other important factors to
consider are the patients developmental stage,
health status, and their occupations, such as
school, sports, and extracurricular activities. The
young child in particular may present with unique
challenges such as poor tolerance for wearing
J.M. Dorich and C. Shotwell
orthotics and difculty with understanding the
need for an orthotic (Ho et al. 2010). These chal-
lenges can lead to decreased compliance with
orthotic wear and the need for problem-solving
effective options for increasing adherence to
orthotics or using alternative treatment interven-
tions to meet treatment goals.
Reasons Orthotics Are Used
in the Pediatric Population
Orthotics are rarely the sole decision of the
treating therapist. Often, clients are referred to
the occupational therapist by a physician, nurse
practitioner, or physicians assistant speci-
cally for the purpose of orthotic fabrication.
Other times, the therapist through treating the
patient concludes that an orthotic may be a
benecial intervention to achieve goal attain-
ment. This chapter will explore specic types
of orthotics that are used in conjunction with
their purpose. The following are common rea-
sons that orthotics are used in the pediatric
population:
Optimize function
Protect for healing
Decrease pain
Limit deformity
Increase range of motion
In some cases, the style of orthotic is specic,
and the purpose for wearing the orthotic allows for
little adjustment in the orthotic design. For exam-
ple, children are often referred for dorsal blocking
orthotics following a exor tendon injury. In this
example, the purpose of the orthotic is for protec-
tion and the orthotic style and wearing schedule is
prescribed by the postsurgical therapy protocol
and referring practitioner. In other situations, the
style and purpose of the orthotic allow the thera-
pist considerable exibility in orthotic design.
Thorough initial evaluation and continued assess-
ment is essential in choosing the appropriate
orthotic for each child (Basford and Johnson
2002).
7 Orthotics and Casting
Orthotic Design
Styles of Orthotics
Therapists working in a pediatric hand care set-
ting have a variety of options available. Often,
therapists custom fabricate orthotics, typically
from thermoplastic materials or less commonly
with a soft material, such as neoprene. When a
therapist makes a custom orthotic for a child,
they can t the child with the orthotic on the
day they initially provide care to the child. Ther-
apists may also use prefabricated orthotics,
which are those made by an external vendor.
Prefabricated orthotics are available in a wide
variety of styles and materials. Often, a therapist
must measure a child for prefabricated orthotics
and then order them from the vendor, resulting
in a waiting period between sizing the child
for the orthotic and then tting the child with
the orthotic. Therefore, the time frame in
which the child must begin wearing the
orthotic often dictates what type(s) of orthotics
may be an option for the child. When an
orthotic is needed immediately, therapists will
typically custom fabricate an orthotic for the
child. If a waiting period is acceptable between
the initial orthotic appointment and tting the
child with the orthotic, both custom-molded
orthotics and prefabricated orthotics may be an
option.
Orthotics custom fabricated from thermoplas-
tic material can be formed to be the most custom
t to each individual patient and are sturdy rigid
support. Therefore, therapists use thermoplastic
materials when the purpose of the orthotic is to
protect the upper extremity during healing or
provide complete immobilization of specic
joints. Thermoplastic materials are also com-
monly used when the orthotic design requires
contoured tting. Orthotics made from thermo-
plastic materials may be adjusted easily by
reheating the material, making thermoplastic
orthotic material ideal for serial static orthotics.
There are a wide variety of thermoplastic mate-
rials available to fashion orthotics for children.
143
Some materials are perforated that allows for
greater ventilation than solid thermoplastic mate-
rials. If the child needs to wear the orthotic for
long periods of time or has sensitive skin, perfo-
rated materials may be best. Many materials
come in a variety of color options helping to
make the orthotic more child friendly by allowing
the child to help design their orthotic.
Therapists can construct some styles of orthot-
ics from neoprene, velfoam, or other soft mate-
rials. Most often soft materials are used when
fabricating orthotics in very young infants, espe-
cially in the neonatal intensive care environment
because of the infants small size and delicate
skin. While these soft materials do not provide
the same degree of rigid support as thermoplastic
materials, neoprene and similar materials may be
adequate to prevent joint deforming positions in
neonates and very young infants. Neoprene also
can be used to construct small thumb supports that
support the rst CMC joint in abduction when an
infant has a Blauth type I or II hypoplastic thumb,
clasped thumb, or abnormal muscle tone in the
thumb that limits the childs pincer grasp. Addi-
tionally, prefabricated versions of thumb supports,
such as the McKie thumb orthotic (Duluth, MN),
are available and have been found to improve
hand function in children with cerebral palsy
who have thumb-in-palm deformity (Fig. 1;
Ten Berge et al. 2012).
In some cases, a child may not tolerate the
rigidity of a thermoplastic orthotic or the care
team may desire for the child to have increased
support at a joint without complete immobiliza-
tion of the joint. In these instances, neoprene
may also be the material of choice. For example,
a child with substantial ligamentous laxity and
chronic wrist pain who consistently has pain
with writing and poor CMC joint stability may
experience less pain when using a neoprene
wrist and thumb spica orthotic. Benik
(Silverdale, WA) is a vendor that makes a wide
selection of prefabricated and custom-sized neo-
prene orthotics in pediatric sizes. Other vendors
make neoprene orthotics that are available in
adult sizes that may t children nearing skeletal
maturity.
144 J.M. Dorich and C. Shotwell

Types of Upper Extremity Orthotics
and Casts
When considering what type of orthotic or cast to
prescribe, it is helpful to consider what purpose
the orthotic/cast will serve. If orthotics are needed
to optimize function of the upper extremity, the
Fig. 1 A Mckie splint is a prefabricated soft splint that
helps to support the thumb in abduction for improved
functional grasp
role of the orthotic is typically either to provide
increased stability or to assist with active range of
motion. Orthotics to support the interphalangeal
(IP) joints, metacarpophalangeal (MCP) joints,
and thumb carpometacarpophalangeal (CMC)
joint and/or wrist are the most common styles
of orthotics used for the purpose of providing
stability at a joint or multiple joints for improved
function in cases where there is no neurologic
involvement. Table 1 above outlines the most
common styles of orthotics used for improving
hand function by providing increased joint stabil-
ity and the various material options for the orthot-
ics. The specic styles of orthotics use for
improving function by either providing joint sta-
bility or assisting active range of motion when
nerve injury is presented are in Table 3.
Pediatric occupational therapists may also use
an orthotic, such as a resting hand orthotic, or a
cast (Fig. 2) to constrain the unaffected upper
extremity for the purpose of improving hand func-
tion in children with hemiplegia (Aarts
et al. 2010). This is typically performed in cases
where a child has sustained an injury to the
peripheral nervous system resulting in upper
extremity impairment, such as an obstetrical bra-
chial plexus birth palsy (Abdel-Kafy et al. 2012),
or when an insult to the central nervous system
has resulted in unilateral upper extremity impair-
ment such as cerebral palsy (Huang et al. 2009).
With constraint-induced movement therapy, the
therapist applies a cast or an orthotic to the unaf-
fected upper extremity to facilitate increased use

Table 1 Orthotics for joint stability for improved function

Orthotic style
Short opponens orthotic
Wrist and thumb spica
Volar based
Radial based
Wrist immobilization orthotic
(or wrist cock-up orthotic)
Volar
Dorsal
Figure of eight orthotics
Structures immobilized
First CMC joint and thumb MCP
Wrist and rst CMC joint and
thumb MCP
Wrist
Distal interphalangeal (DIP) or
proximal interphalangeal (PIP)
joints
Material options
Thermoplast, neoprene, neoprene with
thermoplastic insert
Thermoplast, neoprene, neoprene with
thermoplastic insert or neoprene with metal
stay
Thermoplast, neoprene, neoprene with
thermoplastic insert, or neoprene with metal
stay
Oval-8, thermoplast, silver ring orthotics
7 Orthotics and Casting 145

Fig. 3 An ulnar gutter splint includes the ring nger and

Fig. 2 A cast can be placed on the unaffected extremity to small nger in safe position and wrist in neutral to slight
promote increased functional use of an affected upper hyperextension
extremity with a constraint-induced movement therapy
protocol

of the impaired upper extremity. A variety of pro-

tocols for constraint therapy exist ranging from
application of a cast that is not removable to
application of a constraint cast or orthotic for a
specied amount of time or set of activities on a
regular basis (Huang et al. 2009).
Orthotics that are used for protection (Figs. 3
and 4) of the upper extremity after an injury or
surgery must be designed to include the specic
structures that should be immobilized for healing.
Often these orthotics are fabricated out of thermo-
plastic materials so that they have the rigidity
necessary to provide the degree of immobilization
adequate to promote continued healing of the
injured or surgically repaired structures. Similarly,
Fig. 4 A nger gutter splint serves to immobilize one
orthotics that are being used to provide pain relief digit. It can be donned with Velcro straps as shown or
are constructed to immobilize or position the using tape
146 J.M. Dorich and C. Shotwell

Table 2 Common orthotics used to promote healing

Orthotic style Structures immobilized Variations
Ulnar gutter Fourth and fth digits with MCPs in 5070 of Hand based: most proximal end is just distal to
exion and IPs extended; wrist is in neutral to the wrist exion crease
15 of extension Forearm based: wrist included in neutral to
15 degrees of extension
Third nger included: referring provider may
request that the orthotic is extended radially to
include the third digit
Finger gutter Can include one or multiples the following Sometimes the clinician will extend a nger
joints of: DIP, PIP, MCP of one digit gutter splint to be hand based in children with
small hands to facilitate the splint staying on
Radial gutter Second and third digits with MCPs in 5070 of Hand based: most proximal end is just distal to
exion and IPs extended; wrist is in neutral to the wrist exion crease
15 of extension Forearm based: wrist included in neutral to
15 degrees of extension
Resting hand Wrist (neutral to 1520 of extension) and digits The thumb may be omitted if the nature of
(extension or 1015 arc of digit exion) and injury/surgery does not require immobilization
thumb (palmar abduction) of thumb
Safe position: MCPs are positioned in 50-70
degrees of exion and IPs are in full extension.
Indicated when prolonged immobilization is
desired so that collateral ligaments of MCPs do
not tighten
Thumb spica Wrist in 015 of extension; thumb in palmar Hand based: the wrist is not included
abduction with IP free IP included: the IP joint of the thumb is
immobilized
Dorsal MCPs included in safe position (5070 of This splint may be modied in several ways:
blocking exion); IPs in neutral extension; wrist in neutral Hand based or forearm based
orthotic PIP extension may be blocked to prevent full
PIP extension
Specic digits may be excluded from the
splint (such as the index nger) to allow greater
function if the precautions allow for this
Wrist Wrist in 015 of extension and neutral Volar based
immobilization deviation Dorsal based: allows palm of hand free for
greater sensory input to the hand

upper extremity to allow inamed structures to
rest. Table 2 below provides an overview of the
most common orthotics used to promote healing.
Orthotics may be used to limit deformity in
cases of neurologic impairment (Langlois
et al. 1989), either that of the central nervous
system affecting the upper extremity or peripheral
nerve impairment in the upper extremity. Table 3
outlines those orthotics most often used with
peripheral nerve injury. In cases where there is
an upper motor neuron injury causing abnormal
muscle tone in the upper extremity, the most
effective orthotic design to limit deformity is
usually that which includes all structures contrib-
uting to the loss of range of motion (Figs. 5 and 6).
The clinician must carefully assess the joint
mobility to determine the presence of tendon
tightness and/or joint tightness and assess
which muscle/tendon groups should be placed
on stretch with the orthotic design. Finally, in
cases of severe tissue trauma, such as a burn
(Feldmann et al. 2008) or Volkmann ischemic
contracture, positioning in an orthotic may serve
to limit contracture and deformity.
Orthotics or casts may be used to improve joint
range of motion in cases where tissue tightness
7 Orthotics and Casting
Fig. 5 This is a Benik elbow splint. It is made of neoprene
with a moldable thermoplastic insert so that the splint may
be positioned to match the childs positioning need
Fig. 6 This is a Benik resting hand splint made from
neoprene with a moldable thermoplastic insert. The splint
may be molded by the therapist to achieve the desired
position of support
limits range of motion (Bhat et al. 2010; Lucado
and Li 2009). In cases where therapists apply
orthotics to improve joint mobility, consultation
with the referring provider is recommended to
ensure that the range of movement is not limited
by a bony block or joint incongruity. Therapists
may employ one of several orthotic/cast interven-
tions with the goal of improving joint range of
motion:
1. Serial static orthotics: When serial static
orthotics are employed to improve joint mobil-
ity, a static splint, such as a nger gutter splint
or elbow extension splint, is fabricated to posi-
tion a stiff joint at its end range. The static
147
splint is then serially remolded over a series
of therapy sessions into increased joint range
until the desired passive range of motion is
achieved or a plateau occurs. Often static
orthotics are worn at nighttime to allow the
child movement during the daytime (Schultz-
Johnson 2002). Serial static orthotics are a
good option in cases where the child is
returning for regular therapy visits since the
splint must be adjusted by the therapist to
achieve a greater stretch. Because they are
simple in design and lack movable compo-
nents, they do not pose considerable safety
risks when used with children.
2. Static progressive orthotics: Static progres-
sive splinting is the use of inelastic compo-
nents such as hook and loop tapes, static
line, progressive hinges, turnbuckles, screws,
and gears to apply torque to a joint in order to
statically position it as close to end range as
possible and thus increase passive range of
motion (Schultz-Johnson 2002). With a static
progressive orthotic, the principal of tissue
elongation through low load and prolonged
stretching is applied to improve joint mobility
(McClure et al. 1994). Therapists may custom
fabricate static progressive orthotics (Fig. 7) or
use either prefabricated orthotics (Fig. 8).
Static progressive orthotics may be prescribed
to be worn anywhere from three 30-min ses-
sions per day (Doornberg et al. 2006) to as
much as 23 h per day (Schultz-Johnson 2002).
3. Dynamic orthotics: Dynamic splints (Fig. 9)
employ elastic components, such as rubber
bands or springs, to provide a passive assist
to joint motion or mobilize a stiff joint
(Schultz-Johnson 2002). Both prefabricated
and custom fabricated styles of dynamic
orthotics are options. When dynamic orthotics
are being used for the purposes of increasing
joint mobility, they are worn for a specied
amount of time to achieve a sustained stretch.
They can be removed between wearing periods
to allow for range of motion exercises and/or
functional use of the upper extremity.
4. Serial casts: Serial casts (Fig. 10) may be
applied to be worn without removal until the
next therapy visit or they can be bivalved so
148
that they may be removed and worn for a
portion of each day or night. Serial casts may
be used to decrease joint tightness that limits
mobility after a trauma such as a stiff PIP joint
Fig. 7 This is a custom-molded static progressive PIP
exion splint for the middle nger. The turnbuckle on the
volar surface of the splint allows the splint to be serially
adjusted into increased PIP exion as the child is able to
tolerate an increased stretch
Fig. 8 The JAS elbow
splint is a prefabricated
static progressive elbow
splint that may be used for
improving elbow exion or
elbow extension
J.M. Dorich and C. Shotwell
lacking extension (McClure et al. 1994). They
may also be used to reduce joint contractures in
the upper extremity that have resulted from
spasticity in conditions such as cerebral palsy
(Pohl et al. 2002).
The decision as to which style of orthotic to use
for increasing joint mobility is complex in nature.
A variety of factors guide the therapist and care
team in determining which orthotic design might
be the best for a particular child. The therapist
should obtain a thorough assessment of the childs
tolerance for orthotics, ability to wear an orthotic
for the advised wearing schedule, caregiver sup-
port, skin integrity, and ability to follow directions
for monitoring orthotics and performing neces-
sary adjustments to orthotics to determine if any
of the above orthotic options is appropriate for the
child.
Therapy Evaluation for Orthotics
Occupational Profile
Gathering the appropriate information from the
child and family is essential in choosing the
appropriate orthotic (Hanna and Rodger 2002).
7 Orthotics and Casting
Fig. 9 Prefabricated dynamic splints may be used during
the daytime to improve PIP extension
Fig. 10 Casts, such as this elbow extension cast, may be
applied serially in an increased stretch to improve range of
motion when a child has persistent joint tightness
It is important to realize that each child has unique
needs that require thought and clinical judgment
from the therapist. When the purpose of the
orthotic is for function rather than protection, an
occupational prole or a client/family interview is
benecial in determining the right orthotic for that
child/family (DeGrace 2003). Some of the com-
mon questions to keep in mind while evaluating
the patient are:
What is the goal of the orthotic (e.g., is it to
limit deformity or improve function during a
particular task)?
What are the occupations (e.g., student, athlete,
musician, etc.) of the patient?
149
Is this a long-term or short-term option?
Will the child benet more from a daytime
wearing schedule or nighttime?
What is a realistic wearing schedule for the
child, considering developmental stage and
occupations?
The occupational prole will provide the
therapist greater insight to the specic needs
of each individual child, their goals, and the con-
text in how the orthotic will be helpful in their
lives.
Health Status
The childs health status typically has a substan-
tial impact on the appropriate orthotic style.
The childs health status informs the therapist of
what precautions must be considered when
choosing an orthotic and may also provide infor-
mation as to how the child may be able to tolerate
the orthotic. The childs health status may
include their diagnoses, previous surgeries, and
current or previous therapies. Skin integrity is an
important factor of a childs health status. New-
born and younger children may have more sen-
sitive skin than teenagers. It is not uncommon for
a child to have sensitive skin that breaks into rash
easily. A good question to ask the caregivers is
whether the child sweats excessively or often.
Too much moisture may lead to skin breakdown.
Discomfort can lead to difculty with tolerating
the orthotic and poor compliance. The patient
may have extensive regimental care that may
affect the wearing schedule of the orthotic.
These considerations will factor into the wearing
schedule and the type of orthotic material that is
chosen. In order for a patient and family to be
compliant with orthotic wear, it must be tolerated
by the child and t into their daily routine
(Callinan and Mathiowetz 1996).
Level of Function
It is important to assess the childs current level
of function. The childs level of function will
150
help you to determine if he or she will be donning/
dofng the orthotic independently or if they will
need assistance from a caregiver. Additionally, the
child may be able to take off the orthotic and may
attempt to do so rather than wearing it as pre-
scribed. These factors may affect the overall
design and wearing schedule of the orthotic
(Ho et al. 2010).
When recommending an orthotic to improve
function, it is benecial to observe the child
complete tasks while wearing the orthotic and
without the orthotic (Goodman and Bazyk
1991). Sometimes this can be done with sample
orthotics, taping, or with the patients previous
orthotic. In other cases, it can only be done
once the orthotic has been donned on the child.
Often a patients positioning may be improved
in an orthotic; however, function may be
compromised. For example stabilizing a wrist
may impede a tenodesis grasp. In some
instances, several orthotics are trialed before
deciding upon the optimal orthotic. Sometimes,
the therapist determines it is best to not use an
orthotic (Frohlich et al. 2012).
Goals/Outcome Measures
Many therapists nd it benecial to use a formal
assessment tool to gather information about a
childs functional abilities and limitations. A fre-
quently used tool is the Canadian Occupational
Performance Measure (COPM) (Case-Smith
2003; Dedding et al. 2004). Other outcome
measures may also be used (see chapter Out-
come Measures). Outcome measures are useful
tools in determining how the patients function is
affected by his or her diagnosis and whether an
orthotic is indicated to help improve function.
The COPM may also be helpful to determine the
patients and familys goals. Often a patient will
come to the evaluation with the goal of having
the same orthotic that he or she has always had.
Sometimes after reviewing the outcome measure,
it is discovered that a different orthotic may
be more suitable. Outcome measures can be
readministered at a later date to determine the
effectiveness of the orthotic.
J.M. Dorich and C. Shotwell
Orthotics for Specific Conditions
Congenital Differences
Children with congenital differences may benet
from upper extremity orthotics to improve func-
tion, limit deformity, or provide protection follow-
ing surgical reconstruction of their upper
extremity. The most common congenital differ-
ences for which orthotics are used include longi-
tudinal differences, camptodactyly, thumb
hypoplasia, and preaxial (radial) polydactyly.
Orthotics are also used in the treatment of amni-
otic band syndrome with associated nerve com-
pression and congenital clasped thumb. Less
commonly, orthotics are used with syndactyly
releases.
Longitudinal Deficiency: Radial
and Ulnar
When children have a radial or ulnar longitudinal
difference, a wrist immobilization orthotic may be
used to prevent a xed wrist radial or ulnar devi-
ation contracture, respectively. The wearing
schedule of a wrist immobilization orthotics for
a child with an untreated longitudinal difference
depends on whether or not the child is more func-
tional when wearing a wrist orthotic that aligns
their wrist in more neutral deviation. When chil-
dren achieve greater function with their wrist
positioned by an orthotic, then day wear of the
orthotic is recommended. Kennedy (Kennedy
1996) found that children experienced increased
functional use of their affected upper extremity
when wearing a neoprene wrist support (Fig. 11).
Furthermore, older children being treated with a
neoprene wrist orthotic reported a preference for
the neoprene orthotic over prior wrist orthotics
fabricated from thermoplastic materials. When
children will not tolerate wearing an orthotic dur-
ing the day, night wear of the orthotic to provide a
sustained stretch into neutral alignment is used to
prevent a xed deviation contracture. If a child has
a wrist centralization procedure, a wrist immobi-
lization orthotic for supporting the wrist in neutral
alignment is employed once the child completes
an adequate period of immobilization in a
7 Orthotics and Casting
Fig. 11 Therapists may fabricate soft neoprene wrist sup-
ports such as the one depicted to support neutral wrist
alignment
postsurgical cast. A circumferential (a.k.a. clam
shell) style orthotic and an ulnar gutter with radial
return (in the case of radial longitudinal decien-
cies) are commonly used styles of orthotics
to maintain neutral wrist alignment. Goldfarb
et al. (2011) outlines a protocol of wearing the
orthotic at all times except for AROM and light
play until 6 months out from surgery and then
transitioning to night wear until skeletally mature.
Camptodactyly
Conservative treatment of congenital
camptodactyly is the standard of practice since
surgical results are uniformly disappointing
(Goldfarb et al. 2011). Resolution or reduction
of the PIP exion contracture maybe achieved
through serial static extension orthotics (Benson
et al. 1996; Bonhomme et al. 2013). Bonhomme
et al. (2013) used night orthotics with serial
151
adjustments for increased extension and found it
took an average of 7 months for 62 % of
subjects to achieve full PIP extension and 38 %
to achieve PIP extension of 10 or better. In this
study, an extension orthotic with the MCP and
wrist held in neutral or slight extension was used.
Surgical intervention is reserved for only those
cases which do not respond favorably to conser-
vative intervention (Benson et al. 1996). When
surgical reconstruction is performed, serial orthot-
ics or casts prior to surgery may help to lessen the
joint contracture and lengthen tight tissues prior to
surgery (McFarlane et al. 1992). Once the pin is
pulled after surgical reconstruction, an extension
orthotic is worn continually for 2 weeks, and then
transition to a night orthotic is suggested. Night
orthotics are continued for 36 months following
surgery (McFarlane et al. 1992). Alternatively,
Goldfarb et al. (2011) use a protocol of wearing
a hand-based or forearm-based orthotic at all
times except for hygiene and ROM exercises
from 2 to 6 weeks after surgery and then switching
to night orthotic that is continued until 12 weeks
out from surgery or longer if an extension lag
persists. They also use a dynamic extension
orthotic during the day beginning at 6 weeks
post-op if a considerable lag in extension is
present.
Thumb Hypoplasia
With thumb hypoplasia, Blauth type I and II,
orthotics that support the thumb in palmar abduc-
tion can improve function for activities requiring
gross grasp or palmar pinch grasp. Typically soft
neoprene styles of orthotics are adequate to pro-
vide this level of improved function. A wrist
and thumb spica orthotic is used in the postoper-
ative rehabilitation of children who undergo
surgical management of a hypoplastic thumb,
either a pollicization or thumb reconstruction.
While timing of transition from a postoperative
cast to an orthotic can vary, it most often occurs
46 weeks following surgery. Continual orthotic
wear aside from hygiene, AROM, and light func-
tional activities/play is used for 24 weeks fol-
lowing surgical reconstruction, and a night
orthotic is continued until 3 months out from
surgery (Goldfarb et al. 2011).
152
Preaxial Polydactyl
When a child undergoes surgical reconstruction of
their thumb for preaxial polydactyly, the child is
transitioned from the postoperative cast to a
thumb spica around 4 weeks following surgery.
Initially, the orthotic is worn at all times except for
hygiene, range of motion exercises, and super-
vised light functional use of the hand. Six weeks
following surgery, the child can be transitioned
to a night wearing schedule that is continued up
to 3 months from surgery if the child is at risk
of developing a rst webspace contracture
(Goldfarb et al. 2011).
Congenital Clasped Thumb
Orthotics have shown to be an effective interven-
tion for resolving grade I congenital clasped
thumbs without an associated soft tissue contrac-
ture (Lin et al. 1999; Tsuyuguchi et al. 1985). Lin
et al. (1999) describes a protocol of using a short
opponens orthotic that holds the thumb in MCP
extension and radial abduction, while Tsuyuguchi
et al. (1985) used a forearm-based orthotic with
the thumb MCP and IP joints in neutral and the
CMC in abduction. Both found that with full-time
wear of the orthotic, it took 36 months for the
child to achieve consistent active MCP extension
of the thumb. They also both continued with night
orthotics for an additional 13 months.
When surgical reconstruction of a clasped
thumb is necessary, the child is placed into a
forearm-based thumb spica orthotic after pins are
pulled. Goldfarb et al. (2011) recommends the
child wears the orthotic with the thumb supported
in radial and palmar abduction at all times aside
from hygiene, range of motion exercises, and light
functional use or play until 2 months following
surgery. Their protocol involves continuing with
the orthotic at night until the child is 3 months
from surgery. Tsuyuguchi et al. (1985) initiated
nighttime orthotics only once pins are pulled and
continued with night orthotics until the child was
6 months from surgery.
Amniotic Band Syndrome
Amniotic band syndrome may result in a child
having motor loss from a band compressing a
peripheral nerve (Kozin 2005). In such cases,
J.M. Dorich and C. Shotwell
upper extremity orthotics may be part of the treat-
ment plan used to preserve joint mobility until
active movement is restored. Because orthotics
are employed when the child is newborn, a
forearm-based resting hand orthotic is most effec-
tive. If the radial nerve is involved, the MCP joints
and wrist are positioned in extension and thumb in
radial deviation. If motor function of the ulnar
and/or median nerve is affected, then the MCPs
should be placed in 6070 of exion with the IPs
extended to prevent a claw deformity.
Syndactyly
Orthotics are not typically necessary following a
syndactyly reconstruction, unless the surgery
involves the rst webspace. When surgical revi-
sion occurs in the rst webspace, a c-bar (Fig. 12)
or thumb spica with a c-bar is used to maintain the
rst webspace once the postoperative dressing is
removed (Coombs et al. 1994). Typically, a night-
time wearing schedule is adequate and is contin-
ued until 36 months out from surgery (Goldfarb
et al. 2011). If the child is not actively moving the
thumb into abduction and opposition regularly
with functional use of the affected, then orthotics
may be used during the day as well.
Therapists may use a resting-hand-style
orthotic as the base of support for an elastomer
scar pad that is worn at nighttime when a syndac-
tyly reconstruction involves a webspace other
than the rst. Using a highly perforated orthotic
material as a base for moldable elastomer products
allows for a custom-formed elastomer scar pad to
be securely afxed to an infant or toddlers hand
following surgery, decreasing the choking risk
posed by a scar pad.
Customized Functional Orthotics
for Children with Congenital Differences
Pediatric hand therapists may be called upon to
design orthotics to allow a child with an upper
extremity congenital difference to participate in
specic activities. Figure 13 is an example of an
orthotic designed to assist a child with
symbrachydactyly in riding a bicycle. While
children with congenital differences in their
upper extremities are quite adept at achieving
functional goals, anatomical differences, such as
7 Orthotics and Casting 153

Fig. 12 A rst webspace

c-bar splint may be used
when the depth of a
webspace needs to be
maintained

asymmetrical limb length or underdeveloped

digits, may impede children in a few very specic
activities. In such cases, therapists employ activity
modications, which may include uniquely
designed orthotics to allow a child to use specic
tools.

Peripheral Nerve Injury

When children experience trauma affecting a

Fig. 13 Children with congenital impairment or other
conditions that impact their ability to use an upper extrem-
ity for a particular activity may require a customized splint
to facilitate participation in a desired activity. The above is
a splint made so that a child with a congenital impairment
could ride his bike
peripheral nerve, the pattern of motor loss is spe-
cic to the level of nerve injury and specic
nerve(s) involved. Table 3 outlines the styles of
orthotics employed in providing care to children
recovering from a peripheral nerve injury. While
these are the typical styles, therapists take into
consideration the patients functional abilities and
limitations, speed of recovery, and patient/family
preferences and resources when determining the
best option for each specic child.
If the nerve does not fully return, then the child
may undergo tendon transfers to regain motor
function lost from the nerve injury. A child must
have good passive joint mobility prior to tendon
transfers. If contractures are present, orthotics or
serial casting may be used to achieve improved
154 J.M. Dorich and C. Shotwell

Table 3 Orthotics for peripheral nerve palsies

Nerve
involved Pattern of motor impairment Recommended orthotics
Radial Absent or weakened: wrist extension, MCP Benik radial nerve orthotic; Robinson InRigger
nerve extension, and thumb radial abduction (adult sizes only); P-1 blocker immobilization
orthotic with MCPs and wrist in extension wrist
Median Absent or weak: pronation, wrist exion, digit Thumb spica with thumb in palmar abduction
nerve exion of the PIPs and DIPs of the IF and MF,
(high thumb palmar abduction and opposition, thumb
lesion) exion
Median Absent or weak: thumb palmar abduction and Thumb spica with thumb in palmar abduction or
nerve (low opposition short opponens orthotic
lesion)
Ulnar Absent or weak intrinsic muscles resulting in claw Anticlaw orthotic (Fig. 14) for SF and RF (note:
nerve positioning of RF and SF if the child also has median nerve injury, the claw
deformity will be present in the index, middle,
ring, and small nger and the anticlaw orthotic
should include all four digits)
Note: IF index nger, MF middle nger, RF ring nger, SF small nger

Fig. 14 Anticlaw splints

position the MCPs in
exion and facilitate IP
extension. They can be
designed to include the
ulnar two digits for an ulnar
nerve palsy or all four digits
in case of an ulnar nerve and
median nerve palsy

joint mobility prior to surgery. Following at
tendon transfer, the child is typically casted for
46 weeks and then they may be transitioned to a
protective orthotic. The design of the protective
orthotic should be such that the joints are posi-
tioned to keep the all-transferred muscle groups in
a shortened position to prevent overstretching the
transfer (Skirven et al. 2011). Once the child has
learned to activate the transfer independently to
achieve the desired joint movement, then the
orthotic may be transitioned to night wear only.
This typically occurs around 2 months postopera-
tively. After the transfer is strengthened and
the child is using the transfer regularly during
functional tasks, the night orthotic may be
discontinued.
Brachial Plexus Palsy
Children may have an obstetrical brachial plexus
palsy or sustain a traumatic brachial plexus injury.
7 Orthotics and Casting
The pattern of motor and sensory impairment with
brachial plexus palsies follows the distribution of
injured nerve roots or trunks. Often, in the initial
period following a lesion to the brachial plexus,
the child will require a resting hand orthotic to
prevent contractures of the wrist, digits, and rst
webspace (Eng et al. 1996).
When recovery is incomplete, children may
undergo surgical intervention, such as nerve or
tendon transfers. In such cases, orthotics may be
used in the postoperative rehabilitation plan to
protect the extremity. The specic style of orthotic
used depends on the type of procedure performed.
The therapist and surgeon should collaborate to
determine the best orthotic design to provide the
necessary protection and support to the upper
extremity during the postoperative recovery
stage. When splints are necessary following sur-
gery, they are typically implemented 46 weeks
following surgery.
It is common for children who have sustained
an obstetrical brachial plexus to develop an elbow
exion contracture during the recovery process.
The use of botulinum toxin type A combined with
a 4 week serial casting protocol has been found
to be effective in improving elbow extension
(Basciani and Intiso 2006).
Tetraplegia
Children with tetraplegia resulting from a spinal
cord injury (SCI) may have orthotic needs that
vary depending on where they are in the recovery
process and the level of their spinal cord injury.
In early phases of rehabilitation, orthotics may be
necessary to limit joint contractures as the child is
experiencing autonomic storming. Elbow exten-
sion orthotics and/or resting hand orthotics are the
most common needs during this time. As the
childs acute stage of healing stabilizes, children
may continue to benet from orthotics to prevent
deformity as well as orthotics to optimize func-
tion. However, because tenodesis grasp and/or
lateral pinch are compensatory grasp patterns
used by children with SCI, clinicians must care-
fully assess that the orthotic being considered to
improve function does not actually impede
155
functional grasp patterns. When the level of SCI
is incomplete, recovery may occur for several
months past the injury. Once motor and sensory
return plateau, a child may be a candidate for
reconstructive surgery to maximize their upper
extremity function (Waters et al. 1996; Hamou
et al. 2009).
If the child has any joint contractures that
would limit the outcome of tendon transfers,
static progressive orthotics, serial static orthotics,
or serial casts are used to restore joint mobility
(Gellman et al. 1994). Children with tetraplegia
who have undergone tendon transfers to
optimize upper extremity function are generally
immobilized in a postoperative cast for 1 month.
After that time, they transitioned to a protective
orthotic and begin postoperative therapy proto-
cols. Protective orthotics are initially worn at all
times except for performing home program activ-
ities for rehabilitation. By 2 months postoperative,
the child may begin to wean from the orthotic
during the day continuing it for protection when
engaged in activities other than range of motion
exercises and light functional use. By 3 months
postoperative, orthotics are transitioned to night
wear and continued until 46 months out from
surgery (Skirven et al. 2011). The style of orthotic
used depends on the tendon transfer performed.
The orthotic must be designed position the
transferred muscle(s) in a shortened position to
prevent overstretching during the early phases of
rehabilitation.
Cerebral Palsy
Children with cerebral palsy (CP) and associated
muscle spasticity of their upper extremities may
require orthotics for improved function, to pre-
serve joint mobility, to correct deformity, or for
protection following reconstructive surgery to the
upper extremity. The degree to which spasticity
affects upper extremity function varies widely
among children with cerebral palsy. When deter-
mining if a child with cerebral palsy would benet
from upper extremity orthotics for improved func-
tion, it is imperative that therapists and referring
providers observe the childs hand function.
156
Table 4 Common orthotic styles to preserve deformity for children with cerebral palsy
Orthotic
type Role of orthotic
Resting Position wrist to limit exion contracture
hand Provides stretch to the long nger exors that may be
orthotics contributing to wrist exion and tightness with nger
extension
Position thumb in palmar abduction
Elbow Position the elbow in an extension stretch
extension
orthotics
Some children rely on dynamic wrist movement
for a tenodesis grasp and release or other compen-
satory movement patterns that orthotics may
impede. Orthotics can also diminish the sensory
feedback the upper extremity receives in the areas
covered by the orthotic. Children with CP often
have impairment in the sensation of their impaired
upper extremity (Cooper et al. 1995) and may nd
that an orthotic intended for improved function
actually limits function by further altering the
sensory acuity of their upper extremity. If a clini-
cian feels a child with CP may benet from an
orthotic for improved hand function, the clinician
may be able to simulate an orthotic through man-
ual support to observe the child participating in
specic functional activities and compare and
contrast their function with and without the man-
ual support to determine if an upper extremity
orthotic may improve hand function.
There is little published evidence to support the
use of upper extremity orthotics for improved
hand function in children with CP. Ten Berge
et al. (2012) found that children with hemiplegic
cerebral palsy (Manual Ability Classication Sys-
tem (MACS) level 2 and 3) who wore a neoprene
McKie thumb support did display improved func-
tion. The MACS is a scale to classify the ability of
individuals with CP to handle objects used in
daily activities with their hands. Children may
also benet from use of an orthotic for a specic
functional activity or set of functional activities.
For example, a child who uses assistive
J.M. Dorich and C. Shotwell
Style considerations
May be made as either volar or dorsal
based. Dorsal-based styles (Fig. 15) often
stay on children with upper extremity
spasticity better than volar-based styles
Most children who have considerable
weakness in the wrist extensors or a wrist
exion contracture possess tightness of the
long nger exors in addition to the wrist
exors so including a stretch to the ngers
into extension is necessary to limit
contracture
Circumferential style orthotics may be the
most comfortable and best tolerated
technology for communication or written expres-
sion may have increased ease using their index
nger(s) to activate the device when an orthotic
that supports the nger in extension is worn.
The more the disease limits function in the
upper extremity of a child with CP, the greater
the likelihood the child will develop muscle
imbalances that can lead to joint deformity in the
upper extremity. If a child is observed to have
spasticity and muscle weakness that results in
the child regularly positioning their joint(s) in
positions of deformity, then orthotics to preserve
joint mobility may be indicated. Some of the most
common orthotic styles used for preserving joint
mobility are listed in Table 4. When orthotics
are used for the purpose of maximizing joint
mobility, a nighttime wearing schedule is often
recommended (Schultz-Johnson 2002) so that
orthotics do not limit the opportunity for function
during the day. Because of complex medical con-
cerns, some children are unable to tolerate
sleeping in orthotics. In these situations, therapists
may work with families to identify specic day-
time periods during which wearing orthotics
would minimally impact function. Additionally,
if a child requires orthotics bilaterally or for both
the elbows and distal upper extremity, then an
alternating wearing schedule can be used.
Orthotics or serial casting may be used to cor-
rect joint contractures in children with CP. Serial
casting or static serial splinting can be used to
reduce elbow exion and wrist exion
7 Orthotics and Casting
Fig. 15 Dorsal resting
hand splints may be made
custom made from
thermoplastic materials by
therapists
contractures. The use of botulinum injections
paired with serial static thumb splints has been
effective in improving joint range of motion and
hand function in children with thumb-in-palm
deformity (Wall et al. 1993). Additionally, serial
casting has been effective in improving joint
range of motion when paired with botulinum
injections (Desloovere et al. 2001).
Children with cerebral palsy may be candidates
for surgical reconstruction to correct deformity
and/or improve function of their upper extremity.
Some children are candidates for tendon transfers
to optimize hand function. Common transfers are
those to improve wrist extension or to enhance
thumb extension and abduction. Children are typ-
ically immobilized in a cast for 4 weeks and are
then transitioned to an orthotic as they begin the
rehabilitation phase. The wearing schedule for the
orthotic following a tendon transfer is similar to
that described in the above Peripheral Nerve
Injury section.
Joint fusions, particularly in the wrist and/or
thumb, may be used to correct deformity
and/or maximize hand function. Additionally, ten-
don lengthening of the long nger and thumb
exors may be performed. With any of these
reconstructive surgeries, the child is transitioned
to a protective splint 46 weeks following surgery
or once the fusion displays adequate healing to
discontinue casting. The design of the splint
should provide support to any fused joints and
position any lengthened tendons into a stretch.
Splints are transitioned to a night wearing
157
schedule around 2 months following tendon
lengthening. If they are being used following
a joint fusion, then they are discontinued at
812 weeks following surgery.
Arthrogryposis
Conservative management of arthrogryposis
utilizes serial static orthotics to improve joint
mobility in the affected joints of the upper extrem-
ities, especially the elbows, wrists, and thumbs.
The use of orthotics should begin at as early of an
age as possible (Sells et al. 1996; Mennen
et al. 2005) with initiation of orthotics reported
as early as 7 days old (Kamil and Correia 1990).
Serial casting also may be used (Sells
et al. 1996; OFlaherty 2001; Smith and Drennan
2002) as an intervention to increase joint range
of motion in the upper extremity, although casting
is used less frequently than orthotics (Sells
et al. 1996). Children who have distal
arthrogryposis attain greater gains in wrist exten-
sion through serial casting as compared to chil-
dren with amyoplasia, the most common form of
arthrogryposis. Additionally, children with distal
arthrogryposis are less likely to have a recurrence
of their deformity after casting is discontinued as
compared to children with amyoplasia (Smith and
Drennan 2002).
Some children undergo surgical procedures
to facilitate improved joint passive and/or active
range of motion. Van Heest (Van Heest
158
et al. 1998) describes a postoperative protocol of
casting until 4 weeks following surgery followed
by wearing a sling until controlled elbow motion
is achieved, whereas Goldfarb et al. (2011) outline
a protocol for using postoperative orthotics. Their
protocol begins with the use of a static long arm
elbow starting at 2 weeks postoperatively and
worn at all times expect for performing ROM
exercises. At 4 weeks postoperative, static pro-
gressive elbow orthotics are initiated for intermit-
tent daytime wear, while the static orthotic is
continued at all other times. The child may be
gradually weaned from a daytime orthotic starting
around 6 weeks. Nighttime orthotics are often
continued until 6 months or longer (Goldfarb
et al. 2011). For procedures to improve wrist
extension, a wrist immobilization orthotic is
used for protection following removal of the post-
operative cast. The orthotic may be constructed to
have a removable clamshell and is worn at all
times except for performing exercises until
12 weeks following surgery. At this time, it is
continued for night wear until 6 months postoper-
ative (Goldfarb et al. 2011). Similarly, the postop-
erative protocol for surgical reconstruction of the
thumb-in-palm deformity includes fabrication of a
forearm-based thumb spica orthotic once the post-
operative cast is removed. The wearing schedule
is progressed the same as the wrist immobilization
orthotic (Goldfarb et al. 2011).
Muscular Dystrophy and Progressive
Neurologic Conditions
Children with muscular dystrophy or progressive
neurologic conditions are at risk for joint contrac-
tures when their disease process begins to involve
muscle weakness and limited active range of
motion in their upper extremities. Resting hand
orthotics are indicated for children with muscular
dystrophy whose disease process has reached to
the stage where extrinsic exor tightness of the
long nger exors is detected upon clinical exam
(Bushby et al. 2010). While a night wearing
schedule is ideal to provide a sustained stretch,
some children cannot tolerate wearing orthotics at
night as they have limitations in bed mobility and
J.M. Dorich and C. Shotwell
active upper extremity range of motion by the
time orthotics are often initiated. If this is the
case, the therapist collaborates with the child
and family to design a schedule for part-time
day wear of the orthotics. Often children will
prefer to alternate wearing a right and left
orthotic as opposed to wearing them both at the
same time. Finally, children and young adults
may be particular as to the exact position of
their hands in the orthotics, and careful attention
is necessary to achieve a t the child/young adult
can tolerate to facilitate adherence. Resting hand
orthotics fabricated from thermoplastic materials
or custom moldable neoprene orthotics with a
thermoplastic moldable layer are both options
for this patient population.
Use of orthotics for optimizing hand function
in children with muscular dystrophy has not been
described in the literature. This patient population
typically has complex medical concerns (Bushby
et al. 2010) accompanied with decreasing func-
tional independence by the time they are
experiencing limitations in upper extremity func-
tion. While orthotics may be an option to optimize
hand function, therapists should evaluate the
context in which functional orthotics are being
considered and the childs desire for orthotics
before initiating orthotics designed to enhance
upper extremity function (Wang et al. 2007).
Otherwise, the entire process will be futile and
counterproductive.
Trauma
Orthotics for trauma-related injuries are typically
prescribed by the referring practitioner. It is criti-
cal to understand the precautions associated with
the diagnosis and the preferred positioning.
Orthotics for trauma do require a critical thought
process and special attention to skin issues,
edema, and healing wounds. The initial orthotic
for a trauma is typically required for protection
and must be worn at all times, with the exception
of hygiene and guided therapy program (Skirven
et al. 2011). Subsequently, comfort and special
attention to pressure areas and skin problems is
important.
7 Orthotics and Casting 159

It is important to be respectful of childs pain

and the appearance of the hand. Sometimes, this is
the rst time the child has seen their hand since the
injury. It is not unusual for a child to be fearful or
tentative while the orthotic is being made.

Flexor Tendon Injuries

The postoperative protocols for exor tendon

injuries in children may differ depending on
the childs age. Young children are typically
immobilized in a cast for 6 weeks and typically
do not require orthotics. This protocol may also be
used when the clinician assesses the child to be at
high risk of noncompliance with the postoperative
precautions that must be followed when using an
orthotic for protection. School-aged children and
adolescents may be immobilized in a cast follow-
ing surgery for 4 weeks postsurgery and then
referred for an orthotic and treatment. Other
times, adolescent children are initially placed in
bulky dressing and then referred for a dorsal Fig. 16 A dorsal blocking splint is typically used to
protect exor tendon injuries after a period of cast immo-
blocking orthotic (Fig. 16) the following week.
bilization during the early stages of rehabilitation
The following orthotic recommendations are
based on an early mobilization program in a com-
pliant patient (Pettengill 2005). The joints should
be positioned as outlined in Table 5 (Cannon Table 5 Orthotics for flexor tendon injuries
et al. 2001; Skirven et al. 2011). Location of
Protective orthotics are continued until repair Orthotic
6 weeks postsurgery. Night extension orthotics Zones IV Dorsal blocking orthotic with
positioning as follows
may be used to increase range of motion if joint
Wrist approximately 20 exion
contractures are persistent.
MCP joints approximately 5070
exion
PIP and DIP joints full extension
Extensor Tendon Injuries FPL Dorsal blocking orthotic with
positioning as follows
When a child sustains an extensor tendon injury, it Thumb in palmar abduction
is common for the child to be placed in a cast ThumbMP 15 exion
immediately following surgery. The time spent Thumb IP 30 exion
in the cast varies among practitioners, the location Wrist 20 exion
of the injury, and the age of the child. Orthotics are
most often used following cast removal and in
conjunction with a postoperative rehabilitation (see Table 6; Purcell et al. 2000; Cannon
plan. However, occasionally the practitioner et al. 2001). The clinician may also consider
chooses to immobilize the extremity with an using dynamic or serial static orthotics as needed
orthotic alone. The orthotic for an extensor tendon if the child presents with persistent joint tightness
injury is dependent upon the zone of the injury following the healing process.
160
Table 6 Orthotics for extensor tendon injuries
Location of
repair Orthotic regimen
Zone 1 (mallet Volar orthotic immobilizing the DIP
deformity) joint in approximately 15
hyperextension
Conservative Because the nger may be very
management small, often it is benecial to secure
with exible self-adhesive wrap
rather than Velcro straps
Orthotic may need to be monitored
and adjusted if swelling is present
initially
Orthotic discharged at 810 weeks
Zone 2, 3, 4 Gutter orthotic with PIP and DIP
joints in full extension
Orthotic may need to be monitored
and adjusted if swelling is present
initially
Begin to wean from orthotic at 78
weeks
Zone 5 and 6 Wrist positioned in approximately
20 extension and MPs in
0 extension
Begin to wean from orthotic at 78
weeks
Zone 7 and 8 Wrist positioned in approximately
30 extension and MP joints in
0 extension
Wean from orthotic at 78 weeks
EPL Thumb spica with the IP included.
Wrist positioned in approximately
20 extension, thumb in palmar/
radial abduction with IP joint in
slight hyperextension
Wean from orthotic starting at
6 weeks
EPB and APL Thumb spica orthotic with the wrist
positioned in approximately 20
extension, thumb in palmar/radial
abduction
Wean from orthotic starting at
6 weeks
Amputations/Replantation
Replantation of an extremity requires immobili-
zation with casting initially (Cannon et al. 2001).
Orthotics will be indicated after early protective
casting is discontinued to assist with beginning
range of motion and protecting the extremity
J.M. Dorich and C. Shotwell
Table 7 Orthotics for upper extremity amputations
Level and type
of injury Orthotic regimen
Digital May require resting orthotic during
amputation healing phase, depending on referring
practitioners protocol
Digital Protective orthotic. Consider dorsal
replantation blocking orthotic as this will be
needed once ROM is initiated
Wrist in 20 exion
MCPs in 70 exion
6 weeks post-op
Check with referring practitioner to
discharge orthotic
May initiate extension resting
orthotic if tightness is present
Thumb Dorsal blocking orthotic with
replantation Wrist in neutral
Thumb in wide palmar abduction
with no tension on replanted
structures
MP and IP joints of thumb exed
to 15
6 weeks post-op
Check with referring practitioner to
discharge dorsal orthotic
Fabricate webspacer orthotic to be
worn at night to preserve motion
Hand Safe position orthotic. Consider a
replantation dorsal orthotic if possible
Wrist in neutral
MP joints in 70 exion
6 weeks post-op
Check with referring practitioner to
discharge dorsal orthotic
Consider night resting orthotic if
extrinsic exor tightness is present
Arm This is very rare in children
replantation Consider long arm resting orthotic
initially
while waiting for the nerve function to return
(Herbsman et al. 1966). Dynamic orthotics are
frequently recommended in the rehabilitation
phase to address joint tightness (Scheker
et al. 1995). It is suggested to review dynamic
splinting with the referring practitioner prior to
initiating it with the child. Table 7 outlines the
typical types of orthotics used based on the level
of amputation.
7 Orthotics and Casting 161

Table 8 Orthotics for upper extremity fractures Hand Infections

Type of fracture Orthotic regimen
Metacarpal fracture Ulnar gutter, radial gutter When a child has an infection in their upper
(Fig. 17), or resting hand, extremity, immobilization in an orthotic may be
dependent upon which used to provide pain relief and decrease edema.
metacarpal was fractured
(Kuokkanen et al. 1999)
This is particularly true with cases in which acute
Phalanx fracture Safe position orthotic with inammation is present. Orthotics should be
adjacent digit included designed to provide optimal positioning during
Consider ulnar gutter or radial soft tissue healing. For example, if the digits are
gutter orthotic depending on digit included in the splint, safe position of the digits is
fractured
Typically is discharged recommended to prevent MCP tightness. Orthot-
between 6 and 8 weeks ics should be short term and designed to immobi-
Distal phalanx, Protective nger gutter orthotic lize the joints indicated by the referring
Seymour fracture including middle and distal practitioner (Skirven et al. 2011).
phalanx
If it is an avulsion fracture, DIP
should be placed in mild
hyperextension Compartment Syndrome
Scaphoid fracture Thumb spica orthotic (Oskam
et al. 1996) Orthotics are indicated with compartment syn-
Distal radius Wrist cock-up orthotic (von drome due for immobilization following surgery
Keyserlingk et al. 2011) (Noonan and McCarthy 2010). In the case of a
Ulnar styloid Wrist cock-up orthotic
Volkmanns ischemic contracture, in which there
fracture
is often a nerve repair, an orthotic may be used to
protect the hand and limit deformity while waiting
Fractures for nerve function to return. In this case, resting
hand orthotics and safe position orthotics are often
Protocols for orthotic intervention with upper used. Lastly, serial static, static progressive, or
extremity fractures vary among practitioners and dynamic orthotics can be used to increase motion
institutions. Often the child is kept in a cast for the and decrease joint tightness once the period of
rst 46 weeks while the fracture is healing. Once early protective orthotic is discontinued. It is com-
the fracture site shows radiographic healing, the mon to alternate between wearing a dynamic
cast may be discontinued and the child transitioned orthotic during specic times during the day and
to a protective orthotic if tenderness persists a resting orthotic at night.
(JOINT 2006). Table 8 provides an overview of
the common orthotics used following discontinua-
tion of a cast when tenderness persists (Cannon Burns
et al. 2001). After a fracture has healed, orthotics
(serial static, static progressive, or dynamic) may The protocols for using orthotics in the treatment
be indicated to improve range of motion and of upper extremity burns in children can vary
resolve joint tightness when range of motion exer- among practitioners and institutions (Richard
cises alone are not achieving gains in joint mobility. et al. 1997). Orthotics are commonly used during
many phases of the healing process, but particu-
Dislocations larly when grafting has been performed. The
orthotic is often used to help stretch the skin or
Dislocations of the PIP joint are particularly com- graft. A skin graft or healing wound will contract
mon in children and adolescents who are athletes. until it meets an equal and opposing force, which
Table 9 includes the common orthotics used for is often obtained through orthotics (Madhuri and
dislocations of the PIP joint. Dhanraj 1998). The style of orthotic may vary
162
Fig. 17 A radial gutter
splint supports the wrist in
neutral to slight extension
and the index and middle
ngers in safe position
Table 9 Orthotic design for PIP dislocations
Type of
dislocation Orthotic style
Dorsal PIP Dorsal hand-based extension block of
PIP, allowing for active exion of PIP,
DIP, and MCP (Mcelfresh et al. 1972)
May consider gure of eight splint for
blocking
Volar PIP Finger gutter orthotic with DIP left free
depending on structures that need to be
immobilized for healing. Orthotics are commonly
made for the axillary area, elbow, and the hand
(Feldmann et al. 2008). Table 10 includes orthot-
ics that are specic to the hand.
Rheumatic Diseases
The use of orthotics was once a commonly used
intervention in children with rheumatic diseases to
help reduce pain and swelling as well as to limit
deformity-producing positions (Ouellette 1991).
Treatment for rheumatic diseases has changed
drastically over the past 10 years with the aggres-
sive use of biologic medication and disease-
modifying agents. Therefore, the need for upper
extremity orthotics has reduced dramatically.
When considering an orthotic, also consider
J.M. Dorich and C. Shotwell
whether the patient has started medications and
whether the medications have had ample time to
work (Adams et al. 2008). Often an orthotic is no
longer indicated once the medications are
working.
However, upper extremity orthotics are occa-
sionally used. The type of orthotic that is chosen
may depend on what stage the patient is in the
disease process (Forestier et al. 2009). During the
acute or active phase, an orthotic may be indicated
for pain relief, particularly if synovitis or tendon-
itis is present. It is not recommended to stretch the
soft tissue while the disease is active, as this may
increase the pain. Consequently, orthotics may
serve to rest tissues to minimize trauma and pain.
It is important for the child to use the orthotic in
conjunction with an exercise program prescribed
by the therapist as prolonged immobilization
can reduce motion and increase joint stiffness.
Consider that each childs activities and needs
are unique and the wearing schedule should be
tailored specically for each child (Callinan and
Mathiowetz 1996). A common choice is an
orthotic at night or during sleeping hours so as to
encourage active movement and not interfere with
daily activities. If the child has bilateral involve-
ment, consider alternating the orthotics at night to
avoid discomfort or impede independence.
During the later stages or inactive phase of
rheumatic disease, an orthotic may be indicated
7 Orthotics and Casting
Table 10 Orthotics for upper extremity burns
Phase of
healing Orthotic regimen
Acute or Orthotic is used to limit deformity,
emergent phase relieve pain; for protection while
healing
Pre-grafting Orthotic is indicated if range of
phase motion is reduced, typically a safe
position orthotic
With thumb involvement or a palmar
burn, thumb positioned in radial
abduction and extension
Skin grafting Safe position of orthotic immediately
phase after grafting. Often orthotics are
fabricated in the operating room
Orthotic can be removed after 5 days
Rehabilitation Orthotics are used to preserve range
phase of motion and oppose the force of the
contracting scar
MCPs should be positioned in
80 exion, providing maximal
stretch to collateral ligaments
Orthotics are typically static and
sometimes serially adjusted to gain
range of motion
Consider casting as an option to
correct scar contractures
Consider dynamic splinting for
joint tightness (Manigandan
et al. 2005)
for different reasons. For example, many children
will continue to have pain associated with residual
joint damage, tendonitis, or weakness once the
rheumatic disease is no longer active. In this
case, an orthotic (Table 11) may be indicated to
reduce pain during a particular activity such as
playing an instrument or performing household
chores. Additionally, during the inactive phase
of the disease, the soft tissue may be stretched to
help correct tightness or any deformities that may
have occurred during the active disease process.
Joint Hypermobility
The use of orthotics can be benecial for the child
with upper extremity joint hypermobility. Often
people with joint hypermobility demonstrate
decreased joint protection during their daily activ-
ities, which can lead to joint damage and pain
163
Table 11 Orthotics used with the rheumatic disease
Type of orthotic Therapeutic application
Resting hand Select if there is swelling/pain in the
hand and ngers
Recommend to be worn at night
Hand should rest in a comfortable
position with as little deviation as
possible
Wrist Used in active stages for pain
immobilization management and/or tenosynovitis
Indicated in all stages during daily
activities when there is pain,
instability, or weakness in the wrist,
particularly if it helps to improve
performance during functional
activities
Wrist should rest in a comfortable
position with slight extension and as
little deviation as possible
Oval-eight Used in inactive stages to help
(gure of eight) correct boutonniere and swan neck
deformities
Short opponens Used in active and inactive stages
for pain management
This can be a soft or hard orthotic.
Often there is better compliance
with soft orthotics (Callinan and
Mathiowetz 1996)
Indicated in all stages during daily
activities when there is pain,
instability, or weakness in the CMC
joint of the thumb, particularly if it
helps to improve performance
during functional activities
Finger gutter Used during inactive stages for a
limited time frame to help correct
soft tissue deformity in the digit
Elbow extension Used in inactive stages for a limited
time frame to help correct soft tissue
deformity at the elbow. Can be
serially adjusted to improve range of
motion
(Badia et al. 2005). Orthotics are indicated when
the child is unable to demonstrate joint protection
during an activity such as writing (Figs. 18 and
19) or playing an instrument or if the child needs
additional external support to complete the activ-
ity. Orthotics should enable the child to success-
fully complete an activity without pain while
providing more stability to the joint (Frohlich
et al. 2012). Typically, it is recommended for the
child to wear the orthotic during specic tasks or
164
when at risk for hyperextending the joint. Orthot-
ics should be used in conjunction with a strength-
ening program from the occupational therapist.
Table 12 includes common orthotics used in chil-
dren with hypermobility.
Patient and Family Education
Education: Purpose of the Orthotic
When therapists utilize upper extremity orthotics
as a component of a childs therapeutic plan of
care, thorough caregiver education is necessary to
Fig. 18 DIP hyperextension is characteristic of children
with joint hypermobility. This often lends to joint pain with
prolonged periods of writing
Fig. 19 Ring splints, such
as this Oval-8, can be used
to block joint
hyperextension
J.M. Dorich and C. Shotwell
achieve adherence to the wearing schedule, pre-
vent complications, and ensure the family is don-
ning and dofng the orthotic as it was designed to
be used. Therapists educate caregivers on the
treatment goals the orthotic is intended to achieve.
These can include one or more of the following:
Optimize function
Protect for healing
Decrease pain
Limit deformity
Increase range of motion
Compliance is likely to improve when the child
and/or family understands the role the orthotic has
in achieving treatment goals (Chen et al. 1999).
Prescribing Wearing Schedules
The wearing schedule for the orthotic varies
depending on the purpose of the orthotic. When
orthotics are being employed for protection
following an injury or surgery, the wearing sched-
ule is typically dictated by the treatment or
postsurgical rehabilitative protocol. Similarly,
orthotics worn to decrease inammation or pain
may have a wearing schedule prescribed by the
referring provider. In the acute phase of healing,
orthotics may be prescribed as continual wear.
7 Orthotics and Casting 165

Table 12 Upper extremity orthotics used for joint
hypermobility
Oval-8 orthotics, gure of Used to limit thumb IP, child and family identify an alternative wearing
eight-digit orthotics, nger PIP/DIP schedule. Care should be taken to limit the orthot-
silver ring hyperextension
Often used to limit
hyperextension during
writing, typing, ADLs, and
instrument play
Can help to limit swan neck
deformity when present
Soft thumb opponens Used to help support the
orthotic thumb during activities
Often used during writing
and while playing
instruments
MCP gure of eight Used to limit Generally, the wearing schedule for functional
orthotic hyperextension of MCPs
while typing or carrying
objects
Wrist immobilization Used to help provide
orthotic external support during
lifting activities or while
carrying objects such as a
band instrument
commonly recommended. If a child is unable to
tolerate night wear, then the therapist can help the
ics interference with function while maximizing
the childs time in the orthotic. When using
dynamic or static progressive orthotics, therapists
collaborate with the child and family to design a
wearing schedule that allows for enough time in
the orthotic to achieve improved joint mobility.
When orthotics are being employed to opti-
mize upper extremity function, the therapist con-
ducts a thorough patient and family interview to
guide recommendations for a wearing schedule.
orthotics is during activities in which the child
desires improved hand function. It can be helpful
for the therapist to observe the child engaged in
functional use with their orthotics on and off to
guide the child and family in recommendations of
which specic activities the orthotics will help to
provide improved hand function.

As inammation and pain begin to resolve, then

the therapy protocol often progresses to have the
child remove their orthotic for therapist-directed
range of motion exercises and light self-care activ-
ities. The child is increasingly weaned from their
orthotic until they are able to perform their daily
activities and desired roles with resolved or con-
trolled symptoms.
Orthotics that are used to preserve joint mobil-
ity or range of motion are often prescribed to be
worn when the child is sleeping (if tolerated by the
child) or not engaged in functional use of their
affected upper extremity. This is particularly true
when the style of orthotic, such as an elbow
extension orthotic or resting hand orthotic,
would limit the childs functional use of their
upper extremity. Orthotics that are being
employed to increase range of motion can vary
regarding wearing schedule. When serial casting,
the cast can be made to stay on until the next cast
in the series is going to be made or the cast can be
designed to be removable for a more exible
wearing schedule. Serial static orthotics are most
effective if worn 68 h (Tardieu et al. 1988) so
wearing the orthotic at night while sleeping is
Strategies to Achieve Adherence
with Wearing Orthotics
When the purpose of the orthotic allows for a
exible wearing schedule, adherence to the wear-
ing schedule can improve when the therapist col-
laborates with the family to identify a plan for
when the child will wear the orthotic. The thera-
pist may use guiding questions to help the child
and family identify how they may t a
recommended wearing schedule into the childs
schedule (DeGrace 2003).
The style of the orthotic may need to be
designed so that the child is unable to remove
the orthotic, depending on the childs develop-
mental stage. The design of straps or use of a
wrap over the orthotic can help to prevent the
child from removing the orthotic. Safety must be
considered with young children who may still be
mouthing items regularly or limited in the ability
to communicate discomfort in an orthotic. There-
fore, the design should be one which the child
cannot remove small pieces that could be choked
on. Additionally, when a wrap, such as a sock, is
166
used over an orthotic, the child should be closely
monitored the rst few days to ensure no adverse
effects such as compromised circulation.
Education on Weaning from
an Orthotic
When a child is wearing an orthotic for the pur-
pose of protection or rest for healing, the orthotic
is temporary, and as healing progresses, the child
will be weaned from their orthotic. Typically,
children are happy to be released from wearing
upper extremity orthotics and want to discontinue
wearing them as soon as they are directed to do
so. Yet, some children may be fearful of re-injury
or pain and have difculty discontinuing use of an
orthotic. In such cases, therapists can use guiding
questions to lead the child to develop a specic
plan for discontinuing use of the orthotic. For
example, the child might state they will try to
have the orthotic off when they are at home as a
rst step to weaning from the orthotic. Other
children may discontinue wearing the orthotic
completely and nd that their symptoms begin to
return afterwards. In such cases, the therapist may
need to guide the child in identifying specic
activities where the orthotic is still helpful in
managing pain or inammation as the child is
continuing to recover.
Education on Precautions
Children can experience adverse effects with
wearing orthotics and casts. Skin irritation,
compromised circulation, nerve compression, or
increased pain can occur as unintended conse-
quences of wearing orthotics and casts on the
upper extremity. A thorough review of the signs
of adverse reactions as well as how to manage
them if they occur is pertinent to patient and
family education with the use of upper extremity
orthotics and casts. Therapists should also educate
the family and child on care of the orthotic or cast.
Many casting materials cannot get wet and
would need to be removed if they become wet.
Additionally, if a small toy or item gets placed
J.M. Dorich and C. Shotwell
inside a cast, the cast should be removed so that
the skin can be inspected. If a cast breaks, such as
cracking or denting, a pressure area can result
from the change in the casts shape and removal
of the cast is recommended. With thermoplastic
orthotics, families and children must be educated
on how to clean the orthotic in a way that will not
cause skin irritation or negatively impact the
design of the orthotic. Use of a mild soap that
the family knows the child has not had adverse
reactions to in the past and lukewarm water is
recommended for cleaning orthotics. Addition-
ally, excessive moisture can lead to maceration
and compromised skin integrity. Family education
should be provided on drying the orthotic and the
skin thoroughly after washing the extremity
and/or orthotic to prevent skin maceration from
occurring while the child is wearing the orthotic.
Finally, thermoplastic materials are heat sensitive
so families must be educated that orthotics made
from thermoplastic materials must be kept out of
environments where the temperature exceeds
120 F to avoid heat damage to the orthotic.
Practice Donning and Doffing
Removable Orthotics and Casts
After the therapist ts a child with an orthotic or
removable cast, the child and/or family should
practice donning and dofng the orthotic/cast
with the care providers (Chen et al. 1999). This
will ensure that the child/family feel comfortable
with donning and dofng of the orthotic or cast as
well as how to place the extremity accurately
within the orthotic/cast. This allows the opportu-
nity for adjustments to be made to the design of
the orthotic/cast or further teaching to occur to
ensure the child/family can apply the orthotic/
cast easily and accurately.
Educational Supports
Therapists may use visual supports to assist in
educating care providers on the precautions that
should be followed with wearing orthotics/casts
and how to properly don and doff orthotics.
7 Orthotics and Casting
Fig. 20 Straps on splints may be marked to help care
providers know where straps should be placed
Straps can be marked to assist in matching the
strap placement up correctly using coloring cod-
ing on the straps and orthotics with permanent
markers (Fig. 20). Bivalve casts or clamshell
orthotics can also be marked with lines to match
up to ensure the top and bottom pieces are tting
together accurately (Fig. 21). Additionally, a mark
or label may be placed on an orthotic or cast that
coincides with an anatomical landmark to help
with aligning the orthotic/cast properly on the
upper extremity. Pictures or short video clips of
the donning/dofng procedure can be made as
guides for care providers to reference.
Frequently, children have multiple care pro-
viders. Sometimes the care providers who will
be managing the childs orthotic/cast are unable
to accompany the child to the therapy appoint-
ment for direct education. Additionally, families
may benet from materials that they may have to
review after the therapy visit regarding the pre-
cautions, prescribed wearing schedule, and
method for donning and dofng removable
orthotics/casts (Chen et al. 1999). Visual supports,
167
Fig. 21 Marks may be placed on bivalve serial casts to
help care providers with aligning the volar and dorsal
components of the casts for proper positioning
such as pictures with written explanation
(Katz et al. 2006) or video clips, may be used to
aid in providing education to care providers.
Printed educational materials may also be used.
Clinicians should provide contact information so
that families can contact them if an unintended
reaction occurs when wearing an orthotic or cast
on the upper extremity.
Summary
In the pediatric hand population, orthotics may be
used as an intervention as a part of the overall
treatment plan. Orthotics may serve the purpose to
optimize function, provide protection during
healing, rest the extremity for pain relief, limit
deformity, or increase range of motion when
joint deformity is present. The style and type of
orthotic used is widely linked to the purpose of the
orthotic. Additionally, factors such as the childs
support system, tolerance for splints, and overall
168
health status may inuence the design of the
orthotic. Clinicians must carefully asses each
child and consider any precautions noted by the
referring practitioner to determine the optimal
orthotic design and wearing schedule for each
individual child. Lastly, therapists should provide
thorough education to the child and caregivers
regarding the care and wearing schedule for the
orthotic.
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 Occupational Therapy Evaluation
and Treatment 8
Sarah Ashworth, Timothy Estilow, and Deborah Humpl

Contents Activities of Daily Living . . . . . . . . . . . . . . . . . . . . . . . . . . . 185

Introduction to Occupational Therapy
Evaluation and Treatment . . . . . . . . . . . . . . . . . . . . . . . . . 172
Documenting History and Background . . . . . . . . . . . 172
Establishing Rapport with the Child . . . . . . . . . . . . . . 173
Range of Motion Assessment . . . . . . . . . . . . . . . . . . . . . . 173
Strength . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
Observation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
Manual Muscle Testing (MMT) . . . . . . . . . . . . . . . . . . . . .
Myometry . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
Functional Strength Measurements . . . . . . . . . . . . . . . . . .
Transverse, Radial, and Ulnar Deciencies . . . . . . 185
Obstetrical Brachial Plexus Palsy . . . . . . . . . . . . . . . . . 186
Spinal Cord Injuries . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 187
Arthrogryposis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 189
Fracture Intervention . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 189
174
Physical Agent Modalities in Pediatrics . . . . . . . . . . 190
175
176 Compartment Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . 190
176
177 Peripheral Nerve Injuries (PNI) . . . . . . . . . . . . . . . . . . . 191

Muscle Tone . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 180 Duchenne Muscular Dystrophy (DMD) . . . . . . . . . . 192

Pain . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 180 Charcot-Marie-Tooth Disease (CMT) . . . . . . . . . . . . . 193

Evaluation of Sensation . . . . . . . . . . . . . . . . . . . . . . . . . . . . 180 Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 193

Purpose . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 181 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 194
Method . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 181
Sensibility . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 182
Intervention . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 182
Safety Awareness . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 182
Desensitization . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 183
Retraining . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 183
Edema . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 183
Fine Motor Skills . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 184

S. Ashworth (*)
Shriners Hospital for Children, Philadelphia, PA, USA
e-mail: sashworth@shrinenet.org
T. Estilow D. Humpl
The Childrens Hospital of Philadelphia, Philadelphia,
PA, USA
e-mail: estilow@email.chop.edu;
humpl@email.chop.edu

# Springer Science+Business Media New York 2015 171

J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_8
172
Abstract
There are many areas that encompass an occu-
pational therapy evaluation for an adult with
an upper extremity condition. Even though
the assessment and goals are similar, there
are many considerations when evaluating a
child. Assessment techniques, standardized
assessments, and treatment activities are
much different and will require modications
for a child. The physician and therapists
approach to a child needs creativity and
patience for optimal outcomes. The chapter
examines modications to a standard occupa-
tional therapy assessment for a child as well
as pediatric treatment ideas to maximize
outcomes. Common pediatric diagnoses are
also discussed.
Introduction to Occupational Therapy
Evaluation and Treatment
Hand therapy is a crucial part of rehabilitation
following an injury or surgical procedure. There
are well-established guidelines for the assess-
ment of the upper extremity; however, these
tests and measures can be challenging in the
pediatric population. Hand therapists and
surgeons are constantly redening protocols to
maximize functional benet for their patients.
These protocols are well supported in the litera-
ture and rely on simple commands for the patient
to follow. However, what happens when the adult
protocol following a procedure needs modica-
tion to meet the needs of a 3-year-old following a
similar operation? Simple commands such as
raise your arm as high as you can or do like
I do may become very challenging for that
3-year-old for a variety of reasons including the
following: the child may be fearful of this person
in a white coat, the child may be tired as a result
of waiting for an appointment that coincides with
nap time, or the child is just being deant. The
hand team treating a child has to be mindful of a
childs developmental level and utilize creativity
and empathy to maximize compliance for any
S. Ashworth et al.
therapy session. This chapter will outline assess-
ment and treatment regimens for many specialty
diagnoses seen in pediatrics.
Documenting History and Background
Next to the face, the hands are the second body
part that is seen upon greeting another person.
Hands communicate with gestures as well as
hold utensils for feeding, writing, and working.
When a child has a hand difference, he or she may
be shy or fearful to share and limb with the hand
team. In addition, these psychosocial issues may
be magnied in the teenage years when body
image and self-esteem are amplied and affect
peer relationships. The physician and therapist
should employ sensitivity when approaching the
child to build trust and rapport. In some situations,
the hand team may want to discuss with the family
ahead of time how much information and history
should be shared about the condition with the
child present in the room. The history section
should also address the childs goals, as children
can be very sensitive to their differences, and
blunt discussions with the parents without regard
for the child can hinder the dynamics of the
appointment and trust moving forward. It is
benecial if the therapist can offer busy games
to the child while retrieving the history from
the parents. Some good distractors for children
that will also provide clinical information on
the childs hand function may include
interlocking or form-type puzzles, Pegboard
games, and activities of daily living (ADL)
boards with latches or fasteners. When adults
are seeing a hand surgeon or therapist for the
rst time, they will usually act as an indepen-
dent historian at the appointment. In pediatrics,
the child may be accompanied by one or both
parents, grandparents, and even siblings. Each
person may offer a tidbit of information that is
vitally important in decision making for the
surgical or therapeutic plan. It is important for
the therapist and surgeon to acknowledge these
opinions, as it displays empathy and builds trust
in a potentially stressful setting for the child.
8 Occupational Therapy Evaluation and Treatment
Key tenets in documenting the history should
include birth history, developmental history,
any other diagnoses, detailed description of
the trauma or accident leading to the injury,
any other tests performed for orthopedic or
developmental issues, medications, allergies,
and even smoking history if applicable. Family
should relay any therapies the child is currently
receiving, related or unrelated to the visit. History
of hand dominance should be documented, as
some children may tend to alter dominance
based on lack of strength, increased fatigued,
altered sensibility, or visual scanning issues
with writing or eating. Children will quickly
adapt to using the nondominant hand as the
dominant hand in the event of an acquired
injury.
Establishing Rapport with the Child
When a child is visiting a clinic for the rst time,
he may exhibit unpredictable behavior. Often,
children who have multiple congenital anomalies
may see many specialists and may be fearful or
resistant to cooperate from the moment they enter
the hospital. Quickly establishing rapport may
help that child to move past their fears and comply
with directions. Singing silly songs while having
the child perform body motions may be effective
as there are many range of motion commands that
can follow in the song. For example, If you are
happy and you know it, clap your hands, touch
your shoulders, or wiggle your ngers. Using a
nger puppet can grossly check for range of
motion or if it is placed on the childs hand can
facilitate nger isolation. Blowing bubbles can
instantly create a relaxed and nonthreatening envi-
ronment for the patient, and usually the presence
of bubbles can motivate almost any child to reach
in desired planes and point with the desired hand.
Stickers can be randomly placed on areas of the
body for the child to locate. This is benecial to
encourage supination by placing a sticker in the
palm or to elicit external rotation by placing the
sticker on the ear. Children also like money, so a
few coins of different sizes that can be dropped
173
into a bottle can reveal intrinsic muscle strength
and hand preference. Non-latex balloons or even a
surgical glove blown up to mimic a balloon can
instantly get a child to move.
Range of Motion Assessment
Active and passive range of motion (AROM,
PROM) assessment is an essential component of
any upper extremity evaluation. Children can be
difcult to assess due to many factors including
limited attention span, motor development, ability
to follow instructions, fear, or pain. As with any
intervention, it is important to use age-appropriate
language and explain the activity. Beginning with
AROM prior to PROM measurements allows the
child to understand the task and builds trust
(Aaron 2006). Many children develop compensa-
tory movement patterns quickly after injury or
onset of limitations. The therapist or parent may
need to physically stabilize the childs trunk or
adjacent joints to isolate desired motion
(Ashworth and Kozin 2011).
When working with infants, hand measure-
ments may be difcult to obtain due to small
size, so AROM is best assessed through observa-
tion. Desired movements can be facilitated with
tactile stimulation, eliciting reex responses, or
encouraging reach for toys (Fig. 1). With unilat-
eral impairments, the unaffected extremity can
serve as a developmental norm to assess for
decits. Observation of movement should be
completed in different positions to see full
AROM. PROM measurements can be obtained
for the proximal joints and are important for con-
genital contractures to establish baseline for diag-
noses including arthrogryposis and longitudinal
deciencies.
Younger children are often more cooperative
if the assessment is presented as a game or chal-
lenge, such as Simon says or reaching for a
target. Allowing the child to touch the goniometer
and measure a parent or doll can decrease anx-
iety and improve participation. For children with
motor planning difculty, demonstrate the desired
motion. Using mirror movements of both arms
174
Fig. 1 Young child reaching for a toy to encourage active
reach during an assessment
can also help the child elicit the desired motion.
Hand AROM in this age group is often assessed
with observation. PROM in larger joints can be
obtained. Using distraction techniques, such as
music, toys, or videos, may allow the patient to
relax for a more accurate measurement without
co-contraction or resistance to being moved
(Ashworth and Kozin 2011).
Older children and adolescents are better able
to participate in formal goniometric measure-
ments. For younger school-aged children, a play-
ful approach may provide better rapport and
performance. The older children can better follow
directions. This age group can tolerate blocked
and composite motions to differentiate tight struc-
tures for thorough examination.
These techniques to obtain ROM assessment
are also utilized for treatment. Games, toys, and
crafts can elicit desired ROM with creative
positioning of the activity or child. To facilitate
shoulder external rotation and overhead reach, a
poster board can be taped to a vertical surface for a
painting project (Fig. 2). A child working on
opposition can be directed to pick up dice between
thumb and each nger, progressing to the ulnar
S. Ashworth et al.
Fig. 2 Young boy eliciting external rotation by painting
on a vertical surface
side with each turn. Many children have difculty
with stretching. Using distractions such as songs,
bubbles, or a favorite TV show can help the child
tolerate the intervention. Incorporating exercises
into daily life can be difcult for busy families.
Try to focus on the most important exercises and
educate the family on positioning and activities
that will incorporate the desired range of motion
into activities they are already completing.
Strength
Strength is a dynamic element in pediatrics due to
variations in childhood development. It differs
greatly between sexes and age groups and does
not follow a linear pattern, as there are large
increases in strength at various ages in addition
to relative differences between genders (Stam and
Hovius 2010). Strength can be a determining
factor in a childs ability to acquire new skills;
therefore, it is imperative to focus on strength
during the assessment and intervention of a pedi-
atric patient.
Strength, being the bodys ability to generate
force for a short duration, is highly dependent
upon effort. Getting maximal effort out of a
child relies on the assessors ability to ensure the
child feels comfortable, comprehends the direc-
tions, and is compliant with testing protocol.
These factors are necessary to capture valid and
reliable data. Simple strategies such as allowing
8 Occupational Therapy Evaluation and Treatment
the patient to hold, touch, or examine the testing
equipment; demonstrating the test on their par-
ents, siblings, etc.; or allowing them to administer
it to a family member are all good way to increase
their comfort level with the equipment and testing
methods. Decorating testing equipment with
funny faces, stickers, pictures, etc., can make the
device less threatening. Next, the communication
of expectations needs to be multimodal and appro-
priately worded. Verbal instructions should be
combined with visual modeling and hands on
demonstration, as children differ in their ability
to comprehend auditory, visual, and tactile input.
Language should be child appropriate and avoid
medical jargon. Instead of using the joint or muscle
to describe what you want the patient to do, ask
them to touch their nose and keep their nger
there for biceps testing, or ask them to try and
poke you to assess triceps strength. Prior to test-
ing, the patient should be positioned with proper
support for stability in order to isolate the targeted
muscle group. Supine is good for testing the
elbow joint, and seated with foot support for
shoulder, wrist, and hand. Finally, have them
explain what they are going to do and complete
a practice trial. With proper instructions, demon-
stration, and review, valid and reliable strength
testing can be performed on children as young as
3 years old.
Assessment of strength is an essential part of
the upper extremity evaluation and progress mon-
itoring and a key factor in guiding clinical deci-
sions. Strength testing can help identify the need
to develop antagonist muscle strength to counter-
act spasticity for improved range of motion in
children with spasticity, help determine what
may be causing functional limitations in patients
with neuromuscular disease, be performed preop-
eratively as a means to assist in selection of mus-
cles for tendon transfer, and provide essential
baseline data needed to appropriately develop an
intervention plan and set measurable goals.
As a progress monitoring tool, evaluation of
strength allows the clinician to upgrade, down-
grade, or modify provision of therapy based on
patient performance. It can measure the decit
remaining and indicate need for continued inter-
vention, provide a means to measure compliance
175
with home programs, and document the patients
return to premorbid status.
Finally, strength assessments can be used to
evaluate the effectiveness of clinical, surgical,
and pharmacological interventions as part of clin-
ical practice, clinical research, and natural history
studies to learn more about the disease process.
For an assessment protocol to be useful in the
clinical setting, it is important that it is specic to
the clinical characteristics of the disease and is
sufciently sensitive to detect change in muscle
performance using serial measurements. In addi-
tion, it is essential to target muscles that are rele-
vant to upper extremity functional performance. If
unfamiliar with a diagnosis, a literature review
should be completed by the clinician to determine
which muscle groups and joints are affected.
There are various methods to assess strength,
each having their own advantages as well as lim-
iting factors. In order to select the appropriate
method, a clinician has to consider the patient
population, purpose for testing as previously
discussed, and any limiting patient or environ-
mental factors. Strength can be accessed via
observation, hands on testing, and with the use
of standardized equipment or assessments.
Observation
In the case of the fragile infant or a noncompliant
patient due to age/cognitive/behavioral limita-
tions, one needs to be creative in their assessment.
Observation-based strength measurements can
include prone skills (Fig. 3), supine reach, transi-
tional movements, play abilities (Fig. 4), etc. For
example, wheelbarrow walking is a great way to
evaluate proximal shoulder girdle, pectoral, and
triceps strength as well as distal hand strength in
toddlers. While the child is having fun doing the
wheelbarrow, the clinician can observe for any
indication of weakness (collapsing at the elbow,
sting of the hands, turning of the hands in or out,
sliding or shufing of the hands to avoid single
arm weight bearing, etc.). An infants ability to
reach and sustain movement against gravity, push
up into prone or quadruped, and pull to sit
also offer valuable observations of strength.
176
Fig. 3 Prone strength assessment during play
Fig. 4 Strengthening the exors on a vertical swing
S. Ashworth et al.
Observation-based measures are advantageous
because they can be quick and easy, do not require
any specialty training or expensive equipment,
and can be suited to meet the needs of various
patient populations as described above. However,
observations are limited due to their subjective
nature and limited measurability.
Manual Muscle Testing (MMT)
The most common method of evaluating the
strength of separate muscle groups is the Medical
Research Council (MRC) scale (Medical
Research Council 1976), which is used for Man-
ual Muscle Testing (MMT). The scale covers a
range of strength from 0 (no contraction of the
muscle) to 5 (normal strength) in which the ability
to move against gravity and through the full range
of motion are important elements. The scale is a
simple, quick, cost effective, and standardized
towards measuring strength in children who are
able to follow basic instructions such as try to
touch your shoulder as hard as you can for elbow
exion and try to reach the sky as hard as you
can for elbow extension. However, the scale is
not sufciently sensitive to assess strength in very
weak muscles, when movement is possible only in
the gravity eliminated plane and in patients with
contracture, as full range is not possible. MMT is
best used as a quick screen to identify muscle
groups that require further testing with more
objective means of measurement.
Myometry
Similar to MMT, myometry is quick and easy to
administer and provides an objective, highly mea-
surable method of evaluating specic force pro-
duced at a joint.
Myometry has proven to be a highly reliable
and valid method of assessing strength in children
with neuromuscular conditions (Escolar
et al. 2001) and for specic muscle groups has
shown reliability as young as 2 years old (Rose
et al. 2008) . Myometry also helps to differentiate
strength within a MMT grade. For example,
8 Occupational Therapy Evaluation and Treatment
Fig. 5 Hand held myometry for evaluating elbow exion
strength
Van der Ploeg et al. compared the nonparametric
MRC scale with parametric handheld dynamom-
etry and concluded that an MRC grade 4 covers a
wide range of forces ranging from 10 to 250 N
(Van der Ploeg et al. 1984). Myometry is also a
good tool for evaluating patients with decreased
range of motion or contractures that do not allow
for typical MMT. In this case, a set position should
be chosen and documented to ensure serial testing
is accurate. This is especially helpful in evaluating
children during the recovery process in diagnosis
such as obstetric brachial plexus injury, peripheral
nerve injury, stroke, etc. There are many handheld
myometers on the market with a variety of options
and accessories. They can be mounted or hand-
held (Fig. 5), provide comparison of left/right
limbs with % difference, offer change variance
scores within each extremity, and offer storage
options for an entire assessment to eliminate the
need to manually record data after each individual
test. Measurement can be done using the make
or break method. For a make test, the exam- has proven to be effective and safe and provides
iner holds the myometer stable, and the subject
exerts a maximal force against the myometer for a
period of 35 s. For a break test, the subject
exerts a maximal force against the myometer, and
the examiner applies sufcient force to break the
hold. In children, it is especially important to
ensure that a maximal effort was given and the
data collected is valid and repeatable. If at least
two of the three repetitions recorded are not within
approximately 10 % of each other, the trial should
177
be repeated (Phillips et al. 2000). The myometer is
a very effective tool in measuring strength and has
few limitations; however, the nancial cost of the
device may be a limiting factor for small private
practices or individual practitioners.
Functional Strength Measurements
Functional scales should also be considered when
evaluating strength. They combine the functional
movement/task performance aspect of the
observation-based assessments with the measur-
able properties of MMT and myometry. Both
MMT and myometry focus on specic isolated
muscle strength, but do not allow for compensa-
tory movement strategies that children utilize to
perform ADLs. Assessments such as the Spinal
Muscle Atrophy (SMA) Upper Limb Module
(Mazzone et al. 2011) and Jebsen-Test of Hand
Function do capture range of motion and endur-
ance to some extent but also assess functional
strength of the upper extremity that is important.
Test items are generally specic to ADLs or
closely related and allow clinicians to interpret
how performance-based components such as
range of motion, dexterity, strength, and sensory
function impact task performance. Finally, evalu-
ation of specic tasks such as opening a sealed
water bottle and medicine bottle with child proof
caps, tearing open a bag of chips or a yogurt lid,
removing caps from markers, and pulling apart
toys offers good functional strength outcomes
for goal writing.
Intervention for Strength
Resistance training in children and adolescents
an effective way for enhancing motor perfor-
mance (Behringer et al. 2011). Unlike adults,
children often require creative approaches to
strengthening programs as traditional rote exer-
cises tend to be boring and often require focus
that may not be possible for younger children or
those with cognitive impairments/developmental
delays. Although the therapeutic approach
may have to be different, the same principle
apply in terms of using progressive resistance
178
Fig. 6 Prone weight bearing over ball for upper extremity
strengthening
exercises, except in the case of muscular dystro-
phies or other diagnoses where strengthening is
contraindicated. The key is to identify the mus-
cles/muscle groups that need to be addressed and
then create play-based activities that utilize those
muscles in a controlled, gradable, and measurable
fashion. Prone-based play on physioballs (Fig. 6),
swings, wedges, and other prone positions is a
great way to target the chest, anterior deltoid,
triceps, and distal hands. The activity can easily
be graded by varying the level of support and
angle of the body and by adding additional resis-
tance via resistive bands/weights/etc., to the
patient. Resistive pulling activities (tug of war,
climbing, rowing, swings, and scooter play) are
fun ways to target the latissimus, rhomboids,
biceps, and distal hands as well. Younger children
can participate by imitating animal walks, yoga
poses, and playing gaming systems such as Wii
and Xbox Kinect with simultaneous resistance
applied to the desired movement. The Wii Remote
controller can be secured with Fabrifoam to
S. Ashworth et al.
Fig. 7 Child with hemiplegia has a Wii Remote controller
secured with Fabrifoam on the forearm to encourage
shoulder and elbow range of motion while playing a
game (Courtesy of the Childrens Hospital of Philadelphia)
the forearm to enhance shoulder and elbow
movement (Fig. 7).
Consideration of baseline strength and motor
control is necessary to develop the most effective
treatment plan. The ability to actively move a limb
is dependent on many factors that should be care-
fully assessed to determine the appropriate inter-
ventions. Motor control can be impacted due to
traumatic brain injury (TBI), spasticity, pediatric
stroke, etc., which may limit the ability to activate
and recruit appropriate muscles for movement or
relax the antagonist group. In this situation, cou-
pling strengthening activities with biofeedback is
a good way to improve motor control for more
successful strengthening. In the case of peripheral
nerve injury, brachial plexus injury, SMA, and
other neuromuscular disorders, baseline strength
can be substantially impaired to the point where
gravity alone is a barrier to movement. In these
cases, gravity eliminated or reduced movement
planes are ideal for initiating strengthening.
Aquatic sessions can be extremely benecial, as
the buoyancy of the water reduces the impact of
gravity making it easier to move the limb, and the
water can also be used to create assistance or
resistance to movement as needed. Devices that
decrease workload and/or accentuate movement
8 Occupational Therapy Evaluation and Treatment
(Wilmington Robotic Exoskeleton, counterbal-
ance arm slings, Saebo mobile arm support)
allow for initial strengthening to occur where it
would otherwise be impossible. Computer-based
software like the Biometrics E-Link Evaluation
and Exercise System can be used to target strength
Fig. 8 Child positioned at Biometrics work station, mov-
ing a knob that is linked to a game on the monitor for
desired wrist range of motion (Courtesy of the Childrens
Hospital of Philadelphia)
Fig. 9 TRX suspension
push-up
179
in small muscles for cases like nger fractures
where isolated strengthening may be limited
with traditional equipment. Additionally, the
Biometrics offers a surface EMG sensor that
can provide feedback for isolated muscle
activation (Fig. 8).
For athletes and patients able to complete
higher-level strengthening, progressive resistance
exercises (PREs) can be performed with dumb-
bells, resistance bands, and other traditional
strengthening tools. The use of body weight train-
ing with suspension systems like TRX (total body
resistance exercise) (Fig. 9) can provide an excel-
lent opportunity to work on joint stability, exi-
bility, and strength with very little space and
equipment required. Resistance training programs
for youth should follow a training model with a
progressive and systematic variation in exercise
selection, intensity, volume, frequency, and repe-
tition velocity to enhance training adaptations,
reduce boredom, and decrease the risk of overuse
injuries (Plisk and Stone 2003). In addition, pro-
grams need to be tailored to the individual based
on their past exercise experience, motor control,
and physical limitations. Specic exercise volume
(sets and repetitions) and resistance levels (based
off % of 1 rep max) can be found in the Position
statement on youth resistance training: the 2014
International Consensus. Finally, strength goals
should be linked to specic functional impair-
ments and discussed with patients in order to
180
Table 1 Modified Ashworth Scale (MAS) for grading
spasticity
Grade Description
0 No increase in muscle tone
1 Slight increase in muscle tone, manifested by a
catch and release or by minimal resistance at
the end of the range of motion when the
affected part(s) is moved in exion or extension
1+ Slight increase in muscle tone, manifested by a
catch, followed by minimal resistance
throughout the remainder (less than half) of the
ROM
2 More marked increase in muscle tone through
most of the ROM but affected part(s) easily
moved
3 Considerable increase in muscle tone, passive
movement difcult
4 Affected part(s) rigid in exion or extension
illustrate the benet and importance of completion
of exercises to restore independence or maximize
athletic performance. Motivational tools such as
charts, graphs, apps, etc., can help to motivate
patients as well as provide a way to monitor pro-
gress and response to adaptations to the training
program.
Muscle Tone
Children who have the diagnoses of cerebral
palsy, traumatic brain injury, high spinal cord
injuries, pediatric stroke, or an unexplained hyp-
oxic ischemic event may exhibit spasticity in one
to four extremities. Tone is assessed by a quick
range of motion stretch to a joint and can be
measured using the Modied Ashworth Scale
(MAS). It is a six-point scale with numbers rang-
ing from 0 to 4 and may be useful in guiding
outcomes posttreatment (Table 1). The use of the
MAS becomes increasingly important for surgical
options, and it is very useful to determine effects
of pharmacological therapies such as Botulinum
toxin or baclofen pump placement. Therapists
play a crucial role in providing information on
muscle tone to the physicians, as the child may
be nervous in the physician appointment resulting
in increased spasticity compared to baseline.
Also, the surgeon may only briey test AROM
S. Ashworth et al.
of the joint one or two times versus the therapist
who challenges the child by making them exercise
the extremity repeatedly causing fatigue and more
spasticity over time. The child may also exhibit fair
wrist extension with thumb extension with request
from a physician but when that child is challenged
to hold a 4 diameter jar for opening may exhibit
dynamic tone while trying to complete the task. It is
important for the therapist to document these dif-
ferences and relay the ndings to the physician for
the most comprehensive plan for the child. Finally,
video can be an extremely helpful tool to document
quality of movement and progress in therapy and to
provide the physician with a clinical picture of the
patient prior to their appointment.
Pain
A pain assessment is important, as it allows the
clinician to monitor how the child feels at rest,
with movement, during and after stretching, and
during activities of daily living. Pain can hinder
participation, as children are inherently fearful
when they anticipate pain, and this anxiety can
start before getting to the hospital. Cognition and
developmental levels of the child are considered
when assessing the child for pain. There are
numerous pain scales that are uniform across a
healthcare facility to ensure consistency of grad-
ing. It is important for the clinician to document
the timing of the pain, such as before the activity
begins, during the activity, and/or after the activ-
ity. If a heat modality is used prior to an activity,
the therapist should document pain scores pre and
post the modality application. Documenting pain
is also useful in formulating functional goals for
progressing the child in therapy.
Evaluation of Sensation
Evaluation of sensory function is important as our
bodies require a collaborative effort between our
sensory receptors to provide information on pres-
sure, joint position, pain, and temperature to the
brain. Subsequently, the motor neurons respond
accordingly in carrying out the appropriate
8 Occupational Therapy Evaluation and Treatment 181

reaction. The two work synergistically to ensure

smooth coordinated movements of the hand and
upper extremity. A disturbance in sensory func-
tion will impact ne motor skills regardless of
intact motor function. Individuals with impaired
somatosensory feedback have difculty grading
ngertip forces used to grasp and lift objects,
resulting in insufcient nger opening, object
grasp at incorrect contact points, slips at object
contact, temporal delays, and the use of excessive
ngertip forces during ne motor tasks. This is
crucial in pediatrics, as young children are contin-
ually developing their sensorimotor feedback sys-
tem, and altered sensibility will impact skill
acquisition. Sensation can be very difcult to
describe; therefore, the use of keywords (hot/cold,
sharp/dull, rough/smooth, etc.) or comparisons to
tangible objects (soft like a teddy bear, sharp like a
needle, cold like ice cube, etc.) can be helpful in
establishing terminology for children during the
sensory evaluation. Similar to the assessment of
strength, it is necessary to develop trust with the
child and comfort with the testing tools, especially Fig. 10 Functional Dexterity Test
when using sharp instruments (paper clip, dis-
criminating disk, etc.). Finally, it is necessary to
pay close attention to be sure the child is not using descriptions should include location, distribution,
their vision to compensate during the assessment. and extent of sensory impairment. Passive sensi-
A blindfold, parents hand, or piece of paper in bility can be tested using the Semmes-Weinstein
front of the face may be necessary to ensure test- monolaments or the WEST for touch pressure
ing is valid. and the Discriminator for static and moving
two-point discrimination. Active sensibility can
be tested using the Moberg picking-up test or
Purpose general tests of tactile gnosis or stereognosis.
Positional sense can be completed by placing the
Sensory function requires a thorough evaluation. limb in space and asking the child to mimic the
Specic sensory impairments should be identied posture with the opposing limb or moving a nger
along with their functional implications. For in space and asking them whether it is up or
example, can the child feel the opening of the down. Dexterity measures such as the Functional
shirt to button, maintain grasp on a zipper, and Dexterity Test (FDT) (Fig. 10) and 9-Hole Peg
provide ample force to hold a cup without Test (Fig. 11) can also measure impact on hand
dropping it? function due to poor sensibility. The ORiains
Wrinkle Test is an easy way to check for denerva-
tion following a peripheral nerve injury. The ther-
Method apist places the childs hand in warm water
(104  F) for 30 min. The hand is checked for
Sensory assessment should be methodical and wrinkling or pruning consistent with intact sensi-
comprehensive. Both large and small ber nerves bility. The absence of wrinkling infers the absence
need to be tested in a quantitative way, and of nerve supply.
182
Fig. 11 Nine-Hole Peg Test
To ensure children comprehend testing proce-
dure, it is advantageous to perform exaggerated
trials at rst. For example, during two-point dis-
crimination testing, you should start with the
points extremely far apart, or for light touch, a
thicker monolament should be used with a
slow progression over time to a ner one. During
positional sense, use large quick movements
progressing to very slow subtle ones. Also, mak-
ing it fun is a good way to keep the childs interest.
Telling them you are playing detective and they
have to solve the mystery and pretending you are
checking them for superpowers to see if they can
gure out what things are without using their eyes
(stereognosis testing) are useful tactics.
Sensibility
Due to their age/immaturity, children may provide
several challenges during treatment, as therapeu-
tic interventions for sensory impairment require a
high degree of focus and patience. Children often
S. Ashworth et al.
disregard their insensate part. Therefore, it is
imperative to use multimodal interventions to
encourage usage. A child may not want to focus
on their loss of a particular skill, but shifting the
focus to an area of strength can be an empowering
approach to therapy. For example, allowing the
child to use their visual sense to accommodate
for peripheral sensory impairments during tasks
allows them to gain a degree of control over their
rehabilitation and helps reinforce the therapeutic
plan while working to improve performance.
Intervention
Intervention for sensory impairments varies
greatly depending on the clinical presentation
and stage of recovery. The intervention may be
solely for protection (to prevent further injury in
the case of a severe denervation injury or progres-
sive neuropathy, spinal cord injury, etc.) or can
be an active process utilizing a multisensory
approach to foster hand function as reinnervation
occurs. Regardless of the purpose, it is essential to
imbed play in all sensory interventions to make
them fun and to maintain the childs interest and
ultimately compliance with the sensory program.
Safety Awareness
In general, children have a decreased level of
safety awareness, and impaired sensibility poses
a further risk for accident/injury. A review of the
childs daily activities and tasks is performed to
identify potential safety concerns. For example, a
child with peripheral neuropathy may be at risk
for burning or cutting the hand during meal prep-
aration or injure the hand during constructive
tasks, such as wood/metal work. Also, proper
clothing (mittens, gloves, etc.) should be
recommended to prevent injury from the cold
weather. Role-playing can be benecial to rein-
force safety awareness, as it gives the therapist an
opportunity to simulate unsafe situations them-
selves or with dolls/gurines and the child can
correct the therapist by demonstrating proper
safety. Finally, objects can be adapted by
8 Occupational Therapy Evaluation and Treatment
providing texture (Dycem, double-sided tape,
rubber grips, etc.) to provide increased sensory
input to prevent slippage or can be built up (with
foam/rubber handle, adapted utensils, etc.) to
increase the surface area for better holding.
Desensitization
A progressive desensitization program can be
used to treat neuropathic pain. Sensory input
should start with the child in control of the amount
of pressure, duration of stimulation, and the mate-
rial used. Playing treasure hunt is a good way to
expose the hand to various sensory stimuli in a
playful manner. The therapist can hide toys in a
plastic bin of beans, rice, sand, or shaving cream,
and the child has to search for them. For scar
management, a vibrating animal massager can be
used to provide direct pressure over the scar. The
use of distraction techniques should be employed
when it is necessary for the therapist to provide the
desensitization. Watching television, listening to
music, and playing games can all aid in the accep-
tance of desensitization. Finally, a visual timer,
such as the Time Timer , can be used to provide
concrete visual feedback as to when the desensi-
tization will be done. The temperature, texture,
and amount of force used should be sufcient to
create an unpleasant feel for the patient, and the
intensity should be increased slowly over time.
Retraining
As reinnervation occurs, sensory reeducation is
initiated to rene cortical receptive elds with a
higher sensory resolution, improve tendency
towards reversal and normalization of the
distorted hand map, improve processing in the
sensory network at a higher cortical level, and
facilitate interpretation of the distorted hand
map. A multisensory approach facilitates recov-
ery by allowing children to utilize all of their
senses during reeducation. Traditional sensory
reeducation activities can be modied to become
fun learning games. For example, nger paints
can be used to work on touch localization
183
(the child closes their eyes, therapist dabs a
ngerprint on the child, the child then attempts
to dab the same spot) as the paint allows for visual
feedback to help with auto-correction. Tactile
gnosis games should be played with familiar
toys and objects, as they already have established
cortical representations of these objects from pre-
vious use. A mirror can be used in therapy for
mirror visual feedback (MVF) (Ramachandran
and Altschuler 2009). The child utilizes a mirror
box placed in front of them to complete a specied
movement with the uninvolved extremity while
observing the mirror located at midline. The
visual system interprets what is thought to be
movement of the opposing extremity as seen in
the mirror image and stimulates mirror neurons in
the brain potentially helping to relieve pain,
restore volitional control of an affected limb, and
increase functional movement. Biofeedback can
also be extremely helpful to facilitate motor learn-
ing and reinforce movement patterns during reed-
ucation. The auditory feedback combined with
visual feedback is an excellent way to elicit iso-
lated motor control and reinforce recruitment and
activation of the proper muscles. The Biometrics
is a computer-based rehab tool that allows patients
to play video games while utilizing various
methods of control (myoelectric, range of motion,
resistive, etc.) and provides consistent feedback
during the activity to reinforce learning. Finally, in
addition to impairment-based interventions, ses-
sions should include retraining of sensory func-
tion via completion of ADLs to ensure the skills
are generalized for improved ADL performance.
Edema
Edema is common following an injury or after
surgery. Edema may prevent the hand from mov-
ing, result in decrease sensation in the hand, and
may also be symptomatic of an underlying circu-
latory issue. Circumferential tape measurements
are the most practical way to measure edema on a
pediatric hand or digit. The measurement is usu-
ally recorded in centimeters and compared to the
contralateral limb. Documentation of the edema
should include exactly where the tape measure
184
was applied with reference points of distance to
creases, joints, or bony landmarks.
Retrograde massage, positioning, and the use
of compression wraps, tapes, or garments are use-
ful options include Coban wraps, Ace wraps, and
Tubigrip. Massage can be demonstrated on dolls
or a stuffed animal, and the patient can practice on
the therapist to demonstrate competence.
Fine Motor Skills
Assessment of ne motor skills in children should
include the following: reach, object acquisition,
manipulation, release, and bimanual hand use.
Accuracy and speed are often used as parameters
for evaluation (Aaron 2006). Many factors may
limit ne motor control such as muscle weakness,
AROM/PROM limitation, diminished sensibility,
central nervous system impairment, anatomic
differences, and pain. Consideration of typical
development of hand skills is important when
evaluating infants and children. These skills can
be assessed with observation of functional hand
use and a variety of standardized tools.
Standardized tests of hand function are useful
for establishing baseline prior to surgical or ther-
apeutic intervention, for identifying impairments,
and for monitoring progress. Many of the standard
adult hand assessments can be used with children
and adolescents. If pediatric norms have not been
established, it is still be useful to track individual
changes over time. The Box and Blocks Test
(Fig. 12) and 9-Hole Peg Test are quick exams to
assess grasp and release. They both have pediatric
norms for 6- to 19-year-olds and 4- to 19-year-
olds, respectively (Mathiowetz et al. 1985; Poole
et al. 2005). Video exams are valuable to compare
performance over time and allow for more in
depth review of non-scored areas. Manipulation
assessments, such as the Minnesota Rate of
Manipulation Test or the Functional Dexterity
Test (FDT), allow for greater review of hand
object manipulation. The FDT is standardized
for children as young as 3 years old (Aaron and
Jansen 2003; Aaron 2006).
Pediatric hand function assessments often take
more time to administer but have been developed
S. Ashworth et al.
Fig. 12 Box and Blocks Test
specically for children. The Assisting Hand
Assessment (AHA) was developed to assess func-
tion and outcomes for interventions for unilater-
ally impaired children aged 18 months to 12 years.
These children may have a ceiling effect with
functional or developmental tests due to the
unimpaired upper extremity compensating for
the affected limb. The AHA is most commonly
used with children with hemiplegic cerebral palsy
or brachial plexus injuries and involves a video-
recorded semistructured play session with scoring
after the session. Formal training is required and
costly (Krumlinde-Sundholm et al. 2007). The
Shriners Hospital for Children Upper Extremity
Evaluation (SHUEE) is a tool specic to cerebral
palsy designed to evaluate the potential for
improved hand function with changes to proximal
joints. The examination is videotaped and scored
after the session. It allows the surgeon and thera-
pist to have a standard approach to hand surgery
for children with cerebral palsy and assess bene-
ts of procedure such as tendon transfers or
arthrodesis (Davids et al. 2006).
To assess or treat a child with ne motor de-
cits, it is important to review typical development
of hand skills. These skills change rapidly
during the rst years of life. Newborns posture
primarily with sted ngers around the thumbs
with occasional reexive extension. The grasp
reex emerges by 1 month and diminishes by
45 months. This reex causes the infant to
grasp in response to pressure stimulus in the hand.
8 Occupational Therapy Evaluation and Treatment
Volitional grasp emerges between 4 and 7 months
and begins with ulnar palmar grasp. Grasp shifts
to the radial side of the hand beginning to incor-
porate the thumb in radial palmar grasp. As the
child develops more control, the grasp moves
away from the palm (radial digital) and ulti-
mately away from proximal support of the
forearm (superior pincer) by the rst birthday.
Voluntary release begins from 7 to 10 months.
Transferring objects from hand to hand emerges
at 46 months. When facilitating grasp, release
or manipulation skills using a variety of sensa-
tions can keep an infant interested. For new-
borns, gentle tactile stroking along the dorsum
of the hand and ngers can be utilized to elicit
reexive extension to assess nerve function. Pro-
viding toys with auditory and visual sensations
or different textures may engage the child. Once
children have been introduced to solids, puff cereals
or other parent-approved snacks are an excellent
tool to assess small item grasp (Case-Smith 2006;
Pehoski 2006).
Into the toddler years, the child develops better
control of release and hand manipulation of
objects. By 3 years, children begin to develop
simultaneous but different bimanual skills,
required for stringing beads or cutting with scis-
sors while controlling the paper. A toddler can be
given small blocks to stack to assess targeting and
release. Presenting coins and a piggy bank allows
for observation of digital grasp and release
(Pehoski 2006). Preschoolers to early school-
aged children continue to rene their skills and
speed with hand functions. A component of skill
acquisition is determining appropriate force for
grip. During this time, children begin developing
writing skills. By 67 years, dynamic tripod grasp
on writing implements is present (Ziviani and
Wallen 2006). Observation of coloring and activ-
ities requiring in hand object manipulation are
appropriate for this age range. Providing a variety
of different sized, textured, and weighted objects
can be used to encourage differentiation of
grip force.
For older children and adolescents, observa-
tion of ne motor play or self-care tasks provides
insight into hand skills in addition to the standard-
ized measures above. Observe opening and
185
closing a variety of fasteners, writing, utensil use
for eating, or tool use for work/play. Hiding small
objects in putty for a scavenger hunt can show
nger isolation, grasp patterns, hand manipula-
tion, and strength decits.
Activities of Daily Living
ADLs are described as any activity that allows an
individual to function in his daily routine includ-
ing self-care, chores, work duties, and leisure
skills. For a baby, it may be holding a bottle with
two hands. For a preschooler, it may be getting
dressed and zipping a coat, and for an adolescent it
may be using a cell phone to text message friends.
Assessing ADLs gives the team a better clinical
picture of goals for therapy and future surgical
considerations. It can also help the clinician look
at the childs quality of movement when complet-
ing a task. Even though a caregivers report is
helpful in the assessment that report may yield a
much different clinical picture than the childs
actual abilities. There are many standardized
ADL assessments for pediatrics and adults includ-
ing the Pediatric Evaluation of Disability Inven-
tory (PEDI) for ages 6 months to 7 years, the
Hawaii Early Learning Prole (HELP) Checklists
for ages 03 and 36, and the Disabilities of the
Arm, Shoulder, and Hand Measure for older ado-
lescents. Clinical observations of ADL skills can
reveal a childs bilateral coordination, quality of
performance, and compensatory techniques.
A costume box can be engaging for a child to
assess the activity of dressing. Fastener boards
that have various buttons, zippers, and shoe
laces are also effective. Small individual snack
bags and sealed water bottles can reveal func-
tional pinch and hand strength with opening.
Transverse, Radial, and Ulnar
Deficiencies
Transverse deciencies can occur at any level in
the upper extremity. The most common level is
below elbow (BE) at the proximal third of the
forearm (Fig. 13; Moran and Tomhave 2011).
186
Fig. 13 Short congenital below elbow transverse de-
ciency with nubbins present along distal residual limb
(Courtesy of Shriners Hospital for Children, Philadelphia)
In a 2006 multicenter study performed at ten
Shriners Hospitals for Children, adolescents with
congenital BE amputations were assessed com-
pleting activities with and without prostheses.
The study found the subjects performed the same
or better without prostheses for test activities.
Many reported they primarily used the prosthesis
for specic tasks (i.e., sports) or appearance.
While prosthetic options and training are impor-
tant, interventions should also include adaptive
techniques to maximize independence with the
residual limb (James et al. 2006).
Radial and ulnar deciencies vary in severity
from mild wrist angulation to involvement of
digits, wrist, forearm, and elbow (Fig. 14). Early
therapy focuses on splinting to correct or prevent
progression of deformity and initiating stretching
program. Children with radial deciency often
have absent or hypoplastic thumbs and develop
scissor pinching prehension patterns. Scissoring
may occur between the index and long ngers and
between the ring and small ngers. The assess-
ment must include and inventory of which digits
are preferred and have best quality of movement.
The quality of the index nger motion prior to
surgery correlates with the function after
S. Ashworth et al.
Fig. 14 Young male with bilateral radial deciency and
bilateral hypoplastic thumbs demonstrating expanded web
space between index and long digits (Courtesy of Shriners
Hospital for Children, Philadelphia)
pollicization (Kozin et al. 1992). For pollicization
candidates, encouragement of index and long n-
ger scissor pinching prior to surgery assists with
postoperative retraining. Buddy taping the long
and ring ngers together can facilitate this desired
pinch pattern for small object play such as bead-
ing. If the child has limited elbow exion, they
may require the wrist deviation for hand to mouth
ADL. This lack of elbow exion is a relative
contraindication to wrist centralization. A trial of
splints to simulate position of wrist after surgery
can promote elbow exion and provide an oppor-
tunity to assess potential functional loss. Children
with ulnar deciency may be missing digits
including the thumb. In addition, elbow function
is highly variable depending on the individual
anatomy (Fig. 15). Children with radial or ulnar
deciencies may need adaptive equipment or
techniques for specic activities as they grow.
These often consist of long handled ADL equip-
ment, toileting aides, or uniquely designed equip-
ment based on the childs and family goals (Kozin
2003).
Obstetrical Brachial Plexus Palsy
Children with obstetrical brachial plexus palsies
(OBPP) can vary greatly in their upper extremity
function depending on the number of nerve
roots injured and extent and location of damage.
The therapists assessments aid in tracking
8 Occupational Therapy Evaluation and Treatment
Fig. 15 Six-year-old male with left ulnar deciency and
radioulnar synostosis (Courtesy of Shriners Hospital for
Children, Philadelphia)
neurological recovery over time. The Active
Movement Scale (AMS) and Toronto Scale are
specic tools for use with infants (Bae et al. 2003;
Curtis et al. 2002). These observation scales
assess movement with gravity minimized or
against gravity to measure strength in specic
movements. The Modied Mallet Classication
tool is used with older children who can follow
directions to primarily assess shoulder move-
ments (Fig. 16; Bae et al. 2003).
Frequent PROM is critical for maintaining
joint ROM, particularly in joints with unbalanced
motor function. For example, children with a C5
and C6 injury will have full internal rotation
(IR) strength and lack external rotation (ER).
The shoulder can quickly develop an IR contrac-
ture. Early external rotation PROM with manual
scapular stabilization is important to prevent
187
posterior humeral head subluxation from a
glenohumeral IR contracture. In addition to
stretching program, some children may be appro-
priate for early splinting about the upper extremity
(Ashworth and Kozin 2011). A dorsal wrist cock-
up splint can help prevent wrist drop. A supinator
strap can help to position the forearm in supina-
tion and prevent a forearm contracture.
Intervention focuses on facilitating
age-appropriate developmental movements and
play. Weight bearing is encouraged for strength-
ening throughout the arm and shoulder girdle.
Close observation of compensatory movement
patterns is important. Early incorporation of
trunk stabilization decrease compensatory move-
ments can help before they develop and become
difcult to relearn. See Chapters Neonatal
Brachial Plexus Palsy, Microsurgery for
Obstetrical Brachial Plexus Palsy,
Glenohumeral Joint Secondary Procedures
for Obstetrical Brachial Plexus Birth Palsy, and
Secondary Procedures About the Elbow, Fore-
arm, Wrist, and Hand, for details on medical
management and surgical intervention for obstet-
rical brachial plexus palsies.
Spinal Cord Injuries
Children with spinal cord injuries (SCI) present
with unique upper extremity challenges. After
SCI, increased upper extremity weight-bearing
activities such as wheelchair propulsion, transfers,
bed mobility, and upright mobility put all patients
with SCI at risk of overuse injuries and joint pain.
Early focusing on good biomechanics, proper
seating position, and exercise program in rehabil-
itation can establish good habits for joint
preservation.
Cervical spinal cord injuries are especially
devastating. Upper extremity goals include
maintaining full range of motion, maximizing
strength of innervated muscles, and regaining
function. Controversy exists over splinting a posi-
tion to encourage tightening of the long digit
exor tendons for tenodesis function versus
splinting to maintain full passive movement. We
prefer to maintain supple ngers and reserve
188 S. Ashworth et al.

Modification Mallet classification

(grade I = no function, Grade V = normal function)
Grade I Grade II Grade III Grade IV Grade V

Global Not No
Normal
abduction testable function

<30 30 to 90 >90

Global external Not No Normal

rotation testable function <0 0 to 20 >20

Not No
Hand to neck Normal
testable function
Not possible Difficult Easy

Hand on spine Not No Normal

testable function

Not possible S1 T12

Not No
Hand to mouth Normal
testable function
Marked Partial <40 of
trumpet sign trumpet sign abduction

Not No
Internal rotation
testable function
Cannot Can touch with Palm on bolly,
touch wrist flexion no wrist flexion

Fig. 16 Modied Mallet classication

splinting to encourage long nger exor tendon
tightening only for children with grade 3 MMT
or greater wrist extension that are not tendon
transfer candidates or expected to have addi-
tional recovery. Diligent PROM is necessary to
prevent joint contractures (Hentz and Leclercq
2002; Peljovich et al. 2011). Functional splints
are used to stabilize joints or substitute for weak-
ness. These include dynamic tenodesis splints,
opponens splints, and metacarpophalangeal
extension block splints. Older pediatric patients
may be candidates for neuroprotheses utilizing
functional electrical stimulation for grasp/release
(Ragnarsson 2008).
Tendon transfers can improve UE function and
ability to participate in self-care and meaningful
tasks (Lamberg and Friden 2011; Mulcahey 1996;
Peljovich et al. 2011). Postoperative treatment is
arduous and gradually incorporates light play,
art, or functional activities to motivate and engage
young patients. Biofeedback can improve muscle
recruitment for children. See chapter Upper
8 Occupational Therapy Evaluation and Treatment
Fig. 17 Young girl using
the table as an elbow exion
assist for feeding
Limb Reconstruction in Persons with
Tetraplegia, for specic evaluation and proce-
dure details.
Arthrogryposis
Arthrogryposis is a descriptive term for multiple
congenital contractures. It has been identied as a
component in hundreds of diagnoses (Bamshad
et al. 2009). See chapter Arthrogryposis, for
more details.
Upper extremity assessment consists of
AROM/PROM, ADLs, hand function assess-
ments, upper extremity use for mobility, and
observation of compensatory movements for
UE function. Children with arthrogryposis
often develop complex movement patterns
using momentum and external surfaces to posi-
tion their hand for tasks. Common limitations
include lack of active or passive elbow exion,
wrist exion contractures, and limited digit
function. Thumbs are often adducted and within
the palm.
Early stretching and splinting are necessary for
optimizing upper limb function. Considerable
improvements can be made in the rst 6 months
to 2 years of life with diligent ROM program,
splinting, or casting (Mennen et al. 2005;
Zlotolow and Kozin 2012). Adaptive techniques
and equipment are often utilized for ADL.
Common techniques include using a table to
bend elbow for feeding, use of cuffs or adapted
189
handles to hold items, and devices to assist with
perineal hygiene (Fig. 17).
Specic surgical options will be detailed in
chapter Arthrogryposis. Tendon transfer
options are often limited due to absent or weak
donor muscles. Open dialogue between the
patient/family, surgeon, and therapist is important
to maximize the childs function, whether through
surgery or conservative management. For exam-
ple, increased wrist extension may provide
improved grip strength, but losing exion may
limit reach to mouth or perineal area. When pos-
sible, simulation of postoperative position with
splints can aid in the decision-making process.
Fracture Intervention
Passive and active range of motion may be insti-
tuted when cleared by the physician. The goal of
passive and active range of motion is to minimize
adhesion formation and to maximize movement of
the joint. Excessive PROM can damage the joint
and decrease the trust between therapist and child/
family. Distraction techniques such as interactive
tablets, videos, or word games can be very helpful
to improve PROM. Wrapping a nger with Coban
or Fabrifoam in the desired plane of movement for
15 min or more may give a prolonged stretch while
playing a game. Therapy aides are also helpful to
play music or a game with the kids using one arm.
Card holders and one-handed games like Connect
Four or Trouble can allow a child to play a game
190
Fig. 18 Infant holding aa Oball with evident exor
digitorum profundus activation with play
one handed while participating in PROM stretches.
Babies can often be distracted while bottle feeding
or with light-up switch toys. To encourage active
range of motion for the elbow, placing Velcro darts,
foamy pieces, or pieces of Play-Doh on a childs
shoulder or behind their back to bend elbow to grasp
and release is effective to encourage more end range
motion.
Zoom ball has two handles and a football-
shaped object that relies on two people to move
the football back and forth. Zoom ball is able to
elicit movement from shoulder, scapular, and
forearm muscles as well as provide as a grip
strengthening activity.
Supination and pronation can be encouraged
by a spiral glowing magic wand where the child
has to rotate the palm up and down to see the
liquid contents move back and forth. The Oball
series is an open webbed ball that is versatile
(Fig. 18). Because the ball is an open weave,
it can encourage a small child to isolate exor
digitorum profundus, thumb interphalangeal ex-
ion, and bilateral play skill. Various peg boards
S. Ashworth et al.
with differing diameters of pegs, dice games, and
craft projects with beads and small sequins can
also be benecial for rehabilitation.
Physical Agent Modalities in Pediatrics
Physical agent modalities can be useful in the
rehabilitation of child. However, the therapist
should be aware that the parameters for a child
may be completely different than in adults. Hot
packs, ice massages, and parafn baths can be
useful; however, a childs skin is more sensitive
and can potentially burn and harm quickly.
The average toweling layers needed for moist
hydrocollator heat packs are six to eight, so
adding two or more extra towels will help ensure
the skin does not burn. Parafn baths also have an
intense heat of 130140  F, which can be intimi-
dating to a child. To decrease the intensity of the
heat, it is benecial to cut strips of paper toweling
and place these strips in the wax bath. The thera-
pist can remove the strips from the bath, and the
affected body part can be mummy wrapped with
the warm wax paper towel strips (Fig. 19). Subse-
quently, the limb is wrapped in plastic wrap to seal
in the heat and then covered with a towel. Coban
can also be used over the wax strips to increase
stretch on a stiff hand. Heat modalities should not
be considered if the child is unable to verbalize
discomfort and has altered sensation or edema in
the affected joint (Michlovitz and VonNieda 2006).
Ultrasound can be contraindicated as there are
conicting studies supporting disruption of growth
plates. Neuromuscular electric stimulation (NMES)
may be benecial to facilitate exercising a muscle,
with special care to slowly acclimate the child to the
device, the electrodes, and the intensity of the stim-
ulation. Playful stickers can be placed on the elec-
trodes and referring to the unit as a tickle machine
will often allay fears of the child (Fig. 20).
Compartment Syndrome
Compartment syndrome is an increase in pressure
in the fascial compartments and can occur any-
where in the upper extremity (Taras et al. 2002).
8 Occupational Therapy Evaluation and Treatment
Fig. 19 Therapist is dipping paper towels in parafn wax
to wrap a small childs hand to apply heat properties with
less heat intensity for sensitive skin
Fig. 20 Child has electric stimulation electrodes deco-
rated with stickers over the infraspinatus motor point
191
Common causes are trauma, arterial injury,
sequelae from anticoagulation, and tight cast
application following trauma. Patients may pre-
sent with pain out of proportion to their injury,
paresthesias, paralysis of muscles, and pallor.
Children react differently with increasing analge-
sia, agitation, and anxiousness. Children with
these ndings should be urgently referred back
to the physician for immediate assessment.
Patients with compartment syndrome are typically
referred for services following fasciotomy. Eval-
uation of the wound, edema, and range of motion
is of primary importance. As the healing process
continues, assessment of the skin integrity with
methods to limit scar hypertrophy, maximize skin
mobility, and prevent adhesions should be
employed. Circumferential measurements can be
taken to evaluate edema. Scar assessment can
be assessed with photography that can be helpful
in documenting progress. Compartment syn-
drome intervention starts with wound care and
gentle range of motion. Subsequently, scar man-
agement, strengthening, ADL training, and
splinting are added as healing progresses. Children
are often guarded with movement following an
injury so distraction can be valuable. For example,
placing the arm in water can ease movement, and
covering the scar/wound with loose stockinette to
minimize wound visualization can be helpful. In
addition, encouraging the child to play games on
the Nintendo Wii or Xbox Kinect with the injured
arm is an effective way to distract a child. Scar
massage may be initiated by the child or parent
until trust is built between the therapist and a
younger child.
Peripheral Nerve Injuries (PNI)
The evaluation and treatment of a child after repair
of a PNI can be divided into three phases. During
phase one, the emphasis is on immobilization of
the injury for about 3 weeks to protect the nerve
repair while mobilizing the non-involved joints
to maintain AROM/PROM and reduce edema.
Following immobilization, phase two begins with
an emphasis on facilitating movement as recovery
and function occurs. A/PROM of the involved
192
joints, prehension patterns, and ne motor skills
should be assessed to establish a baseline and
measure progress. The 9-Hole Peg Test (Poole
et al. 2005), Purdue Pegboard, Functional Dexter-
ity Test (Aaron and Jansen 2003), and Jebsen-
Taylor Test of Hand Function (Jebsen
et al. 1990) are pertinent assessments to measure
motor recovery depending upon the nerve
(s) injured. During the mobilization phase, inter-
ventions include splinting to improve function,
biofeedback, and NMES to assist with motor
learning and control and place and hold tech-
niques to foster muscle activation. Finally, phase
three aims to maximize performance of ADLs by
improving muscle strength and sensory function.
Individual-based outcome measures, such as the
Canadian Occupational Performance Measure
(COPM) (Law et al. 1990), should be used to
assess ADL performance. Other measures include
handheld dynamometry and myometry for
strength and the dexterity measures as previously
discussed. Sympathetic function can quickly be
assessed with the wrinkle test (Vasudevan
et al. 2000), and a comprehensive sensory evalu-
ation should be performed. Progressive resistive
strengthening with TheraBand, dumbbells, cuff
weights, and computer-based training software
(BiometricsTM) should be utilized along with
sensory retraining strategies to improve hand
function for ADLs.
Duchenne Muscular Dystrophy (DMD)
Duchenne muscular dystrophy is a rapidly
progressing muscular dystrophy that affects
males primarily and is often diagnosed as early
as 35 years of age. Upper extremity function is
initially impacted due to proximal weakness lim-
iting the ability to reach overhead, followed by
decreased ability to bring the hand to the face for
self-feeding and grooming. Eventually, there is
loss of nger function and prehension. Contrac-
tures can be present throughout the upper extrem-
ity, further limiting passive movement. Evaluation
should focus on proximal range of motion/
S. Ashworth et al.
Fig. 21 Wilmington Robotic Exoskeleton (WREX)
strength at the shoulder and elbow to determine
how these limitations impact the ability to access
items in the environment, complete dressing, self-
feeding, and other ADLs. The Box and Blocks
Test (Fig. 20) is a way to quantify the speed/
efciency of upper extremity reach. Assessment
of ne motor function is integral with special
attention to the ability to isolate the ngers for
operation of keyboard and touch screen software
and to grasp to control the joystick on power
wheelchair, writing implement, and utensils. In
addition, passive composite extension should be
assessed to check for contractures of the long
nger exors that can meaningfully impair hand
function. Therapeutic intervention should aim to
maximize passive and active range of motion,
minimize contracture, and provide adapted equip-
ment and compensatory methods to maintain
independence. Aquatic therapy can be benecial
as it allows the arms to move in a reduced gravity
environment (Cup et al. 2007. Devices such as the
Wilmington Robotic Exoskeleton (WREX)
(Rahman et al. 2007; Fig. 21) can allow for ease
of upper extremity movement for completion of
ADLs. Upper extremity orthotics can be used for
nighttime splinting for contracture management
along with a daily home exercise program (HEP)
including passive range of motion exercises.
If hand function is minimal, the use of high and
low tech assistive technology can be benecial.
Touch screen software (cell phones, tablets, and
computers) and voice-activated computer pro-
grams (Dragon Naturally Speaking) can allow
for improved performance in the areas of
8 Occupational Therapy Evaluation and Treatment 193

communication, education, and employment.

Basic adapted equipment using cuffs, built-up
handles, and modied clothing can allow for
increased independence with self-care tasks.

Charcot-Marie-Tooth Disease (CMT)

CMT is a genetically based progressive peripheral

neuropathy with length-dependent impairment of
motor and sensory function resulting from either a
primary axonal or demyelinating neuropathy
(Wilmshurst and Ouvrier 2011). Patients present
with atrophy of the hand musculature (Fig. 22),
decreased thumb mobility, poor intrinsic nger
function, clawing of the digits, and diminished
positional and tactile sensitivity. The most
common functional limitations due to these
impairments are the inability to open resistive
containers, poor hand manipulation for the com-
pletion of ne motor tasks, difculty picking up
small objects, and trouble grading movement and
force due to sensory disturbances resulting in
dropping of items and the necessity for visual
afferent with regard to ne motor tasks. Assess-
Fig. 22 Thenar and intrinsic wasting due to CMT
ment of hand function should include items from
the Charcot-Marie-Tooth disease pediatric scale
(CMTPedS) (Burns et al. 2012) and assessment from tendon transfer and referral to a hand surgeon
of thumb mobility, intrinsic function, and sensory is appropriate (Estilow et al. 2012).
examination including sharp/dull, vibratory and
positional sense, two-point discrimination, and tem-
perature sensation. Patterns of weakness and sen- Summary
sory disturbance should be described by distribution
of impairment. In addition to standard clinical The evaluation and treatment of a pediatric patient
instruments and assessments, the Rotterdam require careful planning and consideration. Even
myometer (Molenaar et al. 2008) and Rydel-Seiffer though some of the assessment and treatment
tuning fork (Martina et al. 1998) are two specialized options may appear similar to those used with
assessment tools that can provide a more accurate adults, there are a wide variety of ways to adapt
measure of impairment. Interventions should focus them to meet the needs of this unique population.
on maximizing hand strength, thumb mobility, and Successful outcomes will rely on a team
development of compensatory patterns as needed. consisting of the family, therapist, physician, and
Functional splinting to provide thumb opposition child. Children will respond to therapy as long as
and prevent ulnar clawing should be completed the therapist is patient, caring, and innovative.
along with resting splints to maintain intrinsic plus Patience is a virtue when caring for children;
position and preserve thumb web space. Patients however, the rewards are exponential for both
with considerable hand dysfunction may benet the child and the therapist.
194
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 Part VI
Congenital Differences
 Introduction to Congenital Differences
and Genetics 9
Heather Lochner

Contents Abstract
Introduction to Classication Systems . . . . . . . . . . . . 199 The history of classication systems for upper
extremity congenital differences will be
History of Classication Systems . . . . . . . . . . . . . . . . . . 200
discussed, including a review of the historical
International Federation of Societies American and European classication litera-
for Surgery of the Hand (IFSSH) ture and terminology. The origin of the
Classication . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 202
Swanson International Federation of Societies
Inuence of Developmental Biology for Surgery of the Hand classication system is
and Genetics . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 204
reviewed, as well as its limitations in practical
Current Proposed Classication System . . . . . . . . . . 207 application. Based upon recent information in
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 211 developmental biology and genetics, the more
contemporary classication systems are
presented that attempt to organize associated
congenital upper extremity diagnoses within
common etiologic groupings. The proposed
Oberg, Manske, and Tonkin classication sys-
tem takes this new scientic data into consid-
eration and is presented as a potential
replacement for the current IFSSH classica-
tion system.

Introduction to Classification Systems

Classication systems are designed to improve

communication among clinicians regarding the
specic descriptive features of a condition. There-
fore, a classication system should encompass the
full spectrum of morphological abnormalities of a
condition and be easy to remember and use. How-
H. Lochner
ever, prior classication systems were often
Department of Orthopaedic Surgery, Hand Surgery
Division, Johns Hopkins University, Baltimore, MD, USA not universally understood, due to large variation
e-mail: hlochne1@jhmi.edu in terminology. Prior lack of understanding
# Springer Science+Business Media New York 2015 199
J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_9
200
regarding the etiology of various congenital
anomalies has also led to confusion and variations
in classication systems.
History of Classification Systems
There is quite an extensive history of the classi-
cation of congenital anomalies, dating back to the
mid-nineteenth century. However, the identica-
tion of congenital upper limb anomalies existed in
biblical times, as Goliath was thought to come
from a family of macrodactyly with polydactyly
(Upton 1990). Par, in 1634, documented limb
malformations, which were thought to occur
from bad thoughts or deeds. (Upton 1990,
5214). In 1829, Saint-Hilaire described the
vices of conformation as either slight or severe
(Kelikian 1974; Manske and Oberg 2009, 3).
These were deformities that were either visibly
apparent or caused limitations of function. Otto,
Table 1 (Kelikian, 1974, 54)
Saint-Hilaire (1832)
The limb as a whole Ectromely
Hemimely
Phocomely
Fingers Numerical Decrease: ectrodactyly
Increase: polydactyly
Volume Decrease: atrophy
Increase: hypertrophy
Table 2 (Kelikian, 1974, 55)
Polaillon (1884)
Arrested development
Excessive development Polydactyly
Increased number of phalanges
H. Lochner
in 1831, grouped his vices of organization into
10 distinct variations: number, size, form, posi-
tion, connection, color, consistency, continuity,
texture, and content (Kelikian 1974, 52; Manske
and Oberg 2009). Saint-Hilaire further focused on
the variations of number and size in 1832 and
rst used the terms ectromelia, hemimelia, and
phocomelia (Table 1) (Buck-Gramcko and
Ogino 1996; Kelikian 1974; Manske and Oberg
2009). These terms were used to describe absence
of the whole, half, and intermediary segments of
the extremity. He also described anomalies in
number of digits when considering polydactyly
and ectrodactyly. His variations of size included
atrophy and hypertrophy of the digits. French
authors to follow adopted much of his classica-
tion method (Kelikian 1974). In 1869, Fort added
the categories of axial deviation and adhesion, or
syndactyly (Kelikian 1974; Manske and Oberg
2009). Polaillon, in 1884, discussed developmen-
tal arrests and excesses (Table 2) (Kelikian 1974;
Manske and Oberg 2009). Examples of arrests
included conjoined, abbreviated, and absent
digits, while excesses included polydactyly and
macrodactyly. He also made reference to radial,
central, and ulnar components. During the same
time, Leboucq noted the longitudinal arrangement
of defects (Kelikian 1974; Manske and Oberg
2009).
In 1902, Nichols produced a more durable
classication system from the American authors
(Kelikian 1974). His classication system was
Ectrodactyly Total
Partial
Dactylosis
Amputations
Brachydactyly
Syndactyly
Radial Extra thumb
Bid thumb
Ulnar Well-formed extra nger
Pedunculated postminimus
Central Mirror hand
9 Introduction to Congenital Differences and Genetics
Table 3 (Kelikian, 1974, 56)
Ahstrom (1965)
Decit Split hand
deformities Axial defects
Amputations
Symbrachydactylism
Syndactylism
Annular grooves
Surplus Involving certain type of tissues only
deformities Involving whole Digits
structures Phalanges
Rays
Combination deformities (ulnar dimelia)
Dynamic deformities in which loss of function is the chief
deciency
modied over the years and republished in 1923
with new subdivisions of decient and excessive
development, subclassied into number and size
of the affected part. This system was represented
in 1965 by Ahstrom, in a more sophisticated form
(Table 3) (Kelikian 1974).
In 1914, Potel identied longitudinal and trans-
verse anomalies (Kelikian 1974; Manske and
Oberg 2009). He described three subgroups of
transverse deciencies: (1) total or complete
limb absence, (2) radicular or cases where one or
more segments are missing between the hand and
shoulder, and (3) terminal or absence of hand,
ngers, or phalanges. Longitudinal defects were
subclassied as (1) absence of the radius,
(2) absence of the radius and corresponding part
of the hand, (3) absence of the ulna, and
(4) absence of the ulna and one or more
corresponding ngers. Jones, in 1920, diagramed
the axis of the hand passing through the third
metacarpal and long nger (Kelikian 1974).
Radial structures were termed preaxial and ulnar
structures postaxial. Kanavel, in 1932, referred to
hypoplasia and hyperplasia, rather than decient
or excessive development. He stated, Congenital
malformations of the hand are due to varying
degrees of growth impairment having origin in
germ plasm. Moderate growth impairment ends
in tissue disorientation. Severe growth impair-
ment ends in aplasia and hypoplasia (Kelikian
1974, 61). The origin of hyperplasia was less
discernible to him at that time. Cutler modied
201
Kanavels classication in 1942, but his original
classication system was republished by Paletta
in 1953 (Kelikian 1974).
Other authors attempted to produce more sim-
plied classication systems. Annandale, in 1866,
divided congenital anomalies of the digits into
six headings: (1) hypertrophy, (2) deciencies,
(3) supernumerary ngers, (4) union, (5) contrac-
tions, and (6) tumors (Kelikian 1974). This
classication was repeatedly included in later
writings by Blum (1882), Anderson (1897), and
Tubby (1912), with only minor changes (Kelikian
1974). Browne, in 1933, produced a classication
system for congenital anomalies in general and
later modied this in 1939 to include (1) polydac-
tyly, (2) webbed ngers, (3) hypertrophy, (4) atro-
phic changes, (5) molding deformities, and
(6) acrocephalosyndactyly (Kelikian 1974).
German contributions in the congenital classi-
cation literature were made in the late nineteenth
and early twentieth centuries. In 1895, Kummel
described three categories of congenital anoma-
lies: (1) defect malformation, (2) syndactyly, and
(3) polydactyly (Manske and Oberg 2009). These
categories were later modied by Nigst in 1927.
Muller, in 1937, reported that malformations
could present as a spectrum of varying degrees,
or in varying stages of development (Buck-
Gramcko and Ogino 1996; Manske and Oberg
2009). The teratological progression concept
allowed anomalies to be graded by morphological
severity and allowed anomalies to be grouped by
appearance, simplifying the classication system
(Buck-Gramcko and Ogino, 1996; Manske and
Oberg 2009).
ORahilly, in his classication system of long-
bone deciencies, expanded upon intercalary de-
ciencies in 1951 (Kelikian 1974; Knight and Kay
2000). He referred to Jones preaxial and postaxial
terminology but felt that hemimelia could be
transverse or longitudinal (paraxial) (Kelikian
1974; Knight and Kay 2000). Longitudinal
defects were divided into terminal and intercalary,
with terminal longitudinal defects lacking the
radius or ulna and all distal structures, while inter-
calary longitudinal defects might lack one of those
intermediate structures (Knight and Kay 2000).
He also described coherence of normally
202 H. Lochner

Table 4 (Kelikian, 1974, 63)

Frantz and ORahilly (1961)
Terminal Transverse Amelia (absence of limb)
Hemimelia (absence of forearm and hand)
Partial hemimelia (part of forearm is present)
Acheiria (absence of hand)
Complete adactylia (absence of all ve digits and their metacarpals)
Complete aphalangia (absence of one or more phalanges from all ve digits)
Longitudinal Complete paraxial hemimelia (complete absence of one of the forearms and elements
and corresponding portion of the hand)
Incomplete paraxial hemimelia (similar to above but part of defective element is present)
Partial adactylia (absence of one to four digits and their metacarpals)
Partial aphalangia (absence of one to four digits and their metacarpals)
Intercalary Transverse Complete phocomelia (hand attached directly to the trunk)
Proximal phocomelia (hand and forearm attached directly to the trunk)
Distal phocomelia (hand attached directly to the arm)
Longitudinal Complete paraxial hemimelia (similar to corresponding terminal defect but hand is more
or less complete)
Incomplete paraxial hemimelia (similar to corresponding terminal defect but hand is
more or less complete)
Partial adactylia (absence of all or part of metacarpal)
Partial aphalangia (absence of proximal or middle phalanx or both from one or more
digits)

adjacent structures or synostosis, under fusion
hemimelia (Kelikian 1974, 62).
Frantz and ORahilly, in 1961, created a widely
accepted classication system based upon
ORahillys earlier publication with two main
groups, terminal and intercalary (Table 4) (Burtch
1966; Chung 2011; Frantz and ORahilly 1961;
Kelikian 1974; Knight and Kay 2000). However,
the terminology used by these authors was not
accepted in Europe due to the use of terms such
as hemimelia. Burtch revised this classication
system in 1966 to eliminate the controversial
terms and retained four of the main categories:
terminal transverse, terminal longitudinal, interca-
lary transverse, and intercalary longitudinal defects
(Burtch 1966; Knight and Kay 2000). The termi-
nology was modied, and the concept of a central
longitudinal deciency, or cleft hand, was intro-
duced (Burtch 1966; Knight and Kay 2000).
ORahilly in 1969, and numerous other
authors, realized that Although the delineation
of limb anomalies may be based . . . on clinical,
functional, teratogenic, embryological or genetic
criteria, the only satisfactory criterion of
classication at the present time is anatomic
(Kelikian 1974, 67; ORahilly, 1969, 15). He also
recommended that in each anatomic category, it
should be identied whether the anomaly is iso-
lated or part of a syndrome with other systems
involved (Kelikian 1974; ORahilly 1969).
International Federation of Societies
for Surgery of the Hand (IFSSH)
Classification
Multiple classication systems resulted from
these predecessors; however, none were univer-
sally adopted. These systems did not include all
congenital anomalies, and terminology varied
greatly between hand surgeons from Europe and
the United States. Most hand surgeons adopted
the classication system proposed by Swanson in
1964. This system was based on the assumption
that anomalies should be grouped by the anatomic
part of the limb affected during development.
He created six basic categories in his initial sys-
tem: failure of differentiation of parts, arrest of
9 Introduction to Congenital Differences and Genetics 203

development, duplications, overgrowth, congeni- Table 5 Swanson/International Federation of Societies

tal circular constriction band syndrome, and gen- for Surgery of the Hand (IFSSH) Classification (Swanson,
1976, 9)
eralized skeletal defects. His system introduced
the concept of embryologic failure and allowed a I. I.Failure of formation of parts (arrest of
development)
large group of conditions to be included in the
A. Transverse deciencies
failure of differentiation group. Swansons classi-
B. Longitudinal deciencies
cation system also provided a comprehensive
1. Phocomelia
but simple system, which classied both complex 2. Radial
and simple diagnoses, using straightforward lan- 3. Central
guage (Chung 2011; Entin et al. 1972; Kelikian 4. Ulnar
1974; Knight and Kay 2000; Manske and Oberg II. I.Failure of differentiation (separation) of parts
2009; Sammer and Chung 2009; Swanson 1976; A. Synostosis
Swanson et al. 1983). B. Radial head dislocation
This classication system was reviewed by the C. Symphalangism
American Society for Surgery of the Hand D. Syndactyly
(ASSH), the International Federation of Societies E. Contracture
for Surgery of the Hand (IFSSH), and the Interna- 1. Soft tissue
tional Society for Prosthetics and Orthotics a. Arthrogryposis
(ISPO) and modied to include a seventh cate- b. Pterygium
gory, undergrowth. The resulting classication c. Trigger
system was published in 1974 in the orthotics d. Absent extensor tendons
e. Hypoplastic thumb
and prosthetics literature and as the inaugural
f. Clasped thumb
issue of the American volume of the Journal of
g. Retroexible thumb
Hand Surgery in 1976 (Manske and Oberg 2009;
h. Camptodactyly
Swanson 1976). This was subsequently referred
i. Windblown hand
to as the IFSSH classication system and included 2. Skeletal
the seven major categories, with subcategories, a. Clinodactyly
subclassications, and anatomic levels of anoma- b. Kirner deformity
lies and diagnoses (Table 5) (Knight and Kay c. Delta bone
2000; Manske and Oberg 2009; Swanson 1976; III. Duplication
Swanson et al. 1983). A. Thumb
Authors who have found it difcult to classify B. Triphalangism/hyperphalangism
complex cases, particularly with respect to cleft C. Polydactyly
hand and symbrachydactyly, have criticized the D. Mirror hand
IFSSH classication system. Knight and Kay IV. I. Overgrowth (gigantism)
published a more detailed version of the classi- A. Limb
cation system in 2000, which attempted to include B. Macrodactyly
all congenital anomalies within the seven catego- V. I. Undergrowth (hypoplasia)
ries. The authors remarked that the IFSSH system VI. Congenital constriction band syndrome
VII. Generalized skeletal abnormalities
was articial and that a distinction between failure
of formation and differentiation does not truly
exist. They felt these two categories overlapped Ogino 1996; Chung 2011; De Smet et al. 1997;
and represented a spectrum of deformity. In addi- Knight and Kay 2000; Lamb 1990; Manske and
tion, the authors noted that complex deformities Oberg 2009; Ogino et al. 1986; Sammer and
might have combinations of malformations, Chung 2009).
which do not belong to any one category. It is The Japanese Society for Surgery of the Hand
truly a morphological classication system with- also published a modication of the IFSSH clas-
out considering etiology (Buck-Gramcko and sication system, with the addition of two new
204 H. Lochner

Table 6 Abbreviated Japanese Society for Surgery of the congenital anomalies. This caused many authors
Hand modification (Sammer and Chung, 2009, 155) to reexamine the current IFSSH classication sys-
Main category Example tem and attempt to apply it to this new knowledge.
I. Failure of formation Symbrachydactyly Tonkin, in 2006, recognized the problems that
II. Failure of differentiation Radioulnar synostosis arose when attempting to classify deformities in
III. Duplication Radial polydactyly, a morphologically based system, given our
ulnar polydactyly,
increasing understanding of genetic causation
mirror hand
(Manske and Oberg 2009; Tonkin 2006). This
IV. Abnormal induction of Cutaneous syndactyly,
digital rays osseous syndactyly, was clearly apparent when examining typical
central polydactyly, cleft hand and atypical cleft hand. Considering
typical cleft hand the JSSH recommendations regarding a new
V. Overgrowth Macrodactyly group, abnormal induction of rays, Tonkin rec-
VI. Undergrowth Brachydactyly, ognized that syndactyly, central polydactyly, and
clinodactyly
clefting should be grouped together; however, the
VII. Constriction band Constriction band
syndrome genetic etiology behind the phenotype was
VIII. Generalized skeletal Achondroplasia extremely varied with a complex pathway rather
IX. Others than one specic gene abnormality. Because of
this complex interaction in hand development,
he found that simply moving these diagnoses
categories: abnormal induction of rays and
from one group to another within the IFSSH clas-
unclassiable cases (Table 6) (Sammer and
sication system in order to attempt to introduce
Chung 2009). This system attempted to include
some understanding of etiology would not sufce,
the concept of causation into the abnormal induc-
and a major reassessment was needed. Alterna-
tion group as was recently suggested in experi-
tively, he proposed a new classication with a
mental studies. This category included simple
descriptive focus, which identied the location
and complex syndactyly, central polydactyly-
of deformity with an additional subclassication
cleft hand-osseous syndactyly complex, and
based upon the tissue involved and its specic
triphalangeal thumb. The authors had previously
features (Manske and Oberg 2009; Tonkin
noted that typical cleft hand often involves
2006). He suggested that a descriptive classica-
absence of the central rays, polydactyly and syn-
tion system could provide a universal language
dactyly, which were classied under three differ-
for discussion, which would not attempt to
ent groups in the IFSSH system. Their new group
explain causation for the deformity (Table 7)
attempted to include all components after
(Tonkin 2006, 489 and 495).
embryologic studies supported the common etiol-
In 2009, Manske and Oberg reviewed the
ogy. However, the revised classication system was
IFSSH classication system and published
not universally accepted, as differences between
updated information regarding the etiology of
abnormal induction and abnormal formation were
various congenital upper limb conditions. They
not thought to be signicant (Chung 2011; Manske
also proposed a modication to the IFSSH classi-
and Oberg 2009; Naruse et al. 2007; Sammer and
cation system to accommodate for this
Chung 2009).
expanding knowledge of developmental biology
(Table 8) (Manske and Oberg 2009).
Influence of Developmental Biology The authors rst discussed Group 1: failure of
and Genetics formation. This group is further divided into lon-
gitudinal and transverse deciencies, with longi-
As knowledge was gained in the eld of Genetics, tudinal including radial, central, and ulnar
increasing information became available which deciencies. Transverse deciencies may be ter-
gave insight into some of the mechanisms of minal or intercalary, depending upon the location
9 Introduction to Congenital Differences and Genetics 205

Table 7 A practical description of upper limb anomalies Table 7 (continued)

(Tonkin, 2006, 495-6)
(B) Transverse deciency
(I) Abnormalities of zeugopod (arm) (1) Transverse absence
(A) Hypoplasia/aplasia (2) Brachydactyly
(1) Shoulder (C) Intersegmental deciency
(2) Arm (1) Symbrachydactyly
(3) Poland syndrome (2) Brachydactyly
(B) Transverse deciency (D) Synostosis
(1) Transverse arrest (1) Carpal
(C) Intersegmental deciency (2) Metacarpal
(1) Phocomelia (3) Symphalangism
(2) Symbrachydactyly (4) Phalangeal complex syndactyly
(D) Synostosis (E) Overgrowth
(E) Overgrowth (1) Macrodactyly
(1) Hemihypertrophy (F) Duplication
(F) Duplication (1) Radial polydactyly
(1) Whole arm (2) Central polydactyly
(G) Anomalies of soft tissue (3) Ulnar polydactyly
(1) Arthrogryposis (4) Mirror hand
(H) Complex osseous anomalies (G) Anomalies of soft tissue
(I) Constriction ring syndrome (1) Simple syndactyly
(J) Congenital tumorous condition (2) Camptodactyly
(II) Abnormalities of stylopod (forearm) (3) Distal arthrogryposis
(A) Hypoplasia/aplasia (4) Clasped thumb
(1) Radial deciency (5) Trigger digit
(2) Ulnar deciency (6) Cleft hand complex
(B) Transverse deciency (H) Complex osseous anomalies
(1) Transverse absence (1) Complex syndactyly
(C) Intersegmental deciency (2) Clinodactyly
(1) Symbrachydactyly (3) Triphalangism
(D) Synostosis (4) Cleft hand complex
(1) Elbow synostosis (5) Hyperphalangism
(2) Forearm synostosis (I) Constriction ring syndrome
(E) Overgrowth (J) Congenital tumorous conditions
(1) Hemihypertrophy
(F) Duplication
(1) Ulnar duplication
of cellular abnormality in the developing limb
(G) Anomalies of soft tissue
bud. Recent studies suggested that intercalary
(1) Arthrogryposis
deciencies are difcult to explain in develop-
(H) Complex osseous anomalies
mental biology and may actually represent a
(1) Madelung deformity
(I) Constriction ring syndrome
severe form of longitudinal deciency. Radial
(J) Congenital tumorous condition dysplasias result from decreased limb volume
(III) Abnormalities of autopod (wrist and hand) with intact sonic hedgehog (SHH) expression,
(A) Hypoplasia/aplasia resulting in a spectrum of reduction in tissue and
(1) Radial deciency limb length, preserving the posterior and ulnar
(2) Ulnar deciency elements. Animal models utilizing reduction in
(3) Brachydactyly apical ectodermal ridge (AER) broblast growth
(continued) factor produce progressive reduction in length as
206
Table 8 Proposed modifications to the IFSSH classifica-
tion (Manske and Oberg, 2009, 15)
I. Failure of axis formation and/or differentiation
Radial longitudinal deciency
Radial-ulnar synostosis
Ulnar longitudinal deciency
Transverse deciency (including symbrachydactyly)
Dorsal-ventral deciency
II. Failure of hand-plate formation and/or differentiation
Syndactyly
Apert syndrome
Central deciency (cleft hand)
Camptodactyly
Clinodactyly
Clasped thumb
Hand-plate synostoses
Metacarpal synostosis
Carpal synostosis
III. Duplication
Radial polydactyly (including triphalangeal thumb)
Ulnar dimelia
Ulnar polydactyly
IV. Overgrowth
V. Amniotic band sequence
VI. Generalized skeletal abnormalities
is seen clinically in radial dysplasia. This includes
thumb hypoplasia, radial deciencies, and proxi-
mal radial longitudinal deciency, previously
referred to as phocomelia. Many molecular path-
ways may affect cell growth and apoptosis, caus-
ing alterations in limb volume often seen in the
variety of presentation of radial dysplasia with
various syndromes (Al-Qattan et al. 2009;
Manske and Oberg 2009; Riddle and Tabin 1999).
Central deciencies or cleft hand results
from suppressed development of bone and central
soft tissue structures. This also has a wide presen-
tation clinically but does not include deciencies
of the wrist. Defects in the central or medial AER
result in central clefting but can also present in
association with syndromes exhibiting genetic
mutations in AER function. Central deciencies
are also associated with central polydactyly and
syndactyly, as was demonstrated by Ogino in
1990, in an animal model utilizing rat litters, indi-
cating a common etiology (Manske and Oberg
2009; Ogino 1990). This resulted from diffuse
H. Lochner
apoptosis of the limb-bud ectoderm and meso-
derm, reduced factor expression in limb organiza-
tion, and interruption of the AER. Based upon this
new information, it was suggested that a separate
grouping might be more appropriate for central
deciency. Many authors had previously pro-
posed this; however, distinction between cleft
hand and atypical cleft symbrachydactyly was
not clearly understood, but the two are now
known to represent discrete hand deformities
(Al-Qattan et al. 2009; Manske and Oberg 2009;
Naruse et al. 2007; Ogino 1990).
Manske and Oberg noted that ulnar decien-
cies also comprise a spectrum of abnormalities
that follow the ulnar longitudinal axis of the
arm; however, they can also include the radial
aspect of the hand. Recent developmental biology
studies suggest this is explained by disruption in
the zone of polarizing activity (ZPA) along the
postaxial border of the limb bud. Since the ZPA
is responsible for ulnarization of the developing
limb through expression of SHH, interruption in
the timing, degree, and duration of SHH expres-
sion can produce the variability seen in ulnar
deciencies. Animal models suggest that SHH
may also play a role in limb proliferation, where
loss of SHH may reduce volume. Involvement of
the hand seems to occur with variability in the
timing and extent of SHH loss (Al-Qattan
et al. 2009; Manske and Oberg 2009; Riddle and
Tabin 1999).
Transverse deciencies include terminal and
intercalary deciencies. Two types of terminal
deciencies due to failure of formation have
been identied, symbrachydactyly and transverse
arrest (or congenital amputation). Manske and
Oberg remarked that the IFSSH classication
places the two types into separate categories
(Manske and Oberg 2009). However, the authors
believed that symbrachydactyly is most likely a
distal form of transverse deciency, while trans-
verse arrest may be a more proximal one. This is
supported in a study by Kallemeier in 2007 which
noted that 93 % of transverse arrest extremities
show evidence of terminal digital elements as is
seen in symbrachydactyly (Kallemeier et al. 2007;
Manske and Oberg 2009). Another animal study
by Summerbell in 1974 suggested that transverse
9 Introduction to Congenital Differences and Genetics
deciency resulted after the removal of the AER
from developing wing buds (Manske and Oberg
2009; Summerbell 1974). The level of resulting
truncation correlated to the timing of AER
removal in the study. This can also result after
removal of broblast growth factor, which pro-
motes proliferation of mesoderm tissue. Newer
data from Winkel et al., in 2008, demonstrated a
link between Wnt signaling and a mutated recep-
tor in symbrachydactyly (Manske and Oberg
2009; Winkel et al. 2008). It is now apparent that
Wnt is important in AER-related broblast
growth factor expression and function.
Manske and Oberg also noted that intercalary
deciencies or phocomelia has long been consid-
ered a segmental transverse deciency, as initially
described by Frantz and ORahilly (Frantz and
ORahilly 1961; Manske and Oberg 2009). How-
ever, the authors note these deciencies do not
include true segmental defects, since the limb is
abnormal proximal and distal to the affected seg-
ment. In addition, a large portion of extremities
diagnosed as phocomelia cannot be easily classi-
ed in any preexisting system. It is more likely
that many of the phocomelias actually represent a
severe form of radial longitudinal deciency
instead and should be classied accordingly
(Manske and Oberg 2009).
In Group II, failure of differentiation, Manske
and Oberg commented that most conditions
represented abnormalities of development rather
than differentiation. However, they noted that
they did share a common element of disruption
of the hand plate. Syndactyly is noted to occur
with disruption of interdigital bone morphogenic
protein (BMP) signaling and abnormal broblast
growth factor. It can be an isolated nding or as
part of failure of formation and duplication with
other conditions. Other diagnoses the authors
attribute to disruption of hand-plate formation
included Apert syndrome, clinodactyly,
camptodactyly, clasped thumb, and various syn-
ostoses (Al-Qattan et al. 2009; Manske and Oberg
2009).
Group III includes duplication, which is rela-
tively well understood developmentally. In animal
models, ectopic SHH increases the limb volume
to yield radial polydactyly but may range in
207
spectrum from an extra digit to mirror hand and
ulnar dimelia. Triphalangeal thumb and radial
polydactyly can result from mutations in the
SHH-specic regulatory region. The authors also
state that defects in SHH antagonists may also
produce overexpression of SHH leading to radial
and ulnar polydactyly. However, this is clearly not
the whole explanation as mutations in a variety of
genes are noted in polydactyly and appear to be
unrelated to the SHH pathway (Al-Qattan
et al. 2009; Manske and Oberg 2009).
Manske and Oberg went on to summarize the
remainder of the IFSSH classication system
Groups IVVII but did not propose any changes
to those subgroups. They modied Group I to
failure of axis formation and/or differentiation,
based upon the common etiology affecting those
conditions. The authors felt that synostoses of the
radius and ulna would more appropriately t in
this category as they appeared to be more related
to longitudinal deciencies. Symbrachydactyly
was also moved to this group as previously
discussed (Manske and Oberg 2009).
The authors modied Group II to failure of
hand-plate formation and/or differentiation, since
they felt this would represent most of those
included conditions. It also included all
malformations that targeted the hand; therefore,
central deciency was moved to this group. They
recognized that information regarding SHH
misregulation might induce radial polydactyly
and triphalangeal thumb, which could be included
in Group II. However, their modication kept it in
Group III duplication until further information on
limb development was identied, recognizing that
further investigation was necessary (Manske and
Oberg 2009).
Current Proposed Classification
System
In 2010, Oberg, Manske, and Tonkin (OMT) col-
laboratively created a new classication system
based upon prior insight into previous classica-
tions and the enlarging fund of knowledge
in development biology (Table 9) (Oberg
et al. 2010). The OMT classication system uses
208 H. Lochner

Table 9 Oberg, Manske, and Tonkin (OMT) classifica- dysmorphology terminology, in order to describe
tion (Oberg et al, 2010, 2073) the axis of formation/differentiation and the limb
I. Malformations segment predominantly affected. It also takes into
A. Failure of axis formation/differentiation entire upper consideration the underlying molecular and
limb
genetic etiology of the anomalies. This new clas-
1. Proximal-distal outgrowth
sication system has been proposed as a replace-
Brachymelia with brachydactyly
Symbrachydactyly
ment for the Swanson/IFSSH classication
Transverse deciency system (Oberg et al. 2010).
Intersegmental deciency The authors noted that IFSSH Group 1 was
2. Radial-ulnar (anteroposterior) axis comprised of consistent abnormalities affecting
Radial longitudinal deciency the formation of the proximal limb, while Group
Ulnar longitudinal deciency 2 emphasized abnormalities of the hand plate.
Ulnar dimelia However, both of these groups included abnor-
Radioulnar synostosis
malities due to failure of formation/differentiation
Humeroradial synostosis
rather than two different etiologies. Likewise,
3. Dorsal-ventral axis
Group 3, duplication, resulted from similar etiol-
Nail-patella syndrome
B. Failure of axis formation/differentiation hand plate ogies of failures of differentiation and formation
1. Radial-ulnar (anteroposterior) axis affecting the limb axes. They also noted that none
Radial polydactyly of the IFSSH groups effectively addressed the
Triphalangeal thumb breadth of abnormalities found in cleft hands.
Ulnar polydactyly When evaluating the existing IFSSH Group 4,
2. Dorsal-ventral axis overgrowth, the authors felt that this was a
Dorsal dimelia (palmar nail) descriptive term, which did not actually represent
Hypoplastic/aplastic nail
an etiology for the abnormality. Likewise, IFSSH
C. Failure of axis formation/differentiation unspecied
axis
Group 5, undergrowth, was very nonspecic
1. Soft tissue regarding the genetic etiology of the category.
Syndactyly Some of the undergrowth conditions such as
Camptodactyly brachydactyly would actually be better placed
2. Skeletal deciency with the molecular pathways involving transverse
Brachydactyly or longitudinal deciencies. The IFSSH Group
Clinodactyly 7, generalized skeletal disorders, was also noted
Kirner deformity to have a broad and diverse grouping of
Metacarpal and carpal synostosis
syndromic diagnoses, which was not based upon
3. Complex
any clear morphological or causative grouping
Cleft hand
Synpolydactyly (Oberg et al. 2010).
Apert hand The authors rst created new categories for the
II. Deformations OMT classication system that fell under the
A. Constriction ring sequence major headings of Malformations, Deformations,
B. Arthrogryposis and Dysplasias based upon the etiology of the
C. Trigger digits abnormality. They chose to use dysmorphology
D. Not otherwise specied terms to better group the various upper limb
III. Dysplasias
abnormalities into common etiologies, stating:
A. Hypertrophy
A malformation is an abnormal formation of a
1. Macrodactyly
2. Upper limb
body part or complex tissue. Deformation differs
3. Upper limb and macrodactyly from a malformation as the insult occurs after
B. Tumorous conditions normal formation. A dysplasia is an abnormality
in the size, shape, and organization of the cells
9 Introduction to Congenital Differences and Genetics
within a tissue (Oberg et al. 2010, 2072). They
noted that disruption of the molecular pathways
determining proximal-distal, anteroposterior, and
dorsal-ventral axes caused consistent abnormali-
ties affecting the entire limb (Oberg et al. 2010).
The OMT classication therefore divides the
malformations group into failure of axis forma-
tion/differentiation depending upon the limb seg-
ment affected, specically, the entire upper limb,
hand plate, and hand-plate-unspecied axis. Within
the rst two subgroupings, the authors further
divided the categories into proximal-distal
outgrowth, radial-ulnar axis, and dorsal-ventral
axis. The third group is further delineated into soft
tissue, skeletal, and complex (Oberg et al. 2010).
Within the subcategory 1A failure of axis for-
mation/differentiation entire upper limb, the
authors have included brachydactyly, which
impacts the entire upper limb, as well as trans-
verse deciencies, radial and ulnar longitudinal
deciencies, radial and ulnar duplications, and
defects of the dorsal-ventral axis, such as nail-
patella syndrome. Therefore, all malformations
that alter the upper limb symmetry and have a
comprehensive effect on limb development are
included (Oberg et al. 2010).
In subcategory 1B, failure of axis formation/
differentiation hand plate, the OMT classica-
tion includes preaxial and postaxial polydactyly,
triphalangeal thumbs, and dorsal dimelia. All of
these malformations can be attributed to disrup-
tion of an axial signaling pathway, point muta-
tions, or duplications in limb-specic SHH
regulation which induce ectopic SHH expression.
Subcategory 1C, failure of hand-plate formation/
differentiation unspecied axis, includes those
hand-plate defects which are not related to axis
disruption. These include those abnormalities that
involve hand-associated pathways, which regu-
late phalanx and interdigit formation and designa-
tions, including synpolydactyly and cleft hand as
previously demonstrated by Ogino (Naruse
et al. 2007; Ogino 1990; Oberg et al. 2010).
Deformations are identied as the second
category within the OMT classication system.
These abnormalities typically result from disrup-
tion or deformity of any portion of a limb that has
209
already formed, including constriction ring
sequence, trigger ngers, or arthrogryposis.
Arthrogryposis is thought to occur mid-gestation
after the joints and skeleton have already formed,
while trigger digits typically present in childhood
after birth. In this category, the authors did not
include those conditions resulting from infection,
vascular insults, or mechanical damage since they
do not typically produce a consistent pattern of
limb abnormality. Alternatively, they placed them
under subcategory D, not otherwise specied
(Oberg et al. 2010).
The third category of the OMT classication
system is dysplasias, which includes a unique
group of conditions associated with cellular atypia
or tumor formation. This includes hypertrophy or
macrodactyly due to an as yet unidentied factor
causing cellular dysplasia. However, the authors
recognized that this category may include condi-
tions that have yet to be fully understood in terms
of their etiology and may represent a malforma-
tion or deformation. Future insights from devel-
opmental biology are needed to fully understand
their causation (Oberg et al. 2010).
Recently, in 2013, Tonkin et al. published an
assessment of the proposed OMT classication
system in order to evaluate its efcacy. Two
hand surgeons, one hand surgery fellow, and a
resident physician rst attempted to place all
hand diagnoses listed in the Swanson/IFSSH clas-
sication system into the OMT classication sys-
tem to determine interobserver reliability. This
process was repeated 3 months later to establish
intraobserver reliability. In the second part of the
study, 101 patients with 150 limb abnormalities
were classied into both the IFSSH and OMT
classication systems over a 5-month time period
in 2012. The nal component of the study
involved a questionnaire, which was sent to all
members of the Congenital Hand Anomalies
Study Group (CHASG) regarding their experi-
ence, preference, and assessment of the two clas-
sication systems (Tonkin et al. 2013).
The authors found that it was possible to
transfer the diagnoses from the IFSSH classica-
tion system to the OMT classication system.
Intraobserver reliability was relatively high
210 H. Lochner

Table 10 Refinement of OMT classification (extended Table 10 (continued)

version) (Tonkin et al. 2013 1852-53)
3. Dorsal-ventral axis
I. Malformations i. Dorsal dimelia (palmar nail)
A. Failure of axis formation/differentiation entire upper ii. Hypoplastic/aplastic nail
limb iii. Arthrogryposis
1. Proximal-distal axis 4. Unspecied axis
i. Brachymelia with brachydactyly a. Soft tissue
ii. Symbrachydactyly i. Syndactyly
iii. Transverse deciency ii. Camptodactyly
Amelia iii. Thumb in palm deformity
Clavicle iv. Deviated nger without skeletal deformity
Long/short above elbow b. Skeletal deciency
Long/short below elbow i. Clinodactyly
Wrist ii. Kirner deformity
Proximal-distal carpal row iii. Metacarpal and carpal synostosis
Metacarpal c. Complex
Proximal/middle/distal phalanx i. Cleft hand
iv. Intersegmental deciency ii. Synpolydactyly central
Phocomelia (total/proximal/distal) iii. Apert hand
2. Radial-ulnar (anteroposterior) axis II. Deformations
i. Radial longitudinal deciency A. Constriction ring sequence
Thumb hypoplasia (with proximal limb B. Trigger digits
involvement)
C. Not otherwise specied
ii. Ulnar longitudinal deciency
III. Dysplasias
iii. Ulnar dimelia
A. Hypertrophy
iv. Radioulnar synostosis
1. Whole limb
v. Humeroradial synostosis
i. Hemihypertrophy
Elbow ankyloses
ii. Aberrant exor/extensor/intrinsic muscles
3. Dorsal-ventral axis
2. Partial limb
i. Nail-patella, Fuhrmann, and Al-Awadi syndromes
i. Macrodactyly
ii. Arthrogryposis
ii. Aberrant intrinsic muscles of hand
iii. Absent/hypoplastic extensor/exor muscles
B. Tumorous conditions
4. Unspecied axis
1. Vascular
i. Undescended shoulder (Sprengel)
i. Hemangioma
ii. Abnormal shoulder muscles
ii. Malformation
B. Failure of axis formation/differentiation hand plate
2. Neurological
1. Proximal-distal axis
i. Neurobromatosis
i. Brachydactyly
3. Connective tissue
ii. Symbrachydactyly
i. Juvenile aponeurotic broma
iii. Transverse deciency
ii. Infantile digital broma
Wrist
4. Skeletal
Proximal-distal carpal row
i. Osteochondromatosis
Metacarpal
ii. Enchondromatosis
Proximal/middle/distal phalanx
iii. Fibrous dysplasia
2. Radial-ulnar (anteroposterior) axis
iv. Epiphyseal abnormalities
i. Radial (thumb) deciency (no radius
4. Syndromes
involvement)
A. Specied
Absent thumb
1. Apert
Absent/hypoplastic thenar muscles
2. Arthrogryposis
ii. Ulnar deciency
3. Baller-Gerold
iii. Radial polydactyly
4. Bardet-Biedl
iv. Triphalangeal thumb
v. Ulnar polydactyly (continued)
(continued)
9 Introduction to Congenital Differences and Genetics 211

Table 10 (continued) systems. A considerable number of the CHASG

5. Brachmann-de Lange members felt replacement of the IFSSH classi-
6. Carpenter cation system with the OMT classication would
7. Catel-Manzke be appropriate, as the IFSSH system does not
8. Constriction band reect the current understanding of limb develop-
9. Crouzon ment and causation. However, the authors
10. Distal arthrogryposis
acknowledge that problems still remain with the
11. Down
proposed system, as limb development does not
12. Ectrodactyly-ectodermal dysplasia-clefting
13. Fanconi pancytopenia
occur in isolation but is dependent upon multiple
14. Fuhrmann and Al-Awadi factors affecting multiple axes. Additionally, mul-
15. Goltz tiple phenotypes may develop from a specic
16. Gorlin genetic abnormality, or a single phenotype may
17. Greig cephalopolysyndactyly occur with multiple varying genetic failures. After
18. Hajdu-Cheney much discussion of the various upper limb abnor-
19. Holt-Oram malities, an extended version of the OMT classi-
20. Larsen cation system was rened by the authors to better
21. Leri-Weill dyschondrosteosis
accommodate all diagnoses (Table 10). This clas-
22. Levy-Hollister
sication system is currently under review by the
23. Moebius sequence
24. Multiple synostoses
IFSSH for future consideration. Clearly, advances
25. Nager in developmental biology and genetics will con-
26. Nail-patella tinue to require recurrent assessment and modica-
27. Noonan tion of classication systems for upper limb
28. Oculo-auriculo-vertebral spectrum (Goldenhar abnormalities (Tonkin et al. 2013).
syndrome)
29. Oculodentodigital
30. Oral-facial-digital
31. Oto-palato-digital References
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malities into the OMT classication system. cies. J Bone Joint Surg Am. 1961;43:120224.
Kallemeier PM, Manske PR, Davis B, Goldfarb CA. An
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assessment of the relationship between congenital
to lack of understanding of the specic nature of transverse deciency of the forearm and
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growing hand. London: Harcourt; 2000. p. 12535.
Lamb DW. Congenital malformation of the upper limb.
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Embryol Exp Morphol. 1974;32:65160.
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 Hypoplasia, Brachydactyly, and Other
Failures of Formation 10
Scott A. Riley and Ronald Burgess

Contents Abstract
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 214 Congenital anomalies affect 12 % of new-
borns, and approximately 10 % of these chil-
Hypoplasia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 214
Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 218
dren have upper extremity abnormalities.
Brachydactyly and Symbrachydactyly . . . . . . . . . . . . . . 218 Congenital anomalies of the limb are second
Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 223 only to congenital heart disease in the inci-
Cleft Hand . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 223 dence of birth malformations (Bamshad
Technique: Snow-Littler Reconstruction of
Cleft Hand . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 228
et al. Pediatr Res 45:291299, 1999). The clin-
Transverse Deciencies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 230 ical manifestations of these anomalies in chil-
Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 232 dren are extremely variable, and as such,
Phocomelia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 233 classifying specic patterns of deformities
Amelia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 234
Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 234
remains an ongoing challenge. However,
approximately 1015 % of these congenital
Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 234 upper extremity anomalies can be grouped
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 235 into a broad category that represents underde-
velopment and/or failure of formation within
portions of the upper limb. The deformities
represented in this category can range any-
where from a smaller than normal digit (hypo-
plasia) to a complete absence of the extremity
(amelia).
To the medical provider who is unaccus-
tomed to evaluating and caring for these chil-
dren, describing these anomalies can be
difcult, and formulating potential treatment
plans is often a difcult task. Therefore, the
goals of this chapter are to (1) present accepted
terminology used to dene specic conditions,
(2) discuss the common clinical features, and
(3) provide treatment recommendations for
S.A. Riley (*) R. Burgess
some particular diagnostic subtypes seen in
Hand and Upper Extremity Surgery, Shriners Hospital for
Children-Lexington, Lexington, KY, USA this category of deformities.
e-mail: sariley@shrinenet.org; ronburgessmd@gmail.com

# Springer Science+Business Media New York 2015 213

J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_10
214
Introduction
For the past 50 years, limb malformations in the
upper extremity have been classied according to
the predominant anomaly identied. Most con-
genital upper extremity anomalies can t into
one of seven general categories as dened by the
International Federation of Hand Societies
(Table 1; Swanson 1976). Some upper extremity
anomalies are easier to classify than others. For
example, a transverse deciency in the upper
extremity is classied as a Type I failure of
formation. The clinical presentation of this condi-
tion is usually straightforward, so categorizing the
anomaly is not difcult. However, if the limb also
has residual nubbin-like structures, like those seen
in symbrachydactyly, the classication is more
challenging (Cheng et al. 1987). Then consider
an anomaly like cleft hand, classically thought to
represent a longitudinal-central failure of forma-
tion (Type I); but it can also present with anatomic
ndings that show characteristics of hypoplasia
(Type IV undergrowth) as well as syndactyly
(Type II failure of separation). All in all, there
are many instances where descriptive classica-
tion schemes do not work very well.
While it is interesting to discuss how different
upper extremity anomalies can t in to certain
classication systems, it should be stated that as
the understanding of developmental biology
improves, these systems will continue to evolve
over time (Manske and Oberg 2009). A more
recent scheme for classifying congenital hand
anomalies, referred to as the Oberg, Manske, and
Tonkin (OMT) system (Oberg et al. 2010; Tonkin
et al. 2013), has proposed three distinct categories
that distinguish malformations from those that
represent deformities or dysplasia (Table 2). Ulti-
mately, the hope is that by continuing to explore
these developmental differences, the physician
community may be able to offer better treatment
protocols for a child with a specic deformity.
Rather than delving into these categorical differ-
ences, the goal of this chapter is to develop an
appreciation of the more common anomalies asso-
ciated with both undergrowth and failures of for-
mation in the upper extremity. To accomplish this,
S.A. Riley and R. Burgess
the focus will be on the clinical presentation,
associated ndings, and treatment considerations
for these conditions.
An additional point to consider is that cleft
hands, transverse deciencies, and phocomelia
are among the most obvious and disguring of
all congenital anomalies. With the advent of
advanced prenatal imaging, the accuracy in diag-
nosing congenital upper extremity anomalies in
utero is improving. As such, parents may consult
the orthopedic surgeon about these disorders in
the prenatal period. For a source of guidance
about these matters, Bae, et al. provide an excel-
lent discussion about the current status of prenatal
screening as well as ethical and treatment consid-
erations involved with the implementation of this
technology (Bae et al. 2009).
Hypoplasia
Hypoplasia is dened as a small or underdevel-
oped body part. In the upper extremity, any ana-
tomic segment such as a digit, a hand, or even the
entire arm may be considered hypoplastic when
compared to its normal counterpart in the contra-
lateral extremity. Despite its small size, a hypo-
plastic body part may well be very functional, and
when possible, every effort should be attempted to
preserve or augment these affected, yet functional
units.
The exact incidence of hypoplasia in the upper
extremity is difcult to determine because in
many congenital anomalies that are encountered,
a hypoplastic anatomic part can also be identied
as a part of the main condition. However, if one
considers isolated hypoplasia of a single upper
extremity segment with no other associated mus-
culoskeletal decits, the condition represents
about 8 % of all congenital upper extremity anom-
alies (Giele et al. 2001). Yet it is suspected that this
number may be articially small due to
underreporting of hypoplasia when the upper
extremity remains very functional.
The inheritance patterns of hypoplastic anom-
alies are thought to be sporadic in nature unless
the condition is associated with a specic syn-
drome. For example, many children with Feingold
10 Hypoplasia, Brachydactyly, and Other Failures of Formation 215

Table 1 Embryological classification of congenital anomalies

Classication Subheading Subgroup Category
I. Failure of formation
A. Transverse arrest
1. Shoulder
2. Arm
3. Elbow
4. Forearm
5. Wrist
6. Carpal
7. Metacarpal
8. Phalanx
B. Longitudinal arrest
1. Radial deciency
2. Ulnar deciency
3. Central deciency
4. Intersegmental Phocomelia
II. Failure of differentiation
A. Soft tissue
1. Disseminated (a) Arthrogryposis
2. Shoulder
3. Elbow and forearm
4. Wrist and hand (a) Cutaneous syndactyly
(b) Camptodactyly
(c) Thumb-in-palm
(d) Deviated/deformed
digits
B. Skeletal
1. Shoulder
2. Elbow Synostosis
3. Forearm (a) Proximal
(b) Distal
4. Wrist and hand (a) Osseous syndactyly
(b) Carpal bone synostosis
(c) Symphalangia
(d) Clinodactyly
C. Tumorous
conditions 1. Hemangiotic
2. Lymphatic
3. Neurogenic
4. Connective tissue
5. Skeletal
III. Duplication
A. Whole limb
B. Humeral
C. Radial
D. Ulnar
1. Mirror hand
(continued)
216
Table 1 (continued)
Classication Subheading
E. Digit
IV. Overgrowth A. Whole limb
B. Partial limb
C. Digit
V. Undergrowth
A. Whole limb
B. Whole hand
C. Metacarpal
D. Digit
VI. Constriction band syndrome
VII. Generalized skeletal
abnormalities
syndrome (inherited as autosomal dominant) are
found to have small second and fth ngers as
part of the disorder. Thumb hypoplasia, which is
discussed in detail in another chapter, can be seen
in association with Fanconi anemia, an autosomal
recessive disorder. As such, if hypoplasia is seen
in conjunction with anomalies, a formal genetic
consultation is probably indicated, and additional
diagnostic testing may be needed to rule out other
organ system involvement.
The clinical presentation of a child with hypo-
plasia can be quite variable. Obvious size differ-
ences are usually easy to detect, but the best way
to identify subtler forms is to directly measure the
region of concern and compare it to the contralat-
eral upper extremity. In these mild forms of hypo-
plasia, radiographs may aid in conrming the
diagnosis. In addition to noting potential size dif-
ferences, comparison radiographs can also show
other anomalies such as missing carpal bones,
carpal coalitions, and malformed phalanges.
Additional testing modalities such as MRI and
CT scanning are usually not necessary for diag-
nostic purposes.
Any treatment considerations for the patient
with hypoplasia should focus on maximizing
S.A. Riley and R. Burgess
Subgroup Category
1. Polydactyly (a) Radial (preaxial)
(b) Central
(c) Ulnar (postaxial)
1. Macrodactyly
1. Brachysyndactyly
2. Brachydactyly
function of the involved extremity. Often, this
will require following the patient for an extended
period of time, before recommending any surgical
interventions, in order to understand just how the
child uses the extremity most efciently. In addi-
tion to getting subjective input about hand usage
from the family, occupational therapists can pro-
vide objective functional data by administering
age-appropriate testing modalities and thus offer
valuable insights during this process.
Most patients with mild hypoplasia are very
high functioning and require no surgical treatment
for the condition. These children may need to alter
the manner in which a task needs to be performed;
yet they adapt very well in most task-oriented
situations. There are, however, a few instances
where surgery can offer benet to these patients.
One example would be if a child has a small,
oppy digit that gets in the way of gripping or
pinching tasks. In this case, hand use is being
compromised, and amputation of that digit should
be considered. In contrast, a small, stable nger
with good tendon function might be made more
useful by bone lengthening (Arata et al. 2011),
and reconstruction is the best surgical option for
this particular digit. It is equally important that the
10 Hypoplasia, Brachydactyly, and Other Failures of Formation 217

Table 2 Oberg, Manske, and Tonkin (OMT) classification

1. Malformations
A. Failure of axis formation/differentiation entire upper limb
1. Proximal-distal outgrowth
Brachymelia with brachydactyly
Symbrachydactyly
Transverse deciency
Intersegmental deciency
2. Radial-ulnar (anteroposterior) axis
Radial longitudinal deciency
Ulnar longitudinal deciency
Ulnar dimelia
Radioulnar synostosis
Humeroradial synostosis
3. Dorsal-ventral axis
Nail-patella syndrome
B. Failure of axis formation/differentiation hand plate
1. Radial-ulnar (anteroposterior) axis
Radial polydactyly
Triphalangeal thumb
Ulnar polydactyly
2. Dorsal-ventral axis
Dorsal dimelia (palmar nail)
Hypoplastic/aplastic nail
C. Failure of axis formation/differentiation unspecied axis
1. Soft tissue
Syndactyly
Camptodactyly
2. Skeletal deciency
Brachydactyly
Clinodactyly
Kirners deformity
Metacarpal and carpal synostoses
3. Complex
Cleft hand
Synpolydactyly
Apert hand
2. Deformations
A. Constriction ring sequence
B. Arthrogryposis
C. Trigger digits
D. Not otherwise specied
3. Dysplasias
A. Hypertrophy
1. Macrodactyly
2. Upper limb
3. Upper limb and macrodactyly
B. Tumorous conditions
218
Fig. 1 Fourteen-year-old
female with bilateral ring
and small short metacarpals
otherwise known as
knuckle-knuckle-bump-
bump (Courtesy of
Shriners Hospitals for
Children, Philadelphia)
patients family has a very clear understanding of
the expected goals of any type of surgery. Maxi-
mizing the childs use of the extremity should be
the primary goal, and focusing on this goal may
help dismiss any unrealistic expectations regard-
ing treatment outcomes.
Treatment Strategy
Because the majority of children with hypoplasia
of a portion of the upper extremity are very high
functioning, they require no surgical care. None-
theless, there are two instances where surgical
treatments are helpful. The rst indication is to
remove a oppy digit that is interfering with the
functional abilities of the hand. In such cases,
excising the affected digit and reconstructing the
local web space are recommended as surgical
care. A short course of occupational therapy fol-
lowing the procedure may be helpful as well. The
second circumstance, which is discussed in
another chapter, involves reconstructing and
improving the function of a hypoplastic thumb.
Brachydactyly and Symbrachydactyly
After considering hypoplasia of the upper extrem-
ity in general, a discussion of the more common
S.A. Riley and R. Burgess
clinical types is appropriate. Flatt reported that out
of 2,758 collected cases of congenitally anoma-
lous hands, brachydactyly (5.2 %) and thumb
hypoplasia (3.5 %) were the most frequently
seen undergrowth conditions (Flatt 1994a).
Because thumb hypoplasia is discussed as a sep-
arate topic elsewhere, this section will focus on
brachydactyly and symbrachydactyly.
The term brachydactyly as translated from
Greek means short nger. While the whole
digit may appear to be small, on radiographic
examination, an individual bone segment is often
smaller than normal. When the phalanges are
involved, the middle phalanx is the most com-
monly affected bone. In this instance, the condi-
tion may be referred to as brachymesophalangia
(short middle phalanx). Short metacarpals can be
seen in association with some syndromes (e.g.,
Turners), so the term brachymetacarpia may be
encountered in the literature (Fig. 1). It should be
stated that these terms are purely descriptive and
do not imply that one type consistently has a more
favorable prognosis than others.
Most forms of brachydactyly are inherited in
an autosomal dominant manner, usually with var-
iable severity. However, there are some cases of
brachydactyly are felt to be sporadic in occurrence
because of no well-documented family inheri-
tance pattern. On the molecular level, alterations
in BMP cartilage-derived morphogenic protein
10 Hypoplasia, Brachydactyly, and Other Failures of Formation 219

Fig. 2 Fourteen-year-old
female with Grebes
chondrodysplasia and
severe brachydactyly
associated with a cartilage-
derived morphogenetic
protein deciency
(Courtesy of Shriners
Hospital for Children,
Philadelphia). (a) Right
hand, (b) left hand, (c) right
foot, (d) left foot

have been shown to be associated with
brachydactyly in humans and mouse models
(Waters and Bae 2012a; Fig. 2). A classication
system for brachydactyly, rst proposed by Bell in
1951 (Fitch 1979), focuses on the anatomic loca-
tion of the hypoplastic segment to categorize the
anomaly (Table 3). This system has been most
useful in the identication of certain inheritance
patterns by being able to follow specic types
along family pedigrees. For example, the most
common types, A3 and D, have been shown to
demonstrate autosomal dominant inheritance with
reduced penetrance. Some populations also have a
relatively high frequency of certain types of
brachydactyly (Temtamy and Aglan 2008). As
far as the clinical presentation is concerned, the
degree to which any digit is involved may range
from a small, but normal appearing nger to a
tiny residual nubbin, which may be nothing
more than a small pouch of skin attached to the
hand.
When evaluating a patient with small digits,
the term symbrachydactyly may be encountered
in the congenital hand anomalies literature.
220 S.A. Riley and R. Burgess

Table 3 Brachydactyly types (After Temtamy and Aglan 2008)

Type Inheritance
A1 Autosomal dominant
Rare
A2 Autosomal dominant
Very rare
A3 Autosomal dominant
Common (up to 21 % in some
populations)
A4 Few pedigrees identied
Rare (autosomal dominant)
B Few pedigrees identied
Rare (autosomal dominant)
C Few pedigrees identied
Rare (? autosomal dominant)
D Autosomal dominant (up to 4 % in
some populations)
E Autosomal dominant
Proposed gene defect Clinical features
Indian hedgehog Short middle phalanges (usually digits
IIV)
BMP receptor on q4 Short middle phalanx (digit II)
Unknown Short middle phalanx (digit V)
Mutation: HOXD13 Short middle phalanges (digits II and V)
Mutation: ROR2 Absence/hypoplasia of terminal digits
(IIV), with nail absence
Distal phalanx of thumb may be
duplicated
Mutation: CDMP1 Brachymesophalangy of digits II, III, and
V
Ring nger (digit IV) is usually normal in
size
Unknown (possible Short distal phalanx of digit I (thumb)
mutation: HOXD13)
Unknown (possible Variable shortening of metacarpals (often
mutation: HOXD13) digit IV)

Fig. 3 Short nger

symbrachydactyly is
characterized by the triad of
syndactyly, brachydactyly,
and symphalangism
(Courtesy of Shriners
Hospitals for Children,
Philadelphia)

As dened, symbrachydactyly is the condition peromelic, oligodactylic, short nger, and

representing hypoplastic digits (brachydactyly),
webbing between ngers (syndactyly), and a gen-
eral hypoplasia of the hand. To avoid confusion,
brachydactyly refers specically to the digit,
whereas symbrachydactyly describes a spectrum
of clinical ndings seen in the hand.
A descriptive classication system has identi-
ed four separate types of symbrachydactyly:
monodactylic (Nguyen and Jones 2009) based
upon the most commonly seen anatomic features
(Figs. 3 and 4). Symbrachydactyly is often seen
unilaterally, and the occurrence is felt to be
sporadic in nature. It is secondary to a failure
of formation, but the exact manner by which
this occurs continues to be investigated.
Symbrachydactyly can be seen in association
10 Hypoplasia, Brachydactyly, and Other Failures of Formation 221

Fig. 4 Monodactylic symbrachydactyly with persevera-

tion of the thumb (Courtesy of Shriners Hospitals for
Children, Philadelphia)

Fig. 5 A young girl with a Polands syndrome denoted by

with other conditions, and the classic example is the absence of the pectoralis muscles and the breast nipple
Polands syndrome (absence of the sternal head of (Courtesy of Shriners Hospitals for Children, Philadelphia)
the pectoralis major muscle with various degrees
of ipsilateral hand hypoplasia; Fig. 5). This con-
dition has recently been reported in association
with transverse deciency at the level of the fore-
arm (Kallemeier et al. 2007). The term atypical
cleft hand is also seen describing cases of
symbrachydactyly.
Although symbrachydactyly and constriction
band syndrome (CBS, discussed in another chap-
ter) can appear similar in clinical appearance, the
conditions have different diagnostic and treatment
Fig. 6 Constriction band syndrome around the index and
implications. There are two distinct differences.
long ngers with normal musculoskeletal structures prox-
First, in patients with CBS, actual bands are usu- imal to the banding (Courtesy of Shriners Hospitals for
ally identied elsewhere in the body. However, in Children, Philadelphia)
symbrachydactyly, only one hand is commonly
affected, and no other bands are seen. Second, While classication schemes regarding
with symbrachydactyly, the anatomic structures brachydactyly and symbrachydactyly are helpful
proximal to the small digits are also hypoplastic in describing the childs clinical presentation,
or abnormal. In CBS, the musculoskeletal struc- these systems are unable to predict the childs
tures proximal to a constriction band are normal in ability to use the hand. Any surgical recommen-
size (Fig. 6). dations should focus on improving a childs
222
functional outcome as function trumps form.
Input from the family about hand use, evaluations
from therapists, and direct observation of
the patient are critical in the decision-making
process. Many patients with brachydactyly and
symbrachydactyly are highly functional, adapt
well to their hand anomaly, and do not warrant
surgical intervention. They may occasionally
need an assistive device to help with a specic
task, however; surgery would not appreciably
improve their function. There are cases that war-
rant surgery to augment hand use.
Probably the most common surgical technique
to augment function involves altering the skin
about the affected digit(s) through syndactyly
release and/or web space reconstruction. Because
the care of syndactylized ngers is presented in
another chapter, a brief summary of web space
reconstruction will be discussed here. The main
goals of this procedure are to increase digital
separation (span) and improve the apparent length
of the ngers by providing depth to the web space.
The Z-plasty and its varied modications are the
most common methods used to improve the web
space (Shaw et al. 1973). Strict attention to detail
is needed when designing the angles and arms
length of ap transpositions in order to maximize
skin mobilization. Also, great care must be used
when handling the transposed aps so that tension
on the skin is minimized. In most cases, well-
designed skin aps do not require additional soft
tissue coverage. However, if skin is needed, a full-
thickness skin graft can be used to supplement the
reconstruction. As mentioned in the treatment of
syndactyly, skin grafts should be avoided in the
web space commissure due to their propensity to
contract.
The actual length of a nger can be improved
in two ways. For minor changes in digit length, an
osteotomy and interposition bone grafting can be
used. While this technique allows for immediate
gains in length, the concern is that inserting too
large a graft in a small digit may compromise the
surrounding skin and soft tissue structures leading
to necrosis of the ngertip (Flatt 1994b). An alter-
native is distraction osteogenesis,which involves
gradual lengthening of the bone and the soft tis-
sues. There have been many methods described to
S.A. Riley and R. Burgess
achieve this task (Arata et al. 2011), although the
basic technique requires an osteotomy and a
device to gradually distract (lengthen) the
osteotomy site. The most common types of
distractors are uni-plane external xators,
attached to the bone by small wires. The slow
distraction rate, about 0.5 mm to1mm per day,
allows for both fracture callus formation and the
accommodation of the soft tissues necessary for
bone lengthening. Gains in digital length of up to
3.5 cm have been reported (Seitz and Froimson
1995). After the bone is lengthened to the desired
amount, the distraction ceases and the external
xator is left in place until the regenerate callus
matures to resemble normal bone (i.e., a cortical
shell and medullary cavity). Subsequently, the
device is removed and appropriate therapy is
begun. Early removal can result in callus defor-
mation or overt fracture through the
regenerative bone.
Nonvascularized toe phalangeal transfer is a
method used to achieve immediate length in a
hypoplastic digit. The technique typically
involves harvesting the proximal phalanx from a
toe, then transplanting it within the skin of a
hypoplastic digit. This procedure is controversial
with regard to indications and outcome. The best
results are when the procedure is done before
18 months of age, as the rates of physeal arrest
and phalangeal resorption increase beyond this
age (Nguyen and Jones 2009). Preliminary
nonvascularized toe phalangeal transfer followed
by distraction lengthening of the phalanx has been
performed (Netscher and Lewis 2008). A recent
article has shown that there is potential donor site
morbidity with the nonvascularized toe phalan-
geal transfer, thus narrowing the indications for
he procedure (Garagnani et al. 2012).
The most immediate way to provide both
length and function to the brachydactylous hand
is through microvascular toe-to-hand transfer.
Although this is a technically demanding proce-
dure, it probably provides a child the best oppor-
tunity to develop functional pinch and grasp
(Nguyen and Jones 2009). Most toe-to-hand
transfers are done at the level of the metacarpo-
phalangeal joint, and the second toe is the most
common donor digit. There is no generally
10 Hypoplasia, Brachydactyly, and Other Failures of Formation
accepted age for surgical intervention, but due to
the size of the structures involved, the procedure is
generally performed after 18 months of age. Expe-
rienced surgeons are reporting a successful toe-to-
hand transfer rate of 95 % or more (Jones
et al. 2007), and the long-term outcomes indicate
continued growth of the transferred toe as well as
improved function in the hand (Nguyen and Jones
2009).
Treatment Strategy
For the brachydactylous digit, the mainstay of
surgical treatment for a functioning nger is web
space deepening or reconstruction. This proce-
dure is recommended on one side of a nger at a
time to lessen the chance of venous or arterial
vascular compromise to the digit. The other side
of the digit web is usually reconstructed 3 months
later. Increasing the length of a phalanx or meta-
carpal either by direct grafting or by osteotomy
distraction can improve both function and appear-
ance of the nger. However, the gains in bony
length may result in tendon mechanism dysfunc-
tion. As the bone is lengthened, the adjacent ten-
dons become relatively shorter and can promote
joint contractures.
In the case of symbrachydactyly, the primary
goal is for the hand to be able to hold an object
between two stable posts. For the child that has
good thumb function and a reasonably sized ulnar
digit, performing a web space reconstruction to
optimize the span between the ngers is the only
treatment that is required. If the border digits are
small, it is important to assess the thumb for a
functional carpometacarpal joint that allows a
mobile post. If the thumb acts as a mobile
post, then augmenting the size of the digits can
improve the hands overall function. The accom-
panying digit(s) can be augmented by a
nonvascularized toe phalangeal transfer, on-top-
plasty of adjacent digit, or distraction lengthening
of the metacarpal. For these treatment methods,
key points to remember are:
1. The viability and growth potential of a
nonvascularized toe phalangeal transfer is
223
maximized when done in a patient less than
18 months of age, although the indications
have narrowed.
2. Distraction lengthening, because it is done
gradually and over a time period of up to sev-
eral weeks, requires close monitoring of the
patient.
3. On-top-plasty is a relatively difcult procedure
requiring competency in pediatric hand sur-
gery (Fig. 7).
Cleft Hand
Cleft hand
Nonoperative management
Indications Contraindications
Few indications before surgery None
Determining prehensile pattern of
the child
Cleft hand
Physical/occupational therapy recommendations
Assessment of prehensile function
Education of parents
Participate in the decision-making process
Cleft hand is one of the more striking congen-
ital anomalies encountered by the hand surgeon.
The condition represents a failure of formation
characterized by a longitudinal deciency of the
central rays of the hand. There are two distinct
types of cleft hands typical and atypical cleft
hands. Although these forms can appear similar,
there are important distinctive differences. Atypi-
cal cleft hand is really a form of
symbrachydactyly. Atypical cleft hand is usually
unilateral, presents with a U-shaped central
defect, and occurs spontaneously (Miura and
Suzuki 1984; Fig. 8). The classic cleft hand has
a V-shaped defect in the central portion with var-
iable proximal extension (Fig. 9). Extreme cases
may extend into the carpal bones. The adjacent
border digits can have syndactyly (Kozin 2003).
The typical patient has bilateral involvement,
although the extent is variable. In addition, clefts
of the feet as well as cleft lip and palate are
224
Fig. 7 Three-year-old male with brachydactyly of both
hands (Courtesy of Shriners Hospitals for Children, Phila-
delphia). (a) Right hand is missing the terminal portions of
the thumb, index, and long nger. On-top-plasty with
placement of the index nger on top of the thumb was
recommended. (b) X-rays coincide with clinical picture.
Thumb with adequate carpometacarpal joint and extra
metacarpal between thumb and index. (c) Skin design for
S.A. Riley and R. Burgess
on-top-plasty. (d) Exposure of extra metacarpal between
thumb and index. (e) Removal of extra metacarpal. (f)
Dorsal dissection with preservation of veins. (g) Transpo-
sition of index on top of thumb metacarpal. (h) Skin
closure with wide thumb-index web space and elongated
thumb. (i) X-ray following healing of index metacarpal to
thumb metacarpal. (j) Clinical outcome with ability to open
for pinch. (k) Grasping of sticker
10 Hypoplasia, Brachydactyly, and Other Failures of Formation
associated with the hand anomaly. The incidence
of cleft hands is reported as 1:10,0001:90,000
live births (Barsky 1964; Ogino et al. 1986). The
inheritance pattern is often autosomal dominant
with variable penetrance, although autosomal
recessive and X-linked forms have been
documented. The genetic factors for certain
forms of the condition have been identied in
patient with split-hand-foot malformation (Dlx
homeobox abnormalities) and ectrodactyly-ecto-
dermal dysplasias (transcription factor gene p63
affecting Dlx homeobox regulation) (Waters and
Bae 2012b). Failure in the maintenance of the
Fig. 8 Atypical cleft hand (a.k.a. symbrachydactyly) with
unilateral U-shaped central defect (Courtesy of Shriners
Hospitals for Children, Philadelphia)
Fig. 9 Typical bilateral
cleft hand with V-shaped
defect in the central portion
(Courtesy of Shriners
Hospitals for Children,
Philadelphia)
225
central portion of the apical ectodermal ridge is
the proposed etiology (Al-Qattan and Kozin
2013). In an animal model, chemically induced
abnormalities in digital ray formation have indi-
cated lead to central polydactyly, syndactyly, and
central deciency (Naruse et al. 2007).
Classifying the various forms of typical cleft
hand continues to be a challenging task due to the
wide variability in clinical ndings. The three
most often-referenced classication schemes
describe the deformity according to either the
number of digits missing (Nutt and Flatt 1981),
the number of rays present together with associ-
ated bony syndactyly and polydactyly (Ogino
1990), or the functional integrity of the thumb-
index nger web space (Manske and Halikis
1995). While each of these classication systems
describe the anomaly differently, they are individ-
ually important in that they identify specic ele-
ments seen in cleft hand that need to be addressed
when considering treatment alternatives.
Because the cleft hand deformity can be strik-
ing in appearance, its mere presence can be a
source of stress for the family. During the initial
evaluation, the examiner can comfort the situation
by resisting the temptation to immediately focus
on the hand condition. Also, being sensitive to the
familys fears and concerns can be benecial
toward establishing a good physician-patient rela-
tionship that will last many years. The importance
of allowing time for questions cannot be
226
Fig. 10 X-ray of a 3-year-old with cleft hand.
Anteroposterior view shows a proximal phalanx within
the cleft oriented in a transverse direction (Courtesy of
Shriners Hospitals for Children, Philadelphia)
overemphasized. When it can be arranged, most
families are referred for an evaluation by a genet-
ics specialist. When evaluating the affected hand,
a thorough assessment of every anatomic struc-
ture is critical. Take note of what is present and
what is missing, of which structures look nor-
mal and those that appear abnormal. The location
and size of the cleft is important as well as any
compromise of the thumb-index web space. Most
clefts are found in the center of the hand, and
various forms of additional phalangeal or meta-
carpal irregularities (such as hypoplasia, duplica-
tions, and unusual shapes) can be present (Falliner
2004) (Fig. 10). Obtaining radiographs of the
affected extremities is helpful for assessing bony
anatomy, but the ndings can be misleading in the
very young patient due to incomplete or delayed
ossication of abnormal bony elements. Thus,
serial radiographs as the child grows provide bet-
ter information in regard to potential surgical
treatment plans.
Before discussing specic operative proce-
dures, it is important to state that a number of
these children do not require surgery to improve
their function. In spite of the obvious cleft in the
hand, they are able to pinch and grasp both small
and large objects with remarkable dexterity. Even
hands with a single mobile digit can function at a
high level (Fig. 11). Therefore, any proposed sur-
gery must be designed to enhance function.
S.A. Riley and R. Burgess
Having the patients hand assessed by a qualied
therapist can be very helpful in formulating treat-
ment plans. Lastly, there should be no pressure to
operate early on cleft hands. As the child grows,
observing their abilities over time allows for more
condence when recommending a surgical
procedure.
While there is no single right way to surgi-
cally treat cleft hand, however, there are some
general established treatment principles. These
principles focus on functional improvement and
include altering the conguration of the cleft,
maximizing the position of the thumb, and
improving the functionality of stiff or malaligned
digits (Waters and Bae 2012b). In addition,
improving the aesthetic appearance of the hand,
particularly those with severe deformities, is a
reasonable request of many families. Fortunately,
decreasing the depth of the cleft will enhance both
the appearance and function. However, the family
should be cautioned that surgery done solely for
aesthetic purposes may be disappointing as no
operation will produce normal looking hands.
Before discussing specic surgical procedures,
an additional clinical factor needs to be consid-
ered. Closure of the cleft can be proposed for any
patient with cleft hand. In most hands, reconstruc-
tion of the cleft is a worthwhile endeavor because
holding small objects within the palm is easier and
the aesthetic appearance improved. However, in a
few children (e.g., children with only two or three
digits), the cleft enhances function for grasping
large objects. Cleft closure is contraindicated as
function trumps form.
The technique of cleft closure varies with
extent and particular anatomy. Minor clefts may
be treated by Z-plasty incisions combined with a
hexagonal ap (Barsky ap) from a digit adjacent
to the cleft for reconstruction of the web commis-
sure. A deeper (or wider) cleft may require recon-
struction of intermetacarpal ligament between the
heads of the metacarpals. Local tissue or tendon
graft can be used to connect the metacarpal heads
to lessen the chances of the digits splaying with
hand growth (Ogino 1990). The metacarpal
physis must be avoided during intermetacarpal
ligament reconstruction. If anomalous bony struc-
tures are encountered in the cleft, reconstruction
10 Hypoplasia, Brachydactyly, and Other Failures of Formation
Fig. 11 Six-year-old boy
with bilateral cleft hands
that have only a single
mobile digit that functions
at a high level (Courtesy of
Shriners Hospitals for
Children, Philadelphia). (a)
Clinical picture of both
hands. (b) Holding a
hockey stick. (c)
Wakeboarding
becomes more challenging. These abnormal pha-
langes or metacarpals are often small and poorly
positioned, so excision is usually the best treat-
ment. There are occasions, however, when these
bones provide structural support to a joint and
partial preservation is necessary during cleft
reconstruction. When the middle metacarpal is
completely absent, closure of the cleft space can
be impeded by the carpometacarpal articulations
of the adjacent ngers. For these hands, a
V-shaped osteotomy in the center of the carpals
or transferring the second metacarpal base to a
more central position will allow for improved
closure and digit alignment (Ogino 1990).
As the severity of the cleft increases, the status
of the thumb-index web space usually becomes
more narrowed. The thumb-index web space must
be addressed. If this web interval is merely
227
contracted, employing single or multiple
Z-plasties widens the web space (Riley and
Burgess 2009). However, when the thumb and
index nger are almost syndactylized (Manske
and Halikis Types IIB & III), reconstructing the
web space requires the use of additional skin. In
these cases, additional soft tissue coverage for the
web is achieved by using a dorsal transposition
skin aps (Flatt 1994c).
Eloquent and technically demanding proce-
dures have been described for treating both the
deep cleft and the thumb web contracture simul-
taneously (Snow and Littler 1967; Rider
et al. 2000; Miura and Komada 1979; Upton and
Taghinia 2010). The basic concept is to widen the
thumb-index web space, close the cleft, and resur-
face the thumb-index commissure with skin from
the cleft. In order to optimize hand function,
228
additional abnormalities like interphalangeal joint
exion contractures, angulation deformities, or
anomalous bony structures can also be addressed
at the same time. Subsequent hand therapy and
splints designed to maintain the thumb-index
web space can be benecial. Potential complica-
tions include skin ap necrosis and pin tract
infection.
Technique: Snow-Littler
Reconstruction of Cleft Hand
Cleft hand
Preoperative planning
OR table: regular
Position/positioning aids: supine
Fluoroscopy location: ipsilateral
Equipment: standard, wire driver, K-wires
Tourniquet: sterile
The patient is placed supine on the operating
room table (Fig. 12a, b). The procedure is usually
performed under general anesthesia. A single dose
of intravenous preoperative antibiotics is admin-
istered. The limb is prepped and draped in sterile
fashion. Chlorhexidine gluconate and alcohol
prep (ChloraPrep; CareFusion, Leawood, Kansas,
USA) is preferred, which may be more effective in
eliminating bacteria and avoids iodine that can
migrate beneath the tourniquet and cause burns
(Saltzman et al. 2009). A sterile pediatric tourni-
quet (Del Medical Innovations, Vancouver,
Canada) is placed on the upper arm that exsangui-
nates during application.
The hand is carefully examined in anticipation
of web space widening and cleft closure
(Fig. 12c, d) Initial attention is toward the design
of the palmar-based skin ap from the cleft
(Fig. 12e, f). Lines are drawn along the dorsum
of the ring and index ngers coalescing in a
V-shaped apex and the bottom of the cleft. The
incisions course around the sides of the ring and
small metacarpophalangeal joint toward the palm.
The proximal extent is to the level of the apex of
the dorsal V. A small Barsky ap is designed from
the adjacent ring nger for commissure recon-
struction after clef closure. An additional incision
S.A. Riley and R. Burgess
is necessary to separate or widen the thumb-index
web space. In cases that require only widening, a
straight incision is sufcient. In cases with syn-
dactyly, then a zigzag incision will need to be
incorporated into the design to accommodate syn-
dactyly separation.
The web space ap is elevated rst from the
dorsal side (Fig. 12g). The skin is sharply incised
and dissection carried directly to the paratenon.
The dorsal veins are ligated and preserved within
the ap. Once the dorsal aspect is raised, dissec-
tion proceeds from the dorsal side to the palmar
side (Fig. 12h). On occasion, an artery entering
the ap van be preserved, which changes the ap
from random to axial. This axial ap is much more
robust compared to the random ap. The
neurovascular bundles to the index and ring nger
are identied and protected (Fig. 12i).
Once the ap is raised, attention is directed
toward separation of the thumb and index and
widening of the thumb-index web space. The
skin is incised and the brous interconnections
cut (Fig. 12j). The web space is widened with
slow and deliberate dissection. The princeps
pollicis artery and its braches must be identied.
The intervening muscles, such as the rst dorsal
interosseous and adductor pollicis, may require
release until adequate widening has been obtained
(Fig. 12k, l).
Attention is then directed to the index nger. In
cleft hands with a long nger metacarpal, the
index nger is transpose to the long position. In
cleft hands without a long nger metacarpal, a
closing wedge osteotomy at the base of the index
is performed to close the cleft and align the
nger and widen the thumb-index web space
(Fig. 12m, n). Fixation is usually accomplished
with Kirschner wires (Fig. 12o). Addressing the
bony alignment negates the necessity of
intermetacarpal ligament reconstruction. The sur-
geon must ensure that bony alteration does not
result in digital scissoring.
A small Barsky ap from the long nger is
raised and inset to reconstruct the commissure
(Fig. 12p). The cleft skin is transposed into the
widened thumb-index web space (Fig. 12q). The
skin is trimmed and closed with 50 plain suture
(Fig. 12r, s). Following closure, the tourniquet is
10 Hypoplasia, Brachydactyly, and Other Failures of Formation
Fig. 12 Sixteen-month-old child with bilateral cleft hand
(Courtesy of Shriners Hospitals for Children, Philadel-
phia). (a) Left hand dorsal view. (b) Left hand palmar
view. (c) Narrow thumb-index web space. (d) Intended
cleft closure. (e) Dorsal skin design. (f) Volar skin design.
(g) Flap elevation from dorsal to palmar with preservation
of an axial artery to the ap. (h) Continued dissection along
the palmar aspect of the hand. (i) Protection of the adjacent
neurovascular bundles. (j) Incision for widening of thumb-
index web space. (k) Protection of princeps pollicis artery
and division of intervening muscle. (l) Volar view
229
following adequate release of the thumb-index web
space. (m) Closing wedge osteotomy at the base of the
index to close the cleft and align the nger. (n) Improved
alignment of index nger and cleft closure following
osteotomy. (o) Kirschner wire xation of osteotomy. (p)
Barsky ap elevated from long nger for commissure
reconstruction. (q) Cleft skin rotated into widened
thumb-index web space. (r) Dorsal closure. (s) Palmar
closure. (t) Incorporation of hand into activities of daily
living, such as grasping a large object
230
deated to ensure capillary rell to all digits.
Adequate uffy dressings are necessary to equal-
ize the anterior-posterior and medial-lateral
dimensions of the hand. This dressing allows
uniform compression without constriction.
A long-arm soft cast (3M Scotchcast Soft
Cast Casting Tape, St. Paul, Minnesota, USA) is
applied with the elbow exed to greater than 100
to decrease the chance of accidental removal. This
berglass casting tape does not harden
completely, but remains slightly exible when
cured. More importantly, soft cast can be
unwrapped in the clinic avoiding the petrifying
cast saw. The child is admitted overnight with the
arm elevated to promote venous drainage. The
entire hand can be covered the next day if vascu-
larity has been maintained throughout.
Cleft hand
Surgical steps
Limb is exsanguinated and the tourniquet is inated
Cleft ap design along with skin incision for thumb-
index web space release
Elevate dorsal cleft ap at the level of paratenon with
venous preservation to the ap
Elevate volar portion of ap with protection of index and
longer nger neurovascular bundles
Look for potential arterial axial supply to ap
Incise skin between thumb and index nger
Gradual release and widening of thumb-index web space.
Protect princeps pollicis artery
Index nger transposition or wedge osteotomy dependent
upon osseous anatomy
Kirschner wire xation
Inset cleft ap into thumb-index web space
Cleft closure with Barsky ap for commissure
Skin is closed with absorbable suture, and the limb is
immobilized in a long cast for 4 weeks
Cleft hand
Postoperative protocol
Limb is immobilized in a long-arm cast for 4 weeks
Kirschner wire is removed and a short arm splint is
fabricated
Active range of motion and scar care is initiated
Gradual incorporation of hand into activities of daily
living (Fig. 12t)
Passive range of motion is begun to prevent digital
stiffness once bony union is conrmed
S.A. Riley and R. Burgess
In summary, the child with cleft hand anomaly
has a challenging condition. Some cleft hands
represent functional triumphs and aesthetic
disasters (Upton and Taghinia 2010, p. 480).
Treating cleft hand is not for the inexperienced
surgeon or for the faint of heart. A detailed eval-
uation of the child, his or her hand, and their
functional abilities is critical prior to
recommending any surgery. As function trumps
form, not recommending surgery may be difcult
for the family to accept. When surgery is advised,
one must make certain that the family understands
the details of the procedure and the anticipated
benets. Unrealistic aesthetic and functional
expectations must be avoided. The family must
understand that as the childs hand grows, revision
operations to address joint contractures, web
space contractures, and angular deformities of
the digits may be necessary. Hence, frequent and
regular follow-up evaluations are until the childs
growth is complete.
Transverse Deficiencies
Another failure is congenital transverse de-
ciency. This anomaly can occur at any level from
the phalanges to the humerus and is named
according to the most distal remaining bone seg-
ment. In the typical patient, the condition is uni-
lateral and is not seen with other congenital
abnormalities. This deformity occurs sporadi-
cally, and familial involvement has not been dem-
onstrated. The condition should not be confused
with constriction band syndrome (discussed else-
where in this textbook), whereby the entrapment
of developing tissue by an amniotic band may
lead to a transverse-type amputation of the
extremity. The clinical appearance of these two
conditions is at times quite similar, but if the
diagnosis is in question, the patient with amniotic
band syndrome often shows signs of additional
bands or creases in the affected limb or other
extremities. Another difference is that transverse
deciencies generally occur more proximal com-
pared to amputations secondary with constriction
band syndrome (Ogino and Saitou 1987). In a
review of published series, the incidence of the
10 Hypoplasia, Brachydactyly, and Other Failures of Formation
Fig. 13 A transverse deciency of the proximal one-third
of the forearm with terminal nubbins (Courtesy of Shriners
Hospital for Children, Philadelphia Unit)
transverse deciencies has been estimated to be
about 6 % of all congenital upper extremity anom-
alies (Jain and Lakhtakia 2002).
Amputation at the level of the forearm is the
most commonly encountered transverse de-
ciency of the upper extremity. The proximal
one-third of the forearm is most frequent (Kozin
2003; Fig. 13). The deciency can also be found at
the hand and wrist regions, but transverse loss at
the humeral level is rare (Knight et al. 2012).
There are variations in the underlying bony struc-
tures and the type of tissues seen at the distal
portion of the limb. When the amputation occurs
in the forearm, there have been additional bony
anomalies identied such as radioulnar synostosis
and radial head dislocation (Jain and Lakhtakia
2002). With reference to overlying skin, some
patients have a bulbous-like skin and soft tissue
coverage. Additionally, structures like nger nub-
bins (that may have nails), skin invaginations, and
even varying degrees of bone hypoplasia are
commonly seen. Because the rudimentary
nubbin-like structures are similar to those seen in
symbrachydactyly, recent work has investigated
the relationship between the two conditions. The
authors propose that symbrachydactyly and trans-
verse deciency represent a continuum with dif-
ferent severities of a similar mesodermal process
(Kallemeier et al. 2007). The etiology of the trans-
verse deciency is thought to be due to disruptive
events affecting the apical ectodermal ridge after
the limb bud forms (Al-Qattan and Kozin 2013).
231
In terms of functional adaptation, the child that
presents with an isolated upper extremity trans-
verse deciency is often able to adjust to their
difference. In fact, a recent study evaluated chil-
dren with unilateral congenital below-elbow de-
ciency found that compared to the general
population, they do not perceive their health-
related quality of life to be diminished (James
et al. 2006). Regardless of this fact, there will
inevitably be questions raised about the applica-
bility of a prosthetic device for the child.
The discussion should include that consistent
wearing of any upper extremity prosthesis is a
challenging and unrealistic goal. Of note,
James et al. (2006) looked at various outcome
measures in patients with congenital
below-elbow amputations. These patients took a
battery of assessment examinations, and the
results indicated that non-wearers of a prosthesis
performed as well or better than wearers on both
musculoskeletal health questionnaires and func-
tional tests. In their patient population, actual
prosthetic wear was found to be about 65 %. The
authors concluded that while there may be social
situations where a prosthesis can provide some
benet, the devices themselves did not
signicantly improve the childs function. It is
also important for the family to realize that
because the device covers the residual limb, sen-
sation from the distal aspect of the extremity is
negated. This loss of sensory feedback appears
to be a substantial reason for a childs reluctance
to wear any type of device. The classic recom-
mendation for prosthetic prescription is to t the
patient with a passive terminal prosthesis around
6 months of age to assist with bimanual tasks and
independent sitting. As the child matures and
functional needs change, a more active (body-
powered or myoelectric) terminal device prosthe-
sis is to be used. This standard recommendation is
no longer applicable based upon the data avail-
able. One study examined the types of prostheses
used by below-elbow congenital amputees
who were determined to be consistent wearers.
The authors found that these successful users
generally choose among multiple devices based
upon functional need and as such should be
provided with multiple prosthetic device options
232
Fig. 14 Eight-year-old
competitive swimmer with
right below the elbow
transverse deciency
(Courtesy of Shriners
Hospitals for Children,
Philadelphia). (a) Clinical
picture. (b) Swimming
prosthesis to facilitate
competition
(Crandall and Tomhave 2002). Ultimately, it is
often difcult to predict whether or not a child
will become a consistent prosthetic user. An adap-
tive prosthesis is useful when a child wants to
succeed in a particular task or activity (Fig. 14).
Fitting the child with a device to accomplish a
specic goal is rewarding.
As far as other treatment options are
concerned, there are some specic instances
when surgical intervention may be indicated. For
example, there are cases where the soft tissue
coverage at the terminal end of the amputation
site shows substantial invaginations (or creases)
that can be concerning for potential skin macera-
tion issues. If this becomes a consistent hygiene
problem, excision of the involved skin crease
and rotation of a local full-thickness skin ap to
cover the defect is the most effective treatment.
Another possible surgical intervention that par-
ents may consider involves excising the skin nub-
bins or tiny digits that can occur at the distal
portion of the congenital amputation site. This is a
reasonable treatment alternative, although some
children use their nubbins to assist in the holding
of objects and provide additional sensory feed-
back to the extremity. Lastly, there are more exten-
sive procedures that have been proposed to treat
S.A. Riley and R. Burgess
terminal deciencies. When the deciency occurs
at the hand level, microvascular toe-to-hand trans-
fer has been reported to help supplement the
extremitys function (Kozin 2003). Distraction
osteogenesis is another option for children with
short residual limbs. Lengthening a short amputa-
tion segment allows better prosthesis tting
(Seitz 1989; Jasiewicz et al. 2006; Alekberov
et al. 2000). There are a limited number of cases
reported, but most patients beneted from this
treatment.
Treatment Strategy
The majority of patients with unilateral transverse
deciency require no surgical treatment. Even
children that have tiny digits at the terminal
end of the limb seem to appreciate the added
sensory feedback that these appendages provide
and opt to not have them removed. The exception
to nonsurgical care is in the case of a child with a
short forearm segment in a below-elbow terminal
deciency. Lengthening of this short forearm
yields an improved ability to hold objects in the
crook of the elbow and allows for a greater variety
of prosthetic device alternatives.
10 Hypoplasia, Brachydactyly, and Other Failures of Formation
Fig. 15 Five-year-old child with phocomelia variant and
the hand attached directly to the trunk (Courtesy of
Shriners Hospitals for Children, Philadelphia)
Phocomelia
Phocomelia is a developmental anomaly charac-
terized by the presence of a normal hand and the
absence or hypoplasia of the proximal portion of a
limb. A patient with this anomaly has the physical
appearance of the hand being attached directly to
the trunk (Fig. 15). This failure of formation de-
ciency is classied as an intercalary defect, which
implies the loss of an intervening segment in the
extremity. The condition is a rare deformity, com-
prising less than 1 % of all upper extremity con-
genital anomalies (Flatt 1994a). In about half of
cases that have been identied, the children also
have organ system defects (Bermejo-Sanchez
et al 2011a). There is no specic inheritance pat-
tern for phocomelia as most cases are due to either
a spontaneous mutation or occur as part of a
congenital syndrome. The specic factors that
cause phocomelia continue to be investigated.
Current theories have focused on decient cell
division in the limb bud (Al-Qattan and Kozin
2013), as well as inhibition of angiogenesis
and/or progenitor cell survival in the developing
233
limb (Galloway et al. 2009). Unfortunately, the
anomaly is probably best known for its associa-
tion with maternal use of the drug thalidomide for
emesis during early pregnancy in the late 1950s. It
is estimated that over 10,000 infants may have
been born with severe birth defects related to
thalidomide. Although thalidomide was banned
in 1962, the drug has been used recently as a
treatment for leprosy and cancer. Regrettably,
there have also been new cases of thalidomide-
related phocomelia reported in leprosy-endemic
regions (Bermejo-Sanchez et al 2011a; Vargesson
2009). Strict birth control is necessary for women
taking thalidomide. Other medications have been
implicated as a possible cause of phocomelia. For
example, a recent report described an infant with a
limb reduction disorder similar to phocomelia that
was born to a mother taking carbamazepine for
control of seizures (Dursun et al. 2012).
On the clinical level, phocomelia was origi-
nally described to have three basic morphological
typologies (Frantz and ORahilly 1961). Type I
(sometimes referred to as true phocomelia) is the
form wherein the hand is attached directly to the
trunk and no other intervening osseous structures
are found. The other two types have additional
hypoplastic bone elements that can be identied
between the hand and thorax. A child with type II
phocomelia has the hand and forearm connected
to the trunk; while in type III, the hand is attached
to a humerus. However, our current understanding
of the embryological development of the limb
along three longitudinal axes of formation (see
chapter Embryology) will likely change the
way scientists come to classify phocomelia. In a
recent paper, the authors reviewed 60 cases that
were diagnosed as upper extremity phocomelia.
After a critical analysis of the clinical and radio-
graphic ndings, they determined that phocomelia
may actually represent a spectrum of severe lon-
gitudinal dysplasia because none of their cases
had a true intercalary deciency (Goldfarb
et al. 2005). Another study questioned the exis-
tence of true phocomelia because of the fact that
when intercalary defects are seen, the hand and
glenoid were abnormal as well (Tytherleigh-
Strong and Hooper 2003). Ultimately, it is felt
that as the medical community develops a greater
234
understanding of both the developmental biology
and the specic genetics of this particular limb
deciency, the ability to classify what is called
phocomelia will improve as well.
All discussions about potential treatments for
this condition should focus on making the child as
independent with activities and self-care as possi-
ble. Most commonly, these children will benet
from using prostheses, however; the specic types
prescribed will depend both on his or her func-
tional requirements and the ability of the other
extremities to accomplish those needs. Ample
shoulder control and good stability of the trunk
are advantages to children with phocomelia.
Fitting of the device can be challenging because
of the size and orientation of the residual limb.
Surgical care is rarely needed in this anomaly
(Kozin 2003). There may be certain indications
for surgery to promote prosthetic device wear,
such as excision of painful bony prominences
about the residual limb.
Amelia
Amelia is dened as the congenital anomaly char-
acterized by the complete absence of one or more
limbs. Since this diagnosis is very rare, little infor-
mation has been reported about this condition. A
recent review study from 20 congenital anomaly
surveillance programs found that among both live
and stillbirths, the incidence of amelia is 1.41 per
100,000. The upper limbs are affected slightly
more frequently than the lower extremities, and
single extremity involvement occurs about 65 %
of the time. Interestingly, this same study deter-
mined that the frequency of amelia was higher
among mothers less than 20 years of age, and
69 % of the reported cases had multiple congenital
anomalies including anencephaly, cardiac septal
defects, and other musculoskeletal deformities
(Bermejo-Sanchez et al 2011b).
The exact mechanism causing amelia is
unknown. Because the condition represents a fail-
ure of formation, the prevailing theories focus on
either an error in the molecular biology processes of
the developing limb (such as loss of specic bro-
blastic growth factors (Al-Qattan and Kozin 2013))
S.A. Riley and R. Burgess
or an interruption in the vascular supply to the limb
during the early embryological stages. The actual
causes are likely multifactorial; however, there have
been cases of amelia seen in monozygotic twins
that have vascular anastomoses between their pla-
centas. The subsequent limb anomalies were
thought to be due to alterations in the local arterial
supply (Bermejo-Sanchez et al 2011b).
The treatment principles for the child with
amelia are similar to those mentioned for
phocomelia, except that there are really no surgi-
cal indications. As was seen in patients with trans-
verse deciency and phocomelia, the children
with unilateral upper extremity involvement
adapt remarkably. The option of prosthetic device
use is routinely considered, although there are
unique challenges with regard to device construc-
tion and usage in these patients. Difculties
include the lack of scapulohumeral motion to
power the prosthesis, limited bony and soft tissue
anchor points to keep the device in place, and
limited sensory feedback to the patient. In addi-
tion, for children with bilateral amelia, the pros-
thesis can be heavy and the harnessing
apparatus even more complicated. In cases of
unilateral amelia, the child may decide not to
wear a well-designed prostheses because they
can function well without the device.
Treatment Strategy
Since amelia is such a rare condition, most physi-
cians will never encounter a case in their career.
Even though operative care is not required for
these children, the surgeon and his or her team
play an important role in coordinating additional
evaluation, providing suggestions to enhance
function, and supporting their psychosocial needs.
Summary
Congenital upper extremity anomalies that are
characterized by undergrowth and/or failure of
formation represent a broad category of deformi-
ties. The majority of these malformations occur
in a sporadic fashion without a family history.
10 Hypoplasia, Brachydactyly, and Other Failures of Formation
This group of anomalies has a wide range of
clinical manifestations, and each affected child
presents with unique capabilities and challenges.
As such, treatment strategies must be individual-
ized and proposed only after a careful assessment
of the childs anatomy, functional abilities, and
long-term needs.
Acknowledgements The author would like to acknowl-
edge Brian W. ODoherty, Coordinator of Visual Media
Services at the Shriners Hospitals for Children in Philadel-
phia. His photographic excellence was necessary for com-
pletion of this manuscript.
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 Radial Deficiency
11
Nick A. van Alphen and Steven L. Moran

Contents Preoperative Soft Tissue Distraction . . . . . . . . . . . . . . . . 255

Microvascular Joint Transfer . . . . . . . . . . . . . . . . . . . . . . . . 255
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 238 Timing of Surgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 257
Anatomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 238 Surgical Outcomes and Function . . . . . . . . . . . . . . . . . . 257
Incidence and Etiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 241 Forearm Lengthening . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 259
Management of Complications . . . . . . . . . . . . . . . . . . . . . . 260
Associated Anomalies and Syndromes . . . . . . . . . . . . 242
VACTERL Association . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 243 Conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 261
Holt-Oram Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 243 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 261
Fanconis Anemia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 243
TAR Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 244
Assessment of Radial Longitudinal Deciency . . . 245
Signs and Symptoms and Baseline
Hand Function . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 245
Diagnostic Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 245
Classication . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 246
Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 247
No Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 248
Stretching and Splinting . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 248
Surgical Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 249
Surgical Treatment for RLD Type N . . . . . . . . . . . . . . . . 249
Surgical Treatment of RLD Types 0III . . . . . . . . . . . 249
Surgical Treatment of RLD Types IIIIV . . . . . . . . . . . 250

N.A. van Alphen

Plastic Surgery Department, Mayo Clinic, Rochester,
MN, USA
S.L. Moran (*)
Division of Plastic Surgery and Department of Orthopedic
Surgery, Mayo Clinic, Rochester, MN, USA
Shiners Hospital for Children, Twin Cities, MN, USA
e-mail: moran.steven@mayo.edu

# Springer Science Business Media New York (outside the USA) 2015 237
J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_11
238
Abstract
Radial longitudinal deciency (RLD) is a con-
genital disorder characterized by hypoplasia of
both the radius and soft tissue structures on the
preaxial side of the forearm. Clinical presenta-
tion can vary within a spectrum of severity
ranging from mild cosmetic deformity to com-
plete absence of the radius and thumb. Despite
over 250 years of investigation, current surgi-
cal treatment of this disorder is still inadequate
in restoring normal function and appearance
within the wrist. Shortcomings in surgical
management have made a standardized treat-
ment algorithm for RLD controversial; how-
ever centralization and radialization remain the
mainstay of surgical treatment. This chapter
will attempt to provide an overview of the
deformity and present a review of treatment
options.
Introduction
Radial longitudinal deciency (RLD), also
referred to as radial club hand and radial dysplasia
is a congenital disorder characterized by hypopla-
sia of both the radius and soft tissue structures on
the preaxial side of the forearm. Clinical presen-
tation can vary within a spectrum of severity rang-
ing from mild cosmetic deformity to complete
absence of the radius and thumb. RLD was rst
described by Petit in 1733 in a publication detail-
ing the autopsy ndings of a child with bilateral
upper limb involvement (Petit 1733). Over a cen-
tury later, in 1865, Gruber published the rst
review of the literature noting 14 reported cases
(Kato 1924). Since then, several seminal works
have been published to describe the anatomy,
pathology, and incidence of this condition (Kato
1924; O Rahilly 1947, 1951; Heikel 1959;
Pardini 1968; Skerik and Flatt 1969; Lamb
1977; Bayne and Klug 1987). Despite over
250 years of investigation, current surgical treat-
ment of this disorder is still inadequate in restor-
ing normal function and appearance within the
wrist. Shortcomings in surgical management have
made a standardized treatment algorithm for RLD
N.A. van Alphen and S.L. Moran
controversial. This chapter will attempt to provide
an overview of the deformity and present a review
of treatment options.
Anatomy
Anatomical differences vary according to the
severity of the deformity and can be as mild as a
shortened radius or may be as extensive as the
complete absence of the radius, scaphoid, trape-
zium, and thumb. The malformation can affect the
entire limb from the humerus to the hand includ-
ing all structures on the preaxial side of the fore-
arm and hand. The most common skeletal
presentation is either an absent radius or evidence
of a small proximal radial remnant (Upton 2006).
In those cases where the radius is decient, there
is often a brous anlage, extending from the
diminutive radius to the distal portion of the ulna
(Buck-Gramcko 1975; Lamb 1977; Manske
et al. 1981). This anlage is made of dense brotic
connective tissue and may tether the remaining
radial soft tissue structures to the ulna. The ulna
itself is widened, presumably due to the increase
load passing through the bone. While wider, the
ulna is often bowed radially and rarely grows
more than 16 cm in total length (Heikel 1959).
The cause of ulnar bowing is still unknown, but it
has been speculated that this may be due to soft
tissue tethering distally, lack of radial support, or
an intrinsic abnormality of bone growth (Lamb
1977; Upton 2006). Other osseous abnormalities
can be found in the humerus; these are most fre-
quently found at the elbow and can include abnor-
malities of the olecranon fossa, absence of the
capitulum, absence of the coronoid fossa, and
absence of the medial epicondyle (Heikel 1959;
Flatt 1994; DArcangelo et al. 2000).
Within the wrist, the absence of radial support
creates radial angulation and volar radial displace-
ment of the carpus. This volar radial subluxation
is a major cause of wrist weakness as the majority
of exion force is dissipated over an unstable and
non-articulating carpus. The scaphoid and trape-
zium are often absent. The thumb is often hypo-
plastic or absent, and the remaining radial digits
(particularly the index) may be hypoplastic with
11 Radial Deficiency
Fig. 1 (a, b) Appearance of radial longitudinal deciency.
(a) A 6-month-old boy with bilateral radial longitudinal
deciency. Note associated absence of thumbs, signicant
radial deviation of wrist, shortened forearm, and limited
elbow exion. (b) Late radiographic appearance of a
evidence of joint stiffness or contracture (Pardini
1968; Skerik and Flatt 1969; Lamb 1977; Fig. 1).
The amount of deformity or stiffness within the
radial digits obviously will have a profound
impact on the results of future pollicization
procedures.
In conjunction with the osseous abnormalities,
there are corresponding abnormalities within the
soft tissues. In the most general terms, the muscles
that arise from the medial epicondyle are present
but abnormal and can be fused distally, while the
muscles that arise from the lateral epicondyle are
often absent, accounting for the poor wrist exten-
sion seen in most cases (Bayne and Klug 1987).
Muscles most commonly absent include the
anconeus, supinator, extensor carpi radialis
brevis, and pronator quadratus. The exor carpi
radialis is often absent, and if present, it is hypo-
plastic with an abnormal insertion. The extensor
carpi radialis longus if present is often
nonfunctional (Bora et al. 1981) (Flatt 1994).
Abnormal insertions of the residual radial wrist
exors directly into the carpus have been
observed. Specically, in patients with
thrombocytopenia-absent radius (TAR) syn-
drome, a brachiocarpalis muscle has been
noted originating on the anterolateral aspect of
the proximal humerus and inserting into the radial
side of the carpus (Oishi et al. 2009). This muscle
has been postulated to be a cause of ongoing radial
wrist deviation as well as persistent elbow exion.
239
forearm and wrist in an 18-year-old boy with untreated
Holt-Oram syndrome. Note signicant radial deviation of
the wrist, absence of thumb and index nger, and the lunate
is articulating with the metaphysis of the ulna
It can be divided surgically in an effort to improve
hand and elbow position (Fig. 2). In many cases of
RLD, the exor supercialis muscle may also be
abnormal or fused to the surrounding carpus or
metacarpals (which may complicate future
opponensplasty procedures). Ulnar structures
such as exor carpi ulnaris muscle, extensor
carpi ulnaris muscle, and hypothenar muscles are
preserved. The thumb thenar muscles are
hypoplastic or absent, and their presence is depen-
dent on the amount of osseous hypoplasia seen
within the thumb (Bayne and Klug 1987; Flatt
1994).
With regard to neurovascular structures, the
musculocutaneous nerve is usually absent or aber-
rant with its normally innervated muscles sup-
plied by branches of the median nerve (Flatt
1994). The radial nerve frequently terminates at
the elbow. Within the forearm, due to the absence
of radial structures, the median nerve can lie radial
and more dorsal than expected (Fig. 3). It may
often supply residual radial structures that would
have normally been supplied by the radial nerve.
It also gives off a dorsal sensory branch replacing
the terminal sensory branch of the radial nerve.
The ulnar nerve is usually normal. The radial
artery is frequently absent or hypoplastic being
replaced occasionally by a persistent median
artery while the ulnar artery is normal (Huffstadt
and Broker 1978; Inoue and Miura 1991; Manske
and McCarroll 1998).
240 N.A. van Alphen and S.L. Moran

Fig. 2 (a, b) (a) AP radiograph of the forearm in a 4-year- of the carpus. (b) Intraoperative photograph showing the
old girl with TAR syndrome. The arrow points to a soft appearance of muscle with broad insertion into carpal
tissue density which represents the brachiocarpus muscle capsule
which begins at the humerus and inserts at the radial aspect

Fig. 3 (a) Intraoperative

photograph showing the
dorsal approach to the
carpus in a 12-month-old
during a centralization
procedure. (b) The median
nerve is noted to be radial
(arrow) contributing to the
radial sensation within
the hand

Such anatomical abnormalities lead to a fore-
arm which is short and j shaped. Some component
of wrist radial deviation will be passively correct-
able, but in severe forms the wrist may be radially
deviated beyond 90 and quite stiff. The elbow is
usually held in an extended position and the
childs manipulation of objects is usually
performed with the more mobile ulnar digits
(Fig. 4). In milder forms of RLD where the
thumb is present (but hypoplastic), more normal
oppositional pinch and grasp may be possible, but
cross pinch between the ngers tends to be the
norm. The one exception is seen in children with
TAR syndrome; in these cases, the thumb is
always present and allows for some improvement
in pinch and grasp (Hedberg and Lipton 1988).
Sensation is normal within the hand. Finger
motion is often restricted within in the radial digits
and the joint shape in the radial phalanges is
abnormal, often leading to exion deformities at
11 Radial Deficiency
Fig. 4 Manipulation of smaller objects is usually
performed with ulnar digits as shown in this 3-year-old
girl with untreated RLD and signicant radial deviation of
the wrist
the PIP joint in older children or stiff straight
digits in children presenting at earlier ages (Flatt
1994). Power grip is often compromised by the
instability present at the wrist.
Incidence and Etiology
The incidence of RLD in literature is estimated
between 1:30,000 and 1:100,000 live births (Bod
et al. 1983; Kallen et al. 1984; Wynne-Davies and
Lamb 1985; Bayne and Klug 1987; Urban and
Osterman 1990). Although these statistics show
radial deciency to be rare, it is the most common
of the major longitudinal deciencies (which
includes ulnar dysplasia and central deciency).
The frequency of unilateral and bilateral cases of
RLD is similar (Lamb 1977; Lourie and Lins
1998; Geck et al. 1999). In unilateral cases, the
right side is affected more often than the left side.
There is no difference in incidence between the
sexes for unilateral cases; however, bilateral cases
occur more frequently in male patients at a ratio of
3:2 (Lamb 1977; Bod et al. 1983; Kallen
et al. 1984; Wynne-Davies and Lamb 1985;
Urban and Osterman 1990; Evans et al. 1994;
Upton 2006).
The etiology of radial longitudinal deciency
remains largely unknown. The varied phenotypic
spectrum of the disorder suggests both an
241
environmental as well as genetic component
(de Graaff and Kozin 2009). It is presumed that
RLD is the result of an early disruption in the
sequence of normal limb development as the
upper limb develops from the migration of ecto-
dermal and mesodermal tissue between the fourth
and seventh postconception weeks. It is assumed
that genetic, environmental, or idiopathic factors
can all cause specic developmental defects dur-
ing this period (Lamb 1977; Urban and Osterman
1990).
The formation of the embryonic limb bud is the
rst step toward normal development of the upper
extremity (Daluiski et al. 2001). The apical ecto-
dermal ridge (AER) represents a layer of surface
ectodermal cells at the distal end of the embryonic
limb bud that is responsible for cell signaling and
limb differentiation. In experiments by Saunders,
partial resecting of the AER in dogs resulted in
failure of radial bone development (Saunders
1948). Changes in the distribution of broblast
growth factors from the AER and the underlying
mesoderm can impair the development of longi-
tudinal skeletal formation along the limbs
proximal-distal axis. Niswander and Martin have
shown in both mouse and chicken embryo models
that broblast growth factor four (FGF-4) is capa-
ble of producing proliferation of mesenchymal
cells within the developing limb bud AER.
AER-decient limb buds treated with FGF-4
were capable of developing normal patterns of
limb outgrowth. Niswander and Martin also
showed that bone morphogenic protein two
(BMP-2) was capable of inhibiting this growth
(Niswander and Martin 1993a, b; Niswander
et al. 1993; Daluiski et al. 2001). Studies such as
these help to elucidate the role broblast growth
factors play in the etiology of RLD.
Known factors harmful to the cells within the
AER include X-rays, thalidomide, oral anticoag-
ulants, Coumadin derivatives, and anticonvul-
sants (Elbaum et al. 1995). Ogino and Kato
have shown that WKAH/Hkm rats exposed to
busulfan at weeks 10 through 11 of development
develop limb deformities that are similar to
longitudinal deciencies seen in humans
(Kato et al. 1990; Ogino 2004). In Oginos stud-
ies, limb changes were global suggesting that
242 N.A. van Alphen and S.L. Moran

Table 1 Syndromes associated with RLD with known Mendelian inheritance pattern (From de Graaff 2009)
Syndrome Inheritance pattern
Thrombocytopenia-absent radius syndrome Autosomal recessive
Vertebral anomalies, anal atresia, cardiac abnormalities, tracheoesophageal Autosomal recessive/complex
stula, renal anomalies, and limb anomalies (VACTERL)
VACTERL association with hydrocephalus X-linked
Holt-Oram syndrome Autosomal dominant
Fanconis anemia Autosomal recessive/X-linked
Duane-radial ray syndrome Autosomal dominant
Baller-Gerold syndrome Autosomal recessive
Townes-Brocks syndrome Autosomal dominant
Rothmund-Thomson syndrome Autosomal recessive

Table 2 Syndromes associated with RLD (Upton 2006)

Skeletal Cervical rib-radial ray (Funston syndrome)
Costovertebral dysplasia (Keutel syndrome)
Klippel-Feil syndrome
Cardiac Anemia-triphalangeal thumb (Aase-Smith syndrome)
Cornelia de Lange syndrome
Cutaneous Cutaneous poikiloderma (Rothmund-Thomson syndrome)
Renal Renal-radial ray aplasia (Sofer syndrome)
Craniofacial Acrofacial dysostosis (Nager syndrome)
Acro-reno-ocular syndrome
Craniofacial microsomia
Eye-radial syndrome
Lacrimal auriculo-dento-digital syndrome (LADD or Levy-Hollister syndrome)
Radial ray-choanal atresia
Micrognathia and limb anomalies (Hanhart syndrome)
Mandibulofacial dysostosis (Treacher Collins syndrome)
Oculo-auriculo-vertebral dysplasia (Goldenhar syndrome)
Oro-cranio-digital syndrome (Juberg-Hayward syndrome)
Roberts syndrome

injuries to mesenchymal cells within the AER
may have widespread implications for limb
development.
Associated Anomalies and Syndromes
RLD is rarely an isolated nding and is associated
with several congenital syndromes (Tables 1 and 2)
(Goldfarb et al. 2006a; de Graaff and Kozin
2009). Isolated radial deciency is estimated to
comprise only 830 % of the cases (Goldfarb
et al. 2006b; Koskimies et al. 2011). The fre-
quency of associated anomalies increases with
the severity of RLD (Goldfarb et al. 2006a). Asso-
ciated anomalies are seen in 40 % of patients with
unilateral involvement and 77 % of patients with
bilateral involvement (Lin and Perloff 1985;
Goldfarb et al. 2006a; Koskimies et al. 2011);
(Goldberg and Bartoshesky 1985). Because
greater than 70 % of the cases are associated
with some other anomalies, signs of RLD should
lead the practitioner to search for problems in
other organ systems.
The most common syndromes associated
with RLD include the VACTERL association,
Holt-Oram syndrome, Fanconis anemia, and
thrombocytopenia-absent radius (TAR) syndrome
11 Radial Deficiency
(Riordan 1955; Heikel 1959; Fanconi 1967;
Skerik and Flatt 1969; Lamb 1977, 1991; Manske
et al. 1981; Wynne-Davies and Lamb 1985;
Bayne and Klug 1987; Urban and Osterman
1990; Botto et al. 1997; James et al. 1999, 2004;
Maschke et al. 2007). These syndromes will be
discussed in more detail, while a list of
other associated anomalies is provided in
Tables 1 and 2.
VACTERL Association
VACTERL is an acronym for vertebral anomalies
(V), anal atresia (A), cardiac anomalies (C),
tracheoesophageal stula (TE), renal anomalies
(R), and limb abnormalities (L). Three of these
malformations need to be present to meet the
criteria for the VACTERL association. The most
common occurring anomalies are cardiovascular
defects and renal agenesis; RLD is the most com-
mon occurring limb defect in the VACTERL asso-
ciation, but other hand anomalies can include
thumb hypoplasia and polydactyly (Quan and
Smith 1973). The cardiovascular anomalies
most frequently encountered in these children
are atrial septal defects (ASD) and ventricular
septal defects (VSD) (Buck-Gramcko 1985;
Wynne-Davies and Lamb 1985). A multidis-
ciplinary approach needs to be taken with these
children to determine the appropriate time for
limb surgery.
Holt-Oram Syndrome
Holt-Oram syndrome is an autosomal-dominant
disorder of variable penetrance. It is characterized
by RLD and associated cardiovascular malforma-
tions, most commonly atrial or ventricular septal
defect. Other associated hand anomalies can
include radioulnar synostosis, triphalangeal
thumb, and humeral defects. Occasionally, spinal
malformations may be present. The incidence of
Holt-Oram syndrome is estimated at 1 in 200,000
live births (Holt and Oram 1960; Goldberg and
Bartoshesky 1985; Elbaum et al. 1995; Bossert
et al. 2002).
243
Fanconis Anemia
Fanconis anemia is an autosomal-recessive pan-
cytopenia associated with RLD and is character-
ized by major organ deformities and early-onset
bone marrow failure. The disorder is associated
with multiple mutations in the transcription factor
TBX5, a gene located on chromosome 12q24.1
(Li et al. 1997). Clinical features include growth
retardation, major organ abnormalities, hypo- or
hyperpigmentation of the skin (caf-au-lait spots),
typical facial appearance (small head, eyes, and
mouth), and a predisposition to develop certain
forms of cancers. The risk of squamous cell cancer
of the mucous membranes, liver cancer, and acute
myeloid leukemia is increased several fold in
these individuals. 90 % of children will develop
acute bone marrow failure within the rst decade
of life (Wilks et al. 2012). The pancytopenia can
be treated with bone marrow transplantation, but
even with these efforts, life expectancy is only
estimated at 30 years (range 050) (Wilks
et al. 2012).
Early detection is critical so that bone marrow
transplant can be performed before the patient has
had to receive multiple blood transfusions, which
can complicate future bone marrow transplanta-
tion. Bone marrow transplantations performed
before the age of 10 in these children have
shown improved outcomes (Wilks et al. 2012).
While early detection provides the best chance
of long-term survival, signs of anemia are not
noticeable on standard blood tests at birth due to
the late onset of the anemia. To detect this condi-
tion in infancy, a diepoxybutane (DEB) fragility
testing chromosomal challenge can be conducted
(Esmer et al. 2004). If the test is abnormal, close
hematologic monitoring is recommended to iden-
tify early signs of bone marrow failure as the
child ages (Auerbach et al. 1985; Goldfarb et al.
2006b). In addition, a search for a bone marrow
match can be performed prior to the onset of
pancytopenia.
Fanconis anemia is most commonly associ-
ated with preaxial skeletal deformities and has
been reported to occur in 3.5 % of cases of RLD
but may also be seen in 7 % of patients with other
congenital thumb anomalies. One case has been
244
Fig. 5 Photograph of a
3-year-old female with TAR
syndrome. Note appearance
and position of thumb
documented in a child with unilateral thumb
duplication. Present recommendations include
DEB fragility testing in children with RLD, espe-
cially when associated with abnormal facies or
abnormal skin pigmentation (Esmer et al. 2004;
Upton 2006; de Graaff and Kozin 2009; Webb
et al. 2011; Wilks et al. 2012).
TAR Syndrome
Thrombocytopenia-absent radius (TAR) syn-
drome is characterized by a hypomegakaryocytic
thrombocytopenia in association with RLD
(de Graaff and Kozin 2009; Fig. 5). A
microdeletion in 1q21.1 has been found to be
associated with the development of this disorder
(Klopocki et al. 2007). The incidence is estimated
at 1 per 239,000 to 1 per 500,000 live births
(Hall 1987; Goldfarb et al. 2006b). Clinical man-
ifestations include bruising, severe hemorrhaging,
and, in 30 % of the cases, cardiac and/or renal
abnormalities (Urban and Osterman 1990). Other
associated malformations include varus knee
deformities, knee exion deformities, and internal
tibial rotation (DArcangelo et al. 2000). Only
50 % of children will show signs of thrombocy-
topenia at birth, but 90 % will show abnormalities
by 4 months of age (Hall et al. 1969). The only
surgery which is medically justied before the child
experiences a stabilization of the platelet count is
cardiac surgery. Hematologic treatment of severe
thrombocytopenia consists of platelet transfusions.
N.A. van Alphen and S.L. Moran
Platelet counts can be negatively affected by viral
illnesses, minor trauma as well as any surgery.
A particular phenotypic characteristic for TAR
syndrome is the presence of the thumb despite
radial deciency (Hedberg and Lipton 1988).
Although present, the thumb usually lacks normal
function due to stiffness of the thumb and other
digits (Fig. 5). Most thumbs lack interphalangeal
joint (IP) motion and tend to have an extension
decit at the metacarpal-phalangeal (MP) joint,
resulting in impaired hand grasp of large objects
(Goldfarb et al. 2007).
The frequent association of RLD with the
aforementioned syndromes emphasizes the
importance of a thorough and complete physical
exam. This should include an assessment of facial
features and lower limbs and cardiac and spinal
examination (Goldfarb et al. 2006b). Further test-
ing should include spinal radiographs, an echo-
cardiogram, a complete blood count with
peripheral blood smear, and a renal ultrasound
exam (Elbaum et al. 1995; Goldfarb et al. 2006b;
Maschke et al. 2007; Manske and Goldfarb 2009).
Additionally, close collaboration with a special-
ized pediatrician is necessary to provide an appro-
priate long-term treatment plan. Due to the high
incidence of associated anomalies, many patients
with RLD need specialized cardiac monitoring
while undergoing general anesthesia. Surgical
procedures should be conducted in a specialized
pediatric center capable of monitoring these chil-
dren during the perioperative and postoperative
period (Quan and Smith 1973).
11 Radial Deficiency 245

Assessment of Radial Longitudinal

Deficiency

Signs and Symptoms and Baseline

Hand Function

Radial longitudinal deciency (RLD) is charac-

terized by a radial-deviated wrist and the affected
hand held in exed position. The deformity is
always present at birth but continues to develop
with growth of the limb. If untreated, wrist posi-
tion tends to deteriorate with further radial devia-
tion occurring with growth. Stiffness develops
with increasing palmar subluxation of the carpus
and ulnar bowing during infancy (Lamb 1977).
Associated hand anomalies include thumb hypo-
plasia and stiffness of the radial digits (index and
long most commonly). Forearm length is usually
only 60 % of the unaffected side (Bayne and Klug
1987; Sestero et al. 2006). The extent of hand
disability depends on the severity of the dysplasia.
In severe cases, joint dysfunction can extend
proximal to the elbow and shoulder (Urban and
Osterman 1990).
Diagnostic Imaging
The dening characteristic of RLD is hypoplasia
of the radius. The standard radiographic assess-
ment in RLD patients consists of bilateral
anteroposterior and lateral radiographs of the
arm, forearm, wrist, and hand. The wrist is pref-
erably positioned as straight as possible without
stress for imaging (Manske et al. 1981; Ekblom
et al. 2013).
Several radiographic parameters have been
established to study wrist position preoperatively
and postoperatively (Manske et al. 1981). The
hand-forearm angle (HFA) is used to quantify
the angulation between the hand and forearm.
The HFA can be measured using a standard
anteroposterior (AP) radiograph and is dened as
the angle between the longitudinal axis of the long
nger and the longitudinal axis of the distal ulna
(Manske et al. 1981). In many cases, the ulna will
be bowed toward the hypoplastic or absent radius.
Fig. 6 The hand-forearm angle can be measured radio-
graphically from the angle created between a line placed
axial through the third metacarpal and capitate and a line
placed perpendicular to the ulnar articular surface (also
known as the distal ulna midline). Distance (A) represents
the hand forearm position which measures the distance
from the base of the fth metacarpal to the distal ulna
midline. The ulna bow is measured by the angle created
by the distal ulna midline and a line drawn through prox-
imal midline of the ulna
This will provide a false appearance of the actual
HFA. To standardize the longitudinal axis of the
distal ulna, a line is drawn across the distal epiph-
yseal plate of the ulna. From the middle of the
epiphyseal plate, a perpendicular line can be
drawn, which is dened as the longitudinal axis
of the ulna. The direct angle between this
constructed line and the longitudinal axis of the
long nger measures the HFA (Fig. 6). The hand-
forearm position (HFP) is determined as the
shortest distance between a line drawn through
the longitudinal axis of the distal ulna and the
proximal pole of the fth metacarpal. The HFP
can be determined from a standard AP radiograph.
The base of the fth metacarpal can be deviated
radial or ulnar to the ulnar shaft; therefore, radial
and ulnar displacement is measured as plus or
246
Fig. 7 The original Bayne classication system for RLD (Copyright Mayo Foundation)
minus to this point (Fig. 6). The ulnar bow can be
measured by using the aforementioned method for
determining the longitudinal axis of the distal
ulna. The longitudinal axis of the proximal ulna
can be constructed in a similar way, and the inter-
section of these two lines measures the ulnar bow
in degrees. Ulnar bow is measured on a standard
AP radiograph.
Classification
In 1976, the International Federation of Societies
for Surgery of the Hand classied RLD within
Category I, which describes a failure of formation
of parts (Swanson 1976). In 1987, Bayne and
Klug expanded upon this classication breaking
RLD into four categories based on the radiologic
characteristics of the radius; this classication has
been modied several times since its introduction
(Bayne and Klug 1987). Their description of the
four types of RLD is based on the severity of the
osseous decit (Fig. 7). Type I is dened as a
minimally shortened radius of more than 2 mm
with respect to the ulna (James et al. 1999). There
N.A. van Alphen and S.L. Moran
is no ulnar bowing and elbow function is normal.
Thumb hypoplasia is almost always present and
the radial carpal bones are usually hypoplastic.
Surgical management of the wrist in these cases
is rare, and if necessary, surgery is usually directed
toward improving any abnormalities in thumb
function (Maschke et al. 2007).
In type II, both proximal and distal epiphyses
are affected resulting in a hypoplastic radius. This
type of deciency is commonly referred to as
radius in miniature (Bayne and Klug 1987).
The ulna is usually thickened and bowed toward
the radius. The hand and wrist are subsequently
positioned more radially. Thumb hypoplasia and
an absent scaphoid are common in type II (Lamb
1977). To limit the amount of radial deviation and
to facilitate better surgical results, patients should
be splinted and stretched at an early age. Surgery
in these cases consists of procedures to correct
radial bowing of the ulna and to stabilize the
wrist. Surgical options are described in detail fur-
ther in this chapter.
Type III consists of partial absence of the
radius. This decit can be located in the proximal,
middle, or distal third of the radius, but type III
11 Radial Deficiency
Fig. 8 AP radiographs in a
child with bilateral thumb
hypoplasia and evidence of
an absent scaphoid on the
left or type N deciency
usually presents with a proximal radial remnant
(Manske and McCarroll 1998). Some proximal
remnants may not be visible until older age
(Upton 2006). Thumb hypoplasia or aplasia is
often present, and absence of the scaphoid and
trapezium is frequently seen in this subgroup.
Patients often have tight soft tissue (the anlage)
extending from the remaining radial remnant to
the carpus. This results in the wrist moving into
exion and pronation.
Type IV is characterized by complete absence
of the radius. The hand is severely displaced to the
radial side, and in older patients, the carpal bones
may articulate with the radial border of the distal
ulna. Ulnar bowing is present and normal growth
and development of the ulna is inhibited. Overall
ulnar growth is limited and the forearm rarely
grows to more than 60 % of the contralateral
side (Heikel 1959). Limited elbow motion is
often seen. This type of deformity is always seen
in conjunction with thumb aplasia and limited
motion of the radial digits. The one exception to
this is the patient with TAR syndrome where the
thumb is always present but has limited function
(Hedberg and Lipton 1988).
This Bayne classication was expanded by
James and colleagues (James et al. 1999) to
include patients with hypoplasia of the thumb
without signicant deciency in the radius (clas-
sied as type N) and patients with abnormalities in
the radial carpal bones classied as type 0 (Fig. 8).
In cases of type 0, the radial carpal bones are
247
absent or hypoplastic, but there is no shortening
of the distal radius; however, abnormalities may
exist in the proximal radius such as congenital
dislocation of the radial head or radioulnar synos-
tosis (Maschke et al. 2007). Further modications
were made by Goldfarb and colleagues to address
abnormalities in the humerus. They use the clas-
sication of type V RLD for decits of the prox-
imal humerus, abnormalities of the glenoid,
and distal humerus articulation with the ulna
(Goldfarb et al. 2005). Despite these additions,
types III and IV are thought to be the most com-
mon types of RLD (Upton 2006).
Treatment
There are three treatment options for RLD: (1) no
treatment, (2) stretching and splinting alone, and
(3) surgical correction in conjunction with post-
operative stretching and splinting. The ultimate
goal of any treatment is to improve hand function
(i.e., grip strength and precision pinch), improve
functional length, and improve appearance. An
important aspect of any discussion regarding
treatment should focus on providing the parents
with realistic expectations for immediate and late
posttreatment outcomes and the time required for
each intervention.
Surgical treatment of RLD has changed tre-
mendously over the past 30 years but is still
focused on wrist stabilization and realignment
248
Fig. 9 (ac) Stretching and splinting techniques are very
helpful in preparing the wrist prior to centralization and
radialization procedures and to maintain surgical correc-
tion after surgery. (a) Stretching techniques should be
taught to parents and be performed three to four times a
during infancy and on lengthening the forearm
during adolescence (Taghinia et al. 2007).
Starting at birth, initial close contact with the
coordinating pediatrician can expedite the initia-
tion of treatment. Timing of any surgical interven-
tion must be postponed until all hematologic and
cardiac problems are stabilized. The multidis-
ciplinary needs of these young patients make
treatment in a single pediatric surgical center
desirable for patients and their parents.
No Treatment
No treatment is most appropriate in cases where
patients have substantial associated anomalies
which may result in severe cognitive delays or
life-threatening illness. Bayne and others have
also suggested that treatment may be
contraindicated in cases of mild disease, adult
patients who have adapted to their hand and
wrist deformities, and in patients who have lim-
ited elbow function with the inability to ex
beyond 90 . In such patients, straightening the
wrist may limit toileting and the ability to get the
N.A. van Alphen and S.L. Moran
day. (b) Wrist splints may be applied to push the wrist into
a more neutral position. (c) Splints may be worn after
centralization procedures, as in this child with TAR, to
prevent radial recurrence of wrist position
hand to the mouth (Flatt 1994) (Bayne 1991;
DArcangelo et al. 2000).
Stretching and Splinting
The two methods of nonsurgical treatment are
splinting and stretching of the tight radial soft
tissues (Fig. 9). These treatment modalities are
most appropriate for types I and II deformities.
Therapy can start early after birth. Careful
stretching by the parents or caregivers is thought
to limit the radial contraction deformity and may
create a more central hand-forearm position
(HFP). Stretching of the wrist involves
progressive longitudinal distraction, ulnar devia-
tion, and extension while stabilizing the
ulnocarpal joint (Maschke et al. 2007). The wrist
is to be stretched as close to neutral as possible
using gentle but rm passive traction. Due to the
lack of consensus on the optimal rate for
stretching, the most convenient interval is to com-
bine stretching with an everyday activity such as
diaper changes (Goldberg 1976; Flatt 1994;
Maschke et al. 2007).
11 Radial Deficiency
Serial splinting or casting is the other major
technique for nonsurgical treatment. A radial gut-
ter splint, which leaves the ulnar digits free, can be
used to preserve the correction achieved with
stretching. It is initially recommended to wear
the splint during day and night, only taking the
splint off during dynamic stretching. Once passive
motion of the carpus is achieved, the splint regi-
men can be changed to usage of a splint during the
night or periods of rapid growth (DArcangelo
et al. 2000; Maschke et al. 2007).
Serial casting has been a component of the
treatment of RLD since the publication of Sayre
in 1894. Sayre as well as Riordan used progres-
sive casting and stretching to position the hand
more centrally over the ulna prior to surgical
intervention (Sayre 1893; Riordan 1955). In their
long-term outcome study of the surgical manage-
ment of RLD, Bayne and Klug noted splinting to
be essential to maintaining satisfactory postoper-
ative results (Bayne and Klug 1987). Unfortu-
nately, there are few studies to evaluate the
timing and efcacy of splinting. In addition,
there are no prospective studies that evaluate the
long-term outcomes of splinting alone in compar-
ison to surgical management; however, in
Kotwals and colleagues retrospective analysis
of 446 patients with types III and IV RLD treated
over a 20-year period, they found that patients
treated with surgery had better appearance and
function and performed better with activities
when compared to those treated with stretching
and splinting alone (Kotwal et al. 2012).
Surgical Treatment
Surgical treatment for RLD is indicated when
conservative treatment fails or in cases with
marked wrist angulation (types III and IV). Occa-
sionally, surgery may be required in milder cases
of RLD unresponsive to stretching and casting.
Knowledge of the anatomical variations within
the forearm is critical prior to embarking on any
surgical procedure for RLD. Studies by Kato,
Skerik, Flatt, Pardini, and Kelikian describe the
anatomical variations in detail and should be
reviewed by the inexperienced surgeon (Kelikian
249
and Doumanian 1957; Pardini 1968; Skerik and
Flatt 1969; Kato et al. 1990; Flatt 1994).
Surgical Treatment for RLD Type N
Isolated thumb hypoplasia or aplasia has only
recently been considered to be an element of the
RLD classication. Treatment of thumb hypopla-
sia will be addressed separately in its own chapter
(see chapter Thumb Hypoplasia); however,
in patients with absent thumbs or rudimentary
thumbs with an unstable CMC joint, the conven-
tional surgical treatment is pollicization of the
index nger resulting in a four-ngered hand.
Other surgical techniques like toe-to-hand transfer
are described, but thumb pollicization remains the
gold standard (Upton 2006).
The functional results of pollicization in
patients with RLD do not compare as well as
those with isolated Blauth-type thumb hypoplasia
when examined in long-term follow-up (Clark
et al. 1998; Molenaar et al. 2013). De Kraker
and colleagues examined the outcomes of
24 patients with varying degrees of RLD de-
ciency at greater than 9 years following
pollicization. They found that the overall range
of motion and strength were decreased in compar-
ison to normal controls. Patients with severe RLD
had strength measurements that were signicantly
less than those patients with milder RLD. Despite
these functional differences, the majority of
patients were satised with the functional and
aesthetic outcomes (de Kraker et al. 2013). Poorer
outcomes following pollicization in patients with
severe RLD are most likely related to the stiffness
of remaining radial digits, limited active motion,
and ongoing wrist instability.
Surgical Treatment of RLD Types 0III
Type 0 RLD, which represents the radial-deviated
hand with a normal-length radius, is caused by a
deciency in the radial wrist extensors and exors
(Mo and Manske 2004; James et al. 2004). Not all
patients with type 0, type I, or type II RLD need
surgical correction. Splinting and stretching alone
250
are frequently used to treat these patients. When
patients have a marked radial deviation of the
hand at rest, however, surgery can be indicated
(Wall et al. 2013; Mo and Manske 2004). Mo and
Manske have described correction of RLD type
0 with tendon transfers and soft tissue rebalancing
(Mo and Manske 2004). The procedure is partly
based on the radialization technique of Buck-
Gramcko (Buck-Gramcko 1985). The ECU is
divided leaving a distal stump attached to the
fth metacarpal. It is then transferred into the
dorsal wrist capsule at the level of the third meta-
carpal to help improve any existing extension
decit. The extensor carpi radialis tendons
(if present) are transferred to the distal stump of
the extensor carpi ulnaris (ECU) to transfer the
force of the abnormal forearm muscles to the ulnar
side of the wrist, resulting less radial deviation.
While long-term results were lacking in Mos
study, early postoperative resting wrist angle
improved as did active and passive extension of
the wrist (Mo and Manske 2004).
Treatment of type II RLD has included distrac-
tion lengthening of the hypoplastic radius
(Manske and McCarroll 1998; Matsuno
et al. 2006). Matsuno, in a small series of four
patients, described a technique using external
distractors placed on both the ulna and the radius
in an attempt to correct both radial height and
wrist position. Unfortunately, results were poor
and require extended treatment, with absorption
of regenerate bone occurring after distraction
attempts. If one chooses to utilize this technique,
patients and parents should be aware that delayed
consolidation or malunion of the radius can occur
and that three or more lengthening procedures
may be required to gain and maintain appropriate
length (Matsuno et al. 2006).
Surgical Treatment of RLD Types IIIIV
Types III and IV RLD represent the most common
forms of this anomaly, and hence, the majority of
published surgical literature has focused on the
management of these patients. Historically, surgi-
cal treatment has focused on stabilizing the carpus
on the ulna or on techniques that replace the
N.A. van Alphen and S.L. Moran
missing radius with some form of bone graft
(Albee 1928; Starr 1945; Riordan 1955; Manske
and McCarroll 1998; DArcangelo et al. 2000).
The use of bone grafting fell out of favor after
long-term reports failed to show adequate growth
of the grafts over time (Starr 1945; Riordan 1955).
Currently, centralization and radialization are the
most commonly performed surgical procedures
for the treatment of RLD (Fig. 10).
The premise of centralization is to align the
wrist and third metacarpal over the center of the
ulna. The procedure is designed to provide a sta-
ble base for the carpus, place the hand in a more
useful position, and increase functional forearm
length. Centralization was originally published by
Sayre in 1893, where he described notching the
ulna into the carpus by removing the lunate and
capitate (Sayre 1893). The technique of notching,
while creating a stable ulnocarpal articulation, can
lead to injury of both the ulnar physis and
carpus leading to a reduction in remaining ulnar
growth. Subsequent modications have been
developed to limit ulnar physeal injury, including
removal of only portions of the carpus, osteotomy
of the ulna, and extensive soft tissue release
prior to centralization (O Rahilly 1951; DeLorme
1969; Linge 1969; Bora et al. 1970; Lamb 1977;
Watson et al. 1984; Manske and Goldfarb 2009).
These modications help to decrease the force
required to reduce the carpus. Currently, centrali-
zation techniques are preceded by soft tissue
distraction with or without soft tissue release
(see below) to decrease operative time and
allow for easier wrist rebalancing (Goldfarb
et al. 2006a).
Centralization
Preoperative planning
OR table: regular with hand table or arm board
Position: supine
Fluoroscopy location: at the end of hand table
Equipment: standard, K-wires and power
Tourniquet: yes
Surgery may be performed through a variety of
incisions, including either a straight line radial-
based incision, s-shaped radial incision, radial
incisions with z-plasty closure, transverse dorsal
wrist incision, bilobed ap, or dorsal transposition
11 Radial Deficiency
Fig. 10 Centralization technique involves aligning the
third metacarpal with the central portion of the ulna. Trans-
fer of any remaining radial wrist extensors (ECR) may be
considered as well as advancing ECU distal on the fth
ap (Buck-Gramcko 1985; Evans et al. 1995; Pilz
et al. 1998; Upton 2006; VanHeest and Grierson
2007). These later ap designs try to take advantage
of redundant tissue over the ulnar head which can
be brought to the radial (skin-decient) side of the
carpus following centralization. Manske has
recommended an ulnar approach to allow for ulnar
capsulodesis and excision of excess ulnar skin and
subcutaneous tissue (Manske et al. 1981). Pilz and
colleagues retrospectively reviewed all incisions
used for the management of RLD at their institution
between 1970 and 1996. In this series, all aps
healed with the exception of bilobed aps, where
partial ap loss occurred in more than 50 % of
patients (Pilz et al. 1998). Similar complications
have not been noted by other investigators (Evans
et al. 1995; Wall et al. 2013).
251
metacarpal to improve its mechanical advantage and
improve wrist extension. Illustration also depicts correc-
tion of radial bow with concomitant osteotomy at time of
centralization procedure (Copyright Mayo Foundation)
Once the skin incision has been made, the
median nerve is identied along with the extensor
tendons to the ngers, thumb (if present), and
ECU. Remaining portions of the extensor carpi
radialis brevis (ECRB) and extensor carpi
radialis longus (ECRL) can be saved for transfer
to the ECU tendon at the end of the case. The tight
radial side of the wrist capsule is opened and
any remaining anlage is excised. The ulnar
head can now be visualized allowing the carpus
to be moved over the ulna (Watson et al. 1984).
At this stage of the procedure, Manske and
others have recommended creating a carpal
notch to allow for long-term stabilization of
the wrist. Notching may be done with resection
of the lunate and capitate, but can result in
a chondrodesis of the wrist (Lamb 1977;
252
Manske and McCarroll 1998). Watson and other
authors have not found removal of cartilage to be
necessary if a wide release is performed
(Watson et al. 1984; Buck-Gramcko 1985).
If cartilage is to be removed, judicious removal
is necessary to avoid iatrogenic injury to the ulnar
physis.
Once the ulnar head is exposed, a Kirschner
(K) wire is passed down the center of the ulna
exiting the skin proximally in the forearm.
Correction of the ulnar bow, if greater than 30 ,
can be performed at this point with a corrective
osteotomy within the mid or proximal ulnar shaft.
Once the wire is in appropriate position, the wrist
is reduced onto the distal ulna. The third metacar-
pal should be aligned with the central epiphysis of
the ulna. The cartilage of the distal ulna can be
shaved to allow for a atter surface for wrist bones
to seat onto the ulnar head (Watson et al. 1984;
Bayne and Klug 1987). The K-wire is then driven
through the longitudinal axis of the third metacar-
pal to stabilize hand-forearm position. Alterna-
tively, a K-wire can be placed initially through
lunate into the third metacarpal. The wrist is then
reduced and the K-wire is passed down through
the ulna (Watson et al. 1984; Fig. 11).
Fig. 11 (a) AP radiograph
of a child with Bayne type
III RLD, with mild ulnar
bowing. Centralization is
performed in conjunction
with corrective osteotomy
of the ulna. (b) Radiograph
showing pin placement to
correct ulnar bowing and to
position capitate in center of
ulnar joint surface
N.A. van Alphen and S.L. Moran
Additional procedures may include transfer-
ring the remaining radial tendons to the ulnar
aspect of the carpus, plication of the ulnar capsule,
and excess skin resection (Manske et al. 1981;
Upton 2006). Establishing muscle balance is
important for prevention of recurrence of angular
deformity. The ECU can be shortened or moved
distally on the fth metacarpal to improve its
mechanical advantage. In addition, the FCU can
be attached to the ECU to further enhance wrist
extension and prevent recurrent carpal volar sub-
luxation (Bayne 1991; Manske and McCarroll
1998). Bora and Goldberg have described the
advancement of the origin of the hypothenar mus-
cles onto the ulna to increase their contribution to
ulnar deviation of the wrist (Bora et al. 1970;
Goldberg 1976). One needs to avoid transferring
the abductor digiti minimi in these cases if the
surgeon plans to use this muscle for subsequent
opponensplasty. Bora also described the transfer
of the long and ring nger supercialis tendons to
the dorsum of the wrist for help with wrist exten-
sion and ulnar deviation. We have no experience
with this particular tendon transfer (Bora
et al. 1970).
K-wires are kept in place for 812 weeks.
The longer the K-wire is left in place, the lower
11 Radial Deficiency
the incidence of recurrence but the greater the risk
of K-wire breakage and physeal injury. Early
reports from Delorme and Goldberg recommended
leaving the K-wires indenitely and changing them
as necessary at 1824-month intervals (DeLorme
1969; Goldberg 1976). Once the K-wire is
removed, the patients are given a protective splint
that is worn until skeletal maturity (Bayne and
Klug 1987; de Jong et al. 2012; Fig. 12).
Centralization
Surgical steps
Dorsal ulnar, bilobed, or central incision used for
exposure
Identication of median nerve, tendons, and anlage
Anlage resected, judicious removal of cartilage to allow
for carpal reduction
Osteotomy created to correct radial bow
Carpus reduced onto ulna
K-wire used to reduce carpus as well as correct radial
bow
Soft tissue closure combined with transfer or radial wrist
extensors to ECU, ECU advanced distally on fth
metacarpal, and plication of ulnar wrist capsule
Z-plasty or bilobed ap closure, K-wire cut and buried
below the skin
Centralization
Postoperative protocol
Patient casted for 8 weeks
Removable splint placed at 8 weeks
K-wire removed at 812 weeks
Radial gutter splint worn at night until skeletal maturity
Although centralization has been the most pop-
ular surgical procedure to correct RLD, recent
studies have shown radial recurrence to be com-
mon (Damore et al. 2000). Recurrent radial angu-
lation is estimated in literature to be as high as
50 % in severe cases (Lamb 1977; Goldfarb
et al. 2002; McCarthy et al. 2009). Recurrence
of radial angulation as the child grows has been
correlated with the inability to achieve complete
correction at the time of the initial surgery and the
age at the time of surgery (Geck et al. 1999;
Damore et al. 2000). Ulnar length in surgically
treated forearms can be limited to 4858 % of
normal growth, while untreated forearms may
grow to 64 % of the normal side (Sestero
et al. 2006).
253
In response to the poor outcomes produced in
early centralization reports, Buck-Gramcko intro-
duced the technique of radialization (Buck-
Gramcko 1985). The radialization procedure was
developed to avoid carpal bone resection and ulna
shortening. In this technique, the most radial
aspect of the carpus and second metacarpal are
positioned over the central portion of the ulna
placing the hand in slight ulnar deviation. No
bony resection is performed. As in centralization,
a K-wire is used to stabilize the longitudinal axis
of the second metacarpal and the longitudinal axis
of the distal ulna. A hand-forearm angle in slight
ulnar deviation improves the ulnar directed lever
arm, which now extends out from the ulna to the
base of the small metacarpal. The exor carpi
radialis, brachioradialis, ECRB, and ECRL are
transferred on mass into the ECU to improve
ulnar deviation. The K-wire is left in place for
8 weeks, after which night splints are used until
skeletal maturity (Fig. 13).
Buck-Gramcko reported good outcome in his
original series of radialization but provided few
objective measurements to support his observa-
tion. Wrist range of motion in this series was
reported to range between 40 and 90 of exion
(Buck-Gramcko 1985). In a study by Geck, com-
paring the outcome of 15 radializations and
14 centralizations followed for 50 months, no
differences were seen in outcomes between
radialization and modied centralization. The
risk of reoperation was seen to occur in patients
where surgery had been performed at a younger
age and when the initial postoperative hand-
forearm angle was not corrected beyond 0 (indi-
cating persistence radial deformity at the end of
the case) (Geck et al. 1999). Most recently, Dana
and colleagues have published a series of eight
patients treated with preoperative distraction and
radialization followed for over 2 years (Dana
et al. 2012). Recurrence of radial deformity was
high with seven of the eight patients experiencing
recurrent deformity. The forearm-hand angle was
44 at last follow-up compared to initial 12 post-
operative. Intuitively, radialization provides a less
stabile wrist joint compared to the stiff wrist joint
created with carpal notching; however, more
long-term outcome studies are needed to provide
254 N.A. van Alphen and S.L. Moran

Fig. 12 (continued)
11 Radial Deficiency
a clear picture of the role of radialization in the
surgical treatment of RLD.
Preoperative Soft Tissue Distraction
Centralization in the presence of substantial radial
tightness is associated with higher radial recur-
rence rates (Kawabata et al. 1998). Historically,
splinting and casting have been used to better
position the hand over the ulna prior to centrali-
zation but lack the ability to consistently place the
carpus distal to the ulna (Goldfarb et al. 2006a).
Kessler was the rst to use preoperative soft tissue
distraction to position the hand in a more favor-
able position prior to centralization (Kessler
1989). Numerous studies have reported on the
use of this technique prior to correction proce-
dures for RLD (Smith and Greene 1995; Vilkki
1998a; Sabharwal et al. 2005; Goldfarb
et al. 2006a; de Jong et al. 2012). In Goldfarbs
study of six patients, the average surgical time for
centralization was normally 150 min but
decreased to 90 min with the use of preoperative
distraction (Goldfarb et al. 2006a).
Distraction can be performed with unilateral,
ring, or multiaxial devices. The taut radial anlage
is released and the distractor device is placed after
the pins have been positioned under direct vision
(Taghinia et al. 2007). The parents are educated on
the details of the device and distraction usually
proceeds at a rate of 1 mm a day. Patients return to
the clinic once a week for radiographic monitor-
ing of progress. The average duration of distrac-
tion is variable and has been reported to be
between 21 and 130 days (Sabharwal et al. 2005;
Taghinia et al. 2007). Some studies advocate leav-
ing the distractor in place for up to 4 weeks after
reaching a neutral wrist position in order to reduce
swelling at the time of surgery (Sabharwal
Fig. 12 (a) Radiograph and (b) photograph of a
12-month-old girl with Bayne type IV RLD undergoing a
centralization technique as described by Watson. (c) Sep-
arate radial and ulnar z-plasty incisions are used to expose
the critical structures. (d) Finger extensors and the ECU are
identied through the ulnar incision and protected (blue
vessels loops). (e) The wrist capsule is opened and
255
et al. 2005). Although most published series
report only small numbers, we feel soft tissue
distraction to be indicated in all cases of severe
RLD where the wrist cannot be brought easily
over the ulnar head preoperatively (Fig. 14).
Microvascular Joint Transfer
A variety of bone grafts have been tried to recon-
struct the missing radius, including bular shaft,
bula with the distal epiphysis, and portions of the
ulna (Albee 1928; Starr 1945; Riordan 1955;
Heikel 1959). Most of these techniques were
abandoned due to complexity, nonunion, and the
inability of the bone to grow with the patient.
Microsurgical techniques allow for the transfer
of vascularized bone grafts that can include a
viable physis. Based on the earlier work of
Albee and Heikel, Vilkki has utilized the
vascularized second metatarsophalangeal joint
(MTP-joint) transfer to stabilize the carpus in
cases of RLD (Albee 1928; Heikel 1959; Vilkki
1998a, b).
The second MTP-joint is transferred to create a
new radial column within the wrist. This new
radial column consists of the metatarsal and
proximal phalanx of the second toe, which is
transferred to the distal ulna. The Y-shape recon-
struction provides support for the carpus with
growth potential in both the ulna and new radial
column (Vilkki 1998). The benets of this tech-
nique include avoiding injury to the carpus and
ulnar physis, preservation of wrist motion, and
preservation of ulnar growth (Vilkki 2008; de
Jong et al. 2012).
This surgical technique involves two stages.
In the rst stage, soft tissue distraction is used
to align the carpus with the ulna and to create a
space for the MTP-joint transfer in the distal
retracted to expose carpus (to the right of lower retractor)
and ulnar head (arrow). The remaining anlage and wrist
extensor tendons are released through the radial incision
(shown in Fig. 3a). (f, g) Photographic and (h) radiographic
appearance at 6 months prior to pollicization and at (i)
12-year follow-up
256
Fig. 13 Radialization technique involves aligning the
index nger metacarpal with the central portion of the
ulna. As in centralization, radial-sided wrist extensors can
be transferred to the ulnar aspect of the hand. Illustration
forearm. The second stage involves transferring
the MTP-joint to the affected forearm. The meta-
carpal is stabilized to the ulna with K-wires or
screws, and the toe is vascularized by end-to-end
anastomosis with the median artery. A small skin
paddle is often used to monitor the transferred
joint, in addition to providing additional soft tis-
sue coverage over the tight radial aspect of the
wrist (de Jong et al. 2012).
In Vilkkis long-term follow-up of 19 wrists
followed for over 11 years, average nal hand-
forearm angle was 28 of radial deviation. Total
active wrist motion averaged 83 (range,
30115 ). Overall ulnar growth averaged
15.4 cm and overall relative ulnar length was
67 % of the contralateral side (range, 5178 %).
Of the original nine patients, reported in 1998
N.A. van Alphen and S.L. Moran
also depicts correction of radial bow with concomitant
osteotomy at time of radialization procedure (Copyright
Mayo Foundation)
series, seven were noted to have an increase in
radial deviation of their wrist during the follow-up
period; however, this change averaged only 12
over a follow-up of 15.2 years. An improved
reach of 620 cm was noted in comparison to
the untreated other affected hand (Vilkki 1998,
2008). Pin tract infections were the most common
complication with vascular complications
being rare (Vilkki 1998; de Jong et al. 2012).
This procedure is considered a novel alternative
for centralization, and early reports show less
recurrent deformities compared to traditional cen-
tralization techniques (Vilkki 1998); however,
disadvantages include the necessity of two surgi-
cal procedures, the need for microvascular sur-
gery, and the sacrice of the second toe for
reconstruction (Fig. 15).
11 Radial Deficiency
Fig. 14 (ad) Pre-centralization soft tissue distraction can
be performed with a number of devices to aid in position-
ing carpus over the ulna and reducing the need for carpal or
ulnar cartilage resection. (a) Radiographic and (b) clinical
image of a Kessler frame used for distraction. Soft tissue
Timing of Surgery
There is no consensus on a specic timeframe for
surgical intervention, but in general, wrist central-
ization or radialization is often performed
between 6 and 18 months of age (Bayne and
Klug 1987). Surgery is performed with the goal
that the infant will have a stable wrist for bimanual
activities as well for aiding in early ambulation.
Delayed cases of RLD involving toddlers and
young adults are usually associated with a more
severe contraction of radial structures including
the neurovascular bundle, which complicates the
surgery (Buck-Gramcko 1985). Bayne noted that
50 % of the good to excellent results were seen in
children treated before 3 years of age (Bayne and
Klug 1987). This age group may also represent a
subset of patients that are brace compliant, which
was also found to contribute to better results
(Bayne and Klug 1987). As children move past
the age of 8, centralization becomes more difcult
and children are at higher risk of suppressed ulnar
growth following the procedure (Lamb 1977).
Additional procedures for thumb deciency are
257
and anlage release are performed at time of distractor
placement. Newer frames can allow for unidirectional or
bidirectional distraction. (c) Preoperative radiographic and
(d) nal photographic image of a 5-year-old with RLD
treated with biaxial distraction prior to centralization
usually performed before the child enters pre-
school at 25 years of age. Correction of the
short forearm after centralization may be
performed between 8 and 14 years of age (Peter-
son et al. 2007).
RLD treatment with microvascular joint trans-
fer is usually performed between the ages of 2.5
and 4 (de Jong et al. 2012). This time is needed for
the limb to grow in order to accept the placement
of a distraction apparatus and to allow increased
size of the toe and vessels to facilitate microvas-
cular surgery.
Surgical Outcomes and Function
There are few comparative outcome studies to
evaluate the function of the untreated and treated
RLD hand. Kotwals retrospective analysis exam-
ining 137 nonoperatively managed hands com-
pared to 309 hands managed with centralization
or radialization found that the subjective,
objective, and functional measures were signi-
cantly better in the operative group. Improved
258
Fig. 15 (a) Radiograph of a child with Bayne type III
RLD. (bc) Radiographs 2 years following Vilkki proce-
dure showing correction of radial deviation and volar
alignment, nger and wrist range of motion, and
grip strength were related to improvement in func-
tional score. Surgery improved appearance and
functional ease of performance of activities
when compared with nonoperatively managed
hands (Kotwal et al. 2012).
N.A. van Alphen and S.L. Moran
carpal subluxation. (d) Clinical appearance of hand
2 years following surgery
Although not considered a direct complication
of surgical treatment, the main problem in surgical
treatment of RLD is recurrence of the radial devi-
ation. Many have described that the outcome fol-
lowing surgery tends to worsen over time (Heikel
1959; Manske et al. 1981; Bayne and Klug 1987;
11 Radial Deficiency
Kawabata et al. 1998; Geck et al. 1999; Damore
et al. 2000; Goldfarb et al. 2002). Different mod-
ications over the years, such as tendon transfers,
are usually surgeon specic and confound objec-
tive comparison between different studies. Fur-
thermore, the high incidence of associated
anomalies limits objective outcome studies
(Damore et al. 2000). Recurrence of radial defor-
mity after surgical treatment is related to several
factors, such as preoperative hand-forearm angle,
amount of intraoperative correction, and compli-
ance with follow-up treatment. In patients with
severe RLD and high preoperative hand-forearm
angle, the greatest amount of correction can be
achieved with surgery. Nevertheless, this group of
patients has the highest recurrence rate (Damore
et al. 2000).
Although recurrence over time seems inevita-
ble, surgical treatment of RLD should still be
considered as the best treatment option for severe
RLD patients. Surgical treatment of RLD results
in improved appearance, function, and better per-
formance in daily activities when compared with
nonsurgically treated hands (Kotwal et al. 2012).
It is also interesting to note that in Baynes study,
most of the untreated adult patients did not request
surgery for functional reasons, but would have
preferred surgery for aesthetic improvement
(Bayne and Klug 1987).
Despite residual or recurrent deformity follow-
ing surgery, overall adaptability by these children
is impressive. Buffart performed a functional out-
come study of 20 patients with RLD to assess
impairment in hand function and link these limi-
tations to activities of daily living. Results showed
the average grip and pinch strengths were 36 %
and 30 % of reference values, respectively. There
was a decreased motion at the metacarpo-
phalangeal and proximal interphalangeal joints
compared to normal values. Decreases in grip
and pinch strength as well as joint motion were
related to the severity of the RLD. Despite these
limitations, the mean assisting hand assessment
(AHA) score was high (85.5 points) as was the
mean prosthetic upper extremity functional index
(PUFI) score (81.8 points), both measured on a
0100-point scale. Signicant relationships were
found between impairments in hand function and
259
activity performance; however, despite marked
impairments in hand function, children with radial
deciency performed functional activities fairly
well. Relationships between impairments in hand
function and limitation of activities were not lin-
early correlated implying considerable ability to
adapt (Buffart et al. 2008).
Similar functional studies by Ekblom and col-
leagues have investigated the relations between
deformity and the activity among individuals
with RLD. In this study of 20 children with vary-
ing severity of RLD, they noted that the mean total
active motion of the wrist (49.6 ) and digits
(44.7 ) were less than norms. The mean hand-
forearm angle was 34 radial. Ulnar length ranged
from 40 % to 80 % of age-related norms. Grip
strength (mean 2.7 kg) and Box and Block Test
(mean 33.8 blocks/min) were considerably lower
than for age-related norms. The mean score for the
AHA was 55.9, much lower than in Buffarts
study. The AHA score had a signicant relation-
ship with the total range of motion of digits. Hand-
forearm angle did not show any signicant rela-
tionship with Box and Block Test or AHA score.
The researchers concluded that in RLD, total
range of motion of the digits and wrist may be of
more importance to the childs activity and partic-
ipation than the angulation of the wrist (Ekblom
et al. 2013).
Forearm Lengthening
Forearm distraction lengthening has been
recommended by several authors to improve
upon short forearm length as the child approaches
skeletal maturity. Catagni reported on the results
of ve patients with improvements ranging from
4 to 13 cm of length (Catagni et al. 1993). Farr
reported an average increase in forearm length of
7 cm with improvement in ulnar bowing and
hand-forearm angle. Peterson and colleagues
also reported an average of 4.4 cm of added length
in 13 distraction procedures (Peterson et al. 2007).
Multiple complications were seen in all series
with pin infection being the most common (Peter-
son et al. 2007; Farr et al. 2012). Delayed union,
nonunion, and recurrence of deformity have also
260
Fig. 16 (ad) Late forearm lengthening (after centraliza-
tion) can be accomplished with many different external
distraction devices. (a) Preoperative radiographs of a
14-year-old girl with TAR syndrome who underwent
5 cm of axial distraction using monorail xator. (b, c)
been reported. In certain instances, plate xation
is recommended after lengthy distraction to avoid
late fracture (Farr et al. 2012; Fig. 16).
Management of Complications
A variety of complications can occur with the
procedures described above, but the most com-
mon and plaguing issue is recurrent radial devia-
tion. Other complications include iatrogenic
injury to the ulnar physis, carpal growth centers,
or cartilage. Hand ischemia has been reported in
cases of attempted centralization, resulting in
aborting the procedure (Bayne and Klug 1987).
Other complications following radialization and
centralization include broken and infected
N.A. van Alphen and S.L. Moran
Pins are placed above and below the carpus to limit amount
of radial wrist deviation and exion which may occur
during distraction process. (d) A plate is placed on the
ulna at the time of distractor removal to prevent recurrence
or fracture within newly generated bone
hardware (Manske et al. 1981; Buck-Gramcko
1985; Bayne and Klug 1987; DArcangelo
et al. 2000; Goldfarb et al. 2002; Kotwal
et al. 2012). Wrist fusion can be considered in
skeletally mature patients or ulnocarpal epiphy-
seal arthrodesis in the skeletally immature patient
if marked recurrent radial deviation is problem-
atic. Indications for arthrodesis include radial
angulation of greater than 45 and the inability
to actively extend the wrist with a xed exion
deformity of 25 or more (Pike et al. 2010).
The most frequent complication of
pre-centralization soft tissue distraction is pin
tract infection of the external distraction device
(Kessler 1989; Smith and Greene 1995;
Sabharwal et al. 2005; Peterson et al. 2007;
McCarthy et al. 2009; Dana et al. 2012). These
11 Radial Deficiency
infections can usually be treated with antibiotics.
Persistent infection or pin loosening requires
removal of the specic pin. In addition, nger
and metacarpal joint tightness can occur during
the distraction process requiring slowing of the
distortion rate and therapy.
Conclusion
RLD remains a difcult problem resulting in func-
tional limitations, particularly in bilateral cases.
This deformity is commonly associated with other
organ anomalies that need to be identied and
treated prior to hand surgery. While centralization
remains the most commonly performed treatment
modality to date for RLD, other procedures such
as vascularized epiphyseal transfer may provide a
more durable option in the future. Better outcome
measures will be needed to truly measure surgical
success. Prospective multicenter studies will help
to determine the subset of patients that benet
from surgery and methods to decrease the recur-
rence of radial deviation.
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 Ulnar Deficiencies
12
Mohammad M. Al-Qattan and Abdullah Al-Thunyan

Contents Abstract
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 265 Ulnar ray deciency is a rare anomaly occur-
Epidemiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 266 ring in 1:50,000 to 1:100,000 live births. It is
Etiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 266 usually seen as sporadic but may also be seen
Embryology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 266 as familial or syndromic. Embryologically,
Clinical Features and Classication Systems . . . . . . . 267
Which Classication System Should Be Used ulnar ray deciency occurs secondary to a de-
for Research? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 267 ciency of the Sonic Hedgehog that is the main
Functional Assessment of the Limb in controller of the anteroposterior axis of limb
Patients with Ulnar Ray Deciency and development. The clinical features are classi-
Indications for Surgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 269
Surgical Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 271 ed by various classication systems of the
Summary and Conclusions . . . . . . . . . . . . . . . . . . . . . . . . . . 274 deformities seen at the shoulder, elbow, fore-
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 274
arm (i.e., degree of deciency of the ulna),
ngers, and thumb. Functional assessment of
the limb/hand dictates the indications for sur-
gery. Surgical management includes: excision
of the ulnar anlage, wedge osteotomy for bow-
ing of the radius, de-rotational osteotomies,
excision of the radial head, one-bone forearm
operation, thumb/rst web space reconstruc-
tion, forearm lengthening, and other proce-
dures such as correction of concurrent
syndactyly and clinodactyly.

Introduction

No conict of interest. This work was supported by the Ulnar ray deciencies have been given other
College of Medicine Research Center, Deanship of names such as ulnar longitudinal deciencies,
Scientic Research, Riyadh, Saudi Arabia.
postaxial longitudinal deciencies, ulnar
M.M. Al-Qattan (*) A. Al-Thunyan dysmelia, and ulnar club hand. The term describes
Divisions of Plastic Surgery, King Saud University and
a rare congenital upper limb abnormality affecting
King Fahad National Guard Hospital, Riyadh,
Saudi Arabia the entire upper limb (and not only the ulnar ray)
e-mail: moqattan@hotmail.com; althunyan@hotmail.com with a broad spectrum of severity.
# Springer Science+Business Media New York 2015 265
J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_12
266 M.M. Al-Qattan and A. Al-Thunyan

Table 1 Syndromes associated with ulnar ray deficiencies

Syndrome
Weyers oligodactyly syndrome
Cornelia de Lange syndrome (mutation of
the cohesion complex)
Femur-bula-ulna syndrome (proximal
femoral focal deciency)
Ulnarmammary syndrome (Schinzel
syndrome) (TBX3 mutation)
Langer mesomelic dysplasia (mutation of
both copies of the SHOX gene)
Pillay syndrome (opthalmo-mandibulo-
melic dwarsm)
ReinhardtPfeiffer mesomelic dysplasia
Al-Qattan palmar duplication syndromeb
(WNT7A mutation)
a
Note that mutations of a single copy of the SHOX gene cause LeriWeill dyschondrosteosis
b
In the genetics literature, this syndrome is also known as Al-Awadi syndrome or Fuhrmann syndrome when the
phenotype is severe or mild, respectively
Clinical features
Ulnar and bular ray deciencies along with midline craniofacial defect
such as single central incisor and cleft lip/palate
Ulnar ray deciency, developmental delay, microcephaly, thick
eyebrows meeting at midline, short upturned nose, long philtrum,
excessive body hair, visual/auditory abnormalities
Deciency of ulna, bula, and femur
Ulnar ray deciency, breast/nipple hypoplasia/aplasia
Ulnar and bular ray deciency, severe short stature mainly to very
short legs (mesomelic)
Ulnar and bular ray deciency, blindness, fusion of
temporomandibular joints
Ulnar and bular ray deciency, shares some of the features of
LeriWeill dyschondrosteosisa
Double palm (i.e., the dorsal aspect of the hand has no nails or hair; with
the appearance of thick palmar skin on the dorsum), severe lower limb
deciency, variable degree of ulnar ray deciency

Epidemiology (as part of a syndrome). Familial cases frequently

Ulnar ray deciency is rare and it occurs in
1:50,000 to 1:100,000 live births (Froster and
Baird 1992). Longitudinal ulnar defects are
much rarer than longitudinal radial defects. The
occurrence of the former is one for every 410
occurrences of the latter (Ogden et al. 1976;
ORahilly 1951). The largest two series in the
literature are those of Al-Qattan et al. (2010) and
Swanson et al. (1984) reporting on 72 and
65 cases, respectively. Other reports only included
small case series (Blair et al. 1983; Broudy and
Smith 1979; Carroll and Bowers 1977; Spinner
et al. 1970; Straub 1965). Males are slightly more
frequently affected than females (3:2 ratio), and
the left side is slightly more commonly affected
than the right side. About one fourth of cases have
bilateral involvement.
Etiology
Ulnar ray deciencies may be sporadic (most
cases), familial (non-syndromic), and syndromic
have ulnar deciencies involving all four limbs
suggesting an unidentied gene mutation
(Roberts 1886; Wulfsberg et al. 1993). There is a
long list of syndromes in which an ulnar ray
deciency is a concurrent feature, and these
syndromes are listed in Table 1. Finally, ulnar
ray deciency may be seen as a frequent or
constant feature of other congenital hand anoma-
lies such as the ulnar-sided cleft anomaly
(Al-Qattan 2013a).
Embryology
Ulnar ray deciencies occur as a result of de-
ciency of the Sonic Hedgehog (SHH) (Al-Qattan
et al. 2010; Al-Qattan and Kozin 2013). SHH is
situated in the zone of polarizing activity (ZPA)
within the posterior part of hand paddle. However,
SHH diffuses out of the ZPA and acts as a
diffusable protein. SHH is considered as the
main controller of anteroposterior axis of limb
development. Three basic principles should be
known regarding this axis of development.
12 Ulnar Deficiencies
Firstly, the normal development of the thumb and
radial ray requires the absence of SHH anterior
expression and the presence of mesodermal
GLI3R/SALL4 and ectodermal FGF8 expression
anteriorly. In contrast, the normal development of
the ngers and ulnar ray (including the ulnar side
of the carpus and ulna) requires a graded SHH
expression with highest concentration at the little
nger and lowest concentration at the index n-
ger. Secondly, any deciency of SHH will lead to
ulnar ray deciency. In contrast, any overactivity
(or increased expression) of SHH will lead to the
ectopic anterior expression of SHH, and this will
lead to various types of radial polydactyly
(Al-Qattan 2013b). Thirdly, maintenance of
SHH activity is mediated by two main players:
(a) broblast growth factor 4 (FGF4) within the
posterior ectoderm, known as the FGF4-SHH
loop, and (b) the wingless WNT7A protein in
the dorsal ectoderm (Al-Qattan 2011). Therefore,
in Al-Qattan palmar duplication syndrome (see
Table 1), loss of function of the dorsalizing gene
WNT7A will not only lead to palmar duplication
but also to ulnar ray deciency (Al-Qattan 2011).
Similarly, the main pathology in ulnar-sided cleft
hand deformity is suppression of FGF4 within the
posterior ectoderm (Al-Qattan 2013a). Ulnar ray
deciency is a common concurrent feature in
ulnar-sided clefts because of a defective FGF4-
SHH loop (Al-Qattan 2013a).
Ulnar ray deciency is much less common than
radial ray deciency. Ogino and Kato (1988)
explained this by stating that the critical
(in utero) period of ulnar deciency was earlier
than that of other abnormalities and it
corresponded to a period of high mortality rate
of the fetus. The authors also induced ulnar ray
deciency in rats when a teratogen (busulfan) was
given between 9 and 10 days of gestation and
induced radial ray deciency when the same
drug was given between 10 and 11 days.
Embryologically, the ulnar artery develops
before the radial artery and before the develop-
ment of the ulnar ray (Al-Qattan and Kozin 2013).
This explains the ndings of Inoue and Miura
(1991) who carried out angiography in patients
with ulnar ray deciency and found that the ulnar
artery was the main artery to the hand and that
267
50 % cases had absent or severely hypoplastic
radial artery.
Clinical Features and Classification
Systems
Patients with ulnar ray deciency should have
complete history and systemic examination to
document familial and syndromic cases. Careful
clinical and radiological examination of the
lower limbs is required since bular hypoplasia
and focal femoral dysplasia are known concurrent
features (Fig. 1). Appropriate referrals to the
craniofacial team may also be required (see
Table 1).
The affected upper limb should have full clin-
ical, radiological, and functional assessment (see
Table 2). Shoulder instability is rare and was only
reported in a single child out of 72 cases of the
series reported by Al-Qattan et al. (2010).
The elbow may be classied as per Kummel
classication (Kummel 1895) to identify
radiohumeral synostosis (Fig. 2) and radial head
dislocation (Table 2).
The ulna may be normal and this was recently
stressed upon by Havenhill et al. (2005) and
was given the term type zero ulna. Ogden
et al. (1976) classied abnormalities of the ulna
from hypoplastic ulna to absent ulna (Table 2).
Ogino and Kato (1988) provided a classica-
tion of ulnar ray deciency according to the
degree of deciency of the ngers (Table 2).
Later, Cole and Manske (1997) stressed on con-
current thumb/rst web deciencies and realized
that surgical correction is frequently done for this
deciency. Hence, the authors recommended a
separate classication devoted to the thumb
(Table 2).
Which Classification System Should Be
Used for Research?
This is a controversial issue. Al-Qattan
et al. (2010) recommended combining the
existing ve classication systems shown in
Table 2. For example, an affected limb may be
268
Fig. 1 Unilateral ulnar ray deciency with ipsilateral focal
femoral dysplasia. (a) The hand with absent 2 ngers but
excellent function. (b) X-ray of the hand showing a slightly
classied as shoulder-Al-Qattan type I, elbow-
Kummel type III, ulna-Ogden type II, ngers-
Ogino/Kato type II, and thumb-Cole/Manske
type II.
Lorea et al. (2004) offered more detailed
descriptive information about the shoulder,
elbow, forearm, wrist, and hand. This may be
more comprehensive for research, but it did not
gain popularity probably because the identica-
tion of the individual features of its ve compo-
nents is via a variety of numbers and letters that
are not very workable.
The most commonly used single classication
system in scientic papers on ulnar ray deciency
is the modied Bayne classication which is
shown in Table 3. The original Bayne classica-
tion combines Ogden classication of the ulna and
M.M. Al-Qattan and A. Al-Thunyan
short ulna. (c) The deformity of the right lower limb. (d)
X-ray showing the femoral dysplasia. In this case, lower
limb management is the priority
adds a group with radiohumeral synostosis. In the
modied Bayne classication, another two groups
are added: type O ulna (Havenhill et al. 2005) and
the phocomelic type (Goldfarb et al. 2005;
Tytherleigh-Strong and Hooper 2003). In 2003,
Tytherleigh-Strong and Hooper (2003) noted that
some cases were previously diagnosed as
phocomelia, but careful examination of the
X-rays revealed a radiohumeral synostosis with
absent ulna. Hence the proper diagnosis should
have been ulnar ray deciency and not
phocomelia. This was later conrmed by Goldfarb
et al. (2005) who also noted that this apparently
single bone occupying the arm/forearm resem-
bled a humerus proximally and a radius distally.
This bone will frequently have a large medial
epicondyle at the level of what has been the
12 Ulnar Deficiencies 269

Table 2 Assessment/classification/grading of the clinical features of ulnar ray deficiency

Area of interest Authors Description/classication
Shoulder Al-Qattan et al. (2010) I stable (normal) shoulder
II shoulder instability
Elbow Kummel (1895) I normal
II radiohumeral synostosis
III radial head dislocation
Ulna Ogden et al. (1976); Havenhill et al. (2005) O normal
I hypoplastic ulna
II partial aplasia of the ulna (distal part)
III absent ulna
Fingers Ogino and Kato (1988) I little nger hypoplasia
II little nger absent
III absent ulnar two ngers
IV absent ulnar three ngers
V absent all four ngers
Thumb/rst web Cole and Manske (1997) I normal thumb/1st web
deformity II mild deciency
III moderate to severe deciency
IV absent thumb

differentiate it from the true phocomelia

(Al-Qattan et al. 2013).

Functional Assessment of the Limb in

Patients with Ulnar Ray Deficiency and
Indications for Surgery

Full functional assessment of the affected limb/

Fig. 2 Ulnar ray deciency with radiohumeral synostosis.
Note the absent ulnar 3 ngers and the hypoplastic ulna
elbow (Fig. 3). These patients are now put in
a separate group as type V, Bayne (Table 3).
In the genetics literature, the term apparent
phocomelia of ulnar ray deciency is used to
hand is essential not only for documentation and
research but also because it dictates indications for
surgery (Table 4). The range of motion at every
joint is documented. Power and pinch grips
should also be measured. Activity questionnaire
is also suggested by Blair et al. (1983).
The overall function of the limb may also be
documented as per the classication of Al-Qattan
et al. (2010). These authors recommended catego-
rizing the overall function into four groups as
good, fair, poor, or nil if the hand is used
effectively in daily activity, only used occasion-
ally to help the contralateral normal hand, rarely
used in daily activity, or if there is transverse
arrest at the wrist, respectively. Note should
be given transverse arrest at the wrist is also seen
in ulnar ray deciency (Swanson et al. 1984;
Fig. 4).
270 M.M. Al-Qattan and A. Al-Thunyan

Miller et al. (1986) introduced a specic clas- dislocation into excellent and poor function and
sication for the overall function of the limb in patients with radiohumeral synostosis into poor
patients with ulnar ray deciency (see Table 4). and good function (Fig. 3). The authors noted that
The classication divides patients with radial head poor function in patients with radial head disloca-
tion is usually associated with a completely absent
Table 3 The modified Bayne classification which is com- ulna and cubital webbing, while poor function in
monly used in clinical series patients with radiohumeral synostosis is associ-
Classication Description ated with hand on ank deformity. At rest, the
Original Bayne classication I hypoplasia of the hand in the latter group of patients faces posteri-
(Bayne 1982) ulna orly to lie on the ank.
II partial aplasia of Finally, the hand surgeon should specically
the ulna
examine the wrist and hand for deformities that
III absent ulna
considerably affect hand function such as marked
IV radiohumeral
synostosis ulnar deviation, which is usually progressive
Modied Bayne classication O normal ulna because of the presence of an ulnar anlage.
(Goldfarb et al. 2005; I hypoplastic ulna These patients usually have partial absence of
Havenhill et al. 2005; II partially aplastic the ulna, and the brous anlage connects the
Tytherleigh-Strong and ulna distal ulna to both the distal radius (across the
Hooper 2003)
III absent ulna epiphyseal line) and the carpal bones ulnarly.
IV radiohumeral Obviously, early excision of the anlage should
synostosis
be considered. The missing ulnar ngers
V phocomelic
ulnar ray deciency
have little effect on hand function and hence
surgery is not indicated for absent ulnar ngers.

Fig 3 A case of bilateral ulnar ray deciency: A function despite the absent elbows bilaterally. (b) X-ray of
phocomelic type on the right side (Bayne V) and right upper limb showing an apparently single bone
radiohumeral synostosis (with hypoplastic ulna) on the occupying the arm/forearm. Note the long medial
left (Bayne IV). (a) Clinical appearance. Note the good epicondyle at the level of what has been the elbow
12 Ulnar Deficiencies 271

Table 4 Functional assessment of patients with ulnar ray deficiency

Authors Assessment/classication
Blair et al. (1983) (A) Subjective assessment by activity questionnaire
(B) Objective measurements of range of motion/power grip/pinch grip
Al-Qattan (A) Good overall function: the limb/hand is used effectively in daily activity
et al. (2010) (B) Fair overall function: the limb/hand is used occasionally mainly as a helper to the
contralateral normal hand
(C) Poor overall function: the limb/hand is rarely used in daily activity (most of these patients
have hand on ank deformity)
(D) No functional use: ulnar ray deciency in forearm with transverse arrest at the level of the
wrist
Miller et al. (1986) (A) Radial head dislocation with delayed ossication of ulna and excellent function
(B) Radial head dislocation with absent ulna and poor limb function
(C) Radiohumeral synostosis with poor limb function because of hand on ank deformity
(D) Radiohumeral synostosis with good function

Fig. 4 A case of bilateral ulnar ray deciency with transverse arrest at the wrist on the left side. (a) Clinical appearance of
the left side. (b) X-ray appearance. Note the hypoplastic radius and absent ulna

In contrast, thumb/rst web space deformities Surgical Management

(Table 2) should be treated surgically prior to
2 years of age. Finally, other concurrent digital
abnormalities such as syndactyly, clinodactyly,
and polysyndactyly may be seen in up to 55 %
patients (Al-Qattan et al. 2010), and these defor-
mities are also treated surgically to improve
function.
A. Excision of the ulnar anlage only
Patients who present early, excision of the
bro-cartilaginous anlage should be done
prior to 6 months of age. An ulnar incision is
made and identication of the ulnar artery/
nerve should be done rst. The anlage should
272 M.M. Al-Qattan and A. Al-Thunyan

Fig. 5 A case of hand on

ank deformity. The
function of the limb is
greatly improved by
de-rotation osteotomy of the
humerus. (a) Preoperative
appearance. The child
rarely used the hand which
is placed backwards on the
ank. (b) Preoperative
X-ray. Note the
radiohumeral synostosis.
(c) Postoperative
appearance. The child is
now frequently using the
hand in daily activities. (d)
Postoperative X-ray
showing xation of the
osteotomy with the a plate
and screws

be totally excised from its three attachments:
the distal end of the ulna, the radial epiphysis,
and the carpal bones. A temporary K-wire is
used to immobilize the wrist in zero10  of
radial deviation. An ulnar gutter splint is used
after removal of the K-wire. This procedure is
most commonly done in infants with type II
Bayne (Bayne 1982).
B. Excision of the ulnar anlage with wedge
osteotomy of the radius
In patients who present late or with substantial
bowing of the radius, excision of the ulnar
anlage should be combined with a closing
wedge osteotomy of the radius. The choice of
xation of the osteotomy (K-wires or plates)
will depend on the age of the child.
C. De-rotational osteotomies
This is usually indicated in patients with
radiohumeral synostosis with internal rota-
tional deformity of the shoulder and in patients
with hand on ank deformity (Fig. 5). The
de-rotational osteotomy may be done at the
level of the humerus or the forearm (Carroll
and Bowers 1977). For every 1 mm of
de-rotation of the humerus, about 10 of pos-
ture change is seen clinically (Al-Qattan 2002).
Excessive de-rotation should be avoided. Fixa-
tion should be done by plates and screws.
12 Ulnar Deficiencies 273

Fig. 6 Unilateral ulnar ray

deciency with: absent
ulna, radial head
dislocation, absent ulnar
2 ngers, and tight rst
web/hypoplastic thumb. (a)
Clinical appearance. (b)
X-ray. Note that the radial
head dislocation is not
limiting elbow extension,
and hence, no surgery is
indicated at the elbow. The
absent ngers do not require
reconstruction. The priority
here is rst web space
reconstruction and
opponensplasty

D. Excision of the radial head

Radial head dislocation does not usually
require surgery. However, some older children
with radial head dislocation will suffer from
limitation of elbow extension as well as limi-
tation of forearm rotation secondary to the
dislocation. In these cases, excision of the
dislocated radial head will improve function
if there is a stable forearm.
E. The one-bone forearm operation
This is indicated in patients with radial head
dislocation, forearm instability, and with marked
limitation of elbow extension. These patients are
not candidates for simple excision of the radial
head because of the forearm instability.
The procedure of one-bone forearm is best
done through an ulnar incision. Following
excision of the ulnar anlage, a proximal trans-
verse osteotomy of the radius is done. The
distal radial diaphysis is brought into align-
Fig. 7 Ulnar ray deciency with syndactyly requiring
ment with the remaining proximal ulna. Fixa- release
tion of the radius to the ulna is done by
longitudinal intramedullary K-wires (Smith F. Thumb/rst web space reconstruction
and Greene 1995). Extraperiosteal excision of Procedures to the thumb/rst web space should
the proximal radius is done at the same time or be done using the principles of surgery for
at a second stage through a direct incision thumb hypoplasia (see chapter Thumb
several months later (Bayne 1982). Hypoplasia). Widening of the rst web space
274
Fig. 8 Bilateral ulnar ray deciency. (a) The left side required no surgery, but the right side required clinodactyly
correction and thumb/rst web space reconstruction. (b) Correction of clinodactyly by wedge osteotomy
may be done using Z-plasty, the 5-ap
technique, a skin graft, or a skin ap depending
on the severity of the rst web deciency
(Fig. 6).
The thumb is frequently in the same plane as
the ngers. Rotational osteotomy of the thumb
ray may be done either at the level of metacar-
pal or the proximal phalanx.
In cases of severe thumb hypoplasia with a
nonfunctioning basal joint and in cases of oat-
ing or absent thumb, pollicization of the index
nger should be done either using the classic
Buck-Gramcko technique (Buck-Gramcko
1971) or the newly described simpler tech-
nique of Al-Qattan (2012) without
interosseous muscle or extensor tendon
repositioning.
G. Forearm lengthening
Forearm lengthening may be used for prelim-
inary soft-tissue distraction (Smith and Greene
1995) or for lengthening of the bones of the
forearm (Horii et al. 2000).
H. Other procedures
Corrections of other concurrent congenital
anomalies are main indications for surgery
which include correction of syndactyly
(Fig. 7), clinodactyly (Fig. 8), and
polysyndactyly (Al-Qattan et al. 2010).
M.M. Al-Qattan and A. Al-Thunyan
Occasionally, excision of oating ulnar digit
is indicated for aesthetic reasons.
Summary and Conclusions
This chapter reviews the epidemiology, etiology,
embryology, clinical features, classication sys-
tems, functional assessment, and surgical man-
agement of patients with ulnar deciency.
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 Syndactyly
13
M. Claire Manske and Charles A. Goldfarb

Contents Abstract
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 277 Syndactyly, or webbed digits, is one of the
Pathoanatomy and Applied Anatomy . . . . . . . . . . . . . . . 278 most common congenital hand anomalies.
Assessment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 279 While syndactyly most often occurs as an
Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 283 isolated condition, it may be associated with
Operative Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 285
other upper extremity musculoskeletal
Surgical Procedure: Simple and Complex anomalies, including cleft hand, ulnar
Release (Incomplete and Complete) . . . . . . . . . . . . . . . 285
Preoperative Planning . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 285
longitudinal deciency, symbrachydactyly,
Positioning . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 286 and synpolydactyly. Syndactyly may involve
Preferred Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 293 the skin only or may include the bone. Treat-
Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 294 ment with digit separation and web space
reconstruction is designed to maximize func-
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 294
tion and appearance. There are a variety of
described surgical techniques to accomplish
these goals. Outcomes vary depending on the
web space involved and type of syndactyly,
among other factors.

Introduction

Syndactyly, or webbed digits, is one of the most

common congenital hand anomalies, occurring in
1 in 2,000 live births (Flatt 1977; Percival and
Sykes 1989). It is twice as common in males as
in females (Kay et al. 2011) and occurs bilaterally
in 50 % of affected patients. It occurs ten times
more frequently in Caucasian patients compared
to African American patients (Flatt 2005). Syn-
M.C. Manske C.A. Goldfarb (*) dactyly most commonly occurs sporadically, but
Department of Orthopaedic Surgery, Washington
1040 % of affected individuals have a family
University School of Medicine, Saint Louis, MO, USA
e-mail: goldfarbc@wudosis.wustl.edu; history of syndactyly. In those with a family his-
manskem@wudosis.wustl.edu tory, most are affected in an autosomal dominant
# Springer Science+Business Media New York 2015 277
J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_13
278
fashion, with variable expressivity and incom-
plete penetrance.
While syndactyly most often occurs as an iso-
lated condition, it may be associated with other
upper extremity musculoskeletal anomalies,
including cleft hand, ulnar longitudinal de-
ciency, symbrachydactyly, and synpolydactyly.
Syndactyly may also present as part of Poland
syndrome, a hypoplasia of the pectoralis major
muscle. Additionally, acrocephalosyndactyly dis-
orders (e.g., Apert syndrome, Chotzen syn-
drome), acrocephalopolysyndactyly disorders
(Noack syndrome, Carpenter syndrome) , and
orofaciodigital and oculodentodigital disorders
often include syndactyly (Flatt 2005; Jones
et al. 2011). Finally, amniotic band syndrome
may present with a terminal syndactyly in which
the digits are conjoined distally by a soft tissue
bridge and are fenestrated proximally (Patterson
1961). This differs from the other syndactylies as
this acrosyndactyly is a deformation related to
scarring in utero rather than a malformation, char-
acterizing other syndactylies.
In isolated syndactyly, the long-ring nger web
space is the most commonly affected (57 %),
followed by the ring-small nger web space
(27 %). Involvement of the index web spaces is
less frequent (index-long web 14 %; thumb-index
web space 3 %). In syndromic syndactyly, the
involvement of web spaces is more evenly distrib-
uted, although a similar pattern of involved digits
is noted, with 41 % ring-long, 27 % ring-small,
23 % index-long, and 9 % thumb-index web space
involvement (Flatt 2005).
Pathoanatomy and Applied Anatomy
Syndactyly results from a failure of digital separa-
tion and web space formation. The complex pro-
cess of hand and digit development begins with
the outgrowth of the somatic and lateral plate
mesoderm into the overlying ectoderm to form
the apical ectodermal ridge (AER) (Al-Qattan
and Kozin 2013). This developing limb bud rst
appears at 26 days of gestation with three axes of
limb growth: proximal-distal, anterior-posterior,
and dorsal-ventral. By day 33, the digital rays are
M.C. Manske and C.A. Goldfarb
present in the hand paddle. Digital separation typ-
ically begins by gestational day 47 and is complete
by day 54, proceeding from distal to proximal.
Sonic hedgehog (SHH) interacts with bone mor-
phogenetic proteins (BMP) to inuence the forma-
tion and differentiation of digits, in part by
inducing apoptosis in the interdigital mesenchyme
cells (Oberg et al. 2010). Failure of apoptosis of
the interdigital mesenchyme is thought to be
responsible for syndactyly (Al-Qattan and Kozin
2013; Al-Qattan et al. 2009).
A vascular insult to the progress zone of the
mesoderm has been proposed as the etiology/
pathogenesis of symbrachydactyly, Poland syn-
drome, and Apert syndrome. Bavinck and Weaver
described subclavian artery supply disruption
syndrome, in which the occlusion of the subcla-
vian artery and its branches at specic time points
in embryonic development leads to predictable
upper extremity defects (Bavinck and Weaver
1986).
The genetic basis of some of the syndromic or
associated syndactylies has been elucidated. The
abnormality responsible for Apert syndrome is a
missense substitution in the broblast growth fac-
tor receptor 2 gene on chromosome 10q26, either
Pro-253 Arg or Ser-252 Trp. The former of these
two mutations results in severe syndactyly, while
the latter of these two is associated with craniofa-
cial defects (Wilkie et al. 1995). Synpolydactyly
may be caused by an autosomal dominant muta-
tion with variable penetrance at one of three loci.
SPD 1 is caused by a mutation in the HOXD13
gene at the 2q31 locus, SPD 2 results from a
FBLN1 mutation at the 22q13.31 locus, and
SPD3 is associated with a mutation at the
14q11.2-q12 locus (Malik and Grzeschik 2008).
The anatomic structures involved in the
syndactylized digits are variable and depend on
the severity and complexity of the syndactyly (see
section Classication). There is always cutane-
ous involvement, and although the skin and sub-
cutaneous tissues are normal, the amount of skin
covering the conjoined digits is inadequate to
circumferentially cover each digit individually
as the circumference of two separated digits is
30 % greater than two syndactylized digits
(Tonkin 2009). Additionally, the fascia bridging
13 Syndactyly
the interdigital space is thickened beyond the nor-
mal Grayson and Cleland ligaments. Thickening
of the palmar fascia can also be seen, especially in
the rare cases when syndactyly involves the
thumb-index interspace (Ezaki 1998). Depending
on the complexity of the syndactyly, other struc-
tures affected may include nail, vessels, nerves,
tendons, and bone.
Assessment
Clinical and Radiologic Evaluation
A complete clinical evaluation includes an assess-
ment of the web space or spaces involved, the
extent of the syndactyly (complete or incomplete),
and whether the syndactyly is cutaneous or com-
plex (bony involvement). Presence of synonychia
(conjoined nails) or absence of differential motion
between digits indicates complex syndactyly. An
Allen test of the syndactylized digits may provide
insight into the vascular supply to each digit. The
presence of other musculoskeletal or craniofacial
anomalies is noted, given the association of syn-
dactyly with other syndromes. If a syndrome is
suspected, a full clinical exam, including evalua-
tion of the entire affected extremity, contralateral
extremity, chest, bilateral lower extremities, and
face, is performed. Moreover, digits that are short-
ened, in addition to being syndactylized, should
alert the clinician to possible symbrachydactyly or
an acrosyndactyly, which affect operative man-
agement. In these children, the pectoralis major
is evaluated, as its absence or hypoplasia indicates
Poland syndrome.
Radiographs of the affected hand evaluate
osseous involvement that may include bony
fusion, extra digits (synpolydactyly), accessory
bones, or other articular deformities. Although
rarely indicated, ultrasound, magnetic resonance
imaging, or angiography may be useful in
extremely complex cases to evaluation tendon
and vascular anatomy.
Classification
Historically, International Federation of Socie-
ties for Surgery of the Hand (IFSSH) classica-
tion system, proposed by Swanson in 1964 and
279
accepted by the IFSSH in 1976, has been utilized
to categorize upper extremity anomalies. This
system includes seven categories based on mor-
phologic appearance with syndactyly classied
as failure of differentiation (Swanson 1976).
Recently, the Swanson classication was modi-
ed to incorporate an improved understanding of
the genetic and molecular basis of specic con-
genital anomalies (Oberg et al. 2010). This clas-
sication system, based on developmental
biology, contains three broad categories
(malformations, deformations, and dysplasias),
each with multiple subtypes. In this system, syn-
dactyly is classied as a malformation, with the
subtype failure of hand plate formation/differen-
tiation. A benet of this system is that it attempts
to categorize deformities based on developmen-
tal biology and, thus, should make classication
more precise, avoiding the problem of a defor-
mity falling into multiple categories or no cate-
gories at all.
Syndactyly is most commonly characterized
by the anatomic structures involved and the
extent of digital union. According to this system,
syndactyly is described as simple (Fig. 1) or com-
plex (Fig. 2) and complete (Fig. 1) or incomplete
(Fig. 3). Simple (or cutaneous) syndactyly
involves only skin and subcutaneous tissue,
whereas complex syndactyly implies fusion of
underlying bone with possible involvement of
tendons, nerves, and vessels. Complex syndactyly
most commonly involves the fusion of the
distal phalanges, but may also include the
presence of accessory phalanges. Complete
syndactyly refers to digits that are joined the full
length of the nger to the distal ngertip, whereas
incomplete syndactyly ends proximal to the
ngertip.
Kozin proposed a modication of this classi-
cation scheme to include the digits involved and
the severity of the interconnection (Kozin 2001).
Kozins classication system provides guidance
into the timing and optimal technique for
operative intervention. Simple syndactyly
(SS) was divided into standard (s), complicated
(c), and urgent (u) subtypes. Complex syndactyly
(CS) also was further subdivided into standard (s),
complicated (c), and unachievable (u) subtypes.
280 M.C. Manske and C.A. Goldfarb

Fig. 1 (a and b) Simple or cutaneous syndactyly. This is a complete syndactyly, best demonstrated from the palmar
surface (Copyright Charles A. Goldfarb, MD)

Fig. 2 (a) Clinical appearance of complex syndactyly (b) Radiographs show connection between distal phalan-
demonstrating synonychia and tight approximation of ges (Copyright Charles A. Goldfarb, MD)
distal phalanges (copyright Charles A. Goldfarb, MD).
13 Syndactyly 281

Fig. 4 A three-year-old with ulnar deciency and syndac-

Fig. 3 Incomplete syndactyly of the ring and small ngers tyly. Distal phalangeal tufts are fused and a common n-
(Copyright Charles A. Goldfarb, MD) gernail or synonychia crosses the bony mass (Courtesy of
Shriners Hospital for Children, Philadelphia)

Simple Syndactyly (SS)

Standard (SSs): Straightforward syndactyly on
a non-border digit. Surgery can be delayed
until 18 months of age or later.
Complicated (SSc): Simple syndactyly associ-
ated with additional soft tissue interconnec-
tions, syndromes (e.g., Poland syndrome,
central deciency), or additional bony ele-
ments (e.g., hypoplasia). Treatment is individ-
ualized and neurovascular anomalies may
exist.
Urgent (SSu): Soft tissue syndactyly of border
digits or digits of unequal length, girth, or joint
level. It requires early separation to prevent
angular and rotational deformity of the tethered
digit.

Fig. 5 A three-year-old with amniotic band disruption

Complex Syndactyly (CS)
Standard (CSs): Complex syndactyly of adja-
cent phalanges without additional bony abnor-
malities (e.g., without delta phalanx,
symphalangism).
Complicated (CSc): Complex syndactyly
associated with bony interconnections
(e.g., transverse phalanges, symphalangism,
polysyndactyly) or syndromes (to include
amniotic constriction band). Treatment is
sequence and complicated syndactyly (Courtesy of
Shriners Hospital for Children, Philadelphia)
individualized with the consideration that
digits may function better as a unit (Figs. 4
and 5).
Unachievable (CSu): Complex syndactyly
with severe abnormalities of the underlying
bony structure that often prohibits formation
of a ve-digit hand.
282
Fig. 6 A two-year-old with syndactyly combined with
duplication of the ring nger (synpolydactyly) (Courtesy
of Shriners Hospital for Children, Philadelphia). (a):
Tentamy and McKusick classied isolated,
non-syndromic syndactyly to include ve differ-
ent types (Flatt 2005; Tentamy and McKusick
1978; Tonkin 2009).
Syndactyly type I (SD I, zygodactyly) is the
most common type, affecting the long-ring
web space with possible involvement of the
second and third toes. It may be complete or
incomplete and bilateral or unilateral and
usually autosomal dominant with variable
penetrance.
Syndactyly type II (SD II, synpolydactyly) also
involves the long-ring web space and possibly
the fourth and fth toes, but includes a dupli-
cation of the ring nger within the
syndactylous web (Fig. 6).
Syndactyly type III (SD III) is characterized by
syndactyly at the ring-small nger web space.
It may occur as an isolated syndactyly but is
also seen in oculodentodigital syndrome.
M.C. Manske and C.A. Goldfarb
Dorsal view of hand with syndactyly. (b): X-ray reveals
ring nger duplication with awkward interconnections
Syndactyly type IV (SD IV, Haas type) is
extremely rare and involves complete syndac-
tyly of all digits.
Syndactyly type V (SD V) involves fusion of
the metacarpals and metatarsals, most com-
monly of the ring and small ngers and third
and fourth toes.
Since their original description, four additional
types have been added to the Tentamy and
McKusick classication (Jordan et al. 2012).
Syndactyly type VI (SD VI, Mitten Hand)
Syndactyly type VII (SD VII, Cenani-Lenz)
Syndactyly type VIII (SD VIII)
Syndactyly type IX (SD IX, Mesoaxial)
Within complex syndactyly, Apert
acrosyndactyly has been further classied.
The three types of (Fig. 7) syndactyly all involve
13 Syndactyly
complex syndactyly of the second through fth
digits with increasing involvement of the thumb:
type 1 (spade hand) has a free thumb with a
narrow rst web space, type 2 (spoon hand) has
a simple syndactyly of the thumb to the index
nger, and type 3 has a complex syndactyly of
the thumb to the index nger (Upton 1991). This
classication has been further modied by Van
Heest, based on angular deformity of the
metacarpophalangeal joint and rotation deformity
of the digits. Treatment goals and procedure rec-
ommendations are based on this classication
system.
Outcome Assessment
Although there is no outcome scale or tool
designed specically to assess the results of syn-
dactyly treatment, there is general consensus on
important outcome measures. Range of motion,
digit deformity (angulation and rotation), scar
quality (Vancouver Scar Scale), and web creep
are all utilized in assessing outcomes.
Range of motion can be assessed via measure-
ment of the individual joints, but total active
motion, as described by Strickland and Glogovac,
is commonly utilized. Total active motion is the
sum of active exion of the PIP and DIP joint
minus composite extension decits; normal is
175 (Strickland and Glogovac 1980).
Digit deformity is an assessment of both the
rotation and angulation of the digit. Rotation
(supination or pronation) describes the plane of
the distal phalanx relative to the plane of the
proximal phalanx; a goniometer placed on the
dorsal surface of the distal phalanx is to measure
the degree of difference in these planes (Goldfarb
et al. 2012). To determine digital angulation, the
deviation of the longitudinal axis of the distal
phalanx from the longitudinal axis of the proximal
phalanx is measured with a goniometer.
Additional assessment measures may be uti-
lized to evaluate the outcomes of syndactyly
reconstruction. Although originally designed for
assessment of scars in burn patients, the Vancou-
ver scar scale (VSS) has been applied to the
assessment of syndactyly to provide an objective
measure of scar quality compared to normal skin
283
(Goldfarb et al. 2012; Lumenta et al. 2010).
This scale incorporates four variables including
vascularity, height/thickness, pliability, and
pigmentation and assigns points to each parameter
to generate a composite score (Sullivan
et al. 1990).
Web creep is the distal migration of the com-
missure after syndactyly reconstruction. The most
commonly used scale to assess creep is a ve-
point scale described by Withey that grades
creep based on the location of the distal commis-
sure relative to the proximal interphalangeal (PIP)
joint (Withey et al. 2001). Zero points are awarded
to a normal web with soft skin, 1 point for thick-
ening of the web without advancement, 2 points
for web creep one-third of the distance from the
palmar metacarpophalangeal (MCP) joint to the
PIP joint crease, 3 points for web creep two-thirds
of the distance from the palmar MCP to the PIP
joint crease, and 4 points for web creep from the
palmar MCP to PIP joint crease.
Treatment
Nonoperative Management
Indications/Contraindications
The goal of syndactyly management is to create a
more functional and aesthetically normal hand. As
such, operative intervention is often indicated;
however, in certain situations surgery is either
contraindicated or not warranted.
An absolute contraindication to surgical inter-
vention is the presence of medical comorbidities
that render the patient unable to undergo anesthe-
sia. A relative contraindication is the presence of
digits that lack the requisite anatomic structures,
including muscles, tendons, and ligaments,
to function as stable, independent, mobile
digits when separated. This can occur in
symbrachydactyly, synpolydactyly, or other com-
plex syndactylies, in which the digits may be more
functional as a unit. While surgery may improve
hand appearance, it would not improve, and may
impair, function. The presence of a super digit, in
which two metacarpals support a single oversized
284 M.C. Manske and C.A. Goldfarb

Fig. 7 A two-year-old with

Apert syndrome (Courtesy
of Shriners Hospital for
Children, Philadelphia). (a):
Typical facies. (b): Dorsal
view right hand with severe
syndactyly including
thumb-index web space.
(c): Palmar view right hand
with deep crevasses. (d):
Dorsal view of left hand.
(e): Palmar view of left hand
13 Syndactyly
Table 1 Syndactyly nonoperative management
Indications
Simple, incomplete syndactyly without functional
impairment
Medical comorbidities prohibiting surgery
Digits lacking anatomic structures to enable independent
digital function
Super digit
digit (type I super digit) or a single metacarpal
supports multiple digits (type II super digit), is
also a relative contraindication (Wood 1990).
If one of the digits becomes stiff, atrophic, and
aesthetically displeasing, surgical separation may
be warranted. Finally, surgery may not be
warranted in patients who experience minimal
functional impairment. This is most often the
case for simple, incomplete syndactylies as surgi-
cal intervention may improve the aesthetic
appearance of the hand, but it would not substan-
tially alter function. Careful discussion with
patients and families regarding the benets and
risks of surgical intervention in these cases is
undertaken before proceeding (Table 1).
There are no recognized nonoperative treat-
ment modalities for the management of
syndactyly.
Operative Treatment
Indication/Contraindications
Syndactyly of any type can be an indication for
surgery, most importantly if it impairs function.
Numerous techniques have been described to
reconstruct syndactylized digits; these techniques
vary based on the digits involved and the com-
plexity of deformity. There are three basic objec-
tives in syndactyly reconstruction: separation of
the digital skin, reconstruction of the web space,
and soft tissue coverage of the released digits.
In complex syndactyly, a fourth objective,
separation of the nail and osseous connection
with nail wall reconstruction must also be
achieved (Table 2).
285
Table 2 Syndactyly operative management
Indications Contraindications
Syndactyly causing Simple, incomplete
functional impairment syndactyly without functional
impairment
Medical comorbidities
prohibiting surgery
Digits lacking anatomic
structures to enable
independent digital function
Super digit
Surgical Procedure: Simple
and Complex Release (Incomplete
and Complete)
Preoperative Planning
Prior to operative intervention, several factors
are considered, including the timing of
surgery and the order in which the web spaces
will be addressed when multiple digits are
involved.
Although the precise age at which surgery
should be performed remains an open question
that is inuenced by a number of factors,
consensus is emerging. Most surgeons advocate
performing syndactyly reconstruction prior to
2 years of age, as patterns of hand function
develop between 6 and 24 months (Hutchinson
and Frenzen 2010). Additionally, performing
surgery before school age, 5 years in most
cases, may avoid social stigma and lessen
psychological concerns (Vekris et al. 2010). For
most syndactylies, surgery is considered after
6 months of age to minimize anesthetic risk
and allow sufcient growth to facilitate operative
intervention. When syndactyly involves border
digits (i.e., thumb-index or ring-small nger
syndactyly), earlier surgical intervention,
between 3 and 6 months old, is considered as
the differential digital lengths may result in
angular and/or rotational deformity as the shorter
digit tethers the longer one (Fig. 8).
286 M.C. Manske and C.A. Goldfarb

Syndactyly involving multiple digits is simple syndactyly release. Standard operating

typically performed in a staged fashion to
minimize the risk of vascular compromise to
digits supplied by a single artery. When all
digits are involved, reconstruction of the rst
and third web spaces, followed by release of the
second and fourth web spaces 34 months
later, has been recommended (Hutchinson and
Frenzen 2010).
Prior to the procedure, the patients clinical
history, physical exam, and radiographs are
reviewed. The pediatrician and anesthesia team
clears syndromic patients for surgery. The operat-
ing room setup requires a standard operating bed
and hand table. Fluoroscopy is not required for
equipment is utilized (Table 3).
Positioning
Positioning is identical for all syndactyly releases
and reconstructions. The patient is positioned
supine on a standard operating table tted with a
hand table. The patient is close to the edge of the
operating table on the affected side, to allow the
entire involved upper extremity to be positioned
on the hand table. The skin graft donor site is
sterilely prepared and draped.
Surgical Approach

Technique: Simple Complete Syndactyly

Fig. 8 Syndactyly between digits of unequal length leads
to tethering of the longer digit, which results in a exion
contracture and rotational deformity (Courtesy of Shriners
Hospitals for Children, Philadelphia)
The key to a successful syndactyly reconstruction
depends on the recreation of the commissure.
Optimally, the commissure slopes 4550 from
dorsal to palmar and extends one half the length
of the proximal phalanx (Kozin 2001). Numerous
ap designs for the reconstruction of the commis-
sure have been described, including dorsal rectan-
gular aps, palmar rectangular aps, and
interdigitating V-aps (Upton 2006). Many favor
the dorsal rectangular ap, as originally described
by Flatt, as it is thought to best recreate the natural
dorsal to palmar inclination of the web space
(Tonkin 2009).
The commissural reconstruction may be
altered in an attempt to avoid the need for skin
grafts (Fig. 9). In this case, rather than use the
typical dorsal rectangular ap or dorsal/volar
V-aps, additional skin is brought into the eld
to resurface the commissure. The dorsal-
metacarpal island ap described by Sherif

Table 3 Simple syndactyly release preoperative planning

Preoperative planning
OR table: standard operating table with hand table on the patients affected side
Position/positioning aids: the patient is resting supine on the OR table with the affected upper extremity supinated on
the hand table
Fluoroscopy location: not required for simple syndactyly release
Equipment: 15c scalpel blades, bipolar electrocautery, tenotomy scissors, absorbable suture
Tourniquet (sterile/non-sterile): non-sterile or tourniquet applied to affected upper extremity. Tourniquet position
should not preclude access to the antecubital fossa for harvesting of full-thickness skin graft
13 Syndactyly
Fig. 9 Measuring the distance around the digits with a
tape measure and comparing to the distance around indi-
vidual digits exemplies the skin deciency in syndactyly
(Courtesy of Shriners Hospitals for Children, Philadelphia)
(Hutchinson and Frenzen 2010; Sherif 1998)
includes a dorsal ap centered 0.51 cm proximal
to the metacarpophalangeal joint that is mobilized
on its pedicle and advanced into the commissure.
Modications of this ap design include a rotation
of the transposition ap 180 (Aydin and Ozden
2004) or a hexagonal shaped ap (Hsu
et al. 2010). The local advancement ap described
by Ekerot is a rectangular ap with triangle-
shaped wings that form the base and the sides of
the commissure, respectively (Ekerot 1996). The
triangular wings from the dorsal ngers obviate
the need for skin grafting of the proximal ngers
adjacent to the commissure.
No matter the type of commissural ap, an
important goal is to avoid creep of the web space
over time. Tension-free closure is perhaps the
most important technique believed important to
this goal. Additionally, computer modeling sug-
gests that the avoidance of a at base of the
commissural ap helps as well. Any break in the
palmar edge of the web space will decrease ten-
sion (Miyamoto et al. 2010).
Division of the length of the digits is accom-
plished using a zigzag incision to avoid the crea-
tion of a linear scar. The volar and dorsal incisions
are designed to interdigitate. The base of each ap
typically does not extend beyond the midline of
the nger. Depending on the amount of available
287
skin, aps may be raised so that one digit receives
near-complete coverage with local skin while the
other requires skin grafting, or aps can be raised
equally from both digits and thus both digits
require skin grafting (Buck-Gramcko 1985; Ton-
kin 2009). In patients without interphalangeal
joint motion such as Apert syndrome, straight
line incisions to divide the ngers can be
performed as scar contractures are not a concern.
After the aps have been elevated, some sub-
cutaneous fat may be removed from the full length
of the digits and along the neurovascular bundles.
Defatting decreases the volume of the digit and
allows for improved local skin coverage and may
reduce the need for skin grafting (Greuse and
Coessens 2001). Care must be taken to preserve
the subdermal plexus on each ap to assure via-
bility of the aps.
The digits are separated from distal to proximal
and the neurovascular bundles are identied. There
may be a distal bifurcation of the neurovascular
structures. When the nerve bifurcates distally,
intraneural dissection allows easy maintenance of
each digital nerve. When the bifurcation of the
common digital artery into the proper digital arter-
ies occurs distally, the proper digital artery to one of
the digits must be ligated to allow separation of the
digits. A vessel clamp is applied to one of the
proper digital arteries, the tourniquet deated, and
the digit is evaluated. The proper digital artery on
the other side of the digit typically provides suf-
cient blood ow and allows the clamped artery to
be sacriced. It is important to document ligation of
the artery, especially in patients who will undergo
syndactyly release on the other side of the same
digit in the future.
After the digits have been separated, the posi-
tion of the interdigitating aps is evaluated and the
need for additional skin coverage assessed. If
there is insufcient coverage with local skin,
full-thickness skin grafts are harvested and
applied. Full-thickness grafts have been shown
to decrease the incidence of web creep, exion
contracture, and reoperation rate compared to split
thickness skin grafts (Percival and Sykes 1989).
Skin grafts may be taken from the antecubital
fossa, the hypothenar eminence, the volar wrist,
or the inguinal region; we prefer to use the
288 M.C. Manske and C.A. Goldfarb

Fig. 10 Dorsal commissure ap begins at the level of the Fig. 11 Palmar rectangular ap is fashioned to resurface
metacarpal heads and includes two-thirds the length of the the proximal area of a digit adjacent to the commissure
proximal phalanx (Courtesy of Shriners Hospitals for Chil- (Courtesy of Shriners Hospitals for Children, Philadelphia)
dren, Philadelphia)

antecubital fossa because the pigmentation

closely matches that of the recipient site, there is
minimal hair growth, the donor site heals well,
and the proximity of the donor site simplies the
surgery.
Finally, the aps and grafts are sutured into
place with dissolving sutures. First the commis-
sure ap is advanced into the web space, followed
by reapproximation of the interdigitating aps
along the length of the nger. We prefer to use
50 Vicryl Rapide suture (Ethicon Inc, Blue Ash,
Ohio, USA) (Figs. 1018).
Conjoined nails may be present and require
reconstruction. We use a scalpel to incise the
synonychia, and then ag and pennant pulp Fig. 12 From the dorsal side, the volar neurovascular
aps, as described by Buck-Gramcko, are structures are visualized (Courtesy of Shriners Hospitals
designed to recreate the lateral nail folds. These for Children, Philadelphia)
laterally based rectangular and pennant aps
are elevated from the hyponychia, and after Bulic 2013; Golash and Watson 2000).
digit separation, the aps are rotated into Meticulous repair is required to create satisfactory
position along the newly divided edge of the lateral nail folds that become crucial to a
formerly conjoined nail (Buck-Gramcko 1988; satisfactory aesthetic outcome (Tables 4 and 5).
13 Syndactyly
Fig. 13 The length and location of the volar aps can be
checked after elevation of the dorsal aps (Courtesy of
Shriners Hospitals for Children, Philadelphia)
Fig. 14 Lateral spreading of the digits places the inter-
vening tissue under tension and facilitates digital separa-
tion (Courtesy of Shriners Hospitals for Children,
Philadelphia)
Technique: Simple Incomplete Syndactyly
The basic concepts for incomplete syndactyly
reconstruction are the same as for a complete
syndactyly reconstruction. Specically, the com-
missural ap is created to resurface the web space,
289
Fig. 15 The bifurcation between the common and proper
neurovascular structures requires identication (Courtesy
of Shriners Hospitals for Children, Philadelphia)
Fig. 16 The adjacent sides of the separated digits are
defatted prior to insetting of the aps (Courtesy of Shriners
Hospitals for Children, Philadelphia)
and zigzag incisions divide the remainder of the
syndactyly with appropriate interdigitations. The
reconstructive choices include those mentioned
above as well as local aps for smaller incomplete
syndactylies including z-plasty variants. Our pref-
erence for incomplete syndactyly short of the
proximal interphalangeal joint is the three-square-
290
Fig. 17 Dorsal appearance after ap closure and full-
thickness skin grafting (Courtesy of Shriners Hospitals
for Children, Philadelphia)
Fig. 18 Volar appearance after ap closure and full-
thickness skin grafting (Courtesy of Shriners Hospitals
for Children, Philadelphia)
ap method described by Bandoh. It is a straight-
forward technique that creates a reliable, aesthet-
ically pleasing web space while avoiding the need
for skin grafts. It creates a dorsal rectangular ap
M.C. Manske and C.A. Goldfarb
and two additional aps to cover the inner digits.
Specically, three rectangular aps from the
dorsal, volar, and interdigital surfaces of the
interdigital space are used to reconstruct the web
space (Bandoh et al. 1997; Fig. 19a, b).
Technique: Thumb-Index Syndactyly
The management of a thumb-index syndactyly is
different from other digits due to its size, skin
conguration, and importance for function.
Reconstruction of the rst web space is almost
always required as limitations of this web space
are poorly tolerated. The goal is not only to release
the thumb and index ngers but also to both widen
and deepen this web space with a soft, compliant
skin ap. For incomplete, simple syndactyly of
the rst web, options include a two-ap Z-plasty, a
four-ap Z-plasty (Woolf and Broadbent 1972), or
a ve-ap Z-plasty a double Z-plasty combined
with a central Y-V advancement (Hirshowitz
et al. 1975). All techniques allow release and
deepening of the web space without skin grafting.
For complete syndactyly of the rst web space,
four- or ve-ap Z-plasty results in an insufcient
release or recurrent contracture. This has led to the
use of either a dorsal rotational advancement
ap (Buck-Gramcko 1988) or reverse radial fore-
arm aps as described by Gulgonen for recon-
struction of the thumb-index web space in
symbrachydactyly patients (Gulgonen and
Gudemez 2007). Ghanis modication of Buck-
Gramckos dorsal rotational ap broadens the
apex and lengthens the ap (Ghani 2006)
(Fig. 20).
Technique: Complex Syndactyly
Complex syndactylies range from a simple osse-
ous union to the complicated interconnections of
the spade, mitten, or rosebud hand of Apert
acrosyndactyly. Consequently, operative manage-
ment of complex syndactylies varies with the
complexity of the deformity. The treatment of
Apert acrosyndactyly is beyond the scope of this
chapter (see chapter Syndromes Associated
with Syndactyly).
Treatment of complex syndactyly with an osse-
ous union of the distal phalanx proceeds as for
complete syndactyly with the addition of the bony
13 Syndactyly 291

Table 4 Simple syndactyly release surgical steps

Surgical steps
Dorsal commissure incision at of the distance from the MCP joint to the PIP joint
Zigzag incisions both dorsally and volarly along the length of the syndactyly
Hemostasis achieved with electrocautery
Elevation of volar and dorsal skin aps and web space ap
Dissection through subcutaneous tissue from distal to proximal to identify and separate neurovascular bundles to each
digit
Division of the subcutaneous tissue and fascia connecting the digits
Advancement of the dorsal rectangular ap into the web space to the palmar digital crease
Harvest full-thickness skin graft if inadequate local skin coverage (antecubital fossa, hypothenar eminence, wrist crease,
inguinal region)
Interdigitating and web space aps are sutured into place with simple sutures using 50 absorbable suture
Tourniquet is deated and vascular supply to the digits is assessed
Placement of non-constrictive postoperative dressing consisting of nonadherent petroleum gauze over the
incisions, gauze strips in the web spaces between the digits, and elastic bandage around the hand to the level of the
forearm or arm

Table 5 Simple syndactyly release postoperative protocol

Postoperative protocol
Keep operative dressing in place until postoperative follow-up at 3 weeks
Dressing removed at rst postoperative visit; warm water soak may be helpful in removing the dressing
Digits evaluated for infection, ap necrosis, graft failure
Scar and web space massage initiated with the assistance of a hand therapist if available
Splint may be fabricated to support reconstructed web space with odoform pressure mold in some cases
Patients may resume activities as tolerated and should be encouraged to use their hand

Fig. 19 (a and b): Planned box ap reconstruction of web creep after syndactyly reconstruction (Copyright Charles
A. Goldfarb, MD)
292
Fig. 20 (a and b): First web space reconstruction using dorsal ap, Ghani ap (Courtesy of Shriners Hospitals for
Children, Philadelphia)
Fig. 21 Nail fold reconstruction using coronal Buck-
Gramcko pulp aps (Courtesy of Shriners Hospitals for
Children, Philadelphia)
connection separation. The conjoined nails are
divided sharply using a small osteotome as a
knife to divide the phalanges. The lateral nail
folds are resurfaced as described above with a
M.C. Manske and C.A. Goldfarb
ag and pennant technique (Fig. 21). This step is
crucial in minimizing nail abnormalities and n-
gertip deformities. In complex syndactylies, there
is a greater incidence of distal bifurcation of the
neurovascular structures (Eaton and Lister 1990).
Therefore, great care is taken to identify, preserve,
and separate the neurovascular supply to each of
the newly separated digits.
Treatment Specific Outcomes
Outcomes: Simple Syndactyly
Reconstruction
The outcomes of simple syndactyly correction tend
to be good. Most reports mix the outcomes of
complete and incomplete syndactylies. Lumeta
et al. reported good long-term outcomes of cutane-
ous syndactyly reconstruction in 26 web spaces
using palmar and dorsal triangular aps for com-
missure reconstruction, augmented with groin full-
thickness skin grafts as necessary (Lumenta
et al. 2010). There was a low incidence of web
creep (Fig. 22), good scar quality, and no signi-
cant differences in nger motion, power, or
13 Syndactyly
Fig. 22 Web creep after syndactyly separation (Courtesy
of Shriners Hospitals for Children, Philadelphia)
two-point discrimination compared to the contra-
lateral digits. Vekris also reported good to excellent
results of syndactyly reconstruction using skin
grafts in 131 patients (Vekris et al. 2010). There
was a better aesthetic appearance and a lower revi-
sion rate with a dorsal rectangular ap and two
triangular aps compared to two triangular aps
alone (3 % revision rate and 63 % revision rate,
respectively). However, complex syndactyly and
delayed time to treatment were both associated
with less satisfying aesthetic outcomes.
Several authors report good outcomes with
syndactyly reconstruction using a graftless tech-
nique. Sherif reported on the use of a dorsal-
metacarpal island ap for commissural recon-
struction on 12 patients. This limited report
showed good outcomes without recurrence or
web creep. Ekerot, utilizing an extended dorsal
interdigital ap, also showed reliable results with-
out early complications and rare web creep
(Ekerot 1996). Hsu et al. used a dorsal v-y
advancement ap and reported good outcomes
with a mixture of incomplete and complete syn-
dactylies (Hsu et al. 2010). Two of 25 web spaces
required revision.
293
Table 6 Syndactyly potential pitfalls and prevention
Potential
pitfall Pearls for prevention
Web space Tension-free commissure
creep reconstruction. No linear commissural
reconstruction
Contracture Avoidance of linear scars and use skin
grafts as needed
Nail Meticulous reconstruction of lateral
irregularity nail wall with either rotation ap or
full-thickness skin grafts
Vascular Careful handling of neurovascular
compromise bundles with intraoperative assessment
as necessary
Outcomes: Complex Syndactyly
Several authors report worse outcomes with com-
plex syndactyly. Percival and Sykes reviewed
100 patients and 218 web spaces and found the
presence of complex syndactyly, a small sub-
group, to be associated with a higher incidence
of web creep, exion deformity, and need for
additional surgery (Percival and Sykes 1989).
Goldfarb et al. in a study focused on the outcomes
of complex syndactyly reconstruction at long-
term follow-up found that rotational and angular
deformities were common (Goldfarb et al. 2012).
Similarly, Mallet et al. found poorer aesthetic and
functional outcomes including a higher rate of
clinodactyly, exion contractures, and ngernail
differences with worse digital motion compared to
the simple syndactyly cohort (Mallet et al. 2013).
Preferred Treatment
We prefer a Flatt type reconstruction with a dorsal,
rectangular commissural ap and full-thickness
skin grafts for the majority of our syndactyly
reconstructions. We harvest the skin graft from
the antecubital crease as we nd the site provides
a color-matched skin graft with little donor site
morbidity and ease of harvest. We also believe
that a Sherif graftless technique, a dorsal-
metacarpal ap, can provide an excellent aesthetic
outcome for some patients. We combine this tech-
nique using the dorsal-metacarpal ap with loose
closure and defatting on children with some skin
redundancy of the syndactyly. For incomplete
294
Table 7 Syndactyly complication management
Common
complication Management
Contracture Avoidance of linear scars and skin
grafting as necessary. When present,
z-plasties can correct contracture and
restore motion
Web creep Tension-free closure can help avoid
web creep. When it occurs, revision
syndactyly treatment is performed
(such as a Bandoh ap)
Keloid Consider the use of methotrexate in
formation high-risk children such as those with
previous keloid or super digits
Muzaffar et al. (2004), Tolerton and
Tonkin (2011)
syndactyly short of the proximal interphalangeal
joint, we believe the Bandoh box ap provides a
straightforward technique without skin graft and
with an excellent aesthetic outcome (Table 6).
Surgical Pitfalls and Prevention
See Table 7.
Summary
Syndactyly is a common congenital anomaly that
affects hand function appearance and function.
Border digits and multiple syndactylies may be
addressed at a younger age (6 months or younger),
while common long-ring syndactylies may be
treated electively at 18 months or older. Multiple
reconstructive techniques have been described,
and all share the need for restoration of the nger
and web space anatomy with tension-free closure
of aps and grafts. Complex syndactyly outcomes
are more guarded and associated with residual
nger deformities.
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 Syndromes Associated
with Syndactyly 14
Lorenzo Garagnani and Gillian D. Smith

Contents Holt-Oram Syndrome (HOS; HOS1; Heart-Hand

Syndrome; Cardiac-Limb Syndrome; Atriodigital
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 298 Dysplasia) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 310
Syndactyly Classication . . . . . . . . . . . . . . . . . . . . . . . . . . . 300 Jackson-Weiss Syndrome (JWS; Craniosynostosis,
Midfacial Hypoplasia and Foot Abnormalities) . . . . . 311
Syndromes Associated with Syndactyly . . . . . . . . . . 301 Microphthalmia with Limb Anomalies
Amniotic Bands Syndrome (Constriction Rings (Waardenburg Anophthalmia Syndrome;
Syndrome; Congenital Constricting Bands; Anophthalmia-Syndactyly; Ophthalmoacromelic
Amniotic Bands Sequence; ABS; Syndrome; OAS) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 312
Streeter Anomaly) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 302 Moebius Syndrome (MBS; Moebius Sequence) . . . . 312
Apert Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 303 Oculodentodigital Dysplasia (ODDD;
Carpenter Syndrome (Acrocephalopolysyndactyly ODD Syndrome; Oculodentoosseous Dysplasia;
Type II; ACPS II) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 305 ODOD) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 313
Cenani-Lenz Syndactyly Syndrome (Cenani-Lenz Oral-Facial-Digital Syndromes (OFD) . . . . . . . . . . . . . . 313
Syndrome, CLS, CLSS) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 305 Pallister-Hall Syndrome (PHS) . . . . . . . . . . . . . . . . . . . . . . 315
Deletion of 2q37 (Monosomy 2q37) . . . . . . . . . . . . . . . . 306 Pfeiffer Syndrome (Acrocephalosyndactyly Type 5;
Diploid/Triploid Mosaicism (Diploid/Triploid ACS5; Noack Syndrome) . . . . . . . . . . . . . . . . . . . . . . . . . . . . 315
Mixoploidy; 2n/3n Mixoploidy) . . . . . . . . . . . . . . . . . . . . . 306 Poland Syndrome (Poland Sequence; Poland
Ectrodactyly-Ectodermal Dysplasia-Cleft Lip/Palate Syndactyly; Poland Anomaly) . . . . . . . . . . . . . . . . . . . . . . . 316
Syndrome (EEC Syndrome) . . . . . . . . . . . . . . . . . . . . . . . . . 306 Rubinstein-Taybi Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . 317
Filippi Syndrome (Syndactyly Type 1 with Saethre-Chotzen Syndrome (SCS;
Microcephaly and Mental Retardation; Scott Acrocephalosyndactyly Type 3; ACS3; Chotzen
Craniodigital Syndrome with Mental Syndrome; Acrocephaly, Skull Asymmetry, and
Retardation) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 308 Mild Syndactyly) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 318
Fraser Syndrome (Cryptophthalmos-Syndactyly Smith-Lemli-Opitz Syndrome (SLOS; SLO
Syndrome) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 308 Syndrome; RSH Syndrome; 7-dehydrocholesterol
Goltz Syndrome (Focal Dermal Hypoplasia; FDH; Reductase Deciency, Rutledge Lethal Multiple
FODH; DHOF; Goltz-Gorlin Syndrome) . . . . . . . . . . . 308 Congenital Anomaly Syndrome; Polydactyly, Sex
Greig Cephalopolysyndactyly Syndrome (GCPS; Reversal, Renal Hypoplasia, and Unilobar Lung
Polysyndactyly with Peculiar Skull Shape) . . . . . . . . . 310 Lethal Acrodysgenital Syndrome) . . . . . . . . . . . . . . . . . . . 319
Split Hand/Foot Malformation 3 (SHFM3;
Chromosome 10q24 Duplication Syndrome) . . . . . . . 320
Synpolydactyly . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 320
L. Garagnani (*) Timothy Syndrome (TS, Long QT Syndrome
Department of Hand Surgery and Microsurgery, University with Syndactyly, Long QT Syndrome 8, LQT8) . . . . 321
Hospital Policlinico di Modena, Modena, Italy Triploidy Syndrome (Triploid Syndrome;
e-mail: lorenzogara@hotmail.com Triploidy; Chromosome Triploidy Syndrome;
G.D. Smith 3n Syndrome) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 321
Department of Plastic and Reconstructive Surgery,
Great Ormond Street Hospital, London, UK
e-mail: gill.smith@gosh.nhs.uk

# Crown Copyright 2015 297

Published by Springer Science+Business Media New York 2015. All rights reserved.
J.M. Abzug et al. (eds.), The Pediatric Upper Extremity, DOI 10.1007/978-1-4614-8515-5_14
298 L. Garagnani and G.D. Smith

Assessment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 322 Abstract

Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 322 Syndactyly is one of the most common upper
limb congenital anomalies, occurring either as
Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 323
alone or in association with other abnormali-
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 323 ties. Several multiple malformation syn-
dromes where hand and/or foot syndactyly
are present have been described in the scien-
tic literature. Sporadic and inherited forms
have been identied, and among those that are
inherited, new mutations continue to be dis-
covered. The advances in the knowledge of
these anomalies, fetal ultrasound screening,
and new molecular genetic techniques
potentially allow for early diagnosis, although
antenatal diagnosis remains infrequent. New
genetic mutations and malformations associa-
tions continue to present to the clinicians,
making the diagnostic process complex and
challenging. Syndactyly phenotypes may
vary considerably, especially in the syndromic
forms, ranging from simple incomplete to
complex and complicated types. Treatment
of syndromic syndactyly needs to be accu-
rately integrated within a plan tailored on
each single patient, due to the coexistence of
other associated anomalies whose treatment
may take priority over syndactyly.

Introduction

Syndactyly is among the most common congeni-

tal anomalies, with an estimated incidence of
1 in 2,0003,000 live births (Schwabe and
Mundlos 2004) and a higher prevalence
among males (Castilla et al. 1980). Hand or foot
syndactyly can present either as an isolated
malformation or in association with other
malformations of the limbs and may be part of a
multiple congenital anomaly syndrome
(Kumar 2008). In about 1040 % of cases, this
anomaly is familial (Marks et al. 1995). The
spectrum of the anomalies ranges from cases
with simple interdigital skin webbing to cases
with complex bone fusions. The variability of
the types of syndactyly reects the variability of
the causes of these anomalies that may present as
14 Syndromes Associated with Syndactyly 299

Table 1 JSSH modified I. Failure of formation of parts (arrest of development)

classification of the congenital
(A) Transverse deciency
differences of the upper
extremity (2000) (B) Longitudinal deciencies
(C) Phocomelia
(D) Tendon or muscle dysplasia
(E) Absent nail or skin
II. Failure of differentiation of parts
(A) Synostosis
(B) Radial head dislocation
(C) Symphalangism
(D) Contracture
(E) Tumorous conditions
III. Duplication
(A) Thumb polydactyly
(B) Central polydactyly
(C) Polydactyly of the little nger
(D) Opposable triphalangeal thumb
(E) Other types of hyperphalangism
(F) Mirror hand
IV. Abnormal induction of digital rays
(A) Soft tissue
(B) Bone
V. Overgrowth
(A) Macrodactyly
(B) Hemihypertrophy
VI. Undergrowth
(A) Microcheiria (hypoplastic hand)
(B) Brachydactyly
(C) Clinodactyly
VII. Constriction band syndrome
(A) Constriction ring
(B) Lymphedema
(C) Acrosyndactyly
(D) Amputation type
VIII. Generalized skeletal abnormalities and a part of syndrome
IX. Others (including unclassiable cases)

sporadic or inherited as autosomal dominant, forms of symbrachydactyly have been transferred

autosomal recessive, or X-linked. Hand syndac-
tyly has been classied as resulting from the
failure of differentiation between two or more
digits under the International Federation of
Societies for Surgery of the Hand classication
(Knight and Kay 2000; De Smet 2002),
based on Swansons original classication
(Swanson 1976; Swanson et al. 1983). In 2000,
the Congenital Hand Committee of the Japanese
Society for Surgery of the Hand proposed a
modied classication (Table 1) in which all
to the failure of formation group (group I), and a
new group IV of abnormal induction of digital
rays including cutaneous syndactyly (subgroup
A) and osseous syndactyly (subgroup B) has
been introduced (Congenital Hand Committee of
the JSSH 2000; De Smet 2002). The
malformations group (group 1) of the Oberg,
Feenstra, Manske, and Tonkin (OMT) classica-
tion (Table 2) comprises all forms of syndactyly
within its subgroups (Oberg et al. 2010;
Tonkin et al. 2013).
300
Table 2 OMT classification of the congenital anomalies
of the hand and upper extremity (2010)
1. Malformations
A. Failure of axis formation/differentiation entire
upper limb
1. Proximal-distal outgrowth
2. Radioulnar (anteroposterior) axis
3. Dorsoventral axis
B. Failure of axis formation/differentiation hand
plate
1. Radioulnar (anteroposterior axis)
2. Dorsoventral axis
C. Failure of hand plate formation/differentiation
unspecied axis
1. Soft tissue
2. Skeletal deciency
3. Complex
2. Deformations
A. Constriction ring sequence
B. Arthrogryposis
C. Trigger digits
D. Not otherwise specied
3. Dysplasias
A. Hypertrophy
1. Macrodactyly
2. Upper limb
3. Upper limb and macrodactyly
B. Tumorous conditions
described since Temtamy and McKusicks origi-
nal classication:
Syndactyly Classification
Syndactyly is anatomically subdivided into sim-
ple incomplete (Fig. 1a) and complete (Fig. 1b)
forms where only the soft tissues are fused, com-
plex forms with adjacent bone fusion like
acrosyndactyly (Fig. 1c), and complicated forms
where the anatomy is completely subverted
(Fig. 1d). Temtamy and McKusick in 1978 pro-
posed a clinical classication of syndactyly, based
on the affected digits. They subdivided syndac-
tyly into ve groups:
Syndactyly type 1 (SD1): the most common
type is sporadic or inherited (autosomal domi-
nant with incomplete penetrance), affecting the
third web in the hand and/or second web in the
foot; it may be incomplete or complete, and
L. Garagnani and G.D. Smith
most of the times it is bilateral. Inherited
forms have been further subdivided into
four subgroups depending on the different com-
binations of hand and foot involvement, one of
which (Castilla type) is peculiar as it is charac-
terized by fourth web involvement in the foot.
Syndactyly type 2 (SD2): synpolydactyly
(SPD), affecting the third web in the hand and
the fourth web in the foot (Fig. 2a, b), it pre-
sents with partial or complete duplication
within the syndactylous webs. Several sub-
types have been described due to the heteroge-
neity of the spectrum of anomalies.
Syndactyly type 3 (SD3): sporadic or
syndromic (oculodentodigital syndrome),
affecting fourth web in the hand bilaterally.
Syndactyly type 4 (SD4): rare type,
autosomal dominant inherited, presenting
with complete syndactyly of all digits in
the hand.
Syndactyly type 5 (SD5): rare type, autosomal
dominant or X-linked recessive inherited. This
type of syndactyly is characterized by metacar-
pal and metatarsal synostoses and usually
affects the fourth web in the hand and third
web in the foot.
Four additional types of syndactyly have been
Syndactyly type 6 (SD6): very rare,
characterized by unilateral second to fth n-
ger fusion.
Syndactyly type 7 (SD7): this is the very rare
Cenani-Lenz syndrome, autosomal recessive
inherited, with complicated anomalies that
make the hands and feet appear as indistinct
masses of digits (see section Cenani-Lenz
Syndrome) (Fig. 3a, b).
Syndactyly type 8 (SD8): very rare, fourth and
fth metacarpal synostosis.
Syndactyly type 9 (SD9): mesoaxial synostotic
syndactyly (MSSD), described only in two
families. Described features include complex
third web syndactyly with proximal phalanges
size reduction, hypoplastic thumbs and hallu-
ces, second and fth ngers middle phalangeal
14 Syndromes Associated with Syndactyly 301

Fig. 1 (a) Simple

incomplete syndactyly
(Courtesy Marisa Mancini,
Medical Illustrator,
Modena, Italy). (b) Simple
complete syndactyly
(Courtesy Marisa Mancini,
Medical Illustrator,
Modena, Italy). (c)
Complex syndactyly
(acrosyndactyly) (Courtesy
Marisa Mancini, Medical
Illustrator, Modena, Italy).
(d) Complicated syndactyly
(synpolydactyly) (Courtesy
Marisa Mancini, Medical
Illustrator, Modena, Italy)

hypoplasia/aplasia, simple incomplete or com- bilaterally. The spectrum of anomalies is highly

plete syndactyly of the toes.
Syndromes Associated
with Syndactyly
Syndactyly, as part of a syndrome, may present
either as a sporadic or a genetically inherited
anomaly and may involve the webs in different
combinations in the hands and the feet, often
variable, as any subtype of syndactyly may occur,
ranging from simple incomplete forms to complex
and complicated forms, including those associ-
ated with clefting of the hands and feet, polydac-
tyly, and oligodactyly. The associated syndromes
include a large number of different clinical enti-
ties, and the continuous discovery of new syn-
dromes renders this a fascinating subject of
investigation. The most important syndromes
associated with syndactyly are listed, with
302
Fig. 2 (a) Radiographic
appearance of a hand in a
case of synpolydactyly. (b)
Radiographic appearance of
feet in a case of
synpolydactyly
considerations that may specically affect man-
agement and consideration for surgery and anes-
thesia highlighted:
Amniotic Bands Syndrome
(Constriction Rings Syndrome;
Congenital Constricting Bands;
Amniotic Bands Sequence; ABS;
Streeter Anomaly)
[MIM 217100]
Etiology/inheritance: sporadic, with no evidence
of a clear genetic origin. There are extrinsic theo-
ries of formation, where rupture of the amnion
with subsequent constriction of body parts
displaced through is causative, and intrinsic theo-
ries, related to loss of signaling, possibly due to a
vascular problem creating malformations.
L. Garagnani and G.D. Smith
Estimated incidence: 1 in 1,2001 in 15,000
live births.
Findings: ringlike constrictions that may be
located anywhere in the body but more frequently
in the limbs (Fig. 4), congenital amputation,
syndactyly, polydactyly, encephalocele, facial/
orbital clefts, eyelid coloboma, cleft
lip/palate, ectopic heart, abnormal lung lobation,
thoracoschisis, abdominoschisis, gastroschisis,
omphalocele, bladder exstrophy, and scoliosis.
Diagnosis: based on the typical clinical ndings at
birth. When unilateral, this is often confused with
symbrachydactyly, but the presence of multiple
rings and the absence of distal nail elements in
amputations should clarify this. Prenatal diagnosis
is possible in some cases by ultrasound examination
(the earliest documented detection at approximately
12 weeks gestation).
14 Syndromes Associated with Syndactyly
Fig. 3 (a) Hand
appearance in a case of
Cenani-Lenz syndrome. (b)
Radiographic appearance of
the hand
Fig. 4 Acrosyndactyly and amniotic bands
303
Apert Syndrome
[MIM 101200]
Etiology: sporadic or autosomal dominant
(FGFR2 gene mutation, chromosomal location
10q26.13). Apert syndrome appears to occur
more often in children of older fathers.
Estimated incidence: 1 in 160,0001 in 200,000
live births.
Features: craniosynostosis, corpus callosum
agenesis, midfacial hypoplasia (Fig. 5a), pulmo-
nary agenesis, cardiac defects, genitourinary
anomalies, esophageal atresia, tracheoesophageal
stula, cervical vertebrae fusion, developmental
delay, complex syndactyly (hands and feet), and
symphalangism of the interphalangeal joints of all
304 L. Garagnani and G.D. Smith

Fig. 5 (a) Facial appearance in Apert syndrome. (b) Type (e) Radiographic appearance of type 2 hands. (f) Type
1 hand in Apert syndrome. (c) Radiographic appearance 3 hand in Apert syndrome. (g) Radiographic appearance
of type 1 hand. (d) Type 2 hand in Apert syndrome. of type 3 hand
14 Syndromes Associated with Syndactyly
the ngers except the little nger distal
interphalangeal (DIP) joint.
Their craniofacial anomalies make them prone
to airway problems including obstructive sleep
apnea, and raised intracranial pressure. The treat-
ment of these entities takes priority over the hands.
Aperts hand syndactyly is subdivided into
three types, according to Uptons classication
(1991):
Type 1 (spade hand): the thumb is free but
radially deviated due to a delta proximal pha-
lanx, and the palm is at with complex
acrosyndactyly (osseous or cartilaginous) of
third web, simple syndactyly of fourth web,
and syndactyly of the second web which may
be simple or complex (Fig. 5b, c).
Type 2 (mitten hand): simple syndactyly of
rst web with short radially deviated thumb,
cupped palm with complex acrosyndactyly of
second and third web, and simple complete
syndactyly of fourth web (Fig. 5d, e).
Type 3 (rosebud hand or hoof hand): com- syndactyly is less severe.
plete syndactyly of all the digits, with complex
(and often complicated) syndactyly of rst,
second, and third web, and simple syndactyly
of fourth web. There is a common nail, prone to
paronychial infections, and it may be difcult
to distinguish the individual digits within the
syndactyly mass, either clinically or radiolog-
ically (Fig. 5f, g).
Capitohamate carpal coalition is frequent as is
the presence of fourth and fth metacarpal synos-
tosis, which becomes more evident radiologically
with increasing age. Index nger deviation due to
an abnormal epiphysis in the proximal phalanx
may occur, particularly in type 3 hands.
The foot deformities are similar to those affect-
ing the hands, with multiple tarsal coalitions,
radiologically more evident with age. There is
multidigit syndactyly with symphalangism, meta-
tarsal synostosis, and occasional polydactyly.
Diagnosis: based on the typical clinical ndings at
birth of the facial appearance combined with
multidigit syndactyly in all limbs. Genetic testing
can conrm the diagnosis.
305
Carpenter Syndrome
(Acrocephalopolysyndactyly
Type II; ACPS II)
[MIM 201000]
Etiology/inheritance: autosomal recessive
(RAB23 gene mutation, chromosomal location
6p11.2).
Features: syndactyly (hands and feet),
camptodactyly, brachydactyly (hands and feet),
polydactyly (hands and feet), obesity (from child-
hood), craniosynostosis, hydrocephalus, brachy-
cephaly, mandibular/maxillary hypoplasia and
other distinctive facial features (low-set ears,
abnormal eye shape, at nasal bridge), cardiac
defects (including dextrocardia in some cases),
corneal opacity, genital anomalies in affected
males (most frequently cryptorchidism), situs
viscerum inversus in some cases, and mild to
severe intellectual disability in some cases.
Many of the same considerations apply to these
children as to the Apert patients, but their hand
Diagnosis: based on clinical and radiological
features and genetic testing.
Cenani-Lenz Syndactyly Syndrome
(Cenani-Lenz Syndrome, CLS, CLSS)
[MIM 212780]
Etiology/inheritance: autosomal recessive
(LRP4 gene mutation, chromosomal location
11p12-p11.2).
Estimated incidence: unknown. Extremely rare
syndrome, with only around 30 cases described
in the literature.
Features: bilateral complex syndactyly of the
hands and feet with characteristic symmetrical
appearance. There is multidigit complex syndac-
tyly with carpal, metacarpal, and phalangeal syn-
ostoses; toe syndactyly with tarsal, metatarsal, and
phalangeal synostoses; and frequent oligodactyly.
Symphalangism may be present. The forearms are
also affected, with radial and ulnar hypoplasia,
radial head dislocation, radioulnar synostosis,
and brachymesomelia. Facial dysmorphism
306
(ptosis, prominent forehead, at nasal bridge,
hypertelorism, downslanting palpebral ssures)
has been described in some cases. Renal anoma-
lies (agenesis and/or hypoplasia) have been dem-
onstrated to affect over 50 % of CLS families
(Li et al. 2010).
These patients, who have little in terms of
muscle and tendon attachments distally, combined
with their complex synostoses, may get minimal
gain from surgery, and the surgeon should be
cautious in his or her prognosis for functional
improvement after surgery.
Diagnosis: clinical and radiological features and
genetic testing.
Deletion of 2q37 (Monosomy 2q37)
Etiology/inheritance: sporadic (chromosomal dis-
order, characterized by deletion of chromosome
band 2q37).
Estimated incidence: 1 in 10,000 live births.
Features: microcephaly or macrocephaly, typical
facial dysmorphism (round cheeks/round face,
upslanting palpebral ssures, prominent fore-
head, midfacial hypoplasia, enophthalmos,
sparse scalp hair, sparse and arched eyebrows,
depressed nasal bridge, decient nasal alae,
V-shaped nose tip, prominent columella, thin
vermillion border of the lips, high-arched palate),
fth nger clinodactyly, small feet and/or hands,
hands/feet syndactyly, persistent fetal nger
pads, single palmar crease, wide set or distally
placed nipples, and supernumerary nipples.
Proximal implantation of the thumbs and short
toenails has been described in a case. Eczema and
hypotonia are frequent ndings. Congenital
heart malformations and gastrointestinal, genito-
urinary, and central nervous system
malformations have been reported. Seizures
have also been described, and behavioral anom-
alies (a distinct subtype of autism) associated
with this disorder have been reported. Albright
hereditary osteodystrophy-like phenotype
(AHO3) is part of this spectrum, and its features
are including developmental delay or intellectual
decit, short stature, tendency towards obesity
with age, and brachymetaphalangism.
L. Garagnani and G.D. Smith
Diagnosis: based on clinical and radiological
features and genetic testing.
Diploid/Triploid Mosaicism (Diploid/
Triploid Mixoploidy; 2n/3n Mixoploidy)
Etiology/inheritance: chromosomal disorder
(46XX/69XXY), with a mosaic morula (2n/3n)
due to partial recovery of a triploid conception
(see section Triploidy Syndrome). The fetus
may not survive.
Features: hands/feet syndactyly, body and/or facial
asymmetry, hypotonia, truncal obesity, growth
delay, hyperpigmentation or hypopigmentation of
the skin, malformed low-set ears, small phallus,
micrognathia, and variable intellectual disability
(van de Laar et al. 2002; Rittinger et al. 2008).
Diagnosis: clinical features may lead to suspicion.
Only genetic testing can conrm the diagnosis
(amniocentesis, karyotype of a newborn baby or
miscarried/stillborn fetus).
Ectrodactyly-Ectodermal Dysplasia-
Cleft Lip/Palate Syndrome (EEC
Syndrome)
Due to genetic variability different forms of this
disorder are known:
Ectrodactyly-Ectodermal Dysplasia-cleft
lip/palate syndrome 1 (EEC1)
[MIM 129900]
Ectrodactyly-Ectodermal Dysplasia-Cleft
Lip/Palate syndrome 3 (EEC3)
[MIM 604292]
Etiology/inheritance:
EEC1: autosomal dominant with incomplete
penetrance (linked to chromosomal location
7q11.2-q21.3)
EEC3: autosomal dominant with incomplete
penetrance (TP63 gene mutation, chromo-
somal location 3q28)
14 Syndromes Associated with Syndactyly 307

Fig. 6 (a) Clinical appearance of the right hand ectrodactyly in a case of EEC syndrome. (b) Radiographic appearance of
the right hand. (c) Clinical appearance of the left hand ectrodactyly. (d) Radiographic appearance of the left hand

Features: EEC is characterized by high variabil-
ity, and no sign appears to be obligatory for the
diagnosis (Fryns et al. 1990). Described features
are syndactyly of the hands/feet, ectrodactyly of
the hands/feet (Fig. 6ad), maxillary and mild
malar hypoplasia, malformed/small ears, hear-
ing loss, at nasal tip, cleft lip, cleft palate,
absence of Stensen duct, xerostomia,
microdontia, teeth anomalies (hypodontia,
partial anodontia, enamel hypoplasia), dental
caries, lacrimal duct abnormalities, blepharo-
phimosis, blue irides, blepharitis, dacryocystitis,
photophobia, sparse eyebrows, sparse eyelashes,
sparse and thin scalp hair, sparse pubic and
axillary hair, slow hair growth, light colored
hair, fair and thin skin, periorbital hyperpig-
mentation, dystrophic/pitted/thin nails, hypo-
plastic nipples, micropenis (male),
cryptorchidism (male), transverse vaginal sep-
tum (female), renal agenesis/dysplasia,
hydronephrosis, duplicated collecting system,
megaureter, ureterocele, vesicoureteral reux,
bladder diverticula, holoprosencephaly, mental
retardation in some cases, growth hormone de-
ciency, hypogonadotropic hypogonadism, and
central diabetes insipidus.
Diagnosis: based on clinical and radiological
features (high variability) and genetic testing.
308
Filippi Syndrome (Syndactyly Type 1
with Microcephaly and Mental
Retardation; Scott Craniodigital
Syndrome with Mental
Retardation)
[MIM 272440]
Etiology/inheritance: autosomal recessive
(unknown genetic cause).
Features: hands/feet syndactyly, polydactyly,
microcephaly, growth delay, low weight,
distinctive facial features (micrognathia, short
philtrum, broad forehead, high frontal hairline,
hairy forehead, prominent columella, broad nasal
bridge, hypoplastic nasal alae), and variable
mental retardation/intellectual disability. Other
reported features included phalangeal and meta-
carpal hypoplasia, cryptorchidism (male), teeth
and hair abnormalities, hypertonia of the limbs,
central hypotonia, carpal synostosis, radial head
dysplasia and elbow dislocation, anteverted ears
with hyperconvoluted helix, thin lips, generalized
hirsutism, ventricular septal defect of the heart,
Chiari type I brain malformation, cerebellar atro-
phy, diffuse enlargement of subarachnoid spaces
and lateral ventricles, megacisterna magna, and
arachnoidal cyst. Dystonic movements, dystonic
tongue protrusion, and seizures have also been
reported.
Diagnosis: based on typical clinical and radiolog-
ical features.
Fraser Syndrome (Cryptophthalmos-
Syndactyly Syndrome)
[MIM 219000]
Etiology/inheritance: autosomal recessive (homo-
zygous or compound heterozygous mutation in
FRAS1 located on chromosome 4q21, FREM2
on chromosome 13q13, or GRIP1 on chromo-
some 12q14).
Features: Fraser syndrome is a heterogeneous
disorder, with several different features reported
in different individuals. Reported features have
been cryptophthalmos (this nding is not essen-
tial, as affected individuals may not have it)
(Koenig and Spranger 1986; Thomas
L. Garagnani and G.D. Smith
et al. 1986), hypertelorism (Fig. 7a), blindness,
absent or malformed lacrimal ducts, middle end
external ear malformations, hearing loss or con-
genital deafness, hypoplastic notched nares, broad
and low nasal bridge, midline nasal cleavage,
microcephaly, unusual hairline (hair growth on
temples), high palate, cleft lip/palate, laryngeal
stenosis or atresia, renal hypoplasia or agenesis,
umbilical abnormalities, small penis (male),
hypospadias (male), cryptorchidism (male),
bicornuate uterus (female), vaginal atresia
(female), clitoral enlargement (female), imperfo-
rate anus or anal stenosis, pubic symphysis
diastasis, meningomyelocele, encephalocele, and
mental retardation in some cases. Rate of prenatal
death is 25 %, while 20 % of affected individuals
die before age one.
Diagnosis: diagnostic criteria were established
by van Haelst and Scambler in 2007:
Major criteria: syndactyly (Fig. 7b, c, d),
cryptophthalmos, urinary tract abnormalities,
ambiguous genitalia, laryngeal and tracheal
anomalies, and positive family history
Minor criteria: anorectal defects, dysplastic
ears, skull ossication defects, umbilical
abnormalities, and nasal anomalies
All the other malformations and anomalies
were considered uncommon. Diagnosis can be
made if either 3 major, or 2 major and 2 minor,
or 1 major and 3 minor criteria are present in the
same individual.
Goltz Syndrome (Focal Dermal
Hypoplasia; FDH; FODH; DHOF; Goltz-
Gorlin Syndrome)
[MIM 305600]
Etiology/inheritance: sporadic (95 % of cases), or
X-linked dominant (PORCN gene mutation, chro-
mosomal location Xp11.23) with in utero lethality
in males. Affected males are all results of new
mutation.
Features: poikiloderma with focal dermal hypo-
plasia, atrophy and linear pigmentation of the skin
with fatty tissue herniation through the dermal
14 Syndromes Associated with Syndactyly 309

Fig. 7 (a) Facial

appearance in Fraser
syndrome. (b) Clinical
appearance of the left hand
(dorsal view) (c) Clinical
appearance of the left hand
(palmar view). (d)
Radiographic appearance of
the left hand

defects, multiple papillomata of the mucous mem-
branes (external and internal) and skin, hands/feet
syndactyly (Fig. 8), polydactyly, oligodactyly,
brachydactyly, hypoplastic phalanges/metacar-
pals/metatarsals, and split hand. The lobster-
claw hand (i.e., combination of split hand, ray
absence, and syndactyly) is a typical feature of
FDH, and similar deformities have been reported
also affecting the foot. Longitudinal striation of
the long bones (osteopathia striata) has been
reported as a common nding. Mental retardation
is frequent.
Other reported anomalies include short stature,
mild microcephaly, facial and/or skull asymmetry,
pointed chin, narrow nasal bridge, notched nasal
alae, broad nasal tip, low-set ears, narrow auditory
canals, hearing loss, eye colobomas (iris and cho-
roid), aniridia, ectopia lentis, optic atrophy,
microphthalmia, anophthalmia, strabismus, nystag-
mus, reduced visual acuity, apocrine gland anoma-
lies, telangiectasia, hydrocystomas, cleft lip/palate,
hypodontia, partial anodontia, enamel hypoplasia,
malocclusion, delayed teeth eruption, notched inci-
sors, sparse and brittle hair, head and pubic
310
Fig. 8 Radiographic appearance of a hand in Goltz syndrome
alopecia, dystrophic or absent nails, midclavicular
hypoplasia/aplasia, rib hypoplasia, scoliosis, spina
bida occulta, congenital hip dislocation, joint lax-
ity, skeletal asymmetry, pubic bone diastasis (failure
of fusion), supernumerary or hypoplastic nipples,
breast asymmetry, intestinal malrotation, anteriorly
displaced anus, rectal diastasis, hiatus hernia, dia-
phragmatic hernia, umbilical hernia, omphalocele,
inguinal hernia, horseshoe kidney, hydronephrosis,
bid ureter, hypoplasia of the external genitalia
in the female, cryptorchidism (male),
myelomeningocele, hydrocephalus, agenesis of
corpus callosum, Arnold-Chiari malformation, and
mental retardation in 15 % of cases. Giant cell
tumors of bone may also occur.
Diagnosis: based on typical clinical and radiolog-
ical features and genetic testing.
Greig Cephalopolysyndactyly
Syndrome (GCPS; Polysyndactyly
with Peculiar Skull Shape)
[MIM 175700]
Etiology/inheritance: autosomal dominant (GLI3
gene mutation, chromosomal location 7p13).
L. Garagnani and G.D. Smith
Features: frontal bossing, scaphocephaly and
hypertelorism, pre-/postaxial polydactyly, and
syndactyly. The expressivity of the phenotype is
variable and may include trigonocephaly, high
forehead, broad nasal root, broad thumbs and
halluces (Fig. 9a, b, c, d), camptodactyly, umbil-
ical hernia, inguinal hernia (male), agenesis of the
corpus callosum, seizures, hydrocephaly, and
rarely mild mental retardation (psychomotor
development is usually normal in individuals
affected by GCPS).
These individuals may be unaware that
they have a syndrome as this gene has
high penetrance and its features may be
mild so they are considered as a normal family trait.
Diagnosis: based on family history, typical
clinical and radiological features, and genetic
testing.
Holt-Oram Syndrome (HOS; HOS1;
Heart-Hand Syndrome; Cardiac-Limb
Syndrome; Atriodigital Dysplasia)
[MIM 142900]
Etiology/inheritance: autosomal dominant
(TBX5 gene mutation, chromosomal location
12q24.21).
Features: the characteristic phenotypical features
of HOS are thumb anomalies (typically hypopla-
sia) and ostium secundum atrial septal defect. The
thumb may present as a triphalangeal and
non-opposable digit and be bid or absent. Other
reported anomalies are syndactyly, radial and
ulnar anomalies, carpal bones anomalies,
phocomelia of the upper extremity, pectoralis
major muscle aplasia, pectus excavatum/
carinatum, thoracic scoliosis, vertebral anomalies,
ventricular septal defect, hypoplastic left heart
syndrome, and patent ductus arteriosus. The car-
diac and hand defects are variable in severity so a
parent may not be aware that they themselves are
affected if their own abnormality is mild.
Diagnosis: based on family history, typical clin-
ical and radiological features, and genetic
testing.
14 Syndromes Associated with Syndactyly 311

Fig. 9 (a) Clinical appearance of a hand in Greig syndrome. (b) Radiographic appearance of a hand. (c) Clinical
appearance of the feet in Greig syndrome. (d) Radiographic appearance of a foot

Jackson-Weiss Syndrome (JWS; Features: craniosynostosis, midfacial hypoplasia,

Craniosynostosis, Midfacial Hypoplasia broad and medially deviated great toes,
and Foot Abnormalities) short/broad metatarsals, tarsal coalition
(tarsonavicular, calcaneonavicular), and
[MIM 123150] webbing/simple syndactyly of second web in
Etiology/inheritance: autosomal dominant the foot.
(FGFR2 gene mutation, chromosomal location Diagnosis: based on family history,
10q26.13. An FGFR1 gene mutation, chromo- phenotype assessment that should include radio-
somal location 8p11.23-p11.22, has been found graphic evaluation of the feet, and molecular
in one patient with JWS phenotype). genetic tests.
312
Microphthalmia with Limb Anomalies
(Waardenburg Anophthalmia
Syndrome; Anophthalmia-Syndactyly;
Ophthalmoacromelic Syndrome; OAS)
[MIM 206920]
Etiology/inheritance: autosomal recessive
(SMOC1 gene mutation, chromosomal location
14q24.2).
Features: anophthalmia (unilateral/bilateral) in
87 % of cases, microphthalmia, small orbits,
downslanting/short palpebral ssures, prominent
forehead, prominent philtrum, retrognathia,
low-set/posteriorly rotated ears, at nasal bridge,
short nose, ared nostrils, cleft lip/palate, high-
arched palate, fourth and fth metacarpal synos-
tosis, camptodactyly, single transverse palmar
crease, hand/foot syndactyly, clubfoot, foot
oligodactyly, wide rst web in the foot, postaxial
polydactyly in the foot, hip dislocation, bowed
tibia, short femur/tibia, and hypoplastic bula.
Mental retardation has been reported in approxi-
mately 47 % of cases. Interruption of inferior vena
cava with azygos continuation has also been
reported in one individual (Tekin et al. 2000).
Diagnosis: based on typical clinical and radio-
logical features and genetic testing.
Moebius Syndrome (MBS; Moebius
Sequence)
[MIM 157900]
Etiology/inheritance: sporadic (most cases), or
autosomal dominant (unknown gene, possible
chromosomal location on 13q12.2-q13). Cases of
association with Poland syndrome/sequence have
been described (Moebius-Poland syndrome). A
sequence differs from syndrome in that the associ-
ated features are all related to a single primary
event causing secondary defects, rather than a col-
lection of associated features whose causation is
not sequential on a single primary event.
Features: characteristic features are congenital
palsies affecting the abducens (VI) and facial
(VII) cranial nerves. Other cranial nerves may
also be affected, with variable pareses and
related dysfunctions. Variable associated features
L. Garagnani and G.D. Smith
include limb deformities/hypoplasia and orofacial
dysmorphism.
The Moebius Syndrome Foundation Research
Conference in 2007 dened Moebius syndrome as
a congenital, nonprogressive facial weakness with
limited abduction of one or both eyes; its additional
features may include hearing loss and other cranial
nerve dysfunction, as well as motor, orofacial,
musculoskeletal, neurodevelopmental, and social
problems. The degree and type of cranial nerves
involvement is variable, ranging from relatively
benign anomalies like facial paresis/mask-like
facies, ocular palsies, unilateral tongue paresis,
weak bite, and absence of jaw rotation during
chewing, to respiratory and feeding problems in
infancy and dysphagia. The other reported associ-
ated phenotypic features are micrognathia (64 % of
affected individuals), external ear defects (47 %),
hypertelorism (25 %), congenital extraocular mus-
cles brosis (9 %), epicanthal folds (89 %),
microphthalmia, lacrimal duct defects, strabismus
(Duane retraction syndrome, esotropia, exotropia),
attened nasal bridge (81 %), hypoplasia of the
tongue (77 %), palatal weakness, high-arched pal-
ate (61 %), bid uvula (11 %), teeth defects, short
neck, pharyngeal weakness, hypogonadotropic
hypogonadism, small penis (male), hypodeveloped
scrotum (male), hypoplasia of the testes (male),
arthrogryposis (6 %), lower limb deformities
(61 %), lower limb hypoplasia, pes planus, talipes
equinovarus, limbs anisomelia, and hand deformi-
ties (61 %) including syndactyly, brachydactyly,
clinodactyly, camptodactyly, ectrodactyly, phalan-
geal hypoplasia, metacarpal hypoplasia/aplasia,
low-set thumbs, and clenched hands. Central ner-
vous system problems may include hypotonia in
infancy, delayed motor development, language
delay, mild mental retardation, clumsiness
(82 %), poor coordination (83 %), abnormal gait
(67 %), axial imbalance (34 %), and dysdiado-
chokinesis (63 %). Magnetic resonance imaging
(MRI) of the central nervous system may show
the absence of the facial nerve, brainstem hypopla-
sia, and congenital abnormalities of the posterior
fossa.
Diagnosis: based on typical clinical and radiolog-
ical features.
14 Syndromes Associated with Syndactyly
Oculodentodigital Dysplasia (ODDD;
ODD Syndrome; Oculodentoosseous
Dysplasia; ODOD)
[MIM 164200]
Etiology/inheritance: autosomal dominant
(majority of cases) or autosomal recessive
(GJA1 gene mutation, chromosomal location
6q22.31).
Features: typical facial appearance with variable
ocular, dental, and digital anomalies.
Syndactyly type 3 of fourth and fth ngers is
the most common digital malformation, the third
nger may also be affected, and associated
camptodactyly is common. Midphalangeal hypo-
plasia has also been reported. Other reported asso-
ciated anomalies are microcephaly; hearing loss
(conductive); microcornea; microphthalmia; short
palpebral ssures; epicanthal folds; glaucoma;
cataract; iris anomalies; small or thin anteverted
nares; hypoplastic and thin nasal alae; narrow
nasal bridge; cleft lip/palate; broad alveolar
ridges; taurodontism (only 1 case reported);
hypodontia (microdontia); partial anodontia;
enamel hypoplasia; caries; premature dental loss;
skull and vertebral hyperostosis; hip dislocation;
cubitus valgus; broad tubular bones of the limbs;
toes syndactyly (third and fourth toes);
palmoplantar keratoderma; ne, dry, and sparse
slow-growing hair; hyperactive deep tendon
reexes; paraparesis; tetraparesis; ataxia; spas-
ticity; dysarthria; basal ganglia calcication; and
cerebral white matter abnormalities.
Diagnosis: based on family history, clinical and
radiological features, and genetic testing.
Oral-Facial-Digital Syndromes (OFD)
Due to high genetic and clinical variability,
although 13 forms of OFD syndromes have been
documented, only those with syndactyly are
listed:
OFD1 (oral-facial-digital syndrome type 1;
OFDS1; Papillon-Leage and Psaume
syndrome)
313
[MIM 311200]
Etiology/inheritance: X-linked dominant (OFD1
gene mutation, chromosomal location Xp22) with
in utero lethality in males.
Features: typical facial, oral, and digital
malformations. Facial anomalies include frontal
bossing, asymmetry, microretrognathia, and
malar bones hypoplasia. Oral malformations
include tongue hamartoma (70 %), lobulated
tongue (3045 %), bid tongue (3045 %),
tongue nodule, median cleft lip (45 %),
pseudocleft of upper lip, cleft palate, high-arched
palate, hyperplastic oral frenuli, buccal frenuli,
thickened alveolar ridges, and irregular lip mar-
gins. Digital abnormalities (45 %) include syndac-
tyly, clinodactyly, brachydactyly, pre-/postaxial
polydactyly (rare), irregular pattern of radiolu-
cency and/or spicule-like formation in metacar-
pals and phalanges.
Central nervous system involvement is present
in 40 % of cases, with anomalies that include
abnormal gyrations, corpus callosum absence,
heterotopias of the gray matter, hydrocephalus,
arachnoid cysts, cerebellar abnormalities,
hypothalamic hamartoma, porencephaly,
myelomeningocele (rare), stenosis of the aque-
duct of Sylvius (rare), seizures and variable men-
tal retardation (40 %). Polycystic kidney disease
is present in 50 % of cases (adult onset), and
brocystic liver in 45 % of cases. Toes abnormal-
ities (25 %) include hallux duplication and pre-/
postaxial polydactyly. Other reported anomalies
are intrahepatic bile ducts dilatation and beading,
hepatic brosis, cardiac anomalies, microceph-
aly, short stature, low-set ears, hearing loss,
epicanthus, hypertelorism, telecanthus,
downslanting palpebral ssures, broad nasal
bridge, hypoplastic cartilage of nasal alae, anom-
alous anterior teeth (like absent lateral incisors),
enamel hypoplasia, supernumerary or missing
teeth, caries, ovarian cysts, dry scalp, upper face
and ears milia (infancy), dry and sparse hair,
alopecia.
OFD2 (oral-facial-digital syndrome type 2;
OFDS2; Orofaciodigital syndrome II; Mohr
syndrome)
314
[MIM 252100]
Etiology/inheritance: autosomal recessive.
Features: short stature, hypertelorism,
telecanthus, hypoplasia of the zygomatic arch,
maxillar hypoplasia, wormian bones (cranium),
hearing loss (conductive), low nasal bridge,
broad or bid nasal tip, hypertrophic frenula in
mouth, bid tongue, tongue nodules/lobulated
tongue, high-arched palate, cleft palate, cleft lip
(midline), teeth anomalies (absent lateral inci-
sors), pectus excavatum, scoliosis, bone
metaphyses aring or irregularity, preaxial poly-
dactyly of hands/feet, postaxial polydactyly of
hands (bilateral), syndactyly, brachydactyly, fth
nger clinodactyly, partial hallux duplication,
broad cuboid rst metatarsal, extra cuneiform
bone in foot, hydrocephaly, and porencephaly.
Majority of individuals have normal intelligence.
OFD4 (Orofaciodigital syndrome IV; OFDS4;
oral-facial-digital syndrome type 4; OFD syn-
drome with tibial defects; Mohr-Majewski
syndrome; Baraitser-Burn syndrome; OFD
syndrome, Baraitser-Burn type)
[MIM 258860]
Etiology/inheritance: autosomal recessive
(TCTN3 gene mutation, chromosomal location
10q24.1).
Features: short stature, micrognathia,
hypertelorism, epicanthal folds, low-set ears,
cleft palate, high-arched palate, tongue nodules/
lobulated tongue, pectus excavatum, short tibiae,
preaxial and/or postaxial polydactyly of hands
and feet, hands/feet syndactyly, hand
brachydactyly/clinodactyly, cerebral atrophy, and
porencephaly. Other reported anomalies in single
cases include anal atresia, laryngeal dysplasia,
talipes equinovarus, deafness, ocular colobomas,
liver anomalies, hepatic brosis, renal anomalies,
brain malformations, cystic kidney disease,
ambiguous genitalia, and elbows and knees
dislocation.
OFD6 (oral-facial-digital syndrome type 6;
OFDS6; Varadi-Papp syndrome; Varadi syn-
drome; polydactyly, cleft lip/palate or lingual
lump, and psychomotor retardation)
L. Garagnani and G.D. Smith
Fig. 10 Clinical appearance of a hand in OFD syndrome
[MIM 277170]
Etiology/inheritance: autosomal recessive.
Features: short stature, failure to thrive,
micrognathia, low-set ears, posteriorly rotated
ears, hearing loss (conductive), hypertelorism,
esotropia, epicanthal folds, nystagmus, broad
nasal tip, cleft lip/palate, high-arched palate, oral
frenula, lobed tongue, lingual/sublingual nodules,
renal dysplasia or agenesis, preaxial/central/post-
axial polydactyly of hands (Fig. 10), preaxial
polydactyly of feet, hands/feet syndactyly, hand
clinodactyly/brachydactyly, central Y-shaped
metacarpal, central nervous system anomalies
that include hypoplastic cerebellar vermis with
clinical signs of cerebellar defect, hypotonia,
developmental delay and mental retardation.
Other reported variable anomalies include con-
genital heart anomalies, cryptorchidism, genital
anomalies, and occasional hypothalamic
hamartoma.
OFD9 (oral-facial-digital syndrome type 9;
oral-facial-digital syndrome with retinal abnor-
malities; orofaciodigital syndrome with retinal
abnormalities), originally reported as OFD8
[MIM 258865]
Etiology/inheritance: autosomal recessive (origi-
nal hypothesis X-linked recessive).
Features: short stature, hypertelorism, synophrys,
telecanthus, strabismus, retinal colobomas (cardi-
nal feature, dened by Gurrieri et al. in 1992 as
retinochoroidal lacunae of colobomatous origin
similar to those found in Aicardi syndrome),
14 Syndromes Associated with Syndactyly
retrobulbar cysts, low-set ears, broad or bid nasal
tip, median cleft lip, cleft palate, high-arched palate,
tongue lobulation, hamartoma of the tongue, oral
frenula, dental anomalies, hypoplasia of the epiglot-
tis, milia, short tibiae, hand polydactyly, hands/feet
syndactyly, bid halluces, forked metatarsal, and
developmental delay. Hypothalamic hamartoma
and microcephaly have also been reported.
OFD13 (Degner syndrome)
Etiology/inheritance: autosomal recessive.
Features: distinguishing features are brachyclino-
syndactyly and leukoaraiosis (seen on brain MRI
scan), associated to psychiatric symptoms and
epilepsy.
Diagnosis: each form of OFD presents with typi-
cal clinical and radiological features. Genetic test-
ing should take into account the high genetic
variability of these syndromes.
Pallister-Hall Syndrome (PHS)
[MIM 146510]
Etiology/inheritance: autosomal dominant (GLI3
gene, chromosomal location 7p14.1). This very
rare ciliopathy may be lethal neonatally.
Features: a characteristic of this syndrome is a
hypothalamic hamartoma that may vary from
asymptomatic to causing life-threatening seizures
and hormonal disturbances. Other reported
anomalies of the central nervous system are
holoprosencephaly, pituitary aplasia/dysplasia.
Other possible features include intrauterine
growth retardation, short nose, at nasal bridge,
anteverted nares, microtia, absent external audi-
tory canals, posteriorly rotated ears, natal teeth,
multiple buccal frenula, microglossia, cleft
lip/palate, laryngeal cleft, bid or hypoplastic epi-
glottis, abnormal lung lobation, ventricular septal
defect of the heart, proximal aortic coarctation,
patent ductus arteriosus, imperforate anus, hypo-
plastic testicles (male), micropenis (male), kidney
anomalies (renal dysplasia, ectopia),
hemivertebrae, ribs fusion, hip dislocation, distal
limbs shortening, subluxation of the radius, adre-
nal gland hypoplasia, and dysplasia/aplasia of the
315
thyroid. There are abnormalities of respiratory
function with frequent chest infections. The hand
abnormalities are bilateral and symmetrical and
involve complex syndactyly, postaxial polydac-
tyly, oligodactyly, short fourth metacarpals, and
camptodactyly (Fig. 11a, b). The feet are similarly
affected, with syndactyly and postaxial polydac-
tyly. The individuals who survive require hor-
mone replacement.
Diagnosis: based on family history, clinical and
radiological features, and genetic tests. This syn-
drome may be suspected at birth due to the typical
features, and early further investigations are
required to exclude the possible life-threatening
anomalies. Any child with this condition must be
monitored for hormone imbalance problems,
including hypopituitarism that may become evi-
dent during growth.
Pfeiffer Syndrome
(Acrocephalosyndactyly Type 5; ACS5;
Noack Syndrome)
[MIM 101600]
Etiology/inheritance: autosomal dominant
(FGFR1 gene mutation, chromosomal location
8p11.23-p11.22, or FGFR2 gene mutation, chro-
mosomal location 10q26.13). Additional genetic
heterogeneity and sporadic cases have also been
demonstrated.
Features: craniosynostosis (coronal with or with-
out sagittal suture) with characteristic hands and
feet anomalies. Hand/foot anomalies are
represented by broad thumb/broad hallux, partial
ngers/toes syndactyly, hands/feet brachymeso-
phalangia (Fig. 12ac). Other features include
radiohumeral elbow synostosis, turribrachycephaly,
cloverleaf skull (occasional), maxillary hypoplasia,
mandibular prognathism, hypertelorism, shallow
orbits, downslanting palpebral ssures, eye propto-
sis, strabismus, small nose, low nasal bridge,
choanal atresia/stenosis, high-arched palate, dental
crowding, laryngo-/tracheo-/bronchomalacia, car-
tilaginous trachea, hydrocephalus, Arnold-Chiari
malformation, and mental retardation (occasional).
Three subtypes of Pfeiffer syndrome have been
described (Cohen 1993):
316
Fig. 11 (a) Clinical
appearance of a hand in
Pallister-Hall syndrome. (b)
Radiographic appearance of
hands in Pallister-Hall
syndrome
Type 1: mild autosomal dominant
Type 2: sporadic, with cloverleaf skull, elbow
ankylosis, and early demise
Type 3: sporadic, with craniosynostosis, and
early demise
From an anesthetic point of view, they have the
same airway issues that are seen in the Apert patient.
Diagnosis: based on family history, clinical and
radiological features, and genetic tests.
Poland Syndrome (Poland Sequence;
Poland Syndactyly; Poland Anomaly)
[MIM 173800]
Etiology/inheritance: sporadic. Familial cases
(pedigrees compatible with autosomal dominant
L. Garagnani and G.D. Smith
inheritance) have also been reported. Male to
female ratio is 3:1. Cases of association with
Moebius syndrome have been described
(Poland-Moebius syndrome). Subclavian artery
disruption in embryogenesis has been postulated
as the pathogenetic cause.
Features: unilateral hypoplasia or absence of
pectoralis musculature (most often sternocostal
head of pectoralis major muscle) associated
with ipsilateral hand anomalies. Poland syndrome
is most frequently right sided (75 % of cases). Hand
anomalies include syndactyly, brachydactyly, and
oligodactyly. The wrist function depends on the
distal deciencies in the hand. If the deciency is
severe, the wrist may lie in exion, and there may
be a xed exion deformity of the wrist. The most
common hand deformity is short nger-type
symbrachydactyly, where there is a mildly
14 Syndromes Associated with Syndactyly 317

Fig. 12 (a) Radiographic appearance of hands in Pfeiffer syndrome. (b) Clinical appearance of feet in Pfeiffer syndrome.
(c) Radiographic appearance of the feet

hypoplastic thumb, small palm, multidigit simple Rubinstein-Taybi Syndrome

incomplete syndactyly, and brachymesophalangia,
sometimes with symphalangism in the radial digits
(Fig. 13). However, any type of hand deformity can
occur, some of which may be difcult to classify.
Dextrocardia is present in left-sided cases.
Other features include Sprengel anomaly,
hypoplastic/fused ribs, unilateral nipple/areola
hypoplasia or absence, unilateral breast absence,
latissimus dorsi/serratus anterior/infraspinatus/
supraspinatus/deltoid muscle hypoplasia, and
hemivertebrae.
Diagnosis: based on clinical and radiological
features. The mode of genetic transmission of
familial cases has not been determined.
Estimated incidence: 1 in 125,000 live births.
Due to its genetic variability, two forms of
Rubinstein-Taybi syndrome have been
documented:
Rubinstein-Taybi syndrome 1 (RSTS1,
Rubinstein syndrome; broad thumb-hallux
syndrome; broad thumbs and great toes,
characteristic facies, and mental retardation)
[MIM 180849]
Etiology/inheritance: autosomal dominant
(CREBBP gene, chromosomal location 16p13.3).
318
Fig. 13 Short nger-type symbrachydactyly in Poland
syndrome
Features: postnatal growth retardation, obesity
after puberty, short stature, microcephaly, large
anterior fontanelle, late closure of fontanelle,
agenesis of corpus callosum, mental retardation,
and characteristic facial appearance (highly
arched eyebrows, long eyelashes, downslanting
palpebral ssures, beaked nose with nasal septum,
broad nasal bridge, highly arched and narrow
palate, mild micrognathia, characteristic
grimacing/abnormal smile with almost closing of
the eyes, low-set ears). Other described anomalies
include deviated nasal septum, nasolacrimal duct
obstruction, strabismus, cataracts, glaucoma,
coloboma, dental crowding, talon cusps on
teeth, crossbite, enamel hypoplasia/discoloration,
hirsutism, hearing loss, genital anomalies in the
male (hypospadias, shawl scrotum, cryptorchi-
dism), joint hypermobility, patellar dislocation,
small iliac wings, delayed skeletal maturation,
scoliosis, sternal anomalies, large foramen
magnum and/or parietal foramina in the skull,
cutaneous cafe-au-lait spots and/or capillary
hemangiomas, and patent ductus arteriosus.
Atrial and/or ventricular septal heart defects
L. Garagnani and G.D. Smith
may be present. The thumbs and great toes
are symmetrically short and radially deviated,
with 40 % having a delta proximal phalanx
(Fig. 14a, b). Rarely, the thumbs are ulnar devi-
ated. There may be syndactyly, clinodactyly, and
polydactyly attening of the nger nails and hypo-
plasia of the toenails. Single transverse palmar
creases may be noted. Pes planus and plantar crease
between rst and second toes have also been found.
The affected individuals have an increased risk
of tumor formation and may be prone to keloid
formation and recurrent infections (respiratory
infections, otitis, and other infections). Neurological
abnormalities include severe expressive speech
delay, seizures, poor coordination and hypotonia,
hyperreexia, and EEG anomalies.
Rubinstein-Taybi syndrome 2 (RSTS2)
[MIM 613684]
Etiology/inheritance: autosomal dominant
(EP300 gene, chromosomal location 22q13.2).
RSTS2 is signicantly less common than
RSTS1 and appears associated with a milder
phenotype, with a less severe facial
dysmorphism and milder mental retardation, but
microcephaly and facial bones anomalies may be
severe.
Diagnosis: based on family history, clinical and
radiological features, and genetic testing. EEG
anomalies may be present.
Saethre-Chotzen Syndrome (SCS;
Acrocephalosyndactyly Type 3; ACS3;
Chotzen Syndrome; Acrocephaly, Skull
Asymmetry, and Mild Syndactyly)
[MIM 101400]
Etiology/inheritance: autosomal dominant
(TWIST1 gene, chromosomal location 7p21.1;
FGFR2 mutation, chromosomal location
10q26.13 identied in at least one individual
with SCS phenotype).
Estimated incidence: 1 in 25,0001 in 50,000 live
births.
14 Syndromes Associated with Syndactyly
Fig. 14 (a) Radial deviation of the thumb in Rubinstein-Taybi syndrome. (b) Radiographic appearance of the hand
Features: acrocephaly, brachycephaly,
plagiocephaly, hypertelorism, shallow orbits,
facial asymmetry, at face, high and at forehead,
low-set hairline, maxillary hypoplasia, small/low-
set ears, long/prominent ear crus, deafness, stra-
bismus, downslanting palpebral ssures,
buphthalmos, ptosis, S-shaped blepharoptosis,
anomalies of the lacrimal duct, optic atrophy,
beaked nose, thin/long/pointed nose, cleft palate,
narrow palate, craniosynostosis (coronal,
lambdoid, and/or metopic), intracranial hyperten-
sion, acrocephaly, late closure of fontanelles,
parietal foramina, short stature, large ischia,
small ilia, radioulnar synostosis, hand and
foot syndactyly (usually second web in the hand
and third web in the foot), bid distal phalanges of
second and third ngers, brachydactyly,
clinodactyly of the fth nger, broad thumb,
broad hallux, hallux valgus, and mild/moderate
mental retardation in some patients. Vertebral
fusions, congenital heart problems, anal atresia,
cryptorchidism (male), renal anomalies, dental
malocclusion, enamel hypoplasia, hyperdontia,
319
and abnormally shaped teeth have also been
described.
Diagnosis: based on family history, clinical and
radiological features, and genetic testing.
Smith-Lemli-Opitz Syndrome (SLOS;
SLO Syndrome; RSH Syndrome;
7-dehydrocholesterol Reductase
Deficiency, Rutledge Lethal Multiple
Congenital Anomaly Syndrome;
Polydactyly, Sex Reversal, Renal
Hypoplasia, and Unilobar Lung
Lethal Acrodysgenital Syndrome)
[MIM 270400]
Etiology/inheritance: autosomal recessive
(DHCR7 gene mutation, chromosomal location
11q13.4). The mutation is responsible for a de-
ciency of 7-dehydrocholesterol reductase, an
enzyme involved in cholesterol synthesis.
Estimated incidence: 1 in 20,0001 in 40,000 live
births.
320
Features: multiple congenital malformations and
mental retardation. SLOS usually manifests itself
at birth in severe form with stillbirth or neonatal
death from the associated cerebral and cardiac
anomalies, but in milder forms may present later
in life.
SLOS phenotypic features include as follows:
low birth weight (<2.5 Kg), poor growth and
developmental delay, short stature, microcephaly
(80 % of cases), second and third toes syndactyly
(95 % of cases), short and broad/overriding
toes, metatarsus adductus, talipes calcaneovalgus,
postaxial polydactyly of hands/feet, short thumbs,
proximal placement of thumbs, short limbs,
stippled epiphyses of bones (epiphyseal dysplasia
punticularis), subluxation/dislocation of the hip,
micrognathia, bitemporal narrowing, low-set/
posteriorly rotated ears, hypertelorism, palpebral
ptosis, epicanthal folds, cataract, strabismus,
anteverted nares (90 %), broad and at nasal
bridge, cleft palate (1/3 of affected individuals),
tongue hypoplasia, broad alveolar margins,
large central anterior teeth, dental crowding,
ventricular/atrial septal defect of the heart, aortic
coarctation, patent ductus arteriosus, lung
hypoplasia, incomplete lung lobulation, pyloric
stenosis, intestinal malrotation, poor sucking
reex/feeding difculties, gastroesophageal
reux, frequent vomiting, constipation, cryptor-
chidism (male), genital anomalies in 70 % of
affected males (hypospadias, micropenis,
hypoplastic or bid scrotum, microurethra,
ambiguous genitalia), renal agenesis, cystic
kidneys, hydronephrosis, obstructed ureteropelvic
junction, eczema, facial capillary hemangioma,
photosensitivity, blonde hair, central nervous
system anomalies (hydrocephalus, hypoplastic
frontal lobe, periventricular gray matter
heterotopias), hypotonia in early infancy,
hypertonia in childhood, seizures, abnormal
sleep pattern, mental retardation, and behavioral
and psychiatric manifestations (aggressiveness,
self-harm).
Diagnosis: the diagnosis is conrmed by low
cholesterol levels and elevated plasmatic and
tissue levels of 7-dehydrocholesterol, by means
of molecular diagnostic techniques. Prenatal
L. Garagnani and G.D. Smith
diagnosis can be established by means of
morphological studies, elevated levels of
7-dehydrocholesterol in the amniotic uid, chori-
onic villus sampling, and molecular diagnostic
techniques.
Split Hand/Foot Malformation 3
(SHFM3; Chromosome 10q24
Duplication Syndrome)
[MIM 246560]
Etiology/inheritance: autosomal dominant with
incomplete penetrance (chromosomal location
10q24).
Features: median clefts of the hands and feet,
syndactyly, phalangeal, metacarpal and metatarsal
hypoplasia and/or aplasia, and preaxial involve-
ment of the upper limbs described in 60 % of cases
(preaxial polydactyly, proximal placement of the
thumb and/or triphalangeal thumb, rst ray
aplasia) (Elliott et al. 2005). Other associated phe-
notypic patterns have been described, including
mental retardation, ectodermal and craniofacial
ndings, and orofacial clefting (Elliott and
Evans 2006).
The strong family history in most cases means
the surgeon will be confronted with parents who
already have strongly held beliefs on the benet or
otherwise of intervention, colored by their own
personal experiences which do not necessarily
translate to the current needs of their child.
These children are usually fully physically func-
tional without intervention but may nd social
functioning in the future difcult due to their
obvious deformities.
Diagnosis: based on family history, clinical and
radiographic features, and genetic testing.
Synpolydactyly
Due to genetic variability, different forms of this
disorder are known:
Synpolydactyly 1 (SPD1; syndactyly type 2;
syndactyly type II)
14 Syndromes Associated with Syndactyly
[MIM 186000]
Etiology/inheritance: autosomal dominant
(HOXD13 gene mutation, chromosomal location
2q31.1).
Features: bilateral third and fourth nger syndac-
tyly and bilateral fourth and fth toe syndactyly,
mesoaxial polydactyly in the hand with partial or
complete duplication in syndactylous web, postax-
ial or preaxial (rare) polydactyly in the foot, clino-/
camptodactyly of the fth nger, brachymeso-
phalangia of the fth nger and of second to fth
toes, six metacarpals/metatarsals, and Y-shaped
metacarpals. Carpal and tarsal bones are normal.
Frequently poor movement in the third but espe-
cially fth (ring) nger, even after release, with
tendon, bone, and neurovascular anomalies present
in these digits. Parental guilt, and desire for a better
functional and esthetic outcome than their own,
frequently pushes the childs treatment.
Synpolydactyly 2 (SPD2; synpolydactyly, 3/3-
prime/4, associated with metacarpal and meta-
tarsal synostoses)
[MIM 608180]
Etiology/inheritance: autosomal dominant
(FBLN1 gene disruption, chromosomal location
22q13.31). FBLN1 disruption is associated with a
reciprocal translocation t(12,22) (p11.2; q13.3).
Features: bilateral synpolydactyly of third and
fourth nger, bilateral syndactyly of second,
third, and fourth toes, fourth and fth metacar-
pal/third and fourth metatarsal synostosis. The
malformations are symmetric.
Synpolydactyly 3 (SPD3)
[MIM 610234]
Etiology/inheritance: autosomal dominant (chro-
mosomal location 14q11.2-q12). Described in
16 individuals over six generations, in a large
Pakistani family (Malik et al. 2006).
Features: complex syndactyly of third and fourth
ngers and postaxial toes synpolydactyly are the
cardinal features. Other features include simple
syndactyly, symphalangism, clinodactyly,
camptodactyly, and metacarpal anomalies.
321
Diagnosis: based on family history, clinical and
radiographic features, and genetic testing.
Timothy Syndrome (TS, Long QT
Syndrome with Syndactyly, Long QT
Syndrome 8, LQT8)
[MIM 601005]
Etiology/inheritance: autosomal dominant
(CACNA1C gene, chromosomal location
12p13.33). Two types (types 1 and 2) have been
described due to mutations in a transcript variant
of the same gene. TS often results in early child-
hood death.
Features: multiorgan dysfunction that includes
long QT syndrome with lethal arrhythmias (ven-
tricular tachyarrhythmia is a frequent cause of
sudden cardiac death in affected individuals), con-
genital heart defects (patent ductus arteriosus,
patent foramen ovale, ventricular septal defect,
tetralogy of Fallot, hypertrophic cardiomyopa-
thy), hand and foot syndactyly, at nasal bridge,
low-set ears, thin upper lip, round face, intermit-
tent/intractable hypoglycemia, immune de-
ciency, cognitive abnormalities, seizures, and
autism.
Death from cardiac arrhythmia under
anesthesia for hand surgery is a possibility if
the subtle dysmorphia seen in these
children does not prompt electrocardiogram
(ECG) and cardiac ultrasound investigations
prior to surgery.
Diagnosis: based on family history, neonatal
ECG, cardiac ultrasound, clinical features, blood
tests that may identify hypoglycemia, and genetic
testing.
Triploidy Syndrome (Triploid
Syndrome; Triploidy; Chromosome
Triploidy Syndrome; 3n Syndrome)
Etiology/inheritance: extremely rare chromo-
somal disorder (69XXY), invariably fatal, with
almost all fetuses dying in utero. Cases of individ-
uals surviving a few months have been rarely
322
reported. Diploid/triploid mosaic individuals have
more chances to survive (see section Diploid/
Triploid Mosaicism).
Features: large placenta with hydatidiform
changes, intrauterine growth retardation (IUGR),
syndactyly, omphalocele, cardiac defects, hydro-
cephalus, holoprosencephaly, genitourinary
anomalies, and club feet.
Diagnosis: this condition may be suspected on the
basis of routine screening tests, but only genetic
testing can conrm the diagnosis (amniocentesis,
karyotype of a newborn baby or miscarried/still-
born fetus).
Assessment
Prenatal diagnosis of limb and other associated
anomalies may be made during fetal ultrasonog-
raphy screening, but, for the majority of cases,
neither the limb deformity nor the associated syn-
drome is recognized. This includes syndromes,
such as Apert syndrome, where the phenotype is
obvious at birth but the condition is rare and the
prenatal screening is directed at life-threatening
abnormalities. In those patients with a family his-
tory, antenatal detection is much more likely as the
scanning will also be directed towards looking for
similar abnormalities in the fetus.
Depending on the ndings, a diagnostic
hypothesis may be formulated, and further inves-
tigations like chorionic villus sampling (CVS),
amniocentesis, or percutaneous umbilical cord
blood sampling (PUBS) may be performed. A
multidisciplinary team that comprises the obste-
trician/neonatologist, the clinical geneticist, the
clinical psychologist, the pediatrician, and the
surgeons and physicians that will be involved in
the postnatal treatment should be involved. In
those cases where a denite prenatal diagnosis is
made, the parents must be informed of the possi-
ble associated anomalies and related treatment
options. Genetic counseling of the family is
required for all syndromic forms of syndactyly,
and a pedigree drawn on the family history must
be created. Molecular genetic tests should be
performed to detect the specic genetic mutations
in inherited cases if this is done in the family
L. Garagnani and G.D. Smith
prior to the pregnancy, it may be possible to diag-
nose on the fetal cells from maternal blood. An
early, honest, and appropriately supportive dia-
logue with the parents and families of the affected
individuals is warranted, and psychological
assessment and counseling of these families is
paramount. Physical and radiographic examina-
tion of the hands and feet affected by syndactyly is
required. A thorough neonatal ultrasound assess-
ment should always be carried out in these
patients, and targeted radiographic examination
of the spine, skull, chest, pelvis, and limbs may
be required when associated anomalies are
suspected. Neonatal electrocardiogram (ECG)
may be required to detect a congenital long QT
syndrome or arrhythmias, bearing in mind that a
completely normal ECG may be seen with multi-
ple types of congenital heart defects; hence, car-
diac ultrasound is additionally indicated. Blood
tests may also show abnormalities, such as hypo-
glycemia in Timothy syndrome. Additional exam-
inations such as MRI (like in Moebius syndrome)
and CT may be used to detect or better dene the
associated anomalies in selected cases.
Treatment
In the syndromic forms of syndactyly, a multidis-
ciplinary approach with agreement on the order of
priorities is paramount: life-threatening condi-
tions and malformations should be addressed
rst. The surgical release techniques used in
syndromic forms of syndactyly do not substan-
tially differ from those used in the isolated forms
of syndactyly. Syndromic features like laryngo-,
tracheo-, and bronchomalacia, congenital heart
defects, respiratory and renal anomalies, and
insufciency must be compatible with the anes-
thetic and surgical maneuvers; otherwise, syndac-
tyly release has to be delayed or avoided. Timing
of release in the hand is crucial, once these condi-
tions are addressed: complete syndactyly of the
fourth web, acrosyndactyly of the central ngers,
complete syndactyly of digits of different length
that may develop secondary clino- and/or
camptodactyly, and some forms of complex syn-
dactyly that may worsen or cause secondary
14 Syndromes Associated with Syndactyly
deformities of the neighboring rays warrant early
surgical release. The complex and complicated
forms require osteotomies to separate the neigh-
boring digits and remodelling/corrective
osteotomies and/or excision of anomalous or
supernumerary rays. Skin grafting to obtain ade-
quate coverage of the skin defects is often
required, due to shortage of skin especially in the
complex and complicated types of syndactyly.
Alternative techniques, using dermal substitutes
instead of the skin grafts (Landi et al. 2014), or
progressive soft tissue distraction of a web with a
specially designed external xator that allows to
achieve skin expansion prior to release of the
syndactyly (Habenicht 2000), have also been
developed. Skin expansion by tissue expanders
has not achieved good results in the hand, partic-
ularly in cases, such as in Apert syndrome, where
skin shortage is a major issue. Despite optimal
assessment and intervention, the hands in some
syndromes, like Cenani-Lenz, are extremely chal-
lenging from which to get any useful function, and
the outcome is likely to result in the child still
requiring the use of a bimanual grasp. Intervention
for foot syndactyly is seldom needed from a func-
tional point of view when syndactyly affects the
toes: the rationale of surgery may be merely cos-
metic in such cases. Walking and balance prob-
lems, like it may occur in the complex forms with
metatarsal/midfoot involvement, warrant surgical
treatment, like in those Apert syndrome cases
where the second and third metatarsals may
become prominent and painful to walk
on. Cosmetic reduction or interruption of a com-
mon broad toenail may also be performed in Apert
patients. In the future, advances in the molecular
genetic techniques may allow genetic treatment of
those anomalies that are inherited.
Summary
The heterogeneity of syndromes associated with
syndactyly makes the diagnosis and treatment of
each of these conditions a unique challenge for the
surgeon. This eld is in continuous evolution:
reports of new cases of syndromic patients
affected by syndactyly continue to enrich the
323
international literature on this subject, and the
scientic advances are pushing the boundaries
towards early diagnosis and treatment. For the
surgeon, the importance of diagnosis lies in the
associated features of these syndromes and their
implications for anesthesia and for the long-term
prognosis for hand function in the child, which is
related to the prognosis for cognitive function as
well as the anatomical limb anomalies.
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 Duplication
15
Goo Hyun Baek

Contents Abstract
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 326 Congenital duplication of hand differences
includes radial, central, and ulnar polydactyly
Radial Polydactyly . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 326
Terminology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 326
and the mirror hand. This article focuses on
Epidemiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 326 surgical techniques for various clinical cases
Classication . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 326 as well as clinical features. Most of polydac-
Preoperative Evaluation and Discussion with tyly children can be treated with the excision
the Parents and Patient . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 327
Timing of Surgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 328
and reconstruction technique. Main principles
Surgical Techniques . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 328 of this procedure are arthroplasty, corrective
osteotomy, and tendon realignment. The orig-
Central Polydactyly . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 348
Epidemiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 349 inal technique of Bilhaut-Cloquet procedure
Classication . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 350 was modied to overcome common complica-
Surgical Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 351 tions such as joint stiffness, physeal growth
Ulnar Polydactyly . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 356 disturbance, and nail-plate deformity. Two
Epidemiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 356 types of modied techniques, one for Wassel
Classication . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 356 type 2 and the other for Wassel type 4, are
Surgical Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 357
introduced. On-top plasty technique for certain
Mirror Hand . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 361 patients with radial polydactyly in which better
Terminology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 361
distal part of one thumb is transposed to the
Epidemiology and Classication . . . . . . . . . . . . . . . . . . . . 362
Surgical Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 364 better proximal part of the other thumb is
presented. Classication and surgical strate-
Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 366
gies for central polydactyly and ulnar polydac-
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 366 tyly were reviewed.
The mirror hand deformity is a very rare
type of congenital hand differences. A report
of four cases is the largest series ever reported.
Because of its rarity, most surgeons should
depend on literatures for surgical planning.
Some confusion of terminology such as mirror
hand, ulnar dimelia, and multiple hands were
G.H. Baek
discussed, and classication and surgical prin-
Department of Orthopedic Surgery, Seoul National
University College of Medicine, Seoul, South Korea ciples were reviewed.
e-mail: ghbaek@snu.ac.kr

# Springer Science+Business Media New York 2015 325

J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_15
326
Introduction
According to the IFSSH (International Federation
of Societies for Surgery of the Hand) classication
of congenital hand anomalies (Upton 1990),
duplication can be subdivided into six catego-
ries (Table 1).
This chapter will discuss the various diagnoses
that can occur from duplication as well as their
treatments, including radial polydactyly, central
polydactyly, ulnar polydactyly, and ulnar dimelia
(mirror hand).
Radial Polydactyly
Terminology
Radial (preaxial) polydactyly is sometimes called
as thumb duplication, split thumb, or bid thumb.
The meaning of duplication in the dictionary is
one of two things exactly alike or a doubling.
However, it is uncommon in radial polydactyly
that the size and shape of the two thumbs are both
same and normal. Furthermore, triplication of
the thumb was found in 9 out of 121 patients
with radial polydactyly in the Netherlands
(Zuidam et al. 2008). Split thumb has a nuance
that it can be a complete thumb if the two com-
ponents are combined. However, this is not a
common situation. Bid, which means sepa-
rated into two parts, may have the same nuance
as split thumb. When there is no bony connection
between the two thumbs, as in pedunculated type
of radial polydactyly, split or bid thumb may not
be an appropriate description. When the parents
of a baby with radial polydactyly hear that
their baby has a duplicated, split, or bid Rubinstein-Taybi syndrome.
thumb, they might simply think that ablation or
combination of two parts is enough for surgical
treatment.
Although radial polydactyly or polydactyly of
the thumb is not a better description for this entity,
it just means many thumbs which may lessen
the misunderstandings of the parents.
G.H. Baek
Table 1 Duplication
1. Whole limb
2. Humerus
3. Radius
4. Ulna
(a) Mirror hand
(b) Other
5. Digit
(a) Polydactyly
(1) Radial (preaxial, 1st ray, including triphalangeal
thumb)
(2) Central
(3) Ulnar (postaxial, 5th ray)
(4) Combinations
6. Epiphyseal (extra)
(a) 1st ray
(b) 2nd ray
(c) Other
Epidemiology
The incidence of radial polydactyly is reported at
0.081.4 per 1,000 live births (Sesgin and Stark
1961; Temtamy and McKusick 1978). It is a com-
mon congenital difference of the upper extremity
in all races, and about 20 % of them occur bilat-
erally. In Asian countries such as Japan, Korea,
and Hong Kong, polydactyly is more common
than syndactyly (Ogino et al. 1986; Baek
et al. 1997). Syndactyly was more common than
polydactyly in the study from University of Iowa
(Wassel 1969). Most radial polydactyly occur
sporadically. However, when it is associated
with triphalangeal thumb, a higher hereditary pre-
disposition has been identied. Radial polydac-
tyly can occur in syndromic diseases such as
Fanconis anemia, Holt-Oram syndrome, and
Classification
The Wassel classication for polydactyly of the
thumb (Wassel 1969) has been widely used,
although several other classication systems
15 Duplication
Table 2 Wassel classification for polydactyly of the
thumb (Wassel 1969)
Type 1: A bid distal phalanx with a common epiphysis
which articulates with a normal proximal phalanx
There may be one common nail, but usually there are two
distinct nails with a groove between them
Type 2: A completely duplicated distal phalanx
Each distal phalanx usually has its own epiphysis that
articulates with the normal proximal phalanx
Type 3: A duplicated distal phalanx with a bifurcated
proximal phalanx
The distal phalanges usually diverge from the
longitudinal axis, or they may be parallel
Type 4: A complete duplication of the proximal phalanx
Each proximal phalanx has its own epiphysis or a
common epiphysis that articulates with a normal
metacarpal or a metacarpal slightly widened to
accommodate both proximal phalanges
Type 5: A bifurcated rst metacarpal
Each head of the bifurcation articulates with a duplicated
proximal phalanx that has its own epiphysis
Type 6: Complete duplication of the entire rst digit
One side may be more rudimentary than the other
Type 7: A triphalangeal thumb or elements of a
triphalangeal thumb accompanied by a normal thumb
have been proposed (Temtamy and McKusick
1978; Chung et al. 2013). This classication sys-
tem is simple in application and communication
(Table 2). Wassel type 4 is the most common type,
representing 2943 % of all polydactyly of the
thumb, while type 1 is the least common (Wassel
1969; Al-Qattan 2010).
The Wassel classication is based on an assess-
ment of the skeleton. In young children whose
skeleton is immature, the true nature of the thumbs
may not be apparent. For instance, Wassel type
1 polydactyly can be classied as a type 2 until
ossication of the distal phalangeal epiphysis
becomes apparent (Tonkin 2012). Wassel type
7 polydactyly which has a triphalangeal compo-
nent shows diverse manifestations and is further
subclassied into six types (Wood 1978).
There are some types of radial polydactyly
unclassiable by the Wassel system. Peduncu-
lated type, triplicated thumb, or extra thumb that
does not have bony connection with the main
thumb cannot be classied.
327
Preoperative Evaluation and
Discussion with the Parents and Patient
When a baby with radial polyactyly and his/her
parents visit the outpatient department (OPD), it is
important to inspect both hands of the patient.
Most of the babies with unilateral involvement
show smaller sizes of affected thumbs than those
of contralateral normal thumbs. Thus, the parents
should understand that even if the more dominant
one is preserved in the affected thumb, it will be
smaller in length and girth when compared to the
unaffected side.
In babies with bilateral involvement, the nail
size of index nger can be a reference to judge the
size of the affected thumbs. The width of the index
nger nail is about two thirds of that of the thumb
in normal babies.
Active motion of each joint is hard to observe
because the babies usually clench their hands.
Passive motion and varus and valgus stress tests
of the joints, palpation of tendons (especially
exor tendons), and observation of skin crease
may be helpful to evaluate the polydactylic
thumbs. Little or absent passive motion at bifur-
cation site of minor thumb (mostly radial one)
may suggest odd numbered Wassel type 1, 3, or
5. It is much easier to reconstruct a thumb that has
stable joints in radioulnar plane. If the exor ten-
don is palpable while moving the joint passively,
good active motion can be expected postopera-
tively. When the skin crease is faint or absent,
there is a strong possibility that the affected joint
does not have effective motor power.
Simple radiographs are a prerequisite for Wassel
typing and surgical planning. Although it is not easy
to obtain a true PA and lateral view of the affected
thumbs, it is absolutely necessary for surgical plan-
ning. Radiographs of normal side in unilateral cases
are also important in assessing the size and shape of
bones and joints of the affected thumbs compara-
tively. Medical photos are also needed for documen-
tation and later evaluation of surgical outcome.
Before surgery, the parents should be informed
that even if the thumb is successfully
reconstructed, it will not be the same as the
328
contralateral normal thumb in terms of function
and appearance.
In a study of 66 years of experiences for sur-
gery of the duplicated thumb (Townsend
et al. 1994), there were 52 % of patients with
unacceptable results, 18 % of them unsalvageable
by secondary surgery. More recently, the compli-
cation rates appear to be lowering. When initial
surgery was planned to restore all anatomic ele-
ments, the need for secondary surgery was
unusual (Waters and Bae 2012, p. 41). The pri-
mary issues affecting appearance after surgery for
radial polydactyly were reduced nail width and
angulation at interphalangeal joint. Reconstructed
Wassel type 7 thumbs had lower satisfaction score
than other types (Goldfarb et al. 2008). The
patients and their parents sometimes complain of
the postoperative long arm cast; however, a short
arm cast can be easily removed in young children.
Timing of Surgery
There has been no consensus on the timing of
operation for radial polydactyly. In the textbook
of Greens operative hand surgery, it is
recommended to perform surgery at about 1 year
of age before the development of thumb-index
nger pinch (Kozin 2011). In the textbook of
Campbells operative orthopedics, it is
recommended to perform surgical reconstruction
when the child is about 18 months old, but no later
than 5 years old if possible (Jobe 2011). Indebted
to recent advancement in pediatric anesthesia,
most surgeries can be performed safely if the
patient does not have serious comorbidity, such
as cardiac anomaly or pancytopenia. In certain
cases, Wassel type 7, for example, bony shape of
the delta bone, is sometimes important for surgical
planning in which the surgical timing is better to
be postponed until it is clearly visible in radio-
graphs. Thus, timing of surgery depends on gen-
eral condition of the patients, priority of surgery in
patients with multiple associated anomalies, types
of radial polydactyly, and equally importantly
surgeons preference. There is no gold standard
for surgical timing of radial polydactyly.
G.H. Baek
However, earlier surgery is recommended when
surgical planning is completed and the structures
of the thumb are large enough to manipulate
surgically.
The same principles of timing of surgery can
be applied to central polyactyly and ulnar
polydactyly.
Surgical Techniques
The goal of surgical reconstruction is to make a
straight, mobile, and stable thumb of good appear-
ance in size and shape. However, there is no hand
surgeon that can make a normal thumb. We are
trying to make a better thumb in a given situation,
not the best or perfect thumb.
The patients with radial polydactyly show
diverse manifestations, from a rudimentary oat-
ing type to a complex one. Ligation or simple
excision may be enough for oating types of
radial polydactyly. However, simple ablation of
one digit has not produced satisfactory outcomes
in most cases of radial polydactyly. Resultant
deviation, stiffness, and/or ligamentous instability
of the thumb is commonplace. Although surgical
concepts and techniques are still evolving, there
are several reconstructive strategies to achieve a
functionally and aesthetically acceptable thumb.
Surgical techniques to reconstruct radial poly-
dactyly can be classied into ve types ligation,
simple excision, excision and reconstruction,
combination procedures (Bilhaut-Cloquet opera-
tion), and on-top plasty.
Radial polydactyly
Surgical techniques
Ligation
Simple excision
Excision and reconstruction
Combination procedure (Bilhaut-Cloquet operation)
On-top plasty
The surgical wound is usually closed with
absorbable 5-0 or 6-0 sutures. If the wound is
closed with nonabsorbable sutures, sedation of
the patients may be needed for stitch removal. A
long arm thumb spica cast with more than 90 of
15 Duplication 329

Fig. 1 A pedunculated and

partly necrotic radial
polydactyly of a newborn
baby. Vascular structures
are seen in the stalk

elbow exion is recommended postoperatively, Ligation

because a short arm thumb spica cast or a long
arm cast in a position of less elbow exion can be
easily taken off. Duration of immobilization for
patients undergoing corrective osteotomy and/or
reconstruction of collateral ligament should be
46 weeks depending on the patient age. Postop-
erative therapy is not necessary in most patients.
Radial polydactyly
Operative treatment
Surgical
techniques Indications
Ligation Pedunculated type
Simple excision No bony connection between two
polydactyly thumbs. Dominant
thumb shows good stability,
motion, and appearance
Excision and Most of radial polydactyly
reconstruction
Combination Both thumbs are hypoplastic and
procedure almost symmetric, especially, when
the nail width is less than 2/3 of
contralateral normal side in
unilateral cases and when the nail
width is less than that of index
nger in bilateral cases
On-top plasty One thumb has well-developed
proximal part and poorly developed
distal part with absent or
hypoplastic nail. The other thumb
has poorly developed proximal part
and better distal part including nail
and pulp
In a pedunculated type of radial polydactyly, liga-
tion at the base as close as possible to its root with
5-0 or 6-0 Nylon with or without local anesthesia
can be performed in the outpatient department or
nursery (Fig. 1). A ligated hypoplastic thumb will
mummify and usually falls off within 2 weeks
(Fig. 2). A nubbin or dimple usually remains
afterward (Fig. 3). Even in pedunculated type of
polydactyly, painful neuroma may develop after
the ligation, which is an indication for surgical
exploration (Leber and Gosain 2003).
Simple Excision
When there is no bony connection between two
polydactyly thumbs and a dominant thumb shows
good stability, motion, and appearance, simple
excision under general anesthesia is indicated
(Figs. 4, 5, and 6).
Technique: An elliptical incision is made
around the minor thumb. The soft tissue pedicle
usually contains neurovascular structures. To
avoid bleeding, the vessels should be ligated or
cauterized. To prevent painful neuroma, the nerve
should be identied, sharply transected, and
embedded in the soft tissue.
Excision and Reconstruction
Most of radial polydactyly can be successfully
treated by the excision and reconstruction tech-
nique (Fig. 7). Main components of this procedure
330
are arthroplasty, corrective osteotomy, and tendon
realignment. When one of the two polydactylic
thumbs is well developed and the other one less
developed, this technique is indicated. However,
when both polydactylic thumbs are hypoplastic,
this technique results in a small thumb which is
sometimes smaller than the index tip. Surgical
Fig. 2 Ligated thumb became mummied and fell off
9 days after the ligation
Fig. 3 An asymptomatic
dimple is remained after the
ligation
G.H. Baek
technique for Wassel types 1 and 2 is similar. Also
similar surgical technique can be applied to Wassel
types 3, 4, 5, and 6. For the diversity of clinical
features, surgical technique for Wassel type 7 should
be individualized case by case.
Arthroplasty: Arthroplasty consists of joint sta-
bilization by ligamentoperiosteal ap (Manske
1989) and partial excision of excessive portion
of phalangeal or metacarpal head that articulates
with the two thumbs.
Two thumbs articulate with a single proximal
phalangeal head in Wassel type 1 or 2 radial
polydactyly and on a single metacarpal head in
type 3 or 4. During dissection of minor thumb,
distal insertion of collateral ligament should be
preserved with adjacent periosteal tissue for later
reconstruction. This ligamentoperiosteal ap is
reattached to base of phalangeal bone of the
retained main thumb after removal of the minor
thumb. The phalangeal or metacarpal head, when
minor thumb is removed, is relatively large for
the remained dominant thumb. This size
mismatching may cause angular deformity
and/or bony prominence if it is not corrected.
Thus, excessive portion of the head needs to be
shaved or removed. Sometimes, a separate facet
that articulates with the radial thumb to be
deleted is observed. This facet can be used as a
guideline to cut excessive portion. Conventional
oscillating saw or osteotomes cannot be used for
small phalangeal bones of young children. Their
phalangeal bones are soft enough that shaving of
15 Duplication
Fig. 4 A minor radial thumb is attached to the robust main
thumb
Fig. 5 There was no bony
connection between two
thumbs
articular cartilage and partial ostectomy can be
performed by a small rongeur or a No. 15 surgical
blade. Excessive tension of the reconstructed
collateral ligament to correct angular deformity
at the joint level is not recommended because the
deformity is likely to recur and stiffness of the
331
Fig. 6 Good appearance after simple excision
joint may occur. However, angular deformity of
less than 10 at the joint level can be corrected by
this arthroplasty procedure. A longitudinal
Kirschner wire (K-wire) is inserted to protect
the reconstructed collateral ligament.
Corrective Osteotomy: Angulation at
interphalangeal joint and reduced nail width are
primary issues affecting appearance after the sur-
gery (Goldfarb et al. 2008). More than 20 of
angular deformity is not acceptable to most
patients and parents. The angulation can be
corrected by closed wedge osteotomy. Double-
level osteotomy at proximal phalangeal and meta-
carpal levels can be indicated to align severe
divergent-convergent Wassel type 4.
Tendon Realignment: Abnormal insertions of
exor pollicis longus (FPL) and/or extensor
pollicis longus (EPL) are not uncommon in radial
polydactyly, especially in Wassel type 4. The FPL
tendon attaches not only at its customary insertion
but also into the extensor by a tendon that passes
around the radial aspect of the thumb. This anom-
alous muscle abducts the thumb instead of exion
and is called as pollex abductus (Tupper 1969;
Lister 1991). The abnormal alignment of FPL
332 G.H. Baek

Fig. 7 Ligamentoperiosteal ap was raised to reconstruct radial collateral ligament of MP joint. Metacarpal head was
excised partially to t base of dominant proximal phalanx. Corrective osteotomy was added to make a straight thumb

and/or EPL may cause gradual angular deformity

even after successful bony alignment has been
achieved by corrective osteotomy. When there
are abnormal insertions of FPL and/or EPL ten-
dons, the insertion sites should be realigned to
achieve a good exion-extension arc. The abnor-
mal insertion can be completely detached and
reattached into the correct position. The phalan-
geal bones of young children are soft that the
tendon can be sutured into the distal phalanx
using 4-0 or 5-0 Nylon. When the phalangeal
bone is too hard to be sutured by Nylon suture, a
pullout suture technique can be used. When the
distal portion of the tendon is bid and inserted
into both polydactylic thumbs, it usually inserts at
ulnar side of the radial thumb and radial side of the
ulnar thumb. If the radial thumb is to be removed,
the tendon is detached from the insertion of radial
thumb and reattached into the ulnar side of dom-
inant ulnar thumb like Y shape to balance the
vector forces (Figs. 8 and 9).
In Wassel type 4, 5, 6, or 7 polydactyly of the
thumb, some of thenar muscles insert into the
radial side thumb. In most cases, the radial Fig. 8 The tendons frequently bifurcated distal to the MP
thumb is removed and the ulnar thumb is joint, and insert to the side of each distal phalanx
15 Duplication
Fig. 9 The tendon
insertion of minor thumb is
detached and reattached to
the main thumb in Y shape
to balance the vector force
reconstructed. The insertion site of thenar muscles
on the radial thumb should be identied and dis-
sected carefully for later reattachment to the main
ulnar thumb.
Excision and reconstruction
Surgical components
Arthroplasty: joint stabilization by ligamentoperiosteal
ap and partial excision of excessive portion of
phalangeal or metacarpal head
Corrective osteotomy for angular deformity
Tendon realignment when FPL/EPL insertions are
abnormal
Surgical Technique (Wassel Type 2): A
9-month-old boy showed hypoplastic Wassel
type 6 radial polydactyly on right thumb and
Wassel type 2 on left (Fig. 10). The nail size,
length, and girth of ulnar side thumb of left hand
were good enough to perform the excision of
radial thumb and reconstruction procedure.
Although the epiphyses of phalanges and
333
Fig. 10 Wassel type 2 polydactyly of left thumb
metacarpal are not observed in simple radiograph
(Fig. 11), it is easy to decide the surgical plan as
arthroplasty with or without tendon realign-
ment. Corrective osteotomy will not be necessary
because angular deformity at the IP joint is mini-
mal. A racquet-shaped incision was designed
(Fig. 12). A zigzag incision has an advantage to
prevent possible scar contracture, but this technique
is not easy to apply to a small-sized thumb less than
an inch in length in infant age. The dissection was
deepened to expose distal phalangeal bone of the
radial thumb, and a ligamentoperiosteal ap was
raised using No. 15 blade (Fig. 13). Distal phalanx
of the radial thumb was cut to be removed, and
articular surface of this radial thumb was seen
(Fig. 14). Articular surface for the radial thumb
was cut using No. 15 blade (Fig. 15). The consis-
tency of phalangeal bone in infant age is soft
enough to be cut by surgical blade. There was no
malalignment of tendon found. After the
arthroplasty procedure, the articular surface of the
proximal phalanx t that of ulnar thumb (Fig. 16).
334
Before reconstruction of the collateral ligament, a
0.7 mm K-wire was inserted longitudinally to pro-
tect it. The ligamentoperiosteal ap for reconstruc-
tion of the collateral ligament was attached to the
Fig. 11 Ulnar distal phalanx showed better conguration
Fig. 12 Skin incision
G.H. Baek
new insertion site by 5-0 absorbable suture
(Fig. 17). Direct suture of skin edge to the nail
does not achieve normal lateral nail fold. A suture
needle was introduced about 3 mm away from the
skin ap to be sutured to the nail, and it came out
1 mm away from the skin ap to simulate the lateral
nail fold. Then the needle was passed through the
nail, and tied (Fig. 18). The reconstructed thumb
looked straight and reconstructed lateral nail fold
looked adequate (Fig. 19). On a postoperative
radiograph, shaved proximal phalangeal head t
distal phalanx so well that IP joint looked natural
(Fig. 20).
Surgical Technique (Wassel Type 4): An
11-month-old boy showed a divergent-convergent
Wassel type 4 radial polydactyly. The radial
thumb of left hand was hypoplastic, but the ulnar
thumb showed good size and shape (Fig. 21).
There was 40 of angular deformity at the IP
joint of ulnar thumb that needed corrective
osteotomy at proximal phalangeal neck level
(Fig. 22). Medical photos and simple radiographs
suggested strong possibility that arthroplasty of
MP joint and tendon realignment of EPL, FPL,
and thenar muscles were necessary for proper
reconstruction. Proximal phalangeal head of the
ulnar thumb was underdeveloped, suggesting a
potential recurrence of angular deformity postop-
eratively. A racquet-shaped incision was designed
(Fig. 23). The EPL tendon was bifurcated at MP
15 Duplication
Fig. 13 A
ligamentoperiosteal ap
was raised (forcep)
Fig. 14 The articular
surface for removed radial
distal phalanx was observed
joint level and inserted into both thumbs. The
insertion site of radial EPL slip was detached
(Fig. 24) and sutured to the ulnar side of dominant
thumb to balance the extension force (Fig. 25).
The FPL tendon showed the same pattern. The
insertion site of radial FPL slip was detached and
tagged with suture for later reattachment into the
ulnar side of dominant thumb (Fig. 26). The
abductor pollicis brevis muscle insertion into the
radial thumb was detached from the proximal
phalangeal base for later reattachment into the
335
reconstructed thumb. The radial thumb was
removed leaving ligamentoperiosteal ap for
later reconstruction of the MP joint. The portion
of metacarpal head to be resected was lined, and a
ligamentoperiosteal ap was raised and preserved
(Fig. 27). Excessive portion of articular cartilage
and bone was removed by No. 15 blade and small
osteotomes (Fig. 28). Then proximal phalanx
was dissected subperiosteally for an ulnar-based
closed wedge osteotomy to correct angular defor-
mity at the IP joint (Fig. 29). It is convenient to
336
Fig. 15 This portion was
cut using No. 15 blade
Fig. 16 Now the articular
surfaces of the IP joint t
very well
perform ulnar-based closed wedge osteotomy
from ulnar side incision. However, simultaneous
medial and lateral incision on the same thumb
may jeopardize blood circulation. The MP joint
was xed in a reduced position with a K-wire, and
also osteotomy site of proximal phalanx was xed
with an additional K-wire (Fig. 30). Finally the
FPL tendon detached from the radial thumb was
reattached into the ulnar side of reconstructed
distal phalanx. The abductor pollicis brevis ten-
don detached from radial thumb as well as the
G.H. Baek
previously raised ligamentoperiosteal ap for col-
lateral ligament reconstruction was reinserted into
the base of proximal phalanx (Fig. 31). Alignment
and appearance of the reconstructed thumb
(Fig. 32) and the immediate postoperative radio-
graph (Fig. 33) showed reasonable result.
Surgical Technique (Wassel Type 7): A
12-month-old girl showed a radial polydactyly
bid at metacarpal shaft level. The ulnar thumb
had delta middle phalanx with angular deformity
(Figs. 34 and 35). A racquet-shaped incision was
15 Duplication 337

Fig. 17 A K-wire was

inserted longitudinally, and
the ap was reattached to
the new insertion site

Fig. 18 To simulate lateral

fold of the nail, suture
needle was introduced
about 3 mm away from the
skin ap, and it came out
1 mm away from the skin
ap and then passed
through the nail

made along the radial thumb, and it was excised
by dividing bony connection at metacarpal shaft
level. Another straight incision was made along
the radial side of the ulnar thumb to excise the
delta middle phalanx. After excision of the delta
bone, the radial collateral ligament was sutured in
proper tension. When the patients age is less than
6 years, simple excision of the delta bone yields a
good result (Hovius et al. 2004). A longitudinal
K-wire was inserted to protect reconstructed
radial collateral ligament of IP joint (Fig. 36).
Three years after the operation, alignment and
range of motion was good (Figs. 37 and 38).
Combination Procedure (Modified
Bilhaut-Cloquet Procedure)
The original Bilhaut-Cloquet procedure
(BC procedure) consists of resection of the central
portion of duplicated segment and the coaptation
of outer parts of bone, soft tissue, and nail tissue
for the treatment of radial polydactyly (Bilhaut
1889). This procedure has advantage in obtaining
338 G.H. Baek

Fig. 19 The lateral nail

fold looked natural after
reconstruction

Fig. 20 Shaved proximal phalangeal head t distal pha-

lanx on a postoperative radiograph

a good-sized thumb with good IP joint stability.

However, it often develops complications of joint
stiffness, physeal growth disturbance, and nail-
plate deformity (Miura 1982; Tada et al. 1983;
Townsend et al. 1994). The original technique
was modied to reduce these complications
(Baek et al. 2007, 2008).
There is no absolute indication for modied
BC procedure. However, when both thumbs are
Fig. 21 Wassel type 4 radial polydactyly of an 11-month
old boy
hypoplastic and show almost symmetric appear-
ance, this procedure is indicated. Especially, when
the nail width is less than 2/3 of contralateral
normal side in unilateral cases, and when the nail
width is less than that of index nger in bilateral
15 Duplication 339

cases, this technique is recommended. This modi-

Fig. 22 The ulnar side thumb showed better bony devel-
opment, although there was 40 of angular deformity at the
IP joint
ed BC procedure is different from the originally
described method because it is an extra-articular
procedure; the IP joint is reconstructed with one
thumb and the other thumb contributes to only part
of the distal phalanx for stability (Figs. 39 and 40).
Both dorsal and volar incisions are necessary for
this procedure (Figs. 41 and 42). To prevent
so-called seagull deformity of the reconstructed
nail, the contour of nail bed can be manipulated.
For example, more round contour of the nail bed
can be achieved by bending two parts more volarly.
To make one smooth semicircular nail bed in the
transverse plane, slight volar axial rotation is
required (Fig. 43). Bony union between two distal
phalangeal parts usually occurred within several
months and rarely can take up to a year.
For Wassel type 4 polydactyly, the original
technique had been tried (Hartrampf et al. 1974;
Samson et al. 2004; Tonkin and Bulstrode 2007)
and the authors reported good alignment and good
joint stability. The central portions of two distal
phalanges as well as those of the two proximal
phalanges should be resected for classic BC oper-
ation in Wassel type 4. However, it is almost
impossible for phalangeal bones of bid thumbs

Fig. 23 Skin incision

340
Fig. 24 The EPL tendon to
the radial thumb was
detached
Fig. 25 The detached
tendon was sutured to the
ulnae side of ulnar thumb
to be mirror images. A step off between fused two
proximal phalangeal bones at the IP joint is inev-
itable when the MP joint was coapted congru-
ously. Otherwise, shaving of distal articular
cartilage or shortening of one proximal phalan-
geal bone at shaft level is necessary.
When two bisected proximal phalanges are
coapted, articular surface of proximal portion
will be congruous because the MP joint is more
important than IP joint functionally. There should
G.H. Baek
be length mismatch between two portions at the IP
joint level if two bones are not exactly the same
height. If distal articular portion of proximal
phalanx of dominant thumb is preserved and the
same part of proximal phalanx of shorter minor
thumb is removed, reconstructed thumb will
have a stable MP joint and mobile IP joint
(Fig. 44). Above modied BC technique is
applied to at the IP joint and classic BC technique
at the MP joint.
15 Duplication 341

Fig. 26 The FPL insertion

to the radial thumb was
identied and tagged with
suture for later reattachment

Fig. 27 Excessive portion

of metacarpal head on
which the removed radial
thumb sit was marked. A
ligamentoperiosteal ap
was raised from radial
proximal phalanx (left skin
hook)

Combination procedure 1-month-old boy showed Wassel type 2 thumb

Comparison of original BC procedure with modied BC polydactyly on left hand. The distal phalangeal
procedure epiphysis of the radial thumb showed abnormal
Original BC procedure Modied BC procedure triangular shape (Baek et al. 2006), while that of
Intra-articular procedure Extra-articular procedure ulnar thumb looked small and normal (Fig. 45).
Frequent IP joint stiffness Mobile IP joint Thus, the ulnar thumb was chosen to be the main
Possible epiphyseal plate No epiphyseal plate thumb of which most of the parts including IP
injury of distal phalanx injury of distal phalanx
joint would be preserved. The size of the nail of
Seagull deformity, common Seagull deformity,
uncommon
polydactylic thumbs was smaller than those of
index ngers (Fig. 46). Under tourniquet control,
Surgical Technique (Wassel Types 2, 3) (Baek the nail plates were removed. Then soft tissues
et al. 2007; Baek et al. 2008): A 2-year- and including skin and nail bed were removed along
342
Fig. 28 Excessive portion
of metacarpal head was
excised
Fig. 29 Neck of proximal
phalanx was exposed
subperiosteally for
corrective osteotomy
with the incision line. The bases of the two distal
phalanges were separated carefully. The main
articulating digit, the ulnar side in this case,
contained a major part of the distal phalangeal
bone with the overlying nail bed. The radial
minor thumb was made into a llet ap containing
only small extra-articular part of the distal phalan-
geal bone supporting the incised nail bed and the
collateral ligament attached to the proximal pha-
lanx. Articular facet of proximal phalanx for
minor radial thumb was shaved. The radial side
of the main digit tuft was also trimmed with a
G.H. Baek
small rongeur to better approximate with the
remaining portion of minor thumb.
The two distal phalangeal bones are approxi-
mated and maintained by 5-0 Nylon suture, one or
two transverse Kirchner wires, or a spinal needle
in a small thumb. The nail fold as well as nail bed
was repaired with 8-0 Nylon sutures (Fig. 47).
Removed nail was trimmed and reinserted into
the reconstructed nail fold for internal splint.
Two months after the operation, bony union was
observed between two portions of distal phalan-
ges and alignment was good (Fig. 48).
15 Duplication 343

Fig. 30 The osteotomy site

was xed with a K-wire.
Additional K-wire was
inserted into the MP joint
for healing of reattached
ligament and tendon. The
FPL tendon from removed
thumb was reattached to the
ulnar side of remaining
thumb

Fig. 31 The abductor

pollicis brevis tendon
detached from radial thumb
(right forcep) as well as the
previously raised
ligamentoperiosteal ap
(left forcep) were reinserted
into the base of proximal
phalanx

Three months after the operation, the new nail
grew well without deformity and IP joint motion
was good (Figs. 49 and 50).
In a Wassel type 3 polydactyly, all the proce-
dures are same as those of Wassel type 2 except
the minor thumb is osteotomized at its bifurcation
level. When there is more than 20 of angular
deformity at the IP joint, a closed wedge
osteotomy is performed at the proximal phalanx
of the retained thumb. One or two K-wires are
inserted to stabilize the osteotomy site. All the
K-wires are removed 46 weeks after the
operation, even if the bony bridge is not observed
between coapted distal phalanges because it will
eventually show bony union.
Surgical Technique (Wassel Type 4): A 1-year-
and 8-month-old boy showed Wassel type
4 thumb polydactyly on left hand (Fig. 51). Dor-
sal and volar skin incisions were designed
(Figs. 52 and 53). Soft tissues were removed
(Fig. 54), and central portion of bid proximal
phalanges were resected (Fig. 55). Two parts of
proximal phalanges were coapted using 4-0
Nylon to make MP joint congruously (Fig. 56).
344
Fig. 32 Postoperative photo
Fig. 33 Postoperative radiograph
The EPL and FPL were realigned as previously
described. For IP joint, same modied technique
as in Wassel type 2 was applied. After skin clo-
sure, removed nail was trimmed and reinserted
G.H. Baek
Fig. 34 A 12-month-old girl with Wassel type 7 polydac-
tyly. The ulnar triphalangeal thumb had delta middle pha-
lanx causing angular deformity
(Fig. 57). Three years after the operation, both IP
and MP joints looked congruous (Fig. 58).
Although 15 of extension lag at the IP joint
was noted and the size of left thumb was little
bigger than right normal side, exion arc of both
MP and IP joints was equal with that of normal
side (Figs. 59 and 60).
On-Top Plasty
In certain patients with radial polydactyly, one
thumb has well-developed proximal part and
poorly developed distal part with absent or hypo-
plastic nail. On the other hand, the other thumb
has poorly developed proximal part and better
distal part including nail and pulp. In this situa-
tion, better distal part of one thumb is transposed
to the better proximal part of the other thumb. The
transposed distal portion should have its
neurovascular bundle preserved during transposi-
tion. The location of feeding artery to the
15 Duplication 345

Fig. 35 Preoperative
medical photo

Fig. 36 The middle delta

bone was excised, and the
collateral ligament was
sutured in proper tension

Fig. 37 Three years after

the operation, alignment of
the thumb was straight
346 G.H. Baek

Fig. 38 Range of motion

was good

Fig. 39 Modied BC
procedure for Wassel type 2.
The colored area is resected,
and the two distal
phalangeal bones are
combined extra-articularly
to preserve IP joint motion
and to prevent epiphyseal
plate injury

transposed distal part can be traced by ultrasonog-
raphy. At least one artery must be preserved.
Surgical Technique: A 1-year- and 3-month-
old boy showed Wassel type 2 thumb polydactyly
on left hand and hypoplastic polydactyly
unclassiable by Wassel system on the right
hand (Fig. 61). Radial thumb of right hand had
good proximal phalanx and MP joint; however,
distal phalanx was invisible and nail was absent.
The ulnar thumb had good nail and pulp, but only
15 Duplication 347

Fig. 40 Modied BC
procedure for Wassel type 3.
The corrective osteotomy of
the proximal phalanx is
performed when there is
more than 20 of angular
deformity

Fig. 41 Dorsal incision for modied BC procedure of

Wassel type 2

Fig. 42 Volar incision for modied BC procedure of

Wassel type 2

had distal phalanx and remnant of small proximal

phalanx (Figs. 61 and 62). The nail and pulp of the
ulnar thumb was isolated like a neurovascular
bundle. Distal portion of radial thumb was dis-
sected and removed. Cartilaginous portion of dis-
tal phalanx was identied and preserved for later
fusion with transposed part. Along the ulnar
border of the radial thumb, a longitudinal incision
was made and deepened to embed the
neurovascular bundle to the transposed part.
A longitudinal K-wire was inserted to x trans-
posed part (Fig. 63). Adequate blood circulation
348 G.H. Baek

Fig. 43 Slight volar axial

rotation is required to make
one smooth semicircular
nail bed

Fig. 45 The distal phalangeal epiphysis of the radial

Fig. 44 Modied BC procedure for Wassel type 4. The
articular surface of the MP joint is adjusted rst after removal
of central portions of two proximal phalanges. There is length
discrepancy between two portions at the IP joint level if two
bones are not exactly the same height. If distal articular
portion of proximal phalanx of dominant thumb is preserved,
remaining procedure is the same with that of type 2
to the distal part was conrmed after tourniquet
release (Fig. 64). Three years after the operation,
the transposed part was growing well (Figs. 61 and
65). There was 45 of active exion at the MP joint
but no active motion at the IP joint (Figs. 66 and 67).
thumb showed abnormal triangular shape in this Wassel
type 2 polydactyly
Central Polydactyly
Central polydactyly is duplication within the
hand as opposed to duplication along the borders
of the hand such as of the thumb and little nger.
It includes duplications of index, long or ring
rays. The ring nger is most commonly involved,
followed by the long nger and the index nger.
15 Duplication
Fig. 46 The nail size of the
polydactylic thumbs was
smaller than those of index
ngers
Fig. 47 The nail bed was
repaired with 8-0 Nylon
sutures
Epidemiology
Over half of the patients with central polydactyly
had positive family histories for similar deformi-
ties, suggesting autosomal dominance inheritance
(Wood 1971). However, there was no family his-
tory in 12 patients with central polydactyly in
Japan (Tada et al. 1982). It may occur in high
hereditary predisposition, or sporadically. Associ-
ated musculoskeletal anomalies are cleft hand, sim-
ple or complex syndactyly, and polydactyly of toes.
349
In the review of 202 polydactylic digits from
144 patients at the University of Iowa, there were
41 digits of central polydactyly while there were
60 digits of ulnar polydactyly (Wood 1971). How-
ever, in the review of 238 patients with polydac-
tyly at Osaka University, there were 12 patients
with central polydactyly while there were
8 patients of ulnar polydactyly (Tada et al.
1982). Many authors reported the incidence of
central polydactyly was less common than that
of ulnar polydactyly (Wood 1971; Watt and
350 G.H. Baek

Chung 2009; Walters and Bae 2012). Central
polydactyly was more common than ulnar poly-
dactyly in Japan (Tada et al. 1982; Ogino
et al. 1986).
According to the IFSSH classication of congen-
ital hand anomalies, central polydactyly was classi-
ed as a member of duplication (Upton 1990).
Some authors proposed that central polydactyly,
syndactyly, and cleft hand be classied as abnormal
induction of nger rays (Ogino et al. 1986; Satake
et al. 2009). Theses proponents have observed bilat-
erally affected patients, in which cleft hand, central
polydactyly, and syndactyly of central nger rays
were also present in various combinations.

Classification

Stelling (1963) and Turek (1967) have classied

Fig. 48 Two months after the operation, bony union was
achieved
polydactyly into 3 main types. Type 1 is an extra
soft tissue mass not adherent to the skeleton. Type
2 is duplication of digit or part of digit that has
normal components and articulates with an
enlarged or bid metacarpal or phalanx. Type 3 is
rare and consists of a complete digit with its own
metacarpal and all the soft tissues involved.
This classication system was modied only
for central polydactyly (Graham and Ress 1998).
The essential elements of this classication sys-
tem for central polydactyly are three parts. In
type I, duplicated digits could be rudimentary
masses devoid of other tissue elements. In type
II, there is subtotal duplication with some normal
elements that typically articulate with a bid or

Fig. 49 Three months after

the operation, the new nail
grew well
15 Duplication
Fig. 50 The contour of the
nail was smooth
Fig. 51 Wassel type 4 polydactyly of left thumb
broad metacarpal. In type III, there is duplication
of the entire osteoarticular column including the
metacarpal, which results in a completely normal
extra digit.
Surgical Treatment
Type I central polydactyly that does not have bony
connection to the adjacent nger can be simply
operated with excision under general anesthesia.
351
The soft tissue pedicle usually contains
neurovascular structures. The vessels should be
ligated or cauterized, and the digital nerve should
be transected proximally and imbedded in the soft
tissue.
For type III central polydactyly, ray amputa-
tion is recommended. Reconstruction of web
space and repair of transverse metacarpal liga-
ment are important procedures during ray ampu-
tation. Sometimes, corrective osteotomy for
acceptable axial alignment is needed.
352 G.H. Baek

Fig. 52 Dorsal skin

incision

Fig. 53 Volar skin incision

Fig. 54 Soft tissues

including parts of nails were
removed
15 Duplication 353

Fig. 55 Resected central

portion of bid proximal
phalanges

Fig. 56 Coaptation of two

parts with Nylon suture.
Note the articular surfaces
of metacarpal head and
proximal phalanx (only
radial side is seen) met
congruously

Fig. 57 Postoperative
photo
354 G.H. Baek

Surgical outcome of type II central polydac-
tyly, especially complex synpolydactyly, is unsat-
isfactory in many cases. Clinical studies on
surgical outcomes of central polydactyly are
rare, likely related to the suboptimum outcome.
Tada et al. (1982) reported the outcome of
12 patients with central polydactyly. All the
patients required secondary operation for exion
contracture and/or angular deformity. Even after
the multiple surgeries, their surgical outcomes
were generally poor. Thus, ray amputation that
results in three ngers has been recommended
for this complex type II central polydactyly
(Graham and Ress 1998; Watt and Chung 2009;
Waters and Bae 2012, p. 43).
Clinical features of type II central polydactyly
are so diverse that it is impossible to describe all
surgical techniques in detail. Every case should be
individualized for surgical planning; however,
basic principles for reconstruction are the same
as for those of radial polydactyly-arthroplasty,
corrective osteotomy, and tendon realignment.

Central polydactyly
Surgical technique
Type I central polydactyly: simple excision
Type II central polydactyly: ray amputation or excision
and reconstruction
Type III central polydactyly: ray amputation

Surgical Technique (Type II): A 1-year and

Fig. 58 Both MP and IP joints locked congruous 3 years
after the operation
8-month-old boy showed bilateral type II poly-
dactylies of index ngers. Complete simple syn-
dactyly was associated at right rst web space and
left third web space (Fig. 68). Each index nger
had duplicated distal phalanges and one middle
phalanx of small delta bone with angular

Fig. 59 The size of the

reconstructed thumb was
slightly bigger than right
normal thumb, and about
15 of extension lag was
noted at the IP joint
15 Duplication
Fig. 60 Further exion of
the left thumb was normal
Fig. 61 Distal phalanx of radial thumb of right hand is not seen, and ulnar thumb was not connected to the radial one
deformity at distal interphalangeal (DIP) joint
(Fig. 69). On the radial side of index nger, a
racquet-shaped incision was made. The radial dis-
tal phalanx was excised after raising a ligamento-
periosteal ap that will be used for reconstruction
of the radial collateral ligament of the DIP joint.
An open wedge osteotomy was performed at the
delta middle phalanx, and bone chips harvested
from the excised phalanx were used for bone
355
graft. A longitudinal K-wire was inserted and
radial collateral ligament was reattached to the
radial side of the remaining distal phalanx. Four
months after the operation, rst web syndactyly of
right hand was released with double z-plasty, and
third web space of left hand was released with
division and full-thickness skin graft from groin.
Ten years after the operation, active range of
motion of MP and PIP joints of both index ngers
356
Fig. 62 The radial thumb showed better length and girth;
however, nail was absent. The ulnar thumb had nail and
pulp although it was oating type
Fig. 63 Distal part of ulnar thumb was transposed to the
radial thumb, like a neurovascular island ap
G.H. Baek
was good (Figs. 70 and 71), although DIP joints
showed bony ankylosis (Fig. 72).
Ulnar Polydactyly
Ulnar polydactyly, often called as postaxial poly-
dactyly, is a duplication of the little nger, ranging
from a small skin appendage on the ulnar aspect of
the hand to a well-formed ulnar digit articulating
with the fth or sixth metacarpal.
Epidemiology
In a prospective screening study of 11,161 new-
borns, the prevalence rate of type B ulnar poly-
dactyly was 1 in 143 live births of black infants
and 1 in 1,339 live births of white infants.
Seventy-six percent of the patients showed bilat-
eral involvement, and 86 % had a family history of
the anomaly (Watson and Hennrikus 1997). The
prevalence of ulnar polydactyly is not common in
Asian infants, even rarer than central polydactyly
(Tada et al. 1982; Ogino et al. 1986).
In African-American infants, most of ulnar
polydactyly occur as an isolated pattern. How-
ever, in Caucasians, it is often associated with
considerable systemic anomalies (Watson and
Hennrikus 1997). Inheritance pattern is autosomal
dominant with incomplete penetrance in most
patients. Autosomal recessive inheritance has
been reported and it is often associated with
syndromic presentations (Radhakrishna
et al. 1997; Mollica et al. 1978; Merlob
et al. 1981).
Classification
The classication for polydactyly (Stelling 1963;
Turek 1967) can be applied to ulnar polydactyly
as well. Type 1 is an extra soft tissue mass not
adherent to the skeleton like a oating or pedun-
culated appendage. Type 2 is duplication of digit
or part of digit that has normal components and
articulates with an enlarged or bid metacarpal or
15 Duplication
Fig. 64 Circulation to the
transposed part was
conrmed after tourniquet
release
Fig. 65 Bony growth of the transposed part was observed
phalanx. Type 3 is a complete digit with its own
metacarpal and all the soft tissues involved.
However, the classication by Temtamy and
McKusick (1978) is more commonly used. Type
A is a well-formed duplication of digit articulating
with the fth or sixth metacarpal. Type B is a
357
rudimentary underdeveloped digit attached to the
ulnar border of the hand by a skin bridge including
neurovascular bundle.
Surgical Treatment
Type B ulnar polydactyly can be treated with
suture ligation in the outpatient department or in
the newborn nursery. The parents should be
advised of two important factors before the sur-
gery. One, there is a strong likelihood that a bump
will persist after the digit falls off. Two, there is a
small possibility of the child having discomfort
when placing the ulnar border of the hand against
a hard surface secondary to neuroma formation.
Surgical excision of type B polydactyly is
recommended when the extra digit is not
completely ligated or the appearance of the
bump is unsatisfactory (Abzug and Kozin 2013).
Residual bump after suture ligation was reported
in 43 % with an average diameter of 2 mm ranging
from 1 to 6 mm (Watson and Hennrikus 1997).
When surgical excision is indicated, identication
and high transection of the digital nerve to the
extra digit is important to prevent painful
neuroma.
For type A ulnar polydactyly, excision and
reconstruction technique is recommended in
most cases. When two digits sit on one metacarpal
head, the ligamentoperiosteal ap should be
358 G.H. Baek

Fig. 66 Transposed
portion grew well, and good
alignment was maintained
3 years after the operation

Fig. 67 There was 45 of

active exion at the MP
joint, although no motion at
the IP joint

Fig. 68 Bilateral type II

polydactylies of index
ngers with complete
simple syndactyly of right
rst web space and left third
web space
15 Duplication 359

Fig. 69 Each index nger

had duplicated distal
phalanges sharing one
middle delta phalanx

Fig. 70 Good alignment of

both index ngers and well
maintained web spaces
10 years after the operation

Fig. 71 There was no

active motion at the DIP
joints, but the PIP and MP
joints showed good range of
motion
360
Fig. 72 Middle phalanges
of both index nger were
short and their physes were
not observed
Fig. 73 Type A ulnar
polydactyly of right hand
raised from the MP joint of extra digit and also
insertion of abductor digiti minimi (ADM) should
be detached carefully. These ligamentoperiosteal
ap and detached ADM tendon will be reattached
to the base of the fth proximal phalanx of the
preserved ray.
Ulnar polydactyly
Surgical technique
Type A: excision and reconstruction
Type B: ligation or excision
G.H. Baek
Surgical Technique (Type A): A 1-year- and
2-month-old girl showed a type A ulnar polydac-
tyly of right hand (Fig. 73). Her right fourth toe
showed symphalangism of interphalangeal joints
with hypoplasia of the distal phalanx. Bony hypo-
plasia of both duplicated little ngers was
observed in simple radiographs, although radial
fth nger showed full range of active motion at
MP and IP joints (Fig. 74). An elliptical incision
was made around the ulnar polydactylic digit
(Fig. 75). Digital vessels to the sixth digit were
15 Duplication
identied and cauterized (Fig. 76). The digital
nerve was also dissected and transected high
(Fig. 77). Duplicated exor and extensor tendon
to sixth digit were identied and transected
(Figs. 78 and 79). Passive exion and extension
of the fth nger could be checked by pulling
these tendons. Realignment of the tendons is
Fig. 74 Bony hypoplasia was observed from both dupli-
cated little ngers although radial fth nger had full range
of active motion
Fig. 75 Skin incision
361
seldom necessary in reconstruction of type A
ulnar polydactyly. The ADM tendon and ulnar
collateral ligament of MP joint, which inserted at
sixth proximal phalangeal base was raised
(Fig. 80). After excision of sixth nger, a longitu-
dinal K-wire was inserted into the MP joint. Then
the ADM tendon and ligamentoperiosteal ap was
attached to the proximal phalangeal base of fth
nger (Fig. 81). Rotational deformity of the
reconstructed fth nger was checked (Fig. 82).
Sometimes, corrective osteotomy for angular
deformity and/or rotational deformity of the fth
nger is needed.
Mirror Hand
Terminology
Mirror hand (Fig. 83), commonly called ulnar
dimelia, is a rare congenital difference character-
ized by duplication of the ulna, ulnar carpal bones,
and ulnar-sided ngers and absence of the thumb
and radius. There are usually seven ngers with a
central long nger and lesser ngers descending
from either side of it. Number of ngers varies
from six to eight.
Several types of mirror hand variants were
reported. A boy with eight digits and normal
362
Fig. 76 Digital vessels to
the removed nger was
cauterized
Fig. 77 Digital nerve was identied and cut proximally
radius and ulna was reported (Yang et al. 1996). A
baby with eight digits and an ulna and a hypoplas-
tic radius was reported (Al-Qattan et al. 1998). A
boy with seven digits, fused two hypoplastic radii,
and one ulna was reported (Bhaskaranand
et al. 2003). Thus, there were mirror hand patients
who had either normal, hypoplastic, or fused
radius. Ulnar dimelia is not a correct description
G.H. Baek
for these patients, because they have only one
ulna. To avoid some confusion, this condition
could be called thumbless mirror hand.
In the literatures review by Yang et al. (1996),
there had been six reported cases of multiple
hand deformity without the mirrored symmetry
in the hand and forearm in these cases; there
was complete duplication of the hand
including thumb. This deformity is called
multiple hand.
A case of upper limb triplication with radial
dimelia was reported (Mennen et al. 1997). There
were two left upper extremities, and the left upper
limb had two radii, one ulna, and ten digits includ-
ing two thumbs. Two little ngers formed syndac-
tyly sharing common metacarpal and proximal
phalanx. Ulnar thumb was a hypoplastic oating
type. This condition can be called as mirror hand
with thumbs.
Epidemiology and Classification
Only 60 cases of mirror hand have been reported
since its original description in the sixteenth cen-
tury (Wood 1993). Since 1993, 14 cases of mirror
hand have been reported from PubMed search as
of August, 2013.
15 Duplication
Fig. 78 Flexor tendon to
the sixth nger was cut at its
bifurcation site
Fig. 79 Extensor tendon
was cut at its bifurcation site
Al-Qattan et al. (1998) classied mirror
hand multiple hand spectrum into ve types.
Type 1 is a classic mirror hand which has two
ulnae and multiple ngers. Type 2 shows
multiple ngers with two ulnae (one of the ulnae
is vestigial) and a radius. Type 3 shows multiple
ngers with one ulna and a radius. Type 4 is
syndromic mirror hand, such as Sandrow syn-
drome or Martin syndrome. Type 5 is multiple
363
hand that shows complete duplication of the
hand including the thumb with a normal
forearm. This classication system is useful to
understand classic mirror hand deformity and its
variations. However, there are cases that cannot
be classied by this system. For example, this
patient with two well-developed ulnae (none of
them was vestigial) and a hypoplastic radius
(Fig. 84).
364 G.H. Baek

Fig. 80 The ADM tendon

and ulnar collateral
ligament of MP joint was
raised from sixth proximal
phalanx

Fig. 81 Then they were

attached to the proximal
phalanx of fth nger

Surgical Treatment et al. 2013). Because of its rarity, most surgeons

depend on the literature for surgical planning.
A report of four cases of mirror hand deformity is In some patients with mirror hand, shoulder
the largest series ever reported (Al-Qattan motions are limited and bony hypoplasia is
15 Duplication
Fig. 82 Rotational
deformity was checked
before skin closure
Fig. 83 A mirror hand
with eight ngers
present (Jafari and Shari 2005). The elbow
motions, both exion-extension and rotation, are
usually limited because two ulnae abut each other.
Proximal portion of radial ulna can be resected
to improve the elbow motion. Tsuyuguchi and
colleagues reported a patient that required repeat
surgery due to excessive new bone formation after
excision. Eight months after the second operation,
the elbow motion was 40/105 of exion, 30
pronation, and 50 supination (Tsuyuguchi et al.
1982).
365
In the wrist and forearm, the extensor muscles
are decient or absent (Barton et al. 1986a). Thus,
active extension of the wrist is impossible, and the
hand is usually held in exion and radial devia-
tion. A wrist exor can be transferred dorsally to
improve the exion deformity of the wrist. Most
authors recommended pollicization as the primary
procedure. From the three radial ngers, the digit
that shows the best motion, stability, and appear-
ance is chosen for pollicization (Barton
et al. 1986b). Alternatively, amputation of the
366
Fig. 84 Two
well-developed ulnae and
one hypoplastic radius were
observed in this patient.
Who had a pollicization
procedure before
radial ngers and keeping one of them in place
without pollicization has been reported. If
required, a secondary operation to rotate the new
thumb into pronated position can be performed
(Al-Qattan et al. 2013).
Summary
Polydactyly of the thumb show wide spectrum of
clinical manifestations from a pedunculated type
to complex types like triplicated thumb or
divergent-convergent Wassel type 4. Ligation in
the outpatient department or excision under local
or general anesthesia is indicated for pedunculated
types of radial polydactyly. Simple excision under
general anesthesia is indicated when there is no
bony connection between two polydactylic
thumbs and the dominant thumb shows good sta-
bility and appearance. Excision and reconstruc-
tion technique is the most common procedure
used for radial polydactyly. The main principles
are arthroplasty, corrective osteotomy, and tendon
realignment. The original Bilhaut-Cloquet proce-
dure had several drawbacks, such as joint stiff-
ness, physeal growth disturbance, and nail-plate
deformity. Two modications of the original
G.H. Baek
Bilhaut-Cloquet techniques have been introduced
to overcome the drawbacks of the original proce-
dure. One modied technique is for Wassel type
2 and the other modication for Wassel type
4. On-top plasty is indicated when one thumb
has well-developed proximal part and poorly
developed distal part and the other thumb has
poorly developed proximal part and better distal
part including nail and pulp.
Central polydactyly includes duplications of
index, long or ring rays. Surgical outcome of
complex types of central polydactyly are unsatis-
factory in many cases. Most of the ulnar polydac-
tyly can be successfully treated by ligation, simple
excision, or excision and reconstruction.
The mirror hand deformity is rare and most
surgeons should rely on the literature and their
experience for surgical planning. Ablation and
pollicization are commonly recommended.
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 Macrodactyly
16
Amit Gupta and Charity S. Burke

Contents Abstract
Introduction to Macrodactyly . . . . . . . . . . . . . . . . . . . . . 369 Macrodactyly is an extremely rare congenital
anomaly causing either static or progressive
Pathoanatomy and Applied Anatomy Relating
to Macrodactyly . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 371
overgrowth. This overgrowth can involve a sin-
gle digit or an entire extremity. Macrodactyly
Assessment of Macrodactyly . . . . . . . . . . . . . . . . . . . . . . . 371 can be an isolated phenomenon or part of a
Treatment Options . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 378 syndrome. The exact cause of this overgrowth
Nonoperative Management of Macrodactyly . . . . . . . 378 remains unknown, but there are continued stud-
Operative Management of Macrodactyly . . . . . . . . . . . 379
Nerve Decompression . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 380
ies at the molecular level to determine a com-
Epiphysiodesis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 380 mon pathway to link all types of macrodactyly.
Debulking . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 380 The treatment options at this time are obser-
Shortening Osteotomies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 380 vation if the overgrowth is static and not causing
Phalangectomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 384
Amputation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 384
functional impairment, or surgical to try to
improve function and cosmesis. Splinting and
Conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 387
therapy may be attempted, but there is no evi-
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 387 dence to support their use. Surgical options
range from nerve decompression, epiphysio-
desis, debulking, terminalization, middle
phalangectomy, osteotomies, and amputation.
If the amputation involves the thumb, then
pollicization may be considered.
There is no standardized tool to evaluate sur-
Electronic supplementary material: The online version
gical outcomes for surgery treating macro-
of this chapter (doi:10.1007/978-1-4614-8515-5_16)
contains supplementary material, which is available dactyly. The condition is so rare, and varies so
to authorized users. Videos can also be accessed at widely from case to case, that it remains one of
http://www.springerimages.com/videos/978-1-4614-8513-1. the most challenging congenital hand anomalies
A. Gupta (*) that pediatric hand surgeons face.
Department of Orthopaedic Surgery, University of
Louisville, Louisville, KY, USA
e-mail: armhand@gmail.com
C.S. Burke
Introduction to Macrodactyly
Louisville Arm and Hand, Norton Orthopaedic Care,
Louisville, KY, USA Children are supposed to be born symmetrical, and
e-mail: charitysburke@gmail.com it is taken for granted that as they grow, their

# Springer Science+Business Media New York 2015 369

J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_16
370
corresponding limbs, ngers, and toes will match in
size. On rare occasions something goes wrong.
(Flatt 1994, p. 317)
Macrodactyly has been dened a multitude of
ways, but regardless of how inclusive or exclu-
sive the denition is made, it is still one of the
rarest of the upper extremity anomalies.
Macrodactyly is derived from the Greek word
macro meaning large and daktylos meaning
digit. Occasionally in the literature, the terms
megalodactyly, gigantism, macrodystrophia
lipomatosa, dactylomegaly, gargantuan, and
macrodactylia brolipomatosis will also be uti-
lized to describe this same entity. Flatt noted that
this enlargement of tissue may involve a single
digit, several digits, the whole limb, or even one
half of the body (Flatt 1994, p. 317). In 1969,
El-Shami proposed that true macrodactyly must
include enlargement of the skeleton and not sim-
ply the soft tissues (El-Shami 1969). This deni-
tion includes a spectrum of pathology including
lipomatous overgrowth or nerve territory-oriented
macrodactyly (NTOM); Klippel-Trenaunay-Weber
syndrome; congenital lipomatous overgrowth, vas-
cular malformations, and epidermal nevi (CLOVE
syndrome); hemihypertrophy/hemihyperplasia;
Maffucci syndrome; Ollier disease; Proteus syn-
drome; Parkes Weber syndrome; and neurobro-
matosis but excludes arteriovenous stulas,
lipomatosis, neurobromas, and neurilemmoma
of the bone.
The rst description of macrodactyly recorded
in the literature is from Germany in 1824 (Klein
1824). Over the next 50 years, there was a grow-
ing belief that this entity was closely connected
to nerves. It was noticed that the overgrowth
often followed the pattern of a peripheral nerve.
von Recklinghausens description of neurobro-
matosis in 1874 helped endorsed this dogma as
macrodactyly was sometimes also present in these
patients who had abnormal nerves (Recklinghaus
1982). Due to its rarity and diversity, there were
sparse discoveries concerning the exact cause for
this obscure phenomenon for the next 150 years.
Up until 1974, there were only approximately
300 cases of macrodactyly reported in the litera-
ture that included cases of both the hands and feet
and included all types (Kelikian 1974).
A. Gupta and C.S. Burke
There is ongoing research on the molecular
level to determine if there is some common
denominator of a receptor or signaling pathway
that triggers this cellular dysplasia. In 2012, Lau
et al. in Boston isolated pleiotrophin (PTN) in the
surgical specimens of patients treated for
macrodactyly. PTN is a heparin-binding growth
factor that regulates mitogenic function
(Lau et al. 2012). Around this same time in
Japan, Miura et al. isolated a mutation of the
natriuretic peptide receptor 2 gene in a family in
which there was macrodactyly involving their
feet along with tall stature and scoliosis for three
generations. Transgenic mice were then created
that showed similar nding as affected humans
that had this mutation. An increase in cyclic aden-
osine monophosphate (cAMP) in the cartilage of
the growth plates of these mice has lead to ongo-
ing research that might target NPR2 to help treat
macrodactyly in the future (Miura et al. 2012).
The most common type of macrodactyly is
Flatt type I, nerve territory-oriented macrodactyly
(NTOM), or lipobromatous hamartoma of nerve
(Flatt 1994; Kelikian 1974). It has a male predom-
inance of 3:2. Ninety percent of the time is unilat-
eral and there is no evidence of familial
inheritance (Kelikian 1974). This type of
macrodactyly is caused by a spontaneous genetic
mutation causing somatic activation of P13K/
AKT cell signaling pathway. This new break-
through was made in Texas by comparing the
genetic makeup of resected nerve tissue of a
patient and then comparing it to their blood
DNA and looking for differences. This mutation
was also found in the nerve tissue of ve other
affected individuals. This gene mutation seems to
activate E542K, H1047L, or J1047R, which lead
to a gain-of-function mutation. There is a hope
that this mutation can be targeted and used to treat
or prevent overgrowth in the future (Rios
et al. 2013).
Congenital upper extremity epidemiology
studies done in Japan, Australia, and the United
States have all concurred that macrodactyly is the
rarest of the recorded International Federation for
Societies for Surgery of the Hand (IFSSH) upper
extremity anomaly groups. These studies show
that it is, at most, only 1 % of all upper extremity
16 Macrodactyly
Table 1 Epidemiology of IFSSH overgrowth/macrodactyly
Author(s) Publication Country Percent
Ogino JHS 1986 Japan 0.5 %
et al.
Leung JHS 1982 Hong 0.5 %
et al. Kong
Flatt 1994 United 0.9 %
States
Giele et al. JHS 2001 Australia 0.8 %
anomalies seen by hand surgeons and possibly as
low as 0.5 %. When Lamb reported the compiled
data from seven clinics across the world in 1982,
he found that macrodactyly seemed to have no
predilection for any geographic region or ethnic
group (Lamb et al. 1982). According to Giele
et al. 2001, the incidence of macrodactyly
among the general population in Australia is
0.002 % (Table 1).
Pathoanatomy and Applied Anatomy
Relating to Macrodactyly
In the extremity affected by macrodactyly, the
radial side is more frequently affected than the
ulnar side coinciding with the median nerve
being involved 85 % of the time and the ulnar
nerve only 15 % (Flatt 1994). The soft tissue
involvement tends to be greater on the volar side
of the hand as well, but the nail and dorsal skin
may thicken. This imbalance may lead to hyper-
extension of the digit. For most types of
macrodactyly, the enlargement is more pro-
nounced distally. It is three times more common
for the macrodactyly to affect multiple digits
rather than a single digit alone. These affected
digits are beside each other and most commonly
are the index and middle nger (Wood 1993).
There has yet to be a report of macrodactyly
affecting two digits with a normal digit between
the two enlarged digits. If left to their own
devices, macrodactylous digits may begin to
show signs of vascular insufciency. The affected
phalanges show thickening of their cortex and
widening of their medullary canals as they ll
371
Number of children with macrodactyly/number of children
with abnormal extremities/number of children examined
5/943
2/326
26/2,758
5/509/257,430
with fatty marrow. Often, enlargement of the
bony skeleton does not proceed symmetrically,
leading to deviation of the digit either radially or
more commonly, in the ulnar direction (Flatt
1994).
With regard to NTOM, the perineurium is the
most abnormal component of the nerves affected.
There is also an increase in the number of axons
and the size of each individual axon (Rios
et al. 2013).
Other types of macrodactyly that are part of a
syndrome have a broad spectrum of
pathoanatomy. A multidisciplinary approach to
these patients should be undertaken to not only
care for their enlarged hand but also safely care for
the entire child (Table 2).
Assessment of Macrodactyly
With advancement in ultrasound techniques,
macrodactyly can sometimes be diagnosed in
utero (Yuksel et al. 2009), but typically is diag-
nosed at the time of birth by simple visualization
and comparison to surrounding digits or contra-
lateral unaffected digit. If the enlarged digit is not
part of a syndrome, but is rather an isolated entity,
the child must be monitored to see if the digit
(s) grows in proportion to the child or not. Usually
around the age of 23, the rate of growth, whether
it is a static or progressive macrodactyly, will
become evident by simple examination. The
child should be followed at set intervals during
these rst couple of years determined by the anx-
iety of the parents, the magnitude of the enlarge-
ment, and the rate of growth.
 372

Table 2 Comparison of overgrowth syndromes

Klippel-
Trnaunay Parkes Weber Lipomatous CLOVE Maffucci
syndrome syndrome overgrowth syndrome Hemihypertrophy Proteus syndrome syndrome
Capillary stain Present, deep Present, pink, Rare Present, pink, Uncommon Present, pink, often Absent (rarely
purple often diffuse diffuse, truncal diffuse reported)
Progressive Present Present Present, Present, often Either overgrowth or Absent at birth, Secondary to
overgrowth symmetric extensive undergrowth haphazard, unrelenting, enchondroma
disproportional growth, moderate
to massive
Hemodynamics Slow-ow Fast-ow Slow-ow Slow-ow Slow-ow Slow-ow Slow-ow
Vascular VM, LVM AVM, AVF Absent VM, LVM, LM Absent VM, LM, CM VM within bone,
anomalies hands common
Associated Common: GI, Rare, Cobb Absent No visceral Skin, CNS, heart, GU, Cerebriform soles/ None
anomalies GU, genitalia syndrome dental, others palms, linear nevi,
dysregulated fat, lung
cysts, facial neoplasms
Limb Moderate, Arm, leg length Moderate, nerve Moderate Macrodactyly, Major, asymmetrical Disproportionate
enlargement disproportionate discrepancy territory- syndactyly,
digits and toes oriented polydactyly, club feet
common
Limb affected Upper 5 %, Upper 23 %, Equal Equal Arms, hands equally Arms/legs, hands/feet All
lower 95 % lower 77 % equal
Skeletal Unusual, can Common, direct Common, Marked Hip dysplasia, Skull hyperostosis, Gross distortion,
changes occur with large involvement and symmetric scoliosis, lower scoliosis, increased vertebral asymmetry, limb
lesions demineralization limb bone age megaspondylodysplasia length
discrepancy,
scoliosis, short
stature, fractures
A. Gupta and C.S. Burke
Macrodactyly Diffuse Minimal to Moderate to Diffuse or longer Digits longer with Asymmetrical, Digits longer
16

enlargement moderate gigantic with exion exion or extension moderate to gigantic with asymmetric
contractures contractures masses, increased
size due to tumors
Dysregulated Present within Present Common, entire Present Multiple lipomas Lipomas, regional fat Present
fat malformation limb and axilla absence occasionally
Macrodactyly

Nerve Absent Absent Intermittent Intermittent Absent, compression Absent Absent

involvement neuropathies with
contractures
Muscle Displaced by Displaced by Absent Absent Common, atavistic Absent None
anomalies VM, LVM malformation intrinsic and extrinsic
muscles
Associated Absent Absent Absent Absent Renal, adrenal and Ovarian, parotid Chondrosarcoma
neoplasms CNS (17.530 %),
many others les
frequently
(ovarian, CNS)
Coagulopathy Present Normal Normal Normal Normal Normal None
Cardiac failure No Yes No No No No No
Clinical Stable Often progressive Good, related to Progressive Progressive exion, Progressive growth of Good with local
prognosis deterioration, steal size and weight deterioration extension and affected regions therapy; Ollier
phenomenon. of limb with extensive intrinsic contractures disease patients
Prognosis is worse limb demonstrate
with CHF or involvement increased
amputation problems
(continued)
373
 374

Table 2 (continued)
Klippel-
Trnaunay Parkes Weber Lipomatous CLOVE Maffucci
syndrome syndrome overgrowth syndrome Hemihypertrophy Proteus syndrome syndrome
Risks DVT risk Infection, CHF, Secondary Scoliosis, Neoplasms Neoplasms, PE, Pathologic
increased post op gangrene arthritis pulmonary (pheochromocytoma, depression fractures
compromise Wilms tumor,
hepatoblastoma)
Genetics Unknown Unknown Unknown Unknown Unknown Unknown Unknown

Abbreviations: AVM arteriovenous malformation, CHF congestive heart failure, CM capillary malformation, CNS central nervous system, DVT deep vein thrombosis,
GI gastrointestinal, GU genitourinary, LM lymphatic malformation, LVM lymphaticovenous malformation, PE pulmonary embolism, VM vascular malformations
(Reprinted from Hand Clinics, 25, Kevin C. Chung, Matthew J. Carty, Amir Taghinia, Joseph Upton, Overgrowth Conditions: A Diagnostic and Therapeutic Conundrum,
pp. 229245. Copyright (2009), with permission from Elsevier)
A. Gupta and C.S. Burke
16 Macrodactyly
The child should be watched playing and
performing ne and gross motor activities with
the arm and hand usually best accomplished with
strategic bribery involving toys, stickers, and/or
suckers. It should be noted whether the child uses
the enlarged digit(s) or bypasses them entirely.
During examination of the hand in these children,
it is important to realize that overgrowth is not
limited to a single digit or there is circumferential
equal involvement even in a single digit. For
example, the ulnar aspect of the index nger and
radial portion of the middle nger may be
enlarged and growing and deviating away from
each other. This overgrowth will often extend into
the palm and even up as high as the axilla. The fat
distribution in the nger, palm, forearm, and arm
is rarely circumferential but rather concentrated
near the affected nerve and usually more severe
distally. The shoulder, elbow, wrist, and all digits
should be taken through a full active and passive
range of motion to check for any loss of motion or
scissoring of the digits.
The enlargement of the nerve and surrounding
fat places the child at high risk for compressive
neuropathies at the carpal, cubital, and/or radial
tunnel. It is difcult to diagnose peripheral neu-
ropathies in children. Wounds from chewing,
biting, disuse, or ignoring the digit can all be
signs that the child has lost sensation in the
affected digit. The wrinkling test may prove use-
ful if the child is amenable. The test requires
warmwater submersion of the digit for 5 min to
assess for skin wrinkling. Lack of wrinklings is
indicative of loss of sympathetic innervation
and nerve innervation. Nerve conduction studies
and electromyography can be helpful to diagno-
sis and/or conrm peripheral neuropathies.
Electrodiagnostic studies in children may require
sedation, although oversedation will affect
the study quality. Infants under 1 year of age
can usually be tested without any sedation
(or sedation only for the parents), while the
2- to 6-year-old children are the most likely to
need mild sedation. A patient-savvy neurologist
may succeed without pharmacologic aide by rst
doing the nerve conduction study and then elec-
tromyography using single ber stimulation
techniques (Hays et al. 1993).
375
Serial radiographs should be taken to monitor
bony growth to help provide information
concerning angular deformities. Serial studies
also help guide the timing of epiphysiodesis. The
same-sex parent should be examined and even
radiographed if needed to provide a reference for
the approximate nal desired size of the digit for
the child.
Magnetic resonance images can be useful for
cases in which there is some component of vascu-
lar malformation in the affected limb.
When examining macrodactyly that is part of a
syndrome such as Maffucci syndrome or neuro-
bromatosis, the physician must have a heightened
awareness of the possibility of malignant transfor-
mation of a tumor of the hand or other body part.
A substantial increase in pain may be the harbin-
ger of the transformation of a plexiform neuro-
broma to a neurobrosarcoma, or a change in a
skin lesion in Maffucci syndrome may signal the
sarcomatous degeneration of a hemangioma or
lymphangioma.
Other than grouping the types of overgrowth,
currently there is no classication system that can
guide treatment algorithms and compare treat-
ment outcomes.
The three most accepted classication systems
for overgrowth were published by De Laurenzi,
Temtamy and McKusick, and Flatt, which divided
digit overgrowth into groups based on growth
rate, syndromal distinction, and disease process,
respectively. Currently, Flatts classication with
Uptons modication is the most widely used
when discussing macrodactyly.
Feriz, Werthemann, and Laurenzi recognized
that not all large digits in the newborn grew at the
same rate or for the same length of time. They
were able to identify static macrodactyly or
macrodactylia simplex congenital and differenti-
ate it from the progressive macrodactyly or
macrodystrophia lipomatosa progressiva (Feriz
1925; Werthemann 1952).
Temtamy and McKusick recognized that
macrodactyly could be part of a syndrome or an
isolated anomaly. They further subdivided iso-
lated macrodactyly into true macrodactyly or
pseudomacrodactyly. The authors declared that
macrodactyly caused by hemangiomas, soft tissue
376 A. Gupta and C.S. Burke

tumor, edema from constriction band syndrome, Type I is gigantism and lipobromatosis. This
arteriovenous aneurysms, or stulas lead to soft type is equivalent to Kelikians nerve territory-
tissue increase in size. However, these etiologies oriented macrodactyly (NTOM). This group is
do not cause bone enlargement and therefore are further divided into static and progressive forms
not truly macrodactyly, hence the term pseudoma- that were noted in earlier publications (Figs. 1
crodactyly. They recognized partial gigantism, and 2).
neurobromatosis, Ollier disease, Maffucci syn- Gigantism and neurobromatosis comprise
drome, Klippel-Trenaunay-Weber syndrome, and type II. Neurobromatosis is an autosomal domi-
congenital lymphedema as all syndromes that nant trait, but 50 % of cases are new mutations
could have macrodactyly as part of the spectrum (Temtamy and McKusick 1978). Patients present
of anomalies (Temtamy and McKusick 1978; with classic caf au lait spots numbering greater
Table 3). than six, molluscum brosum, and multiple neu-
Flatt devised a classication system that robromas on peripheral nerves. Plexiform neu-
divided macrodactyly into three types (Table 4). robromas often lead to macrodactyly along with
osteochondral masses arising from the physis of
the metacarpals and phalanges.
Table 3 Classification of macrodactyly
Type III is rare and is known as gigantism and
Author (s) Publication Classication criteria digital hyperostosis. Kelikian previously had
De G Med Milan Growth rate called this exostotic digital gigantism. The nerves
Laurenzi 1962
in these affected digits appear normal, but
Temtamy March of Dimes. Syndromal or
and Birth Defects nonsyndromal strangely enough, the pattern of overgrowth still
McKusick 1978 tends to favor median nerve distribution. The
Flatt The Care of Disease processes digits do not just grow longer and larger, they
Congenital Hand types IIII, type IV tend to become nodular and deformed. Unlike
Anomalies 1994 modication added
by Upton (2005)
other macrodactyly, which tends to most severely
affect distally, the distal phalanx is typically

Table 4 Flatts classification system of macrodactyly

Type Name Cause Features
I Gigantism and Mutation causing somatic activation Male/female ratio 3:2
lipobromatosis of P13K/AKT cell signaling pathway 810 % syndactyly rate
Follows peripheral nerve/nerve territory-
oriented macrodactyly
Fatty inltrates noted in affected nerves
II Gigantism and Neurobromatosis/plexiform Autosomal dominant inheritance
neurobromatosis neurobroma May be bilateral
May have large osteochondral masses near the
epiphysis of both the phalanges and metacarpals
III Gigantism and Unknown No hereditary pattern known
digital Bilateral involvement but asymmetric
hyperostosis Badly misshapen ngers
IV Gigantism and Unknown No inheritance pattern known
hemihypertrophy Hands may not get as large as other types
Flexion contractures of ngers
Thumb adduction
Ulnar drift of ngers
Abnormal extrinsic and intrinsic insertions
16 Macrodactyly
Fig. 1 Dorsal view of
static NTOM involving the
long nger
Fig. 2 Volar view of static
NTOM involving the long
nger
spared in type III. Hypertrophic cartilage may
be found along with osteochondral masses.
Interestingly, this syndrome can also include
hemihypertrophy and elbow problems. Radial
head dysplasia and subluxation along with loose
bodies in the elbow are often found. There can be
striking loss of range of motion with this type of
macrodactyly. This is especially evident at
puberty when there is a sudden increase in the
size of periarticular osteochondral masses that
results in a mechanical block to motion.
377
Upton modied Flatts original classication
system to add a fourth type known as gigantism
and hemihypertrophy (Upton 1990). The enlarge-
ment of the digits tends to be less severe than that of
NTOM or neurobromatosis, but the children tend
to develop exion contractures especially of the
metacarpophalangeal joints. Ulnar deviation of
the digits is also common. Abnormal muscle ori-
gins, insertions, and intrinsic muscle hypertrophy
lead to noticeable enlargement of the thenar and
hypothenar eminences. A thumb adduction
378 A. Gupta and C.S. Burke

Fig. 3 Examining a childs hand function with

macrodactyly after middle nger ray resection. Attention
is particularly paid to the index nger which is also affected
and not being used

contracture develops in adolescence. Although the

hand may not be as impressively large in this type of Fig. 4 Hemihypertrophy
macrodactyly, the proximal forearm and arm may
become massively enlarged (Figs. 3 and 4).
and 2 patients requested amputation (poor result).
There is also no standard outcome tool to com-
There were no reported functional outcomes for
pare the outcomes of treatment to different surgi- these patients such as range of motion or hand
cal options other than simple measurements of the
capabilities (Kotwall and Farooque 1998).
digit size and range of motion. Akinci et al. (2004)
Functional outcome tools such as the
study of seven digits treated with a debulking (the ABILHAND-Kids, Quality of Upper Extremity
concave side of the digit was preserved on its
Skills Test (QUEST), the Melbourne Unilateral
neurovascular bundle and appropriate soft tissue
Upper Limb Function (MUUL), and the Pediatric
removal from the opposing side) found that the Outcomes Data Collection Instrument (PODCI)
average reduction in circumference of the ngers
may be utilized in the future to compare outcomes.
were 44 % and 35 % of length. The two thumbs of
These measures have shown promise in children
the study were reduced in size by an average of with cerebral palsy and other upper extremity abnor-
37 % in circumference and 15 % in length. Range
malities (Kunkel et al. 2011). Their applicability to
of motion and two-point discrimination were
children with macrodactyly has yet to be shown.
found to be the same or nearly the same as before
surgery (Akinci et al. 2004). Kotwall et al. in 1998
in a series of 21 ngers and toes classied a good Treatment Options
operative result following debulkings to be a
reduction in digit size by 50 % or more, a satis- Nonoperative Management
factory result as 2550 % but with angular defor- of Macrodactyly
mity, and a poor result as a digit that was
cosmetically unacceptable requiring amputation. Nonoperative management of static NTOM may
The results were humbling with 12 patients be acceptable if the digit does not impede
graded as good, 7 patients deemed satisfactory, hand function due to bulk or overlap
16 Macrodactyly 379

(Table 5; Video 1). Nonoperative management upon a static digit compared to progressive
may be best while trying to delineate progressive macrodactyly. The same-sex parents same digit
from static NTOM in the rst couple of years of should be used as a guide for ultimate expected
life. Compressive gloves and garments have size of the childs nger.
been suggested to attempt to retard growth Treatment of progressive NTOM or macro-
driven by vascular issues, but no published evi- dactyly associated with hemihypertrophy or
dence supports this treatment. There has been no other syndromes is extremely challenging. Due
success shown with radiation therapy or any to the rare nature of the problem and the great
medical treatments to date. variety of its presentation, namely, the growth rate
and extent of involvement, every case must
receive individualized treatment. A thorough
Operative Management
examination and detailed surgical planning is
of Macrodactyly
mandatory as the ultimate outcome is difcult to
predict and multiple procedures are necessary.
Treatment for decreasing digit bulk and length
Expectations of the patient and family must be
should be delayed until it becomes apparent
guarded as normalcy is unachievable. Basic prin-
whether the macrodactyly is static or progressive
ciples and techniques for treating the growing
if it is type I. More predictable outcomes can be
hand that is growing abnormally large are
expected if surgical intervention is performed
debulking, osteotomies, nerve resection with or
without grafting, epiphyseal arrest, excision of
osteochondromas, entire bone resection, decom-
Table 5 Nonoperative management of macrodactyly
pression of compressive neuropathies, terminaliza-
Indications Contraindications tion, and differing levels of amputation to maintain
Static NTOM with full Static NTOM with hand function (Table 6). Unfortunately, even
function scissoring, overlap, or
bulk limiting function amputation of the involved digit may not com-
Therapy/splinting Loss of range of motion pletely rid the child of their problems as neighbor-
due to osteochondromas, ing ngers and proximal tissues can be affected.
joint subluxation There have been numerous surgical treatments
Compression garments No evidence to support designed to deal with macrodactyly. There is still
vascular malformations/
no perfect surgery to combat this varied and
edema
difcult issue. In fact, effective treatment has

Video 1 Static NTOM

treated nonoperatively as
the patient has full range of
motion and full function of
the enlarged long nger
380 A. Gupta and C.S. Burke

Table 6 Types of surgeries for macrodactyly with wide exposures and utmost care due to the
Type of surgery Advantages Disadvantages enlarged nerves and aberrant anatomy.
Nerve Symptoms relief No change in
decompression disease
process Epiphysiodesis
Epiphysiodesis Short recovery Time sensitive
Debulking Decrease in girth Minimal
Epiphysiodesis is the most straightforward proce-
length
decrease dure for these children. The epiphysiodesis should
Scar tissue be performed at all phalanges and metacarpal
Possible when the childs nger reaches the same length
vascular insult as the same-sex parents digit. Epiphysiodesis can
Terminalization Short recovery Loss of nail/ be accomplished through drilling, burring, or an
poor aesthetic osteotome.
result
Middle Maintained nail Joint
phalangectomy and joint reconstruction
Osteotomy Correct angulation Possible Debulking
nonunion
Resection of Better able to Loss of joint Soft tissue debulking procedures can be done
portion of correct length at function alone or at the time of epiphysiodesis or
phalanges multiple levels Scar tissue osteotomy. There are multiple approaches and
Recovery time
techniques, but common principles include
Amputation Recovery time Aesthetic
concerns
debulking only one side of a digit at a time and
Loss of length allowing 36 months between surgeries. Tech-
niques that include a unilateral soft tissue resec-
tion include those described by Bertilli, Fujita,
Hoshi, Akinci, and Ogino. These procedures can
remained elusive and the ultimate outcome is fre- also include bony work to provide shortening. In
quently disappointing to the patient and the sur- 1974 Edgerton and Tuerk described complete
geon. All procedures risk viability of the digit and resection of one side of the enlarged digit includ-
usually induce additional stiffness. No surgery ing the skin. This technique was utilized to
cures the underlying disease process. Surpris- decrease the ap necrosis seen from other tech-
ingly, even resection of the affected nerves and niques (Edgerton and Tuerk 1974). Upton advo-
ligation of the blood vessels and other soft tissue cates aggressive debulking procedures prior to the
techniques have been rendered ineffective. Most child starting school to try to decrease social anx-
children routinely require multiple procedures and iety caused by a disturbingly enlarged digit (Carty
the results are still unpredictable. For this reason, 2009). Debulking can be done through a mid-axial
the molecular work discussed earlier to prevent or Bruner-style incision. The volar incision may
overgrowth is crucial. provide slightly better exposure; however, the
mid-axial scar is less likely to hypertrophy.

Nerve Decompression
Children with macrodactyly have a high rate of
compressive neuropathies that require decompres-
sion. Ongoing compression will lead to long-term
decits with altered sensation that is detrimental to
development of hand function. Carpal, cubital, and
radial tunnel syndrome should be decompressed
Shortening Osteotomies
The most accepted osteotomies involve fusing the
distal phalanx in its entirety onto a shortened
middle phalanx (Table 7). (Barsky 1967) was the
rst to describe this technique, while Flatt
advocated concomitant lateral debulking and
 16

Table 7 Surgical techniques to manage macrodactyly

Author Publication Technique Advantages Disadvantages
Macrodactyly

Barsky JBJS 1967 First stage distal middle phalanx resected and made conical, Preserved nail bed Loss of DIP joint
distal phalanx and nail fused to shortened middle phalanx with good blood
supply
Flatt The Care of the First stage distal middle phalanx resection, nail narrowing, FDP Loss of DIP joint
modication Congenital Hand shortening if >3 cm shortening and FDS compromised. Second
of Barsky Anomalies 1994 stage redundant dorsal skin excision
Fujita Keisei Geka 1983 Resection of tip of distal phalanx and unilateral soft tissue Maintained range Limited
resection Motion Shortening
Potential
Hoshi Keisei Geka 1973 Resection of distal aspect of middle phalanx and distal tip of distal Increased shortening Loss of DIP joint
phalanx with unilateral soft tissue resection
Rosenberg Hand 1983 Elevated nail bed on axial island ap Preserved nail bed Nail bed can enlarge itself when
transferred
Akinci JHS 2003 First procedure concave side neurovascular bundle and adequate Simplied surgical Loss of DIP joint
tissue preserved with resection of all other soft tissue, DIP joint planning Angulatory
fusion with shortening. Second procedure skin ap resection Deformities
Equired
Subsequent
Surgeries
Bertilli JHS [AM] 2001 Resection osteotomies of distal portion of proximal and middle Longitudinal and Loss of DIP joint
phalanx with resection of DIP joint and unilateral soft tissue transverse size
resection reduction
Ogino Congenital Resection of proximal phalanx, DIP joint osteotomy and fusion, Shortening at multiple Loss of DIP joint
Malformations of the distal phalanx bony tip resection along with unilateral soft tissue levels
Hand and Forearm 1998 resection
Tsuge Plast Reconstr Surg 1967 First stage tip plasty (dorsal composite ap of nail inset into Shortening at multiple Necrosis of dorsal ap
dorsal aspect of middle phalanx) and arrest of phalangeal physis at levels Safer for toes
all levels. Second stage dorsal skin excision
(continued)
381
Table 7 (continued)
382

Author Publication Technique Advantages Disadvantages

Millesi Symposium on Described for thumb Stiff IP joint
reconstructive hand Longitudinal nail ridge
surgery 1974 Transverse K-wires through
distal phalanx which may allow
continued transverse growth
Interosseous wires more reliable
Kotwall/Tan JBJS 1998/Scand J Plast Middle phalangectomy
Reconst Surg 2006;
40:362365
Edgerton and Symposium on Complete excision of skin and fat with debulking and full Need for full thickness Decreased ap necrosis from
Tuerk Reconstructive Hand thickness skin grafting skin graft long aps from contralateral side
Surgery 1974 Risk of skin graft of nger
necrosis
Jebson and Green Operative Hand Terminalization with amputation of the distal phalanx and Quick recovery Cosmetically noticeable due to
Louis Surgery, Fifth Edition surrounding soft tissue nail loss
2005 (pages 19481949)
Jebson and Green Operative Hand Ray amputation Most denitive Psychological coping with loss
Louis Surgery Fifth Edition treatment for single of digit
2005 (pages 19501964 digit
Quick recovery Decreased hand breath leading
to decrease in grip strength
Kozin Clin Orthop Surg 2012 Thumb amputation and pollicization Allows for large Need for healing of osteotomy
resection of affected Risks of percutaneous pin
digit(s)
Improved function of Need for physical therapy for
hand by creating retraining of digit pollicized
usable, opposable digit
Carty et al. Hand Clinics 2009 Thumb amputation and toe transfer Allows for large Risk of failed free tissue transfer
resection of affect
digit(s)
Improved hand Risks of percutaneous pin
function by creating Potential foot complications
usable, opposable digit Need for physical therapy for
retraining of toe
A. Gupta and C.S. Burke
16 Macrodactyly
Fig. 5 Barsky digital reduction. Skeletal reduction. (a)
One type of reduction of the massive ngertip involves
aggressive pulp resection, isolation of the remaining nail
complex including dorsal cortex of the distal phalanx as a
dorsal pedicle, and transfer on top of the middle phalanx.
(b) Another strategy involves bone resection, dorsal skin
and tendon resection, and transfer as a palmar pedicle. The
skin excess in both methods is removed secondarily. (c)
For massive thumb overgrowth, skeletal reduction can be
shortening of the exor digitorum profundus if the
shortening was greater than 3 cm and the exor
digitorum supercialis was compromised by
the osteotomy. This method of distal phalanx pre-
serves the nail germinal matrix but sacrices the
distal interphalangeal joint. See Fig. 5.
Tsuge developed an alternate technique to
accomplish the task of shortening the digit but
383
achieved with longitudinal, oblique, or central longitudinal
excisions. Motion at the interphalangeal joint is invariably
diminished or completely lost as these children grow and
develop secondary osteoarthritis (From Upton J. Failure of
differentiation and overgrowth. In: Mathes SJ, Hentz VR,
editors. Plastic Surgery. 2nd edition. Volume VIII: The
hand and upper limb, Part 2. Philadelphia: WB Saunders;
2005. pp. 301, 302, 305, 315; with permission)
saving the nail bed. The dorsal one-third of the
distal phalanx cortex along with the nail are trans-
posed onto the distal aspect of the middle phalanx
and the remainder of the distal phalanx is ampu-
tated (Tsuge 1985). See Fig. 5.
Rosenburg described a nail preserving tech-
nique by elevating the entire nail bed as an axial
island ap. This allows for bony resection as
384
Fig. 6 Patient 1. (a) Progressive macrodactyly of the right
thumb in a 12-year-old girl. Her thumb was 13 % longer
and 75 % larger in circumference than the opposite, and the
joints had very limited range of motion. (b) The slice taken
from the convex (radial) side includes the remnants of the
needed and placement of nail bed in desired loca-
tion (Rosenberg et al. 1983).
Millesi described an elegant debulking and
osteotomy for thumb preservation. The procedure
requires a longitudinal osteotomy of the distal pha-
lanx that decreases girth but also creates a longitu-
dinal nail crease much like a Bilhaut-Cloquet
procedure. The proximal phalanx is shortened and
decreased in width by a transverse osteotomy. The
nail is decreased in length along with the pulp by
distal resection (Millesi 1974; Fig. 6).
Techniques by Bertilli, Fujita, Hoshi, Akinci,
and Ogino all rely on unilateral debulking in com-
bination with shortening osteotomies at one or
more levels. Many methods utilize Barsky or
Tsuges distal techniques. Authors of these
methods suggest measuring the contralateral
digit and nail bed prior to debulking of the
affected digit to utilize it as a template. The nail
can also be partially resected, leaving a more
proper-sized nail with reconstruction of the lateral
nail folds with local tissue as described by Golash
(Golash and Watson 2000).
Phalangectomy
Middle phalangectomy has been described by
Kotwall and Tan. This procedure relies on
A. Gupta and C.S. Burke
nail, nail plate, and phalanges and the neurovascular bun-
dle in one piece, and the remaining tissues and phalanx
were left on the concave side with the intact neurovascular
bundle to form a thumb looking like the opposite (Akinci
et al. 2004b, p 1011)
complete removal of the bone with suturing of
the joint capsule from the distal phalanx to the
proximal phalanx and pin stabilization for 46
weeks. This bone is removed as part of the second
debulking procedure. The authors did not report
range of motion at the newly created join. They
emphasized that the patients did not have good
preoperative motion and that aesthetic improve-
ment is their aim. The authors caution to only
remove about 1020 % of the fat with each
debulking to ensure leaving enough fat with the
skin to prevent necrosis, which occurred in two
ngers, did have skin necrosis that healed with
dressing changes (Kotwall 1998; Tan 2006;
Figs. 7 and 8).
Amputation
Amputation of the digit can occur through the
distal phalanx and is known as terminalization
(see previous discussion). Although aesthetically
not as pleasing as some of the more elaborate
procedures, terminalization is effective and has a
short recovery time. The procedure decreases the
length, but not circumference.
Amputation through the middle or proximal
phalanx is an option but is rarely utilized since
ray resections offer increased appearance and
16 Macrodactyly 385

Fig. 7 Patient
3. Presurgical planning for
the middle nger. (a) A ap
is prepared from the nearby
eponychium to use during
the closure of the wound of
the shortened and narrowed
nail. (b) Palmar view of the
incisions (Akinci
et al. 2004b, p 1014)

Fig. 8 Technique of bone shortening with removal of a

whole middle phalanx (Copyright JBJS Kotwall 1998)

Fig. 9 Ray amputation of middle/long nger

function in the majority of cases. A ray amputa-

tion done for the index nger will also increase the
thumb-index web space. Middle and ring nger
ray amputations may require adjacent digit trans-
position to close the defect and improve appear-
ance (Fig. 9).
For thumb amputations, there has been recent
work to promote pollicization or even possible
toe-to-thumb transfers in this setting to regain
function (Kozin 2012; Upton 2009). These
techniques are surgically demanding but have
added to the ever-growing list of tactics utilized
for this challenging problem.
Ray amputations often provide the most expe-
dited recovery and deliver excellent aesthetic out-
come and functional results with the least amount
of surgeries. Ray amputations should always be
considered a valid alternative to salvage (Fig. 10).
386 A. Gupta and C.S. Burke

Fig. 10 (continued)
16 Macrodactyly
Fig. 10 Amputation and pollicization for macrodactyly (Provided by Dr. Scott Kozin, Philadelphia Childrens Shriners
Hospital)
Conclusion
Macrodactyly is a rare phenomenon that is slowly
being unraveled from a molecular and genetics
standpoint. Future treatment will be directed at
the underlying molecular and/or genetic problem.
Until that time, there will remain a multitude of
surgical techniques to salvage these overgrown
digits. There are no valid comparison studies to
prove superiority of any given procedure. Macro-
dactyly associated with a syndrome requires multi-
disciplinary management.
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 Thumb Hypoplasia
17
Scott H. Kozin and Dan A. Zlotolow

Contents Abstract
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 389 Thumb hypoplasia may be an isolated nding
or associated with varying degrees of radius
Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 390
hypoplasia. The classication of thumb hypo-
Classication/Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . 391 plasia is relatively straightforward, and the
Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 393 treatment paradigm is well dened. Further-
Thumb Reconstruction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 394 more, the outcome is predictable as long as
Pollicization . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 397 the principles of management are followed. In
Outcome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 404 contrast, the treatment of radius hypoplasia
Thumb Reconstruction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 404 remains difcult, and obtaining a consistent
Pollicization . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 404
favorable outcome is elusive as detailed in the
Complications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 410 chapter covering radius and ulnar deciencies.
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 412 This chapter will focus entirely on the thumb
with a focus on diagnosis, classication, and
treatment to maximize the most important digit
of the hand. As humans, we can function
extremely well with a thumb and an adjacent
post for prehension. Without a thumb, we are
relegated to a primitive hand lacking an oppos-
able digit with diminished function.

Introduction

Thumb hypoplasia is considered part of radial

deciency even with normal forearm architecture.
Electronic supplementary material: The online version Therefore, associated anomalies need to be con-
of this chapter (doi:10.1007/978-1-4614-8515-5_17) sidered during the initial evaluation. The workup
contains supplementary material, which is available
must be inclusive of entities such as VACTERL
to authorized users. Videos can also be accessed at
http://www.springerimages.com/videos/978-1-4614-8513-1. association, thrombocytopenia-absent radius (TAR)
syndrome, Holt-Oram syndrome, CHARGE syn-
S.H. Kozin (*) D.A. Zlotolow
Shriners Hospitals for Children, Philadelphia, PA, USA drome, and Fanconi anemia (FA) (Table 1 and
e-mail: skozin@shrinenet.org; dzlotolow@yahoo.com Fig. 1). A workup of all potentially affected
# Springer Science+Business Media New York 2015 389
J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_17
390
organ systems can be lifesaving and should
include a renal ultrasound, a cardiac echocardio-
gram, spine radiographs, a complete blood count,
and a chromosomal challenge test (Soldado
et al. 2013).
Failure to make the diagnosis of an associated
syndrome will have lifelong repercussions for the
Table 1 Syndromes or associations with radial deficiency
Syndrome or association Characteristics
Holt-Oram Heart defects, most
commonly cardiac septal
defects
Thrombocytopenia- Thrombocytopenia present
absent radius (TAR) at birth (may require
syndrome transfusions), but improves
over time
VACTERL association Vertebral abnormalities,
anal atresia, cardiac
abnormalities,
tracheoesophageal stula,
esophageal atresia, renal
defects, radial dysplasia,
lower limb abnormalities
Fanconi anemia Aplastic anemia not present
at birth, develops about
6 years of life
CHARGE syndrome Coloboma of the eye, heart
defects, atresia of the nasal
choanae, retardation of
growth and/or
development, genital
and/or urinary
abnormalities, and ear
abnormalities and deafness
Fig. 1 A 3 year-old child
with CHARGE syndrome
(Courtesy of Shriners
Hospital for Children,
Philadelphia)
S.H. Kozin and D.A. Zlotolow
child, family, and physician. This consequence is
especially evident in children with FA that can be
diagnosed with a chromosomal challenge test.
Early diagnosis allows for years of preparation
to search for an appropriate bone marrow match
that may prevent the child from succumbing to
aplastic anemia. Innovative techniques, such as
PGD (preimplantation genetic determination),
allow the parents to have another child without
FA, but with matching blood characteristics.
Hence, cord blood can be harvested and used as a
donor for the affected FA child. Many hand sur-
geons and pediatricians fail to consider the diagno-
sis of FA at the time of thumb reconstruction or
consultation, leaving the child and their family to
suffer the consequences of a painful terminal ill-
ness that could otherwise have been treated.
Diagnosis
The diagnosis of thumb deciency is dependent
upon the extent of deciency and the presence or
absence of any associated anomalies. Thumb hypo-
plasia and profound radial deciency are usually
diagnosed shortly after birth as the forearm is
foreshortened and the hand deviated in a radial
direction. In addition, orid thumb hypoplasia is
recognized early in infancy as there is marked
asymmetry between the thumbs. In contrast, mild
thumb hypoplasia may go unrecognized for years
and not present until adolescence, even if more
17 Thumb Hypoplasia 391

severe hypoplasia is diagnosed on the contralat- Table 2 Thumb deficiency classification and treatment
eral side. This late presentation often is confusing paradigm
to the patient and disconcerting to the parents. The Type Findings Treatment
parents cannot understand how they did not rec- I Minor generalized No treatment
ognize their childs discrepancy before adoles- hypoplasia
cence and often relate the thumb hypoplasia to II Absence of intrinsic thenar Opponensplasty
muscles
remote trauma. Our explanation is quite simple;
First web space narrowing First-web
as one gets older the activities of daily of life release
become more complicated, and therefore subtle Ulnar collateral ligament UCL
differences are not recognizable until the child is (UCL) insufciency reconstruction
older. For example, buttoning, tying, and III Similar ndings as type II A:
keyboarding are tasks performed as the child plus: Reconstruction
becomes older and are not performed during Extrinsic muscle and tendon B: Pollicization
abnormalities
infancy and early childhood. This explanation
Skeletal deciency
obviates any feeling of guilt and allows the family A: Stable CMC joint
to focus on the treatment to remedy the problem. B: Unstable CMC joint
Diagnosis is based upon a careful physical IV Pouce ottant or oating Pollicization
examination and corresponding x-rays. The length, thumb
girth, motion, and stability are important elements V Absence Pollicization
of the examination. The length and girth are usually
compared to the contralateral side. The normal
thumb length is just proximal to the proximal the thenar muscles. As stated previously, this
interphalangeal (PIP) joint of the index nger. slight degree of hypoplasia may present later in
X-ray length is approximately 70 % of the length life as the complexities of daily hand function
of the adjacent index nger proximal phalanx. The become more intricate and complicated. Type I
thumb girth and nail width as a percentage of thumb hypoplasia may or may not warrant treat-
corresponding levels of the index nger girth and ment depending on the impact on function. Inca-
nail width are approximately 133 % and 105 %, pacitating lack of opposition requires a tendon
respectively (Goldfarb et al. 2005). The overall transfer to enhance thumb function.
thumb motion is assessed along with movement at Type II hypoplasia has distinct ndings that
the carpometacarpal (CMC), metacarpophalangeal direct treatment (Fig. 2). There is absence of the
(MCP), and interphalangeal (IP) joints. Similarly, thenar muscles innervated by the recurrent branch
the stability of each of these joints is assessed by of the median nerve (abductor pollicis brevis,
clinical examination. Instability of the MCP and/or opponens pollicis, and supercial head of the exor
CMC joints is commonly associated with thumb pollicis brevis) (Fig. 2a). The ulnar nerve-
hypoplasia and directly inuences the surgical innervated deep head of the exor pollicis brevis
treatment paradigm. An inventory of present and is usually present and provides MCP joint exion.
absent intrinsic and extrinsic muscles is a necessary The MCP is often unstable with incompetency of
part of the examination, as this is critical to plan- the ulnar collateral ligament (Fig. 2b). The MCP
ning the surgical reconstruction. joint can also be globally unstable with incompe-
tency of both the ulnar and radial collateral liga-
ments. The thumb-index or rst web space is
Classification/Diagnosis narrowed, and the skin is tight (Fig. 2c). The web
space can appear less narrow as the unstable MCP
The classication of thumb hypoplasia truly joint drifts into valgus during the examination.
guides management (Table 2) (Kozin 2010a; Type III hypoplasia has the same characteris-
Soldado et al. 2013). Type I hypoplasia has tics as type II hypoplasia as well as extrinsic
minor generalized hypoplasia usually affecting muscle and tendon deciencies. Absence or
392
Fig. 2 A 2- year-old with type II left thumb deciency (Courtesy of Shriners Hospital for Children, Philadelphia). (a)
Absent thenar muscles. (b) Ulnar collateral ligament insufciency. (c) Narrow thumb-index web space
hypoplasia of the extrinsic extensors (extensor
pollicis longus and extensor pollicis brevis) or
exors (exor pollicis longus) requires a careful
examination of the active movement at the MCP
and IP joints. Type III hypoplasia is also
subdivided into IIIA and IIIB, which is a critical
demarcation in the treatment paradigm. A type
IIIA thumb has a stable CMC joint that is worthy
of reconstruction and provides a useful prehensile
thumb. A type IIIB thumb has an unstable CMC
joint that requires ablation and pollicization. The
unstable CMC joint precludes reconstruction as
the surgery cannot overcome the basal joint insta-
bility. A simple explanation to parents is that this
effort would be similar to trying to build a house
without a stable foundation. Multiple valiant sur-
gical attempts to reconstruct the CMC joint have
paled in comparison to a successful index nger
pollicization with respect to outcome and function
(Kozin et al. 1992; Kozin 2012).
S.H. Kozin and D.A. Zlotolow
Differentiating between a type IIIA (stable) and
a type IIIB (unstable) thumb can be difcult in
some cases, but crucial to formulating a treatment
plan. Because the rst metacarpal base and the
trapezium ossify around 6 years of age, radio-
graphs are unreliable for evaluation of the CMC
joint. In a very unstable CMC joint, the metacarpal
base tapers to a point rather than having the typical
metaphyseal are (Fig. 3). However, this plain
x-ray nding is inconsistent. Advanced imaging
studies, such as ultrasound or MRI, can be helpful
to evaluate the integrity of the cartilaginous meta-
carpal base and the trapezium (Soldado et al. 2013).
Repeat examinations, however, may be equally as
reliable to detect a thumb unworthy of reconstruc-
tion. If the child completely bypasses her or his
thumb in favor of scissor pinch, the index nger
pronates, and the index-long web space widens,
then thumb reconstruction is contraindicated, and
index pollicization is preferred.
17 Thumb Hypoplasia
Fig. 3 X-ray of a 2-year-old revealing a thumb metacarpal
that tapers to a point is indicative of an unstable
carpometacarpal joint (Courtesy of Shriners Hospital for
Children, Philadelphia Unit)
Type IV hypoplasia represents a substantial
deciency known as a pouce ottant or oating
thumb (Fig. 4). The thumb remnant is attached to
the hand solely by its neurovascular bundle and
skin. The thumb is oppy and is not incorporated
into prehension. Type V is complete absence of
the thumb. On occasion, there will be a small
nubbin remnant present. Scissor pinching is
performed between the index and long nger.
Thumb hypoplasia can also be found in children
with ulnar deciency. A similar treatment paradigm
is applied. Pollicization is indicated for marked
thumb hypoplasia. The surgical technique is similar
with minor modications, such as skin design.
The standard surgical indications for polliciza-
tion can be expanded to include a thumb smaller
than a small nger (Kozin 2012). Reconstruction
of a small hypoplastic thumb even with a stable
CMC joint will not function like pollicization of a
mobile index nger (Foucher et al. 2001). This
393
Fig. 4 A 1 year-old with severe type IV thumb hypoplasia
(pouce ottant or oating thumb) (Courtesy of Shriners
Hospital for Children, Philadelphia Unit)
decision requires a heart to heart conversation
with the parents. Parents usually come to appreci-
ate that function trumps form and that thumb
ablation and index pollicization will result in
enhanced function versus reconstruction of a
small scrawny thumb. When in doubt, parents
are offered the opportunity to discuss their deci-
sion with other parents who have made a similar
difcult decision.
Treatment
There is little role for nonoperative management
of the hypoplastic thumb. An experienced thera-
pist evaluation, however, is invaluable. Their
input can be helpful by providing support for the
family and facilitating the decision-making pro-
cess. On occasion, preoperative fabrication of a
thumb spica splint may stabilize an unstable MCP
joint. Following surgery, the therapists role esca-
lates to act as a primary provider of care that
directly impacts the success of the procedure
(Tables 3 and 4).
The classication schema guides treatment.
Type I hypoplasia usually does not warrant surgi-
cal intervention. Type II and IIIA hypoplasia often
requires surgery to provide stability, improve
motion, and enhance function. Type IV and V
hypoplasia requires index nger pollicization as
long as the index is t to be a thumb. A stiff
394
Table 3 Thumb hypoplasia: Nonoperative management
Indications Contraindications
Few indications None
Unstable joints
Determining the degree of thumb
hypoplasia and prehensile pattern
of the child
Table 4 Thumb hypoplasia: Physical/occupational ther-
apy recommendations
Assessment of thumb hypoplasia and thumb usage
during prehension
Thumb spica splint
Education of parents
Participate in the decision-making process
index nger will make a stiff thumb that may not
improve hand function. Therefore, the risk/benet
ratio requires calculation and consideration. The
operation should follow the Peter Carter 24 rule.
In other words, for some children surgery can
indeed be done to them, but it may not neces-
sarily be for them.
Thumb Reconstruction
Thumb reconstruction for type II and IIA hypo-
plasia requires addressing all the elements that are
decient. The narrowed thumb-index web space,
MCP instability, and thenar muscle absence all
require treatment. Thumb-index web space
narrowing is treated with skin rearrangement and
soft tissue release. A four-ap Z-plasty lengthens
the tight skin and provides a rounded contour to
the web space. The deeper soft tissue release
includes the investing fascia around the adductor
pollicis. The princeps pollicis artery and its
branches must be identied and protected before
division of the fascia. An extremely tight web
space may also require partial release of the
adductor muscle and/or rst dorsal interosseous
muscle.
MCP instability requires stabilization. Unidi-
rectional instability with an incompetent ulnar
collateral ligament is the most common nding.
Ligament reconstruction can be performed with
S.H. Kozin and D.A. Zlotolow
the residual length of donor tendon from the oppo-
sition transfer. Bidirectional or global instability is
more difcult to manage. Reconstruction of both
the radial and ulnar collateral ligaments with ten-
don graft has been described. MCP joint
chondrodesis is preferred to achieve rm stability
and provide a stable fulcrum for the opposition
transfer to function. The details of this procedure
are covered in chapter Arthrogryposis. UCL
insufciency can be secondary to an anomalous
connection between the exor pollicis longus and
extensor pollicis longus muscles (a.k.a. pollex
abductus) (Tupper 1969; Fitch et al. 1984; Lister
1991). This pollex abductus attenuates the UCL
over time and prevents active interphalangeal
joint motion. Surgery must release the pollex
abductus at the time of UCL reconstruction.
Thenar absence requires a tendon transfer to
provide thumb opposition. There are a variety of
donor muscle-tendon units to choose from
including exor digitorum supercialis (long or
ring), abductor digiti minimi, extensor carpi
ulnaris, and extensor indicis proprius. Each
donor muscle-tendon unit has its advocates,
advantages, and disadvantages. The long or ring
nger exor digitorum supercialis (FDS) is
preferred secondary to its length, technical ease,
power, expendability, and synergism. If the FDS
is unavailable, the other donor options are
considered.
Technique: Flexor Digitorum
Superficialis Opponensplasty with UCL
Reconstruction (Opposition and UCL
Reconstruction Video)
The patient is placed supine on the operating room
table (Video 1). The procedure is usually
performed under general anesthesia. A single
dose of intravenous preoperative antibiotics is
administered. The limb is prepped and draped in
sterile fashion. Chlorhexidine gluconate and alco-
hol prep (ChloraPrep; CareFusion, Leawood,
Kansas, USA) is preferred, which may be more
effective in eliminating bacteria and avoids iodine
that can migrate beneath the tourniquet and cause
burns (Saltzman et al. 2009; Table 5)
The limb is exsanguinated and the tourniquet
is inated. The incisions are carefully drawn
17 Thumb Hypoplasia
Video 1 Opposition and
UCL Reconstruction
Table 5 Thumb OR table: regular
reconstruction: Preoperative
Position/positioning
planning
aids: supine
Fluoroscopy location:
ipsilateral
Equipment: standard,
drill, K-wires
Tourniquet: sterile
(Fig. 5). The narrowed thumb-index web space is
widened via a four-ap Z-plasty. Each limb of the
Z-plasty should be equal in length except for the
radial limb that is extended in a proximal direction
to expose the UCL and the MCP joint. The ring
nger FDS tendon is isolated at the base of nger
and in the distal forearm. A short oblique incision
is made at the base of the ring nger, and a zigzag
or oblique incision is performed along ulnar por-
tion of volar forearm. The exor carpi ulnaris
(FCU) tendon is also isolated. An additional skin
incision is made along the radial side of the thumb
MCP joint to expose the eventual site for FDS
tendon attachment. The ring nger FDS tendon is
identied in at the base of the nger and separated
from the exor digitorum profundus (FDP) ten-
don. The FDS tendon is also isolated in the fore-
arm (Fig. 6). The FDS tendon is tagged with a
suture and cut at the base of the ring nger while
protecting the underlying FDP tendon. An Allis
tissue forceps (Jarit, Hawthorne, NY) is placed
around the ring FDS tendon in the forearm and
395
used to roll the tendon through the carpal tunnel
and into the forearm (Fig. 7). Failure of the FDS
tendon to roll into the forearm requires assessment
for FDP-FDS attachments and/or division of
Campers chiasm.
A loop of FCU tendon is used to construct a
pulley for the FDS tendon. The distal 23 cm of
FCU tendon is isolated. The radial one-half of the
FCU tendon is harvested preserving its distal
attachment to the pisiform (Fig. 8). The radial
one-half of the tendon is passed through the
retained FCU at the pisiform to fashion a loop.
The radial one-half of the tendon is sutured to the
retained FCU at the pisiform. The ring nger FDS
tendon is passed through the FCU loop in prepa-
ration of transfer (Fig. 9).
A subcutaneous tunnel is made between the
radial side of the thumb and the volar forearm
incision for passage of the ring nger FDS tendon.
The FDS tendon is passed through the FCU loop
under the skin to the radial side of the thumb
(Fig. 10). The metacarpal head is isolated and
a 0.4500 Kirschner wire drilled across the metacar-
pal head parallel to the joint surface (Fig. 11).
Mini-uoroscopy is used to ensure appropriate
Kirschner wire position. The wire is directed
from the volar aspect of the radial side of meta-
carpal to the ulnar and dorsal aspect. The hole is
enlarged with a drill bit to allow passage of the
FDS tendon. The MCP joint is reduced and stabi-
lized with a longitudinal 0.04500 Kirschner wire
396 S.H. Kozin and D.A. Zlotolow

Fig. 5 Four-ap Z-plasty

to widen the narrowed
thumb-index web space
(Courtesy of Shriners
Hospital for Children,
Philadelphia)

Fig. 6 Ring nger FDS

tendon is identied in at the
base of the nger and within
the forearm (Courtesy of
Shriners Hospital for
Children, Philadelphia)

drilled antegrade from the tip of the thumb across
the MCP joint. The wire is cut short and a Jurgan
Pin Ball (RFO Medical Company, London, UK)
applied. The FDS tendon is passed through the
drill hole to the ulnar side of the thumb for liga-
ment reconstruction (Fig. 12). If the tendon is too
large in diameter, one FDS slip can be removed.
At this point, the wrist is placed into slight
extension, and the FDS tendon tensioned until
the thumb positions into opposition. Tenodesis is
used to assess tension, and once correct tension is
achieved, the FDS tendon is sutured to the sur-
rounding bone and periosteum along the radial
side of the thumb. This maneuver sets the tension
in the opposition tendon transfer. The remaining
FDS tendon along the ulnar side of the thumb is
used to reconstruct the UCL. The FDS tendon is
directed to the base of the proximal phalanx and
sutured directly into the bone. Usually, there is
additional length to pass the FDS tendon back
onto itself to complete a double-stranded repair
(Fig. 13).
The skin is closed with absorbable suture,
and the limb is immobilized in a long-arm thumb
spica cast for 3 weeks. The Kirschner wire is
removed, and a short-arm thumb spica splint is
fabricated. Active motion and therapy are initiated
(Tables 6 and 7).
17 Thumb Hypoplasia 397

Fig. 7 Ring nger exor

digitorum supercialis
tendon rolled into volar
forearm incision (Courtesy
of Shriners Hospital for
Children, Philadelphia)

Fig. 8 One-half of the

distal 23 cm FCU tendon
is used to construct a pulley
for the FDS tendon
(Courtesy of Shriners
Hospital for Children,
Philadelphia)

Pollicization Technique: Pollicization (Pollicization

Pollicization is indicated for type IIIB, IV, and V
hypoplasia. The technique is similar with subtle
variations depending upon whether a hypoplastic
thumb is present or absent. In a type IIIB hypo-
plasia, vascularized fat from the ablated thumb
can be used to augment the thenar eminence
(Upton et al. 2008; Soldado et al. 2013). In a
type IVor V hypoplasia, this option is unavailable.
Video)
The current technique of pollicization repre-
sents a consolidation of contributions from sur-
geons over the last 100 years (Littler 1976;
Kozin 2012). Following general anesthesia, the
child is placed in the supine position (Video 2).
Preoperative antibiotics are routinely adminis-
tered. The extremity is prepped and draped in
sterile fashion. A sterile pediatric tourniquet
398 S.H. Kozin and D.A. Zlotolow

Fig. 9 Ring nger FDS

tendon is passed through the
FCU loop (Courtesy of
Shriners Hospital for
Children, Philadelphia)

Fig. 10 FDS is tendon is

passed through the
subcutaneous tunnel to the
radial side of the thumb
(Courtesy of Shriners
Hospital for Children,
Philadelphia)

(Del Medical Innovations, Vancouver, Canada)
is placed on the upper arm that exsanguinates
during application. Exsanguination should be
gentle to maintain some lling of the digital ves-
sels for ease of identication.
The skin incision must be nimble to allow easy
index nger transposition and construction of a
sufcient thumb-index web space. The skin inci-
sion proposed by Marybeth Ezaki and Peter Carter
is employed as it allows more glabrous skin to be
placed along the palmar aspect of the index nger
(Kozin 2010a; Fig. 14). The palmar skin is incised
rst, and the radial neurovascular bundle is iso-
lated. In children with type IIIB or IV hypoplasia,
the single vessel to the hypoplastic thumb can be
traced to the radial neurovascular bundle of the
index nger to facilitate identication (Fig. 15).
The dissection next proceeds in an ulnar direction
to identify the common digital vessels to the
index-long web space. The proper digital nerves
to the ulnar side of the index and the radial side of
the long nger are isolated at the same time of
17 Thumb Hypoplasia 399

Fig. 11 Kirschner wire is

drilled across the
metacarpal head parallel to
the joint surface (Courtesy
of Shriners Hospital for
Children, Philadelphia)

Fig. 12 FDS tendon is

passed through the
metacarpal head to the ulnar
side of the thumb (Courtesy
of Shriners Hospital for
Children, Philadelphia)

vessel identication. Proximal microdissection is tension-free index nger pollicization

necessary to further separate the proper digital
nerves prior to pollicization. Occasionally, there
is a neural loop that encircles the artery that must
be addressed to prevent constriction of the artery
during index nger transposition. The smaller of
the two nooses is usually cut to prevent any arte-
rial compression. Alternatively, the nerve fasci-
cles can be split from each other to allow
recession of the artery more proximally. The
proper digital artery to the long nger is
ligated with a ligature clip (Fig. 16). This allows
vascularized by the radial digital artery and the
common digital artery to the index-long web
space.
The rst annular pulley of the index nger is
incised to avoid buckling of the exor tendons
after index nger shortening. The intermetacarpal
ligament is divided. The dorsal skin incision is
sharply elevated with preservation of as many
dorsal veins as possible (Fig. 17). The index
extensor tendons are inspected, and any intercon-
nections released to allow a direct pull to the index
400
Fig. 13 FDS tendon is
used to reconstruct the
UCL. Note longitudinal
Kirschner wire exiting tip of
the thumb (Courtesy of
Shriners Hospital for
Children, Philadelphia)
Table 6 Thumb reconstruction: Surgical steps
Limb is exsanguinated and the tourniquet is inated
Four-ap Z-plasty of thumb-index web space with release of
tight fascia
Ringer FDS tendon is isolated at the base of nger and in the
distal forearm
Isolate thoracodorsal neurovascular pedicle
Ring FDS tendon pulled through the carpal tunnel and into
the forearm
Radial one-half of the distal 23 cm FCU tendon made into a
loop for the FDS tendon
Ring nger FDS tendon passed through the through FCU
loop
Skin incision along the radial side of the thumb MCP joint
Subcutaneous tunnel is made between the radial side of the
thumb and the volar forearm FCU loop
FDS tendon is passed under the skin to the radial side of the
thumb
Metacarpal head is isolated and a 0.45 Kirschner wire
drilled across the metacarpal head parallel to the joint surface
Hole is enlarged with a drill bit to allow passage of the FDS
tendon
MCP joint is reduced and stabilized with a longitudinal
0.045 Kirschner wire drilled antegrade from the tip of the
thumb across the MCP joint
FDS tendon is passed through the drill hole to the ulnar side
of the thumb for ligament reconstruction
FDS tendon is sutured to the surrounding bone and
periosteum along the radial side of the thumb while setting
the appropriate tension in the tendon transfer
FDS tendon is directed to the base of the proximal phalanx
and sutured directly into the bone to reconstruct the UCL
Skin is closed with absorbable suture and the limb is
immobilized in a long-arm thumb spica cast for 3 weeks
S.H. Kozin and D.A. Zlotolow
Table 7 Thumb reconstruction: Postoperative protocol
Limb is immobilized in a long-arm thumb spica cast for
3 weeks
Kirschner wire is removed and a short-arm thumb spica
splint is fabricated
Tendon transfer ring and reeducation instituted
Protective extension block splinting of the thumb is
continued until 3 months after surgery
nger. The extensor or exor tendons are not
shortened as these musculotendinous structures
adapt over time.
The rst dorsal and palmar interossei muscles
are dissected to their attachments into the extensor
hood (Figs. 18 and 19). The interossei muscles
and tendons are released with a portion of the
hood in expectation of transfer. The tendons
must be carefully dissected from the MCP joint
collateral ligaments. The neurovascular bundles
must also be sheltered during elevation of the
interossei. Prior to cutting the metacarpal, the
eventual insertion sites for the tendon transfers
are identied and tagged within the extensor
mechanism over the proximal interphalangeal
joint (Fig. 20). This facilitates later transfer of
the rst dorsal and rst palmar tendons into the
radial and ulnar lateral bands, respectively.
The index nger must be shortened. A ne-
bladed saw is used to cut the metacarpal base in a
17 Thumb Hypoplasia 401

Video 2 Pollicization

Fig. 14 A 3 year-old with type IIIB hypoplasia (Courtesy along the palmar aspect of the index nger. (b) Incision
of Shriners Hospital for Children, Philadelphia) (a) Volar between the hypoplastic thumb and index nger. (c) Dorsal
skin incision that allows more glabrous skin to be placed incision design

perpendicular direction through the metaphyseal physis is identied. The distal cut is directly
are. A rongeur can widen the base by pedaling through the physis using a knife. This cut leads
the surrounding cortex. The metacarpal is dis- to physeal ablation (epiphysiodesis) to prevent
sected in a proximal to distal direction, and the undesirable growth of the index metacarpal.
402
Fig. 15 Single vessel to the hypoplastic digit is traced to
the radial neurovascular bundle of the index nger, and the
common digital vessels to the index-long web space are
identied (Courtesy of Shriners Hospital for Children,
Philadelphia)
Fig. 16 Ligation of the proper digital artery to the long
nger with ligature clips (Courtesy of Shriners Hospital for
Children, Philadelphia)
The metacarpal bone from its base to the epiphysis
is then removed.
The normal index MCP joint hyperextends,
and the normal thumb CMC joint does not hyper-
extend. To equalize this discrepancy, the index
MCP joint is xed into hyperextension at the time
of pollicization. This is achieved by suturing the
S.H. Kozin and D.A. Zlotolow
Fig. 17 Elevation of the dorsal skin incision with preser-
vation of as many dorsal veins as possible (Courtesy of
Shriners Hospital for Children, Philadelphia)
Fig. 18 Dissection of the rst dorsal interosseous muscle
with a portion of the extensor hood (Courtesy of Shriners
Hospital for Children, Philadelphia)
MCP joint into hyperextension using a
nonabsorbable suture material passed through the
epiphysis and dorsal capsule. Afterward, a
Kirschner wire is through the metacarpal epiphysis,
into the proximal phalanx, and out the PIP joint
using a wire driver. This Kirschner wire is used as a
joystick to simplify index nger positioning.
Interosseous sutures can be added to augment the
17 Thumb Hypoplasia 403

xation (Fig. 21). The index metacarpal epiphysis
is aligned anterior to its metaphyseal base with
meticulous positioning into 45 of abduction and
between 100 and 120 of pronation. Once the
position is deemed satisfactory, the Kirschner
Fig. 19 Dissection of the rst volar interosseous muscle
with a portion of the extensor hood (Courtesy of Shriners
Hospital for Children, Philadelphia)
wire is drilled retrograde across the metacarpal
base into the carpus (Figs. 22 and 23). The
Kirschner wire is cut short and a Jurgan Pin Ball
(Jurgan Development & Manufacturing, Madison,
Wisconsin, USA) is applied. Further stability is
obtained via tendon transfer of the rst dorsal
interosseous to the radial lateral band and the rst
palmar interosseous to the ulnar lateral band about
the PIP joint (Fig. 24). The skin is carefully inset
with absorbable suture. Any redundant skin is
excised. Whenever possible, the rst web space
suture line is advanced dorsal to avoid suture mate-
rial and scar within the commissure (Figs. 25 and
26). The tourniquet is deated and the thumb
observed for 5 min. The arterial circulation usually
returns quickly, although vasospasm can result.
Time, warm soaks, and patience routinely lead to
resolution. Persistent lack of blood inow requires
exploration for arterial kinking or iatrogenic injury.
Venous congestion is more common, which can
require application of a looser dressing and/or
release of any taut sutures. The postoperative dress-
ings are crucial. Adequate uffy dressings are nec-
essary to equalize the anterior-posterior and
medial-lateral dimensions of the hand. This
allows uniform compression without constriction.

Fig. 20 Elevation of the

rst dorsal and rst palmar
tendons from the
metacarpal and tagging of
their eventual insertion sites
(Courtesy of Shriners
Hospital for Children,
Philadelphia)
404
Fig. 21 A Kirschner wire
is driven through the
metacarpal epiphysis, into
the proximal phalanx, and
out the PIP joint using a
wire driver. This Kirschner
wire is then used as a
joystick to position the
index nger (Courtesy of
Shriners Hospital for
Children, Philadelphia)
A long-arm soft cast (3M Scotchcast Soft Cast
Casting Tape, St. Paul, Minnesota, USA) is applied
with the elbow exed to greater than 100 to
decrease the chance of accidental removal. This
berglass casting tape does not harden completely,
but remains slightly exible when cured. More
importantly, soft cast can be unwrapped in the
clinic avoiding the petrifying cast saw. The child
is admitted overnight with the arm elevated to
promote venous drainage. The thumb can be cov-
ered the next day if vascularity has been maintained
throughout (Tables 8 and 9).
Outcome
Thumb Reconstruction
The results for thumb reconstruction for types II
and IIIA are difcult to interpret as comparison
between studies is difcult due to the heterogene-
ity of the patient populations, severity of hypopla-
sia, and reconstructive techniques. In general,
results of thumb reconstruction for hypoplasia
are uniformly good compared with preoperative
S.H. Kozin and D.A. Zlotolow
function as long as the surgical plan addresses
each decient component of the thumb (Kozin
2010b) (Fig. 27). A mild degree of MCP joint
stiffness is commonly seen after ligament recon-
struction, however; this stiffness has diminutive
impact on functional outcome provided the
interphalangeal and CMC joints are supple.
The results of tendon transfers, bone lengthen-
ing, and joint stabilizations for higher-grade hypo-
plastic thumbs are poor compared with the
results of pollicization (Aliu et al. 2008). Children
who require a 5-digit hand for cultural reasons,
however, may gain some benet from these
procedures.
Pollicization
The results after pollicization depend primarily on
the status of the transposed index nger and sur-
rounding musculature. Pollicization of the index
nger provides better functional and aesthetic
results in isolated thumb hypoplasia compared to
patients with an associated hypoplastic or absent
radius. A mobile index nger moved to the thumb
17 Thumb Hypoplasia 405

Fig. 22 Once the index has

been positioned in adequate
abduction and pronation,
the Kirschner wire is drilled
retrograde across the
metacarpal base into the
carpus. The Kirschner wire
is then cut short and a
Jurgan Pin Ball applied
(Courtesy of Shriners
Hospital for Children,
Philadelphia)

Fig. 23 Frontal view

reveals adequate pronation
(Courtesy of Shriners
Hospital for Children,
Philadelphia)
406 S.H. Kozin and D.A. Zlotolow

Fig. 24 Tendon transfer of

the rst dorsal interosseous
to the radial lateral band and
the rst palmar interosseous
to the ulnar lateral band
about the PIP joint
(Courtesy of Shriners
Hospital for Children,
Philadelphia)

Fig. 25 The skin is

carefully inset to maximize
the rst web space and
optimize the appearance
(Courtesy of Shriners
Hospital for Children,
Philadelphia)
17 Thumb Hypoplasia
Fig. 26 Final closure with
glabrous skin along the
undersurface of the thumb
(Courtesy of Shriners
Hospital for Children,
Philadelphia)
provides stability for grasp and mobility for ne
pinch and shows progressive improvements in
function comparable with normal development
(Kozin et al. 1992; Fig. 28). In contrast, a stiff
index nger in the thumb position provides a
stable thumb post for gross grasp but will not
participate in pinch (Egloff and Verdan 1983;
Manske et al. 1992; Percival et al. 1991; Sykes
et al. 1991). Scissoring of adjacent digits is usu-
ally preferred for ne pinch if dexterity of the
pollicized index nger is compromised. Children
with a stiff, weak, malpositioned, diminutive,
and/or unstable new thumb will often bypass the
thumb and begin to scissor pinch between the long
and the ring nger.
A recent study correlated the primary care-
takers subjective impressions of outcome to objec-
tive measures and found that children who had
difculty peeling a sticker from its backing tended
to scissor pinch between the long and ring ngers
and ignored the thumb for small object manipula-
tion. In these children, the new thumb IP and MP
motion was poor (Zlotolow et al. 2014).
407
Likewise, children with a preoperative ulnar-
sided grasp pattern, as is often seen in patients
with radial longitudinal deciencies, will main-
tain their ulnar-sided grasp after pollicization and
ignore the new thumb. Patients with mirror hands
or ve-ngered hands tend to have suboptimal
results as well, although the reasons for this are
unclear (Manske et al. 1992). It may be that the
pollicized digit in these patients is more akin to a
long nger than an index nger and may therefore
lack the same independence of function.
The results of pollicization persist into adult-
hood, with long-term studies showing continued
functional use of the thumb (Clark et al. 1998). In
most cases where the child uses the index nger as a
thumb preoperatively, pollicization results in good
to near-normal hand function in the long term. With
no associated conditions to limit outcomes, the
child can expect about 1/3 of the strength of an
unaffected thumb. Large object manipulation is
typically better than small object, but overall
approximately 95 % of patients incorporate their
thumbs into daily activities (Manske et al. 1992).
408
Table 8 Pollicization: Surgical steps
Limb is gently exsanguinated and the tourniquet is
inated
Skin incision proposed by Ezaki and Carter
Palmar skin is incised and the radial neurovascular
bundle is isolated
Dissection proceeds in an ulnar direction to identify the
common digital vessels to the index-long web space
Proper digital nerves to the ulnar side of the index and the
radial side of the long nger are isolated
Proper digital artery to the long nger is ligated with a
ligature clip
Incise rst annular pulley of the index nger
Divide intermetacarpal ligament between the index and
long nger
Dorsal skin incision is sharply elevated with preservation
of dorsal veins
The rst dorsal and palmar interossei muscles are
released with a portion of the hood
Metacarpal index nger is shortened by a saw cut at the
metaphyseal are and a knife cute through the physis
leading to an epiphysiodesis
Index MCP joint is xed into hyperextension using a
nonabsorbable suture material passed through the
epiphysis and dorsal capsule
Kirschner wire drilled antegrade through the metacarpal
epiphysis, into the proximal phalanx, and out the PIP
joint
Kirschner wire is used as a joystick to align the index
metacarpal into 45 of abduction and between 100 and
120 of pronation
Kirschner wire is drilled retrograde across the metacarpal
base into the carpus
Transfer the rst dorsal interosseous to the radial lateral
band and the rst palmar interosseous to the ulnar lateral
band about the PIP joint
Skin is closed with absorbable suture with resection of
any redundant skin. Kirschner wire is cut short
The objective and aesthetic outcomes follow-
ing pollicization have been compared to normal
thumbs (Goldfarb et al. 2007). In general, the
pollicization was slightly longer and smaller in
girth. The most frequently cited negative percep-
tions of the thumb were decreased girth, excessive
length, and angulation.
Timing of the surgery has not been shown to
affect functional outcomes, although most clini-
cians feel that early surgery may be better to take
advantage of cortical plasticity. Others have
argued that social factors should be the impetus
S.H. Kozin and D.A. Zlotolow
Table 9 Pollicization: Postoperative protocol
Limb is immobilized in a long-arm thumb spica cast for
4 weeks
Kirschner wire is removed and a short-arm thumb spica
splint is fabricated
Occupational therapy is started with an emphasis on
thumb usage
Initial goal is large object acquisition followed by smaller
objects and eventually ne pinch
Standard wound care is also performed consisting of scar
massage and softening techniques
Protective splinting of the thumb is continued until
3 months after surgery
for early surgery, since the index nger is already
cortically represented as a thumb if the child uses
it as a thumb. Pollicization merely makes the
index thumb work more like a true thumb
(Manske et al. 1992).
Cortical plasticity and motor relearning are
postulated to play a critical role in functional
development following pollicization. There is a
large region of the sensorimotor cortex homuncu-
lus devoted to the hand, and much of it is devoted
to the thumb. Researchers are trying to understand
the changes in sensorimotor cortex following
injury, repair, and reconstruction (Anastakis
et al. 2008). Techniques include transcranial mag-
netic stimulation (TMS), electroencephalography,
magnetoencephalography (MEG), functional
MRI (fMRI), structural MRI (sMRI), and positron
emission tomography (PET) (Gevins et al. 1995;
Friston et al. 1991). Human cortical plasticity is a
complex process that involves the unveiling of
formerly dormant connections and sprouting of
active afferents from nearby cortical and/or sub-
cortical territories.
Following hand transplantation, the original
sensorimotor cortex map for hand activation is
restored (Giraux et al. 2001). The transplantation
reverses the sensorimotor cortex loss following
the hand amputation. Similarly, successful toe
transfer produces temporal activation within the
sensorimotor cortex consistent with cortical plas-
ticity (Manduch et al. 2002). Functional MRI has
conrmed that when a patient learns to use their
toe transfer, there is an expansion in their motor
cortical representation. Practice amplies the
17 Thumb Hypoplasia 409

Fig. 27 A 10 year-old
child status post left FDS
tendon opposition transfer
(Courtesy of Shriners
Hospital for Children,
Philadelphia) (a) Excellent
opposition to the small
nger. (b) Improved ability
to acquire large objects

Fig. 28 A 3 year-old s/p index pollicization depicted in surgical technique (Courtesy of Shriners Hospital for Children,
Philadelphia) (a) Overall appearance. (b) Movement pattern. (c) Pinching a sticker
410 S.H. Kozin and D.A. Zlotolow

changes within the SMS cortex. As the new motor Table 10 Pollicization pitfalls and complications
skill is mastered, there is a subsequent decrease in Complication Etiologies Management
the amount of cortical representation (Anastakis First web Insufcient web Therapeutic
et al. 2008; Manduch et al. 2002). The precise space space modalities
effects of pollicization have yet to be studied, contracture reconstruction or Revision web
loss of skin ap space deepening
however; similar sensorimotor cortex changes
via z-plasty or
are likely. Without a doubt, fundamental alter- dorsal rotational
ations must occur in the sensorimotor cortex as ap
the index nger becomes a thumb. Stiffness May be ascribed Therapy
Another factor to consider when deciding the to preoperative Inherent stiffness
condition of the not correctable
timing of surgery is the size of the hand. While the
index nger or Surgical
precise age to perform surgery is unknown, the secondary to adhesions can be
advantages of early surgery are mitigated by scarring related to treated by
the anatomical size of the neurovascular and surgery tenolysis
musculocutaneous structures. Pollicization at Excessive Failure to ablate Epiphysiodesis
about 12 years of age allows some hand growth length index metacarpal and ostectomy of
and still takes advantage of toddler brain plastic- growth plate metacarpal
Malrotation Technical error Rotational
ity. Late presentation is not a contraindication for
(under or over osteotomy
pollicization, however; the adolescent is likely has rotation) or loss of
less plasticity and is often astonished about the xation during
change in his or her hand. Preoperative discussion postoperative care
and reviewing pictures is mandatory to lessen the Lack of Primary deciency Opposition
opposition in intrinsic muscles tendon transfer
surprise following dressing removal. or inability to
reconstruct
interossei
Complications

Complex procedures can lead to big and small

complications. Acute complications are related
to vascular compromise. Arterial insufciency
results in failure of the thumb to pink up and
requires immediate exploration for arterial injury
or kinking. The underlying problem needs to be
rectied to preserve the arterial inow to the
thumb. The second acute problem involves
venous egress as the thumb turns blue. Rectica-
tion requires a series of steps including loosening
of the dressings or release of taught sutures. Sub-
sequently, strict elevation is necessary to promote
venous drainage. Loss of the index nger due to
vascular compromise after pollicization is rare,
with an estimated incidence of 0.2 % in experi-
enced hands (Buck-Gramcko 1971).
Long-term complications are related to scar-
ring, stiffness, bony problems, and limited motion
(Table 10). Scarring within the rst web space
Fig. 29 Over-rotation of the index nger can occur and
requires therapeutic modalities, such as silicone can result in a thumb that is poorly aligned for pinch
elastomer and massage. Dense recalcitrant (Courtesy of Shriners Hospital for Children, Philadelphia)
17 Thumb Hypoplasia
Fig. 30 A 6 year-old with a long thumb attributed to
ongoing growth at the metacarpal physis (Courtesy of
Shriners Hospital for Children, Philadelphia) (a) Clinical
scarring requires revision surgery to resurface the
web space. Stiffness may be inherent to the index
nger and not amenable to treatment or iatrogenic
and related to the surgery. Iatrogenic stiffness
requires therapy to mobilize the thumb. Persistent
stiffness may have a surgical solution, such as
tendinous adhesions. Careful examination is nec-
essary to determine the root cause. Bony problems
can be related to instability, malpositioning of the
thumb, or length. CMC joint instability is
411
appearance of elongated thumb. (b) Bony resection and
epiphysiodesis. (c) Skin reduction. (d) Final appearance
uncommon even with a brous union (Locher
et al. 2012). Symptomatic instability, however,
warrants revision surgery. A thumb positioned in
too much supination or pronation requires
osteotomy and repositioning (Fig. 29). A long
thumb attributed to ongoing growth at the metacar-
pal physis requires epiphysiodesis and possible
shortening (Fig. 30). Limited active motion in the
presence of acceptable passive motion is amenable
to tendon transfer, such as an opposition transfer.
412
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Fitch RD, Urbaniak JR, Ruderman RJ. Conjoined exor
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PR. Normative values for thumb length, girth, and
width in the pediatric population. J Hand Surg
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 Constriction Band Syndromes
18
Gloria Gogola

Contents Abstract
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 413 Constriction band syndrome is a congenital
condition with a wide spectrum of clinical pre-
Etiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 414
sentation. The physical appearance ranges
Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 415 from a mild hourglass-type circumferential
Relevant Anatomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 417 indentation in the skin to complete digit or
limb reduction. A deep band may result in
Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 419
distal lymphedema, nerve compression, defor-
Classication . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 419 mity, and acrosyndactyly.
Treatment Options . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 419 There is such great variation that no two
Nonoperative Management . . . . . . . . . . . . . . . . . . . . . . . . . . 419 individuals will have the identical deformity,
Operative Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 419
posing a treatment challenge for hand sur-
Constriction Band Excision . . . . . . . . . . . . . . . . . . . . . . . . 421 geons. Operative treatment is by necessity as
Preoperative Planning . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 421 varied as the clinical presentation. The specic
Technique . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 422
Postoperative Care . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 423 surgical strategy must be tailored to each indi-
Outcomes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 423 vidual and ranges from emergent limb-sparing
Acrosyndactyly Separation . . . . . . . . . . . . . . . . . . . . . . . . 424
band release to elective aesthetic band exci-
Preoperative Planning . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 424 sion. Timing of surgical intervention is impor-
Technique . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 425 tant and determined by the clinical
Postoperative Care . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 425 presentation. Management of constriction
Reconstructive Procedures for Digital band syndrome is focused on optimizing func-
Deciency . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 425 tion, facilitating growth and development, and
Management of Complications . . . . . . . . . . . . . . . . . . . . 426 improving aesthetic appearance.
Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 427
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 428 Introduction

Constriction band syndrome (CBS) is a congenital

limb difference presenting with circumferential
bands causing an hourglass-like appearance in
the affected limbs. The severity of the deformity
G. Gogola
Shriners Hospital for Children, Houston, TX, USA is very variable, ranging from mild skin indenta-
e-mail: ggogola@shrinenet.org tion to complete limb reduction (Fig. 1ac).
# Springer Science+Business Media New York 2015 413
J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_18
414 G. Gogola

Fig. 1 (ac): Examples of

the spectrum of clinical
presentation of constriction
bands. (a) Constriction
bands causing hourglass
deformity at the base of the
ngers. (b) Deeper bands on
the central digits with distal
deformity; the thumb and
small nger are not affected.
(c) Acrosyndactyly with
phalangeal reduction of the
long, ring, and small
ngers. The index nger
has a prominent band at the
base, and the thumb is not
involved (Courtesy of
Shriners Hospitals for
Children, Houston)

Infants may present with swelling, deformity, or
fusion of the digits (acrosyndactyly) distal to the
site of constriction (Askins and Ger 1988; Foulkes
and Reinker 1994; Kawamura and Chung 2009).
Constriction band syndrome was rst described in
the 1800s. Chaussier in 1812, Montgomery in
1832, and Simonart in 1846 all reported on
encircling bands with the potential to cause distal
deformities and intrauterine amputations (Moses
et al. 1979; Montgomery 1832; Foulkes and
Reinker 1994). However, CBS was possibly rec-
ognized much earlier by Hippocrates, the most
observant human in history, who reportedly
noted the presence and appearance of constriction
bands on the extremities (Light 1991). CBS
occurs in 1/1,2001/15,000 live births (Garza
et al. 1988; Foulkes and Reinker 1994; Kawamura
and Chung 2009). It is believed to cause 1 in
70 spontaneous abortions by strangulation of the
umbilical cord or from massive body wall
involvement incompatible with life (Byrne
et al. 1982; Kalousek and Bamforth 1988). CBS
is a sporadic condition, with no sex predilection.
While no distinct risk factors have been dened
(Goldfarb et al. 2009), nearly 60 % of documented
cases have some type of abnormal gestation his-
tory (Askins and Ger 1988; Foulkes and Reinker
1994).
Etiology
CBS represents a separate category in the
Swanson/International Federation of Societies
for Surgery of the Hand (IFSSH) classication
for congenital hand differences (Swanson 1976;
18 Constriction Band Syndromes
De Smet 2002). The etiology still remains contro-
versial, a fact reected in the plethora of terminol-
ogy for this condition: amniotic band syndrome,
constriction ring syndrome, Streeters dysplasia,
Simonarts bands, annular groove, amniotic band
disruption complex, limb body wall complex,
ADAM (amniotic deformity, adhesions, mutila-
tions), and amnion rupture sequence. The dif-
culty lies in nding a single mechanism that
accounts for the disparate anomalies associated
with CBS in up to 70 % of cases. These anomalies
include clubfoot deformity, leg length discrep-
ancy, cleft lip, cleft palate, and visceral and body
wall defects (Moses et al. 1979; Foulkes and
Reinker 1994). Aberrant embryonic morphogen-
esis (intrinsic theory), fetal vascular compromise,
and mechanical deformation of normally devel-
oped structures (extrinsic theory) have all been
proposed.
The intrinsic theory was rst suggested by
Streeter, who felt that there was a primary meso-
dermal defect resulting in malformations during
embryogenesis (Streeter 1930). Others have
supported the concept that the abnormal tissue at
the site of constrictions represents abnormal dis-
tribution of apoptosis, or programmed cell death,
a normal embryological process (McKenzie
1975). Mild constriction bands look like naturally
occurring skin creases, but in abnormal locations
(Moran et al. 2007). A second theory for the
etiology of CBS is that of vascular disruption or
hemorrhage within the limb bud mesenchymal
tissues after the condensation of the digital rays,
from either intrinsic or extrinsic forces (Van Allen
1981; Halder 2010). Kino supported this theory
with an experimental animal model (Kino 1975).
Lockwood added to the idea of a teratogenic event
causing a malformation based on studies of mono-
zygotic twins with CBS (Lockwood et al. 1989).
The currently best-accepted theory, proposed
by Torpin in 1965, states that the constrictions are
a result of extrinsic forces and events causing
deformation of a normally developing fetus
(Torpin 1965). Through the rst trimester of preg-
nancy, the fetal membranes have two distinct
layers, the chorion and the amnion. The extrinsic
theory states that a partial rupture of the inner
amnion prior to the fusion of the two layers results
415
in brous chorionic bands that can encircle and
trap some part of the fetus. The gestational age at
which the amniotic rupture occurs is thought to
be critical in determining the severity of the
resulting deformation. Rupture before 45 days
gestation can lead to central anomalies, CNS
malformations, and truncal defects, because
during the 5th week skeletal elements develop
from mesodermal condensations appearing along
the long axis of the limb bud (Higginbottom
et al. 1979). Later rupture is more likely to affect
the limbs. Later, as the fetus grows but the bands
do not, the bands become constricting, resulting
in distal deformation, acrosyndactyly, vascular or
neural compromise, or complete limb or digital
reduction. If the amniotic bands are swallowed
while still partially attached to the chorion, the
tether may lead to bizarre facial clefts and palate
deciencies that are not along the embryological
planes of facial closure (Bouguila et al. 2007). A
possible explanation for the non-band-related
effects of a partial amniotic rupture is a transient
oligohydramnios from extravasation of amniotic
uid. Until the chorion adjusts its permeability,
the developing fetus has restricted space,
resulting in compressive deformation forces.
This may contribute to the severity of clubfeet
deformities seen with CBS (Allington et al. 1995;
Gomez 1996) (Fig. 2a, b).
Diagnosis
CBS is present at birth and is diagnosed based on
clinical features and appearance. There is a predi-
lection for the distal segments of the limbs, with
the hand and ngers involved up to 80 % of the
time (Tada et al. 1984). In a study of 364 patients
with constriction bands, 64 % of the bands were
located in the upper limb; of those, 2 % were on
the arm, 7 % on the forearm, and 55 % in the hand.
The lower limb bands were similarly concentrated
in the distal part (Flatt 1977). The central digits are
most commonly involved. The thumb is least
affected, likely due to its in-palm fetal position,
where it is relatively protected by the ngers
(Light and Ogden 1993; Foulkes and Reinker
1994). The clinical presentation can have any
416
Fig. 2 (a) Clinical
photograph of an infant
with a deep constriction
band with distal edema and
clubfoot deformity. The
other leg has a milder
clubfoot deformity without
constriction bands. (b) The
affected leg 9 months post
band excision. The
lymphedema has
signicantly improved;
treatment for the clubfoot
deformity is ongoing
(Courtesy of Shriners
Hospitals for Children,
Houston)
variation along a spectrum that includes mild
hourglass-type circumferential indentation in the
skin, deep circumferential cleft, distal deformity
such as lymphedema, distal acrosyndactyly, and
complete digit or limb reduction (Fig. 1ac).
Moderate to severe constriction bands cause
brous scar formation that can lead to distal vas-
cular, lymphatic, and neural impairment. Temper-
ature gradients have been measured across
constricting bands due to vascular insufciency
across the band (Flatt 1977). Initially mild distal
symptoms may progress as the child grows. When
the bands are at the level of the digits, nail defor-
mity is often present (Moses et al. 1979). With
bands proximal to the wrist crease, neuropraxia or
more severe nerve injury can result. A study on
45 patients with CBS found that 23 % had sensory
decits (Moses et al. 1979). Of note, every patient
with a sensory decit in that series also had a
notable temperature gradient (average 2.4
Celsius) at the site of the constriction ring.
Multiple cases of mild to substantial ulnar,
median, and radial nerve compression have been
reported (Weeks 1982; Tada et al. 1984; Uchida
and Sugioka 1991; Light and Ogden 1993;
Weinzweig and Barr 1994; Jones et al. 2001;
Beidas et al. 2010). A careful neurological exam
is mandatory, albeit it is difcult to perform in
young children. Sensory and even mild motor
disturbances are challenging to diagnose in
infants and are probably underreported. It is
G. Gogola
helpful to look for subtle signs or abnormal resting
posture, such as an intrinsic minus position and
inability to extend the nger interphalangeal
joints, denoting an ulnar nerve palsy, or wrist
drop with inability to extend the metacarpo-
phalangeal joints, denoting a radial nerve lesion.
Depending on the timing of the onset of band
formation in utero, nerve compression may
already have been present for a considerable num-
ber of months on a childs rst day of life. Jones
reported on a case where a deep constriction band
causing ulnar nerve compression was noted on an
18-week ultrasound. That meant that when the
infant was born at term, the ulnar nerve palsy
had actually been present for 5.5 months (Jones
et al. 2001). Early recognition of nerve dysfunc-
tion associated with constriction bands is
extremely important. Careful preoperative assess-
ment will guide subsequent reconstruction.
CBS should be distinguished from
symbrachydactyly and transverse deciency.
Symbrachydactyly is typically unilateral and
affects the entire hand. The hand is small, yet the
distal phalanges have formed ngernails, as ecto-
dermal development progresses despite mesoder-
mal disruption (Moran et al. 2007).
Symbrachydactyly may occur with Polands
sequence and other conditions of vascular insuf-
ciency (Ogino and Saitou 1987). The presence of
bands on multiple other sites in an asymmetric
pattern distinguishes CBS. Single digits can be
18 Constriction Band Syndromes
Fig. 3 Clinical photograph of a constriction band causing
acrosyndactyly. Black arrows show epithelialized sinus
tracts between the syndactylized ngers (Courtesy of
Shriners Hospitals for Children, Houston)
affected in isolation in CBS, and even when the
digits appear normal distal to a phalangeal band,
the nails are often absent or abnormal. The
acrosyndactyly seen in CBS is different from a
complex syndactyly resulting from failure of dif-
ferentiation. In CBS, it is felt that normal digital
separation via apoptosis has already occurred
when an abnormal force results in distal fusion.
This is supported by the ndings of epithelialized
patent sinus tracts between digits, lack of bony
fusion, and the involvement of nonadjacent digits
as well as adjacent ones (Fig. 3).
Transverse limb deciency, a failure of forma-
tion, is also typically unilateral, with the proximal
forearm being the most commonly affected site, as
opposed to the ngers in CBS. The proximal limb
is abnormal. Transverse deciencies in the digits
often take the form of disarticulations with distal
soft tissue pockets, while constriction band reduc-
tions occur at the level of the phalangeal midshaft
417
with tapering bone stumps (Ogino and Saitou
1987; Kallemeier et al. 2007) (Fig. 4a, b). Both
symbrachydactyly and transverse deciencies can
have terminal structures such as nubbins, rudi-
mentary nger nails, clefts, and pits (which repre-
sent anomalous muscle attachments to the skin)
(Kallemeier et al. 2007) (Fig. 5a, b). In CBS
severe enough to result in an intrauterine amputa-
tion, no such distal structures are present at all.
While symbrachydactyly and transverse limb
deciency are currently classied as different enti-
ties, it is likely they represent different points
along a single continuum (Kallemeier
et al. 2007), distinct from CBS.
Relevant Anatomy
The anatomy relevant to surgery depends on the
area of the body affected by the constriction
bands. Most bands are supercial, and only the
skin and subcutaneous tissue are involved. It is
important to note that the neurovascular bundle
may be displaced superiorly near areas of
banding. Severe constriction bands can cause
neurovascular and lymphatic compromise that
distorts the anatomy distal to the constriction
point.
It is a hallmark of CBS that the anatomical
structures proximal to the constriction band are
completely normal. This is true of limb reductions
resulting from CBS as well, highlighting the
importance of differentiating an intrauterine
amputation from CBS from congenital limb de-
ciency. The reliable presence of normal proximal
anatomy is an extremely important point for sur-
gical planning. But is the proximal anatomy truly
completely normal? Recent studies have scruti-
nized this anatomy more closely. Satake
et al. noted metacarpal hypoplasia in 30 % of
hands with constriction band amputations at the
mid proximal phalanx level (Satake et al. 2012). A
small study of upper and lower limb vascular
anatomy in CBS found an incidence of proximal
vascular abnormality ranging from 100 % in deep
bands and limb reductions to 11 % in supercial
bands (Daya and Makakole 2011).
418 G. Gogola

Fig. 4 (a, b): Radiographic comparison between adactyly
due to constriction band syndrome and failure of forma-
tion. (a) CBS. The digital reduction occurs at the
mid-phalanx level, with tapered distal bone segment and
no nubbins or excess soft tissue distally. Note also that only
the index and long ngers are affected. (b) Failure of
formation. The digital reduction occurs at the MP joint
level, with metacarpal dysplasia and distal soft eshy
skin nubbins. Note that the entire hand is uniformly
affected (Courtesy of Shriners Hospitals for Children,
Houston)

Fig. 5 (a, b): Examples of transverse limb deciencies with rudimentary nubbins and clefts (Courtesy of Shriners
Hospitals for Children, Houston)
18 Constriction Band Syndromes
Imaging
With advancements of ultrasound technology,
constriction bands, limb deformations, and many
skeletal anomalies can be visualized as early as
the start of the second trimester. Prenatal diagno-
sis of atypical facial clefting should alert clini-
cians to the possibility of CBS, which can help
in prenatal counseling and family preparation.
Limb-threatening constriction bands can be diag-
nosed and theoretically treated, prior to progres-
sion to intrauterine amputation (Soldado
et al. 2009). Doppler ultrasound has demonstrated
restoration of blood ow to a threatened limb after
fetal intervention (Moran et al. 2007). However,
intrauterine surgery is not without risks to fetus
and mom. Postnatally, plain radiographs of the
affected hand are helpful in preoperative planning
when acrosyndactyly is present; otherwise, imag-
ing is not routinely utilized.
Classification
Given the wide variation of clinical presentation
where no two patients will have the exact same
pattern, number, or severity of constriction bands,
CBS is difcult to classify. While multiple classi-
cation systems have been proposed, none have
been universally adopted. The two most helpful
classications are descriptive one is based on the
depth of band involvement, and the other is based
on the severity of the deformity distal to the band.
Isacsohn divided constriction bands into ve
groups depending upon the depth of band involve-
ment (Isacsohn et al. 1976). In group 1, the band is
a shallow groove in the skin; in group 2 the band
involves the subcutaneous tissue and muscle; in
group 3 the band extends to bone; in group 4 there
is bony pseudarthrosis; and in group 5 an intra-
uterine amputation occurred. This classication
highlights the fact that increasing depth of band
involvement results in increasing severity of distal
lymphatic, vascular, and neural compromise.
The most widely quoted classication system
is the Patterson classication, which is based on
419
Table 1 Patterson classification for constriction bands
Group 1 Simple constriction rings
Group 2 Constriction rings with distal deformity
and/or lymphedema
Group 3 Constriction ring with acrosyndactyly
I Distal fusion with well-formed web
spaces
II Distal fusion with incomplete web
space formation
III Distal fusion with sinus tract between
digits; no web space
Group 4 Intrauterine amputation
the pattern of deformity distal to the constriction
band (Patterson 1961) (Table 1).
Treatment Options
Nonoperative Management
Nonoperative management is appropriate for the
two ends of the constriction band spectrum the
very mildly affected and the limb or digital ampu-
tations. Supercial bands without lymphedema or
neurological compromise require no operative
treatment. The prognosis for isolated supercial
extremity bands is excellent with no functional
decits, although there can be aesthetic concerns.
For patients with digital amputations, careful
assessment of their hand function is required. If
the function of the hand is acceptable, no treat-
ment is needed (Wiedrich 1998; Kawamura and
Chung 2009). It is better to adapt tools and equip-
ment to t the childs needs than to undertake
complex reconstruction that cannot result in a
normal hand and will still require adaptive equip-
ment (Fig. 6a, b).
Operative Treatment
Indications
Acute vascular compromise, severe lymphedema,
and distal nerve compression are denite indica-
tions for surgical intervention. The presence of an
420
Fig. 6 (a, b): Example of modication of sports equip-
ment. (a) The patients ngers are too short to keep a
standard baseball glove from falling off his non-dominant
left hand when he catches a ball. (b) The volar wrist
Fig. 7 (a, b): Preoperative
(a) and postoperative (b)
views of asymptomatic
constriction bands treated
electively to improve the
contour of the ngers
(Courtesy of Shriners
Hospitals for Children,
Houston)
acrosyndactyly prompts reconstruction for func-
tional improvement as well as for the prevention
of deformity with subsequent digital growth. Mild
to moderate bands that were initially asymptom-
atic should be reevaluated for surgical interven-
tion if there is a change, such as onset of cold
intolerance or deformity with growth. The desire
for a more aesthetic contour to the limb or digit is a
valid indication to excise a constricting band
(Fig. 7a, b); however, purely aesthetic excision
should be avoided if there is a tendency for hyper-
trophic scar formation. Complex reconstruction
G. Gogola
component, stitched into the glove, provides an alternative
mechanism of support to keep the glove on his hand while
catching a ball (Courtesy of Shriners Hospitals for
Children, Houston)
should be avoided if no clear functional benet
can be expected.
Timing of Surgical Intervention
In the rare cases where an infant is born with
constriction bands causing acute vascular com-
promise or severe distal edema, the bands should
be released within the rst hours or days of life as
a limb-sparing procedure (Fig. 8). Nerve and dis-
tal soft tissue reconstruction is performed in a
subsequent second stage. In the majority of
cases, the limb is adequately perfused and the
18 Constriction Band Syndromes
Fig. 8 Infant with a deep constriction band around the
proximal left arm. The band was urgently incised shortly
after birth as a successful limb-sparing procedure. In sub-
sequent staged procedures, the band was fully excised and
nerve grafting performed. The lymphedema resolved and
protective sensation to the hand was achieved, but there
remains minimal motor function of the wrist and hand.
Note the presence of bilateral clubfeet (undergoing casting)
(Courtesy of Shriners Hospitals for Children, Houston)
timing of constriction band excision is determined
by the depth of the band and the severity of distal
deformity. The presence of distal lymphedema
signals venous or lymphatic obstruction, or both.
These bands should be excised within the rst
3 months of life to allow for resolution of the
edema and prevent secondary brosis. Clinical
evidence of nerve dysfunction should also prompt
intervention as early as possible, preferably within
the rst 3 months of life. Depending on the onset
of the limb constriction in utero, nerve compres-
sion could already be of several weeks or
months duration on the day of birth, which
increases the urgency of band excision to release
nerve compression. If acrosyndactyly is present,
reconstructive surgery is recommended between
6 months and 1 year of age to allow for untethered
normal longitudinal bone growth. When the con-
striction is minor and the surgical goals purely
aesthetic, the procedure may be performed at any
age. In these elective cases, many surgeons
choose to wait until the child is several years of
age and their baby fat has dispersed. If a child has
other conditions that also require treatment within
a certain time frame, such as cleft lip or palate or
clubfeet, it is imperative to communicate with the
421
other surgeons regarding the timing of surgeries.
If possible, procedures should be scheduled so as
to maximize treatment during each anesthetic
exposure.
Constriction Band Excision
Preoperative Planning
Constriction band excision is performed under
loupe magnication, with the patient positioned
supine and the affected limb on a pediatric hand
table if one is available. An appropriately sized
pediatric tourniquet is required. The location of
the band on the limb determines whether a sterile
or nonsterile tourniquet is needed. Deep rings
with neurovascular compromise may require
nerve excision and grafting, necessitating micro
instruments, appropriate suture or brin glue, and
preoperative selection of nerve graft.
Deep bands with distal edema are best released
in two stages, with only 50 % of the band initially
excised. An interval of 612 weeks before the
remaining band is excised allows the cutaneous
circulation to reestablish and the scar to soften
(Kawamura and Chung 2009). Bands in series
are also treated in a staged manner if the Z-plasties
for each band would interfere with each other
(Upton and Tan 1991). Proximal bands, bands
requiring nerve exploration or grafting, deep
bands without lymphedema, and supercial
bands in any location may be safely excised in a
single stage (Hall et al. 1982; DiMeo and Mercer
1987).
As with all congenital hand cases, preoperative
counseling of the family is imperative. The dis-
cussion of treatment goals and realistic expecta-
tions depends upon the severity of the deformity
and decits. If there is a severe constriction with
considerable nerve palsy, full muscle recovery
may not occur. An acrosyndactyly with short
digits and cluster of bulbous terminal tips will
not look normal after syndactyly separation,
although it will result in a much more functional
hand. The family should be advised that second-
ary procedures may be necessary as growth
occurs.
422 G. Gogola

Technique intervening band must be entirely removed to

The most widely used technique is that of Upton
and Tan, which entails complete band excision,
mobilization of the subcutaneous tissue as a sep-
arate layer, and Z-plasty skin closure (Upton and
Tan 1991) (Table 2). An appropriate-sized tourni-
quet is inated after gentle or no exsanguination
so as to better visualize the vascular structures. A
transverse incision is made on either side of the
constriction band at the point the skin begins to
invaginate into the cleft (Fig. 9a, b). The
Table 2 Surgical steps
Incise skin at point of invagination proximal and distal to
cleft
Completely excise skin within cleft
Release all deep brous constrictive tissue
Specically decompress neurovascular structures if band
severity warrants
Mobilize subcutaneous layer proximal and distal to cleft
Incise skin for Z-plasties
Close subcutaneous layer separately
Transpose Z-plasty aps and close skin
avoid simply repositioning the abnormal tissue
and leaving a residual defect. All abnormal brous
tissue must be excised, going as deep as necessary.
Any deep fascial constriction is released. Skin
aps are mobilized proximally and distally. The
subcutaneous tissue is mobilized from the skin
above and the tissue below (Fig. 9c). Debulking
of the distal segment may be necessary, especially
when bulbous deformation is present.
Neurovascular bundles should be examined
and any persistent fascial bands or points of com-
pression released. Nerves affected by severe
bands may require more than external neurolysis.
The preoperative clinical assessment, the appear-
ance of the nerve upon visual inspection, and
(if available) intraoperative electrical stimulation
of the nerve across the compression site all aid in
deciding if excision of the compression point is
indicated, along with primary repair or nerve
grafting. Care should be taken to preserve subcu-
taneous veins to prevent postoperative venous
congestion. The mobilized proximal and distal
subcutaneous tissue is advanced across the band
constriction site and sutured as a separate layer

Fig. 9 (ae): Cross-

sectional drawings of
surgical excision of
constriction bands. (a) Side
view of a band at the mid-P2
level. (b) The dotted lines
represent the point at which
the skin invaginates into the
cleft, which is where the
circumferential incisions
proximal and distal to the
band should be made. All of
the skin between the dotted
lines, as well as the
underlying constrictive
tissue (marked with small
xs), must be excised. (c)
The subcutaneous layer is
mobilized and sutured
across the cleft. (d) The skin
is incised and the Z-plasty
aps mobilized. (e) The
Z-plasty aps are
transposed and sutured
without tension
18 Constriction Band Syndromes
(Fig. 9c). This step corrects the contour deformity
caused by the band. The overlaying skin is then
incised in Z-plasties (Fig. 9d), which are trans-
posed and closed without tension using 4-0 or 5-0
absorbable suture (Fig. 9e). Excess skin is
trimmed as needed during skin closure. Serial
Z-plasties are made circumferentially at the level
of the arm or forearm. In the digits, the Z-plasties
can be placed specically over the lateral borders
of the digits to minimize visible dorsal scarring.
Sterile, non-constricting dressings are applied,
and a splint is applied. For infants and toddlers,
the splint should not only immobilize the opera-
tive site but also control the elbow in 90 of
exion to make it more difcult for the patient to
wriggle out of the splint.
While the technique detailed above is the most
widely and commonly used, various modications
of varying complexity have been proposed to try to
optimize the aesthetic outcome. These variations
include creating subcutaneous dermofat aps and
closing the skin with rectangular-shaped aps
(Mutaf and Sunay 2006), notched triangular aps
(Tan and Chiang 2011), and linear circumferential
skin closure after aggressive excision of all
constricting tissues (Habenicht et al. 2013).
Postoperative Care
The postoperative splint is removed after 1014
days. Postoperative care consists primarily of
standard scar management beginning after the
incisions are completely healed and the sutures
resorbed. Parents are taught to massage the scar
daily with lotion. Silicone gel sheets or elastomer
molds are placed over the incisions nightly for
3 months. Patients are encouraged to avoid sun
exposure to the surgical site for 36 months. The
goal is to keep the resultant scars soft, pliable, and
at. Once the Z-plasties are healed, there are no
restrictions and patients resume activities to their
own tolerance. Some residual edema may persist,
especially with deep bands or after the rst stage
in planned two-stage approach. In these cases
compressive elastic bandage wrapping or a com-
pressive sleeve for 46 weeks facilitates edema
resolution.
423
Outcomes
Most authors report high success rates after con-
striction band excision, meaning that the constric-
tion deformity does not recur and that patients and
families are satised with the appearance of the
limb (Moses et al. 1979; Upton and Tan 1991;
Foulkes and Reinker 1994; Wiedrich 1998;
Kawamura and Chung 2009) (Fig. 10a, b).
Habenicht et al. reviewed their outcomes for lin-
ear circumferential skin closure without
Z-plasties. After 6.5-year follow-up, they found
no recurrence or scar constriction with growth and
noted that there was a more aesthetic scar appear-
ance than that obtained with Z-plasties. It is
important to note that they emphasized radical
excision of all constricting tissues, multiple lon-
gitudinal fasciotomies, and mobilization of the
subcutaneous layer prior to closure (Habenicht
et al. 2013). These points may differentiate their
technique from the simple circumferential skin
closure that consistently resulted in scar contrac-
ture and recurrence of the invagination deformity.
Even the change to simple W-plasty and Z-plasty
closures tended to indent over time as commonly
reported in the 1940s, leading to Upton and Tans
modication of mobilizing the subcutaneous tis-
sue as a separate layer prior to skin closure (Upton
and Tan 1991). No specic outcomes measuring
dexterity, function, or scar qualities have been
detailed. Many patients with deep rings complain
of some cold intolerance after band excision,
although the abnormal appearance of those digits
after cold exposure tends to be more concerning
than the sensation of cold intolerance itself (Upton
and Tan 1991). Pain has not been reported as a
complaint after constriction band excision.
When looking specically at outcomes in
patients with recognized neurological compro-
mise preoperatively, simple early release alone
between 3 weeks and 60 months of age does not
result in neurological improvement in long-term
follow-up studies (Jones et al. 2001). In a sum-
mary of 12 cases of peripheral nerve motor palsy
with CBS reported in 6 papers, 83 % of patients
did not show any neurological improvement after
band release and neurolysis of the involved nerve
or nerves (Jones et al. 2001). Weinzweig and Barr
424
Fig. 10 (a, b): (a) Clinical
photograph showing a
constriction band of the
forearm. (b) 2 years post
band excision (Courtesy of
Shriners Hospitals for
Children, Houston)
reported on a case with a deep band at the distal
1/3 of the humerus with no distal edema and
complete radial and incomplete median and
ulnar nerve palsies. The child was treated at
14 weeks of age with single-stage complete band
excision, nerve decompression and neurolysis,
and Z-plasties. He underwent serial EMGs that
showed improvement in nerve conduction out to
19 months postoperatively. There was clinical
improvement though not resolution noted at
4 years postoperatively (Weinzweig and Barr
1994). In another report, 3 of 4 patients with
constriction bands proximal to the wrist had
peripheral nerve palsies not improved by surgical
decompression (Uchida and Sugioka 1991).
Acrosyndactyly Separation
Preoperative Planning
Dobyns taught that the number of ngers is not as
important as their length, bulk, stability, spacing,
and control. The surgical plan for separation of
G. Gogola
distally fused digits is guided by the appearance
and severity of the deformity. It is helpful to
consider these hands in stratied groups by sever-
ity, much like the subtypes of Group 3 in
Pattersons classication (Table 1). For well-
formed digits with good web spaces of the proper
depth, the separation is fairly straightforward.
Narrow skin bridges between digits can be
divided; full-thickness skin grafts are used to
cover interdigital skin defects. A greater amount
of distal deformity presents more of a challenge.
Nonadjacent ngers may be fused together. The
digits may not be side by side in a single plane, but
rather stacked on top of each other. It may be quite
difcult to sort out which distal tip goes to which
digit. Decisions should be based upon resulting
length and stability. Preservation of the distal tips
is preferable over amputation to preserve length.
The most involved type are the hands with short,
single-phalanx digits fused together with no web
space, only an epithelialized sinus tract between
ngers. The sinus tract is typically distal to the
level of the proper web space. These hands often
present with a deep band and multiple bulbous
18 Constriction Band Syndromes
Fig. 11 Example of a right hand with constriction band
resulting in an acrosyndactyly with multiple bulbous nub-
bins. The thumb, which is unaffected, is on the right side of
the photograph, exed at the IP joint (Courtesy of Shriners
Hospitals for Children, Houston)
deformities that look like a bunch of grapes
(Fig. 11). These digits should be separated in
staged fashion so as to not operate on adjacent
web spaces at the same time. It may not be prudent
or even possible to try to preserve all of the distal
tissue. It is best to use the portions of the nubbins
that provide the best soft tissue coverage over the
tapered bone ends, and delete the oppy,
unsupported nubbins (Light and Ogden 1993).
The surgical goal is separation of the digits and
creation of an adequately deep web space
(Fig. 12ac). These children may benet from
later secondary procedures to further deepen the
web spaces or lengthen the existing digits.
Technique
Any standard technique to separate a syndactyly
may be used. Although the complicated
acrosyndactyly can be more demanding, all of
the established principles for syndactyly separa-
tion apply. The ngers are separated using
Z-plasty aps, with care taken to identify and
425
protect the digital neurovascular bundles.
The existing sinus tract is excised, as it is typically
located too distal and is too narrow to be a good
commissure (Miura 1984). A dorsal skin ap is
raised to create a broad web space. The creation of
eponychial aps is often not necessary as the
distal tips do not have ngernails. The digits are
defatted to aid in closure. The skin Z-plasties are
mobilized and sutured with ne absorbable
suture. Full-thickness skin graft is used to cover
the remaining open interdigital areas. Sterile
conforming dressings and a protective splint are
applied.
Postoperative Care
The postoperative splint is removed to check the
skin grafts at 1 week and then reapplied for 12
additional weeks. Once the skin grafts and
Z-plasties are completely healed, full mobilization
is permitted. There are no restrictions, and patients
resume their typical activities to their own toler-
ance. Contoured elastomer molds are used in the
web spaces nightly for 3 months.
Reconstructive Procedures for Digital
Deficiency
The management of intrauterine digital amputa-
tions is focused on optimizing hand function,
especially power grasp and precision pinch.
Improving aesthetic appearance is a secondary
goal, and while worthwhile, noteworthy improve-
ments are more difcult to achieve as the hand
will still appear different. Fortunately, thumb is
often spared, likely due to its shorter relative
length or protected in-palm fetal position. Many
procedures for thumb and nger augmentation or
reconstruction have been described. As is often
the case, the simplest procedures are the most
benecial in this population. Web space deepen-
ing effectively lengthens the digits and has been
shown to improve function (Miura 1984). Meta-
carpal distraction lengthening, particularly of the
thumb, is another useful and reliable technique to
achieve functional improvements. This is best
426
Fig. 12 Clinical photograph of a patient with a constric-
tion band of the ring nger and an acrosyndactyly with
digital deciency of the index and long ngers. (a)
reserved for patients over 8 years of age (Miura
1984; Ogino et al. 1994; Kawamura and Chung
2009), as the success rate is higher in older chil-
dren than in infants and toddlers (Wiedrich 1998).
Non-vascularized phalangeal toe transfers are
rarely indicated in CBS, because, unlike
symbrachydactyly, the terminal digits do not
have a distal soft tissue envelope to accommodate
a toe phalanx (Fig. 4a, b). Also unlike other con-
genital limb deciencies, the anatomic structures
proximal to the constriction band are reliably pre-
sent and well formed. That makes CBS the best
congenital indication for a toe-to-hand transfer,
particularly in the case of a thumb amputation
proximal to the metacarpal-phalangeal joint with
G. Gogola
Preoperative, (b) immediate postoperative result, and
(c) 4-year follow-up (Courtesy of Shriners Hospitals for
Children, Houston)
intact thenar musculature (Kawamura and Chung
2009).
Management of Complications
Table 3 lists common surgical pitfalls and how to
avoid them. The most worrisome complication of
constriction band excision and/or acrosyndactyly
release is vascular compromise of the digits. The
risk can be minimized by being meticulous in the
surgical dissection of the different tissue layers
and working under loupe magnication. Gentle
or no exsanguination before inating the
tourniquet allows better visualization of the dorsal
18 Constriction Band Syndromes 427

Table 3 Potential pitfalls and prevention

Potential pitfall Pearls for prevention
Injury to Recognize that the neurovascular
neurovascular bundle may be displaced near
structures areas of banding
Use loupe magnication
Unrecognized Release all constricting tissue,
nerve compression including deep to band itself
Skin ap necrosis Close skin without tension
Avoid tight circumferential
dressings
Lack of adequate Deate tourniquet prior to nal
hemostasis skin closure to conrm
hemostasis
Shear stress on graft Good splint immobilization

veins for preservation. It is important for skin ap

and graft viability to achieve a tension-free skin
closure. Postoperative venous congestion can be
improved by judiciously removing one or several
sutures. The postoperative dressings and splint are
quite important and can be challenging. Tight
circumferential dressings must be avoided, but
the dressings and splint need to adequately immo-
bilize the limb. The treatment for necrosis of the
skin aps of full-thickness skin grafts depends
upon the age of the patient and particularly upon
the size of the area affected. Allowing healing by
secondary intention works well with small areas
and younger patients. Large areas of loss require
repeat grafting. Similarly to other types of syn-
dactyly, web creep is common with later growth
spurts and is treated with web space deepening.
As expected, very deep bands and multiple adja-
cent bands are the most difcult to correct and
most prone to develop complications.
Summary
Future research on CBS includes continued inves-
tigation into the etiology of the condition, an
ongoing question since the 1930s. There may
well be a multifactorial etiology resulting in a
spectrum of clinical presentation. The most inter-
esting thing on the horizon is surgical treatment of
severe constriction bands prenatally. The eld of
fetal surgery has advanced to the point of
Fig. 13 Clinical photograph of a 5-month-old infant with
a residual constriction band at the wrist after surgical
release in utero at 23 weeks gestation (Courtesy of Shriners
Hospitals for Children, Houston)
expanding the indications for fetal surgical treat-
ment from life-threatening conditions to limb-
threatening conditions. With the improvements
in prenatal ultrasound, it is possible to monitor
limb perfusion distal to a visible band as well as
evaluate the potential for nerve compression. The
goal of in utero treatment is to release a severe
band with nerve or vascular compromise,
preventing an impending limb amputation and
allowing for normal in utero growth and develop-
ment of the limb. Bands may not be completely
resolved after fetal intervention; however, the
postnatal outcome can be converted from a limb
deciency to a reconstructable functional limb
(Soldado et al. 2009) (Fig. 13). Very real risks,
such as premature rupture of membranes, preterm
labor, chorioamnionitis, and fetal loss, have not
yet been reduced to the point of fetal surgery being
the mainstream treatment for CBS
(Crombleholme et al. 1995; Quintero et al. 1997;
Sentilhes et al. 2004).
428
In summary, constriction band syndrome has a
wide spectrum of clinical presentation. Manage-
ment is focused on improving function, facilitat-
ing normal development, and providing better
aesthetic appearance. The treatment plan must be
specic to each individual, as no two affected
children will look exactly the same, making CBS
a creative challenge to treat. Timing of initial
reconstruction is dependent on the presentation
and symptoms. All patients with constriction
band syndrome should be serially monitored
until skeletal maturity for secondary issues such
as recurrent deformity and web creep.
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 Metabolic and Endocrine
Abnormalities 19
Krister Freese and Arabella Leet

Contents Pathophysiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 439

Clinical Features . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 439
Osteogenesis Imperfecta . . . . . . . . . . . . . . . . . . . . . . . . . . . 432 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 440
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 432
Pathophysiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 432 Osteopetrosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 440
Diagnostic Evaluation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 433 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 440
Medical Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 433 Pathophysiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 440
Surgical Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 434 Clinical Features . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 440
Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 441
Rickets . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 435
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 435 Infantile Cortical Hyperostosis
(Caffey Disease) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 442
Vitamin D Deciency Rickets . . . . . . . . . . . . . . . . . . . . . . 435 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 442
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 435 Pathophysiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 442
Pathophysiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 435 Clinical Features . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 442
Clinical Manifestations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 435 Medical Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 443
Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 436
Sclerosteosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 443
Hypophosphatemic Rickets . . . . . . . . . . . . . . . . . . . . . . . . 437 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 443
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 437 Pathophysiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 444
Pathophysiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 437 Clinical Features . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 444
Clinical Features . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 437 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 444
Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 438
Camurati-Engelmann Disease . . . . . . . . . . . . . . . . . . . . . 445
Vitamin D-Dependent Rickets Type I . . . . . . . . . . . . . 438 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 445
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 438 Pathophysiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 445
Pathophysiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 438 Clinical Features . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 445
Clinical Features . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 439 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 446
Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 439
Melorheostosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 446
Vitamin D-Dependent Rickets Type II . . . . . . . . . . . . 439 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 446
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 439 Pathophysiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 447
Clinical Features . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 447
Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 447
K. Freese (*) Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 449
Division of Orthopaedic Surgery, University of Hawaii,
Honolulu, HI, USA References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 449
e-mail: krister_f@yahoo.com
A. Leet
Department of Orthopaedic Surgery, Shriners Hospital for
Children-Honolulu, Honolulu, HI, USA
e-mail: aleet@shrinenet.org

# Springer Science+Business Media New York 2015 431

J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_19
432
Abstract
The evaluation and management of endocrine
and metabolic bone diseases affecting the
upper extremity is complicated by the rarity
of these entities and the lack of evidence to
guide treatment. In general, large studies with
high-quality evidence about the treatment of
the diseases covered in this chapter are not
available. However, upper extremity surgeons
who treat children may encounter the diseases
described below. Many of these patients will
have functional limitations. Without further
exploration, it will continue to be difcult to
optimize the treatment of patients with meta-
bolic and endocrine abnormalities affecting the
upper extremity.
Osteogenesis Imperfecta
Introduction
As a general rule, patients with the more severe
phenotypes of osteogenesis imperfecta (OI) have
more upper extremity deformity than those with a
milder burden of disease (Amako et al. 2004).
Upper extremity surgical correction for deformity
or fracture is performed much less frequently than
lower extremity surgery (Amako et al. 2004; Root
1981; Leet et al. 2013); however, children with
upper extremity deformity show decreased func-
tion in the domains of self-care and mobility based
on pediatric outcome tools. The idea that upper
extremity surgery can improve function in
patients with OI is tantalizing but has yet to be
conclusively proven.
Pathophysiology
Osteogenesis imperfecta is a hereditary disorder
caused by many different gene mutations. They
are most commonly found in the gene for type
1 collagen. Bone samples from OI patients show a
high bone turnover state with histology showing a
marked increase in osteoclasts (Glorieux
et al. 1998). Microfractures can cause the bone
K. Freese and A. Leet
to bend and deform more slowly over time, while
acute fractures can lead to malunion and subse-
quent increased deformity (Leet et al. 2013).
Patients with osteogenesis imperfecta have a
clinical phenotype consisting of bone fragility
manifests by low bone mass, frequent fractures,
and skeletal deformity. Other features include
short stature, blue sclera, and wormian bones of
the skull. OI is classied by the Sillence classi-
cation, which initially was described as four clas-
sic phenotypes, of which type 3 was the most
severe. Recently, three new phenotypes have
been added to the Sillence classication scheme
based on distinctions in genetic testing and histol-
ogy (Amako et al. 2004). The number of muta-
tions for OI is over 300 and each year new
mutations are discovered. OI is inherited in an
autosomal dominant transmission pattern with
parents and children sharing the same mutation.
However, when comparing generations, a huge
difference in disease burden is revealed; thus,
genotype does not correlate well with phenotype
in this condition.
Upper extremity clinical ndings: In a study of
159 patients with osteogenesis imperfecta,
59 (37 %) were found to have upper extremity
involvement (Amako et al. 2004). Of those with
upper extremity involvement, 39 were found to
have a deformity of the humerus and 25 of these
39 patients (64 %) had bilateral humeral involve-
ment (Fig. 1). Deformity in the humerus was
almost always a varus deformity (95 %). Along
with the varus deformity, posterior bowing
occurred most commonly when the deformity
was multiplanar (Amako et al. 2004 and Root
1981).
Deformities of the radius and ulna occur less
frequently than deformities of the humerus with
each forearm bone involved in 18 % and 19 % of
patients, respectively (Amako et al. 2004). No
matter what upper extremity bone is studied, the
magnitude of angular deformity correlates with
the severity of the disease phenotype (Amako
et al. 2004); thus, the most pronounced upper
extremity deformities were seen in the patients
with type 3 OI.
In another study of 489 OI patients, radial head
subluxation or dislocation was found in 83 patients
19 Metabolic and Endocrine Abnormalities
Fig. 1 Right and left humeri of a patient with osteogenesis imperfecta
(17 %) (Fassier et al. 2007). Radial head defor-
mity correlated to severity of disease with the
radius dislocating either posteriorly or
posterolaterally. Patients with type V OI seem
particularly prone to radial head deformity
(Fassier et al. 2007).
Deformity of the upper extremity has func-
tional implications. In 159 children given the
PEDI outcome tool, mean self-care and mobility
scores were negatively correlated to the amount of
angular deformity as measured on the radiographs
(Amako et al. 2004). Despite this functional loss,
upper extremity surgery is performed much less
frequently than the correction of deformity in the
lower extremities of OI patients (Root 1981).
Diagnostic Evaluation
Osteogenesis imperfecta is most often diagnosed
by sequencing for gene mutations in patients who
have a suspicious phenotype. An avulsion fracture
of the olecranon has been described as being
pathognomonic for osteogenesis imperfecta
(Stott and Zionts 1993). Occasionally, skin bro-
blasts are obtained from biopsy specimens and
studied; however, this more traditional means of
433
making a diagnosis is required much less often as
the specic genetic makeup of OI is now better
understood. Imaging can include a skull lm for
wormian bones. Most OI patients are imaged pri-
marily with plain lms of areas of bone deformity.
Child abuse is often a possible diagnosis in chil-
dren with frequent fractures and distinguishing
children with brittle bones from those who are
subject to frequent trauma can be a difcult task.
Medical Treatment
Bisphosphonates are analogs of pyrophosphate that
bind to the hydroxyapatite crystals in the bone
matrix. Pamidronate, the rst bisphosphonate
used for the treatment of OI, has been shown to
decrease the number of fractures and increase bone
mass using meta-analysis techniques combining
data from multiple randomized clinical trial
(Phillipi et al. 2008). Bisphosphonates can have
some serious toxicity and clinical trials continue
to be conducted to determine when to initiate treat-
ment, what is the optimal dosage and route of
administration, and how much total drug should
be given. Among reported complications of
bisphosphonates is an increase in nonunion at the
434
Fig. 2 Postoperative humerus radiograph after
intramedullary rodding
site of osteotomies, but not an increased rate of
fractures (Munns et al. 2004). Therefore, thought-
ful planning as to the administration of a
bisphosphonate around the time of surgery should
be discussed as a team and perhaps the drug held
until there is radiographic callus visible at the
osteotomy site.
Surgical Treatment
Humeral rodding can be approached by an
anterolateral incision over the apex of the main
deformity. The radial nerve must be identied
and protected and in cases of severe deformity
may not be in the expected anatomic location.
Multiple osteotomies may be required to
straighten the segment. A telescopic or solid rod
can be placed from proximal to distal (Fig. 2).
The rod should be directed down the medial
column of the distal humerus, if possible, to
account for the recurrence of varus with growth
of the patient (Fig. 3; Root 1981). Postoperative
K. Freese and A. Leet
Fig. 3 Recurrent varus deformity after humeral rodding
management consists of a sling and swathe that
can be removed when the patient is comfortable.
As in lower extremity treatment, the need for
additional rodding procedures is often necessary
as the deformity tends to return over time, partic-
ularly in growing children (Amako et al. 2004;
Root 1981).
Rodding the forearm can be difcult as the
patients who require this procedure are likely to
have severe deformity coupled with canals that
are so small and deformed that placing a rod inside
the canal is inherently challenging. Rod choice is
limited to solid rods in the forearm. Root
et al. suggest osteotomies of the radius and ulna
with rodding of the ulna only (Root 1981). The
forearm should be protected in a splint for a few
weeks after surgery. In several series, rodding of
the bones of the forearm was found to have poor
results and caution was advised when considering
the procedure. However, most of these articles
predate combined medical and surgical manage-
ment and newer studies are required to determine
if surgery is helpful once the biology of the bone is
altered.
19 Metabolic and Endocrine Abnormalities
Rickets
Introduction
Rickets is the result of decreased mineralization of
trabecular bone and is characterized by growth
retardation and bony deformity. Histologically,
an increase in the amount of unmineralized oste-
oid is the hallmark of the disease. Rickets is a
disease exclusively of childhood as it affects the
growing physis. Osteomalacia is the adult coun-
terpart and occurs after the cessation of growth.
There are a number of causes of rickets all of
which affect bone homeostasis. Bone mineraliza-
tion requires tight regulation of calcium and phos-
phate. As such, any number of disorders that
interrupt the homeostasis of calcium and phos-
phate may result in rickets. Historically, the most
commonly encountered form of rickets was the
result of vitamin D deciency. The most com-
monly encountered form of inherited rickets is
X-linked hypophosphatemic rickets. Other forms
of rickets occur as a result of vitamin D resistance,
defects in phosphate metabolism, and renal
abnormalities.
Vitamin D Deficiency Rickets
Introduction
Vitamin D deciency rickets has decreased in
incidence with the increased use of dietary vita-
min D supplementation. However, cases still
occur and can lead to considerable morbidity.
Infants who are exclusively breastfeed and
whom do not receive vitamin D supplementation
are at increased risk. In addition, children
living at northern latitudes, who have decreased
sun exposure either for cultural reasons or
because of geography, have darker skin, and
those fed a strict vegan diet are also at increased
risk. A recent increase in the use of sunblock
to prevent skin cancer has had as an
unintended consequence a decrease in vitamin D
production.
435
Pathophysiology
Vitamin D is obtained from two sources. Vitamin
D3, cholecalciferol, is produced in the skin after
sun exposure. Alternatively, vitamin D2,
ergocalciferol, is obtained from the diet. Both
cholecalciferol and ergocalciferol undergo similar
processes to achieve their active state and have
equivalent biologic potency. Initially, vitamin D is
hydroxylated in the liver to 25-hydroxyvitamin D.
25-hydroxyvitamin D undergoes a second
hydroxylation in the kidney to its active form,
1,25-dihydroxyvitamin D. 1,25-dihydroxyvitamin
D increases absorption of calcium from the gastro-
intestinal tract and increases resorption of calcium
and phosphorus from bone. The net effect is
increased serum calcium. As serum calcium levels
fall, parathyroid hormone (PTH) levels increase in
an effort to maintain normal serum calcium levels.
As a consequence of increased circulating PTH,
urinary excretion of phosphate increases and
serum concentrations of phosphate decrease. With
decreasing levels of calcium and phosphate, PTH
promotes calcium loss from the bone. As this sever-
ity of vitamin d deciency increases, rachitic bone
changes become readily apparent on radiograph
and clinical manifestations soon follow.
Clinical Manifestations
Vitamin D deciency rickets has a wide spectrum
of presentations. The most common piece of
patient history is failure to supplement vitamin D
during breastfeeding (96 %). A recent review of
cases of nutritional rickets over a 10-year period
found that 81 % of cases occurred in African
American patients, 14 % occurred in patients of
Arab descent, and only 2 % occurred in Caucasian
patients (Lazol et al. 2008). Most cases presented
during fall, winter, or spring, while only 14 % of
cases occurred during the summer months.
On evaluation, affected children may demon-
strate development delay, decreased growth, and
failure to thrive. 47 % and 31 % of patients are
below the fth percentile for height and weight,
respectively (Lazol et al. 2008). Genu varum of
the legs is not uncommon, occurring in 58 % of
436
Fig. 4 Typical radiograph
of the distal radius and ulna
in a patient with vitamin D
deciency rickets (Courtesy
of Michael Meagher, MD,
Shriners Hospital for
Children Honolulu)
patients. Other clinical ndings include frontal
bossing, rachitic rosary, scoliosis, ligamentous
laxity, and hypotonia. Patients may present with
seizures and tetany as a result of hypocalcemia
(Joiner et al. 2000). Vitamin D also plays a role in
ones ability to mount an immune response. An
increased rate of pneumonia in patients with
rickets has been observed (Muhe et al. 1997).
Other patients may come to clinical attention
after incidental radiographic ndings lead to a
diagnosis of rickets. The most common
presenting sign is wide swollen joints (74 %)
(Lazol et al. 2008).
Radiographic evaluation will demonstrate typ-
ical changes at the distal metaphyses of long
bones, most often the distal radius and ulna
(Fig. 4). These changes include metaphyseal wid-
ening, cupping, and fraying of the metaphysis
(Chavhan et al. 2010). Repeat radiographs as
soon as 23 weeks after the initiation of treatment
can demonstrate improvement. Other radio-
graphic features commonly seen include rib ar-
ing, widespread osteopenia, and multiple fractures
in various stages of healing. This last feature may
lead to a diagnostic dilemma with child abuse and
osteogenesis imperfecta also included in the
differential.
The diagnostic evaluation should include
serum calcium, phosphorus, PTH, and alkaline
K. Freese and A. Leet
phosphatase concentrations. Renal function
should be evaluated to rule out other causes of
rickets. Finally, 25-hydroxyvitamin D and 1,25-
dihydroxyvitamin D levels should be obtained.
The laboratory ndings will demonstrate
decreased 25-hydroxyvitamin D, normal or
increased 1,25-dihydroxyvitamin D, increased
PTH, and decreased calcium and phosphorus.
The alkaline phosphatase level will be increased
in 98 % of cases (Lazol et al. 2008).
Treatment
The rst priority of treatment of vitamin
D-decient rickets is to prevent the complications
of hypocalcemia, seizures and tetany. Initially,
treatment involves replacement of calcium. The
second goal of treatment is to replenish vitamin D
levels leading to improvement in calcium absorp-
tion from the gastrointestinal tract. Daily treat-
ment with vitamin D should be continued until
serum alkaline phosphatase levels have returned
to normal and radiographic evidence of disease
has resolved. During this time, oral calcium sup-
plementation is recommended. In general, the
prognosis for patients treated for vitamin D de-
ciency rickets is good. Bony deformities slowly
improve over the course of months to years.
19 Metabolic and Endocrine Abnormalities
In severe cases of genu valgum or varum, severe
upper extremity deformity, and short stature, the
deformities may not resolve. A review of the
literature has not produced evidence for the surgi-
cal management of patients with residual upper
extremity deformity.
Preventing rickets is an important and achiev-
able goal. Emphasizing the importance of vitamin
D supplementation to breastfeeding mothers can
decrease the incidence of rickets. The American
Academy of Pediatrics in its 2008 recommenda-
tions states that children from infancy to adoles-
cence should receive 400 IU of vitamin D daily. In
a recent study, only 1125 % of infants less than
11 months of age were meeting the daily require-
ment for vitamin D (Perrine et al. 2010). This
emphasizes the need for continued improvement
in vitamin D supplementation.
Hypophosphatemic Rickets
Introduction
Hypophosphatemic rickets is an inherited disease
characterized by rickets, increased renal clearance
of phosphate, and short stature. The disease is also
known as vitamin X-resistant rickets and is most
commonly inherited as an X-linked dominant
mode of transmission. The X-linked dominant
form of hypophosphatemic rickets accounts for
80 % of cases of hypophosphatemic rickets.
Hypophosphatemic rickets can also be inherited
as an autosomal dominant disease with reduced
penetrance and in a rare autosomal recessive form.
The disease occurs more frequently in females
(70 %) than males (30 %) (Bhadada et al. 2010).
The incidence of hypophosphatemic rickets is
1 per 21,000 people.
Pathophysiology
X-linked hypophosphatemic rickets is caused by
a mutation in the phosphate-regulating gene with
homologies to endopeptidases on the X chromo-
some (PHEX). In 87 % of familial cases of
hypophosphatemic rickets and 72 % of sporadic
437
cases, a mutation in the PHEX gene is found
(Gaucher et al. 2009). The mutation in PHEX
leads to its inactivation, which alters phosphate
homeostasis. Approximately 8085 % of phos-
phate is resorbed in the kidney (Santos et al.
2012). In its normal state, the PHEX gene prod-
uct decreases the synthesis of broblast growth
factor 23 (FGF-23). FGF-23 is produced by
osteocytes, osteoblasts, and odontoblasts. An
increased circulating level of FGF-23 is directly
responsible for both phosphaturia and decreased
1-alpha hydroxylation of 25-hydroxyvitamin D
(Aono et al. 2009). FGF-23 acts on the proximal
renal tubule to downregulate the sodium phos-
phate cotransporter. Interestingly, cortical bone
mineral density seems to be decreased while tra-
becular bone mineral density seems to be
increased in hypophosphatemic rickets (Cheung
et al. 2013).
Clinical Features
X-linked hypophosphatemic rickets has a mean
age at diagnosis around 1 year of age (Patzer
et al. 1999). The most common clinical features
of X-linked hypophosphatemic rickets are growth
disturbances causing disproportionate short stat-
ure, rickets leading to lower extremity deformi-
ties, and muscle weakness. Interestingly, the
serum phosphate levels are similar in males and
females. However, the bony deformities are more
severe in males. The growth abnormalities seen in
hypophosphatemic rickets are variable and cases
of normal adult height have been reported (Santos
et al. 2012). Leg length is more severely affected
than either trunk length or arm length (ivinjak
et al. 2010). At presentation, the average stature of
children with hypophosphatemic rickets is 2.48
standard deviations below controls. Arm length
and leg length are 1.81 and 2.90 standard devia-
tions below the normal population, respectively
(Zivicnjak et al. 2010). This disturbance in growth
appears to worsen during adolescence. The
patients may also have abnormalities of their
teeth. Many patients present with pathologic frac-
tures and approximately half of patients will have
bone pain.
438
The laboratory evaluation of patients with
X-linked hypophosphatemic rickets demonstrates
a number of abnormalities. Hypophosphatemia is
universally found. Inappropriately increased
levels of phosphate are found in the urine despite
the hypophosphatemia. The patients will exhibit
decreased or inappropriately normal levels of
1,25-dihydroxyvitamin D. FGF-23 levels are ele-
vated. Alkaline phosphatase may be elevated or
normal and serum calcium is usually normal.
Treatment
The treatment of X-linked hypophosphatemic
rickets is usually with high-dose oral phosphate,
calcium, and 1,25-dihydroxyvitamin D supple-
mentation. This approach will allow the rickets
and hypophosphatemia to improve. However, it
does not fully correct the growth abnormalities
and has been associated with nephrocalcinosis
and secondary hyperparathyroidism in a consid-
erable number of patients. Approximately 50 % of
treated patients develop nephrocalcinosis. This is
not seen in untreated patients (Kooh et al. 1994).
The hyperparathyroidism in hypophosphatemic
rickets can lead to a number of complications
including arterial hypertension, ectopic cardiac
calcication, and other cardiac abnormalities
(Schmitt and Mehls 2004).
Interestingly, treatment of hypophosphatemic
rickets with calcitriol and high-dose phosphate
leads to an increase in serum FGF-23 levels (Imel
et al. 2010). Treatment starting at younger ages has
been shown to improve the patients growth param-
eters at skeletal maturity and decrease the radio-
graphic ndings associated with rickets (Makitie
et al. 2003). In an effort to improve longitudinal
growth, treatment with growth hormone supple-
mentation has been attempted. A recent random-
ized controlled trial demonstrated increased growth
without progression of bowing deformities or body
disproportion in patients treated with growth hor-
mone (Zivicnjak et al. 2011).
The surgical treatment of hypophosphatemic
rickets is aimed at correction of deformities
K. Freese and A. Leet
caused by the disease. The majority of these
occur in the lower extremities. Early medical
treatment has been shown to decrease the need
for future surgical intervention (Evans et al.
1980). Of note, the ability to heal osteotomies
and fractures may be impaired in patients with
hypophosphatemic rickets. As in other metabolic
bone diseases, surgical intervention is best
preformed as near to skeletal maturity as possible
in order to decrease the likelihood of recurrence.
The recurrence rate after surgery has been
reported to be as high as 90 % (Petje
et al. 2008). Both the correction of angular defor-
mity and lengthening procedures have been suc-
cessfully performed (Fucentese et al. 2008). In
one small series of 8 patients undergoing 28 sur-
geries, there were no cases of nonunion
(Fucentese et al. 2008). Fassier-Duval rods have
been used in conjunction with Ilizarov frames to
correct lower extremity deformity in patients
with hypophosphatemic rickets. In a small series
of six operations, 50 % of cases had complications
(Birke et al. 2011). Although no series exist on the
treatment of upper extremity deformity in this
condition, experience with care of the
lower extremities speaks to the difculty
of treating bony deformity in patients with
metabolic bone disease and serves as a reminder to
undertake surgical intervention judiciously.
Vitamin D-Dependent Rickets Type I
Introduction
Vitamin D-dependent rickets type I (VDDR-I) is a
rare genetic disease caused by a mutation in the
1-hydroxylase gene. The disease is inherited in
an autosomal recessive fashion.
Pathophysiology
Patients with VDDR-I have an inherited defect in
vitamin D metabolism. 1,25-dihydroxyvitamin D
19 Metabolic and Endocrine Abnormalities
is the active form of vitamin D and is a key
regulator of calcium and phosphate metabolism.
The precursor to the active form of vitamin D is
made in the skin by the effect of ultraviolet light
on 7-dehydocholesterol. Subsequently, vitamin D
undergoes two successive hydroxylations to form
1,25-dihyroxyvitamin D. The rst occurs in
the liver and the second occurs in the proximal
renal tubule of the kidney. The genetic defect in
VDDR-I is most often a loss of function mutation
in the 1-hydroxylase gene. 1-hydroxylation
is the rate-limiting step in the formation of
1,25-dihydroxyvitamin D. Subsequently, patients
are unable to produce the active metabolite of
vitamin D. The 1-hydroxylase enzyme is a mem-
ber of the cytochrome P450 family.
Clinical Features
Patients with VDDR-I typically present with fail-
ure to thrive, muscle weakness, and symptoms of
hypocalcemia. They may present with seizures.
They often have skeletal changes typical of rickets
including genu varum or valgum, rachitic rosary,
and pathologic fractures. Finally, they may have
secondary hyperparathyroidism. As this is a
genetic disorder, the patients typically present in
infancy.
Laboratory ndings will allow the diagnosis to
be elucidated. The patients will have low or no
detectable serum 1,25-dihydroxyvitamin D with
normal to increased levels of 25-hydroxyvitamin
D levels. Calcium and phosphate levels will be
decreased. Conversely, parathyroid hormone
and alkaline phosphatase levels will be elevated.
Treatment
VDDR-I is treated with the replacement of
1,25-dihydroxyvitamin D in physiologic doses.
This has been shown to cause remission of the
disease including correction of the laboratory
parameters and reversal of the rachitic bony
changes.
439
Vitamin D-Dependent Rickets Type II
Introduction
Vitamin D-dependent rickets type II (VDDR-II) is
a rare autosomal recessive disorder caused by a
mutation in the vitamin D receptor. The disease
results in end-organ resistance to 1,25-
dihydroxyvitamin D. VDDR-II is further classi-
ed by the presence (type IIA) or absence of
alopecia (type IIB).
Pathophysiology
VDDR-II is caused by a mutation in the vitamin D
receptor gene found on chromosome 12 (Malloy
et al. 1990; Feldman et al. 1982). The disease is
inherited in an autosomal recessive fashion.
Several mutations have been described leading
to variability in the patients response to 1,25-
dihydroxyvitamin D. The most common muta-
tions result in a defective steroid binding domain
in the vitamin D receptor (Malloy et al. 1990).
These patients have no detectable 1,25-
dihydroxyvitamin D activity.
Clinical Features
The onset of disease is usually early in infancy.
The patients have a severe rickets diathesis and
present with severe bony changes and symptoms
of hypocalcemia. A distinguishing feature of
VDDR-II is the associated nding of total scalp
and body alopecia. This is seen in a signicant
proportion of patients but not universally.
Patients may be born with hair and later go on
to develop alopecia (Al-Khenaizan and Vitale
2003). Importantly, patients with alopecia
may have worse disease phenotype than those
without alopecia. The patients may also exhibit
signs and symptoms of immunodeciency (Soni
et al. 2008). Although the exact mechanism
linking active vitamin D and the hair follicle is
440
unknown, the vitamin D receptor is found in the
dermal papillae of the hair follicle as well as in
keratinocytes of the outer root sheath.
The diagnosis can be made based on the clin-
ical history and laboratory evaluation. The
patients have similar laboratory abnormalities as
VDDR-I with one exception. The sine qua non is
an elevated level of 1,25-dihydroxyvitamin D.
Treatment
The treatment of VDDR-II involves replacement
with either high-dose vitamin D analogs or cal-
cium supplementation (Kruse and Feldmann
1995). VDDR-II varies in its responsiveness to
vitamin D supplementation. In some cases, intra-
venous calcium replacement may be required to
reverse clinical symptoms. Interestingly, treat-
ment of VDDR-II does not reverse the associated
alopecia.
Osteopetrosis
Introduction
Osteopetrosis is an uncommon genetically
inherited disease resulting in abnormally dense
bone. The entity is also known as marble bone
disease or Albers-Schonberg disease. Its inci-
dence ranges from 1 in 250,000 live births in the
autosomal recessive form to 5 per 100,000 live
births in the autosomal dominant form. The auto-
somal recessive forms tend to result in more
severe disease and, if untreated, are fatal in
infancy.
Pathophysiology
Osteopetrosis varies substantially in severity
between different types of the disease. This vari-
ation is a reection of the multiple different
genetic etiologies. At least 10 different genes
have been implicated in the pathogenesis of
osteopetrosis. Mutations in T-cell immune regula-
tor 1, RANK, RANKL, CLCN7 (a chloride
K. Freese and A. Leet
channel), and carbonic anhydrase II, among
others, have been described (Michou and Brown
2011). All of these genes in one way or another
impact the function of osteoclasts, ultimately
leading to dysregulation of bone homeostasis.
Osteoclasts are multinucleated cells that originate
from mononuclear precursors. Loss of function
mutations in RANK and RANKL leads to the
rare osteoclast-decient form of osteopetrosis
(Guerrini et al. 2008). The more common
osteoclast-rich forms of osteopetrosis are most
frequently a result of mutations in CLCN7. Loss
of this chloride channel function prevents acidi-
cation of the resorption lacuna. Subsequently,
bone demineralization is signicantly impaired.
Interestingly, only 70 % of cases of osteopetrosis
are explained by a currently known mutation
(Stark and Savarirayan 2009).
Clinical Features
Osteopetrosis is characterized by increased bone
density leading to a variety of clinical problems.
The disease can range from fatal in infancy
to asymptomatic. The more severely affected
patients have macrocephaly and other craniofacial
abnormalities. Of particular importance are the
systemic changes that occur as a result of the
disease. The bone marrow is replaced by bone
leading to anemia and immunodeciency. A com-
monly seen feature of many sclerosing bone dis-
eases is cranial nerve compression secondary to
stenosis at neuroforamina. This is also seen in
osteopetrosis. The skeletal ndings are common
among all subtypes of osteopetrosis though their
severity varies. The skeleton shows increased
bone density with diffuse sclerosis; there can be
modeling deformities at the metaphysis, a predis-
position to pathologic fractures, and osteomyeli-
tis. The most common modeling defect is the
Erlenmeyer ask seen at the distal femur.
Another common radiographic feature is the
bone in bone appearance of the vertebrae, ilium,
and phalanges. Patients are at risk for dental
caries.
There are three main types of osteopetrosis:
autosomal recessive osteopetrosis, intermediate
19 Metabolic and Endocrine Abnormalities
osteopetrosis, and autosomal dominant
osteopetrosis. The subtypes will be discussed in
the following paragraphs.
Autosomal recessive osteopetrosis, sometimes
known as malignant osteopetrosis, becomes clin-
ically apparent in the rst few weeks to months of
life. It is a life-threatening disease. Pancytopenia
leads to profound immunodeciency and
extramedullary hematopoiesis occurs in the liver
and spleen. Subsequently, hepatosplenomegaly is
present in most patients (Del Fattore et al. 2006).
The patients have an increase in bone quantity, but
the bone is brittle, leading to pathologic fractures.
Patients may also have macrocephaly and frontal
bossing. Cranial nerves are entrapped at their
foramina and 78 % of patients have hearing loss.
In addition, patients may present with hydroceph-
alus secondary to outow obstruction in the pos-
terior fossa (Al-Tamimi et al. 2008). Laboratory
evaluation is positive for hypocalcemia. The
patients may have symptoms related to their hypo-
calcemia including seizures and hyperparathy-
roidism. Patients with the autosomal recessive
form of the disease who are untreated have a
shortened lifespan.
There are several subtypes of autosomal reces-
sive osteopetrosis. The rst is neuropathic, which
is characterized by neurodegeneration and sei-
zures despite normal calcium levels. A second
subtype is associated with renal tubular acidosis
and has a less severe clinical course (Cotter
et al. 2005). In this rare form of the disease,
profound hematopoietic deciencies are
uncommon.
An intermediate form of osteopetrosis has been
described. Similar to infantile osteopetrosis, this
disease is inherited in an autosomal recessive
manner. However, the disease has its onset in
childhood. The children may have some
disturbance in hematopoiesis, but it is usually
mild. The other features of the disease tend to be
much less severe than that seen in infantile
osteopetrosis.
The last form of osteopetrosis is the autosomal
dominant form, which presents in adolescence.
The disease has variable penetrance with 90 %
of all patients with known mutations demonstrat-
ing the phenotype. In autosomal dominant
441
osteopetrosis, most ndings are related to the
skeleton. The patients may develop hip osteoar-
thritis, pathologic fractures, scoliosis, and dental
caries. Only 5% of affected people have hearing or
visual loss. Unlike the autosomal recessive forms
of osteopetrosis, patients typically have a normal
life expectancy. The diagnosis of autosomal dom-
inant osteopetrosis can be conrmed with an ele-
vated tartrate-resistant acid phosphatase serum
level (Waguespack et al. 2002).
Treatment
The treatment of osteopetrosis is largely aimed at
treating the complications of the disease. No
completely effective medical treatment has been
developed to date. High-dose steroids have been
used with mixed results. Interferon gamma has
been used with some success in patients with the
infantile malignant form of the disease (Key
et al. 1995). Interferon gamma has been shown
to decrease bone density, increase the medullary
space, increase the size of neuroforamina, and
decrease the rates of infection in patients with
osteopetrosis (Driessen et al. 2003). Seizures are
managed with calcium and vitamin D supplemen-
tation. In cases of symptomatic anemia, the
patients may require blood products. Patients
should be followed at regular intervals for evalu-
ation of hearing and visional loss. In patients with
the malignant autosomal recessive form of
osteopetrosis, hematopoietic stem cell transplan-
tation has been used with some success. In the
ideal situation with the availability of an identi-
cally matched donor, 73 % of patients were
disease-free at 5 years (Driessen et al. 2003).
The overall survival in one study, including
122 patients who had undergone stem cell trans-
plantation, was 46 % at 5-year follow-up
(Driessen et al. 2003). The expected outcome of
transplantation is prevention of disease progres-
sion not reversal of already apparent disease man-
ifestations. For instance, the rate of vision
impairment was 52 % in patients treated at less
than 3 months of age, while it was 86 % in patients
older than 6 months (Driessen et al. 2003). Few
patients demonstrate improvement in vision after
442
stem cell transplantation. As such, stem cell trans-
plantation ideally occurs prior to 3 months of age.
The surgical management of complications from
osteopetrosis is particularly challenging. Fre-
quently, patients with osteopetrosis have osteoar-
thritis and pathologic fractures. In cases were
fracture xation is required, nonunion or delayed
union is common. A recent report of operative
xation of a humerus fracture treated for non-
union after initial conservative therapy suggests
a role for BMP in cases of atrophic nonunions
(Raq et al. 2009). Intraoperatively, one should
expect difculty with drilling and reaming
(Nakayama et al. 2007). Lastly, treating surgeons
should be aware that patients with osteopetrosis
are at increased risk for infection after surgical
procedures.
Infantile Cortical Hyperostosis
(Caffey Disease)
Introduction
Infantile cortical hyperostosis (ICH) is a genetic
disease belonging to the family of sclerotic bone
disorders and is characterized by massive new
subperiosteal bone formation. Caffey initially
described the entity in 1945. Patients typically
have involvement of the diaphysis of long
bones, but the clavicles, the scapulae, the ribs,
and the mandible may also be involved. The
onset of disease usually occurs in infancy, usually
around 2 months of age, with resolution of symp-
toms during early childhood. However, recent
reports have indicated that infantile cortical
hyperostosis may recur as late as 12 years after
initial presentation. A prenatal form of infantile
cortical hyperostosis has also been described
(Kamoun-Goldrat et al. 2008). This prenatal
form may present either as a severe lethal hyper-
ostosis or as mild uncomplicated disease similar
to the traditional disease. There does not appear to
be an increased prevalence in any one ethnic
group.
K. Freese and A. Leet
Pathophysiology
Infantile cortical hyperostosis is caused by a muta-
tion in the COL1A1 gene. Infantile cortical hyper-
ostosis demonstrates variable penetrance and is
inherited in an autosomal dominant fashion. The
responsible mutation causes a missense mutation
(3040C ! T). This mutation results in an arginine
being substituted for a cysteine at the 836th amino
acid residue and changes the triple-helical domain
of 1 chains. The exact reason this change in
collagen structure results in hyperostosis is
unknown. However, it is interesting to note that
infants treated with prostaglandin E for ductus-
dependent heart disease form new periosteal bone
that is similar in distribution to that seen in infan-
tile cortical hyperostosis. Treatment of patients
with infantile cortical hyperostosis with indo-
methacin has been shown to decrease circulating
levels of prostaglandin and improve symptoms.
The relationship between COL1A1 and prosta-
glandin in ICH has not been determined.
Although the prenatal forms of infantile corti-
cal hyperostosis appear phenotypically similar to
the infantile form, they do not always share the
same genetic etiology. A recent report has identi-
ed the causative mutation in infantile cortical
hyperostosis in a case of fatal severe prenatal
cortical hyperostosis (Kamoun-Goldrat et al.
2008). Case reports have hypothesized both auto-
somal dominant and autosomal recessive modes
of inheritance.
Clinical Features
The infant with infantile cortical hyperostosis pre-
sents with the rapid onset of swelling of the jaw
and face, followed by swelling of the affected
extremity. The infant may avoid movement of
the involved limbs. The patient may be febrile
and have increased inammatory markers includ-
ing the erythrocyte sedimentation rate, C-reactive
protein, and the white blood cell count. In some
cases, the patient may be anemic. Given this con-
stellation of symptoms, an infectious etiology is
19 Metabolic and Endocrine Abnormalities
often sought rst. However, characteristic radio-
graphic images will usually divulge the diagnosis.
Radiographs will demonstrate massive new
subperiosteal bone formation that spares the
epiphysis. The cortex of the bones appears thick-
ened and the soft tissues surrounding the bones
are not necessarily edematous. Radiographic
changes typically lag 23 weeks behind initial
clinical presentation. Previous reports have esti-
mated that the clavicles are involved 50 % of the
time, the ribs 33 % of the time, and the scapulae in
25 % of patients (Caffey 1957). The mandible is
involved in 7090 % of cases (Shandilya
et al. 2013).
The natural history of Caffey disease is vari-
able: while some patients have resolution of
symptoms within a week of disease onset, other
patients have a waxing and waning course with
resolution of swelling in one body area followed
by a new onset of swelling in another location.
There does appear to be evidence that families
with the COL1A1 mutation may have joint
hyperlaxity and may be more susceptible to frac-
tures (Gensure et al. 2005). Others have reported
short stature, increased rates of dental caries, and
persistent bone deformities in association with
infantile cortical hyperostosis (Suphapeetiporn
et al. 2007). In fact, patients as old as 76 years of
age have been reported to have residual deformi-
ties from Caffey disease.
A second form of infantile cortical hyperosto-
sis has been described also with a prenatal onset.
Within the prenatal form of the disease, two sub-
types have been identied. The rst is a mild
subtype that results in disease that mimics the
infantile onset disease. These patients were born
at term and had relatively limited skeletal involve-
ment (Schweiger et al. 2003). Many of these
infants had pulmonary hypoplasia. Conversely,
those fetuses affected by the severe form of dis-
ease rarely survive to birth. Polyhydramnios was
commonly the rst detected abnormality and was
present in 85 % of cases (Schweiger et al. 2003).
All patients affected with the severe form of dis-
ease were born prematurely and most had exten-
sive skeletal involvement. In those few infants
443
with severe disease that survived, remarkable
improvement of the skeletal disease was seen.
Medical Treatment
The mainstay of treatment for infantile cortical
hyperostosis has historically been supportive
care and pain control. Multiple authors have
reported the efcacy of treatment with nonsteroi-
dal anti-inammatory medications and antipy-
retics (Thometz and DiRaimondo 1996).
Glucocorticoids have been used successfully in
cases recalcitrant to NSAIDs. In one particular
case, the authors found that prednisolone was
ineffective in preventing multiple recurrences in
a set of affected twins (Couper et al. 2001). The
children were subsequently treated with indo-
methacin successfully. Of note, the patients had
multiple steroid-related complications and no
adverse effects from indomethacin. This group
of patients may also benet from referral to a
medical geneticist for counseling and diagnostic
testing.
Orthopedic care is highly individualized based
on the patients individual disease manifestations.
Radioulnar synostosis and radial head disloca-
tions have been described with some frequency.
These deformities along with joint contractures
may require operative intervention. It should be
reiterated that infantile cortical hyperostosis is a
benign self-limiting disease that spontaneously
resolves in most patients and invasive procedures
are rarely required.
Sclerosteosis
Introduction
Sclerosteosis is a member of the sclerosing bone
dysplasia family. The disease is inherited in an
autosomal recessive fashion and is the result of
mutations in the SOST gene. The clinical mani-
festations of sclerosteosis include progressive
bone overgrowth leading to facial abnormalities
444
and cranial nerve entrapment. A considerable pro-
portion of patients also have syndactyly most
commonly of the index and middle ngers. This
rare disease is found most commonly in the Afri-
kaner population of South Africa but has been
seen in Spain, Brazil, Germany, Japan, Senegal,
and the United States of America (Hamersma
et al. 2003; Bueno et al. 1994). The incidence of
sclerosteosis is approximately 1 per 75,000
people in the Afrikaner population.
Pathophysiology
The mutation in the SOST gene prevents secretion
of the sclerostin gene product. All SOST muta-
tions described to date have resulted in a loss of
gene function. Sclerostin is normally a product of
osteocytes that has been shown to negatively reg-
ulate bone formation (van Bezooijen 2004).
SOST has been shown to downregulate bone for-
mation through inhibition of the Wnt pathway.
Others have shown that SOST expression by oste-
ocytes depends on mechanical loading. In bones
that are unloaded, SOST expression is
upregulated, leading to decreased bone deposi-
tion. Finally, in SOST knockout mice, disuse
osteopenia was not induced by bone unloading
(Lin et al. 2009).
Clinical Features
Patients affected by sclerosteosis are normal at
birth except for syndactyly, which is present in
76 % of patients (Hamersma et al. 2003). Patients
begin to manifest other symptoms of the disease in
early childhood usually around 5 years of age. The
most serious clinical manifestation is a result of
hyperostosis of the calvarium leading to poten-
tially fatal increases in intracranial pressure. The
patients may also demonstrate mandibular over-
growth and generalized hyperostosis of long
bones. Patients tend to be tall and resistant to
fractures. Bone mineral density measured by
DEXA scan shows that Z scores in affected indi-
viduals range between +7.73 and +14.43 in the
lumbar spine (Gardner et al. 2005). Heterozygotes
K. Freese and A. Leet
do not demonstrate clinical manifestations of the
disease but do have bone mineral densities that are
greater than the general population. The patients
commonly have facial nerve palsies and hearing
loss secondary to compression of the seventh and
eighth cranial nerves, respectively. Most affected
patients do not live a normal lifespan. Those
patients with elevated intracranial pressure have
an average lifespan of 33 years. Other manifesta-
tions of the disease in the upper extremity include
radially deviated distal phalanges and dysplastic
nails.
The diagnosis can be made based on the clin-
ical features of the disease. The patients may have
elevated alkaline phosphatase levels and urinary
cross-linked N-telopeptide levels on laboratory
evaluation. DNA sequencing can be used to con-
rm the diagnosis in suspected cases.
Radiographic features of the disease included
hyperostosis and sclerosis of the calvarium and
base of the skull with loss of the diploic space. The
diaphyses of long bones are more involved than
the metaphysis and epiphysis. The ribs and clav-
icles tend to be broad and dense, while the scapula
and pelvis demonstrate sclerosis without an
increase in bone size. The vertebral bodies are
relatively spared while the posterior elements
show typical bony changes of sclerosis. The
bony changes progress over the rst three decades
and then become static thereafter (Beighton
et al. 1976).
Treatment
Management of patients with sclerosteosis begins
with close surveillance. The patient should have
neurologic and audiologic examinations annually.
Signs of increased intracranial pressure should
prompt neurosurgical evaluation. Surgical decom-
pression of compressed cranial nerves and
craniectomy for increased intracranial pressure
are often required. Some have recommended pro-
phylactic craniectomy in all patients in their sec-
ond decade (du Plessis 1993). The patients may
require decompression of their spinal cord in
adulthood. Bony surgery is often very difcult
and prolonged given the density of their bone. In
19 Metabolic and Endocrine Abnormalities
fact, commonly, the patients calvarium is 34 cm
thick. Lastly, the hand surgeon may be the rst
physician to evaluate these patients, as they are
normal at birth except for syndactyly of the n-
gers. The syndactyly ranges from slight webbing
to complete complex syndactyly and usually
involves the index and middle ngers. The syn-
dactyly is treated as it would be in other patient
populations.
Camurati-Engelmann Disease
Introduction
Camurati-Engelmann disease is a genetic disorder
resulting in hyperostosis of the long bones and
skull. It is also commonly known as progressive
diaphyseal dysplasia. The disease is caused by a
mutation in the TGFB1 gene and is inherited in an
autosomal dominant fashion with reduced pene-
trance. Previously, ribbing disease, which appears
radiographically similar but has its onset after
puberty, was thought to be a distinct entity. How-
ever, ribbing disease is caused by the same genetic
mutation of TGFB1. The incidence of Camurati-
Engelmann disease is 1 per 10,000 (Wallace
et al. 2004).
Pathophysiology
Ninety-four percent of families affected with
Camurati-Engelmann disease have mutations in
the TGFB1 gene. There are some families with
the phenotype that do not have an identiable
mutation. Thus, there may be some genetic het-
erogeneity or the families may have been
misdiagnosed.
TGFB1 is normally secreted as an inactive
complex consisting of two latent peptides and
two active peptides. Mutations detected to date
have one of two effects. Either the mutation
leads to decreased secretion of TGFB1 or the
mutation causes decreased dimerization between
the latent and active peptides (Janssens 2005). In
the former case, TGFB1 activity is increased but
the exact mechanism is unknown. In the later case,
445
increased active TGFB1 is secreted from the cell.
Radiographic evidence suggests that osteoclastic
remodeling of bone is decreased and that osteo-
blastic bone formation is increased. TGFB1 has
also been shown to stimulate osteoprogenitor cells
to differentiate into osteoblasts and increase col-
lagen production. Interestingly, several polymor-
phisms in TGFB have been shown to regulate
bone mineral density and risk of osteoporosis.
Lastly, TGFB1 activity has been shown to
decrease both adipogenesis and myogenesis
(Choy and Derynck 2003; Zhu et al. 2004). This
would explain the clinical features of decreased
strength and body fat.
Histologic examination of bone biopsy speci-
mens from patients with Camurati-Engelmann
disease reveals increased numbers of osteocytes
with trabecular thickening and irregularity
(Ozturkmen and Karamehnetoglu 2011). The
biopsy also demonstrates decreased haversian
systems and sclerosis (Wallace et al. 2004).
Clinical Features
Patients with Camurati-Engelmann disease have
proximal muscle weakness, a wide-based gate,
and headaches in addition to widespread hyperos-
tosis of the long bones. The muscle weakness can
lead to a misdiagnosis of muscular dystrophy in
some patients. The patients may demonstrate the
Gower maneuver to go from sitting to standing.
The hyperostosis involves the diaphysis and can
spread to the metaphysis as the disease progress.
The epiphysis is spared. Initially, the femur and
tibia are affected. With progression of the disease,
the bula, humerus, ulna, and radius can also
become involved (Janssens 2005). Involvement
of all bones has been described (Wallace
et al. 2004). The disease is usually bilateral and
symmetric. The rate of progression is variable and
may be rapid or slow. The skull is involved in
54 % of patients while the pelvis is involved in
63 % of patients (Janssens 2005). The average
onset of symptoms is 13.4 years old. The age of
onset and rate of progression of disease cannot be
correlated to the patients specic TGFB1 muta-
tion (Janssens 2005). Ninety percent of patients
446
report bone pain with many patients reporting
sporadic episodes of incapacitating pain. The
patients bones are often tender to palpation.
Given the patients increased bone mineral den-
sity combined with good bone quality, there may
actually be a decreased incidence of fracture.
However, some researchers have reported delayed
bone healing when fractures do occur. The
patients may have a variety of other skeletal man-
ifestations including kyphosis, scoliosis, genu
valgum, pes planus, and frontal bossing.
As in sclerosteosis, cranial nerve foramina may
become stenotic leading to cranial nerve dysfunc-
tion. This occurs in 38 % of patients. A substantial
proportion of patients have hearing loss. In cases
where the orbit is involved, vision problems may
occur as well.
Extensive laboratory evaluations have been
done in most patients with Camurati-Engelmann
disease and in most cases, the results were
unremarkable. However, a small proportion of
patients may be anemic. In most cases, the alka-
line phosphatase is normal.
Treatment
The initial management of patients with
Camurati-Engelmann disease is aimed at deter-
mining the extent of disease. The patient should
undergo a complete neurologic examination
including hearing tests and ophthalmologic exam-
ination. The patient should also have a skeletal
survey to determine the extent of bony involve-
ment. Inammatory markers, such as erythrocyte
sedimentation rate and C-reactive protein, can be
used as a measure of disease activity. Bone scin-
tigraphy can be useful to detect disease at a young
age. Bone scans will show increased uptake prior
to radiologic evidence of sclerosis (Janssens
2005).
The treatment of Camurati-Engelmann disease
is aimed at symptomatic relief and prevention of
long-term complications. The mainstay of treat-
ment of symptoms is corticosteroids. Multiple
authors have reported relief of bone pain and
improved strength with the use of corticosteroids.
Prednisolone is the steroid of choice and the
K. Freese and A. Leet
patients should be tapered to the lowest dose that
relieves symptoms. Interestingly, glucocorticoids
have been shown to shift TGB1 binding from
receptors that have signaling capability to recep-
tors that are non-signaling. In addition, to
improved symptoms, radiographic evidence of
improvement after steroid treatment has been
noted in some patients. Deazacort has also been
used in the treatment of Camurati-Engelmann dis-
ease with similar effectiveness and less side
effects (Bass et al. 1999). Losartan is another
potential therapeutic agent and can be tried in
patients who do not tolerate corticosteroids. The
drug is known to have anti-TGFB effect and may
be particularly well suited to preventing myopa-
thy associated with Camurati-Engelmann disease
(Cohn et al. 2007). After the initiation of cortico-
steroids, the patients must be monitored for hyper-
tension. In addition to steroids, NSAIDs and
antipyretics can also be used for symptomatic
relief. Bisphosphonate treatment of Camurati-
Engelmann disease has been attempted with lim-
ited or no success (Castro et al. 2005).
In several cases, surgical treatment has been
attempted. Reaming of the medullary canal has
been reported in at least two cases (Ozturkmen
and Karamehnetoglu 2011; Beals et al. 2002). In a
case in which the tibia was reamed for recalcitrant
pain, the patient had complete resolution of her
symptoms. The patient was symptom-free 5 years
after surgery (Ozturkmen and Karamehnetoglu
2011). In addition, neurosurgical intervention
may be required in patients with cranial nerve
foraminal narrowing.
Melorheostosis
Introduction
Melorheostosis is a rare benign nonfamilial scle-
rosing bone disorder. The disease is characterized
by hyperostosis and brosis of surrounding soft
tissues. The incidence of the disease is about 0.9
per million individuals. It appears to affect both
sexes equally. Approximately half of patients pre-
sent with monostotic disease.
19 Metabolic and Endocrine Abnormalities
Pathophysiology
The etiology of melorheostosis is not known at
this time. In the past people of have observed that
the disease seems to occur in a sclerotomal distri-
bution. Hence, some authors have hypothesized
that the disease occurs secondary to a disorder of
the nervous system. Others feel that
melorheostosis is due to mosaicism of a gene
mutation that would otherwise be lethal. Recent
studies have implicated a mutation of the LEMD3
gene as a causative factor in melorheostosis,
osteopoikilosis, and Buschke-Ollendorff syn-
drome (Hellemans et al. 2004). The normal func-
tion of LEMD3 is to inhibit TGF-beta and BMP.
However, in a follow-up study by the same
authors, LEMD3 mutations were not implicated
in cases of sporadic melorheostosis (Hellemans
et al. 2006). Another protein implicated in the
pathogenesis of melorheostosis is ig-h3. This
protein is downregulated in melorheostosis. In
vitro studies have demonstrated that ig-h3
inhibits bone formation (Kim et al. 2000).
Histologic features of the disease include dense
irregular sclerotic lamellae, large amounts of
non-mineralized osteoid, and numerous osteo-
clasts. The large amounts of osteoid and increased
numbers of osteoclasts suggest dysregulation of
both bone formation and resorption.
Clinical Features
Melorheostosis usually presents in childhood.
4050 % of cases are diagnosed prior to the age
of 20 years. Most patients present with progres-
sive joint contractures and limb swelling. Pain is a
variable presenting symptom, which seems to be
more common in adults with melorheostosis. The
disease is most commonly found in the lower
extremities but can be found in any bone
(Freyschmidt 2001). The axial skeleton is usually
spared, but when spine disease occurs, the patient
may develop neurologic decits. Involvement can
be monostotic, polyostotic, monomelic, or gener-
alized. The skull and ribs are affected least often.
In addition to the sclerosis with
melorheostosis, a number of other deformities
447
have been described in association with this dis-
ease. These include genu valgum, equinus, foot
deformities, and patellar dislocations. Growth dis-
orders in the affected extremity often bring the
child to the attention of a physician. There may
be changes in the skin overlying the affected
bones. The skin may be edematous and erythem-
atous. It is not uncommon to nd an ossied soft
tissue mass and in some series, the rate is as high
as 27 %. Ossication of the soft tissues often
occurs prior to bony disease. This can complicate
the diagnosis. Finally, vascular anomalies are
associated with melorheostosis. These include
hemangiomas, arteriovenous malformations, glo-
mus tumors, and aneurysms. The natural history
of melorheostosis is chronic recurrent disease
with periods of exacerbation and periods of qui-
escence (Abdullah et al. 2011; Long et al. 2009).
Even after surgical treatment, the disease tends to
recur.
Most laboratory studies, including calcium,
phosphorus, and alkaline phosphatase, are within
normal limits. The classic radiograph demon-
strates segmental, unilateral disease with irregular
sclerosis that looks like hot dripping wax (Fig. 5).
In fact, the name melorheostosis is derived from
the Greek words melos, meaning limb, and
rhein, meaning ow. There is typically a narrow
transition zone between normal and abnormal
bone. The hyperostosis can be endosteal or peri-
osteal. In children, the disease is more commonly
endosteal (Younge et al. 1979). Bone scans will
demonstrate focal areas of increased uptake in all
areas of diseased bone. MRI and CT can be used
to further delineate bone involvement, evaluate
the surrounding soft tissues, and assess spine for
stenosis. There is decreased signal compared to
normal bone on all MRI sequences.
Treatment
Treatment of melorheostosis is mostly symptomatic.
Children typically present with contractures, limb
deformity, or limb length inequality, while adults
typically present with pain and stiffness. Medical
treatment includes NSAIDs and bisphosphonates
for pain relief (Donath et al. 2002). Others have
448
Fig. 5 Melorheostosis of
the ulna, carpal bones, and
metacarpals (Courtesy of
Michael Meagher, MD,
Shriners Hospital for
Children Honolulu)
recommended retraining patients to use their
nondominant upper extremity in cases where their
dominant arm is affected.
Surgical treatment is aimed at increasing func-
tion. Given that the disease is progressive, surgi-
cal intervention should be delayed until skeletal
maturity if possible. In many ways, the disease
mimics arthrogryposis. Surgical techniques used
during the treatment of arthrogryposis may be
applicable in melorheostosis. This may involve
soft tissue procedures to improve range of motion
of contracted joints or bony procedures to correct
deformities. However, surgical releases of soft
tissue contractures have not been universally suc-
cessful. In fact, in one series, 10 surgical releases
of contracted joints were attempted and in 9 of
K. Freese and A. Leet
these cases, the disease recurred (Younge
et al. 1979). Recently, a case report of a patient
with a 90 xed elbow exion contracture sec-
ondary to melorheostosis was treated with surgi-
cal contracture release. At two-year follow-up, the
patient had a signicant improvement in his range
of motion with exion from 20 to 135 (Gong
et al. 2008). Osteotomies to correct deformities
have also met with limited success. In general, the
deformities associated with melorheostosis tend
to recur as children grow. There are cases in which
the disease extends into adjacent joints causing a
mechanical block to motion. Several cases have
been treated successfully with total joint
arthroplasty of both the shoulder and knee
(Moulder and Marsh 2006). Other manifestations
19 Metabolic and Endocrine Abnormalities
of melorheostosis that may require surgical treat-
ment in the upper extremity include carpal tunnel
syndrome and cubital tunnel syndrome as a result
of disease of the adjacent bone and soft tissue. A
recent case described symptoms of median nerve
compression at the elbow from a lesion on the
anterior distal humerus. The nerve was
decompressed and the melorheostotic bone was
removed. The patient had complete resolution of
her symptoms at 1 year (De Vos et al. 2010).
Finally, it is worth noting that several patients
have undergone amputation of an affected extrem-
ity as the extremity has lost all function (Younge
et al. 1979).
Summary
Metabolic bone diseases can affect upper extrem-
ity function, but upper extremity interventions
have only been studied in a limited fashion and
present an interesting new frontier for those inter-
ested in the upper extremity.
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 Genetic Syndromes
20
Julie D. Kaplan and Carol L. Greene

Contents Abstract
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 453 Congenital upper extremity abnormalities are
commonly associated with genetic syndromes;
Important Terminology . . . . . . . . . . . . . . . . . . . . . . . . . . . . 456
accurate identication and diagnosis of a syn-
Etiologies of Malformations . . . . . . . . . . . . . . . . . . . . . . . 457 drome may dramatically impact medical man-
Limb Deciencies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 460 agement for the child and recurrence risk for
the family. In this chapter, commonly used
Synostosis/Syndactyly . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 461
genetic terminology and the utility of genetic
Polydactyly . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 461 testing versus referral to a clinical geneticist is
Brachydactyly . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 461 reviewed. In addition, common genetic syn-
Oligodactyly . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 462 dromes will be reviewed for specic types of
limb abnormalities. After reading this chapter,
Overgrowth . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 462
the orthopedic surgeon should have a better
Skeletal Dysplasias . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 462 understanding of when to recognize a possible
Congenital Contractures . . . . . . . . . . . . . . . . . . . . . . . . . . . 463 genetic syndrome in a child with an upper
Joint Dislocations, Subluxations,
extremity abnormality in addition to when to
and Hyperextensibility . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 463 refer to genetics for testing, diagnosis, and
counseling.
Madelung Deformity . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 465
Conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 465
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 465 Introduction

Abnormalities of the upper extremity can be part

of hundreds to thousands of genetic syndromes.
For example, searching hand abnormality on
OMIM (Online Mendelian Inheritance in Man)
OMIM reference reveals 5,345 results. The
extremities are highly susceptible to
malformations, deformations, and disruptions
J.D. Kaplan (*) C.L. Greene due to their developmental complexity and expo-
University of Maryland School of Medicine, Baltimore,
sure outside of the body wall (Stevenson and Hall
MD, USA
e-mail: jkaplan@peds.umaryland.edu; 2006). Limb abnormalities that are part of genetic
cgreene@peds.umaryland.edu syndromes are most often present at birth, but
# Springer Science+Business Media New York 2015 453
J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_20
454 J.D. Kaplan and C.L. Greene

Table 1 Selected syndromes with limb deficiencies as a common feature

Commonly associated Inheritance
Syndrome Upper limb anomaly features pattern Testing
Cornelia de Lange Absent or hypoplastic ulna; Prenatal growth Autosomal Molecular
oligodactyly retardation, microcephaly, dominant,
congenital heart disease mostly de
(CHD), dysmorphic novo
features, GU anomalies
Fanconi anemia Radial ray deciencies Short stature, Autosomal Chromosome
microcephaly, recessive, breakage,
pancytopenia X-linked molecular
Poland anomaly Distal transverse defect, Ipsilateral pectoral muscle Sporadic Unknown
unilateral symbrachydactyly deciency (Fig. 1), cranial (most) or
nerve palsy autosomal
dominant
Holt-Oram Radial, thenar, or carpal bone CHD (esp. ASD/VSD), Autosomal Molecular
malformation cardiac conduction defects dominant
Thrombocytopenia- Bilateral absence of radii with Thrombocytopenia, other Autosomal Deletion in
absent radius (TAR) thumbs present (Figs. 2 and skeletal anomalies, GU recessive 1q21.1;
3) anomalies (deletion or molecular
mutation)
Roberts Mesomelic shortening Prenatal growth Autosomal Premature
leading to hypomelia, retardation, cleft lip +/ recessive centromere
symmetric tetraphocomelia, palate, dysmorphic facies, separation,
oligodactyly, syndactyly, intellectual disability Molecular
clinodactyly
Adams-Oliver Terminal transverse limb CHD, cutis aplasia Autosomal Molecular
defects, syndactyly, (absence of a portion of dominant or
brachydactyly skin, usually on the scalp) recessive
(Fig. 4)
Amniotic band Amputation of digits and/or Cleft lip +/ palate, club Sporadic Disruption
sequence limbs, constriction bands foot
(Fig. 5)
Nager Radial ray deciency Cleft lip +/ palate, Autosomal Molecular
micrognathia, dysmorphic dominant
features
Thalidomide Variable long bone Ear anomalies, CHD Teratogen N/A
exposure deciencies, oligodactyly
VACTERL Radial aplasia proximally Vertebral anomalies, anal Sporadic Unknown
placed thumb, humeral atresia, CHD, (most),
hypoplasia tracheoesophageal stula, autosomal
renal anomalies dominant, or
X-linked
CHD congenital heart disease, ASD atrial septal defect, VSD ventricle septal defect, GU genitourinary

some only become evident or develop over time;
an important example is the development over
time of restricted joint mobility and carpal tunnel
syndrome in the mucopolysaccharidoses, which
are addressed in chapter Metabolic and
Endocrine Abnormalities. Upper extremity
abnormalities can be minor ndings of interest
primarily to the geneticist in identication of a
syndrome, but many are considerable functional
or aesthetic issues that come to the attention of the
orthopedic surgeon. Overall, 45 % of infants with
malformations will be found to have a genetic
20 Genetic Syndromes
Fig. 1 A girl with Poland anomaly showing right pectoral
muscle hypoplasia and absence of the right nipple (Cour-
tesy of Shriners Hospitals for Children Philadelphia)
Fig. 2 Absent radius in a patient with TAR syndrome
(Courtesy of Shriners Hospitals for Children Philadelphia)
etiology (Thompson and Thompson 2007).
Therefore, identication of the etiology of an
upper extremity abnormality is important as risk
of surgery may be affected once the developmen-
tal process and associated features are recognized.
455
Fig. 3 Radiograph of an individual with TAR syndrome
showing absent radius (Courtesy of Shriners Hospitals for
Children Philadelphia)
Fig. 4 Cutis aplasia of the scalp (Courtesy of Shriners
Hospitals for Children Philadelphia)
For example, the child with Holt-Oram syndrome
is at risk for cardiac arrhythmia and congenital
heart defect.
Syndromes that involve anomalies of the upper
extremity may result from chromosomal, single
gene, and non-Mendelian inheritance or terato-
gens. This chapter will give brief denitions of
important genetic and dysmorphology
456 J.D. Kaplan and C.L. Greene

terminology and describe some of the more com-

mon or important syndromes associated with Important Terminology
upper limb anomalies. This terminology is uti-
lized by the geneticist and may differ slightly
from the terminology applied by the hand
surgeon.
Fig. 5 Terminal limb deciency with constriction bands
caused by amniotic bands (Courtesy of Shriners Hospitals
for Children Philadelphia)
Anomaly: an irregularity of structure with
unspecied etiology
Dysmorphic feature (minor anomaly): an
unusual anatomic feature that is of no
serious medical or cosmetic consequence to
the patient
Malformation: an anomaly resulting
from intrinsic abnormalities in one or
more genetic programs operating in
development
Deformation: an anomaly in a structure that
was programmed to be made correctly but
was altered by external factors
Disruption: an anomaly resulting from the
destruction of structures that developed
normally
Syndrome: a nonrandom pattern of anomalies
that can be attributed to an identiable etiology
or demonstrates a recognizable pattern of
inheritance
Association: a nonrandom pattern of
anomalies that occurs without an
identiable etiology or heritability being
established

Table 2 Selected syndromes with synostosis and/or syndactyly as a common feature

Commonly/variably associated Inheritance
Syndrome Upper limb anomaly features pattern Testing
Apert Cutaneous and/or osseous Craniosynostosis, shallow orbits Autosomal Molecular
syndactyly (Fig. 6) with hypertelorism (Fig. 7), hearing dominant
loss, cleft palate
Adams-Oliver Terminal transverse limb CHD, cutis aplasia Autosomal Molecular
defects, syndactyly, dominant or
brachydactyly recessive
Fibrodysplasia Synostosis, broad thumbs Progressive ossication of soft Autosomal Molecular
ossicans tissue, hearing loss, broad great toes dominant
progressiva
Pfeiffer Syndactyly, broad thumbs, Craniosynostosis, broad great toes, Autosomal Molecular
radiohumeral synostosis hydrocephalus, occasional mental dominant
retardation
Timothy Unilateral or bilateral Prolonged QT interval, CHD, Autosomal Molecular
cutaneous syndactyly hypertrophic cardiomyopathy, dominant
involving ngers two to ve seizures, intellectual disability
CHD congenital heart disease
20 Genetic Syndromes
Fig. 6 The hand of a child with Apert syndrome demon-
strating cutaneous and bony syndactyly (Courtesy of
Shriners Hospitals for Children Philadelphia)
Fig. 7 A child with Apert syndrome showing craniosyn-
ostosis, shallow orbits, and midface hypoplasia (Courtesy
of Shriners Hospitals for Children Philadelphia)
Etiologies of Malformations
Chromosomal abnormalities: abnormalities of
chromosome number or structure (deletions,
duplications, translocations, inversions, and
ring chromosome)
457
Single-gene disorders: genetic conditions
caused by the alteration or mutation of a spe-
cic gene
Teratogenic: of or relating to substances or
agents that can interfere with normal embry-
onic development
Non-Mendelian: a general term that refers to
any pattern of inheritance in which traits do not
segregate in accordance with Mendels laws
(e.g., trinucleotide repeats, imprinting)
In order to help determine the etiology of the limb
anomaly, a careful history, including family his-
tory, should be ascertained. Questions should
elicit information about pregnancy complications,
possible drug or environmental exposures, devel-
opmental and learning difculties, and other
anomalies. Family history should be targeted to
determine if other family members have similar
anomalies, other skeletal anomalies or birth
defects, or developmental delay/intellectual
disability.
A considerable fraction of individuals with
anomalies of the extremities will also have
minor or major anomalies elsewhere that may be
clues to an underlying syndrome (Thompson and
Thompson 2007). The presence of some major
anomalies may be discerned by history taking
(e.g., congenital heart disease), but others will
require careful examination. Therefore, physical
examination should not only be focused on the
extremity or extremities with the anomaly, but on
the entire body with attention to any other minor
or major anomalies that may suggest a syndrome.
In particular, craniofacial anomalies may help
suggest a particular genetic or teratogenic etiol-
ogy. Although the primary care physician typi-
cally identies concerns about a genetic
syndrome, the astute orthopedic surgeon can
make a big impact on a childs health care by
recognizing when a genetic syndrome may be
present.
In addition to growth disturbance and intellec-
tual deciency, there are some key malformations
or dysmorphic features that should lead the ortho-
pedic surgeon to be concerned that a patient could
have one of several common or important genetic
syndromes. Examples include:
458 J.D. Kaplan and C.L. Greene

Table 3 Selected syndromes with polydactyly as a common feature

Inheritance
Syndrome Upper limb anomaly Commonly associated features pattern Testing
Bardet-Biedl Postaxial polydactyly Truncal obesity, renal Autosomal Molecular
anomalies, retinal dystrophy, recessive
mental retardation
Meckel-Gruber Postaxial polydactyly Encephalocele, cystic kidneys, Autosomal Molecular
cleft lip+/ palate hepatic recessive
brosis
Trisomy 13 Postaxial polydactyly Holoprosencephaly, midline Chromosomal Karyotype
cleft lip+/ palate, intellectual
disability
Oral-facial- Preaxial or postaxial Bid or trid tongue, cleft X-linked Molecular
digital polydactyly, syndactyly, palate, brain anomalies, renal dominant
syndrome, type clinodactyly, brachydactyly cysts, duplicated hallux
1
McKusick- Postaxial polydactyly, CHD, GU malformations Autosomal Molecular
Kaufman mesoaxial polydactyly recessive
Short Postaxial polydactyly Lethal skeletal dysplasia (short Autosomal Molecular
rib-polydactyly limbs, short ribs), CHD, recessive (types II, III,
syndromes polycystic kidneys and V)
(types IV)
Simpson- Postaxial polydactyly, Pre and postnatal macrosomia, X-linked Molecular
Golabi-Behmel brachydactyly, cutaneous organomegaly, large mouth,
syndactyly CHD, prolonged QT,
Pallister-Hall Postaxial polydactyly, Hypothalamic hamartoma, Autosomal Molecular
mesoaxial polydactyly bid epiglottis dominant
Fanconi anemia Preaxial polydactyly, Microcephaly, short stature, Autosomal Chromosome
hypoplastic, or absent pancytopenia/bone marrow recessive or breakage
thumbs/radius failure X-linked studies,
molecular
Townes-Brocks Preaxial polydactyly, Imperforate anus, preauricular Autosomal Molecular
triphalangeal thumbs pits/tags, sensorineural dominant
deafness, renal agenesis/
impairment
Carpenter Postaxial polydactyly, Craniosynostosis, CHD, Autosomal Molecular
brachydactyly, clinodactyly, growth and intellectual recessive
syndactyly, camptodactyly disability
Ellis-van Postaxial or mesoaxial Short-limb dwarsm, neonatal Autosomal Molecular
Creveld polydactyly, nail dysplasia teeth, narrow chest recessive
(Fig. 8)
CHD congenital heart disease, GU genitourinary

Short stature in a child with a thumb malforma-
tion should raise concern for Fanconi anemia;
this disorder will have profound implications
for future health as it causes bone marrow fail-
ure in about 90 % of individuals.
Nevus simplex in a child with
hemihypertrophy, whether or not there is tall
stature, should raise concern for Beckwith-
Wiedemann, a syndrome with increased
tumor risk that has a good life expectancy if
the protocols to monitor for tumors are
followed.
Remarkably long eyelashes and the presence
of one long contiguous eyebrow (synophrys) in
a child with ulnar deciency should raise con-
cern for Cornelia de Lange syndrome in which
congenital heart defects are common and may
not have yet been discovered.
20 Genetic Syndromes 459

Genetic testing can be quite complex and

Fig. 8 Postaxial polydactyly and nail dysplasia seen in
Ellis-van Creveld syndrome (Courtesy of Shriners Hospi-
tals for Children Philadelphia)
understanding diagnostic techniques and testing
strategies will help to increase the likelihood of
an accurate diagnosis in a more cost-effective
manner. For an overview of genetic testing,
please refer to Dugan (2013). Briey, if a chro-
mosomal abnormality (e.g., Turner syndrome) is
likely, a routine karyotype is indicated. Chromo-
somal microarrays can identify copy number
changes in smaller regions of the chromosome
that may not be seen under the microscope. If a
single-gene disorder is suspected, molecular
sequencing or deletion/duplication analysis of
that particular gene is required to make a
molecular diagnosis. Many syndromes can be
clinically diagnosed, however, and chromosome

Table 4 Selected syndromes with brachydactyly

Upper limb Commonly/variably associated Inheritance
Syndrome anomaly features pattern Testing
Aarskog Brachydactyly, Short stature, hypertelorism, X-linked Molecular
short/broad hands, dysmorphic features (face and GU)
clinodactyly
Adams-Oliver Terminal CHD, cutis aplasia Autosomal Molecular
transverse limb dominant or
defects, recessive
syndactyly,
brachydactyly
Albright Brachydactyly, Short stature, intellectual disability Autosomal Molecular,
hereditary short metacarpals pseudohypoparathyroidism, obesity dominant, microdeletion
osteodystrophy (esp. fouth and imprinting, in 2q37
fth) microdeletion
Achondroplasia Brachydactyly, Upper airway obstruction, Autosomal Molecular
trident hand, macrocephaly, spinal stenosis, brain dominant
rhizomelic stem compression
shortening
Fibular Brachydactyly Talipes equinovalgus, short Autosomal Molecular
hypoplasia and (complex), short metatarsal, deformed tarsals, recessive
complex metacarpals rudimentary phalanges, absent bula,
brachydactyly displaced patella
(Du Pan)
Robinow Brachydactyly, Hemivertebrae with fusion of thoracic Autosomal Molecular
syndactyly, vertebrae, fused or absent ribs, short recessive
clefting of distal stature, dysmorphic features, CHD,
phalanges cleft lip +/ palate
CHD congenital heart disease, GU genitourinary
460 J.D. Kaplan and C.L. Greene

Table 5 Selected syndromes with oligodactyly as a common feature

Commonly/variably Inheritance
Syndrome Upper limb anomaly associated features pattern Testing
Amniotic Amputation of digits and/or Cleft lip +/ palate, club Sporadic Disruption
band limbs, constriction bands foot
sequence
Adams-Oliver Terminal transverse limb defects, CHD, cutis aplasia Autosomal Molecular
syndactyly, brachydactyly (absence of a portion of dominant or
skin, usually on the scalp) recessive
Cornelia de Absent or hypoplastic ulna; Prenatal growth Autosomal Molecular
Lange oligodactyly retardation, microcephaly, dominant,
CHD mostly de
Dysmorphic features, GU novo
anomalies
Ectrodactyly- SHSF (Fig. 9), syndactyly Cleft lip +/ palate, Autosomal Molecular
ectodermal abnormalities of skin, hair, dominant
dysplasia- and nails
clefting (EEC)
Fanconi Radial ray deciencies Short stature, Autosomal Chromosome
anemia microcephaly, recessive, breakage,
pancytopenia X-linked molecular
Holt-Oram Radial, thenar, or carpal bone CHD (esp. ASD/VSD), Autosomal Molecular
malformation cardiac conduction defects dominant
Nager Radial ray deciency Cleft lip +/ palate, Autosomal Molecular
micrognathia, dysmorphic dominant
features
Poland Distal transverse defect, unilateral Ipsilateral pectoral muscle Sporadic Unknown
anomaly symbrachydactyly deciency, cranial nerve (most) or
palsy autosomal
dominant
Thalidomide Variable long bone deciencies, Ear anomalies, CHD Teratogen N/A
exposure oligodactyly
Roberts Mesomelic shortening leading to Prenatal growth Autosomal Premature
hypomelia, symmetric retardation, cleft lip +/ recessive centromere
tetraphocomelia; oligodactyly, palate, dysmorphic facies, separation,
syndactyly, clinodactyly intellectual disability molecular
VACTERL Radial aplasia proximally placed Vertebral anomalies, anal Sporadic Unknown
thumb, humeral hypoplasia atresia, CHD, (most),
tracheoesophageal stula, autosomal
renal anomalies dominant, or
X-linked
CHD congenital heart disease, SHSF split hand split foot, ASD atrial septal defect, VSD ventricle septal defect, GU
genitourinary

or molecular testing may not be necessary.

Radiographs of the affected limb or a full
skeletal survey can be benecial in making a
clinical diagnosis. Clinical geneticists are
trained to diagnose syndromes and send testing
when and if indicated, so the clinician should
not hesitate to refer to a genetics specialist for
any congenital malformation of the upper
extremity.
Limb Deficiencies
Limb deciencies describe the absence of the skel-
etal and soft tissue components of all or part of a
limb. They are often isolated but may be associated
with other malformations requiring careful exami-
nation for other skeletal anomalies in addition to
anomalies in other systems (Table 1).
20 Genetic Syndromes 461

the most common congenital anomaly of the

Synostosis/Syndactyly
Synostosis is an osseous connection between
bones that are typically separate and is usually
due to the failure of complete segmentation of
cartilaginous template of the skeleton.
Symphalangism is dened as synostosis of pha-
langes resulting from failure of development of
interphalangeal joints. Syndactyly is due to a fail-
ure of the digits to separate during weeks 68 in
embryonic development and may involve the
bone and nails or only soft tissue. Syndactyly is
limbs, occurring in 2/1,0003/1,000 live births
(Schwabe and Mundlos 2004; Table 2).
Polydactyly
Polydactyly occurs from excessive partitioning of
the hands and feet resulting in complete or incom-
plete extra digits. Postaxial polydactyly describes an
extra digit on the ulnar side and preaxial polydac-
tyly is accessory digits on the radial side. Isolated
postaxial polydactyly is common in the African-
American population (Frazier 1960), and postaxial
polydactyly in this population is therefore less likely
to be associated with a syndrome. Mesoaxial or
central polydactyly is the partial or complete dupli-
cation on the 2nd, 3rd, or 4th nger (Table 3).

Brachydactyly

Brachydactyly refers to shortening of the digits

Fig. 9 Split hand split foot (ectrodactyly) seen in EEC
(Courtesy of Shriners Hospitals for Children Philadelphia)
and can be due to underdeveloped, absent, or
abnormally shaped phalanges or metacarpals. Sin-
gle or multiple digits may be involved and short-
ening can be symmetric or asymmetric. Types of

Table 6 Selected syndromes with overgrowth and/or macrodactyly

Commonly/variably associated Inheritance
Syndrome Upper limb anomaly features pattern Testing
Beckwith- Asymmetric Large size, large tongue, Autosomal Molecular,
Wiedemann overgrowth of one or hypoglycemia, omphalocele, dominant, methylation testing,
more limbs visceromegaly imprinting FISH, UPD analysis
Isolated Asymmetric Increased risk for intra- Sporadic Sometimes
hemihypertrophy overgrowth of one or abdominal malignancy chromosomal,
more limbs uniparental disomy
or molecular
Klippel- Limb overgrowth Cutaneous hemangiomas, Sporadic None
Trenaunay- with vascular varicosities
Weber anomalies,
macrodactyly,
Proteus Progressive, Lipoatrophy, cerebriform Sporadic, Molecular
segmental or patchy connective tissue nevi, lipomas, mosaicism
overgrowth, hamartomas, skull exostoses
macrodactyly
Hereditary One or more Short stature, bony deformity, Autosomal Molecular, FISH
multiple osteochondromas, restricted joint motion (Fig. 10) dominant
osteochondromas Madelung deformity
462
Fig. 10 Multiple osteochondromas resulting in bony
deformity (Courtesy of Shriners Hospitals for Children
Philadelphia)
brachydactyly are classied by the clinical and
radiographic patterns of involvement of the digits
(Temtamy and McKusick 1978). Important syn-
dromes with brachydactyly are included here for
completeness although if brachydactyly is the
only orthopedic anomaly, a child is not likely to
be referred to orthopedic surgery. However,
J.D. Kaplan and C.L. Greene
brachydactyly is likely to be noticed in a complete
orthopedic examination and might be an impor-
tant clue to an undiagnosed genetic syndrome
(Table 4).
Oligodactyly
Oligodactyly is a general term that describes the
severe underdevelopment or absence of one or
more digits. Monodactyly refers to the presence
of a single digit. Ectrodactyly is a nonspecic term
that describes the partial or total absence of distal
hand segments (Temtamy and McKusick 1978).
The term ectrodactyly is often unsuitably used to
describe split hand/split foot (SHSF), which is an
absence of the central digital rays (Table 5).
Overgrowth
Limb overgrowth is the enlargement of the skele-
tal and/or soft tissue components of the limb.
Overgrowth may be due to increased size of the
cells (hypertrophy) or increased number of cells
(hyperplasia). The terms hypertrophy and
hyperplasia have been used interchangeably
for decades, so conclusions should not be drawn
about mechanism based on terminology alone.
Overgrowth may be part of generalized over-
growth or isolated limb involvement.
Macrodactyly is the isolated overgrowth of the
phalanges and soft tissues of one or more digits
(Table 6).
Skeletal Dysplasias
The skeletal dysplasias are a heterogeneous group
of disorders that are characterized by the abnormal
bone growth and/or organization. Many are rec-
ognized prenatally, but some do not come to the
attention of the physician until childhood. The
inheritance patterns of these disorders may be
autosomal dominant, autosomal recessive, or
X-linked. Please see chapter Skeletal Dyspla-
sias for more details.
20 Genetic Syndromes 463

Table 7 Selected syndromes with joint contractures

Commonly/variably Inheritance
Syndrome Upper limb anomaly associated features pattern Testing
Amyoplasia Internal rotation and Hip dislocation, Sporadic None
adduction of shoulders, equinovarus, midfacial
extended elbows, and hemangioma,
exed wrists with ulnar gastroschisis, normal
deviation (Fig. 11) intelligence
Distal arthrogryposis, Camptodactyly and ulnar Feet usually involved, Autosomal Molecular
type 1 (DA1) deviation of all ngers, normal intelligence dominant
clenched hand
Congenital contractural Camptodactyly of PIP Marfanoid habitus, Autosomal Molecular
arachnodactyly joints, adducted thumbs crumpled helices, dominant
kyphoscoliosis
Multiple pterygium Dislocated radial head, Cleft palate, vertebral Autosomal Molecular
syndrome exion contractures with anomalies, hearing loss, recessive
pterygia, camptodactyly, scoliosis, normal
syndactyly, intelligence
arachnodactyly
Freeman-Sheldon (distal Contractures of hands Contractures of feet, Autosomal Molecular
arthrogryposis type 2A) (similar to DA1) (Fig. 12) distinctive facies dominant
(whistling face),
scoliosis
Diastrophic dysplasia Large joint contractures, Limb shortening, small Autosomal Molecular
hitchhiker thumbs, chest, cleft palate, cystic recessive
radial dislocation ear swelling
Pena-Shokeir (fetal Flexion contractures, Pulmonary hypoplasia, Autosomal Molecular
akinesia deformation multiple ankyloses, micrognathia, decreased recessive or
sequence) camptodactyly movement in utero, often sporadic
lethal
Mucopolysaccharidoses Joint contractures (esp. Hepatosplenomegaly, Autosomal Biochemical,
(e.g., Hunter and Hurler phalangeal), dysostosis coarse facial features, recessive molecular
syndromes) multiplex progressive pulmonary (most) or
and cardiac disease, X-linked
developmental delay (Hunter)

Congenital Contractures Joint Dislocations, Subluxations,

Congenital contractures may be isolated to one
area of the body or affect multiple part of the
body. Arthrogryposis is dened as congenital con-
tractures in two or more different areas of the body
and can be further categorized by three subgroups:
amyoplasia, distal arthrogryposis, and syndromic
arthrogryposis. Multiple congenital contractures
are present when there is decreased fetal move-
ment due to a neuropathic or myogenic process,
connective tissue disorder, space limitation, vas-
cular compromise, teratogenic exposure, or
maternal illness (Table 7) (Bamshad et al. 2009).
and Hyperextensibility
Dislocations and subluxations of the joints are
common in inherited connective tissue disorders,
particularly Ehlers-Danlos syndrome. There are
multiple types of Ehlers-Danlos syndrome, the
most common being the hypermobility type char-
acterized mainly by joint hypermobility with the
absence of skin fragility. The vascular type can
lead to the most morbidity and mortality and
should be recognized as early as possible as man-
agement recommendations can change. The vas-
cular type is characterized by arterial, intestinal,
464 J.D. Kaplan and C.L. Greene

Fig. 13 A girl with Madelung deformity showing a dor-

sally prominent distal ulna

Fig. 11 A girl with amyoplasia demonstrating internal

rotation of the shoulders, extended elbows, and exed wrists
(Courtesy of Shriners Hospitals for Children Philadelphia)

Fig. 12 Distal arthrogryposis in an individual with

Freeman-Sheldon syndrome (Courtesy of Shriners Hospi-
tals for Children Philadelphia) Fig. 14 A radiograph showing Madelung deformity
20 Genetic Syndromes 465

Table 8 Selected syndromes with Madelung deformity

Commonly/variably Inheritance
Syndrome Upper limb anomaly associated features pattern Testing
Leri-Weill Madelung deformity, radial Short stature, scoliosis Autosomal Molecular
dyschondrosteosis bowing, dorsal subluxation of the dominant
ulna
Seckel Madelung deformity, clinodactyly, Severe short stature, Autosomal Molecular
abnormal carpal bones microcephaly, hip recessive
dysplasia
Hereditary multiple One or more osteochondromas, Short stature, bony Autosomal Molecular,
osteochondromas Madelung deformity deformity, restricted joint dominant FISH
motion

and/or uterine rupture in addition to join
hypermobility and translucent skin. An individual
with recurrent dislocations or subluxations and/or
hyperextensible joints should be referred for
genetic evaluation. In addition, a child with a
single dislocation and a positive family history
of dislocations should be referred.
Madelung Deformity
Madelung deformity (Figs. 13 and 14) is an anom-
aly of the wrist caused by asymmetric growth at
the distal radial physis secondary to a partial
ulnar-sided arrest. See chapter Madelungs
Deformity for a more complete description
(Table 8).
Conclusion
Abnormalities of the upper extremity and the
limbs in general are common in many genetic
syndromes. The accurate diagnosis of a syndrome
can benet the patient and the family as there may
be changes in management recommendations and
recurrence risk. The orthopedic surgeon is vital in
recognizing that a syndrome may be the cause of
the patients limb anomaly and referring to genet-
ics for an accurate diagnosis and comprehensive
counseling.
References
Bamshad M, Van Heest A, Pleasure D. Arthrogryposis: a
review and update. J Bone Joint Surg Am. 2009;91
Suppl 4:406.
Dugan SL. Overview of genetic testing. In: Saal RA, editor.
Medical genetics in pediatric practice. Elk Grove Vil-
lage: American Academy of Pediatrics; 2013.
p. 23552.
Frazier TM. A note on race-specic congenital malforma-
tion rates. Am J Obstet Gynecol. 1960;80:1845.
OMIM: Online mendelian inheritance in man. http://www.
ncbi.nlm.nih.gov/Omim/searchomim.html
Schwabe GC, Mundlos S. Genetics of congenital hand
anomalies. Handchir Mikrochir Plast Chir. 2004;36
(23):8597.
Stevenson RE, Hall JG. Human malformations and related
anomalies. 2nd ed. Oxford: Oxford University Press;
2006.
Temtamy SA, McKusick VA. The genetics of hand
malformations. New York: Alan R Liss; 1978.
Thompson and Thompson. Genetics in medicine. 8th
ed. Philadelphia: Saunders; 2007.
 Skeletal Dysplasias
21
Alphonsus K. S. Chong, Rosalyn P. Flores, and
Eng Hin Lee

Contents Upper Extremity Manifestations of SD . . . . . . . . . . . 477

Clinical Presentation and Management . . . . . . . . . . . . . . 477
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 468
Summary and Future Directions . . . . . . . . . . . . . . . . . . 480
Classication . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 468
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 481
Pathogenetics . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 469
Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 469
Prenatal Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 470
Postnatal Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 470
Differential Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 472
General Approach to Treatment . . . . . . . . . . . . . . . . . . 472
Commonly Seen Conditions . . . . . . . . . . . . . . . . . . . . . . . 473
Achondroplasia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 473
Osteogenesis Imperfecta . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 474

A.K.S. Chong (*)

Department of Hand and Reconstructive Microsurgery,
National University Hospital, Singapore
Department of Orthopaedic Surgery, Yong Loo Lin School
of Medicine, National University of Singapore, Singapore
e-mail: alphonsus_chong@nuhs.edu.sg;
doscksa@nus.edu.sg
R.P. Flores
Department of Orthopaedic Surgery, National University
Hospital, Singapore
e-mail: rottifer@gmail.com
E.H. Lee
Department of Orthopaedic Surgery, Yong Loo Lin School
of Medicine, National University of Singapore, Singapore
Division of Paediatric Orthopaedics, National University
Hospital, Singapore
e-mail: eng_hin_lee@nuhs.edu.sg

# Springer Science+Business Media New York 2015 467

J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_21
468 A.K.S. Chong et al.

short stature and disproportion of the head, limbs,

Abstract
and spine. Over 400 types have been described.
Skeletal dysplasias (SD) are a heterogeneous
Birth prevalence of SD recognizable in the neonatal
group of genetic disorders of skeletal growth
period is 2.4 per 10,000 deliveries (Camera
and development resulting in abnormal shape,
et al. 1982). Twenty-three percent are stillborn and
size, and texture of skeleton and characterized
thirty-two percent die during the rst week of life.
by short stature. With recent advances in
Approximately 9.1 per 1,000 die in perinatal period.
research capabilities in genomics and molecu-
Thanatophoric dysplasia and achondrogenesis
lar biology, the genetic basis and molecular
account for 62 % of all lethal cases. Most common
mechanisms underlying many of these dyspla-
SDs seen in clinical practice are achondroplasia and
sias have been elucidated and the classication
osteogenesis imperfecta (OI). These four dysplasias
and nosology have now been expanded to over
represent two-thirds of all the cases of SD.
400 types. Many of the severe forms of SD do
As the clinical manifestations of SD are quite
not survive beyond the rst week of life. The
diverse, one simple way to think about the various
most common conditions seen in the clinic are
types of conditions is to consider that the mani-
achondroplasia and osteogenesis imperfecta,
festations can be due to (a) abnormal bone growth
both of which are caused by gene mutations.
(resulting in abnormal shape and size of the skel-
The diagnosis of SD can often be made pre-
eton), (b) abnormal number of bones (more or less
natally with biometric measurements of the
than normal), and (c) abnormal texture of the
fetus during gestation. After delivery, diagno-
bones (increased or decreased bone density).
sis can be made on clinical and radiological
features with the aid of biochemical investi-
gations and genetic testing. Upper limb man-
Classification
ifestations of SD are also diverse. Commonly
seen conditions in the clinic involving upper
The classication of SD was previously based on
limbs include osteochondroma (Ollier dis-
radiological features, histology, and clinical fea-
ease), hereditary multiple exostoses (HME),
tures. Most were also referred to by their
and brous dysplasia. The management of SD
eponyms, by terms describing a salient feature of
requires a multidisciplinary team approach
the disease, or by the presumed pathogenesis of
involving a pediatric geneticist, molecular
the disease. The fundamental problem encoun-
pathologist, radiologist, and pediatric ortho-
tered previously was that the exact pathogenesis
pedic surgeon. Genetic counseling plays an
of these diseases was rarely known.
important part in the overall management of
In the 1960s and 1970s, it was recognized that
these disorders. Orthopedic management is
genetic skeletal disorders were clinically and
mainly directed at treating the manifestations
genetically heterogeneous and this prompted a
of the disease such as spine and limb defor-
group of international experts from genetics, pedi-
mities and fractures. Current research on treat-
atrics, orthopedics, and radiology to come together
ment by bone marrow transplantation, stem
to reach a consensus on the nomenclature of what
cell therapy, and gene therapy is ongoing.
was then recognized as constitutional (or intrinsic)
disorders of bone (McKusick and Scott 1971).
Introduction The skeletal disorders were initially grouped into
ve categories: osteochondrodysplasias, dyso-
Skeletal dysplasias (SD) are a heterogeneous stoses, idiopathic osteolyses, chromosomal aber-
group of mainly genetic disorders involving dis- rations, and primary metabolic abnormalities.
orders of osteochondral development resulting in Numerous revisions were made in the 1970s to
abnormal shape, size, and texture of the skeleton. early 1990s to address the increasing complexity
Persons with SD can often be recognized by their of information on these skeletal disorders.
21 Skeletal Dysplasias
In 1999, the International Skeletal Dysplasia Soci-
ety (ISDS) was formed, and since then, the Nosol-
ogy and Classication of Genetic Skeletal
Disorders have been delegated to an ad hoc
group within the ISDS. This nosology provides
an overview of recognized diagnostic entities and
groups them according to clinical and radio-
graphic features and molecular pathogenesis.
In the 2010 revision of the nosology, 456 con-
ditions were included and placed in 40 groups
dened by molecular, biochemical, and/or radio-
graphic criteria (Warman et al. 2011).
The following Table 1 gives an overview of the
different groups and their corresponding diagnos-
tic criteria.
Pathogenetics
Recent advances in the understanding of the var-
ious causes of SD have been due in large part to
the study of the underlying molecular mecha-
nisms and gene mutations (Superti-Furga
et al. 2001). SD can be broadly grouped on the
basis of the function of the protein product of the
causative gene. This can be clinically relevant as
many of the disorders caused by genes whose
protein products have similar functions also
share similar clinical characteristics. Five broad
groups can be categorized based on the defective
molecular component and proteins/genes
involved as shown in Table 2 below.
Many of the genes mutated in SD affect
important functions of the growth plate (physis).
A good understanding of growth plate physiol-
ogy will be helpful in working out the possible
effects of gene mutations in various zones of the
physis. For example, in the resting zone, SOX9
gene mutation causes camptomelic dysplasia; in
the proliferative zone, FGFR3 gene mutation
causes achondroplasia, hypochondroplasia, and
thanatophoric dysplasia; in the hypertrophic
zone, PTHR1 gene mutation causes metaphyseal
dysplasia; and in the terminal differentiation
zone, RUNX2 gene mutation causes cleidocranial
dysplasia (Dietz and Mathews 1996; Zelzer and
Olsen 2003).
469
Table 1 Overview of the various groups and diagnostic
criteria adapted from the Nosology and Classification of
Genetic Skeletal Disorders, 2010
Group Diagnostic criteria
18 Based on a common underlying gene or
pathway (e.g., achondroplasia belongs to
group 1 or the FGFR3 chondrodysplasia
group)
917 Based on the localization of radiographic
changes to specic bone structures
(vertebrae, epiphyses, metaphyses,
diaphyses, or a combination thereof) or
of the involved segment (rhizo, meso,
or acro)
1820 Dened by macroscopic criteria in
combination with clinical features (bent
bones, slender bones, presence of multiple
dislocations)
2125, 28 Takes into account features of
mineralization (increased or reduced bone
density, impaired mineralization, stippling,
osteolysis) (e.g., osteogenesis imperfecta
and other disorders of decreased bone
density belong to group 25)
26 Identication of several novel molecular
mechanisms leading to hypophosphatemic
rickets
27 Lysosomal disorders with skeletal
involvement
29 Disorders with so-called abnormal
development of skeletal components
(exostoses, enchondromas, and ectopic
calcication)
30 Overgrowth syndromes with signicant
skeletal involvement
31 Genetic inammatory/rheumatoid-like
osteoarthropathies
3240 Dysostoses, anatomical criteria with
additional criteria reecting principles of
embryonic development such as limb
reduction or hypoplasia (proximal-distal
growth) versus terminal differentiation and
patterning of the digits or joint formation
Diagnosis
The diagnosis of SD usually requires a multidis-
ciplinary approach with the active participation
of a pediatric geneticist, a radiologist, and a
molecular pathologist with special interest in SD
as well as a pediatric orthopedic surgeon.
470 A.K.S. Chong et al.

Prenatal Diagnosis independent variable and the estimated fetal age

as the dependent variable. However, in order to
Long-bone biometry has been used extensively in assess the normality of bone dimensions, the ges-
the prediction of the fetal gestational age. Nomo- tational age is used as the independent variable
grams generally use the long bone as the and the long bone as the dependent variable.
Patients at risk for SD should seek prenatal care
Table 2 Pathogenetics of SD: 5 broad groups based on early to assess all clinical estimators of gestational
the defective molecular component and proteins/genes
age. In the prenatal diagnosis of infants with SD,
involved
there is a discrepancy between fetal size and ges-
Gene or protein tational age. Affected fetuses have been shown to
involved Clinical phenotype
have dramatic deviations from the 5th and 95th
Group 1: defect in structural cartilage proteins
condence limits (Romero et al. 1998). The
Collagen 2 (COL2A1) Achondrogenesis
2, spondyloepiphyseal graphs below show the biometry of the femur
dysplasia, Kniest dysplasia, and humerus during gestation (Figs. 1 and 2).
Stickler syndrome
Group 2: defect in cartilage metabolic pathways
Diastrophic dysplasia Diastrophic dysplasia,
sulfate transporter achondrogenesis IB, Postnatal Diagnosis
atelosteogenesis type
2, recessive multiple History
epiphyseal dysplasia When presented with a child with disproportion-
Group 3: defect in local regulators of cartilage growth
ate short stature, a focused history can give impor-
Fibroblast growth factor Achondroplasia,
receptor 3 (FGFR3) thanatophoric dysplasia,
tant clues as to the differential diagnosis. A
hypochondroplasia complete prenatal history should be obtained,
Group 4: defect in transcription factors including fetal biometry ndings during the pre-
Short-stature homeobox Dyschondrosteosis, Langer- natal ultrasound.
gene (SHOX) type mesomelic dysplasia As a part of the patients perinatal history, it is
Group 5: defect in tumor-suppressor genes important to ascertain the patients birth length, as
Exostosin 1 and 2 Multiple hereditary some patients with skeletal dysplasia may present
exostoses
with short stature at birth (e.g., achondroplasia)

Fig. 1 Biometry of femur during gestation

21 Skeletal Dysplasias
Fig. 2 Biometry of humerus during gestation
while others may have a normal birth length
with subsequent failure of linear growth (e.g.,
pseudoachondroplasia).
Family Evaluation
It is important to take a detailed family history to
determine if there is another family member with
SD in order to assess the mode of inheritance, if
any. SD is often transmitted by Mendelian inher-
itance. A pedigree that includes rst-degree rela-
tives is usually sufcient for screening, but
occasionally, a more extensive family history is
needed. Assessment of parental heights also plays
a role, as the child simply might have familial
short stature (Unger 2002).
Physical Examination
Growth parameters should be measured, which
include the patients height, weight, and head
circumference. For example, in patients with
achondroplasia, the head circumference is greater
than normal while the height is reduced dramati-
cally. It is also important to note the height for age
percentile of the patient. In general, if adult height
is under 150 cm or 5 ft, consideration of SD is
appropriate.
It is also necessary to determine the patients
proportions. The upper segment to lower segment
ratio and the arm span to height ratio are used to
471
document whether the spine or the limb is more
severely shortened. Limb shortening is further
classied depending on which segment is most
affected: rhizomelia (short proximal segments;
i.e., short humerus or femur, as in achondroplasia),
mesomelia (short middle segments; i.e., short
forearm or tibia, as in dyschondrosteosis), or
acromelia (short hands or feet, as in Grebe type
of chondrodystrophy) (Fig. 3).
When the patient is short but the body pro-
portions are normal, the diagnosis is usually
related to constitutional short stature, which may
be due to endocrine disorders, malnutrition, pre-
natal dwarsm, or one of the many dysmorphic
syndromes.
Patients with SD often have dysmorphism or
minor morphologic variations of the bones and
soft tissues. Dysmorphic features associated with
short stature suggest an underlying SD (Beals and
Horton 1995).
The presence of deformities (e.g., spinal defor-
mities such as lordosis, kyphosis, or scoliosis;
varus or valgus deformity of the extremities) in
patients with short stature is also highly sugges-
tive of a diagnosis of an SD.
Imaging Studies
Good-quality skeletal radiographs should be
obtained. It is necessary to request for a limited
472
Fig. 3 Types of
disproportionate limb
shortening
skeletal survey because normal ndings in a spe-
cic region can aid in making a differential diag-
nosis. An SD can almost always be diagnosed on
the basis of ve radiographs: lateral skull,
anteroposterior (AP) view of the pelvis, lateral
lumbar spine, AP view of the hand and wrist,
and AP view of the knee. In general, the dyspla-
sias are classied according to which part of the
skeleton is involved. The pattern of involvement
may include any or all of the following:
spondyloepiphyseal, metaphyseal, and diaphyseal
dysplasia. Aside from the pattern of involvement,
the region affected can also be used to narrow the
differential diagnosis (Unger 2002).
Other Tests
Many of the SD can be diagnosed from clinical
and radiographic features. However, a blood
chemistry prole can be obtained to exclude treat-
able metabolic disorders such as the mucopoly-
saccharidoses and rickets. A bone biopsy is rarely
indicated but may sometimes be useful to conrm
the diagnosis of dysplasia. In some instances,
more detailed genetic testing may be required for
purposes of conrmation of the diagnosis, docu-
mentation, and counseling.
A.K.S. Chong et al.
Differential Diagnosis
In considering the differential diagnosis, a few
important conditions should come to mind.
These are hypothyroidism, nutritional rickets,
failure to thrive, and non-accidental injuries.
General Approach to Treatment
A multidisciplinary approach is usually required
to manage SD as the children often have multiple
problems that require different areas of expertise.
Genetic counseling is an important aspect of man-
agement and a pediatric geneticist would be the
ideal person to conduct this appraisal. In centers
where antenatal diagnostic technology is avail-
able, preimplantation genetic diagnosis could
help the parents to have a normal child. In addition
to genetic counseling, the parents and child will
benet from counseling with respect to potential
medical and social problems that the child could
encounter during growth and development.
More specic to the type of SD, the child
will require management for spine and limb
21 Skeletal Dysplasias
deformities, possible fractures (e.g., in osteogen-
esis imperfecta), limb length discrepancy, and in
some situations limb lengthening for short stature.
Some of these will be covered in greater detail in
the sections below on Achondroplasia and Osteo-
genesis Imperfecta.
Commonly Seen Conditions
Achondroplasia
Achondroplasia is the most common SD, with
an incidence of 1 in 30,000 live births per year
(Shirley and Ain 2009). It is inherited as a fully
penetrant autosomal dominant trait. However,
more than 80 % of the cases are sporadic, wherein
affected individuals are the offspring of parents of
normal stature. Higher paternal age at the time of
conception of patients with achondroplasia sug-
gests that de novo mutations of paternal origin are
involved (Vajo et al. 2000).
Pathogenetics
Achondroplasia is caused by mutations of the
gene encoding broblast growth factor receptor
3 (FGFR3) on the distal short arm of chromosome
4. Most individuals with the clinical features of
achondroplasia have the same mutations that sub-
stitute an arginine for a glycine residue in the
transmembrane domain of the receptor in the
physis. FGFR3 is a negative regulator of chondro-
cyte proliferation and differentiation in the growth
plate. Mutations involving the FGFR3 result in
activation of the receptor and thus viewed as a
gain-of-function mutation (Vajo et al. 2000;
Horton 2006; Laederich and Horton 2010).
The clinical result of this mutation is underde-
velopment and shortening of the long bones
formed by endochondral ossication. It has been
suggested that FGFR3 inhibits both the prolifera-
tion and terminal differentiation of growth plate
chondrocytes and synthesis of extracellular matrix
by these cells. It also proposed that FGFR3 induces
premature terminal differentiation, thereby reduc-
ing the number of cells that contribute to template
synthesis (Laederich and Horton 2010).
473
Prognosis
Affected individuals have normal cognitive devel-
opment, although motor development may be
delayed. Patients with achondroplasia are
healthy compared to patients with other skeletal
dysplasias, but mortality rates in all age groups
are higher than those in the general population
because of sudden death in young infants, central
nervous system and respiratory problems in older
children, and cardiovascular problems in young
adults.
Diagnosis
The clinical manifestations of achondroplasia vary
at different stages of the affected individuals life.
At birth, short stature is evident, with the trunk
length in the lower range of normal and the
extremities shortened in a rhizomelic pattern.
Frontal bossing and midface hypoplasia are also
noted. Delayed motor development or apnea in
infants may be the result of foramen magnum
stenosis leading to cervical myelopathy. Hydro-
cephalus may occur during the newborn and
infantile period.
As the infant grows and begins to sit, thora-
columbar kyphosis may be noted. This is often
due to the infant slumping forward because of
trunk hypotonia combined with a relatively over-
sized head, a at chest, and a protuberant abdo-
men. This decreases with age (most tend to
resolve at 1218 months of age) as trunk strength
improves and the child begins to walk.
As the child continues to grow, genu varum
and lumbosacral hyperlordosis become evident.
The presence of genu varum is a clinical hallmark
of achondroplasia. Its cause remains controver-
sial, with several hypotheses being proposed
such as lateral collateral ligament laxity and bu-
lar overgrowth. Lumbosacral hyperlordosis is the
result of excessive anterior pelvic tilt while stand-
ing, producing a prominent abdomen and buttocks
with hip exion contractures. In adulthood, lum-
bar spinal stenosis may develop.
The rhizomelic shortening of the upper extrem-
ity of affected individuals can create disability
because of the difculty in reaching the top
of the head and the perineum for hygiene care.
474
This can also be exacerbated by elbow exion
contractures and radial head subluxation.
The average nal height of adult individuals
with achondroplasia is 132 cm for males (range:
118145 cm) and 125 cm for females (range:
112136 cm). This corresponds to a height that
is 67 standard deviations below the average for
unaffected individuals (Shirley and Ain 2009).
Management
Management of achondroplasia is directed toward
its different clinical manifestations. Indications
for surgery for the spinal conditions associated
with achondroplasia are not clearly dened,
although neurologic compromise generally war-
rants appropriate surgical intervention. The deci-
sion for limb lengthening is likewise difcult and
controversial, as the lengthening process is long,
arduous, and complicated. In addition, the func-
tional improvement after elective limb lengthen-
ing has not been well established.
Osteogenesis Imperfecta
Osteogenesis imperfecta (OI) is a heritable hetero-
geneous disorder of bone formation that may
affect more than 1 in 10,000 individuals. It is
distinguished by four features that present in the
following causal relationship: genetic disorder,
collagen defect, bone fragility, and frequent frac-
ture. Patients with OI are characterized by having
fragile bones. Many also have dentinogenesis
imperfecta (teeth appear brownish or bluish, soft,
translucent, prone to cavities, and crack easily),
blue sclerae (the effect of light reected from the
underlying choroid and its blood vessels through
the thin sclerae), and scoliosis (Roughley
et al. 2003). Minimal trauma can result in fractures
and bony deformities.
Pathogenetics
OI is a group of disorders caused by inherited or
spontaneous genetic mutations in the COL1A1 or
COL1A2 genes, which are responsible for
encoding the alpha-1 and alpha-2 chains of
type 1 collagen, respectively. Quantitatively or
A.K.S. Chong et al.
qualitatively decient brils are produced by
mutations in these genes, resulting in bone tissue
with altered mechanical properties. Bone tissue
anomalies are the most visible manifestation of
OI. Extraskeletal tissues and organs affected by
type 1 collagen defects include the sclerae, den-
tine, ear ossicles, skin, vessels, capillaries, and
heart valves.
Classification
In 1979, Sillence classied the condition into four
types based on clinical, genetic, and radiographic
ndings. Since then, three more types have been
added. Types I to IV are commonly associated
with mutations in the genes for type 1 collagen,
whereas no type 1 collagen gene mutation has
been detected in types V to VII (Kocher and
Shapiro 1998; Burnei et al. 2008).
Type I
Type I is the most common form of OI, estimated
at 35 per 100,000 births. It is mild in severity
and is inherited in an autosomal dominant
pattern. Patients have blue sclerae and bone fra-
gility and are deaf or have a family history of
presenile deafness. Infants are of normal weight
and length at birth and do not have multiple
fractures. This type is subdivided into type A
and type B according to the absence or presence
of dentinogenesis imperfecta, respectively.
Type II
This is the lethal form of OI, and the pattern of
inheritance is also autosomal dominant. However,
the lack of affected siblings in different series
suggests a new mutation of a dominant gene or a
nongenetic etiology. Its incidence is 1 per
40,00060,000 births. Infants are either stillborn
or die during the neonatal period and are fre-
quently small for gestational age. Blue sclerae
are present. In utero, multiple fractures occur and
the long bones are broad and shortened.
Type III
This is the most severe nonlethal form of OI, and
the pattern of inheritance for this type is autoso-
mal dominant. Its incidence is 12 per 100,000
21 Skeletal Dysplasias
births. During infancy, patients have bluish
sclerae. Later in life, they have normal or pale
blue sclerae. In most patients, the long bones are
shortened and bowed, and multiple fractures are
present at birth. This type is characterized by
progressive deformity of the long bones and
spine. Dentinogenesis imperfecta is also present.
Type IV
This type is rare, with an unknown frequency. It is
the clinically most diverse group and is inherited
as an autosomal dominant disorder. It encom-
passes all those individuals who do not meet the
criteria for types I to III. The phenotype can vary
from mild to severe, and the more severely
affected patients present with fractures at birth
and have moderate skeletal deformity and a rela-
tively short stature. Affected individuals have
blue sclerae at birth that eventually become
white. The long bones are of normal length, but
there may be mild femoral bowing noted. It is
subdivided into type A and type B according to
the absence or presence of dentinogenesis
imperfecta, respectively.
Type V
Type V OI is moderately deforming, and patients
exhibit moderate to severe bone fragility. It is
inherited in an autosomal dominant manner and
is characterized by hypertrophic callus develop-
ment after fracture, calcication of the
interosseous membrane at the forearm, and
hyperdense metaphyseal bands. Blue sclerae and
dentinogenesis imperfecta are absent.
Type VI
This type of OI presents with moderate to severe
skeletal deformity. It is characterized by frequent
fracture, vertebral compression, long-bone defor-
mity, normal-colored sclerae, and the absence of
dentinogenesis imperfecta. Blood tests show
slightly elevated levels of serum alkaline phospha-
tase. Histologic studies show sh-scale-like appear-
ance of the bone lamellae and an abundance of
osteoid, unmineralized bone matrix in the absence
of hypocalcemia or abnormalities in phosphate,
parathyroid hormone, or vitamin D metabolism.
475
Type VII
Patients with this type of OI also have moderate to
severe skeletal deformity and bone fragility.
Unlike other forms of OI, which are autosomal
dominant in transmission, type VII OI is autoso-
mal recessive in inheritance. The distinctive fea-
tures of this type are rhizomelic limb shortening
and coxa vara. Affected individuals lack blue
sclerae and dentinogenesis imperfecta.
A summary of the Sillence classication is
shown in Table 3 below.
Prognosis
Osteogenesis imperfecta is a disease with a wide
range of clinical presentations; thus, the quality of
life of patients with OI is highly variable. Type II
OI is lethal. Type I OI has the best prognosis as the
presence of multiple fractures is uncommon.
Types III to VII OI, although compatible with
life, can lead to signicant handicaps due to mul-
tiple fractures and deformities.
Diagnosis
Prenatal diagnosis of OI can be made with ultra-
sound. Type II OI has been diagnosed before
20 weeks age of gestation, with ndings of long-
bone fractures, angulation, shortening, localized
thickening secondary to callus formation, bowing,
and demineralization (Fig. 4). Multiple fractures
are also noted in the ribs resulting in a narrowed
chest. The skull may be thinner and, in severe
cases, the cranial vault has a wavy outline and is
easily compressible.
Although prenatal ultrasound can accurately
diagnose type II OI, it has been unsuccessful in
diagnosing the other types of OI, as there are
limitations to the evaluation of bone mineraliza-
tion with sonography.
At birth, the Sillence classication helps to
establish the diagnosis, but only when the clinical
signs are obvious. The presence of blue sclerae
and a positive family history are the most reliable
features.
Biochemical and genetic examinations based
on the study of type 1 collagen and DNA may be
necessary to differentiate OI from non-accidental
injury (NAI). These examinations are also useful
476 A.K.S. Chong et al.

Table 3 Sillence classification with additional types more recently described

Type Severity Inheritance Features
I Mild Autosomal Blue sclerae; bone fragility; infants are of normal weight and length at
dominant birth without multiple fractures
Type A: normal teeth
Type B: dentinogenesis imperfecta
II Lethal perinatal Autosomal Blue sclerae, stillborn or neonatal death, multiple intrauterine fractures
dominant
III Severe deforming Autosomal Normal or pale blue sclerae, multiple fractures present at birth,
dominant progressive deformity of long bones and spine, dentinogenesis
imperfecta
IV Mild to severe Autosomal Normal sclerae, moderate skeletal deformity, relatively short stature
dominant Type A: normal teeth
Type B: dentinogenesis imperfecta
V Moderately Autosomal Normal sclerae, moderate to severe bone fragility, hypertrophic callus
deforming dominant development after fracture, calcication of the interosseous membrane
at the forearm, hyperdense metaphyseal bands, normal teeth
VI Moderate to Autosomal Normal sclerae, frequent fracture, long-bone deformity, normal teeth,
severely deforming dominant sh-scale-like appearance of bone lamellae, and an abundance of
osteoid unmineralized bone matrix
VII Moderate to Autosomal Normal sclerae, normal teeth, rhizomelic limb shortening, coxa vara
severely deforming recessive

to distinguish OI from other genetic syndromes

such as hypophosphatasia and vitamin D disor-
ders that are associated with frequent fractures but
exhibit no structural alteration of type 1 collagen.
Bone mineral density (BMD) measurement
can also be used in the diagnosis of OI.
Although measured values can be normal in a
small number of cases, most patients with OI
have BMD values far below the normal range,
which can be a major risk factor for further
fractures.

Fig. 4 Bowed femur in a 12-year-old boy with osteogen-
esis imperfecta
Management
The goals of treatment in children with OI include
reduction of fracture rates, correction and preven-
tion of long-bone deformities and scoliosis, and
improvement of functional outcome.
The use of bisphosphonates in children with
OI, whether in oral (alendronate) or intravenous
(pamidronate) form, has been shown to demon-
strate a decrease in the frequency of fractures as
well as improvement of vertebral bone density
and quality of life (Rauch et al. 2003; DiMeglio
and Peacock 2006). The duration of treatment
must be limited to approximately 2 years, as
bone mineral density tends to stabilize or even
21 Skeletal Dysplasias
Fig. 5 Osteotomy of femur and insertion of Fassier-Duval
(FD) telescoping nail
decrease after the rst 2 years of treatment. It is
also important to note that bisphosphonates inter-
fere with bone formation and resorption; hence,
interference with healing following a fracture or
osteotomy is expected (Munns et al. 2004). Often,
the bisphosphonates are discontinued for short
duration before and after a planned osteotomy.
Surgical management in patients with OI
includes treatment of fractures as well as correc-
tion of long-bone and spinal deformities, followed
by early physical therapy in order to restore the
patient to self-sufciency as completely and rap-
idly as possible. Internal xation of long bones
using intramedullary rods or nails is the most
common surgical treatment for patients with OI.
Multilevel long-bone osteotomies as originally
described by Soeld (Soeld and Millar 1959)
in combination with the use of intramedullary
devices, whether telescopic or non-telescopic,
have been benecial in the restoration of the
bony axis in patients with OI (Fig. 5). In order to
address scoliosis in patients with OI, Luque
instrumentation (Hanscom et al. 1992) is a reliable
option in these patients. Although bone fragility
may be a deterrent to spinal instrumentation, the
477
use of bisphosphonates generally improves the
quality of bone, thereby allowing the use of instru-
mentation (Burnei et al. 2008).
Current research is directed toward the implan-
tation of smart intramedullary rods and bone mar-
row transplantations as viable treatment options
for patients with OI.
Upper Extremity Manifestations of SD
The spectrum of conditions under this diagnostic
umbrella is wide, so the upper limb manifestations
in skeletal are predictably diverse and heteroge-
neous. This situation has been accentuated with
the increased number of skeletal dystoses
included in the latest revision of the classication
of these disorders by the International Skeletal
Dysplasia Society in 2010 (Warman et al. 2011).
The etiology of the upper limb problems is
varied and may be multifactorial. The clinical
problems encountered may be attributed to mass
effect, growth disturbances, propensity to injury,
poor healing capacity, and in some cases malig-
nant change.
Certain deformities are more commonly asso-
ciated with particular diagnoses. This section will
focus on the range of upper limb problems in
patients with SD, and these will be discussed in
the context of the types of underlying conditions
that they are usually associated with.
Clinical Presentation and Management
The diagnosis of SD may be made prenatally, but
more often than not, it may only be obvious at
birth or even later. In some cases, the problem in
the upper limb may be the main reason for the
child to consult a physician. While SDs are
uncommon, with an incidence of about 1 in
5,000 live births (Alanay and Lachman 2011), it
is important to consider the possibility of this
diagnosis when a patient presents with features
of SD or a problem consistent with it.
As discussed above, the clinical evaluation
should include an assessment of height and limb
lengths (including proportions of segments).
478
A radiological assessment of the affected limb is
necessary, and that of the contralateral limb is
often helpful to provide a basis for comparison.
Depending on the condition, the patient may have
generalized laxity (e.g., Marfans syndrome,
hypochondroplasia) or contractures (e.g., campto-
dactyly with SD).
Short Upper Limb and Limb Length
Discrepancy
A short upper limb is part of the presentation of
the short stature in SD. Depending on the type of
short stature, the limb may be rhizomelic,
mesomelic, or acromelic. Limb shortening is
seen not just in disorders like achondroplasia but
also conditions like Ollier disease and hereditary
multiple exostoses (HME). The growth effects of
Ollier disease are more severe than in HME, lead-
ing to a shortened and enlarged bone.
However, bony involvement extends beyond
shortening. There is also associated bone bend-
ing and curvature. This increases the effective
shortening and can limit motion, thus limiting
the available working space of the limb. The
affected regions are also associated with increased
risk of fractures. Limb involvement may be
asymmetric, for example, in Ollier disease and
HME, leading to limb length discrepancy. Apart
from appearance, limb length discrepancy is bet-
ter tolerated in the upper limb compared to the
lower limb.
Joint or Bony Deformity
Shoulder Deformities
Kosenow syndrome, or pelvis-shoulder dysplasia,
is a rare SD that has its main features as bilateral
iliac and scapular hypoplasia (Elliott et al. 2000).
Arm and Elbow Deformities
Achondroplasia is often associated with a short
humerus, posterior bowing of the elbow, and a
subluxation of the radial heads. The elbow defor-
mity results in loss of elbow extension (Kitoh
et al. 2002). The radial head dislocation can
cause a secondary compressive injury to the pos-
terior interosseous nerve.
A.K.S. Chong et al.
Forearm Deformities
In HME, deformities of the forearms are common.
Typically, it appears like a Madelung deformity,
leading to forearm shortening and bowing giving
the appearance of a varus deformity of the forearm
and elbow (see chapter Benign Bone
Lesions). Forearm pronation and supination are
also restricted. Lengthening of the ulna can cor-
rect the deformity to a large extent. Hypoplastic or
absent radii is seen in Fanconi anemia.
Hands
The manifestations in the hand are varied. Achon-
droplasia patients have trident hands, i.e., an
extra space between the 3rd and 4th ray, so that
the ngers are divided into 3 groups. Deformities
include polydactyly, ectrodactyly, brachydactyly,
camptodactyly, synostosis, thumb hypoplasia
(e.g., Holt-Oram syndrome, Fanconi anemia),
and syndactyly (e.g., Apert syndrome). A short
metacarpal is a frequent manifestation in HME
(Pannier and Legeai-Mallet 2008).
In Fanconi anemia, the hand deformity may be
rst presentation of the clinical problem (Kozin
2008). Especially where there are additional nd-
ings such as growth retardation, skin hyperpig-
mentation, and microcephaly, this diagnosis
should be considered and further evaluation done.
Pain
Localized pain may be due to stress fractures,
mechanical effects of swellings on surrounding
structures, or rarely malignant change. It has
been reported that female patients with brous
dysplasia can have increased pain during preg-
nancy and during parts of the menstrual cycle
because of the effect of estrogen on the lesions
(Kaplan et al. 1988).
Fractures and Bony Deformity
Patients with SD may have propensity to fractures
due to localized (e.g., Ollier disease) or general-
ized deciencies in the mechanical properties of
the bone (e.g., osteogenesis imperfecta). On occa-
sion, the development of a fracture due to a trivial
injury may be the trigger to the diagnosis of the
underlying condition. Besides the usual morbidity
21 Skeletal Dysplasias
associated with fractures, there is an increased
likelihood of development of deformity due to
poor bone remodeling in these patients.
In osteogenesis imperfecta (OI), fractures of
the shoulder and upper extremity were found to
be common in an inpatient cohort of OI patients,
with 66 % of them having such an injury (Suko
2004). These patients have a 3337 % incidence
of upper extremity deformities, with the humerus,
radius, and ulna being the most commonly
affected bones. Beyond appearance, upper limb
deformities can affect functional activities and
interfere with activities of daily living.
While most cases of fractures in OI occur in
known patients with the diagnosis, a fracture may
be the initial presentation for the condition. Zionts
and Moon reported a series of patients with OI
who sustained fractures of the apophysis of the
olecranon, mostly after trivial trauma. The frac-
tures occurred at a mean age of 10, and half of the
patients were not known to have OI at the time of
elbow fracture (Zionts and Moon 2002).
Enchondromatosis (Ollier disease) affects the
short tubular bones of the hand and feet, as well as
the extremity long bones. It is not uncommon for
enchondromas to be diagnosed as incidental nd-
ings when an X-ray is done for some other reason.
Enchondromas give rise to focal areas of weak-
ness of tubular bones leading to pathological frac-
tures. When there is a fracture, it is necessary to
treat both the fracture and the underlying
enchondroma. The standard treatment for the
lesion is curettage, bone grafting, osteotomies,
and internal xation. In a case report, it has been
suggested that distraction osteogenesis can be
used to treat the deformity as well as the shorten-
ing following a humeral fracture, as well as con-
version of the diseased to normal bone (Tellisi
et al. 2008).
Fibrous dysplasia is a benign intramedullary
bro-osseous lesion. It most commonly affects a
single bone (monostotic) but can affect multiple
bones (polyostotic form). McCune-Albright syn-
drome is a rare disease that presents with
polyostotic brous dysplasia associated with
caf au lait spots and endocrinopathy. The
monostotic form may be asymptomatic and is
479
Fig. 6 Polyostotic brous dysplasia showing lesions in
the humerus and radius
often diagnosed incidentally, but it can present
with pain, deformity, and pathological fractures.
The risk of fracture is high in polyostotic brous
dysplasia. In a cohort study of 35 patients,
172 fractures were reported at mean follow-up
14.2 years. Of these, 44 of them involved the
humerus and forearm bones (Leet et al. 2004).
Figure 6 shows radiographs of brous dysplasia
involving the humerus, radius, and ulna with the
typical ground-glass appearance.
Nerve Problems
Involvement of the central nervous system is a
well-recognized problem in SD. However, the
peripheral nervous system can also be affected
from the manifestations of the disease. The
peripheral nerve can be affected by traction, com-
pression, or injury at the level of the nerve roots or
nerve itself. Multiple factors can cause nerve injury
including compression or traction due to bony
deformity or swellings. In achondroplasia, the pos-
terior interosseous nerve may be injured with a
radial head subluxation or dislocation. There are
also risks and the possibility of iatrogenic injury
during surgery. For example, patients with SD are
more likely to sustain nerve injury during limb
lengthening procedures (Nogueira et al. 2003).
The development of carpal tunnel syndrome
(CTS) in children is associated with mucopoly-
saccharidosis (MPS). MPS is the most common
480
cause of CTS in children. The condition is caused
by deposition of glycosaminoglycans (GAGs) in
the exor retinaculum and tenosynovium, causing
compression of the median nerve. It should be
noted that CTS presents differently in children.
The typical sensory symptoms are usually absent
and most patients deny any symptoms (Haddad
et al. 1997). The main presenting complaint is
difculty with ne motor activities or manual
clumsiness, which may be rst noticed by the
caregiver. Examination ndings are also different,
with unreliable Phalens and Tinels signs. Early
surgical treatment gives better results, so a high
index of suspicion is necessary and early nerve
conduction studies are useful. The treatment of
choice is surgical release of the carpal tunnel
with concomitant exor tenosynovectomy and
A1 pulley release.
Other Upper Limb Problems
Children with MPS are prone to the development
of trigger digits. The underlying pathology is sim-
ilar to that of carpal tunnel syndrome in these
patients, with deposition of GAGs around the
exor tendon at the level of the A1 pulley. If
untreated, the condition can lead to exion con-
tracture of the digits. The recommended treatment
is release of the trigger with a possible need to
partially resect the exor digitorum supercialis
tendon (Van Heest et al. 1998). The surgery is
often done concurrently with the release of the
carpal tunnel (White and Sousa 2013).
Malignant Change
Malignant change is an uncommon but serious
problem associated with some conditions.
Enchondromatosis (Ollier disease and Maffucci
syndrome) is associated with malignant change
of the enchondroma to chondrosarcoma. The esti-
mated risk varies between 20 % and 50 %.
Maffucci syndrome has been shown to be associ-
ated with a high risk of malignant change, and also
with poorer prognosis (Pannier and Legeai-Mallet
2008).
In hereditary multiple exostoses, there is an
associated risk of malignant transformation of
the osteochondroma (Pannier and Legeai-Mallet
2008). Malignant change is more likely to involve
A.K.S. Chong et al.
the axial bones, such as the pelvis, scapula, ribs,
and spine. Malignant change is rarely seen in
childhood and is more often seen after the end of
growth. In patients with enchondromatosis and
HME, regular surveillance of the lesions is
recommended and it has to be carried out well
into adulthood.
Summary and Future Directions
SDs are a heterogeneous group of mainly genetic
disorders involving osteochondral development
resulting in abnormal shape, size, and texture of
bone and short stature. In the recent Nosology and
Classication of Genetic Skeletal Disorders
(2010), 40 groups have been identied based on
molecular, genetic, and radiological criteria. The
diagnosis and management of SD are best accom-
plished by a multidisciplinary team consisting of a
pediatric geneticist, a molecular pathologist, a
radiologist, and a pediatric orthopedic surgeon.
Genetic counseling forms an important part of
the management. Many of the clinical manifesta-
tions of SD will require the expertise of a pediatric
orthopedic surgeon with special interest in defor-
mity correction and limb reconstruction.
Current genetic approaches in research on
achondroplasia have been directed at interfering
with the synthesis of the FGFR3 gene, or blocking
its activation, or inhibiting its tyrosine kinase
activity, or promoting its degradation, or antago-
nizing its downstream signals. Recent studies in
transgenic mice with high concentrations of brain
natriuretic peptide (BNP) showed increased
physeal activity resulting in increased bone
growth (Nakao-Lab, Kyoto). A C-type natriuretic
peptide (CNP) has been found to antagonize the
effects of FGFR3 on endochondral ossication
through the MAPK-mediated FGFR3 signals.
Rescue of bone growth deciency was seen in
the achondroplastic mice even at a 10-fold lower
dose of CNP (Yasoda et al. 2009). Work is cur-
rently directed at bringing this to clinical trials.
For osteogenesis imperfecta, there have been
some efforts made in using bone marrow trans-
plantation as a way to increase the normal osteo-
blasts in a child with OI. Horowitz et al. (2001)
21 Skeletal Dysplasias
reported on 3 children who underwent bone mar-
row transplantation and showed increased osteo-
blast density, increased mineralization, and
decreased incidence of fractures. These children
apparently also grew in height. There are also
some efforts directed at using gene therapy to try
to convert the more severe types of OI to milder
forms. Gene therapy is quite challenging as there is
a high mutation spectrum in OI (Marini et al. 2010).
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 Toe-to-Hand Transfers in Children
22
Neil Ford Jones

Contents Outcomes of Toe Transfers for Congenital

Hand Anomalies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 507
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 484
Conclusions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 509
Conventional Reconstruction of Amputations
in Children . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 484 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 509
Toe-to-Hand Transfers After Trauma
in Children . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 484
Indications for Toe-to-Hand Transfers
After Trauma in Children . . . . . . . . . . . . . . . . . . . . . . . . . 485
Conventional Reconstruction of Congenital
Hand Differences . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 487
Toe-to-Hand Transfers for Congenital Hand
Differences . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 489
Indications for Toe-to-Hand Transfers for
Congenital Hand Differences . . . . . . . . . . . . . . . . . . . . . . 490
Timing of Toe Transfers for Congenital Hand
Differences . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 496
Preoperative Assessment of a Child for
a Toe-to-Hand Transfer . . . . . . . . . . . . . . . . . . . . . . . . . . . . 496
Anatomy of the Great Toe and Second Toe . . . . . . 499
Surgical Technique . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 499
Dissection of the Donor Foot . . . . . . . . . . . . . . . . . . . . . . . . 502
Dissection of the Recipient Hand . . . . . . . . . . . . . . . . . . . . 503
Closure of the Foot . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 503
Transfer of the Toe to the Hand . . . . . . . . . . . . . . . . . . . . . 504
Outcomes of Toe Transfers for Posttraumatic
Reconstruction in Children . . . . . . . . . . . . . . . . . . . . . . . . 506

N.F. Jones
University of California Irvine, Irvine and Shriners
Hospitals, Los Angels, CA, USA
e-mail: nfjones@uci.edu

# Springer Science+Business Media New York 2015 483

J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_22
484
Abstract
Miraculous improvement in hand function can
be achieved by microsurgical toe-to-hand trans-
fers, either for children who have sustained
severe trauma resulting in amputations of their
thumb or ngers or for children with congenital
absent digits, due to symbrachydactyly, congen-
ital constriction ring syndrome, or transverse
and longitudinal deciencies.
Introduction
Buncke et al. (1966) experimentally performed a
microsurgical toe-to-hand transfer in a monkey
in 1964, and this was followed by the rst micro-
surgical second toe-to-thumb transfer in a human
by Yang in 1966 (Yang 1977). Cobbett (1969)
performed the rst great toe-to-thumb transfer
based on the plantar arterial system, and Buncke
(1973) performed the rst great toe-to-thumb
transfer based on the rst dorsal metatarsal
artery. Great toe transfers have subsequently
evolved to minimize the harvest of tissues from
the foot and to customize the appearance of
the great toe similar to that of the thumb, using
the Morrison wraparound ap (Morrison et al.
1980) and the trimmed toe transfer (Wei et al.
1988; Upton 1988). Great toe and second toe
transfers for posttraumatic reconstruction of
amputations of the thumb as well as single sec-
ond toe, simultaneous double second toe trans-
fers (OBrien 1978; Coskunrat et al. 2005), and
combined second and third toe transfers (Wei
et al. 1989) for posttraumatic reconstruction of
multiple digital amputations have subsequently
evolved for specic indications.
Conventional Reconstruction
of Amputations in Children
Fortunately severe amputations of the thumb
and ngers are relatively rare in children. The
mechanism of injury may be due to lacerations,
N.F. Jones
crush injuries, explosions, and burns or due to
disseminated intravascular coagulation related
to septicemia. For sharp amputations, replanta-
tion should be attempted if at all possible, if
necessary using vein grafts (performed on a
back table) from the dorsal radial artery or even
from the brachial artery at the elbow. Ectopic
replantation in the groin or axilla may be con-
sidered in very unusual circumstances such as
gross contamination. Conventional reconstruc-
tion of non-replantable amputations or failed
replantations includes pollicization of a normal
or injured nger for thumb reconstruction and
distraction lengthening for thumb and nger
reconstruction.
Toe-to-Hand Transfers After Trauma
in Children
Microsurgical toe-to-hand transfers have revolu-
tionized posttraumatic reconstruction in adults
after amputation of the thumb and/or ngers. Toe
transfers should be considered as the optimal tech-
nique for reconstruction of amputated ngers and
thumbs in children, just as in adults. It is a single-
stage procedure that provides greater length and
maintains the potential for growth, compared with
distraction lengthening. For thumb reconstruction,
it preserves all four ngers or the remaining ngers
compared with pollicization.
The second toe is always used for nger
reconstruction and is preferred for thumb recon-
struction in younger children. The second toe
also provides the most inconspicuous donor site
in the foot and is preferred by cultures requiring
the use of sandal footwear. The great toe and its
variations are preferred for thumb reconstruction
in older children, when the appearance of the
new thumb becomes more important and
when multiple ngers are also amputated since
the great toe has a greater area of sensate pulp.
The Morrison wraparound (Morrison et al. 1980)
and trimmed great toe (Wei et al. 1988; Upton
1988) variations of a great toe transfer minimize
the harvest of tissues from the foot and provide
22 Toe-to-Hand Transfers in Children
Fig. 1 (ac) Photographs and radiograph of a 3-year-old boy who sustained a traumatic amputation of his left thumb
the most normal appearance of the reconstructed
thumb. The trimmed toe technique allows the
potential for growth in children, but the Morrison
wraparound does not.
Soft tissue coverage of metacarpal amputation
stumps previously covered with skin grafts may
need to be replaced using a groin ap or a reverse
radial forearm ap, prior to toe transfers to recon-
struct a metacarpal hand. Similarly, severe
thumb-index nger web-space contractures may
need to be released before performing a toe-to-
thumb transfer, utilizing a groin ap, a reverse
radial forearm ap, or a reverse posterior
interosseous artery ap. Alternatively, the toe-
to-thumb transfer and thumb-index nger
web-space release can be performed simulta-
neously, using a small dorsalis pedis artery skin
ap based on the same arterial pedicle or using a
reverse radial forearm ap to cover the web space
and the proximal radial artery to provide arterial
inow to the toe transfer.
Several small series of toe-to-thumb and toe-
to-nger transfers have been reported for posttrau-
matic reconstruction of childrens hands with
485
success rates comparable to adults (Yoshimura
1980, 1984; Vilkki 1989; Shvedovchenko 1993;
Kay et al. 1996; Yu et al. 1999; Wei et al. 1997a;
Wei and Mardini 2003).
Indications for Toe-to-Hand Transfers
After Trauma in Children
The indications for toe-to-thumb transfers after
trauma in children include:
(i) Isolated amputations of the thumb from dis-
tal to the CMC joint out to the accepted
critical functional level at the midpoint of the
proximal phalanx (Figs. 1, 2, 3, and 4). Very
rarely, an emergency toe transfer may be indi-
cated for reconstruction of a degloved thumb
with an intact bony skeleton and tendons.
(ii) Amputations of the thumb and several other
ngers (Figs. 5 and 6).
(iii) Amputation of all ve digits the metacar-
pal hand (Wei et al. 1997b, 1999; Figs. 7, 8,
and 9).
486 N.F. Jones

Fig. 2 (a) Dissection of the rst dorsal metatarsal artery (FDMA) as the arterial supply to a right second toe transfer. (b)
Second toe transfer

Fig. 3 (a) Dissection of the exor pollicis longus tendon pollicis longus tendon. (c) Repair of the exor digitorum
and radial and ulnar digital nerves to the thumb. (b) Repair longus tendon to the exor pollicis longus tendon
of the extensor digitorum longus tendon to the extensor
22 Toe-to-Hand Transfers in Children 487

Fig. 4 (a) Immediate intraoperative photograph of the right second toe transfer to the left thumb. (b, c) One year after
right second toe-to-left thumb transfer

Fig. 5 (a) Eleven-year-old boy who sustained amputations of his right thumb, index, and middle ngers and the distal
phalanx of the ring nger, initially covered with a groin ap. (b) Left second toe transfer

The indications for toe transfers for nger Conventional Reconstruction

reconstruction in children include: of Congenital Hand Differences

(i) Complete or partial amputation of multiple or Conventional non-microsurgical reconstruction

all four ngers but with an intact thumb of congenital absent digits usually involves
(ii) Amputation of all ve digits the metacar- pollicization of the index nger for children
pal hand (Wei et al. 1997b, 1999) born with a hypoplastic or absent thumb
488 N.F. Jones

Fig. 6 (a) Left second toe to right thumb transfer. (b) The left foot donor site

Fig. 7 (ac) Palmar and dorsal views and radiograph of an 8-year-old boy with burns of his left hand resulting in
amputation of all ve digits

(Buck-Gramcko 1971; Manske and McCarroll
1985) and nonvascularized toe phalangeal bone
grafting (Entin 1959; Carroll and Green 1975;
Goldberg and Watson 1982; Buck-Gramcko and
Pereira 1990; Radocha et al. 1993; Cavallo
et al. 2003; Jones 2004; Gohla et al. 2005;
Unglaub et al. 2006) and distraction lengthening
(Matev 1970; Kessler et al. 1977; Seitz and
Froimson 1991; Ogino et al. 1994; Pensler
et al. 1998; Hulsbergen-Kruger et al. 1998; Dhalla
et al. 2001; Miyawaki et al. 2002; Matsuno
et al. 2004; Seitz et al. 2010) of hypoplastic digits
22 Toe-to-Hand Transfers in Children 489

Fig. 8 (a) The thumb-index nger web space was released with full-thickness skin grafts, and the index nger metacarpal
was resected to develop a mobile thumb metacarpal as a foundation for a great toe transfer. (b, c) Left great toe transfer

Fig. 9 (ad) One year following the left great toe-to-left subsequently underwent reconstruction of two ngers
thumb transfer, the child already has pinch between the using a combined second-third toe transfer
great toe transfer and the middle nger metacarpal. He

due to symbrachydactyly, cleft hand, or congeni-
tal constriction ring syndrome.
Toe-to-Hand Transfers for Congenital
Hand Differences
The rst microsurgical toe transfer to reconstruct a
congenital hand difference was performed by
OBrien et al. (1978) in 1977 who successfully
transferred the great toe to reconstruct a
hypoplastic thumb in two children. Yoshimura
(Yoshimura 1980, 1984) performed 13 toe trans-
fers in 12 children, 10 of whom had sustained
traumatic amputations, but 2 had congenital dif-
ferences 1 child had an absent thumb, index, and
middle ngers, and the second child had congen-
ital constriction ring syndrome involving one n-
ger. May et al. (1981) reported bilateral great toe-
to-thumb transfers to reconstruct a 9-year-old boy
with bilateral aplasia of his thumb, index, and
middle ngers. After other case reports (Nyarady
490
et al. 1983; Meals and Lesavoy 1983), several
surgeons have reported larger series of microsur-
gical toe-to-hand transfers to reconstruct various
congenital differences of the hand (Gilbert 1982,
1985; Lister 1988; Eaton and Lister 1991;
Shvedovchenko 1993; Vilkki 1995; Kay and
Wiberg 1996, Kay and McGuiness 1999; Kay
et al. 1996; Van Holder et al. 1999; Foucher
et al. 2001; Richardson et al. 2004; Jones
et al. 2007; Jones and Kaplan 2013).
Indications for Toe-to-Hand Transfers
for Congenital Hand Differences
Microsurgical toe-to-hand transfers for recon-
struction of congenital hand differences have not
evolved to a similar extent as toe transfers for
reconstruction after trauma and remain controver-
sial. Firstly, surgeons may be reluctant to risk the
small but potential loss of a toe transfer in a child
who is already missing one or more digits in their
hands. Secondly, some pediatric hand surgeons
still maintain that children with unilateral absence
of one of more digits adapt to their impairment as
they grow or can be helped with a static or func-
tional prosthesis. Finally, parents may be reluctant
to accept a very complicated reconstructive pro-
cedure that carries a small risk of ending up with a
missing great or second toe as well as more scars
on the hand to show for the failed endeavor.
Rather than just showing pre- and postopera-
tive photographs or videos or fabricating mou-
lages, the authors practice is to arrange for
prospective parents to meet the parents of a child
with a similar congenital hand difference who has
previously undergone a toe transfer, so that the
parents can see for themselves the potential func-
tion and appearance of the reconstructed hand, as
well as speak with the parents about their concerns
(Jones 2013).
Very little has been published on the specic
indications for microsurgical reconstruction of
congenital absent digits. Most pediatricians and
even some hand surgeons do not understand
which children should be considered for micro-
surgical reconstruction. Previous reports of toe
N.F. Jones
transfers for congenital hand differences have all
been based primarily on the specic embryologi-
cal diagnoses, according to the classication of
the International Federation of Societies for Sur-
gery of the Hand (Swanson 1976). However, it is
the authors contention that specic indications
for toe transfers are more logically dened by
the anatomy of the hand anomaly itself, a concept
that is also much more easily understood by refer-
ring pediatricians and surgeons (Jones 2012).
From an analysis of 235 hands in 204 children
born with congenital absent digits over a 15-year
period, Jones and Kaplan (Jones 2012) developed
a simple documentation system, which allows
hand surgeons to describe the morphological or
radiographic appearance or the functional status
of a childs hand with congenital absent digits,
based on which digits are missing and their level
of absence. Each hand can be described by three
letters R (radial), C (central), and U (ulnar) as well
as ve numbers. The rst letter and number des-
ignate which rays are missing, and the second and
third letters and numbers designate which rays are
present. Consequently, an absent thumb would be
designated as R1U4, a hand with a thumb but
absent ngers would be designated as U4R1, and
complete absence of all ve digits would be desig-
nated as R5. The spectrum of radial deciencies
includes R1U4, R2U3, R3U2, and R4U1. There
are 15 morphological phenotypes of congenital
absent digits the three most common phenotypes
being U4R1 (thumb but absence of all four ngers
corresponding to the monodactylous type III
symbrachydactyly), R1U4 (absent thumb), and
R5 (aplastic hand). Unlike most other classica-
tions, this documentation system not only facili-
tates communication between hand surgeons but
also incorporates all the previous subclassication
systems that have attempted to describe congeni-
tal absent digits in radial, central, and ulnar de-
ciencies, symbrachydactyly, and congenital
constriction ring syndrome. However, most
importantly, it has allowed the development of
an algorithm that predicts whether conventional
or microsurgical reconstruction is indicated for
each specic phenotype. Of the 15 phenotypes,
only 7 phenotypes are potential indications for
22 Toe-to-Hand Transfers in Children
Fig. 10 Schematic representation of the indications for microsurgical toe-to-hand transfers for congenital differences of
the hand
Fig. 11 (a, b) Photograph and radiograph of a 2-year-old
girl with congenital constriction ring syndrome affecting
her right thumb. The thumb is missing from just distal to
microsurgical reconstruction with toe-to-hand
transfers: R1U4, R2U3, R3U2, R4U1, R5,
U4R1, and occasionally C3R1U1. For a child
missing a thumb but with four relatively normal
ngers (R1U4), it is intuitive to reconstruct a
thumb to oppose to these ngers, either by
pollicization of the index nger or by a toe-to-
thumb transfer. Similarly for a child missing all
four ngers (U4R1), it is logical to reconstruct one
or two ngers to allow pinch and grasp to the
normal thumb, either by distraction lengthening
or by toe-to-hand transfers.
There appear to be four indications for consid-
ering microsurgical reconstruction of an absent
thumb (Jones 2013; Fig. 10):
491
the metacarpophalangeal joint, but the thenar muscles are
preserved, classied as an R1U4 hand [59]
(i) Isolated absence of the thumb with four
normal or relatively normal ngers, in
which the carpometacarpal joint and base
of the thumb metacarpal and thenar mus-
cles are preserved (R1U4). This is usually
seen in congenital constriction ring syndrome
or occasionally transverse failure (Figs. 11, 12,
and 13). Reconstruction of these thumbs with
a toe transfer is superior to index nger
pollicization, distraction lengthening, or
nonvascularized toe phalangeal bone grafting,
because it provides greater length and the
potential for growth and preserves the full
complement of ngers. A second toe transfer
in a 23-year-old child will provide almost
492 N.F. Jones

Fig. 12 (a, b) The

congenital constriction ring
syndrome also caused an
above knee amputation of
the left lower extremity and
an abnormality of the right
great toe, so only the right
second toe was available for
transfer

Fig. 13 Three years

postoperatively, the right
second toe-to-right thumb
transfer has comparable
appearance to the normal
left thumb and excellent
opposition to the small
nger

5 cm length of bone and soft tissue compared
with only 1317 mm length of a non-
vascularized toe phalangeal bone graft or 3 cm
that can be achieved by distraction lengthening,
albeit with a secondary bone graft procedure. If
the thenar muscles are poorly developed, an
opposition tendon transfer using the exor
digitorum sublimis from the ring nger can be
performed primarily at the same time as the toe
transfer or at a secondary procedure.
However, if there is an isolated absence of
the thumb and four relatively normal ngers,
but the carpometacarpal joint is absent,
pollicization of the index nger remains the
gold standard of reconstruction.
(ii) Absence of the thumb as well as the index
and middle (and ring) ngers but with one
or two ngers remaining on the ulnar side
of the hand (R2U3, R3U2, and R4U1). This
is usually seen with severe radial longitudinal
deciencies or the monodactylous type III
atypical cleft hand form of symbrachydactyly
(Blauth 1971; Fig. 14). A second toe can be
transferred to reconstruct the absent thumb
with minimal donor site morbidity, but the
modied wraparound (Morrison et al. 1980)
22 Toe-to-Hand Transfers in Children 493

Fig. 14 (a, b) Photograph

and radiograph of a 3-year-
old girl with severe
congenital constriction ring
syndrome affecting her
right thumb, index, middle,
and ring ngers. The thumb
is missing from just distal to
the metacarpophalangeal
joint, but the thenar muscles
are preserved and the ring
and small ngers remain
relatively functional

Fig. 15 (a, b) Because the second and third toes in both feet were affected by the congenital constriction ring syndrome, a
right trimmed great toe transfer was designed for reconstruction of the thumb

or trimmed great toe (Wei et al. 1988; Littler
1994) techniques can occasionally be consid-
ered in older children to provide both an excel-
lent functional result and a thumb very similar
in appearance to the contralateral normal thumb
(Figs. 14, 15, and 16). If the carpometacarpal
joint is absent in these severe radial deciency
phenotypes, the second toe metatarsal can be
xed to the scaphoid or spliced into the radial
styloid as described by Vilkki (1989) and by Yu
(Yu et al. 1999). Very rarely, a similar morpho-
logical deformity also occurs in one or both feet
(cleft hand and cleft feet), and the abnormal
great toe is sometimes amputated to facilitate
the tting of shoes. In these situations, the
abnormal great toe can be transferred to recon-
struct the absent thumb (Chang and Jones
2002a).
(iii) Unilateral (or, extremely rarely, bilateral)
absence of the thumb and all four ngers
(R5). This is usually seen with the adactylic
type IV symbrachydactyly (Blauth and
Gekeler 1971) or with transverse failure of
formation.
(iv) Very rare cases in which the thumb or
radial side of the hand becomes totally
494
Fig. 16 Two years
postoperatively, the right
trimmed great toe-to-right
thumb transfer has
comparable appearance to
the normal left thumb and
excellent opposition to the
only normal small nger
Fig. 17 (ac) Palmar and dorsal photographs and radio-
graph of a 6-month-old baby girl with symbrachydactyly
of her right hand, with a normal thumb but absence of all
enveloped by a rapidly growing vascular
malformation which is unresponsive to
embolization or sclerotherapy or by pro-
gressive macrodactyly. This situation can
potentially be salvaged by radical resection
of the thumb but preserving the carpo-
metacarpal joint and base of the thumb meta-
carpal and thenar muscles, followed
immediately by a second toe transfer (Foucher
et al. 2001; Carlsen and Jones 2007).
There appear to be two indications for consid-
ering toe transfers to reconstruct absent ngers
(Jones and Kaplan 2013; Fig. 10):
N.F. Jones
four ngers which are just represented by nubbins, classi-
ed as a U4R1 hand [59]
(i) Absence of all four ngers (from the carpus
out to the base of the middle phalanges) but
with a normal thumb (U4R1). This is usually
seen with the monodactylous type III
symbrachydactyly, transverse failure of for-
mation, congenital constriction ring syndrome,
or severe ulnar longitudinal deciency (Figs. 17
and 18). The location into which the toe is
transferred depends on the anatomy. Microsur-
gical reconstruction of a nger can be accom-
plished by a single second toe transfer into the
middle, ring, or small nger position. Place-
ment of the toe transfer on the ulnar side of the
hand allows both grasp of large objects as well
22 Toe-to-Hand Transfers in Children
Fig. 18 (a) Second toe transfer performed at 2 years of
age. (b, c) Five years after a second toe-to-small nger
transfer, the child has excellent grasp and very precise
pinch between the thumb and second toe transfer. (d) The
as tip-to-tip pinch (Fig. 18b, c). An alterna-
tive option if the parents are agreeable is to
transfer two second toes, either simulta-
neously or sequentially, into the middle and
small nger positions to allow three-point
pinch (Figs. 19, 20, and 21). Occasionally, if
the parents are willing to accept the appear-
ance of the donor foot, combined second and
third toes can be transferred into the middle-
ring or ring-small nger positions. The range
of motion of a toe transfer used for
495
postoperative radiograph shows 90-90 interosseous wire
xation of the second toe metatarsal to the base of the small
nger metacarpal
reconstruction of a nger depends on whether
there is an existing metacarpophalangeal joint
in the hand. If the proximal phalanx of a
toe transfer can be xed to a remnant of
the proximal phalanx of a nger, excellent
motion can be expected at the existing
metacarpophalangeal joint, whereas if the
metatarsal of a toe transfer has to be xed to
a metacarpal in the hand, the motion achieved
at the metatarsophalangeal and proximal
interphalangeal joints is considerably less.
496
Fig. 19 (a, b) Photograph
and radiograph of a 2-year-
old boy with
symbrachydactyly of his
left hand. The thumb is
present, but all four ngers
are absent and just
represented by nubbins,
classied as a U4R1
hand [59]
(ii) Complete absence of all ve digits (R5).
This is usually seen in the adactylic type IV
symbrachydactyly (Blauth 1971) or trans-
verse failure of formation (Fig. 22). Recon-
struction may be accomplished using two
second toe transfers into the thumb and ring
or small nger positions to provide grasp and
tip-to-tip pinch. This can be performed simul-
taneously but is probably better performed
sequentially, so that positioning of the second
transfer into a nger position can be adjusted
to the position and mobility of the rst trans-
fer into the thumb position (Figs. 23 and 24).
Timing of Toe Transfers for Congenital
Hand Differences
The optimal age for performing a toe transfer to
reconstruct a congenital hand difference has not
been dened. Generally, the earlier that a toe
transfer is performed, the better the chance of
cortical integration. Children with a unilateral
congenital hand anomaly should probably be
reconstructed at an earlier age, before the use of
the contralateral normal hand dominates. How-
ever, the real limiting factor in pediatric toe trans-
fers is the size of the donor and recipient vessels,
N.F. Jones
which must be of adequate size to facilitate the
microsurgical anastomoses. Gilbert (1982, 1985)
and Lister (Lister 1988) have performed toe trans-
fers in children as young as 612 months of age,
but toe transfers have also been successful in
older children between 11 and 17 years of age
(Spokevicius 1997). The senior author usually per-
forms toe transfers between 2 and 3 years of age.
Preoperative Assessment of a Child
for a Toe-to-Hand Transfer
After a traumatic injury to a childs hand, there is
always some blame, either attached to or accepted
by one of the parents. The parental response to a
child born with a congenital hand difference
resembles the bereavement response. Each parent
must deal with their own sense of loss, as well as
the obligation to direct the treatment of their child.
Listening to the childs parents and allowing them
to express their understanding of their childs con-
dition and their hopes and expectations for recon-
struction are vitally important. Some surgeons
have recommended that the parents and child
meet with a clinical psychologist prior to consid-
ering a toe-to-hand transfer, and it has been
documented that psychological counseling may
22 Toe-to-Hand Transfers in Children
Fig. 20 (ad) Simultaneous bilateral second toes were transferred into the middle and small nger positions
play an integral role in decision-making in pedi-
atric hand surgery (Bradbury et al. 1994). The
parents should be prepared psychologically for
the substantial amount of time and energy that
will be required during the pre- and postoperative
period. In addition, they must understand and
accept the fact that a toe transfer can fail, leaving
the child with the same decit in the hand but also
now missing a toe in the foot. If the parents and/or
child cannot accept the time commitment and the
potential risk of failure, this surgery should not be
considered.
497
Usually with congenital hand differences, the
child will have been examined by a pediatrician
for other congenital anomalies and possibly even
by a geneticist to exclude any syndrome. A
detailed examination of the involved upper
extremity should be performed or if the child is
very young, observed while playing. More prox-
imal shoulder or elbow abnormalities should be
excluded such as Polands syndrome or radial-
ulnar synostosis. The presence or absence of a
radiocarpal joint as well as the number and length
of any metacarpal bones should be determined.
498
Fig. 21 (a, b) Seven years postoperatively, both ngers have normal sensation and continue to grow and provide three-
point pinch with the mobile thumb
Fig. 22 (ac) Dorsal-palmar photographs and radiograph
of a 2-year-old boy with symbrachydactyly affecting all
ve digits of his left hand. The digits are missing from the
The presence or absence of a thumb and whether
there is a thumb carpometacarpal joint are impor-
tant if a toe-to-thumb transfer is being considered.
Dimpling of the skin in symbrachydactyly may
indicate the presence of exor tendons in the
vicinity of the nubbins.
One or other of the childs feet may be posi-
tioned close enough to the affected hand, so that
the parents and/or child can visualize what the
transferred toe might look like. Occasionally, con-
genital anomalies of the lower extremities may
coexist with upper extremity anomalies and
N.F. Jones
level of the metacarpal bases; classied as an R5 hand
(Jones and Kaplan 2012)
especially with bilateral congenital hand differ-
ences; the availability of donor toes may be lim-
ited because of associated foot anomalies.
Plain radiographs of both hands and both
feet should be obtained to determine the pres-
ence of metacarpals and whether a carpo-
metacarpal joint of the thumb is present or
absent. In children where the carpal or metacar-
pal bones have not yet ossied, an MRI scan may
be considered.
The necessity for preoperative angiography of
either the upper or lower extremity remains
22 Toe-to-Hand Transfers in Children
Fig. 23 (ac) The child underwent staged second toe
transfers, rstly into the thumb position (a) and 6 months
later into the small nger position (b). Six weeks postop-
eratively (c), demonstrated by the presence of remaining
controversial. Greenberg and May (1988) found
a preoperative lateral angiogram of the foot to be
helpful in identifying the location and size of the
rst dorsal metatarsal (FDMA) and rst plantar
metatarsal arteries (FPMA). The author no lon-
ger obtains angiograms but listens to the dorsalis
pedis and rst dorsal metatarsal artery in both
feet with a handheld Doppler probe. The dorsalis
pedis artery can be traced distally into the rst
dorsal metatarsal artery lying between the great
toe and second toe metatarsals using an 8 MHz
pencil Doppler ultrasound probe. The dominance
of the FDMA can be assessed from the presence
or absence of a Doppler signal and the potential
depth of the FDMA from the intensity of the
signal (supercial, within, or deep to the intero-
sseous muscles).
A nal preoperative evaluation should always
be performed by the childs pediatrician a few
days prior to any toe-to-hand transfer to exclude
any viral infection, which might compromise
postoperative respiratory function after a multi-
hour operation.
499
skin sutures and before the child has regained any sensa-
tion in the second toe transfer, he is already able to pinch
between the two toe transfers just by visualizing his hand
Anatomy of the Great Toe
and Second Toe
The arterial anatomy of both the great toe and the
second toe is based on either the rst dorsal
metatarsal artery (FDMA), a continuation of the
dorsalis pedis artery, or the rst plantar metatar-
sal artery (FPMA) (Villen and Julve 2002).
Supercial veins drain to the medial side of the
dorsum of the foot and then into the greater
saphenous vein. Sensibility to the plantar surface
of the great toe or second toe is provided by the
tibial (medial) and bular (lateral) digital nerves
and to the dorsal surface by the deep peroneal
nerve.
Surgical Technique
The child is positioned supine with the arm and
hand on a hand table and the donor foot at the end
of the operating table. General anesthesia is
500 N.F. Jones

thigh, since a skin graft may need to be harvested

later from the thigh or groin.

Toe-to-hand transfer
Preoperative planning for all procedures
OR table: any table for supine position
Positioning/positioning aids: supine with the arm and
hand on a hand table and the donor foot at the end of
the operating table
Equipment: basic and microsurgical instruments,
microscope, tourniquets

Toe-to-hand transfer
Surgical steps donor foot
Fig. 24 (a, b) Six years postoperatively, the child demon-
strates excellent ability to pick up small objects by side-to-
side pinch between the two second toe transfers and strong
grasp to lift up a heavy bottle
occasionally supplemented with an axillary block
or continuous brachial plexus infusion. A Foley
catheter is used to monitor urine output and uid
status, but central venous catheters are not used
routinely. A regular tourniquet is applied to the
upper arm, and a sterile tourniquet is applied to the
8 MHz Doppler ultrasound probe is used to mark the
location of the dorsalis pedis artery and/or the posterior
tibial artery
Location of suitable dorsal veins may be marked by
inating the thigh tourniquet to 100 mmHg after the
draping
Leg elevated but not exsanguinated and the tourniquet
elevated to 300 mmHg
Racquet-shaped incision is used to harvest the great
toe, or a V-shaped incision is used to harvest the
second toe
Dorsal incision is extended to the level of the extensor
retinaculum
Draining veins are dissected in a distal-to-proximal
direction to a dominant dorsal vein
The extensor hallucis longus (EHL) tendon to the great
toe or the extensor digitorum communis tendon (EDC)
to the second toe is dissected in a distal-to-proximal
direction and transected at the level of the extensor
retinaculum
Deep peroneal nerve is identied proximally by
dividing the extensor hallucis brevis tendon and traced
in a proximal-to-distal direction isolating the fascicles
supplying either the great toe or the second toe, which
are divided from the main deep peroneal nerve
Find the rst dorsal metatarsal artery (FDMA) distally in
the web space between the great toe and second toe. The
FDMA may lie in a supercial dorsal, intramuscular, or
plantar position in relation to the interosseous muscles
A communicating branch between the FDMA and the
rst plantar metatarsal artery (FPMA) may be visualized
passing over and distal to the intermetatarsal ligament.
The ligament is divided, and the FPMA is dissected
further proximally through the plantar incision
If the second toe is being transferred, the bular digital
artery to the great toe is ligated and divided. If the great
toe is being transferred, the tibial digital artery to the
second toe is ligated and divided
Select the dorsal or plantar arterial system depending
upon which is dominant
(continued)
22 Toe-to-Hand Transfers in Children
Toe-to-hand transfer
The exor hallucis longus tendon to the great toe or the
exor digitorum longus and exor digitorum brevis
tendons to the second toe are identied through the
plantar incision and are divided as far proximally as
possible
The tibial and bular plantar digital nerves are traced
proximally to their bifurcation from the common digital
nerves. The common digital nerves to the great
toe-second toe web space and the second-third toe web
space are split more proximally using micro-forceps and
micro-scissors before transecting the tibial and bular
digital nerves to either the great toe or second toe
Harvest of a second toe transfer is completed by
osteotomy of the second toe metatarsal at the level of the
metaphyseal are. The great toe is usually harvested at
the level of the base of the proximal phalanx
Tourniquet is then deated and the vascular pedicles
irrigated with 1:20 papaverine solution to relieve any
vasospasm
The foot is wrapped in a laparotomy pad bathed with
warm irrigating solution awaiting hand preparation
Toe-to-hand transfer
Surgical steps hand
The hand and arm are exsanguinated with an Esmarch
bandage and the tourniquet inated
The dorsal aspect of the hand is explored rst, either
through a transverse incision at the level of the wrist or
through a longitudinal incision extending proximally
from the proposed metacarpal stump
The extensor tendons in a congenital hand are almost
universally present, and a recipient extensor tendon is
selected that has satisfactory gliding and excursion
A large dorsal vein is identied and tagged with vessel
loops
Identify the supercial radial nerve or later coaptation
to the deep peroneal nerve or as a nerve transfer to the
plantar digital nerves of the toe transfer
For a toe-to-thumb transfer, the dorsal branch of the
radial artery passing through the anatomical snuff box
is dissected and tagged with vessel loops
Explore the palmar aspect of the hand via a Bruner
zigzag incision
Either the median or ulnar nerve is identied at the
level of the wrist and dissected in a proximal-to-distal
direction to identify common digital nerves or proper
digital nerves for later coaptation to the plantar digital
nerves of the toe transfer
Flexor tendons are dissected either proximal or distal
to the transverse carpal ligament. One exor tendon
with satisfactory gliding and excursion is selected
For a toe-to-nger transfer, the ulnar artery is dissected
through Guyons canal and tagged with vessel loops
(continued)
501
Toe-to-hand transfer
Periosteum over the stump of the selected metacarpal
is elevated, and dorsal-palmar and radial-ulnar drill
holes are made with a 0.035 in. K-wire for subsequent
90-90 interosseous wiring
Toe-to-hand transfer
Surgical steps transfer of the toe to the hand
The relevant structures in the toe transfer are
reidentied the dorsal vein, extensor tendon, deep
peroneal nerve, exor tendons, tibial and bular digital
nerves, and either the dorsalis pedis artery FDMA
system or the FPMA
The position and length of the toe transfer are then
determined by placing the base of the metatarsal (of a
second toe) or the proximal phalanx (of a great toe) at
the proposed osteosynthesis site
Excess length of the metatarsal of a second toe transfer
is removed with an oscillating saw
Dorsal-palmar and radial-ulnar drill holes are made in
the metatarsal or proximal phalanx of the toe transfer
using a 0.035 in. K-wire
Osteosynthesis between the toe metatarsal or proximal
phalanx and the recipient metacarpal or proximal
phalanx is accomplished by 90-90 interosseous wiring
using 26 gauge stainless steel dental wire
The extensor hallucis longus tendon of a great toe
transfer or the extensor digitorum communis tendon of
a second toe transfer is repaired to the selected extensor
tendon in the hand
The toe transfer is positioned in full extension at the
MCP and interphalangeal joints and the selected
recipient tendon at its resting tension
The extensor tendon repair is performed
The exor hallucis longus of a great toe transfer or the
exor digitorum longus tendon of a second toe
transfer, is repaired to the selected recipient exor
tendon in the hand or at the wrist level
Neurorrhaphies of the tibial and bular digital nerves
of the toe transfer to recipient common or proper
digital nerves in the hand are performed
The tourniquet is then deated and the arterial and
venous anastomoses performed
Two surgical teams are utilized to dissect the
hand and foot simultaneously, but dissection
should usually start with the hand in congenital
cases to conrm that recipient tendons and nerves
are available. If only one team is available, the
dissection usually commences with the hand to
conrm that the specic tendons and digital
nerves are available for subsequent tendon and
nerve repairs.
502
Dissection of the Donor Foot
Preoperatively, the 8 MHz Doppler ultrasound
probe is used to mark the location of the dorsalis
pedis artery and/or the posterior tibial artery,
depending on the local anatomy and the needs of
the recipient hand. The location of suitable dorsal
veins may be marked by inating the thigh tour-
niquet to 100 mmHg after the draping but prior to
the start of the surgery. The leg is then elevated but
not exsanguinated in order to leave some blood
within the venous system and the tourniquet ele-
vated to 300 mmHg. A racquet-shaped incision is
used to harvest the great toe (Figs. 8b and 15a). A
V-shaped incision is used to harvest the second toe
(Fig. 12a). The midpoint of the web space
between the great toe and the second toe and the
midpoint of the web space between the second
and third toes are marked, and dorsal and plantar
triangular aps are designed from these two points
proximally to their apex at the level of the
metatarsophalangeal joint. The dorsal incision is
extended proximally in either a longitudinal fash-
ion or a lazy S-shape to the level of the extensor
retinaculum. Draining veins from the apex of the
dorsal ap are dissected in a distal-to-proximal
direction to a dominant dorsal vein, which is dis-
sected further proximally to its origin from the
greater saphenous vein at the level of the extensor
retinaculum.
Either the extensor hallucis longus (EHL) ten-
don to the great toe or the extensor digitorum
communis tendon (EDC) to the second toe is
dissected in a distal-to-proximal direction and
transected at the level of the extensor retinaculum.
The deep peroneal nerve is identied proxi-
mally by dividing the extensor hallucis brevis
tendon and traced in a proximal-to-distal direction
to the proximal margin of the V-shaped dorsal
ap. The fascicles supplying either the great toe
or the second toe are divided from the main trunk
of the deep peroneal nerve.
In the original technique of harvesting a toe
transfer, the dorsalis pedis artery was identied
proximally and dissected in a proximal-to-distal
direction into the rst dorsal metatarsal artery
(FDMA). However, it is faster to identify the
FDMA distally in the web space between the
N.F. Jones
great toe and second toe (Wei et al. 1995). The
FDMA may lie in a supercial dorsal, intramus-
cular, or plantar position in relation to the
interosseous muscles. A communicating branch
between the FDMA and the rst plantar metatar-
sal artery (FPMA) may be visualized passing over
and distal to the intermetatarsal ligament. The
ligament is divided, and the FPMA may be dis-
sected further proximally through the plantar inci-
sion. If the second toe is being transferred, the
bular digital artery to the great toe is ligated
and divided. If the great toe is being transferred,
the tibial digital artery to the second toe is ligated
and divided. The surgeon can then decide whether
the dorsal or plantar arterial system is dominant. If
the FDMA is considered to be large enough and in
a supercial position, the FDMA is dissected in a
distal-to-proximal direction to its origin from the
dorsalis pedis artery, which is itself dissected fur-
ther proximally to the level of the extensor reti-
naculum by dividing the extensor hallucis brevis
tendon (Fig. 2a). If the FDMA is very small in
caliber or lies deeply intramuscularly, the FPMA
is chosen as the dominant arterial system but has
to be extended with an interposition vein graft,
anastomosed on a back table (Brown et al. 2006).
If both the FDMA and FPMA are absent or hypo-
plastic, the great toe may still be transferred using
the medial plantar arterial system (Koman et al.
1985). In children with congenital cleft feet, the
great toe is usually transferred on the posterior
tibial artery pedicle which can be traced distally
to the medial plantar arterial system.
The exor hallucis longus tendon to the great
toe or the exor digitorum longus and exor
digitorum brevis tendons to the second toe are
identied through the plantar incision and are
divided as far proximally as possible. The exor
tendons may be difcult to harvest without creat-
ing a long incision on the plantar surface of the
foot. In order to gain more length, the exor
tendons may be divided through the dorsal inci-
sion after the metatarsal osteotomy.
The tibial and bular plantar digital nerves are
traced proximally to their bifurcation from the
common digital nerves. The common digital
nerves to the great toe-second toe web space and
the second-third toe web space are split more
22 Toe-to-Hand Transfers in Children
proximally using micro-forceps and micro-
scissors before transecting the tibial and bular
digital nerves to either the great toe or second toe.
Each nerve is tagged with a small micro-clip to
facilitate later identication.
Harvest of a second toe transfer is completed
by osteotomy of the second toe metatarsal at the
level of the metaphyseal are with a short, thin,
narrow oscillating saw blade. The great toe is
usually harvested at the level of the base of the
proximal phalanx, as a more proximal osteotomy
through the metatarsal head may interfere with
gait. The tourniquet is then deated and the vas-
cular pedicles irrigated with 1:20 papaverine solu-
tion to relieve any vasospasm, and the foot is
wrapped in a laparotomy pad bathed with warm
irrigating solution.
Dissection of the Recipient Hand
This may be performed simultaneously with the
foot dissection if two teams are available or pri-
marily before the foot is dissected, especially in
congenital cases to ensure that there are adequate
nerves and tendons present in the hand. Even
though the external morphological appearance
and a plain radiograph of the hand are reliable
indications for consideration of a toe-to-hand
transfer, they do not imply availability of internal
recipient structures. In general, the anatomic
structures (tendons and nerves) are more devel-
oped in children with congenital constriction ring
syndrome compared to those children with
symbrachydactyly, hypoplasia, and transverse
deciency. Several authors (Gilbert 1982, 1985;
Kay and Wiberg 1996, Kay and McGuiness 1999;
Foucher et al. 2001; Jones and Kaplan 2013) have
observed that tendons and nerves are more likely
to be normal proximal to a constriction ring com-
pared with symbrachydactyly. However, with
increasing experience of the surgeon, the lack of
suitable recipient tendons and nerves, which may
be encountered in symbrachydactyly, can be
circumvented by using tendon grafts or tendon
transfers and nerve grafts or nerve transfers.
The hand and arm are exsanguinated with an
Esmarch bandage and the tourniquet inated to
503
280 mmHg. The dorsal aspect of the hand is
explored rst, either through a transverse incision
at the level of the wrist or through a longitudinal
incision extending proximally from the proposed
metacarpal stump. The extensor tendons in a con-
genital hand are almost universally present, and a
recipient extensor tendon is selected which has
satisfactory gliding and excursion. A large dorsal
vein is identied and tagged with vessel loops. A
branch of the supercial radial nerve is also iden-
tied for later coaptation to the deep peroneal
nerve or as a nerve transfer to the plantar digital
nerves of the toe transfer. For a toe-to-thumb
transfer, the dorsal branch of the radial artery
passing through the anatomical snuff box is
dissected and tagged with vessel loops.
The palmar aspect of the hand is explored
through a Bruner zigzag incision. Either the
median or ulnar nerve is identied at the level of
the wrist and dissected in a proximal-to-distal
direction to identify common digital nerves or
proper digital nerves for later coaptation to the
plantar digital nerves of the toe transfer (Fig. 3a).
The exor tendons are dissected either proximal
or distal to the transverse carpal ligament, but a
portion of the transverse carpal ligament should
always be left intact to prevent bowstringing. One
exor tendon should be selected which has satis-
factory gliding and excursion (Fig. 3a). For a toe-
to-nger transfer, the ulnar artery is dissected
through Guyons canal and tagged with vessel
loops. The periosteum over the stump of the
selected metacarpal is elevated, and dorsal-palmar
and radial-ulnar drill holes are made with a
0.035 in. K-wire for subsequent 90-90
interosseous wiring. The incisions in the hand
are temporarily closed with staples to prevent
excessive swelling and the tourniquet deated.
Closure of the Foot
Prior to transfer to the hand, the toe transfer
should be pink with normal capillary rell and
should have an excellent Doppler signal. The toe
transfer should be allowed to reperfuse for at least
20 min before reinating the tourniquet on the leg.
The arterial and venous pedicles to the toe are
504
ligated with 4-0 silk ties and reinforced with small
micro-clips. The toe transfer is wrapped in a moist
laparotomy pad and handed to the surgical team
operating on the hand.
The thigh tourniquet is deated and hemostasis
achieved in the donor foot. If necessary, a small
Jackson-Pratt drain is placed. After harvesting the
second toe, the gap between the great toe and the
third toe is closed by repairing the intermetatarsal
ligament using 2-0 or 3-0 Ethibond sutures. The
skin is loosely closed with interrupted 4-0 or 5-0
chromic catgut sutures (Fig. 6b). After harvesting
the great toe, the dorsal and plantar skin can
usually be approximated or the remaining defect
covered with a skin graft. After harvesting the
Morrison wraparound or trimmed toe varia- sor tendon repair is performed with multiple 4-0
tions of a great toe transfer, the proximal-based
ap on the medial aspect of the foot is used to
cover any remnant of the proximal phalanx of the
great toe, and any remaining soft tissue defect is
covered with a skin graft.
Transfer of the Toe to the Hand
Before reinating the arm tourniquet, the relevant
structures in the toe transfer are reidentied the
dorsal vein, extensor tendon, deep peroneal nerve,
exor tendons, tibial and bular digital nerves,
and either the dorsalis pedis artery FDMA sys-
tem or the FPMA (Figs. 8c, 12b, 15b, 18a). The
position and length of the toe transfer are then
determined by placing the base of the metatarsal
(of a second toe) or the proximal phalanx (of a
great toe) at the proposed site of osteosynthesis
with the selected metacarpal, checking the length
and relationship of the toe transfer to the
remaining digits in the hand or to the proposed
location of a future additional toe transfer. Excess
length of the metatarsal of a second toe transfer is
removed with an oscillating saw. Dorsal-palmar
and radial-ulnar drill holes are made in the meta-
tarsal or proximal phalanx of the toe transfer using
a 0.035 in. K-wire, and osteosynthesis between
the toe metatarsal or proximal phalanx and the
recipient metacarpal or proximal phalanx is
accomplished by 90-90 interosseous wiring using
a 26 gauge stainless steel dental wire (Fig. 18d).
N.F. Jones
Alternatively, osteosynthesis can be accom-
plished using crossed K-wires or a longitudinal
K-wire or even a small plate and screws. Any
propensity for hyperextension at the metatarso-
phalangeal joint of the toe transfer is prevented
by suturing the volar plate more proximally to soft
tissues or to the metatarsal itself to limit extension
to a neutral 0 .
The extensor hallucis longus tendon of a great
toe transfer or the extensor digitorum communis
tendon of a second toe transfer is repaired to the
selected extensor tendon in the hand. The toe
transfer is positioned in full extension at the
MCP and interphalangeal joints and the selected
recipient tendon at its resting tension. The exten-
clear nylon mattress sutures and a running locked
suture to allow early motion (Fig. 3b). If a toe-to-
thumb reconstruction is being performed, the
extensor indicis proprius (EIP) tendon to the
index nger may need to be transferred as a
donor tendon transfer. When reconstructing a n-
ger, a quadriga effect, due to tethering of adja-
cent extensor tendons with a common muscle
belly to the absent digits, must be prevented by
releasing adjacent extensor tendons.
Usually only one exor tendon, the exor
hallucis longus of a great toe transfer or the exor
digitorum longus tendon of a second toe transfer,
is repaired to the selected recipient exor tendon
in the hand or at the wrist level. The exor tendon
repair is accomplished using several 4-0 nylon
mattress sutures supplemented with a running
locked suture (Fig. 3c). To prevent any quadriga
effect, adjacent exor tendons that may tether the
newly reconstructed exor tendon to the toe trans-
fer are divided. Occasionally, an interposition
exor tendon graft may be necessary to make up
for any discrepancy in length between the exor
tendon of the toe transfer and the selected exor
tendon at the wrist. If a exor tendon with ade-
quate excursion cannot be identied, a primary
tendon transfer may be required.
Neurorrhaphies of the tibial and bular digital
nerves of the toe transfer to recipient common or
proper digital nerves in the hand are performed
using an end-to-end epineurial technique with
10-0 nylon microsutures under the operating
22 Toe-to-Hand Transfers in Children
microscope. Digital nerve neurorrhaphies can
occasionally be facilitated using brin glue.
Neurorrhaphy of a branch of the deep peroneal
nerve of the toe transfer to a branch of the super-
cial radial nerve in the hand is similarly performed.
If there is a gap between the digital nerves of the toe
transfer and recipient nerves in the hand, interposi-
tion nerve grafts may be necessary, using either the
supercial or deep peroneal nerves harvested as a
nerve graft from the donor foot. If recipient digital
nerves are unavailable in a toe-to-thumb transfer,
end-to-side neurorrhaphies may be required, or a
nerve transfer can be performed using a branch of
the supercial radial nerve coapted to the ulnar
digital nerve of the toe transfer.
Osteosynthesis, extensor and exor tendon
repairs, and neurorrhaphies of the digital nerves
and deep peroneal nerve should hopefully be
performed within a 2-h period of tourniquet ina-
tion. The tourniquet is then deated for the arterial
and venous anastomoses. The venous anastomo-
sis is usually performed rst before the arterial
anastomosis. The draining vein of the toe transfer
is anastomosed end-to-end to the selected vein on
the dorsal aspect of the wrist under the operating
microscope using standard microsurgical tech-
niques using 10-0 nylon microsutures. The recip-
ient vein is then occluded just proximal to the
anastomosis with a small microvascular clamp.
The site of the arterial anastomosis depends on
whether the dorsal or plantar arterial system of the
toe transfer has been harvested and the location of
the recipient radial or ulnar artery. If a long
dorsalis pedis FDMA arterial pedicle has
been harvested, end-to-end or end-to-side anasto-
mosis to the ulnar artery at the wrist or the dorsal
branch of the radial artery in the anatomical snuff
box can be performed. If the short FPMA ped-
icle has been harvested, it can occasionally be
anastomosed to the supercial palmar arch or to
a common digital artery, but it is usually better to
elongate the FPMA with an interposition vein
graft anastomosed on a back table prior to
osteosynthesis (Brown et al. 2006). The vein
graft can then be anastomosed end-to-end to the
dorsal branch of the radial artery in the anatomical
snuff box or the ulnar artery at the wrist. After
completion of the arterial anastomosis, the double
505
approximator clamp is removed, but the single
microvascular clamp on the proximal side of the
venous anastomosis is maintained to create a high-
pressure column of blood owing through the toe
transfer to expand any collapsed vessels due to
vasospasm. The anastomoses are irrigated with
1:20 papaverine solution. The toe transfer should
rapidly turn pink and have excellent capillary rell.
After removal of the microvascular clamp from the
proximal side of the venous anastomosis, the
patency of the arterial and venous anastomoses
can be conrmed by the Acland milking test.
The incisions are loosely closed with inter-
rupted 4-0 or 5-0 chromic catgut sutures. Split-
thickness or full-thickness skin grafts are liberally
used to prevent any tension on the underlying
anastomoses. A sterile pulse oximeter probe is
applied to the nail plate of the toe transfer before
the dressings are applied for continuous postoper-
ative monitoring of the patency of the arterial and
venous anastomoses (Jones and Gupta 2001). The
pulse rate of the toe transfer should correspond
exactly to the pulse oximeter on the opposite hand
and corresponds to the patency of the arterial
anastomosis. Any decrease in the pulse rate com-
pared to the systemic heart rate may indicate that
the arterial anastomosis is compromised. The oxy-
gen saturation (SaO2) corresponds to the patency
of the venous anastomosis, and any decrease in
the oxygen saturation for an extended period of
time when compared to the systemic oxygen satu-
ration may indicate thrombosis of the venous anas-
tomosis. The incisions are covered with antibiotic
ointment and nonadherent gauze dressings and the
hand, wrist, forearm, and elbow immobilized in a
loose plaster of Paris sugar-tong splint. The donor
foot is also immobilized in a posterior plaster of
Paris splint with the foot in neutral position. Both
the hand and foot are elevated and the hand kept
warm with a heating blanket. The child is moni-
tored in a pediatric intensive care unit for up to
3 days postoperatively. The child is kept sedated
using opiates and benzodiazepines as necessary,
and a rst-generation cephalosporin is adminis-
tered intravenously for 57 days postoperatively.
Anticoagulation with dextran 40 (0.36 ml/kg/h) is
continued for 5 days, and low-dose aspirin 81 mg is
started on the rst postoperative day and
506
continued for 4 weeks. The dextran infusion is
reduced to half on postoperative day 6 and
discontinued on day 7. If the clinical appearance
of the toe transfer or the differential pulse oximetry
monitoring indicates compromised perfusion to the
toe transfer, the child should be returned to the
operating room immediately for exploration and
possible revision of the microsurgical anastomoses.
The splints on the hand and foot are removed
1014 days postoperatively, and the take of
any skin graft is assessed. The child can then
begin to ambulate with a bulky sock in a slipper
or sneaker. The hand is protected with a remov-
able thermoplast splint for 2 more weeks when
passive and active range of motion exercises of
the toe transfer are begun.
These children may require secondary teno-
lyses, tendon transfers, bone grafting, arthrodeses,
osteotomies, or scar revisions to reach their optimal
functional and aesthetic outcome.
Toe-to-hand transfer
Postoperative protocol
A sterile pulse oximeter probe is applied to the nail
plate of the toe transfer before the dressings are applied
for continuous postoperative monitoring of the
patency of the arterial and venous anastomoses
Monitor the pulse rate of the toe transfer as it should
correspond exactly to the pulse oximeter on the
opposite hand and corresponds to the patency of the
arterial anastomosis
The hand, wrist, forearm, and elbow are immobilized
in a loose plaster of Paris sugar-tong splint. The donor
foot is also immobilized in a posterior plaster of Paris
splint with the foot in neutral position
Both the hand and foot are elevated and the hand kept
warm with a heating blanket
The child is monitored in a pediatric intensive care unit
for up to 3 days postoperatively
Child is kept sedated using opiates and
benzodiazepines as necessary, and a rst-generation
cephalosporin is administered intravenously for 57
days postoperatively
Anticoagulation with dextran 40 (0.36 ml/kg/h) is
continued for 5 days, and low-dose aspirin 81 mg is
started on the rst postoperative day and continued for
4 weeks
If the clinical appearance of the toe transfer or the
differential pulse oximetry monitoring indicates
compromised perfusion to the toe transfer, the child
should be returned to the operating room for exploration
and possible revision of the microsurgical anastomoses
(continued)
N.F. Jones
Toe-to-hand transfer
Splints on the hand and foot are removed 1014 days
postoperatively. Child can then begin to ambulate with
a bulky sock in a slipper or sneaker
Hand is protected with a removable thermoplast splint
for 2 more weeks when passive and active range of
motion exercises of the toe transfer are initiated
Outcomes of Toe Transfers
for Posttraumatic Reconstruction
in Children
Yoshimura (1980, 1984) performed 13 toe trans-
fers in 12 children, 10 of whom had sustained
traumatic amputations. Shevdovchenko (1993)
reported 103 toe transfers to reconstruct 23 thumbs
and 80 ngers in 66 children aged between 3 and
14 years. Seventeen of these children received toe
transfers for reconstruction of amputated digits.
Wei (Wei et al. 1997a) performed 42 complete
toe transfers in 25 children at an average age of
12 years and a range between 3 and 16 years, for
reconstruction of 11 thumb and 31 nger amputa-
tions over a 4-year period. Preoperative angiogra-
phy of the hands and feet was not performed.
Three children required reexploration for arterial
thrombosis, and one second toe transfer failed
completely. Static two-point discrimination aver-
aged 5 mm. Range of motion averaged 69 at the
MCP joint, 38 at the PIP joint, and 13 at the DIP
joint of ngers reconstructed by second toe trans-
fers and 15 at the IP joint of thumbs
reconstructed by great toe transfers. Radiologi-
cally the toe transfers showed the same growth
as nontransferred toes. They observed that toe
transfers for digital amputations in children dif-
fered from similar toe transfers in adults in two
respects. Secondary tenolyses were rarely
required, and sensory recovery was better. In a
follow-up study over a 7-year period, Wei and
Mardini (2003) performed 48 toe transfers
(including trimmed great toes, wraparound great
toes, single and double second toes, and combined
second-third toes) to reconstruct 11 thumbs and
37 ngers in 33 children at an average age of
12 years and a range from 2 to 16 years. There
22 Toe-to-Hand Transfers in Children
were four reexplorations, three venous thrombo-
ses, and one arterial thrombosis, but no failures.
They also reported that the trimmed toe technique
did not adversely affect growth in children (Wei
and Mardini 2003).
Kay (Kay et al. 1996) performed seven toe
transfers for posttraumatic reconstruction of six
children aged between 2 and 10 years three
after burns, two after trauma, and one after menin-
gococcal septicemia. Five thumbs and two ngers
were reconstructed using 6 second toe transfers
and 1 wraparound great toe transfer. There were
no reexplorations and no failures.
Vilkki (1989) described a very unique tech-
nique for reconstruction of pinch and grip in
patients with amputations at the wrist level. A
three-joint second toe transfer is harvested with a
hemi-pulp ap from the great toe on the same
vascular pedicle. The long metatarsal bone of the
second toe transfer is attached to the palmar aspect
of the distal radius about 7 cm proximal to the
wrist level. The radial half of the distal radius is
resected but preserving the distal radial-ulnar joint
to create an ulnar post, and the hemi-pulp ap is
used to provide sensation over this post. Yu
(Yu et al. 1999) performed 33 toe-to-forearm
transfers for handless forearms from the level
of the metacarpal base to the distal third of the
forearm. In their technique, the distal ulna is elon-
gated with a 3 cm long segment of the ipsilateral
radius, and a second toe is transferred onto the
stump of the distal radius. The radial aspect of the
elongated ulna is covered by the plantar skin ap
connected to the second toe transfer. It was their
opinion that that this reconstructive technique
provided better pinch function between the toe
transfer and the elongated ulna and had better
appearance than would have been possible with
a Krukenberg procedure.
Outcomes of Toe Transfers
for Congenital Hand Anomalies
Gilbert (1982, 1985) reported 21 second toe trans-
fers in 17 children with congenital hand differ-
ences between the age of 18 months and 13 years
to reconstruct 4 thumbs and 17 ngers. Twelve of
507
the anomalies were due to congenital constriction
ring syndrome and ve due to congenital aplasia.
Gilbert found that results were very good
for thumb reconstruction, good in the majority of
nger reconstructions, but poor in aplasia of the
ngers and thumbs. Results were better in chil-
dren with congenital constriction ring syndrome
compared with congenital aplasia, because the
structures were more normal proximally. He felt
that the ideal case was a congenital constriction
ring syndrome amputation in which the meta-
carpophalangeal joint remains intact with a base
of the proximal phalanx.
Lister (1988; Eaton and Lister 1991) reported
12 children between the ages of 10 months and
8 years who underwent a second toe transfer
to reconstruct a congenitally decient thumb
due to transverse arrest (6), congenital constric-
tion ring syndrome (3), and symbrachydactyly
(3). Shevdovchenko (1993) reported 103 toe
transfers in 66 children between 3 and 14 years
of age to reconstruct 23 thumbs and 80 ngers.
Forty-nine children had congenital hand differ-
ences classied as ectrodactyly, brachydactyly,
adactyly, and hypoplasia. Vilkki (1995) per-
formed toe transfers in 30 children with congen-
ital hand differences, 18 of which were
performed for adactyly. Fourteen children under-
went toe transfers for aplasia of all four ngers,
whereas four children underwent a toe transfer
for reconstruction of the thumb. Five toe trans-
fers required reexploration, and one toe transfer
failed. He was able to restore pinch in 14 out of
17 hands.
Kay and Wiberg (1996, Kay and McGuiness
1999; Kay et al. 1996) transferred 66 toes in
40 children, aged 9 months to 14 years. Fifty-nine
toes in thirty-four children were transferred for
congenital differences including symbrachydactyly
(17), transverse deciency (8), complex syndactyly
(3), and congenital constriction ring syndrome (3).
The thumb was the most frequently reconstructed
(27) followed by the ring nger (22). Five children
(12.5 %) required reexploration, but all toe trans-
fers survived. Seventy-six percent of parents
were very satised with the results of surgery,
and most parents and children felt that surgery
had positively affected the childrens psychosocial
508
well-being. Like Gilbert, Kay and Wiberg (1996;
Kay and McGuiness 1999) felt that the ideal indi-
cation for a toe transfer was congenital constric-
tion ring syndrome affecting the thumb, because
all the structures are normal proximal to the
amputation. However, the majority of the chil-
dren had symbrachydactyly, transverse arrest, or
even a monodactylous cleft hand. Kay and Wiberg
(1996, Kay and McGuiness 1999) also empha-
sized the benets of simultaneous double second
toe transfers in 13 children (33 %), providing
savings in resources and minimizing trauma to
the child and family, as well as avoiding the dif-
culty of surgical dissection inherent in a second-
stage procedure. He also advocated the advantage
of a single operative team, which is contrary to
most other series.
Van Holder et al. (1999) reconstructed 14
children with congenital hand differences due
to congenital constriction ring syndrome (3),
symbrachydactyly (2), and transverse failure
(9) with sequential staged double second toe
transfers. All of the toes had sensibility and
mobility and provided improved function and
appearance; however, all patients required at
least one secondary procedure. Foucher et al.
(2001) reported 65 toe transfers in 58 children
with congenital differences of the hand. Symbra-
chydactyly was the most common indication
with 45 children undergoing 51 toe transfers. A
toe transfer was used to reconstruct the thumb in
12 cases, the middle nger in 22 cases, and the
ring nger in 18 cases. Foucher stated that toe-to-
hand transfers are indicated for children with
congenital hand differences classied by the
International Federation of Societies for Surgery of
the Hand (Swanson 1976) as being in category I
(failure of development), category V (under-
growth), and category VI (congenital constric-
tion ring syndrome). Foucher further subdivided
the indications into Blauth and Gekeler (Blauth
1971) types III and IV symbrachydactyly
(45 children, 51 toe transfers) and much less
frequent indications including transverse de-
ciency (two children, three toe transfers),
Manske (Manske et al. 1995) type IIIB thumb
hypoplasia (ve toe transfers), ulnar longitudinal
N.F. Jones
deciency with monodactylous hand (one toe
transfer), central deciency with monodactylous
hand (one toe transfer), and macrodactyly (one
toe transfer). Surprisingly, only two children
with congenital constriction ring syndrome
underwent toe transfers. In contrast to Kays
(Kay and Wiberg 1996; Kay and McGuiness
1999; Kay et al. 1996) contention that simulta-
neous double second toe transfers had signicant
benets, Foucher (Foucher et al. 2001) felt that
the disadvantages were surgeon fatigue, increased
operating time, and morbidity. He therefore advo-
cated sequential second toe transfers to allow better
positioning of the second transfer.
Richardson et al. (2004) felt that severe
monodactylous type III and adactylic type IV
symbrachydactyly (Blauth and Gekeler 1971)
are ideal indications for toe transfers and reported
18 toe transfers in 13 children with symbra-
chydactyly. Six toes were transferred into the
thumb position and 11 into the small nger posi-
tion. Richardson (Richardson et al. 2004) unlike
Kay also felt that if two transfers were required
that this should be performed in two separate
stages. Jones et al. (2007) reported 82 toe transfers
in 68 children with congenital differences of the
hand due to symbrachydactyly, congenital con-
striction ring syndrome, and transverse deciencies
and recommended very specic indications for
considering the use of toe transfers to reconstruct
congenital hand differences in a series of 100 toe
transfers in 82 children (Jones and Kaplan 2013).
In a radiographic study, Chang and Jones
(Chang 2002b) documented excellent potential
for digital growth after toe transfers performed in
children between the ages of 2.8 and 13 years. The
epiphyseal plates remained open in 27 of 28 pha-
langes up to 36 months later. In four children
where radiographic comparison was possible, the
amount of linear growth of the toe transfer was
equal to the contralateral nontransferred toe. Kay
et al. (1996) reported that the growth of a toe
transfer varied between 70 % and 104 % of the
length of the corresponding toe.
Chang and Jones (Chang 2002a) reported three
great toe transfers to reconstruct the thumb in two
children with cleft hand and cleft feet, in which
22 Toe-to-Hand Transfers in Children
the great toe was being amputated to allow the
tting of shoes, illustrating the ultimate use of
spare parts. Both Nyarady et al. (1983) and
Harashina et al. (1994) each described reconstruc-
tion of a hypoplastic thumb in a child using a
second toe transfer. Tu et al. (2004) reported
11 children with hypoplastic thumbs associated
with radial clubhand, who underwent a second
toe transfer to reconstruct the absent thumb because
the childs parents refused pollicization of the index
nger. Five children had Blauth stage IV, ve had
Blauth stage V, and one child had Blauth stage IIIB
thumb hypoplasia (Blauth 1967).
Conclusions
Microsurgical toe-to-hand transfers for posttrau-
matic reconstruction in children and for recon-
struction of congenital hand anomalies will
hopefully continue to evolve to become accepted
into the armamentarium of pediatric hand sur-
geons, although it will continue to remain a tech-
nical challenge requiring a high level of
microsurgical skill on the part of the hand surgeon
and the ultimate trust of the childs parents. Pre-
operative counseling of the parents with the
opportunity to meet and speak with other families
and children who have undergone a toe transfer is
absolutely vital. Most importantly, the parents
should fully understand the slight potential for
failure of the procedure with consequent loss of
the toe transfer. While the indications for toe
transfers are now better dened (Jones and Kaplan
2013), the optimal age for toe transfers remains
unknown. Just like other congenital hand surgery,
most experienced surgeons believe toe transfers
between 2 and 3 years of age have the best chance
of cortical integration, although toe transfers have
been successfully reported in older children
between the ages of 11 and 17 years (Spokevicius
and Radzevicius 1997).
It will always be very difcult to prove conclu-
sively that microsurgical reconstruction of con-
genital hand anomalies is superior to either no
surgical intervention or conventional reconstruc-
tion, but a child who is able to write their name for
509
the rst time, or who can pick up a cup single-
handedly and hold a utensil, or who can now ride a
bicycle provides the hand surgeon with far greater
conrmation than the most sophisticated outcome
instruments. In the future, parental assessment of
their childrens functional outcomes and even the
childs own assessment will be important valida-
tion of these techniques.
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22 Toe-to-Hand Transfers in Children
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Part VII
Nerve
 Nerve Anatomy and Diagnostic
Evaluation 23
Jeffrey A. Stromberg and Jonathan Isaacs

Contents Abstract
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 515 Nerve injuries in the pediatric upper extremity
offer unique diagnostic challenges. Young
Nerve Anatomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 516
patients may not understand the interview
Brief Review of Microanatomy . . . . . . . . . . . . . . . . . . . . 521 questions or be able to adequately express
Pathophysiology of Nerve Injury . . . . . . . . . . . . . . . . . . 521 themselves. The physical exam is often limited
due to fear, anxiety, or the inability to follow
Diagnostic Evaluation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 522
Physical Examination . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 522 commands. As a result of these obstacles,
Electrodiagnostic Testing . . . . . . . . . . . . . . . . . . . . . . . . . . . . 523 nerve injuries amenable to repair might be
Nerve Visualizing Modalities: Ultrasound . . . . . . . . . . 524 observed for unreasonable amounts of time
Nerve Visualizing Modalities: Magnetic
while the patient is put through repeat and
Resonance Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 526
unnecessary diagnostic evaluations. This chap-
Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 526 ter provides a comprehensive description of
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 526 gross and microscopic nerve anatomy, an over-
view of the pathophysiology of nerve injury,
how to conduct a physical exam, and how to
best utilize electrodiagnostic testing and other
imaging modalities such as ultrasound
and MRI.

Introduction

The location and composition of peripheral nerves

are set in utero, and therefore, their anatomic
J.A. Stromberg (*) relationships, motor innervation targets, and sen-
Department of General Surgery, Virginia Commonwealth sory distribution patterns are similar to those seen
University Health Systems, Richmond, VA, USA in adults. Despite these similarities, nerve injuries
e-mail: jstromberg2@mcvh-vcu.edu
in the pediatric upper extremity exhibit unique
J. Isaacs challenges in ascertaining their diagnosis. A
Department of Orthopaedic Surgery, Division of Hand
proper history may be difcult to obtain due to
Surgery, Virginia Commonwealth University Health
Systems, Richmond, VA, USA communication obstacles as the patient may not
e-mail: jisaacs@mcvh-vcu.edu understand your questions or be able to
# Springer Science+Business Media New York 2015 515
J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_23
516
adequately express themselves. The physical
exam is often limited due to fear, anxiety, or the
inability to follow commands. Therefore, to ade-
quately treat the pediatric patient, one must have a
thorough understanding of the anatomy, a strong
comprehension of the mechanism of injury, an
appropriate algorithm of tests, and a high index
of suspicion.
Nerve Anatomy
Nerves of the upper extremity originate from ven-
tral nerve roots typically from C5 through T1,
which then intermingle to form the brachial
plexus in the posterior cervical triangle (Johnson
et al. 2010). The brachial plexus can be further
broken down into nerve roots, trunks, divisions,
and cords from which numerous important nerves
originate (Fig. 1). The proximal aspect of the
brachial plexus runs between the anterior and
middle scalene muscles and courses towards the
axilla and under the clavicle. The infraclavicular
portion of the plexus, known as the cords, forms
the terminal branches making up the major
peripheral nerves of the upper extremity.
The long thoracic nerve (C5, 6, 7), dorsal scap-
ular nerve (C5), and innervation for the scalene
muscles (C5, 6) originate from the nerve roots in
the posterior cervical triangle. The long thoracic
nerve innervates the serratus anterior, which is
responsible for stabilizing the scapula to allow
anteversion of the arm. The dorsal scapular
nerve pierces the middle scalene and innervates
the rhomboid muscles, which stabilize and adduct
the scapula. The superior trunk gives rise the
subclavius nerve (C5, 6) and the suprascapular
nerve (C5, 6). The suprascapular nerve travels
with the suprascapular artery and vein before
innervating the supraspinatus and infraspinatus
muscles which assist with arm abduction and
external rotation. The subclavius nerve often
travels to the subclavius muscle with the phrenic
nerve (Tubbs et al. 2010).
Emerging from the posterior cord are the upper
subscapular (C5, 6), thoracodorsal (C5, 6, 7),
lower subscapular (C5, 6), axillary (C5, 6), and
radial nerves (C5, 6, 7, 8, T1). The thoracodorsal
J.A. Stromberg and J. Isaacs
nerve follows the subscapular artery before inner-
vating the latissimus dorsi muscle which produces
arm adduction. The axillary nerve passes through
the quadrangular space before dividing into an
anterior and posterior branch with the anterior
branch intimately winding around the surgical
neck of the humerus (Fig. 2). The axillary nerve
is mostly a motor nerve providing innervation to
the deltoid, teres minor, and long head of triceps
brachii muscles and therefore inuences arm
abduction, exion, extension, and rotation
(Tubbs et al. 2010). From the medial cord origi-
nate the medial pectoral nerve (C8, T1), medial
brachial cutaneous nerve (C8, T1), medial
antebrachial cutaneous nerve (C8, T1), a portion
of the median nerve (C8, T1) and ulnar nerve
(C8, T1). The medial pectoral nerve assists with
arm adduction by always innervating the
pectoralis minor and frequently the pectoralis
major muscles. The medial brachial cutaneous
nerve courses along the medial side of the proxi-
mal brachial artery and innervates the medial dis-
tal third of the arm. The medial antebrachial
cutaneous nerve (MAC) also initially runs medial
to the brachial artery but transitions to a supercial
position in the middle arm as it runs with the
basilic vein. In the antecubital fossa the MAC
bifurcates to continue its venous relationship
with the volar branch anterior to the median
basilic vein and the ulnar branch posterior to the
median vein. The MAC innervates the volar distal
third of the arm and the ulnar half of both volar
and dorsal surfaces of the forearm (Tubbs
et al. 2010).
The lateral pectoral nerve (C5, 6, 7) and
musculocutaneous nerve (C5, 6, 7), and the
remaining contribution to the median nerve
(C5, 6, 7) arise from the lateral cord (Jacobson
et al. 2010; Tubbs et al. 2010). The lateral pectoral
nerve innervates the pectoralis major muscle
also contributing to arm adduction. The
musculocutaneous nerve penetrates and inner-
vates the coracobrachialis muscle and then tra-
verses the remainder of the arm radially between
the biceps brachii and brachialis muscles, both of
which receive innervation. After the motor inner-
vation is exhausted, the nerve becomes the lateral
antebrachial cutaneous nerve which runs under
23 Nerve Anatomy and Diagnostic Evaluation
Fig. 1 Peripheral nerves of the upper extremity. A sum-
mary view of the brachial plexus organization and the
paths of the ulnar, radial, and median nerves. The site of
each plane transition, identied as where the nerves pierce
through or traverse above or below a given anatomic struc-
ture, along the respective nerves course is represented
517
(Reprinted from Neurology board review: an illustrated
study guide. M Mowzoon, 2007, Rochester, MN: Mayo
Clinic Scientic Press. Copyright by Taylor and Francis
Group LLC Books. Reprinted with permission. License Id:
3254140720553)
518 J.A. Stromberg and J. Isaacs

Fig. 2 Posterior view of

the arm. The axillary nerve
passes through the
quadrangular space and
then wraps around the
surgical neck of the
humerus with the posterior
humeral circumex artery.
Note the early strong
relationship between the
radial nerve and the brachial
artery as they enter the arm
and travel along the spiral
groove of the humerus. The
medial and lateral heads of
the triceps are resected but
can be seen receiving radial
innervation (Reprinted with
permission from Anatomy
and landmarks for branches
of the brachial plexus: a
vade mecum, by
S.R. Tubbs et al. 2010,
Surgical and Radiologic
Anatomy, 32(3), p. 265.
Copyright 2010 by
Springer-Verlag France.
Artist David License Id:
3244201311409)

the bicipital aponeurosis before bifurcating and
innervates the radial half of the volar forearm.
The anterior branch of the lateral antebrachial
cutaneous nerve terminates at the base of the
thumb after traversing anteriorly over the radial
artery at the wrist (Tubbs et al. 2010).
The ulnar nerve proceeds down the arm paral-
lel and posterior to the brachial artery close to the
humerus until the distal third of the arm where it
then turns dorsally away from the artery. The
nerve penetrates the medial intermuscular septum
and lies against the medial head of the triceps
muscle (Jacobson et al. 2010). The ulnar nerve
will then pass under the arcade of Struthers, a
brous band originating from the medial head of
the triceps and inserting onto the medial
intermuscular septum. The arcade of Struthers is
present in approximately 70 % of the population
but more likely to be altogether absent in younger
pediatric patients (Feinberg et al. 1997). At the
elbow the ulnar nerve lies posterior to the medial
epicondyle of the humerus and continues under
the cubital tunnel retinaculum, a short, thin brous
band traversing between the medial epicondyle
and the olecranon (Fig. 1). The cubital tunnel is
immediately distal to the retinaculum with the
23 Nerve Anatomy and Diagnostic Evaluation 519

oor formed by the ulna and the roof by Osbornes

ligament which bridges the two heads of the exor
carpi ulnaris (FCU) (Jacobson et al. 2010). The
anconeus epitrochlearis, an established variant
present in up to one third of the population, is an
accessory muscle proximal to the cubital tunnel
that inserts between the medial epicondyle and the
olecranon and overlies the ulnar nerve parallel to
Osbornes fascia (Jacobson et al. 2010). In addi-
tion to the cubital tunnel itself, the anconeus
epitrochlearis may be a source of ulnar nerve
compression (Stutz et al. 2012). After exiting the
cubital tunnel, the ulnar nerve lies between the
FCU and exor digitorum profundus (FDP) mus-
cle bellies (Figs. 1 and 3). It is here that the nerve
innervates the FCU and the ulnar muscle bellies of
the FDP controlling the ring and little ngers
(Jacobson et al. 2010). At or just proximal to the
level of the wrist, two sensory nerves originate:
the ulnar palmar cutaneous nerve providing sen-
sation for the hypothenar eminence and the dorsal
cutaneous nerve which provides sensation to the
ulnar dorsal surface of the hand, the dorsal little
nger, and a variable portion of the dorsal ring
nger (Engber and Gmeiner 1980; Tagliaco
et al. 2012).
Distally the ulnar nerve joins the ulnar artery
and enters Guyons canal, a tunnel formed by the
pisiform bone, exor retinaculum, and the palmar
Fig. 3 Anterior view of the forearm. In the distal arm, the
carpal ligament. Variably within the canal or median nerve is ulnar to the brachial artery, and after
shortly after exiting, the nerve either bifurcates traversing the elbow, the anterior interosseous nerve is
or trifurcates into primarily sensory or motor closely associated with the respective artery. In the
mid-forearm the ulnar nerve becomes closely associated
nerves. The deeper motor branch passes under
with the ulnar artery as it travels ulnarly between the exor
the exor digiti minimi brevis and abductor digiti digitorum profundus and the exor carpi ulnaris (cut)
minimi to supply the hypothenar muscles, the (Reprinted with permission from Anatomy and landmarks
deep head of exor pollicis brevis, the adductor for branches of the brachial plexus: a vade mecum, by
S.R. Tubbs et al. 2010, Surgical and Radiologic Anatomy,
pollicis, the dorsal and palmar interossel, and the
32(3), p. 265. Copyright 2010 by Springer-Verlag France.
third and fourth lumbrical muscles (Jacobson Artist David Fisher. License Id: 3244201311409)
et al. 2010; Tagliaco et al. 2012; Tubbs
et al. 2010). The supercial sensory portion inner- innervates the medial and lateral heads of the
vates the palmar surface of the distal ulnar palm triceps brachii muscle and the anconeus muscle,
and form the proper digital nerves to the little in addition to emitting several sensory nerves to
nger and ulnar half of the ring nger (Fig. 1) provide sensation to the posterior arm and fore-
(Tagliaco et al. 2012). arm: the posterior cutaneous nerve, the lower lat-
In the arm the radial nerve travels diagonally in eral cutaneous nerve, and the posterior cutaneous
an ulnar to radial direction along the posterior nerve of the forearm (Figs. 1 and 2). In the distal
aspect of the humerus in the spiral groove arm, the radial nerve penetrates the lateral
(Jacobson et al. 2010). Proximally the nerve intermuscular septum and courses under the
520
brachioradialis muscle (Jacobson et al. 2010;
Tubbs et al. 2010). Here the brachioradialis and
extensor carpi radialis brevis and longus muscles
receive their innervation (Tagliaco et al. 2012).
After passing the elbow joint level at the lateral
epicondyle, the radial nerve enters the anterior
compartment of the forearm between the
brachialis, brachioradialis, and extensor carpi
radialis longus muscles before bifurcating into
the supercial radial nerve and the deeper poste-
rior interosseous nerve (PIN).
The PIN travels posteriorly through the arcade
of Frohse between the two heads of the supinator
and then courses in an intramuscular path close to
the radius (Jacobson et al. 2010) (Fig. 1). The PIN
is responsible for providing the innervation to the
entire extensor compartment including the
brachioradialis, supinator, extensor digitorum
communis, extensor carpi ulnaris, extensor digiti
minimi, abductor pollicis longus, abductor
pollicis brevis, extensor pollicis longus (EPL),
and extensor indicis muscles. While the PIN
does not provide any cutaneous innervation, it
does provide sensory feedback from the dorsal
wrist capsule, through the terminal sensory
branch, which travels posterior to the posterior
interosseous membrane and anterior to the EPL
(Smith et al. 2011; Tubbs et al. 2010).
The supercial branch of the radial nerve
briey follows the radial artery before coursing
laterally in proximity to the cephalic vein and then
through the anatomic snuffbox. The supercial
branch is entirely sensory and innervates the
radial aspect of the dorsum of the hand, the dor-
sum of thumb, index, long, and radial half of the
ring ngers proximal to the distal interphalangeal
joint (Jacobson et al. 2010; Tagliaco et al. 2012).
The median nerve descends down the arm
adjacent to the brachial artery, generally lateral
to the artery proximally, and then becomes medial
to the artery distally (Fig. 3) (Jacobson
et al. 2010). The median nerve traverses the
elbow joint level adjacent to the origin of the
pronator teres humeral head and then typically
goes under this muscle belly, although a less com-
mon course is intramuscular through the pronator
teres or brachialis muscles (Jacobson et al. 2010).
Along the forearm the median nerve courses
J.A. Stromberg and J. Isaacs
between the FDP and exor digitorum
supercialis (FDS) (Klauser et al. 2010). The
anterior interosseous nerve (AIN) branches off
radially after the median nerve crosses the two
heads of the pronator teres and enters the FDP
muscle belly before continuing on the volar sur-
face of the interosseous membrane in proximity to
the interosseous artery (Chin and Meals 2004).
The AIN innervates the exor pollicis longus,
FDP of the index and middle ngers, and
quadratus muscles. The AIN terminates as sen-
sory innervation for the radiocarpal, midcarpal,
and carpometacarpal joints (Chin and Meals
2004). The median nerve proper will innervate
the pronator teres, exor carpi radialis, FDS, and
palmaris longus. Alternatively, the FDS may be
innervated by the AIN, a reported variant,
manifesting in weakness of all proximal
interphalangeal joints following an isolated AIN
injury (Chin and Meals 2004). Proximal to the
carpal tunnel, the median nerve gives off the
median palmar cutaneous branch which inner-
vates the thenar eminence and the palmar triangle.
At the wrist, the median nerve enters the carpal
tunnel, which is formed by the carpus on the oor
and the transverse carpal ligament as the roof
(Figs. 1 and 3). Within the tunnel the median
nerve lies supercial and radial, surrounded by
the FPL radially and both FDP and FDS tendons
of the index nger dorsally and ulnarly (Klauser
et al. 2010). After exiting the carpal tunnel, the
median nerve bifurcates into a smaller radial
branch and a larger ulnar branch. The radial
branch rst gives off the recurrent motor branch
for the thenar muscles and then trifurcates into
three proper palmar digital nerves, two to the
thumb and one to the radial side of the index
nger. The recurrent motor nerve innervates the
opponens pollicis, abductor pollicis brevis, and
the supercial head of the exor pollicis brevis
muscles. The proper palmar digital nerves provide
innervation to the entire palmar surface of the
thumb and radial aspect of the index nger
with the index nger nerve also innervating the
rst lumbrical. The larger ulnar branch of the
median nerve further bifurcates into two common
palmar digital nerves, with the rst common nerve
innervating the second lumbrical and forming the
23 Nerve Anatomy and Diagnostic Evaluation
proper digital nerves for the ulnar index nger and
radial middle nger. The other common palmar
digital nerve innervates the ulnar middle and
radial ring ngers after dividing into the respec-
tive proper digital nerves. A dorsal branch from
each proper digital nerve communicates with the
digital nerves of the supercial radial branches to
innervate the dorsal distal phalanx of the index,
middle, and radial half of the ring ngers
(Tagliaco et al. 2012).
It is important to remember that the aforemen-
tioned sensory and motor assignments are typical
but not the rule as variations are commonplace.
One of the more common variants is Martin-
Gruber anastomoses, which involve upper fore-
arm communications between the ulnar and
median nerves; present in upwards of 1731 %
of the population. These communications are fre-
quently unilateral and typically only carry motor
bers. Their presence can clearly confound the
expected presentation of a given nerve injury
(Loukas et al. 2011). The specic sensory and
motor anatomic variants described in the arm,
forearm, and hand are beyond the scope of this
chapter, but the surgeon should always suspect
their presence when the clinical picture does not
conform to the standard expectations.
Brief Review of Microanatomy
Peripheral nerve structure and its response to injury
are important to comprehend as this knowledge
will affect the interpretation of diagnostic tests
and inuence treatment. Each individual axon is
covered by a connective tissue matrix called the
endoneurium. The axons are then grouped into a
fascicle which is enclosed by perineurium. The
interfascicular tissue is the internal epineural layer
with the peripheral nerves outermost sheath being
the external epineurium. The perineurium is the
source of a nerves tensile strength, while the
inner epineurial layer provides for some compres-
sive protection. Therefore, nerves with less inner
epineurium are most susceptible to compression
injury. Blood vessels run longitudinally along
both layers of the epineurium and the perineurium
(Feinberg et al. 1997; Sunderland 1990).
521
Peripheral nerves can be either monofascicular
or polyfascicular. As a general principle, distally
nerves become more polyfascicular with each fas-
cicle corresponding to either a sensory or motor
function, particularly near branch points (Kauf-
man et al. 2009; Sunderland 1990). The specic
motor or sensory designations at certain anatomic
locations can also be estimated to assist with diag-
nosis or repair. Within the median nerve prior to
AIN branch formation, the motor bers
corresponding to the AIN are posterior (Chin
and Meals 2004). After the AIN branch point,
the motor fascicles of the median nerve are local-
ized radially. For the ulnar nerve in the
mid-forearm, the motor group is centrally located
between the outer dorsal and volar sensory fasci-
cles (Kaufman et al. 2009).
Pathophysiology of Nerve Injury
The two most commonly used classications of
nerve injury were developed by Seddon and Sun-
derland. Neuropraxia, or Sunderlands rst-
degree injury, describes an injury of a transient
localized conduction block in the absence of
structural damage (Burnett and Zager 2004;
Sunderland 1990). Axonotmesis, Sunderlands
second-degree injury, occurs with isolated axonal
and myelin injury but the endoneurium is left
intact. Sunderlands third- and fourth-degree inju-
ries are progressively more severe forms of
axonotmesis, as the injury is now associated
with endoneurial damage causing brosis and
loss of the guiding endoneurial tubes. The com-
plete disruption of the axons, endoneurium, and
perineurium across the entire fascicle marks
fourth-degree injuries. Neurotmesis, Sunderlands
fth-degree injury, occurs with complete transec-
tion of the entire peripheral nerve including the
epineurium. Sunderlands (modied) sixth-degree
injury describes a crush or traction injury
resulting in diffuse mixed degrees of nerve injury
(which may result in partial regeneration)
(Hosalkar et al. 2006; Kaufman et al. 2009;
Sunderland 1990).
With all second-degree injuries and greater,
Wallerian degeneration occurs in the axon distal
522
to the site of injury. During this process, which
begins within several hours of injury, the axons
and myelin break down. Schwann cells proliferate
and assist the inux of macrophages in phagocy-
tosis of the debris. After 2 weeks, the endoneurial
tubes begin to shrink. By 58 weeks the
endoneurial tubes will be completely empty. In
third-degree injuries, there is an even greater
inammatory response due to hemorrhage from
the injured blood vessels leading to an inux of
broblasts and subsequent scar formation. The
diameter of the distal endoneurial tubes progres-
sively decreases to a nadir at 34 months post-
injury, but without a regenerating axon becomes
obliterated from progressive brosis by increased
collagen deposition within the endoneurial sheath
over a course of many months (Burnett and Zager
2004; Sunderland and Bradley 1950). In fourth-
and fth-degree injuries, perineurium and epineu-
rium are disrupted which no longer limits the
inammatory response in either the proximal or
distal stumps, often preventing the passage of a
regenerating axon (Burnett and Zager 2004;
Sunderland 1990).
A specialized growth cone arises at the distal
tip of the proximally injured axon. Multiple
lopodia arise from the growth cone and facilitate
the progression of the regenerating nerve through
the Schwann cell tunnels (Burnett and Zager
2004). In third-degree injuries and greater, there
is an increased likelihood that the growing axon
will enter an endoneurial tube different than its
original. In mixed nerves, if a sensory axon enters
an endoneurial tube of a motor axon, that nerve
will fail to mature. While the traditionally stated
rate of nerve regeneration is 1 mm per day, the rate
is in fact variable as the growth rate decreases with
increasing distance from the cell body. The matu-
ration of regenerating nerves comprised of
remyelination and progressive increase in axonal
diameter occurs at a slower rate than axonal
growth. With motor nerves, the muscle itself grad-
ually loses the ability to support functional
reinnervation a process that takes around
18 months (Burnett and Zager 2004; Lee
et al. 2004). Upon reinnervation, collateral
sprouting occurs resulting in groups of
reinnervated muscle bers typically larger and
J.A. Stromberg and J. Isaacs
less organized than pre-injury motor units
(Lee et al. 2004; Wilbourn 2002).
While rst- and second-degree injuries can
likely recover with conservative management,
some third-degree and all of the fourth- through
sixth-degree injuries require surgical intervention
to achieve the best chance of functional recovery
(Kaufman et al. 2009). It is the discretion of the
surgeon to determine if surgery is indicated after
obtaining all pertinent data from the physical
exam and diagnostic tests.
Diagnostic Evaluation
Physical Examination
The history and physical exam are often limited in
the pediatric patient population. The patient may
be nonverbal, uncooperative, or unable to fully
comprehend questioning. The inciting injury
may have been unwitnessed or the functional def-
icit may have been insidiously present for a sig-
nicant amount of time. Pain from other injuries
may confound the exam by producing voluntary
or involuntary weakness (Feinberg et al. 1997).
Static and moving two-point discrimination are
valuable sensory tests in older children though
younger children will struggle in interpreting
what they are being asked. They may not admit
this and will often guess the right answer
(Hosalkar et al. 2006). Intact digital nerves should
successfully distinguish moving points 25 mm
apart at the ngertip and 712 mm apart at the
palmar base (Kaufman et al. 2009). Vibrometers,
which assess large myelinated bers, and
Semmes-Weinstein monolament instruments,
which assess pressure thresholds, are also useful
adjuncts, though again successful evaluation of
these tools is dependent upon the patients age
(Hosalkar et al. 2006; Kaufman et al. 2009;
Werner and Andary 2002). Indirect measurement
of anhidrosis can identify underlying nerve dam-
age. The absence of wrinkled skin after prolonged
submersion in water is suggestive of nerve injury
but may be less sensitive in the acute setting
(Kaufman et al. 2009; Panthaki 2009). Keeping
the test as simple as possible may also be a useful
23 Nerve Anatomy and Diagnostic Evaluation
tactic, such as limiting the assessment to sharp and
dull discrimination and then comparing sensation
of the affected digit with a normal digit.
When evaluating sensation, there are three
areas that are most consistently innervated by a
single nerve: the dorsal rst web space for the
radial nerve, the palmar distal phalanx of the little
nger for the ulnar nerve, and the palmar distal
phalanx of the index nger for the median nerve.
Motor exam should initially assess the resting
posture and nger cascade followed by assessing
passive and active movement from the shoulder to
the distal interphalangeal joints. Strength testing
is best carried out in an organized systematic
approach covering the length of the extremity.
Useful tricks to assess nger abduction or
adduction are to have the patient cross their n-
gers or hold a piece of paper between their ngers.
Adjuncts to an exam for young or uncooperative
patients can include observation of a patient
interacting with a toy or building blocks. Some-
times trying to get the child to reach for keys or a
cookie (while restraining the normal limb) can be
the only way to illicit activity. Obviously pain and
anxiety (including the parents) can make this a
trying exercise for everyone in the room. Repeat
exams (on different visits) can be especially help-
ful in this subset of patients.
In the acute traumatic situation, deep lacera-
tions with any suggestion of nerve injury should
be explored, while closed trauma requires a more
in-depth assessment as described above.
Electrodiagnostic Testing
Electrodiagnostic evaluation with nerve conduc-
tion (NC) studies and electromyography (EMG)
can be particularly helpful tests when the history
and exam are difcult to interpret. However, an
accurate test requires a relaxed patient. A cooper-
ative patient may quickly become uncooperative
once the discomfort of this test is realized. A
reasonable and often-used approach is to perform
the EMG under anesthesia if it cannot be com-
pleted without this intervention though some
portions of the test such as active recruitment
will be lost. EMG and NC studies can be helpful
523
in localizing a lesion and differentiating between
neuropraxic injuries and axonotmetic injuries but
cannot otherwise predict which injuries will spon-
taneously recover and which ones require surgical
intervention.
Variables assessed during a motor nerve con-
duction study include latency, conduction, nerve
conduction velocity, and compound muscle action
potential (Lee et al. 2004). Latency is the time
required to generate a compound muscle action
potential in the target muscle after stimulating the
nerve. The distal latency is the time needed to
stimulate the same muscle from a more distal
location on the motor nerve. The conduction
time is the difference between the latency and
the distal latency and therefore is a direct mea-
surement of conduction along the nerve and inde-
pendent of factors affecting the motor end plate
and subsequent muscle depolarization. Nerve
conduction velocity is determined by dividing
the conduction by the distance between the prox-
imal and distal stimulation sites. The conduction
velocity increases in the presence of myelin and is
also directly proportional to the axonal diameter
due to the decreasing resistance with increasing
axon thickness. The measured nerve conduction
velocity will not diminish until a signicant num-
ber of nerve bers are affected, as this is a mea-
surement of the fastest intact nerve bers. The
waveform of the compound muscle action poten-
tial (also known as an M wave) can yield substan-
tial information. A prolonged duration of the
potential indicates slowing of some nerve bers
as slower conduction will induce muscle contrac-
tion at a later time. A decreased amplitude indi-
cates incomplete motor nerve conduction, either
due to conduction block or axonal loss (Kane and
Oware 2012; Lee et al. 2004; Wilbourn 2002).
F-waves, variable low-amplitude waves from
recurrent discharge of a few motor neurons after
a supramaximal stimulus, are either absent or
mildly prolonged in severe radiculopathy (Kane
and Oware 2012; Lee et al. 2004). Delayed
F-waves may be present in demyelination (Kane
and Oware 2012).
The EMG is the more invasive portion of
electrodiagnostic studies and involves inserting a
needle into the muscle belly to measure the
524
intrinsic electrical activity. EMG assesses the
electrical activity at rest, the motor unit potential
on minimal voluntary contraction, and the recruit-
ment pattern on maximal contraction (if the
patient is awake and cooperative). Insertion of
the needle will normally cause a small temporary
discharge of electrical activity. After the initial
insertional activity, there should be minimal or
no electrical activity in a normal innervated mus-
cle. Increased spontaneous activity due to
hyperexcitable motor end plates is pathologic and
typically a consequence of denervation. Fibrilla-
tions, positive sharp waves, and fasciculations are
commonly present in denervation but are not
unique to this condition. Fibrillations are irregular
spontaneous contractions of individual muscle
bers, whereas fasciculations are spontaneous con-
tractions of the entire motor unit and are therefore
visible and palpable on exam. After approximately
23 weeks post-injury, the motor end plates will
have undergone enough changes such that brilla-
tions and positive sharp waves can be detected.
Like in NC studies, the shape of the motor unit
potential offers further insight into the status of the
motor unit. The amplitude represents the number
of muscle bers activated by stimulation of a motor
unit, whereas the duration represents recruitment
and synchrony of muscle ber ring (Lee
et al. 2004). While acute denervation will result
in a short amplitude and short duration, early
reinnervation will produce a high-amplitude and
long-duration potential due to the surviving nerve
bers branching out to many denervated muscle
bers and increasing their motor unit territory
(each axon now innervates more muscle bers
than they did pre-injury) (Burnett and Zager
2004; Lee et al. 2004; Wilbourn 2002).
The timing of performing electrodiagnostic
tests in relation to the initial injury is crucial to
obtaining an accurate diagnosis due to the tempo-
ral changes of nerve and muscle. Neurotmesis and
axonotmesis are indistinguishable initially since
time is needed to allow for Wallerian degeneration
to occur. For instance, it may take up to 10 days
for the compound nerve action potential to disap-
pear in the distal nerve segment after a fth-degree
injury, though all degrees of injury will exhibit a
conduction block immediately. Hyperexcitable
J.A. Stromberg and J. Isaacs
changes in the muscle such as brillation poten-
tials may not be present for 10 days or more
depending on the distance of the injury from the
target muscle (Kane and Oware 2012; Lee
et al. 2004). Therefore, the initial
electrodiagnostic testing should not be performed
acutely and should be performed roughly 2 weeks
post-injury (Hosalkar et al. 2006).
While providing important and often useful
information, the knowledge obtained with serial
EMG and NC studies obtained should be weighed
against the associated discomfort and risk
(if sedation required). The question should be
asked, how will this information potentially
change my treatment? Obviously differentiating
neuropraxic injuries bodes an excellent prognosis
and diminishes anxiety. However, repeat exams
with this diagnosis do not add much to the plan of
expectant observation. Likewise, once the diag-
nosis of axonotmetic injury has been established,
the remaining clinical dilemma is whether or not
the injury will regenerate on its own or whether
surgical intervention will be necessary. Repeat
testing should be timed with potential
reinnervation. Randomly repeating conduction
studies is rarely helpful. If a nerve injury is
noted to be 4 in. proximal to a denervated muscle,
a new EMG should be delayed until such testing
would conrm or challenge the onset of
reinnervation of that muscle. Since axon regener-
ation occurs at approximately 1 in. per month, in
this hypothetical scenario, repeat testing would be
helpful at 4 months but not at 3 months.
Nerve Visualizing Modalities:
Ultrasound
As the quality and ease of ultrasound imaging
improves, this painless and versatile modality
becomes a particularly attractive diagnostic tool in
evaluating pediatric nerve injuries. When the nerve
of interest can be identied, high-frequency trans-
ducers provide a greater spatial resolution than
magnetic resonance imaging (MRI) and allow for
visualization of submillimeter objects such as digital
nerves and even, potentially, the nerve macrostruc-
ture (Kessler et al. 2012; Kinni et al. 2009; Smith
23 Nerve Anatomy and Diagnostic Evaluation
Fig. 4 Ultrasound image
of traumatized median
nerve. This is a transverse
view of the median nerve
(encircled by white line).
Note the hypoechoic outer
ring of the epineurium with
annular and attened inner
hyperechoic structures
corresponding to the nerve
fascicles (personal image,
none needed)
et al. 2011). Outer and inner epineurial layers are
hyperechoic, whereas the endoneurium and nerve
fascicles are hypoechoic (Fig. 4) (Koenig
et al. 2009). This alternating pattern is responsible
for the striped appearance in a longitudinal view and
honeycombed appearance in a transverse view
(Jacobson et al. 2010; Kessler et al. 2012). The
echotexture of nerves is often comparable to ten-
dons and can be differentiated from tendons by their
immobility during passive movement of joints
(Koenig et al. 2009). To readily differentiate periph-
eral nerves from arteries or veins, which are pulsatile
or compressible, respectively, the Doppler mode can
be used. Arteries or veins also serve as landmarks to
orient the user due to their close association with
nerves throughout the majority of the upper
extremity.
The depth of sound wave penetration is
inversely dependent upon the frequency, with
less penetration as the frequency increases. Alter-
natively, the resolution is directly dependent upon
the frequency, with a greater appreciation for
detail with increasing frequency. This is why
high-frequency transducers in the range of
1518 MHz should ideally be used to visualize
supercially located nerves such as the ulnar
nerve at the elbow or the median nerve through
the carpal tunnel, but lower-frequency transducers
in the range of 912 MHz should be used to
visualize deep tissue nerves such as the brachial
plexus (Koenig et al. 2009).
525
During the evaluation of a nerve injury, ultra-
sonography is often able to identify the location
of injury and offer important information on
nerve integrity. Sonographic characteristics of
an entrapped nerve include decreased
echogenicity and fusiform thickening of the
nerve immediately proximal to the site of com-
pression with distal nerve attening (Jacobson
et al. 2010; Kessler et al. 2012; Kinni
et al. 2009; Koenig et al. 2009). To facilitate
treatment, ultrasound can frequently establish
the source of compression such as ganglia,
tumors, accessory muscle bellies, boney callous,
xation screws or plates, and small structural
tissue such as the ligament of Struthers as well
(Jacobson et al. 2010; Kinni et al. 2009; Koenig
et al. 2009). In trauma, nerve laceration versus
transection can often be differentiated. In the
nonacute setting, the terminal neuroma or the
presence of scar encasement of the injured
nerve may be identied (Martinoli et al. 2011).
Like the use of ultrasound to assess other pathol-
ogies, the quality of the information obtained is
related to operator experience, depth and size of
the structure being investigated, overlying tissue
damage and edema, and the patients body habi-
tus. Thin, small limbs, as seen in pediatric
patients, make pediatrics a particularly appealing
patient population for this modality, and it has
become a valuable assessment tool, though
should not be considered a crystal ball.
526
Nerve Visualizing Modalities: Magnetic
Resonance Imaging
MRI is another useful modality to evaluate for
nerve injury, particularly in this patient population
though, has the disadvantage (compared with
ultrasound) that the child must remain perfectly
still in an often loud and intimidating environ-
ment. MRI can potentially visualize the major
nerves of the upper extremity and localize the
injury. On both T1- and T2-weighted images,
nerves have a similar signal intensity to muscle,
but will not enhance with gadolinium-based con-
trast (Andreisek et al. 2006). Nerves are best visu-
alized by the surrounding circumferential layer of
extraneural fat which distinguishes them from the
neighboring musculature (Andreisek et al. 2006;
Kijowski et al. 2005). Inammation from chronic
nerve compression can cause thickening of the
nerve and increased signal intensity (Kijowski
et al. 2005). While MRI is typically unable to
detect smaller nerves such as the anterior
interosseous nerve, it is still sensitive at detecting
nerve injuries through changes in the muscle (Kim
et al. 2007). In the acute phase, neurogenic muscle
edema may be noted (Andreisek et al. 2006).
Chronic denervation will demonstrate atrophied
muscle with fatty inltrate (Kijowski et al. 2005).
The major obstacle to this modality (also when
compared to ultrasound) is the multiplane nature
of the nerve paths. The nerve cannot typically be
seen in its entirety, although the axial plane is
typically the most practical perspective to assess
the peripheral nerve (Andreisek et al. 2006).
Though loss of continuity can sometimes be visu-
alized, often the tests are indeterminant espe-
cially in areas of complex anatomy such as the
brachial plexus.
Summary
Nerve injuries in general have a better prognosis in
children than adults, but many surgeons are reluc-
tant to perform unnecessary surgery on children.
Nerve injuries amendable to repair are often
observed for unreasonable amounts of time with
J.A. Stromberg and J. Isaacs
the hope of avoiding surgery at all. Repeat and
often pointless testing and diagnostic evaluations
are offered instead. Indeed, this patient population
offers unique challenges to the nerve surgeon try-
ing to adequately assess the risk-benet ratio of
surgical intervention. Injured children are often
unable or unwilling to communicate their subjec-
tive complaints, demonstrate their objective de-
cits, or tolerate appropriate testing. The surgeon
must compensate for these obstacles with a high
index of suspicion, repeat physical examinations,
and the judicious use of sometimes painful studies.
Increased risks of sedation to obtain further infor-
mation must be weighed against the risk of early
surgical exploration. Ultrasound imaging, and to a
lesser extent MRI, may have a greater role in this
particular patient population because of this and
similar dilemmas especially as the quality of these
modalities improves.
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 Ulnar Nerve Injury
24
Angela Wang

Contents Abstract
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 529 The ulnar nerve in children is not commonly
injured. The most common causes of trauma to
Ulnar Nerve Brief Anatomy . . . . . . . . . . . . . . . . . . . . . . . 530
the ulnar nerve in children involve fractures
Physical Examination . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 530 around the elbow and their treatment, namely,
Nerve Conduction Studies . . . . . . . . . . . . . . . . . . . . . . . . . 531 supracondylar humerus and medial
epicondylar fractures. Knowledge of the ulnar
Ulnar Nerve Subluxation . . . . . . . . . . . . . . . . . . . . . . . . . . 532
nerve anatomy and issues that can arise during
Ulnar Nerve Compression . . . . . . . . . . . . . . . . . . . . . . . . . 532 treatment of these fractures can lessen the
Cubital Tunnel Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . 532
Guyons Canal Compression . . . . . . . . . . . . . . . . . . . . . . . . 533 chance of iatrogenic ulnar nerve injury.
Compression by Mass Lesions . . . . . . . . . . . . . . . . . . . . . . 533 The ulnar nerve can also be subject to com-
Other Compressions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 534 pression syndromes in children, primarily at
Injury . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 534 the elbow and less commonly at the wrist.
Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 534 Most often, the cause of nerve compression is
Tardy Ulnar Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 535 not known, but it can be due to fracture
Direct Trauma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 539 malunion or a space-occupying lesion.
Ulnar Pediatric Nerve Tumors . . . . . . . . . . . . . . . . . . . . 541
Direct trauma to the ulnar nerve can be
watched if the nerve is in continuity, or
Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 541
repaired or grafted if the nerve has been sev-
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 541 ered or ruptured. Innovative surgeries includ-
ing nerve transfers have been developed, but
results have only been reported in adults. For-
tunately, ulnar nerve recovery in children tends
to have good results, likely due to the greater
potential for nerve regeneration in the pediatric
Electronic supplementary material: The online version population.
of this chapter (doi:10.1007/978-1-4614-8515-5_24)
contains supplementary material, which is available
to authorized users. Videos can also be accessed at
http://www.springerimages.com/videos/978-1-4614-8513-1.
Introduction
A. Wang
Pediatric peripheral nerve injuries are relatively
Department of Orthopaedic Surgery, University of Utah,
Salt Lake City, UT, USA uncommon. They differ from adult nerve injuries
e-mail: angela.wang@hsc.utah.edu in their potential for greater neural plasticity and
# Springer Science+Business Media New York 2015 529
J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_24
530
quicker axonal regeneration, with rates of axonal
regrowth thought to be as quick as 5 mm/day in
children. The ulnar nerve in particular is a mixed
motor and sensory nerve, with a long distance to
travel to its end motor targets in the hand (intrinsic
muscles). Hence, ulnar nerve injury in adults has
demonstrated less favorable outcomes than those
of median or radial nerve injuries. However, due
the greater potential for nerve regrowth in chil-
dren, an otherwise devastating nerve injury may
ultimately end up with a good result. Ulnar nerve
conditions in children can be largely grouped into
compression (direct or due to mass effect) and
trauma (fracture or directly to the nerve). Primary
nerve tumors are uncommon.
Ulnar Nerve Brief Anatomy
The ulnar nerve arises mainly from the C8 and T1
nerve roots, with a sometimes contribution from
C7 nerve root. These roots contribute to the lower
trunk and subsequently the medial cord of the
brachial plexus. As it courses distally in the
upper arm, the ulnar nerve does not usually give
off any branches proximal to the antebrachial
area, although occasionally a motor branch to
the triceps can be seen. The nerve runs accompa-
nied by the superior ulnar collateral branch of the
brachial artery and later by the ulnar collateral
branch of the radial artery. At the elbow, the
ulnar nerve courses posterior to the medial
epicondyle. In children, the medial epicondylar
ossication center appears at 57 years of age
and completes its fusion to the distal humerus at
1520 years of age. The ulnar nerve passes
through the cubital tunnel behind the medial
epicondyle in the ulnar groove and can give a
few branches to the capsule at this level. The
ulnar nerve then proceeds to provide its rst
motor branch to the exor carpi ulnaris.
In the proximal forearm, the ulnar nerve inner-
vates the exor digitorum profundus to the ring
and small ngers. As it continues distally, approx-
imately 68 cm proximal to the ulnar styloid, the
dorsal sensory branches from the ulnar nerve
proper, which provides sensation to the dorsal
ulnar portion of the hand (usually the small nger
A. Wang
and the ulnar portion of the ring nger to the
proximal interphalangeal joint). The ulnar nerve
then passes through Guyons canal, where it pro-
ceeds to give off a branch to the hypothenar mus-
cle; it then splits into sensory and motor
components. The sensory component provides
volar sensation to the ulnar side of the ring nger
and both sides of the small nger. The motor
branch dives deep and courses across the palm in
a radial direction to innervate the intrinsic muscles
and half of the exor pollicis brevis (the other half
is innervated by the median nerve).
The topography of the ulnar nerve has been
studied with respect to ulnar nerve grafting and
nerve transfers. Proximally, the sensory and motor
bers intermingle with a considerable amount of
ne interplexal connections. As the nerve courses
more distally, the sensory and motor portions
become more distinct. At the level of the elbow,
the motor portion of the ulnar nerve courses more
along the volar side, between two sensory por-
tions. This arrangement changes as the nerve runs
through Guyons canal at the wrist, with the motor
portion then becoming more dorsal and radial in
location (Brown et al. 2009).
Nerve cross-sectional area has been evaluated
and has been found to correlate with increasing
age. Cartwright et al. (2013) found on ultrasound
of normal subjects that nerve cross-sectional size
increased also with body mass index but corre-
lated most closely with age.
Physical Examination
Diagnosis of nerve injuries and specic muscle
testing is challenging in children. The child may
be agitated or uncomfortable. He or she may be
unable to follow commands for sensory and motor
testing. Children often do not understand the
meaning of tingling and numbness (Hosalkar
et al. 2006). These confounding factors can
delay accurate diagnosis. It is imperative to assess
all major nerves (radial, ulnar, and median) to
diagnose motor and sensory decits. In the older
child, two-point discrimination should be tested
with the childs eyes closed. Vibrometry and
Semmes-Weinstein monolament exam can be
24 Ulnar Nerve Injury 531

Fig. 1 Seventeen year-old

male that sustained a medial
arm laceration involving the
ulnar nerve (Courtesy of
Shriners Hospital for
Children, Philadelphia). (a)
Medial arm laceration. (b)
Loss of exor digitorum
profundus function. (c)
Inability to cross ngers. (d)
Positive Froments sign. (e)
Positive Wartenbergs sign

used to test vibration and pressure thresholds,
respectively (Hosalkar et al. 2006). Ulnar nerve
sensory loss includes the small nger and ulnar
half of the ring nger.
The ulnar decits will vary according to the
level of injury (Fig. 1). High ulnar nerve injures
(above the elbow) will negate exor carpi ulnaris,
exor digitorum supercialis (ring and small), and
exor digitorum profundus function along with all
of the ulnar intrinsic muscles within the hand.
Low ulnar nerve injuries (at the wrist) will not
affect those forearm muscles. Low lesions result
in clawing of the ring and small digits as the exor
digitorum profundus provides the deforming
force. The classic ndings of ulnar nerve injury
include the inability to cross ngers and marked
loss of dexterity. The classic signs are Froments
sign (loss of the adductor pollicis and substitution
by the exor pollicis longus) and Wartenbergs
sign (loss of the palmar interosseous and pull of
the extensor digit minimi).
Nerve Conduction Studies
Nerve conduction studies are generally of limited
use in the young pediatric population, largely due
to difculties with patient compliance and toler-
ance. In addition, the clinically observed func-
tional improvement may not always correspond
to electrical improvement and vice versa. In addi-
tion, in order to obtain a good study, sedation is
532
Fig. 2 Ulnar nerves that
subluxate may yield
symptoms requiring
treatment. (a) Ulnar nerve
in a resting position within
the cubital tunnel. (b)
Anterior subluxation with
elbow exion
often required, which confounds the interpreta-
tion. Thus, the routine use of electrophysiologic
studies in children is not universally embraced.
Nevertheless, ulnar mononeuropathy is one of the
more frequently seen pediatric nerve diagnoses,
with the electrophysiologic prognosis being more
favorable in nontraumatic causes as compared to
traumatic ones (Felice and Royden Jones 1996).
In addition, two particular scenarios in children
may cause altered responses with respect to the
results of electrophysiologic studies. Firstly, it has
been found that prenatal alcohol exposure (>2
oz. absolute alcohol/day) in young children can
cause abnormalities in nerve conduction studies,
with slower nerve velocities and smaller proximal
and distal amplitudes as compared to controls
(Avaria Mde et al. 2004). Secondly, it has also
been found that nerve conduction results are
altered in children with Type I diabetes mellitus.
Cenesiz et al. (2003) examined electrophysiologic
studies in forty children with Type I diabetes and
compared the results to a control group of thirty
patients. All nerve conduction values in children
with diabetes mellitus were found to be signi-
cantly lower as compared to those of the control
group, and overall. Sixty percent of diabetic chil-
dren were found to have some type of peripheral
neuropathy.
Ulnar Nerve Subluxation
Ulnar nerve subluxation at the elbow can be a
cause of medial elbow pain, mostly in adults.
This subluxation nding can be seen in children,
A. Wang
although it is usually asymptomatic. Erez
et al. (2012) demonstrated in an ultrasound study
that 37 % of normal children had subluxating or
dislocating ulnar nerves at the elbow. Patients
with unstable ulnar nerves tended to be younger
(aged 610 years) and were more likely to have
generalized ligamentous laxity. Waters and col-
leagues also examined normal pediatric subjects
and found a statistical association between young
age, ligamentous laxity, and ulnar nerve instabil-
ity, as well as a strong presence of bilateral ulnar
nerve subluxation (Zaltz et al. 1996).
Subluxating ulnar nerves that are symptomatic
may need to have treatment similar to that of
cubital tunnel syndrome (Fig. 2). Most cases are
asymptomatic and are simply associated with
young age and ligamentous laxity. This nding
can be important when considering pinning of
supracondylar humerus fractures. If 10 % of all
children have unstable ulnar nerves in exion, a
subluxating or dislocating ulnar nerve could be
particularly at risk with a medial pin.
Ulnar Nerve Compression
Cubital Tunnel Syndrome
The ulnar nerve can be subject to compression at
several sites along its course. At the elbow, the
possible common sites of nerve compression are
(from proximal to distal) the arcade of Struthers
(an overlying fascial layer), the medial head of the
triceps, the medial intermuscular septum, the
medial epicondyle itself (especially if there are
24 Ulnar Nerve Injury
Fig. 3 Sixteen year-old male underwent right ulnar nerve
decompression. An epitrochlear anconeus was found over-
lying the cubital tunnel (Courtesy of Shriners Hospital for
Children, Philadelphia)
bony abnormalities), Osbornes ligament (also
sometimes referred to as Osbornes band or
Osbornes fascia) that overlies the cubital tunnel,
and lastly between the heads of the exor carpi
ulnaris muscle. Collectively, compression of the
ulnar nerve in any of these potential areas can lead
to cubital tunnel syndrome. In addition, exion of
the elbow causes attening of the cubital tunnel,
decreasing its volume and compressing the ulnar
nerve.
Cubital tunnel syndrome is rare in the pediatric
and adolescent population, though it has been
reported in the young, throwing athlete (Godshall
and Hansen 1971). In the average pediatric patient
presenting with symptoms of cubital tunnel syn-
drome, Stutz et al. (2012) found in 39 extremities
that nonoperative treatment (including nighttime
splinting, anti-inammatory medication, and
activity modication) was not uniformly success-
ful, but they still recommended conservative treat-
ment as an initial approach. They did nd that
patients who underwent surgical release
(30 extremities) obtained good relief of symp-
toms. Most of the time, the etiology of cubital
tunnel in the pediatric and adolescent population
is idiopathic. In some cases, the cause may be
related to a subluxating ulnar nerve. Less common
etiologies of compression of the ulnar nerve in the
cubital tunnel area include compression by the
anconeus epitrochlearis muscle (Fig. 3) (Boero
et al. 2009). A single case of cubital tunnel has
533
Fig. 4 Anterior transposition of the ulnar nerve at the
elbow
been reported in a child with Larsens syndrome
and a dysplastic medial epicondyle (Tubbs
et al. 2008).
The surgical options for the treatment of cubital
tunnel are similar to adults with no technique dem-
onstrating superior efcacy. Symptomatic sublux-
ation requires anterior transposition (Fig. 4).
Guyons Canal Compression
At the wrist, the ulnar nerve can become com-
pressed within Guyons canal, which is a bro-
osseous tunnel distal to the wrist exor retinacu-
lum where the ulnar nerve and artery enter the
hand. The roof of Guyons canal is composed of
the volar carpal ligament and the pisohamate lig-
ament. The cause of ulnar nerve compression
within Guyons canal can be idiopathic (most
common) or due to a space-occupying lesion
such as a ganglion or vascular mass (ulnar artery
aneurysm). Entrapment of the ulnar nerve at
Guyons canal in general is also rare in children.
It has been reported in a case associated with
exuberant scar tissue formation after a laceration
over the volar ulnar wrist (Kalaci et al. 2008).
Compression by Mass Lesions
The ulnar nerve can become compressed by a
mass anywhere along its course in the upper
534
extremity. Such lesions can arise from the bone,
the soft tissue, the vascular system, or the joint.
Some of the more commonly seen benign masses
in children include ganglion cysts and
osteochondromas.
Ganglion Cysts
Ganglion cysts are thought to be outpouchings of
the joint lining and are lled with synovial uid.
Most cases associated with ulnar nerve compres-
sion have been reported in the adult literature,
both at the cubital tunnel/elbow region and in
Guyons canal. Only one case has been reported
in a child, in the Japanese literature, which
occurred distally at the wrist (Miwa et al. 1970).
Treatment of symptomatic lesions consists of
excision of the mass, along with nerve exploration
and decompression.
Osteochondromas
Osteochondromas in children can occur either as a
singular lesion or multiple lesions (also known as
multiple hereditary exostosis). They tend to occur
at the physeal region of bones and actively grow
until skeletal maturity, after which point the
lesions become more quiescent. Lesions that
occur about the elbow could have the potential
to cause ulnar nerve stretch or compression, but
this has not been commonly reported, with only
one report in an adult in the Turkish literature
(Karakurt et al. 2004). Excision of the lesion is
recommended if persistent ulnar nerve symptoms
arise, along with exploration and decompression
of the nerve.
Other Compressions
The ulnar nerve can be at risk in the upper
extremity for compression from such entities
as uid accumulation and swelling. Some of
the more unusual cases of ulnar palsy reported
in children due to compression include
intraneural hemorrhage in a hemophiliac
(Cordingly and Crawford 1984) and after intrave-
nous uid extravasation (Dunn and Wilensky
1984).
A. Wang
In addition, children born with congenital con-
striction band syndrome have been reported to
have ulnar nerve involvement (Uchida and
Sugioka 1991; Weeks 1982). An early suspicion
for ulnar nerve involvement is recommended,
with prompt nerve decompression to maximize
the chance for nerve recovery.
Injury
Fractures
Most neuropathies associated with a fracture at the
time of injury are likely neuropraxic and can be
monitored for recovery; however, the ulnar nerve,
due to its relatively supercial location and close
association with the ulnohumeral joint, has a par-
ticular risk of injury with elbow fractures. Entrap-
ment of the ulnar nerve has been reported in an
olecranon fracture (Ertem 2009) and an elbow
dislocation (Reed and Reed 2012), and entrap-
ment with laceration of the ulnar nerve has been
reported in forearm fractures (Stahl et al. 1997).
The following section discusses some of the more
common elbow fractures that may result in ulnar
nerve injury. Careful observation of the initial
fracture pattern and displacement along with a
meticulous physical exam, combined with a
close assessment of clinical recovery, can aid in
decision making regarding potential nerve
exploration.
Supracondylar Humerus Fractures
Supracondylar humerus fractures are the most
commonly seen elbow fractures in children. Frac-
tures needing surgical intervention are often
treated by crossed pinning, but one of the most
worrisome possible complications is that of ulnar
nerve injury associated with the injury itself, or
from xation of the fracture. Fracture pinning is
usually performed with the elbow in the exed
position for fracture reduction, but this
maneuver can cause anterior subluxation of the
ulnar nerve, especially in young, lax children,
which puts the ulnar nerve at direct risk during
medial pinning.
24 Ulnar Nerve Injury
One meta-analysis examining neuropraxias
associated with supracondylar humerus fractures
showed that the overall incidence of ulnar nerve
injury with surgical treatment of supracondylar
humerus fractures was 6 %. The ulnar nerve was
the most frequently damaged nerve, occurring
mostly in exion-type fractures, whereas the ante-
rior interosseous nerve was at higher risk in exten-
sion type fractures (Babal et al. 2010). Eberl
et al. (2011) found a higher rate of iatrogenic
ulnar nerve injury (15 %) in children treated with
crossed pins as compared to .4 % in children
treated by antegrade nailing. However, pinning
is the most common method to surgically treat
supracondylar humerus fractures, and much liter-
ature has been devoted to determining the safest
and most stable pin conguration.
A few meta-analyses of pinning methods have
demonstrated greater fracture stability but up to
fourfold increased risk of iatrogenic ulnar nerve
injury with crossed pins as compared to lateral
pins (Woratanarat et al. 2012; Zhao et al. 2013),
and another meta-analysis suggested there is an
iatrogenic ulnar nerve injury for every 28 patients
treated with crossed pinning as compared with
lateral pinning (Slobogean et al. 2010). Eberl
et al. (2011) also found that medial pinning con-
ferred the highest overall risk of nerve injury,
including the ulnar nerve. In patients demonstrat-
ing neuropathy associated with supracondylar
humerus fractures however, most studies have
shown good results in terms of nerve recovery
(including median, radial, and ulnar nerves) with
observation. Therefore, routine nerve exploration
is not recommended particularly if a mini-open
approach was used (Ramachandran et al. 2006;
Khademolhosseini et al. 2013). In summary, one
should be aware of the increased concern for ulnar
nerve injury with a exion-type supracondylar
humerus fracture, especially with treatment by
medial or crossed pinning, and use of a mini-
open incision should be considered to visualize
the ulnar nerve.
Medial Epicondylar Fractures
Medial epicondyle fractures are relatively uncom-
mon, accounting for only about 20 % of total
535
pediatric elbow fractures. The mechanism of
injury can be by direct trauma, by avulsion, or
associated with an elbow dislocation (roughly
60 % of medial epicondylar fractures are associ-
ated with an elbow dislocation). Surgical indica-
tions include open fractures, an intra-articular
fracture fragment, gross instability, and ulnar
nerve entrapment (Fig. 5).
The incidence of ulnar nerve injury, despite a
close anatomic association with the area of injury,
is relatively low. It has been recommended that the
ulnar nerve be protected during xation, but rou-
tine dissection is unnecessary (Gottschalk
et al. 2012). However, if the medial epicondylar
fragment is small and the injury associated with an
elbow dislocation, late ulnar nerve palsy has been
reported due to a trapped nerve (Haah
et al. 2010; Lima et al. 2013). Therefore, a high
index of suspicion for ulnar nerve entrapment in
these cases is warranted. Clinical results in treat-
ment of medial epicondylar fractures overall are
good; specically with respect to the ulnar nerve,
Kamath et al. (2009) found in their systematic
review no difference in postoperative ulnar nerve
symptoms between fractures treated operatively
versus nonoperatively, regardless of their preop-
erative ulnar nerve symptoms.
Tardy Ulnar Palsy
Tardy ulnar palsy can develop after a child incurs
an elbow injury associated with bony nonunion,
malunion, or simply bony overgrowth with time.
Around the elbow, supracondylar humerus frac-
tures as well as medial or lateral condylar fractures
can heal and proceed to develop an angular defor-
mity, resulting in cubitus varus or valgus, both of
which can be associated with ulnar nerve symp-
toms (Fig. 6).
Cubitus Valgus
Tardy ulnar palsy associated with posttraumatic
cubitus valgus is thought mainly to be due to
stretch on the ulnar nerve due to changes in the
conguration of the distal humerus in the medial
epicondylar region. The normal carrying angle of
536
Fig. 5 Fifteen year-old right hand-dominant male
dislocated right elbow (Courtesy of Shriners Hospital for
Children, Philadelphia). (a) X-rays demonstrate postero-
lateral dislocation with displaced medial epicondyle frac-
ture. (b) Following closed reduction, medial epicondylar
fragment appears to be within the ulnohumeral joint. (c)
Sagittal CT scan cut infers medial epicondyle within the
ulnohumeral joint. (d) Coronal CT scan conrms medial
A. Wang
epicondyle within the ulnohumeral joint. In addition, phys-
ical examination revealed absent sensation in the ring and
small ngers and inability to cross his ngers or contract
his rst dorsal interosseous muscle. (e) Medial incision
with extrication of ulnar nerve traveling into ulnohumeral
joint (Courtesy of Shriners Hospital for Children, Philadel-
phia). (f) AP X-ray after xation with a cannulated screw
24 Ulnar Nerve Injury 537

the elbow is around 11 of valgus, and increasing been reported after surgery by nerve decompres-
amounts of valgus can increase stretch on the sion, both with and without associated corrective
ulnar nerve as the nerve is relatively tethered in osteotomy of the distal humerus (Mortazavi
the area of the cubital tunnel. Good results have et al. 2008; Kang et al. 2013).

Fig. 6 (continued)
538
Fig. 6 Ten year-old boy with left lateral condylar non-
union, progressive valgus, and tardy ulnar nerve palsy
(Courtesy of Shriners Hospital for Children, Philadelphia).
(a) Photograph demonstrating left elbow cubitus valgus
deformity. (b) Anteroposterior X-ray of lateral condylar
nonunion. (c) Child positioned prone on operating table
and anatomic landmarks drawn on posterior elbow. (d)
Fixation of nonunion site with cannulated screws. (e)
Cubitus Varus
The etiology of ulnar neuropathy in cubitus varus
is not as straightforward as in cubital valgus.
Cubitus varus is thought to cause tardy ulnar
palsy not primarily by nerve traction, but second-
arily through changes in the elbow anatomy.
Kwanishi et al. (2013) studied three-dimensional
A. Wang
Dome osteotomy about lateral condylar nonunion site. A
second dome was completed to allow lateral translation of
the distal fragment. The intervening piece of bone is
removed. (f) Medial plate and screw xation. Note lax
ulnar nerve after dome osteotomy. (g) Postoperative
X-ray following screw xation of lateral condylar non-
union and dome osteotomy
bone models of patients with cubitus varus and
found that the posterior trochlea, distal lateral
capitellum, radial head, and ulnar articular surface
were all enlarged compared to the normal side. In
addition, the ulna was shifted more medially and
distally. Some authors have reported muscle
impingement on the nerve with a forward shifting
24 Ulnar Nerve Injury 539

Video 1

of the medial head of the triceps (Ogino

et al. 1986; Spinner et al. 1999), whereas others
have noted subluxation or instability of the ulnar
nerve with compression by brous bands of the
FCU (Abe et al. 1995; Fujioka et al. 1995; Jeon
et al. 2006). In all cases, good results were
obtained by treatment with nerve decompression,
again with or without corrective osteotomy of the
distal humerus.

Direct Trauma

Unfortunately, the relatively supercial position of
the ulnar nerve at the elbow and wrist puts it at risk
during penetrating trauma. Fortunately, as previ-
ously mentioned, children have a greater potential
for nerve regrowth and more favorable plasticity of
the neural circuits than adults that can yield in good
(Video 1) results after severe injury. Complete dis-
ruption of the ulnar nerve is treated with direct
repair if possible or cable grafting for a larger defect
(Fig. 7). More recently nerve transfer of the anterior
interosseous nerve to the motor branch of the ulnar
nerve has been utilized, although the results of
nerve transfers have mainly been reported in adults
(Video 1). The ulnar nerve can be transposed at the
elbow to lessen tension on the repair or grafting
site, and the arm can also be immobilized in a
Fig. 7 Ulnar nerve cable graft
position of elbow and wrist exion to decrease
tension on a nerve repair or graft.
Baysefer et al. (2004) reported on a series of
21 pediatric ulnar nerve explorations and repairs
due to trauma (fracture or laceration) in children
and found better results in lesions requiring only
neurolysis or decompression as opposed to dis-
continuous lesions (Fig. 8). This nding is similar
to outcomes seen in the adult literature, where
neurolysis of intact nerve lesions can usually
have good results over 90 % of the time. Chemnitz
et al. (2013) reported on long-term outcomes after
45 pediatric ulnar and median nerve repairs in the
forearm with an average follow-up of 31 years. In
540 A. Wang

patients less than 12 years of age at the time of

repair, 87 % had complete recovery; in contrast, if
the repair was performed between 12 and 20 years
of age, only 67 % achieved full recovery. There
were no differences in the outcomes between
median and ulnar nerves. Overall motor function
was close to normal, and cold intolerance was not
an issue.
A small series of secondary (delayed) median
and ulnar nerve grafting in children with a mean
follow-up of 7 years showed results were superior
to a comparison group of adults but inferior to those
obtained by primary repair (Ceynowa et al. 2012).
Overall, although the ulnar nerve has relatively
poorer recovery with repair or reconstruction as
compared to the median and radial nerves, largely
due to the distal location of its motor targets,
children can demonstrate good results especially
with younger age at time of injury and an injury in
Fig. 8 Ulnar nerve lesion in continuity treated with
a more distal location.
neurolysis

Fig. 9 Schwannoma of the ulnar nerve. (a) MRI of the lesion. (b) Intraoperative dissection
24 Ulnar Nerve Injury
Ulnar Pediatric Nerve Tumors
Primary nerve tumors in general are not common
in the pediatric population. They have on occasion
been reported in the ulnar nerve: an ossifying
brolipomatous hamartoma has been documented
in a 5-year-old (Drut 1988), and Cavanagh and
Pincott (1977) described a small case series of
pediatric ulnar nerve tumors including a
neurilemmoma (also known as schwannoma, a
tumor which usually arises from a single nerve
fascicle) (Fig. 9) and a neurobroma (a usually
more involved nerve and brous lesion, which can
be solitary or associated with general neurobro-
matosis). Both authors described meticulous dis-
section of the nerve and knowledge of the nerve
anatomy, allowing for sparing of intact branches
whenever possible.
Summary
The pediatric ulnar nerve may be injured due to
trauma, compression, or mass effect. Symptoms
may be acute or seen later in life as a sequel of a
childhood event. The greater neural plasticity in
children affords good results with prompt treat-
ment, even in the setting of nerve disruption.
Intimate knowledge of the ulnar nerve
anatomy can aid in dissection and repair or recon-
struction, thus maximizing the chances for nerve
recovery.
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 Median Nerve Injury
25
Allan Peljovich and Felicity Fishman

Contents Abstract
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 544 Median nerve injuries in children are rare but
can lead to devastating functional decits if
Pathoanatomy and Applied Anatomy . . . . . . . . . . . . 544
they are not recognized and treated promptly.
Assessment of Median Nerve Injury . . . . . . . . . . . . . . 546 These injuries most commonly occur second-
Median Nerve Injury Treatment Options . . . . . . . . 548 ary to fractures, dislocations, penetrating
Nonoperative Management of Median wounds, and lacerations. As the neurologic
Nerve Injuries . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 548 evaluation of children can be challenging, cli-
Operative Treatment of Median Nerve Injuries . . . . . 549
nicians should maintain a high level of suspi-
Surgical Procedure: Tendon Transfers cion for median nerve injury in children
for Irreparable Injury to the Median Nerve . . . . . 556
presenting with injuries in proximity to the
Restoring Median Nerve Muscle Function . . . . . . . . . . 556
Restoring Median Nerve Sensory Function . . . . . . . . . 558 anatomic course of the median nerve. An initial
period of observation for patients presenting
Preferred Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 558
with median nerve palsy following closed frac-
Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 560 tures or gunshot injuries may be warranted.
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 560 However, in patients who demonstrate motor
or sensory decits in the distribution of the
median nerve following open fractures, pene-
trating wounds, or lacerations, a low threshold
for early exploration is appropriate.
EMG/NCV studies can help guide treatment
in patients with delayed return of median
nerve function.
Options for surgical intervention depend
upon the length of the nerve defect and the
time elapsed from injury to operative interven-
A. Peljovich (*) tion. For patients with median nerve defects
The Pediatric Hand and Upper Extremity, Center of
2 cm, options include primary epineural or
Georgia, Atlanta, GA, USA
fascicular repair or use of a synthetic conduit.
e-mail: drp@handcenterga.com
For patients with median nerve defects 2 cm,
F. Fishman
options include allograft, autograft, or nerve
Yale Orthopaedics and Rehabilitation, New Haven,
CT, USA transfers. Patients with late presentations or
e-mail: fsh11@gmail.com; felicity.shman@yale.edu who have undergone a failed nerve repair are
# Springer Science+Business Media New York 2015 543
J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_25
544
candidates for tendon transfers to restore lost
median nerve motor function. Children are
generally believed to have better potential for
motor and sensory recovery than adults follow-
ing injury to the median nerve.
Introduction
Median nerve injury in children is fortunately rare
but can be devastating. Fractures, dislocations, lac-
erations, penetrating wounds, and crush or ampu-
tation injuries are the reported mechanisms of
injury (Hosalkar et al. 2006; Kaufman et al.
2009). Peripheral nerve injuries have been reported
in association with approximately 1020 % of
supracondylar humerus fractures, with the anterior
interosseous nerve and median nerve proper most
likely injured in posterolaterally displaced
extension-type supracondylar fractures, (Hosalkar
et al. 2006). Difculties with examining and
assessing nerve function in young children
mandate a high level of suspicion for median
nerve injury especially those where the median
nerve is particularly at risk, (e.g. volar forearm
and wrist laceration). The approach to treatment is
based upon the acuity of presentation and the
pathoanatomy. Although median nerve injuries
and defects are challenging to treat, it is generally
accepted that children have a superior potential for
recovery than adults and often have satisfactory
results following surgical treatment of median
nerve injuries (Birch and Achan 2000; Chemnitz
et al. 2013; Senes et al. 2009; Barrios and dePablos
2013; Valencia et al. 2005).
Pathoanatomy and Applied Anatomy
The median nerve arises from the medial and
lateral cords of the brachial plexus, with contribu-
tions from C6 to T1. The C6C7 roots contribute
most of the sensory axons, while the C7T1 roots
contribute most of the motor axons (C6 does con-
tribute some motor to exor carpi radialis and
pronator teres) (Hentz and Leclercq 2002; Perez
et al. 2008). The median nerve does not usually
provide motor innervation in the upper arm as it
A. Peljovich and F. Fishman
travels from lateral to medial in the interval
between the biceps and brachialis muscles. At
the level of the coracobrachialis muscle, the
median nerve crosses anterior to the brachial
artery to rest medial to the vessel (Leversedge
et al. 2010). In the antecubital fossa, the median
nerve enters the forearm just deep to the lacertus
brosus (bicipital aponeurosis) before separating
from the vessels and diving between the two
heads of the pronator teres. The lacertus brosus
is an excellent anatomic landmark for securing the
location of the nerve with injuries in the
antecubital area. The nerve continues distally
through exor digitorum supercialis (FDS) and
then lies between the FDS and exor digitorum
profundus (FDP). The anterior interosseous nerve
(AIN) typically arises from the median nerve just
distal to the leading arch of the FDS. As the
median nerve approaches the wrist, it becomes
more supercial and continues distally in the
interval between the FDS and the exor carpi
radialis (FCR) and just deep to the palmaris
longus (PL) as it approaches the wrist. The palmar
cutaneous branch of the median nerve typically
arises 57 cm proximal to the wrist and courses
distally along the antebrachial fascia near the
ulnar side of the FCR tendon (Doyle and Boyte
2003; Leversedge et al. 2010; Mazurek and Shin
2001).
The median nerve becomes more supercial
approximately 5 cm proximal to the wrist and
continues just deep and radial to the tendon of
the PL. The median nerve typically enters the
wrist at the level of the volar wrist crease via the
carpal tunnel as a single trunk, subsequently
dividing into three common digital nerves. The
recurrent branch of the median nerve that inner-
vates the thenar intrinsics (abductor pollicis
brevis, opponens pollicis, and supercial head of
the exor pollicis brevis), most commonly
branches from the main trunk just beyond the
distal aspect of the carpal tunnel ligament. The
rst common digital nerve typically divides into
three proper palmar digital nerves, supplying sen-
sation to the radial and ulnar aspects of the thumb,
the radial aspect of the index nger, and a branch
to the rst lumbrical. The second common digital
nerve splits into the proper digital nerves of the
25 Median Nerve Injury
Fig. 1 High branching
bid median nerve
second web space and supplies small branch to the
second lumbrical. The third common nerve of the
median nerve courses to the third web space and
provides sensibility to the ulnar aspect of the long
nger and the radial aspect of the ring nger
(Doyle and Boyte 2003; Mazurek and Shin 2001).
It is important for the clinician to be aware of
the described variations in the anatomy of the
median nerve in the forearm, wrist, and hand. In
the proximal forearm, a connection between the
median and ulnar nerves is referred to as a Martin-
Gruber connection (1025 %). If a high median
nerve injury occurs in a patient with a Martin-
Gruber anastomosis, the physical exam ndings
may demonstrate loss of function of some of the
intrinsic musculature of the hand (rst dorsal
interosseous, adductor pollicis, abductor digiti
minimi) in addition to the loss of normally median
nerve-innervated musculature. The median nerve
has been observed to branch proximal to the wrist
and therefore be present distally as a bid nerve
(Fig. 1). If an injury occurs to one branch of a bid
median nerve, the corresponding distal sensory
and motor exam may indicate partial denervation
in the median nerve distribution. Although this
physical examination nding could be secondary
to a partial laceration of the median nerve, a high
branching bid nerve should be in the clinicians
differential diagnosis. In the carpal tunnel, the
motor branch can be transligamentous,
545
subligamentous, or extraligamentous. Multiple
recurrent motor branches can arise from the
median nerve trunk. The clinician must be aware
of these patterns during a release of the carpal
tunnel as the transligamentous branch is likely at
a higher risk of iatrogenic injury than an
extraligamentous branch.
A Riche-Cannieu anastomosis commonly
exists between the recurrent motor branch of the
median nerve and the deep branch of the ulnar
nerve (5075 %) within the bers of the exor
pollicis brevis (FPB) that can provide dual inner-
vation of the intrinsic muscles of the hand. In
patients with a Riche-Cannieu anastomosis, the
FPB may remain fully innervated after an injury
to the median nerve. A more distal connection
between the median and ulnar nerves has also
been described. The palmar ulnar-median com-
municating branch of Berrettini is a distal com-
municating branch between the ulnar and median
sensory nerves and may be present in majority of
the population (Doyle and Boyte 2003;
Leversedge et al. 2010).
The intraneural topography of the median
nerve has been studied extensively. At the carpal
tunnel, the median nerve is typically 94 % sensory
and 6 % motor fascicles (Leversedge et al. 2010).
Chow et al. noted that at the level of the wrist, the
median nerve typically consists of three fascicular
groups. The radial aspect of the nerve is composed
546
Fig. 2 (a) Healed laceration with occult median nerve injury in a child presenting with a mutilated index nger. (b)
Intraoperative photograph of occult median nerve injury
of one large fascicular group, containing sensory
and motor fascicles. The ulnar aspect of the
median nerve at the wrist is comprised of two
sensory fascicular groups (Chow et al. 1986).
The median nerve provides innervation to PT,
FDS, FCR, and palmaris longus (PL). The AIN
innervates the exor pollicis longus (FPL), FDP
to the index nger, and the pronator quadratus
(PQ). The recurrent motor branch of the median
nerve innervates the rst and second lumbricals,
abductor pollicis brevis (APB), opponens pollicis
(OP) and the supercial head of the FPB; while
branches from the proper digital nerve to the index
and the common digital nerve to the index/middle
nger innervate the rst and second lumbricals,
respectively. Median nerve decits are generally
classied as high or low, based on their location
proximal or distal to the original of the AIN in the
forearm. High median nerve injuries affect both
the intrinsic and extrinsic median nerve-
innervated musculature with a loss of almost all
of the forearm exor compartment muscles,
intrinsic opposition, FPL, and PQ. Low median
nerve injuries generally result in loss of APB and
OP with partial preservation of FPB function.
Assessment of Median Nerve Injury
Assessing for nerve injuries in children is often
difcult compared to examining skeletally mature
individuals. The particularly difcult component
A. Peljovich and F. Fishman
of assessment is the critical sensory loss to the
thumb and radial digits. The function of the FPB,
still partially intact from the ulnar nerve, can fool
careful observers as to the functioning of the the-
nar intrinsics in infants and toddlers who cannot
easily cooperate during a physical examination. In
fact, direct communication and participation in an
exam is limited in young children. A thorough
physical examination must be performed, and a
high level of suspicion for nerve injuries should be
maintained when a child presents with a penetrat-
ing injury or a fracture commonly associated with
nerve lesions. Any uncertainty can be settled, by
surgical examination of the nerve. Initial assess-
ment in the emergency department of a seemingly
benign puncture or penetrating injury may result
in a missed median nerve laceration and is further
complicated by the childs anxiety in the emer-
gency setting. Children may present with median
nerve lesions from a remote injury. In delayed or
occult presentation of median nerve injury in chil-
dren, neglect or mutilation of an insensate digit in
the median nerve distribution may be the
presenting symptom and the reason for referral
(Birch and Achan 2000) (Fig. 2a, b).
Physical examination of the motor component
of the median nerve is generally performed by
examining the median nerve-innervated muscula-
ture. A reliable motor examination for both extrin-
sic and intrinsic muscles of the median nerve can
be obtained by examining thumb opposition and
exion at the IP joint. In younger children,
25 Median Nerve Injury 547

observation during activities requiring these

motions can provide similar information to exam-
ination of an older child who is able to follow
commands. If the examiner can gain the con-
dence of the child, palpating for muscle contrac-
tion can help differentiate between the integrity of
the median- versus ulnar-innervated thenar intrin-
sics. Grading muscle strength using the standard
British Medical Research Council system is
almost impossible in young children, and the
examiner needs to rely on the presence of muscle
contraction and the childs functional abilities
during tasks to infer strength. Incorporating the
evaluation of a physiotherapist can be useful.
Physical examination of the sensory compo-
nent of the median nerve is more challenging.
Hermann et al. reported that the normal moving
two-point discrimination for children aged 418 is
24 mm. His evaluation of 348 healthy children
and adolescents demonstrated that a moving
two-point discrimination examination was reli-
able in children 6 years of age or older (Hermann
et al. 1996). In children younger than 6 years old, Fig. 3 Three-year-old female with thumb ulnar digital
other methods of assessing sensation and sympa- nerve laceration. The wrinkle test illuminates the injury
as the radial-sided pulp wrinkles in response to uid
thetic function must be utilized. Observation of immersion, while the ulnar side of the thumb pulp does not
sweat in the median nerve distribution, skin wrin-
kling, and timed tactile gnosis can be employed to
further investigate the integrity of the sensory presenting with lacerations, penetrating injuries,
components of the median nerve. Moberg or fractures with high associations with nerve
endorsed the ninhydrin ngerprinting test to lesions, such as posterolaterally displaced
assess sweat patterns in the ngers following extension-type supracondylar fractures, displaced
nerve injury (Moberg 1964). The tactile adher- metaphyseal distal radius fractures, and Galeazzi
ence test is a simple alternative test described to fractures (Hosalkar et al. 2006). When nerve inju-
assess anhidrosis in which a plastic pen can be ries occur in the setting of lacerations, the damage
rolled against the ngertip. Frictional movement is often to the surrounding tendons, muscles, and
with tactile adherence will be noted if sweating is vessels as well, but to parents and some physi-
intact. However, if the skin is dry and lacks inner- cians, the childs ability to grossly ex ngers and
vation, there will be no adherence of the plastic thumb often hides the true nature of the injury.
pen (Harrison 1974). An additional test of This is especially the case in lacerations involving
sudomotor function is the wrinkle test origi- the volar wrist crease, where unless the injury
nally described by ORiain. The patients hand is penetrates deep enough to affect the FDP, the
placed in a basin of warm water for approximately true extent of the injury can easily be overlooked.
30 min. Normally innervated skin will wrinkle, Wounds sustained secondary to glass can be
while the skin in the distribution of the injured deceivingly small and conceal the true extent of
nerve will remain smooth (ORiain 1973) (Fig. 3) the injury (Iconomou et al. 1993). Upper extrem-
(Vasudevan et al. 2000). ity fractures in children are more than twice as
A healthy level of suspicion for nerve injury likely to be associated with nerve injuries as lower
should be held by the clinician for all children extremity fractures (Hosalkar et al. 2006). Up to
548
21 % of extension-type fractures of the
supracondylar humerus, especially those with
posterolateral displacement of the distal fragment
have been associated with AIN injury. Median
nerve injury has also been reported following
diaphyseal fractures of the radius and ulna as
well as Galeazzi fracture-dislocations (Babal
et al. 2010).
In addition to a thorough physical examina-
tion, radiographs of the affected extremity can
provide additional information as to the nature of
the injury. More advanced imaging, such as CT or
MRI, is not generally warranted in the evaluation
of a suspected median nerve injury. Electromyog-
raphy (EMG) and nerve conduction velocity test-
ing can be a valuable tool in assessing median
nerve injuries associated with closed injuries and
with delayed recoveries. The problem with these
studies is that young children do not tolerate the
needle portion of the EMG testing without anes-
thesia therefore, the value of the results must be
considered before ordering an EMG. If required, it
is important to communicate exactly what infor-
mation is needed to help direct the electrophysi-
ologist to avoid unnecessary parts of the
electrodiagnostic testing.
Median Nerve Injury Treatment
Options
Nonoperative Management of Median
Nerve Injuries
An initial trial of observation is often warranted in
median nerve decits associated with fractures, as
they are often lower grade nerve injuries. Careful
neurovascular examination of the involved
extremity should be performed and documented
both before and after a closed reduction is
performed. Nerve injuries in this setting can be
the result of a direct contusion or a traction injury
from displacement of the fracture fragments.
Spontaneous recovery in Sunderland rst-degree
median nerve injuries generally occurs within
3 months as there has been no disruption of the
internal structures of the nerve (Sunderland 1951;
Seddon 1942). Second-degree Sunderland
A. Peljovich and F. Fishman
injuries (axon disrupted, endoneurium intact)
have the potential to recover at approximately
1 mm/day or 1 in./month, and children should be
followed with serial physical examinations during
the rst 36 months following injury. An advanc-
ing Tinels sign can be serially documented in
these children to track the progress of nerve recov-
ery. In such cases, an EMG/NCS study to assess
baseline injury is utilized, but it is important wait
for at least 3 weeks from injury to ensure that any
Wallerian degeneration is underway to obtain an
accurate assessment of nerve function. A repeat
study is used after 46 months (depending on the
anatomic location of the injury) in the absence of
any clinical recovery. Clinical signs of recovery
include advancing Tinels; return of pseudomotor
functions (sweat, skin pads); return of sensation
(often hypesthesia initially), and motor function
(Birch 2011; Kaufman et al. 2009; Waters and Bae
2012) (Table 1).
Outcomes of Nonoperative Management
Our experience with median nerve injury in chil-
dren suggests that the mechanism of injury is the
more important variable in decision-making. As
demonstrated in other studies (Waters and Bae
2012), median nerve dysfunction in the setting
of closed injuries warrants a 46-month period
of observation. On the other hand, median nerve
dysfunction that presents following any manipu-
lation, such as with elbow dislocation or forearm
fractures, merits more aggressive treatment
Table 1 Nonoperative management of median nerve
injury
Median nerve injury: nonoperative management (initial
observation)
Indications Contraindications
Suspected neuropraxia Open fracture associated
associated with fracture with median nerve decit
Gunshot wounds with Laceration explored for
nerve decit additional injured structures
with obvious nerve
transection
Median nerve injury
documented to occur
following closed
manipulation of a fracture or
dislocation
25 Median Nerve Injury
Fig. 4 Median nerve
entrapped within the elbow
joint following closed
reduction of a posterior
elbow fracture-dislocation
involving the medial
epicondyle. The nerve
decit was not recognized
until well after the fracture
had healed
including surgical exploration (Fig. 4). If a childs
injury required manipulation, and there was no
assessment prior to the reduction, than any
median nerve dysfunction is presumed to be new
and merits surgical exploration.
Children with gunshot wounds to the upper
extremity can present a clinical challenge. The
injuries can vary dramatically based on the veloc-
ity of the weapon as well as the proximity of the
weapon to the child at the time the injury was
sustained. A peripheral nerve can be transected
or damaged directly by the projectile, or it can be
injured indirectly by shock wave cavitation, the
energy the projectile emits through the surround-
ing soft tissues (Oberlin and Rantissi 2011). Addi-
tionally, peripheral nerves can be irritated and
injured by shrapnel embedded within the soft
tissues after the gunshot wound is sustained. If a
child presents with median nerve palsy following
injury with a stable bullet that travelled in prox-
imity to the anatomic location of the median
nerve, there is a high likelihood of direct injury
to the nerve, and therefore early exploration and
repair is recommended. With a large cavitation
injury, it is possible that a median nerve palsy
might be secondary and an initial period of obser-
vation may be appropriate (Oberlin and Rantissi
2011; Stoebner et al. 2011). Roganovic speci-
cally reported on a series of Sunderland grade V
median nerve injuries sustained secondary to
549
missiles. He concluded that children have better
recovery following repair of the median nerve in
this clinical scenario and that both the level of the
repair and the time between injury and surgical
repair were independent predictors of successful
outcome for motor and sensory recovery
(Roganovic 2005). It is ultimately up to the
treating surgeons judgment, but such injuries
often require urgent operative treatment, and so
the nerve should be routinely explored in these
situations.
Operative Treatment of Median Nerve
Injuries
The surgical approach to any peripheral nerve
injury is best understood by dividing the injury
into the treatment of acute versus chronic injuries
and sharp lacerations versus stretch/crush injuries
where the zone of nerve injury is greater. The
majority of surgical treatment in acute and sub-
acute injuries involves lacerated median nerves as
the presence of an open wound in the face of a
nerve decit often infers a nerve laceration. In
contrast, the majority of surgery in the chronic
setting manages stretch and crush injuries, as
these are the mechanisms of injury associated
with most closed injuries. The challenge in both
situations is accurately assessing the zone of
550
injury so that the surgeon can condently repair or
graft healthy viable nerve endings resulting in a
greater likelihood of success. The challenge more
often found in the latter situation is assessing the
nerve injury intraoperatively, especially when the
nerve is in continuity and there is a neuroma in
continuity. Another issue critical to the surgical
treatment of the median nerve issue concerns the
estimated time of nerve regeneration. Repaired
and healing nerves advance at a rate of about
1 mm/day. The target muscles for reinnervation
start the process of internal degeneration the
moment of denervation, and it is assumed and
understood that at some point in time, 1821
months from the time of injury, the denervated
muscles undergoes irreversible end-plate demise
while the muscle bers themselves undergo mor-
phological changes that will make them irrevers-
ibly brotic. Moreover, the sooner the muscles are
reinnervated, the greater the potential return of
strength. The surgeon must take these factors
into account as he/she decides the optimum time
to intervene along with the best methods of repair
and reconstruction.
Sharp knife lacerations and penetrating injuries
secondary to glass are a common cause of median
nerve injury in children and typically present soon
after the injury (Iconomou et al. 1993). If a nerve
decit is identied or suspected, surgical explora-
tion is warranted. The laceration may be explored
acutely if other injuries require urgent surgical
intervention (acute hemorrhage) or can be delayed
for up to a week or so. One of the advantages of
delayed exploration is the better ability to gauge
the zone of injury that can extend beyond the
immediate margins of the lacerated nerve ends.
The dissection and identication of the injured
structures is generally easier if performed earlier,
before scar tissue has the opportunity to form, and
the nerve ends retract proximally and distally from
the site of the laceration (Birch 2011; Waters and
Bae 2012). In the setting of an open fracture with
an associated median nerve decit, operative
exploration at the time of fracture debridement
and xation is advised.
Primary repair of the median nerve is usually
possible in the acute and subacute setting without
the need for substantial mobilization, posturing of
A. Peljovich and F. Fishman
the elbow or wrist, or nerve graft/conduit provided
a narrow zone of injury (Kaufman et al. 2009).
Even if the zone of injury is up to 2 cm, or viable
nerve ends are about 2 cm apart, a primary repair
is achievable. If the gap is greater than 4cm;
however, a bridge will be necessary (Hosalkar
et al. 2006). The lacerated nerve ends are prepared
by sharp resection until the organized internal
architecture of the nerve is visualized and appears
healthy (Birch 2011). Less resection is generally
required in both lacerations and acute repairs,
while crush, stretch, and contaminated injuries
can lead to considerable nerve damage proximal
and distal to the visible zone of injury even in the
acute setting. A tension-free repair is critical to the
potential for nerve recovery. During repair of an
acute or chronic nerve injury, elongation of 15 %
results is a severe decrease in blood ow and
therefore a decreased potential for nerve regener-
ation (Clark et al. 1992).
Positioning and exposure of the median nerve
can help facilitate a tension-free repair. With the
patient in the supine position and the operative
arm placed on a stable hand table, exion of the
wrist and hand can help approximate the ends of
the nerve and decrease tension across the repair
site. This position can be maintained
intraoperatively with the use of any commercially
available surgical hand positioner/immobilizer.
Careful mobilization of potential soft tissue
tethers proximal and distal to the repair can also
aid in decreasing tension. Proximal forearm inju-
ries to the median nerve may require division of
the pronator fascia to increase visualization and
ability to mobilize the proximal portion of the
nerve (Pederson and Person 2008). The clinician
should consider release of any known areas of
constriction distal to repair site as compression
has been reported to delay and impair nerve regen-
eration (Johnston et al. 1993). In distal forearm
median nerve injuries, release of the carpal tunnel
should be considered. When injuries to the
median nerve occur at the level of the wrist or
palm, the transverse carpal ligament will likely
need to be released for proper exposure of the
proposed site of repair.
Primary surgical repair can be epineural, fas-
cicular, or group fascicular. For digital nerve
25 Median Nerve Injury
Fig. 5 (a) Median nerve laceration in forearm. (b) Primary epineural repair of median nerve in forearm
lacerations, an end-to-end epineural repair is
performed with 9-0 nylon using the microscope.
An epineural repair with 8-0 nylon can also
be performed for more proximal lacerations to
the median nerve in the forearm and wrist
(Fig. 5a, b). The advantages of the epineural repair
lie in its surgical simplicity and gentleness
compared to other repairs that require internal
dissection and damage to the perineural vessels.
The surgeon can use the epineural vascular anat-
omy to line up vessels as well as aligning matched
fascicles to perform as anatomic a repair as
possible (Tables 2, 3, and 4). A fascicular repair
requires stripping the outer epineurium and then
directly aligning the perineurium of individual
fascicles within the median nerve. This is more
feasible distally in the forearm and wrist level as
the internal fascicles are organized into a more
predictable alignment and there are fewer in num-
ber. A compromise between the two techniques is
that of grouped fascicular repair, which requires
less traumatic dissection but aligns groups of
fascicles with segmental sutures through the
inner epineurium (Issacs 2010). This technique is
particularly useful in the distal median nerve in the
forearm where the intraneural topography is well
documented (Chow et al. 1986). Despite multiple
investigations, the superiority of epineural or
fascicular repairs has not been denitively proven
(Issacs 2010; Orgel and Terzis 1977). Cabaud
et al. demonstrated no statistically signicant
difference in the results of peripheral nerve repairs
551
performed with epineural or perineural fascicular
techniques (Cabaud et al. 1976).
Although microsurgical repair of peripheral
nerves is generally performed using
nonabsorbable microsutures, the development of
brin adhesives offers a sutureless alternative
(Isaacs 2010; Isaacs et al. 2008). Theoretically,
nerve repair utilizing adhesive alone could
decrease operative time and traumatic handling
of the nerve ends. Moy et al. reported an improve-
ment in the speed of repair of peripheral nerves
using a brin seal versus suture, but found
increased reactive changes within the earlier
periods of nerve regeneration histologically
(Moy et al. 1988). However, others have demon-
strated decreased inammatory response and an
environment more conducive to nerve growth
when using brin adhesives to perform a periph-
eral nerve repair (Ornelas et al. 2006; Palazzi
et al. 1995).
Overall, children are believed to have better
potential for functional motor and sensory recov-
ery following nerve injury and nerve repair (Birch
and Achan 2000; Chemnitz et al. 2013; Senes
et al. 2009; Ruijs et al. 2005). Primary repair of
a median nerve laceration in an end-to-end fash-
ion is preferred over use of a graft or conduit.
Hudson et al. found very satisfactory return of
opponens pollicis function (4.5 out of 5) and a
mean static two-point discrimination of 5 mm in
children who underwent primary epineural repair
of the median nerve (Hudson et al. 1997). Tajima
552
Fig. 6 (a) Photograph of a median nerve exploration in
the forearm for a child who failed to recover median nerve
function following a palsy sustained as the result of a
displaced both bones forearm fracture. Intra-operative
nerve testing failed to demonstrate any evidence of con-
ductivity across the zone of injury. (b) Proximal and distal
Table 2 Operative treatment ! primary epineural repair
for median nerve laceration (preoperative planning)
Primary nerve repair for median nerve laceration
Preoperative planning
1. OR table: standard OR table with hand table
attachment (with a stabilizing leg)
2. Positioning: supine
3. Equipment: loupe magnication, operating
microscope, micro instruments, 8-0, 9-0, and 10-0 nylon,
11 blade, sterile tongue depressor, and hand stabilizer for
palmar or digital lacerations
4. Tourniquet: non-sterile
A. Peljovich and F. Fishman
stumps of healthy nerve left once the zone of injury was
sharply resected. This defect is too large for primary repair.
(c) Median nerve is reconstructed using auto-cable grafts
secured at each stump with brin glue and reinforced with
nerve-tubes. (Case and operative photographs courtesy of
Scott Kozin, MD)
reported excellent recovery of moving and static
two-point discrimination in children who
underwent group fascicular repair of the median
nerve at the level of the wrist (Tajima and Imai
1989). In children who sustained penetrating inju-
ries to the median nerve from glass, repair of the
median nerve resulted in very satisfactory sensory
return and acceptable motor function, with the
best results seen in the children who underwent
primary fascicular repair (Iconomou et al. 1992).
In studies that compare the results of repair and
grafting among the various upper extremity
25 Median Nerve Injury
Table 3 Operative treatment ! primary epineural repair
for median nerve lacerations (surgical technique)
Primary nerve repair for median nerve lacerations
Surgical steps (epineural repair)
1. Sterile prep and drape the extremity, inate tourniquet,
place hand in alumi-hand if palmar or digital laceration is
present
2. Extend traumatic laceration ! Bruner or mid-axial on
the hand and digits, straight on the volar forearm with
zigzags around the wrist creases
3. Exploration of wound under loupe magnication for
associated injuries
4. Identify nerve stumps proximally and distally
5. Irrigate and debride wound bed
6. Prepare nerve stumps ! resect unhealthy portions of
nerve using 11 blade on sterile tongue depressor until
healthy organized nerve is observed in proximal and
distal stump
7. Repair any additional injured structures
8. Reapproximate nerve ends using nylon (8-0 in distal
forearm, 9-0 or 10-0 for more distal injuries) using the
microscope. The 9-0 and 10-0 gauges may be the
appropriate size for forearm and wrist level repair in
infants at the surgeons discretion. Place 2 sutures
through the outer epineurium 180 apart. Use neural
vasculature to help orient stumps appropriately
9. Assess the tension on the repair and its stability by
mobilizing the wrist and ngers
10. Irrigate wound
11. Close incision with absorbable suture
Table 4 Operative treatment ! primary epineural repair
for median nerve lacerations (postoperative protocol)
Primary nerve repair for median nerve lacerations
Postoperative protocol
1. Immobilize postoperatively in splint or cast (surgeon
preference). Use long arm cast in young children and any
circumstances where elbow motion applies undue
tension on the nerve repair. Keep ngers and thumb free
(assuming digital nerve not repaired) to allow for some
degree of early nerve/tendon differential gliding. In cases
where arm positioning was required for a direct repair,
one can put some exion on the wrist, but it should not
exceed 3040 of exion
2. Anticipate early mobilization, within 12 weeks of
surgery, depending on the tension of repair and stability
as determined in Step #9. With older children, early
mobilization with splint protection allows differential
nerve/tendon gliding. In younger children, or
noncompliant children of any age, continue cast-type
protection for 3 weeks postoperatively
3. With the aid of a physiotherapist, work on early range
of motion, wound care, strengthening when range of
motion is restored, and neurosensory reeducation
techniques
553
nerves, median nerve injuries tend to respond very
favorably to surgical repair and reconstruction
compared to the radial and ulnar nerves (Murovic
2009).
For segmental defects in the median nerve of
34 cm or greater, a tension-free end-to-end repair
is difcult to achieve primarily. Options for bridg-
ing the defect when positioning the wrist/elbow
and mobilizing the nerve proximally and distally
are insufcient include autograft, allograft, and
nerve conduit. Autografts remain the most thor-
oughly researched and proven of the three options
for repairing longer nerve gaps. Autografts are
obtained from sensory nerves of similar size to
the segment of the median nerve in need of
grafting. The sural nerve can be used (and cabled)
for sections of the median nerve in the forearm.
The medial and lateral antebrachial cutaneous
nerves, the medial brachial cutaneous nerve, or
the posterior interosseous nerve from the dorsum
of the wrist can be employed for more distal areas
of injury to the median nerve in the palm and
digits. Results of median nerve grafting with auto-
graft in children are generally superior to that
found in adults, but are inferior to results of pri-
mary nerve suture. Ceynowa et al. performed a
retrospective review of children and adolescents
who underwent nerve grafting to the median or
ulnar nerves using sural nerve grafts with a mean
graft length of 2.8 cm. Although results were good
overall with a mean DASH score of 14.6 (average
period of observation of 7 years), two-point dis-
crimination return was incomplete. Motor func-
tion was better in patients with median nerve
injuries (Ceynowa et al. 2012). The results of
nerve grafting to restore function in the setting of
long segmental defects (>5 cm) are somewhat
mixed. Long segmental defects can be grafted
with favorable results, but shorter defects seem
to experience better outcomes (Socolovsky
et al. 2011; Karabekmez et al. 2009; Daoutis
et al. 1994).
Allograft presents an alternative to autograft,
without the morbidity or the residual sensory def-
icit left by the harvest of an in vivo graft. Previous
use of allograft has been hindered by considerable
immunogenicity requiring the use of immunosup-
pressive mediations (Tables 5 and 6). Processed
554
Table 5 Operative treatment ! nerve grafting for median
nerve laceration with deficit >2 cm (preoperative
planning)
Nerve grafting for median nerve laceration
Preoperative planning
1. OR table: standard OR table with hand table
attachment (with a stabilizing leg)
2. Positioning: supine
3. Equipment: loupe magnication, operating
microscope, micro instruments, 9-0 and 10-0 nylon,
allograft, collagen conduits
4. Tourniquet: non-sterile (for operative upper and lower
extremity if harvesting sural nerve)
Table 6 Operative treatment ! nerve autograft for
median nerve laceration with deficit >2 cm (surgical
technique)
Median nerve repair: autograft
Surgical steps
1. Steps 16 as per primary epineural repair of median
nerve
2. Measure length of nerve gap
3. Harvest autograft (sural, medial/lateral antebrachial
cutaneous, medial brachial cutaneous, or PIN over
dorsum of wrist) after injured nerve is fully exposed and
prepared. Graft length should exceed measured nerve
decit by at least 15 % to account for shrinkage
secondary to elastic recoil
4. If using multiple grafts to better approximate the size of
the median nerve ! grafts can be cut and then laid
together prior to securing with brin glue. Sew the grafts
to the proximal and distal ends of the median nerve stump
attempting to align fascicles if possible
5. Steps 810 as per primary epineural repair of median
nerve
nerve allografts, consisting of decellularized
predegenerated human nerve tissue, are now
available and do not require suppression of the
patients immune response. Karabekmez et al.
reported return of adequate sensation in hand
and nger segmental nerve defects of 0.53 cm
using decellularized nerve allograft (Karabekmez
et al. 2009). Recent data has shown promising
outcomes in reconstruction of peripheral nerve
gaps from 5 to 50 mm using allograft nerve (Cho
et al. 2012). Whitlock et al. demonstrated superior
outcomes in an animal model with peripheral
nerves coapted utilizing decellularized allograft
to use of a type 1 collagen conduit. However, in
A. Peljovich and F. Fishman
Table 7 Operative treatment ! nerve allograft for
median nerve laceration with deficit >2 cm (surgical
technique)
Median nerve repair: allograft
Surgical steps
1. Steps 17 as per primary epineural repair of median
nerve
2. Measure length of nerve gap and width of nerve ends
3. Select appropriately sized allograft ! sharply trim
allograft to a length to t decit without tension
4. Approximate distal and proximal stumps of median
nerve to allograft with nylon suture (8-0 in forearm, 9-0
or 10-0 for more distal repair) using microscope with two
stitches through the outer epineurium. Repair can be
augmented with brin glue
5. Steps 810 as per primary epineural repair of median
nerve
their study, autograft results were superior to that
of the allograft (Whitlock et al. 2009) (Table 7).
Synthetic bioabsorbable nerve conduits are
readily available and composed of collagen,
polyglycolic acid, (PGA) or caprolactone.
Although often used for digital sensory defects,
synthetic conduits have also been utilized in larger
mixed motor and sensory nerves, such as the
median nerve. It is generally accepted that con-
duits should not be used to bridge nerve decits of
larger than 3 cm. Many studies report encouraging
results of nerve regeneration using conduits; how-
ever, the gaps are often shorter than 3 cm.
Dienstknecht et al. demonstrated that type 1 colla-
gen conduits successfully reconstructed median
nerve segmental defects in the forearm of
12 cm (Dienstknecht et al. 2013).
The ideal nerve conduit would be
nonimmunogenic and resorb at the same rate as
the regeneration of the axons of the nerve being
repaired. Both type I collagen conduits and PGA
conduits are permeable, allowing diffusion to aid
in the regeneration of the entubulated nerve. Col-
lagen generally resorbs within 9 months of
implantation, and PGA resorbs in approximately
6 months (Deal et al. 2012). There are no random-
ized controlled trials comparing the
3 FDA-approved bioabsorbable conduits; how-
ever, individual studies of each material (collagen,
PGA, caprolactone) have each demonstrated good
to excellent results in restoration of sensation
25 Median Nerve Injury
Table 8 Operative treatment ! median nerve repair with
synthetic conduit (surgical technique)
Median nerve repair: synthetic conduit
Surgical steps
1. Steps 16 as per primary epineural repair of median
nerve
2. Measure the length of the nerve gap and the width of
the proximal and distal stumps (most appropriate for
decits of
3 cm)
3. Select appropriately sized conduit and hydrate in saline
for 5 min
4. Trim ends of conduit to t the nerve gap
5. Suture nerve ends to conduit ! Place an 8-0 or 9-0
nylon suture from the outside of the proximal aspect of
the tube into the lumen of the tube. An epineural stitch is
then placed in the proximal median nerve stump, and the
suture is then brought through the lumen from inside to
the outside of the tube, bringing the proximal stump
approximately 45 mm into the lumen of the conduit. A
second simple suture can be placed 180 from the initial
suture from the conduit to the epineurium. This technique
is repeated for the distal aspect of the median nerve stump
6. Flush the tube with saline to prevent an air-block
7. Steps 810 as per primary epineural repair of median
nerve
(Mackinnon and Dellon 1990; Taras et al. 2011;
Bertleff et al. 2005) (Table 8).
Peripheral nerves move with respect to their
surrounding soft tissues in vivo and stretch and
shorten to some degree (Coppieters et al. 2009;
Echigo et al. 2008; Dilley et al. 2003). Preventing
scar formation and adhesions helps to reduce the
risk of creating a new site of tension and/or com-
pression and helps in the overall rehabilitation of
the child. The surgeon must note how range of
motion of the proximal and distal joints affects
tension on the median nerve repair and then use
this intraoperative assessment to guide postoper-
ative care. Allowing the ngers and thumb to
move immediately helps create differential glid-
ing between the extrinsic nger exors and the
nerve, and allowing the wrist and forearm and
elbow to move early in the postoperative period
helps create differential gliding between the two
heads of the pronator and around the biceps ten-
don. It is appropriate to engage the help of a
qualied physiotherapist to help ensure compli-
ance through splinting and by monitoring and
guiding motion. Surgical ndings may mandate
555
Table 9 Operative treatment ! nerve grafting for median
nerve lacerations with deficit >2 cm (postoperative
protocol)
Nerve grafting for median nerve lacerations with decit
>2 cm
Postoperative protocol
Please refer to postoperative protocol for primary
epineural repair
restricting motion to within specic arcs of
motion to minimize tensioning the nerve repair.
Communication and guidance with physiotherapy
helps create the environment to maximize the
childs outcome. Sensory reeducation has also
been shown to improve outcomes, but to a limited
extent (Mavrogenis et al. 2009) (Table 9).
Median Nerve Transfers
The surgeon may be presented with a child who has
sustained a substantial loss of median nerve
substance. Defects larger than 46 cm restored
by nerve grafts are associated with poorer
reinnervation outcomes than smaller defects.
Another difcult situation involves median nerve
lacerations well proximal to the elbow where the
distance from repair to the rst points of innerva-
tion may be longer than what the muscles have for
time to reinnervate (usually associated with other
serious injury involving the ulnar nerve, the bra-
chial artery, and even the musculocutaneous
nerve). In the latter situation, the healing nerve
repair may simply take too long to ever innervate
critical musculature before irreversible end-plate
demise. One option is to resort to tendon transfer
reconstruction early, and the techniques are detailed
below. The other option is to restore critical por-
tions of the median nerve using nearby healthy
nerve tissue. Previously described donor motor
nerves include the motor nerve to the brachialis
muscle and the posterior interosseous nerve (both
the supinator and extensor carpi radialis brevis
branches). Donor sensory nerves include the lateral
antebrachial cutaneous nerve, the supercial radial
sensory nerve, and the common digital nerve to the
small/ring nger (ulnar nerve) (Murphy et al. 2012;
Bertelli et al. 2012; Hsiao et al. 2009; Ducic
et al. 2006; Rapp et al. 1999).
556
Surgical Procedure: Tendon Transfers
for Irreparable Injury to the Median
Nerve
The surgeon may be faced with a child for whom a
median nerve repair and/or reconstruction is no
longer possible or has failed to achieve a satisfac-
tory result. The former is the more common situ-
ation and typically results from a combination of
delayed presentation and delayed recognition.
The time from the injury and presentation either
is too long for a nerve repair/reconstruction to
succeed or extends well beyond 2 years from the
time of injury. This is when the surgeon needs to
specically dene the functional decits the child
is facing, and then construct a plan to improve the
childs situation as needed.
Restoring Median Nerve Muscle
Function
At a minimum, the motor decits of a chronic
median nerve injury include the median-
innervated thenar intrinsics and the radial two
lumbrical muscles. Since the ulnar nerve inner-
vates the interossei, the adductor pollicis, and a
portion of the exor pollicis brevis, the functional
decit is fundamentally limited to thumb opposi-
tion. In the setting of a Martin-Gruber anastomo-
sis, critical lateral pinch function could also be
lost. What a parent might notice, and the surgeon
should ascertain, is any difculties the child has
with large object acquisition, manipulation, and
lateral pinch. Reconstructing the childs ability to
oppose the thumb, thus rotating the thumb oppo-
site the digits for large object grasp and manipu-
lation, could reduce such difculties. In the setting
of a Martin-Gruber anastomosis, additional con-
sideration to restoring thenar adduction could help
reduce difculties with thumb/index pinch. If the
level of chronic median nerve injury extends
proximal to the mid forearm, then additional func-
tional decits could include impairment of object
manipulation and lateral pinch from absent FPL
and FDP index/long function and even severely
A. Peljovich and F. Fishman
Table 10 Operative treatment ! tendon transfers for
median nerve injury (preoperative planning)
Tendon transfers for median nerve injury
Preoperative planning
1. OR table: standard OR table with hand table
attachment
2. Positioning: supine
3. Equipment: loupe magnication, 40 braided
nonabsorbable suture
4. Tourniquet: non-sterile (for operative upper and lower
extremity if harvesting sural nerve)
impaired grasp from loss of FDS function. Loss of
the pronator teres and pronator quadratus are not
often noticed as the brachioradialis acts as a
secondary pronator.
Tendon transfer surgery is the gold standard
in restoring lost function from permanently dener-
vated muscle from median nerve injuries. The
primary functional impairment to restore in
low median nerve decits is opposition
(Table 10). The ability of the thumb to both
palmarly abduct and circumduct is a requisite for
large object acquisition and manipulation. The
key for the surgeon, therefore, is to restore the
thumbs positional mobility. This is accomplished
with a single donor muscle, and previous reports
and studies have used the following: FDS ring,
FDS long, extensor indicis proprius, abductor
digiti minimi, palmaris longus, extensor carpi
ulnaris, and others. The particular donor muscle
chosen for transfer may be inuenced by the pres-
ence of associated nerve injuries/palsies, muscle/
tendon injuries and even the location of any
compromised soft tissues. Familiarity with vari-
ous opponensplasty techniques will allow the sur-
geon to tailor the technique to accommodate a
childs particular needs (Tables 11 and 12). The
one insertional site that is most likely to mimic
opposition is the insertion of the APB. The surgeon
can also insert part of the donor tendon into the
extensor pollicis longus (EPL) or the dorsum of the
thumb metacarpophalangeal (MCP) joint to create
a combined opposition and MCP extension
moment, but this combined insertion is not typi-
cally required for an isolated median nerve palsy.
Finally, the vector of insertion needs consideration.
25 Median Nerve Injury
Table 11 Operative treatment ! FDS ring
opponensplasty (surgical technique)
Tendon transfers for median nerve injury
Surgical steps FDS ring donor muscle
1. Create Bruner-type approach centered about the distal
palmar crease of the ring nger
2. Harvest the FDS of the ring by pulling proximally and
cutting the tendon distal to campers chiasm (to avoid PIP
hyperextension deformity). The A1 pulley can be incised
to gain more length
3. Create another zigzag or transverse incision along the
wrist creases in line with the FCU and pisiform
4. Dissect the FCU while protecting the ulnar
neurovascular bundle. Divide the FCU longitudinally,
and release a distally based strip of the ulnar half such that
there is 34 cm of length. Loop the ulnar strip, and sew it
to itself distally to create a loop of tendon
5. Identify the FDS ring in the distal forearm through the
ulnar incision, and withdraw the FDS ring into and out of
this wound
6. Direct the FDS ring through the loop of FCU
7. Create a mid-axial incision along the radial aspect of
the thumb MCP joint more proximal than distal. Expose
the APB insertion while protecting the dorsal radial
sensory branch of the supercial sensory radial nerve
8. Create a capacious subcutaneous tunnel from the
thumb wound towards the wound where the FCU
is. Tunnel a clamp, i.e., Adson clamp, supercial to the
palmar aponeurosis
9. Using the clamp, or a suture passer, pass the FDS ring
into the palmar tunnel and out of the radial thumb wound
10. Weave the FDS ring into the APB insertion with at
least two weaves. Position the wrist in slight extension,
and the thumb in opposition, and then secure the transfer
at the estimated resting length of the FDS ring
11. Close all wounds, and apply a bulky, nonadherent
dressing and then a short arm thumb spica cast/splint in
the position of the transfer
The best mimic for opposition mandates that the
tendon approach the APB insertion from an area
near the pisiform. The classic opponensplasty
employs a loop of distally based exor carpi ulnaris
insertion (split tendon) to act as a pulley for the
FDS ring to create a specic vector of pull. As the
vector of pull moves radial and proximal, the direc-
tion of pull trends to direct more palmar abduction
and less thumb circumduction (Davis 2011).
In the event of a Martin-Gruber anastomosis
and associated loss of the adductor and rst dorsal
interosseous, pinch can be reconstructed with an
557
Table 12 Operative treatment ! ADM opponensplasty
(surgical technique)
Tendon transfers for median nerve injury
Surgical steps ADM donor muscle
1. Create an extended incision along the hypothenar
eminence from the base of the pisiform towards the ulnar
mid-axial aspect of the small nger proximal phalange
2. Expose the ADM muscle and protect the ulnar digital
nerve to the small nger. Expose the origin of the ADM
such that the ulnar neurovascular bundle, on the deep
surface of the ADM, is protected. Expose the distal
insertion of the ADM into its contribution of the ulnar
lateral band
3. Detach the ulnar lateral band of the small nger, then
continue freeing the ADM from the surrounding
hypothenar muscles
4. Dissect the FCU while protecting the ulnar
neurovascular bundle. Divide the FCU longitudinally,
and release a distally based strip of the ulnar half such that
there is 34 cm of length. Loop the ulnar strip, and sew it
to itself distally to create a loop of tendon
5. Identify the FDS ring in the distal forearm through the
ulnar incision, and withdraw the FDS ring into and out of
this wound
6. Direct the FDS ring through the loop of FCU
7. Create a mid-axial incision along the radial aspect of
the thumb MCP joint more proximal than distal. Expose
the APB insertion while protecting the dorsal radial
sensory branch of the supercial sensory radial nerve
8. Create a capacious subcutaneous tunnel from the
thumb wound towards the wound where the FCU
is. Tunnel a clamp, i.e., Adson clamp, supercial to the
palmar aponeurosis
9. Using the clamp, or a suture passer, pass the FDS ring
into the palmar tunnel and out of the radial thumb wound
10. Weave the FDS ring into the APB insertion with at
least two weaves. Position the wrist in slight extension,
and the thumb in opposition, and then secure the transfer
at the estimated resting length of the FDS ring
11. Close all wounds, and apply a bulky, nonadherent
dressing and then a short arm thumb spica cast/splint in
the position of the transfer
adductorplasty. A common donor motor is the
extensor carpi radialis longus extended either the
use of a tendon graft or by prolonging its length
using a Z-plasty technique. The tendon is is
passed dorsally around the base of the index meta-
carpal and then volarly and transversely to the
thumb metacarpal.
For high median nerve decits, the various
impairments can be reconstructed using a variety
558 A. Peljovich and F. Fishman

of techniques. In these circumstances, the decits Table 13 Surgical pitfalls and prevention
could include loss of palmar grasp and loss or Median nerve injury
difculty with lateral pinch. Both grasp/pinch pat- Potential pitfalls and prevention
terns can be reconstructed provided sufcient Potential pitfalls Prevention
donor motors. Flexor pollicis longus function is Pitfall #1 Misalignment of Careful alignment via an
often restored using the brachioradialis as a donor. fascicles epineural repair using
supercial vascular
Index and long exor profundus function can be
markers or use of group
restored with either a side-side FDP ring/small fascicular repair
transfer, distal interphalangeal joint arthrodeses, Pitfall #2 Tension across Positioning of the limb to
or a combination. To restore nger exion, and repair site decrease tension (exion
thus palmar grasp, in high median nerve palsies, of the wrist and hand),
dissection or proximal and
the four FDP tendons are synchronized to each
distal tissue for improved
other with side to side transfers and then mobi- mobilization, use of
lized in concert by transfer of the ECRL. Forearm conduit if necessary
pronation rarely requires reconstruction, but if Pitfall #3 Assuming Explore early, high
needed, the BR can be passed around the radius absence of injury suspicion for injuries with
supracondylar fractures,
during transfer to the FPL to create a stronger lacerations, and
pronation moment; or, the biceps tendon can be penetrating injuries
re-routed round the radial neck and re-attached to Pitfall #4 Assuming nerve Utilize intraoperative
itself or the biceps tuberosity to yield a pronation in continuity is a nerve studies to assess
moment. functioning nerve nerve

Restoring Median Nerve Sensory

Function
A sensory impairment may also be associated in
the setting of a chronic median nerve injury. Such
a sensory decit can involve some or all of the
thumb, index, and long and radial half of the ring
nger. More proximal injuries will include a patch
of skin at the base of the thenar eminence, but it is
the sensory impairment to the thumb, index, and
perhaps long nger that is the most impairing.
Such a decit, if bothersome, will typically result
in avoidance by the child. If sensation cannot be
restored, such avoidance may be permanent. The
sensory organs for sensory nerves fortunately
remain viable for years after an injury, so even
children who present on a delayed basis can have
sensation restored by nerve repair/reconstruction
long after the muscles pass their time for
reinnervation.
A direct repair of the median nerve, with or
without grafting, is the rst choice in sensory
reinnervation, even if the injury occurred 12
years in the past. The other choice is to perform
a nerve transfer to restore part of the median
sensory function. The LABC has been utilized as
a donor sensory nerve for restoring critical median
sensation. The common digital nerve to the small/
ring has also been used to reinnervate the radial
digital nerve to the index and the ulnar digital
nerve to the thumb in an attempt to restore sensi-
bility to lateral pinch (Table 13 and 14).
Preferred Treatment
For acute closed injuries, an initial period of
observation is preferred. During this period of
time, associated injuries have time to heal, and
physical therapy may be initiated. In fact, ensur-
ing range of motion helps keep denervated muscle
from becoming brotic and maintains differential
gliding between the various muscles and nerves.
A baseline EMG/NCS is ordered if the nerve
injury continues for more than 6 weeks. This
study can help localize the site of injury and also
dene the electrical nature of the injury, i.e., focal
conduction block versus a more severe injury. The
ability to differentiate a conduction block from a
25 Median Nerve Injury
Table 14 Management of complications
Median nerve injury
Common
complication Management
Delayed presentation Thorough initial examination
from missed injury for injuries with high
association with median nerve
injury, small penetrating
injuries, lacerations
Infection Careful irrigation, preoperative
antibiotics
Secondary nerve Begin physiotherapy early
entrapment
Rupture of nerve Assess tension critically
repair intraoperatively, position arm
to surgeons advantage, utilize
graft or conduit if irreparable
without signicant tension
higher level of injury as well as identifying the
presence of median-ulnar nerve connection
requires an experienced and expert electrophysi-
ologist. It is preferable to communicate directly
with the consulted electrophysiologist prior to
testing. Depending upon the site of injury, the
time to when reinnervation would begin is esti-
mated, and a detailed examiniation and repeat
EMG/NCS is obtained (usually 46 months from
injury) assessing for improvement in the muscles
closest to the site of injury. If there is no evidence
of reinnervation, exploration of the nerve should
be undertaken. During surgery, intra-operative
nerve testing can be used if a nerve in continuity
is identied. If a nerve action potential across the
zone of injury is identied, neurolysis alone can
be performed, and a protective nerve wrap or
conduit can be utilized. Otherwise, the zone of
injury is resected and either repaired or grafted
depending upon the defect as discussed further
below (Murovic 2009). On the other hand, if the
repeat EMG/NCS suggests early reinnervation,
observation can be continued for an additional
23 months. Subsequent exploration can be
performed if there is absence of progressive clin-
ical recovery.
For acute open injuries in the forearm or wrist,
preferred treatment is based on the length of the
median nerve defect. For gaps less than 2 cm, a
primary epineural repair is preferred. The repair is
559
performed with appropriate gauged sutures
reinforced with brin glue. Any apparent segmen-
tal defect is rst treated with extended neurolysis
to mobilize the nerve, and the elbow can be posi-
tioned in up to 90100 of exion if this will
enable approximation the nerve ends for primary
repair. If wrist exion is avoidable, this is pre-
ferred. Persistent segmental defects up to 23 cm
are treated with autograft or allograft nerve
grafting, and defects between 3 and 6 cm are
treated with autologous nerve grafting. Nerve
conduits and wraps are used to protect a repair
and or/graft from surrounding tissue if there is a
high risk for postoperative scarring. At 610 cm
defects (Socolovsky et al. 2011), and for any
median nerve injury above the elbow, early
nerve transfer to the median nerve using branches
of the PIN can be considered for augmentation of
potential motor recovery, but the nerve should still
be reconstructed for sensory renervation
(Table 13).
For acute open injuries in the palm or digits,
the median nerve and/or its branches are treated
with primary epineural repair using similar tech-
niques as previously described. Defects 23 cm in
length are treated with a synthetic type 1 collagen
conduit, and defects greater than 3 cm in length
are treated with allograft.
For children presenting late, at 36 months
post injury without any clinical evidence of
nerve recovery, an EMG is obtained for closed
injuries and exploration undertaken if the initial
injury was open. For chronic injuries presenting
between 9 and 12 months from the time of injury,
nerve tranefer is considered depending upon the
location of injury, the results of an EMG/NCS,
and the estimated distance from the nerve injury to
the critical muscles. Children presenting between
12 and 16 months present an ambiguous dilemma
regarding the relative value of nerve tranefer ver-
sus tendon transfer. For children presenting with
chronic median nerve injury beyond 1618
months, tendon transfer surgery is performed to
restore the functional impairments as assessed.
All repairs, grafts, neurotizations, and
neurolyses are treated with early physiotherapy.
It is preferable to assess all repairs, grafts,
neurotizations, and neurolysis for tension and
560
differential soft tissue gliding at the time of sur-
gery. Such assessments help guide postoperative
physiotherapy, with early mobilization always the
goal. It is also preferable to employ sensory reed-
ucation techniques in the hopes of improving sen-
sory outcomes. A qualied physiotherapist is
always employed early in the postoperative period
and subsequently weaned as the child recovers
(Mavrogenis et al. 2009) (Table 14).
Summary
Median nerve injuries in children can result in
substantial functional impairment if they are not
addressed promptly and properly. Clinicians
should have a high level of suspicion for median
nerve injury in patients who sustain lacerations or
penetrating injuries in proximity to the anatomic
course of the median nerve. A low threshold for
early exploration is appropriate in patients who
demonstrate motor or sensory deciencies in the
distribution of the median nerve. If a tensionless
repair is not possible, the clinician should consider
nerve conduits and/or grafting. If the length of the
defect is 46 cm, nerve transfers for median nerve
function is a treatment option. For late presenta-
tions or failed nerve repair, tendon transfers can be
performed to help decrease the functional decits
left by the median nerve injury.
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 Radial Nerve Injury
26
Anjan P. Kaushik and Warren C. Hammert

Contents Abstract
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 563 Radial nerve palsy in the child can be attributed
Epidemiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 564 to a variety of traumatic causes, including
Relevant Pathoanatomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 564 humeral shaft, supracondylar humerus, and
Presentation and Symptoms . . . . . . . . . . . . . . . . . . . . . . . . . 564 Monteggia fractures, as well as congenital
Imaging and Diagnostic Studies . . . . . . . . . . . . . . . . . . . . . 566
and pathologic etiologies. Most pediatric inju-
Etiologies of Pediatric Radial Nerve Palsy . . . . . . . 566 ries to the radial nerve have a favorable prog-
Humeral Shaft Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 568
Supracondylar Humerus Fractures . . . . . . . . . . . . . . . . . . 570
nosis with observation, protective splinting,
Condylar Fractures and Elbow Dislocations . . . . . . . . 571 and therapy. Patients who fail to recover neu-
Monteggia Fracture-Dislocations and Forearm rologic function after 36 months, however,
Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 573 require surgical exploration for neurolysis,
Neonatal, Congenital, and Pathologic Causes . . . . . . . 575
Iatrogenic Causes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 576
nerve repair, or nerve grafting. Primary repair
has better outcomes than grafting or nerve
Treatment Options and Surgical Techniques . . . . . 577 transfers, provided that a well-vascularized tis-
Nonoperative Management . . . . . . . . . . . . . . . . . . . . . . . . . . 577
Nerve Exploration and Neurolysis or Primary sue bed is available and minimal tension is
Nerve Repair . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 578 placed on the repair.
Nerve Grafting . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 580
Nerve and Tendon Transfers . . . . . . . . . . . . . . . . . . . . . . . . . 582
Complications and Management . . . . . . . . . . . . . . . . . . . . 583
Introduction
Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 584
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 584 Pediatric trauma is considered the primary cause
of radial nerve palsy in children, not only from
acute fractures and dislocations of the humerus
and proximal forearm but also from chronic
sequelae of injuries around the elbow. Other eti-
Electronic supplementary material: The online version ologies include iatrogenic, congenital, and patho-
of this chapter (doi:10.1007/978-1-4614-8515-5_26) logic conditions. The prognosis for nerve
contains supplementary material, which is available recovery is generally favorable, particularly in
to authorized users. Videos can also be accessed at
younger age groups. This chapter aims to summa-
http://www.springerimages.com/videos/978-1-4614-8513-1.
rize the medical literature focused on etiologies of
A.P. Kaushik W.C. Hammert (*)
pediatric radial nerve palsy and also describe the
Department of Orthopaedic Surgery, University of
Rochester Medical Center, Rochester, NY, USA various nonoperative and surgical treatments for
e-mail: warren_hammert@urmc.rochester.edu this pathology.
# Springer Science+Business Media New York 2015 563
J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_26
564
Epidemiology
Nerve injuries are more common following upper
extremity fractures than those involving the lower
extremity, occurring in 2.5 % of pediatric extrem-
ity fractures (Omer 1974; Noble et al. 1998). The
most common causes are humerus fractures in the
supracondylar region or shaft, and the most com-
monly injured nerve is the radial nerve, account-
ing for 58 of 162 nerve injuries in a mixed adult
and pediatric trauma population of 5,777 patients
(Noble et al. 1998). The incidence of radial nerve
palsy in humeral shaft fractures is between 12 %
(Shao et al. 2005) and 18 %, with spontaneous
recovery in up to 90 % of these injuries (Hosalkar
et al. 2006). Other fractures around the elbow,
particularly Monteggia fracture-dislocations and
humeral condyle fractures, are also associated
with radial nerve injury (Galbraith and
McCullough 1979).
Injury mechanisms include motor vehicle
trauma, falls from bicycles or playground injuries,
motorcycle and recreational vehicle trauma, gun
violence, assault, and child abuse. Noble
et al. reported an association of peripheral nerve
injuries with head injuries of 60 %, as well as a
high incidence of pelvic and other limb fractures.
Males are 1.55 times more likely to sustain a
nerve injury than females (Hosalkar et al. 2006;
Noble et al. 1998).
Other congenital and pathologic causes of
radial nerve palsy are less common. Neonatal
brachial plexus palsy is reported to occur in
0.42.5 cases per 1,000 births, and isolated palsy
of the radial nerve is even less frequent within this
group, with only a few dozen cases in the litera-
ture (Hayman et al. 1999). One report, however,
suggests that isolated radial nerve injury in new-
borns may be underrecognized or misdiagnosed,
with an incidence of 2.6 % or more (Alsubhi
et al. 2011).
Relevant Pathoanatomy
The radial nerve, a terminal branch of the poste-
rior cord of the brachial plexus, receives contribu-
tions from all nerve roots from C5 to T1. As it
A.P. Kaushik and W.C. Hammert
exits the axilla, it gives off sensory branches to the
posterior arm and innervates the three heads of
triceps and anconeus. The radial nerve travels in a
medial to lateral direction xed within the poste-
rior spiral groove of the humeral shaft (Fig. 1).
This location leaves the nerve vulnerable to con-
tusion or transection in diaphyseal humerus frac-
tures, when it experiences angular deformity and
traction. After it exits the spiral groove 1012 cm
above the lateral epicondyle, the radial nerve
pierces the lateral intermuscular septum and
gives muscular branches to brachioradialis and
extensor carpi radialis longus (Waters and Bae
2012).
As it passes over the anterolateral capsule of
the radiocapitellar joint and radial head, the radial
nerve divides into a supercial branch (SBRN)
and a deep branch, which eventually becomes
the posterior interosseous nerve (PIN) (Fig. 2).
This cutaneous location over the radial head
can leave the radial nerve susceptible to
injury from radial head dislocation. SBRN con-
tinues deep to brachioradialis muscle and
innervates the radial base of the thumb and
dorsal hand. PIN continues through the brous
edge of supinator (known as the arcade of
Frohse, the most common site of radial nerve
compression) and pierces supinator muscle, even-
tually supplying the extensor muscles of the
wrist and hand (Waters and Bae 2012), as depicted
in Fig. 1.
Presentation and Symptoms
Clinically, children with radial nerve palsy present
with decreased use of the affected arm, wrist drop
with weakness and/or loss of nger extension, and
sensory loss (Fig. 3). They may also have defor-
mity and swelling if a fracture is present. In
patients with humeral shaft fractures, distal
motor and sensory function will be lost, and
some weakness of supination may be present,
but elbow extension is spared, as innervation to
triceps occurs proximal to the spiral groove
(Hosalkar et al. 2006). In patients with axillary
compression of the radial nerve, there will also be
weakness of elbow extension and more proximal
26 Radial Nerve Injury 565

Fig. 1 Radial nerve

anatomy and muscle
innervation (Courtesy of
Shriners Hospital for
Children, Philadelphia)
(Copyright Dan
A. Zlotolow)

Monteggia fractures and elbow dislocations. PIN

Fig. 2 The radial nerve passes over the anterolateral cap-
sule of the radiocapitellar joint and divides into a super-
cial branch (SBRN) and a deep posterior interosseous
nerve (PIN) (Courtesy of Shriners Hospital for Children,
Philadelphia)
sensory loss, in addition to the distal decits
(Waters and Bae 2012).
Isolated PIN palsy can occur from injury to the
radial head and proximal radius and ulna, as in
can become entrapped within supinator muscle
under the arcade of Frohse, resulting in loss of
motor function (nger and thumb extension) with-
out sensory involvement. Because the
brachioradialis and extensor carpi radialis longus
muscles are spared, the wrist will deviate radially
upon attempted wrist extension (Waters and Bae
2012; Spinner et al. 1968).
Diagnosis of nerve injuries and specic muscle
testing can be challenging in children. They may
be agitated or unable to follow commands for
sensory and motor testing and may simply not
comprehend the meaning of tingling and numb-
ness (Hosalkar et al. 2006). This can delay accu-
rate diagnosis. It is important to assess all major
nerves radial, ulnar, and median individually
in order to determine motor and sensory decits.
In the older child, two-point discrimination should
be tested with the childs eyes closed, and
vibrometry and Semmes-Weinstein monolament
exam can be used to test vibration and pressure
thresholds, respectively (Hosalkar et al. 2006). In
children with closed head injury and upper
extremity fractures who are obtunded or coma-
tose, initial fracture splinting and serial
566
Fig. 3 Seven-year-old status post left humerus fracture
with high radial nerve palsy (Courtesy of Shriners Hospital
for Children, Philadelphia). (a) Loss of wrist and digital
neurovascular examination is necessary to char-
acterize potential nerve injury.
Imaging and Diagnostic Studies
In traumatic situations, radiographic imaging of
the entire bone, including the joints above and
below the fracture, is recommended. It is impor-
tant to rule out ipsilateral limb fractures or a oat-
ing elbow, such as a concomitant proximal radius
and ulna fracture with a humerus fracture. Elec-
tromyography (EMG) and nerve conduction
velocity (NCV) studies can be useful both as
diagnostic and as surveillance instruments for
radial nerve injury (Hosalkar et al. 2006). Neuro-
physiologic NCV testing can help identify the
nerve branches that have been affected and, com-
bined with distal muscle response to EMG stimu-
lation, can help determine if signal transmission
can occur along a nerve. NCV differentiates
demyelination from axonal loss, and EMG distin-
guishes between neuropraxia versus axonotmesis,
or neurotmesis. When the electrical activity infor-
mation from these needle electrodes is pooled,
pathology can be stratied into conduction
slowing, demyelinating conduction block, or fail-
ure of conduction (Hosalkar et al. 2006).
Early EMG/NCV testing done as a baseline
within 10 days of injury can be used when nerve
continuity is unknown, to determine if the nerve is
A.P. Kaushik and W.C. Hammert
extension. (b) Passive extension of the wrist and attempted
active nger extension produces no metacarpophalangeal
joint extension
transected or contused, which may demonstrate
intact but slowed conduction. Young children
often have difculty tolerating needle studies, so
anxiolytic medication or conscious sedation may
be utilized. A comparison electrodiagnostic study
can be repeated 6 weeks after injury to monitor the
progress of radial nerve recovery and plan surgical
intervention (Hosalkar et al. 2006).
Etiologies of Pediatric Radial Nerve
Palsy
Traumatic injuries are the most common causes of
radial nerve palsy in children. These include acute
fractures of the humeral shaft, supracondylar
humerus, and distal humeral condyles, as well as
elbow dislocations and proximal ulna Monteggia
fractures with associated radial head dislocations.
These injuries are diagnosed on radiographic
imaging and radial nerve injury identied on care-
ful neurologic examination. Unfortunately, some
injuries are missed, such as chronic radial head
dislocation and elbow instability, leading to
delayed diagnosis and treatment of nerve palsy.
Occasionally iatrogenic damage is a cause of
radial nerve injury, for example, in the percutane-
ous treatment of supracondylar and condylar frac-
tures, as well as in correctional osteotomies of the
ulna or humerus (Fig. 4). Tumors, vascular
malformations, and congenital or newborn
26 Radial Nerve Injury
Fig. 4 Four-year-old status post open reduction and inter-
nal xation of a lateral condyle fracture (Courtesy of
Shriners Hospital for Children, Philadelphia). (a) High
radial nerve palsy with loss of wrist and digital extension.
(b) Lateral exploration with nerve discontinuity along with
pathologies are also rare etiologies that are
encountered (Fig. 5). The literature review below
summarizes the outcomes of both operative and
567
large proximal and distal neuromas. (c) Proximal nerve
resected until good viable axons. (d) Distal nerve resected
to good distal axons. (e) Long defect between proximal and
distal stumps. (f) Sural nerve cable grafting
nonsurgical management of these diverse etiolo-
gies of radial nerve palsy. Traumatic causes are
discussed anatomically from proximal to distal.
568
Fig. 5 Ten-year-old with progressive loss of nger and
thumb extension (Courtesy of Shriners Hospital for Chil-
dren, Philadelphia). (a) Video examination with intact
wrist extension, but no extrinsic thumb or nger extension.
Humeral Shaft Fractures
Fracture of the humeral diaphysis is the most
common long bone fracture associated with
nerve palsy, and the radial nerve is at highest
risk, particularly in transverse or spiral fractures
of the middle to distal third of the humeral shaft
(Shao et al. 2005). Most radial nerve palsies
associated with this fracture are from stretch or
contusion and do not require acute exploration.
However, early surgical exploration is warranted
for open humeral shaft fractures and should be
considered when nerve palsy occurs after a
reduction attempt (Hosalkar et al. 2006).
The Holstein-Lewis fracture, a distal third
spiral oblique diaphyseal humerus fracture, has a
A.P. Kaushik and W.C. Hammert
(b) MRI reveals lesion within the posterior interosseous
nerve. (c) Surgical exploration with large Schwannoma
inltrating interosseous nerve. Radial sensory nerve is
spared (red vessel loop)
higher incidence of radial nerve injury, 22 % com-
pared to 8 % for other shaft fractures (Ekholm
et al. 2008).
There has been considerable controversy over
the last few decades regarding expectant versus
early surgical management of radial nerve injury
in these fractures, but a systematic review by Shao
et al. found no statistically signicant difference
in the rate of nerve recovery with nonoperative
and operative treatment (Shao et al. 2005).
Although they found that the total number of
pediatric patients with humerus fractures was
small within the 35 papers included in the study,
they recommended a conservative approach to the
early management of radial nerve palsy in these
fractures for both adult and pediatric patients
26 Radial Nerve Injury 569

Fig. 6 Algorithm for Mulple injuries

Closed Injury Open Injury
management of radial nerve Segmental fracture
palsy caused by humerus Floang elbow
fracture (Courtesy of Major vascular
Shriners Hospital for injury
Observe
Children, Philadelphia) Coma
(Copyright Dan
A. Zlotolow)
Ultrasound within 3 Nerve Fracture immobilizaon
weeks, if possible Exploraon Vascular repair, etc.

Intact Severed
Entrapped
contused Parally severed

Surgical Injury extent Surgical repair

Observe Extricaon
repair indisnguishable impossible

Calculated waing me interval Mark the nerve

Second exploraon
in 2 to 3 months

Improved Unimproved

Tendon transfer aer

fracture healed
No surgical EMG, NAP, NCV
intervenon before exploraon

(Shao et al. 2005). Deferring exploration up to
6 months had no effect on functional outcome,
and thus, it is reasonable to delay exploration 24
months following injury to observe for early signs
of recovery prior to exploration, since 71 % of
patients treated nonoperatively recovered sponta-
neously, and overall recovery has been reported at
88 % (Shao et al. 2005). The authors suggested a
treatment algorithm for radial nerve palsy in
humeral shaft fractures, which is shown in Fig. 6.
As children are becoming increasingly
involved in competitive sports at an earlier age,
unique fracture patterns have emerged. A spiral
fracture of the humeral shaft with a high rate of
radial nerve palsy has been described in throwing
athletes. Ogawa and Yoshida (1998) analyzed
90 patients, 9 of which were children, who
sustained these humeral fractures from throwing
motions that occurred during recreational
baseball. 16 % of all patients had radial nerve
palsy, all of which recovered within 8 months.
The injury usually occurred during the accelera-
tion phase in an overhand or three-quarter over-
hand throw, which resulted in an external
rotational spiral fracture of humeral shaft. Patients
less than 15 years old were more likely to have
fractures in the proximal half of the diaphysis.
Approximately half of the patients were treated
nonoperatively with a functional brace and the
other half with surgical plate xation without
any nerve procedures, and all patients had full
functional recovery (Ogawa and Yoshida 1998).
The literature has limited information speci-
cally regarding the surgical management of radial
nerve injury from humeral shaft fractures in chil-
dren. A case report by Ogawa et al. (2007)
described successful nerve grafting for a 3-year-
old child with a closed humerus fracture resulting
570
in radial nerve palsy. After EMG demonstrated no
recovery of function 3 months post-injury, they
explored the fracture site and noted complete tran-
section of the radial nerve with substantial retrac-
tion of the proximal end. Cable grafting with 8 cm
of sural nerve autograft resulted in successful
recovery of radial nerve function at 6 months
(Ogawa et al. 2007).
Amillo et al. (1993) reported on 12 patients
with radial nerve palsy treated surgically, 4 of
which were pediatric patients. All four children
presented with complete motor and sensory def-
icits, conrmed on EMG at 3 months post-injury.
Two of the four underwent simple neurolysis for
brosis around the radial nerve, one had sural
nerve interfascicular grafting for callus entrap-
ment, and one had tendon transfers because the
nerve had been divided during the injury. Three
had excellent motor and sensory recovery, and
the child who underwent tendon transfers had
good motor function with limited sensation. The
authors emphasized the importance of surgical
exploration if there are no signs of nerve recovery
3 months post-injury, and preoperative EMG
offers useful information about the extent of
nerve damage (Amillo et al. 1993). A similar
study by Larsen and Barfred (2000) evaluated
26 patients with a median age of 21 who had
radial nerve palsy after humeral shaft and
supracondylar humerus fractures. They reported
good nerve recovery within 45 months with
expectant management for 21 patients, and for
the 5 patients who showed no recovery, surgical
exploration, sural nerve grafting, and tendon
transfers also resulted in favorable outcomes
(Larsen and Barfred 2000).
Supracondylar Humerus Fractures
Neurologic injuries from supracondylar humerus
fractures in children are common, with rates rang-
ing from 5 % to 22 % (Culp et al. 1990; Dormans
et al. 1995; Louahem et al. 2006; Babal
et al. 2010). Lesions can be seen in the ulnar,
radial, median, anterior interosseous, and multiple
nerves. Although older studies suggested the
radial nerve was most commonly injured, recent
A.P. Kaushik and W.C. Hammert
meta-analyses have found that the anterior
interosseous nerve is most frequently affected
(34 %) in extension type and the ulnar nerve
(91 %) in exion-type fractures (Babal
et al. 2010). Iatrogenic nerve injuries also occur
in 23 % of patients during the treatment of these
fractures (Gosens and Bongers 2003; Babal
et al. 2010; Royce et al. 1991). Similar to radial
nerve injuries associated with humeral shaft frac-
tures, most radial nerve palsies recover spontane-
ously following closed supracondylar fractures,
so clinical observation is recommended. If no
recovery is seen after a period of expectant man-
agement, surgical options include exploration and
neurolysis, nerve grafting, and nerve or tendon
transfers. Again, nerve traction and contusion are
more common than complete disruption
(Louahem et al. 2006; Gosens and Bongers
2003; Galbraith and McCullough 1979).
Radial nerve injury has been reported in 641 %
of supracondylar humerus fractures (Louahem
et al. 2006; Babal et al. 2010). Posteromedial
fracture displacement is more likely to result in
neurologic injury (Hosalkar et al. 2006).
Culp et al. (1990) retrospectively evaluated six
patients with radial nerve palsy in a series of
101 consecutive supracondylar fractures. They
found that four children recovered spontaneously
within 25 months and one child required
neurolysis, after which he recovered in 6 months.
The nal patient, who had a transected nerve, did
not recover function despite sural nerve grafting
and required tendon transfers (Culp et al. 1990).
When combined with their data for median and
ulnar nerve postsurgical outcomes, they concluded
that neurolysis after a 5-month period of observa-
tion offered good results for recovery of nerve
function in these injuries. Dormans et al. (1995)
found comparable outcomes in their series of
200 Gartland extension type III supracondylar
humerus fractures. They reported ve radial nerve
injuries, all of which recovered without surgery
within 4 months (Dormans et al. 1995). In the series
of 617 patients studied by Gosens and Bongers
(2003), 10 radial nerve injuries were identied.
Nine of these recovered spontaneously after closed
or open reduction and pinning of the supracondylar
fracture and one patient required radial nerve repair
26 Radial Nerve Injury
with an interposition autograft (Gosens and
Bongers 2003). Three of these patients also had
concomitant brachial artery injury, so it is important
to monitor vascular status during the evaluation of
these patients. Recent studies have emphasized the
fact that incidence of vascular and neurologic
injury increase with severity of Gartland classica-
tion of fracture displacement (Tomaszewski
et al. 2012; Gosens and Bongers 2003). Another
study from France involving 210 children with
supracondylar fractures reported a high rate of neu-
rologic injury (22 %), including 13 radial nerve
palsies (Louahem et al. 2006). Prognosis is good
for spontaneous functional improvement, so they
encouraged a more conservative strategy. The rare
complete transections, however, require repair or
nerve grafting.
Another potential cause of radial and other
nerve injuries in supracondylar humerus fractures
is iatrogenic damage during fracture stabilization.
Flynn et al. (1974) published a 16-year follow-up
study that described a blind percutaneous pinning
technique with serial intraoperative radiographs
used to treat 72 children with displaced
supracondylar fractures. Vascular complications
occurred in 18 % of these cases and neurologic
complications in 11 %, including two patients
with radial nerve palsy who recovered spontane-
ously (Flynn et al. 1974). Since then, as uoro-
scopic imaging technology has improved live
fracture visualization, multiple studies have
described iatrogenic complications from pinning
of supracondylar fractures. Royce et al. (1991)
reported four cases of iatrogenic nerve injury,
including three ulnar nerve palsies and one radial
nerve palsy. Injury to the radial nerve in this
patient occurred during placement of the medial
Kirschner wire. After starting the pin in the medial
epicondyle, penetration of the pin through the
lateral cortex and plunging resulted in complete
radial nerve palsy post-op. The nerve was
explored on the same day and found to be in
continuity but contused, with functional return
occurring over 3 months (Royce et al. 1991). A
similar report of iatrogenic radial nerve palsy in
2012 described the exact moment when radial
nerve injury occurred in one patient, when the
medial K-wire exited the lateral humeral cortex
571
and the patients wrist jerked into extension
(Krusche-Mandl et al. 2012). Although the patient
had a postoperative wrist drop, function returned
in 13 weeks. Radial nerve palsy, in addition to
ulnar nerve injury, has also been described during
open reduction and pinning of supracondylar frac-
tures (Brown and Zinar 1995). Medial pins started
too posteriorly on the medial epicondyle can
affect the ulnar nerve, and again, lateral cortical
penetration can be injurious to the radial nerve
(Fig. 7).
Current teaching for placement technique of
the medial K-wire includes making a small inci-
sion along the medial epicondyle to openly retract
the ulnar nerve, as well as to use uoroscopic
imaging to avoid plunging through the
lateral humeral cortex, which can wrap up or add
traction to the radial nerve (Brown and Zinar
1995; Gosens and Bongers 2003; Royce
et al. 1991). Although percutaneous pinning
of supracondylar fractures is a relatively safe,
effective technique, surgeons must be aware
of the 13 % risk of iatrogenic nerve injury
(Gosens and Bongers 2003; Babal et al. 2010;
Royce et al. 1991; Krusche-Mandl et al. 2012;
Brown and Zinar 1995) and be careful in their
operative technique.
Condylar Fractures and Elbow
Dislocations
Dislocations of the elbow and fractures of the
lateral and medial humeral condyle have been
reported as less common causes of radial nerve
palsy. PIN palsy was rst reported in 1936 in a
patient after acute lateral condyle fracture (Wilson
1936), and transient paralysis of the radial nerve
was again described a decade later (McDonnell
and Wilson 1948). Another study presented two
patients with PIN palsy after open treatment of
lateral condyle fractures (Smith and Joyce 1954).
Although tardy ulnar neuropathy is a more com-
mon nerve injury that can occur from lateral con-
dyle nonunion resulting in elbow deformity,
clinicians must also evaluate the radial nerve in
both nonoperative and surgical management of
these fractures (Hosalkar et al. 2006).
572
Fig. 7 Twelve-year-old female level 7 gymnast 5 months
following open reduction and internal xation of right
medial epicondyle fracture associated with an elbow dis-
location (Courtesy of Shriners Hospital for Children, Phil-
adelphia). (a) Clinical examination with absent wrist and
Medial epicondyle fractures can occur in con-
junction with elbow dislocations and may be asso-
ciated with ulnar nerve palsy, but surgical
treatment of these fractures may result in radial
nerve injury. Marcu et al. (2011) describe two
patients who underwent open reduction and
A.P. Kaushik and W.C. Hammert
digital extension. (b) X-ray showing xation with 3.5 mm
cannulated screw. (c) Exploration revealed neuroma
incontinuity. (d) Sural nerve cable grafting. (e) Video dem-
onstrating excellent outcome
cannulated screw xation for medial epicondyle
fractures. In the rst patient, retrograde screw
placement was done through a medial approach,
and care was taken not to pass-point the terminally
threaded guide wire beyond the lateral cortex of
the humerus, but unrecognized advancement may
26 Radial Nerve Injury
Fig. 8 Lateral x-ray
demonstrating anterior
dislocation of the radial
head. This condition can
result in injury to the PIN as
it travels through the
supinator muscle at the
arcade of Frohse (courtesy
of Shriners Hospital for
Children, Philadelphia)
have occurred. The patient had radial nerve palsy
postoperatively despite an uneventful procedure,
and she underwent screw removal and nerve
grafting, eventually recovering full function at
9 months. Similar xation was completed in the
second patient, but intraoperative advancement of
the guide pin occurred laterally, and twitching was
noted within the mobile wad. She had spontane-
ous recovery of wrist extension at 3 months
(Marcu et al. 2011). The authors emphasize the
dangers of cannulated screw xation of fractures,
including unrecognized guide pin advancement and
problems with the pin such as bending or breakage.
They suggest some reasons that can be attributed to
radial nerve injuries and offer techniques to avoid
these problems. The use of terminally threaded pins,
which make it more difcult to feel when the pin
has breached a cortex, can be substituted with
smooth pins, which also cause less soft tissue dam-
age. Another problem with cannulated systems is
that the drill can advance a pin unintentionally while
spinning over the pin. More generous use of live
uoroscopy to avoid unwanted advancement has
been recommended (Marcu et al. 2011). These
challenges and technique suggestions are presented
to avoid radial nerve palsy as a complication.
Other rare injuries can also lead to radial nerve
injury. Abu-Jayyab et al. (2011) published a case
report of an 8-year-old child with combined lateral
condyle and medial epicondyle fractures with tran-
section of the radial nerve. She underwent radial
nerve primary repair and recovered full nerve func-
tion in 6 months (Abu-Jayyab et al. 2011).
573
Monteggia Fracture-Dislocations
and Forearm Fractures
Monteggia fractures of the proximal ulna with
radial head dislocation have frequently been asso-
ciated with radial nerve and PIN palsy in children,
even since Bado classied the lesion in 1962, with
up to 43 % of patients with these fractures having
neurologic injury (Jessing 1975). As the PIN
travels through the supinator muscle adjacent to
the radial head and neck, it is susceptible to injury
with anterior or lateral radial head dislocation and
often gets entrapped at the arcade of Frohse
(Fig. 8; Spinner et al. 1968). If a radial head is
found to be irreducible, the radial nerve may in
fact be entrapped beneath it or wrapped around the
radial neck, and open reduction using an anterior
approach is necessary (Morris 1974). Direct nerve
trauma and traction neuropraxia are both causes of
palsy (Ruchelsman et al. 2009; Stein et al. 1971).
Return of nerve function after closed reduction of
Monteggia fracture-dislocations can either be
acute within a few days or delayed, as reported
by Spinner et al. in three children (1968). Two of
the patients had full motor function within 14 days
after the fracture; however, the other patient took
5 months to regain all function (Spinner
et al. 1968).
Wiley and Galey (1985) published a series of
46 children with Monteggia fractures, of which
4 had either radial nerve or PIN palsy. 48 % of
these children sustained anterior radial head dis-
location (Bado I), 39 % had lateral dislocation
574
(Bado III), 11 % had posterior dislocation
(Bado II), and 2 % had type IV injury with fracture
of the proximal radius. Notably, 41 % of patients
had olecranon fractures. The fractures were
treated with closed or open reduction and pinning,
but none of the nerve palsies required further
surgery and recovered spontaneously.
A favorable prognosis is seen for most children
following closed reduction (Wiley and Galey
1985; Hosalkar et al. 2006), while adults require
advanced surgical intervention more frequently
(Jessing 1975). Stein et al. (1971) presented
seven adult soldiers who sustained Monteggia
fractures with PIN and radial nerve palsy, six of
which were explored operatively and three of
which required tendon transfers secondary to fail-
ure of nerve recovery. They also noted an associ-
ation with ulnar nerve injury (Stein et al. 1971).
Malunion of a Monteggia fracture that occurs
at a young age can lead to acute or tardy progres-
sive radial nerve palsy, with one case reported
65 years after the initial injury, demonstrating
the importance of early recognition and treatment
of this fracture-dislocation (Austin 1976; Holst-
Nielsen and Jensen 1984). Redislocation of the
radial head after closed reduction can occur with
cast immobilization, so serial radiographic
follow-up is required. Ulnar malunion and persis-
tent radial head dislocation can lead to symptom-
atic PIN palsy in children as well, which can be
treated with corrective osteotomy and neurolysis
of the chronically compressed nerve. Ruchelsman
et al. (2009) reported a surgical technique for
management of PIN palsy in a child after chronic
type I Monteggia fracture malunion with persis-
tent anterior radial head dislocation. The patient
had persistent motor weakness 9 months after
injury and was treated with surgical exploration,
where the radial head was found to be dislocated
between the supercial radial nerve and PIN.
Considerable epineural brosis was seen at the
arcade of Frohse, and microsurgical neurolysis
was completed. After extensive release of the
scarred capsule of the proximal radioulnar joint,
an osteotomy and plating of the proximal ulna was
performed, followed by open reduction of the
radial head and annular ligament reconstruction
with ulnar periosteum. Radiocapitellar pinning
A.P. Kaushik and W.C. Hammert
was used to stabilize the reduction. The child
had full restoration of PIN function within a year
after neurolysis and reconstruction (Ruchelsman
et al. 2009). Based on this, the authors favor
simultaneous treatment of the malunion and
nerve compression in order to avoid later tendon
transfers.
In another case series of 13 children, Wang and
Chang (2006) report the outcomes of a similar
procedure consisting of open reduction and ulnar
osteotomy to treat chronic anterior radial head
dislocation. Annular ligament reconstruction was
also performed. The authors found that successful
reconstruction of chronic Monteggia fracture-
dislocations is possible but can have complica-
tions of iatrogenic transient PIN palsy,
redislocation, and delayed union of the osteotomy
(Wang and Chang 2006). One pitfall to discuss is
the complication of iatrogenic PIN palsy that can
occur if corrective ulnar osteotomy is completed
without an open reduction of the radiocapitellar
joint. A case report presented a 6-year-old treated
with osteotomy without exposure of the radial
head (Osamura et al. 2004). The blind reduction
of the radiocapitellar joint resulted in transection
of the PIN. The patient had no recovery of motor
function and, on exploration, the distal nerve
stump could not be identied, so tendon transfers
were performed (Osamura et al. 2004). This
emphasizes the importance of diligent neurologic
examination and surgical exposure in the care of
children with this fracture pattern and radial nerve
injury.
In adults, good outcomes are reported with
simple neurolysis alone rather than ulnar
osteotomy and ligament reconstruction for tardy
PIN palsy in patients with chronic radial head
dislocation (Holst-Nielsen and Jensen 1984). In
children, however, restoration of anatomic rela-
tionships with reconstruction is preferred to allow
normal long-term function.
Other forearm injuries, including diaphyseal
radius and ulnar fractures and Galeazzi fractures
of the radius with distal radioulnar joint disloca-
tion, have also been associated with PIN palsy, but
the incidence is lower secondary to the larger soft
tissue envelope relative to the elbow (Hosalkar
et al. 2006; Moore et al. 1985). Tubbs
26 Radial Nerve Injury
et al. (2013) presented a child who sustained a
proximal radial shaft fracture malunion, which
resulted in recurrent radial head dislocation and
compression of the PIN at the arcade of Frohse.
Exploration and nerve decompression led to full
recovery of PIN function in 5 months (Tubbs
et al. 2013). Injury to the posterior interosseous
nerve occurs more often during open reduction
and xation of Galeazzi and both bone forearm
fractures. Moore et al. (1985) described a large
series of patients with open plating of Galeazzi
fractures with an incidence of 8.5 % for iatrogenic
injury, in which 8 % of subjects were children
(Moore et al. 1985). Median and ulnar nerve
injury or compression is more common than
supercial radial nerve injury in more distal radius
and ulnar fractures.
Neonatal, Congenital, and Pathologic
Causes
Newborn radial nerve palsy is a rare entity, but a
variety of causes can lead to infant wrist drop,
including intrauterine positioning (Alsubhi
et al. 2011), maternal anatomy during vaginal
delivery (Morgan 1948; Alsubhi et al. 2011),
reduced fetal activity from reduced amniotic
uid volume (Lundy et al. 2009), obstetric
humerus fracture (Morgan 1948), constriction
bands (Uchida and Sugioka 1991), infection
(Lejman et al. 1995), tumors, malformations, and
lesions of the spinal cord. Treatment of these
palsies after birth includes splinting the arm with
the wrist and ngers in extension, and therapy to
encourage wrist motion. Fortunately, most new-
borns recover spontaneously from these in utero
or birth injuries to the radial nerve.
Prolonged intrauterine hyperexion of the wrist
with persistent pressure on the exed hand can
result in wrist drop at birth. Cases of bilateral radial
nerve palsy have also been reported, where
prolonged vaginal delivery results in tetanic uterine
spasm that creates a contraction ring of the cervix,
thereby compressing the radial nerves at the level
of the humeri or elbows (Morgan 1948). An abnor-
mal uterine obstruction during labor, known as
Bandls ring, can also have a similar effect.
575
Neonates with isolated radial nerve palsy are
expected to recover without surgical intervention.
Hayman et al. (1999) described four children who
had complete recovery of radial nerve paresis
within 1 month. They demonstrated EMG/NCV
evidence of acute denervation and later
reinnervation and also did skin biopsies of
contused areas in the upper arms of these infants,
which revealed subcutaneous fat necrosis
(Hayman et al. 1999). A larger case series by
Alsubhi et al. (2011) presented 25 children with
isolated radial nerve palsy after delivery, mounting
to an incidence of 2.6 % in a sample size of
953 infants. This isolated palsy may in fact be
underdiagnosed or misdiagnosed, so clinical suspi-
cion should be maintained. Radial nerve palsy was
differentiated from obstetric brachial plexus palsy,
which also has shoulder dysfunction and loss of
elbow exion. Seventeen of the 25 affected new-
borns were noted clinically to have bruising (28 %)
or a rm subcutaneous nodule of fat necrosis (68 %)
in the posterolateral aspect of the arm (Alsubhi
et al. 2011). The major perinatal risk factor for this
injury was prolonged labor or failure of labor to
progress, usually requiring instrumented vaginal
delivery or C-section. Protracted pressure on the
arm or elbow by the pelvic brim was found to be
the source of compression. All of the infants recov-
ered full radial nerve function within 6 months,
mostly before 2 months, so isolated neonatal radial
nerve palsy has a much more favorable prognosis
than lower brachial plexus palsy (Alsubhi
et al. 2011; Lundy 2011; Hayman et al. 1999).
Congenital constriction bands are also known
to cause isolated or multiple nerve palsies of the
upper extremity, and these usually require early
surgery for deep soft tissue releases, Z-plasties,
and nerve decompression or grafting (Fig. 9;
Uchida and Sugioka 1991; Weinzweig and Barr
1994). Intraoperative ndings of the extent of
nerve compression may not match preoperative
clinical examination or EMG ndings, but com-
plete decompression with one-stage or multistage
releases is recommended (Weinzweig and Barr
1994). Sclerema neonatorum, a potentially lethal
condition, is a neonatal pathology with diffuse
subcutaneous fat necrosis that can present with
radial nerve palsy (Coppotelli et al. 1979).
576
Fig. 9 One-year-old male
with amniotic band around
the left arm resulting in
radial nerve palsy (Courtesy
of Shriners Hospital for
Children, Philadelphia)
Traumatic fractures of the limb at birth can also
cause radial nerve palsy, as newborns with humeral
shaft fractures can present with wrist drop (Morgan
1948). Fracture-related palsies usually recover
without surgery. Infection has also been reported
as an etiology of radial nerve palsy, when septic
arthritis of the shoulder in premature infants can
cause compression of the radial nerve near the
triangular interval of the posterior shoulder. The
four children reported in a case series of shoulder
infections recovered by 5 weeks after treatment of
the infection (Lejman et al. 1995). Other potential
causes of radial nerve or PIN palsy in infants and
young children include self-limiting infantile corti-
cal hyperostosis (Caffey disease) (Finsterbush and
Husseini 1979), nerve tumors such as intraneural
perineurioma that require excision and nerve
grafting (Isaac et al. 2004), and vascular
malformations (Regan et al. 1991). Prompt recog-
nition of these conditions can initiate appropriate
workup and treatment.
Iatrogenic causes of radial nerve injury in chil-
dren include prolonged use of a blood pressure cuff
when the cuff is placed too close to the elbow joint
(Tollner et al. 1980), as well as intramuscular injec-
tions (Gaur and Swarup 1996; Pandian et al. 2006;
Finsterbush and Husseini 1979). Injection-related
palsy was associated with administration by inex-
perienced or uncertied medical practitioners and
had a poor prognosis, with only 28 % of patients
having nerve recovery (Pandian et al. 2006).
Appropriate education of health care workers can
help avoid such nerve injuries.
A.P. Kaushik and W.C. Hammert
Iatrogenic Causes
Several iatrogenic causes of radial nerve palsy in
children have been discussed above, including
closed reduction of humeral shaft fractures
(Hosalkar et al. 2006), pinning of supracondylar
humerus (Babal et al. 2010; Royce et al. 1991;
Brown and Zinar 1995) and medial epicondyle
fractures (Marcu et al. 2011), treatment of
Monteggia and Galeazzi fractures
(Osamura et al. 2004; Moore et al. 1985), and
nontraumatic causes (Pandian et al. 2006; Tollner
et al. 1980).
Surgeries for correction of chronic humeral or
ulnar deformities have occasionally resulted in
iatrogenic radial nerve palsy. Lengthening of the
humerus with distraction osteogenesis has the
potential for injury to the radial nerve secondary
to traction on the nerve. Etiologies for humeral
growth arrest and shortening include congenital
defect, infection, trauma, multiple hereditary
exostoses, bone cysts, and radiation therapy. Lee
et al. (2005) reported three cases of radial nerve
palsy in 15 children who underwent the lengthen-
ing procedure. They were found to have transient
neuropraxia, with full recovery of wrist and nger
extension between 1 week and 6 months (Lee
et al. 2005). Injury to the nerve can occur during
the osteotomy or placement of the distraction
device pins, but may also happen in the weeks
afterwards, during the distraction process or
manipulation of the osteotomy site. Ilizarov tech-
nique can also be used for humeral lengthening
26 Radial Nerve Injury
and has a similar risk of transient radial nerve
palsy (Yang and Huang 1997). If palsy develops
during the lengthening period, the surgeon
may choose to decrease the rate of distraction
until the paresis resolves. Overall the authors
found humeral lengthening to be a successful
procedure, allowing children to have better self-
image as well as functional ability and perfor-
mance in sports activities (Lee et al. 2005; Yang
and Huang 1997).
Ulnar osteotomies for Monteggia fracture
malunion or chronic radial head dislocation, as
detailed above, can result in radial nerve palsy or
transection if a closed reduction of the radial head
is completed without open exposure to the
radiocapitellar and proximal radioulnar joints
(Osamura et al. 2004; Wang and Chang 2006).
Treatment Options and Surgical
Techniques
A nonsurgical approach can be taken in the major-
ity of cases of children with radial nerve palsy, as
neurologic recovery occurs within 36 months at
a rate of approximately 1 mm a day (Waters and
Bae 2012). The radial nerve is most commonly
injured by mechanisms of traction or contusion,
resulting in neuropraxic injury, but penetrating
trauma or open fractures to the upper extremity
resulting in weakness and sensory loss in the
radial nerve distribution warrant acute surgical
exploration and nerve repair. In closed trauma,
the absence of radial nerve functional recovery
beyond 6 months warrants surgical exploration
with possible neurolysis and nerve repair, as
untreated radial nerve palsy beyond 12 months is
unlikely to recover (Shergill et al. 2001; Omer
1974). Careful surgical planning is required, and
the upper extremity surgeon must be prepared to
utilize nerve grafting or nerve transfers as options,
based on what is encountered intraoperatively.
Failure of motor recovery after secondary nerve
procedures may necessitate tendon transfers,
accepting sensory loss as a long-term problem.
Fortunately, children tend to recover functionally
from radial nerve injury better than adults.
577
Nonoperative Management
Patients and parents should be well informed and
maintain realistic expectations during the treat-
ment process for a child with radial nerve palsy.
They should understand that expectant manage-
ment is a favorable option in children, particularly
in closed fractures of the upper extremity and in
newborns with compression-related palsy of the
upper arm. Return of sensory and motor function
is expected in 24 months, and delays in recovery
beyond 56 months should prompt surgical
exploration (Hosalkar et al. 2006). Factors that
affect the outcome include age of patient, mecha-
nism of injury, anatomic level of injury, medical
comorbidities, and other associated injuries such
as closed head trauma. Supportive treatment with
splint immobilization and anti-inammatory med-
ications, combined with physical therapy to main-
tain supple range of motion, is benecial when the
child can participate with a therapist (Colditz
1987). Therapy for radial nerve palsy focuses on
limitation of repetitive wrist exion and elbow
extension and emphasizes maintenance of passive
extension of the wrist and digits.
Splinting can be static, dynamic, or a combi-
nation of both. The goal of functional splints is to
maintain range of motion, prevent joint contrac-
tures, and assist with motor function as nerve
recovery proceeds gradually. During radial nerve
palsy, the normal reciprocal tenodesis between
exors and extensors of the wrist is lost, so an
ideal splint should recreate nger exion with
wrist extension and nger extension with wrist
exion (Colditz 1987; Szekeres 2006). A static
volar cock-up wrist splint can help avoid
overlengthening of affected muscles and prevents
overpowering of the wrist extensors by exors.
However, this limits the use of ne manipulative
exion of the ngers and prevents patients from
utilizing their intact palmar sensation (Colditz
1987). Several dynamic splints have also been
proposed by Green, Thompson, and Littler, but
the Thomas, Oppenheimer, and Hollis suspension
splints, which assist in wrist, thumb, and nger
extension during attempted nger exion, are still
used by therapists today (Colditz 1987; Szekeres
2006), as demonstrated in Fig. 10.
578
Fig. 10 Low-prole
neoprene splint to support
wrist in extension and
provide
metacarpophalangeal joint
extension with straps
fastened by Velcro
(Courtesy of Shriners
Hospital for Children,
Philadelphia)
The use of supportive treatments such as ther-
apy and splinting while the radial nerve paresis
resolves is benecial, but some children are too
young to engage in therapeutic exercises or cannot
tolerate bulky dynamic splints, so some physi-
cians prefer a less aggressive approach with static
splinting and observation.
Nerve Exploration and Neurolysis or
Primary Nerve Repair
Indications and Contraindications
Penetrating trauma to the limb and open fractures
resulting in radial nerve palsy warrant acute pri-
mary nerve exploration and repair if required. Any
repairs completed within 1 week are considered
primary (Birch 2010). Delayed secondary nerve
exploration after failure of palsy to resolve can
also reveal a partially or completely lacerated
nerve that needs repair or grafting. Direct repair
of a nerve without tension can be performed when
the nerve gap is less than 2 cm, while any defect
greater than 4 cm usually requires nerve grafting
or transfer. Defects 24 cm present a gray zone, in
which both techniques have been used (Lee
et al. 2008; Hosalkar et al. 2006).
The technique for nerve repair depends on the
size of the nerve and its defect, fascicles involved,
and mechanism of injury (i.e., crush, laceration,
avulsion). Principles for nerve repair include pres-
ervation of epineural vessels that perfuse the
A.P. Kaushik and W.C. Hammert
nerve, identication and preparation of fascicles
in their proper orientation, adequate exposure of
the nerve ends, and nal repair with minimal
tension (Hosalkar et al. 2006; Birch 2010; Clark
et al. 1992). If a single microsuture (8-0 or 9-0)
can hold the ends together, primary repair is
acceptable, but tension on the repair site can com-
promise the outcome, so when present, nerve
grafting should be performed. Crush and avulsion
mechanisms of nerve injury have lower success
rates for recovery, as do more proximal nerve
injuries (Birch 2010).
Preoperative Planning
The surgeon should ensure optimal conditions,
including available microsurgical equipment and
a well-trained operative team during daylight
hours, because the rst attempt at nerve repair
has more predictable outcomes than subsequent
revision nerve surgeries. Fine vascular instru-
ments, microsurgical suture, appropriate needle
holders and forceps, bipolar cautery, brin clot
glue, and nerve collagen conduits are important
to have available for the procedure (Birch 2010).
General anesthesia is preferred to allow
enough time for careful dissection, preparation,
and repair of the nerve. A tourniquet allows a
surgical eld to be unhindered by bleeding ves-
sels, but in more proximal radial nerve injuries, a
tourniquet may not be possible. Magnication
with high-powered loupes or an operating micro-
scope allows more accurate fascicular repair.
26 Radial Nerve Injury 579

Depending upon the location of suspected radial

nerve injury, the patient can be positioned either
supine with a bump (allowing an anterolateral
approach to the radial nerve or PIN), lateral
decubitus, or prone (affording a posterior
approach for more proximal exposure near the
humeral shaft) (Birch 2010; Ingari and Green
2010).

Procedural Details
The radial nerve can be exposed anywhere along
its path from the posterior cord of the brachial
plexus to the distal supinator and beyond, but
approach should be chosen based on the region
of injury. External neurolysis involves release of
all brous tissues causing constriction of the nerve
and careful dissection of the nerve proximally and
distally while maintaining all exiting nerve
branches to individual muscles. This decompres-
sion should be completed for nerves in continuity
and for complete nerve transections prior to repair
(Birch 2010).
For primary repair, nerve ends should be mobi-
lized, and the branching pattern of epineural ves-
sels should be visualized as extensively as
possible. At the zone of injury, nerve ends should
be sharply incised perpendicular to nerve bers to
ensure that healthy nerve tissue can be repaired.
Successive sections of 1 mm should be taken until
uninjured nerve is identied. This is a crucial step
to avoid poor outcomes. If the nerve gap exceeds
24 cm after sequential transections to obtain
healthy fascicles, the surgeon should highly con-
sider a nerve graft or transfer procedure to avoid
excessive tension and impaired blood ow (Clark
et al. 1992). Detailed microsurgical inspection of
the external and internal topography of the nerve
should be completed. The adventitia surrounding
the nerve can be gently retracted to better visualize
the ne reticular network of vessels and also
examine fascicle groups. Undamaged fat can
serve as a suitable wound bed on which to com-
plete the nerve repair (Birch 2010).
Nerve repair is commenced with careful align-
ment of the nerve ends, and 9-0, 8-0, or 7-0 nylon
sutures are placed in the epineurium 180 apart.
With appropriate alignment of epineural vessels,
surface tension will allow easier closure, and
Fig. 11 Primary epineural nerve repair of a nerve lacera-
tion, with accurate orientation using epineural blood ves-
sels as landmarks (Courtesy of Shriners Hospital for
Children, Philadelphia) (Copyright Dan A. Zlotolow)
Fig. 12 Group fascicular repair nerve repair of a nerve
laceration (Courtesy of Shriners Hospital for Children,
Philadelphia) (Copyright Dan A. Zlotolow)
additional epineural sutures are added in modera-
tion to avoid excessive handling of the nerve ends.
Partial nerve injuries can be repaired with simple
epineural suture (Fig. 11). In theory, more accu-
rate motor-to-motor and sensory-to-sensory
reinnervation can occur with group fascicular or
individual fascicular repair (Hosalkar et al. 2006;
Birch 2010), but this creates more internal scar-
ring, thus potentially impeding neuroregeneration
(Fig. 12).
Group fascicular repair requires the appropri-
ate matching of fascicles proximally and distally.
The internal epineurium between fascicular
groups is dissected, and the fascicles or groups
are repaired individually with one or two sutures
each, from deep (farther from surgeon) to super-
cial (closer to surgeon). The external epineurium
is then sutured to reduce tension on the overall
580
repair. If brin glue is used, fewer sutures are
required to complete the repair (Birch 2010).
One method to match motor fascicles more con-
sistently is to use intraoperative nerve stimulation
to evaluate motor response distally, but this tech-
nique is rarely used since children typically do not
tolerate stimulation while awake.
Careful layered subcutaneous and skin closure
is then completed, avoiding extremes of range of
motion of the elbow or shoulder joints to reduce
tension on the repair site. The elbow is splinted in
90 of exion, and the wrist can be splinted in
neutral to slight extension to avoid exion
contracture.
Postoperative Rehabilitation
Plaster immobilization for 3 weeks allows time for
healing of the nerve repair site and the surgical
incision. Goals of the postoperative protocol
should be to maintain joint range of motion
while motor recovery occurs and also to provide
sensory reeducation for the supercial branch of
radial nerve. Although the sensory inputs from
radial nerve are not as crucial as median or ulnar
nerve for protective sensation, accurate fascicle
alignment will allow better reinnervation of skin
receptors. Children also have cortical
neuroplasticity, contributing to improved out-
comes (Fig. 7).
After direct nerve repair, a hinged elbow brace
can be applied at 3 weeks to allow active and
passive exion with a block to extension, to
avoid excess tension on the repair. Progressive
increase in range of motion is allowed over the
rst 6 weeks to avoid adhesions near the nerve
repair site. Between 6 and 12 weeks, unrestricted
motion is allowed, and strengthening of wrist and
nger extensors is emphasized after functional
recovery appears (Birch 2010).
Treatment-Specific Outcomes
Shergill et al. (2001) published a series of
260 adult and pediatric patients (mean age 28)
who underwent either primary repair (9 %) or
nerve grafting (91 %) for injuries to the radial
nerve and PIN. They noted that more proximal
injuries recovered less consistently with nerve
repair or grafting than distal injuries such as
A.P. Kaushik and W.C. Hammert
isolated PIN injury. All nerves repaired after
12 months failed to recover. Open tidy lacera-
tions to the radial nerve had good to fair outcomes
in 79 % of patients, followed by closed traction
injuries such as humeral shaft fractures (58 %
good to fair). Patients with open untidy wounds
such as gunshots or contaminated wounds and
patients with associated vascular injury had
worse outcomes following surgical repair
(Shergill et al. 2001). Although they did not
divide the data to compare pediatric and adult
age groups, other reports have shown that children
fare better after nerve repair than adults (Birch and
Achan 2000; Sairyo et al. 1997).
Nerve Grafting
Indications and Contraindications
Primary nerve repair yields better results than
nerve grafting, but only if there is minimal tension
at the repair site (Slutsky 2005). Segmental nerve
defects greater than 4 cm are not amenable to
primary repair, so nerve grafts or transfers are
usually required. Grafting a healthy nerve to
bridge a short distance offers better outcomes
compared to direct repair of injured tissue. This
reiterates the importance of sequential sectioning
of nerve ends until uninjured fascicles are seen
(Clark et al. 1992). As a general rule, when >10 %
of nerve elongation is necessary to bridge a defect,
or if >4 cm of gap is present, nerve grafting is
advised (Slutsky 2005).
Bridging nerve gaps from 4 to 9 cm or more is
most commonly accomplished with the use of
autograft (Lee et al. 2008). Patients with defects
>10 cm historically have not done as well and
often require tendon transfers (Shergill
et al. 2001). The nerve graft provides a set of
endoneurial tubes through which axonal regener-
ation occurs, and this requires neural growth fac-
tors provided by Schwann cells. Survival of
Schwann cells requires good vascularity to the
entire graft, which occurs from both the proximal
and distal nerve ends of the injured radial nerve
(Slutsky 2005). Vascularity of the tissue bed is
equally as important to promote axonal regenera-
tion. Diffusion from the tissue bed plays a role in
26 Radial Nerve Injury
early nerve nutrition, and endothelial buds emerge
within 3 days after nerve repair (Slutsky 2005).
Preoperative Planning
Surgical equipment, microscope availability, anes-
thesia, and positioning are similar to that described
for nerve repair above. Prior to making incision, the
surgeon should consider potential sources of nerve
autograft and prepare the limb(s) appropriately,
for example, the ipsilateral leg for sural nerve
graft. Large segmental nerve gaps may require
more than one autograft. One should always look
for options to reduce tension on the nerve graft
repair, including extensive nerve mobilization,
positioning of joints, nerve transposition (i.e., radial
nerve through a humeral shaft fracture site in the
acute trauma setting), and bone shortening (Birch
2010; Slutsky 2005).
Potential sources for autograft include the
medial antebrachial cutaneous nerve (direct
branch from medial cord of brachial plexus) and
lateral antebrachial cutaneous nerve (terminal
branch of musculocutaneous nerve) from the
same limb, but further removing sensibility from
the injured limb can be detrimental (Birch 2010;
Slutsky 2005). Therefore, the sural nerve is often
chosen as a good autograft option, allowing up to
40 cm of high-quality nerve tissue from each leg
in adults (Staniforth and Fisher 1978; Lee
et al. 2008). However, sensory loss over the lateral
foot and other donor site morbidities such as neu-
roma, deep vein thrombosis, and hematoma are
prevalent, so patients should be advised about
these potential problems (Staniforth and Fisher
1978). In children, the sensory decit is negligible
as axonal sprouting from adjacent nerves
repopulates the sensory eld (Lapid et al. 2007).
Procedural Details: Preferred Technique
This section focuses upon the use of sural nerve
autograft for the treatment of radial nerve defects.
Placement of the patient in a lateral position or
supine position with a large bump allows access to
the posterior and lateral aspect of the injured arm
as well as the ipsilateral leg from which sural
nerve can be harvested. After tourniquet ination,
a transverse incision is made 1 cm posterior and
proximal to the lateral malleolus. The nerve can be
581
identied distally behind the lateral malleolus,
running immediately lateral to the short saphe-
nous vein. It is then traced proximally and
exposed through a series of short transverse inci-
sions. It travels along the lateral aspect of the
Achilles tendon and becomes more midline, pierc-
ing the deep fascia of the leg proximal to the
musculotendinous junction of the two heads of
gastrocnemius. A longitudinal incision is made
when additional length is required, and the nerve
can be harvested up to its dual origins from the
tibial and common peroneal nerves (Slutsky 2005;
Staniforth and Fisher 1978). The graft should
always be handled carefully in a blood-soaked
moist towel until implantation at the recipient site.
Exposure of the radial nerve injury recipient
site can be completed concurrently when two
surgeons are available or sequentially after
harvesting the sural autograft. Neurolysis from
scar tissue and preparation of the tissue bed and
nerve ends are undertaken as detailed above.
Careful nerve fascicle handling is again empha-
sized. Unscarred synovium or fat is preferred over
disrupted muscle or fascia as a vascularized tissue
bed (Birch 2010; Slutsky 2005). The autograft
length should be at least 15 % longer than the
nerve gap because graft shrinkage occurs after
repair (Birch 2010).
Sural nerve graft can be cut into shorter lengths
with a fresh scalpel to repair individual fascicles
and/or groups of sensory or motor fascicles.
Again, adventitia at the nerve ends should be
retracted 23 mm to allow accurate repair with
8-0 or 9-0 nylon suture. Every attempt should be
made to match fascicles proximally and distally
according to their orientation within the radial
nerve. Because of the size of the sural nerve,
multiple strands (typically 3) are used as a cable
graft. The strands of the sural nerve are joined
with brin glue, allowing easier handling of the
cable graft. The epineurium of the donor sural
nerve is generally sutured to the perineurium of
a fascicle or bundle (Birch 2010). Fibrin glue can
be used to reduce the number of sutures required
and augment the repair. Replacing the nerve graft
repair site within appropriate muscle planes in the
upper arm and forearm ensures protection of the
radial nerve graft.
582
Other graft options include vascularized nerve
autografts (e.g., with short saphenous vein endo-
thelium wrapped around sural nerve), nerve allo-
grafts, and synthetic collagen nerve conduits.
Advantages of vascularized autograft include the
ability to repair within a scarred tissue bed and to
repair larger diameters of nerve (Seckel
et al. 1986). Though no evidence specically
related to radial nerve repair in children is avail-
able, favorable clinical outcomes have been seen
with vascularized nerve grafting in adults. Nerve
allografts are easy to obtain and have no donor site
morbidity. Although they can be useful as con-
duits for axonal regeneration, there is poor
Schwann cell viability and vascularization, so
clinical success, in particular motor recovery, has
been limited with allografts. Tissue-engineered
allograft seeded with bone marrow stromal cells
have shown promising experimental results in
radial nerve defects in other mammals, but further
investigation is needed (Wang et al. 2010). Syn-
thetic nerve conduits can also be worthwhile as
adjuncts to nerve repair or grafting, to promote
availability of neurotrophic factors and foster an
environment for expeditious nerve regeneration.
Similar to allograft nerves, there is insufcient
data regarding motor recovery to recommend the
use of synthetic conduits alone, particularly in
children.
Postoperative Rehabilitation
Postoperative care with initial immobilization and
progressive therapy after nerve grafting is similar
to that of primary nerve repair. Attention must also
be given toward care of the donor site and lower
limb motion if sural nerve is harvested.
Maintaining protected joint range of motion and
sensory reeducation of the upper limb is again
highlighted.
Treatment-Specific Outcomes
Lee et al. (2008) treated six patients with >9 cm
radial nerve defects, one of which was a 14-year-
old with a closed humerus fracture. She
underwent early sural nerve autograft within
2 weeks of injury and had excellent motor and
sensory recovery of function (Lee et al. 2008).
A.P. Kaushik and W.C. Hammert
However, there is limited additional information
on the results of nerve grafting for radial nerve
defects in children. Clinical ndings of radial
nerve grafting in adults suggest slightly more
discouraging outcomes than with direct repair.
Singh et al. (1992) reported that only 57 % of
radial nerve injuries treated with interfascicular
cable graft had good motor recovery and 48 %
had acceptable sensory recovery. Kallio
et al. (1993) found that only 38 % of patients
had a functional motor recovery after radial
nerve grafting. In these studies, preoperative
delay, nerve defects >5 cm, and older age were
risk factors for poorer outcome (Singh et al. 1992;
Kallio et al. 1993).
Nerve and Tendon Transfers
Nerve transfers in the upper extremity have
provided excellent treatment options for brachial
plexus palsy, particularly in restoration of
function of muscles innervated by
musculocutaneous, median, and ulnar nerves.
The radial nerve, however, has not been as
extensively studied, and few reports of nerve
transfers for radial nerve palsy in children are
available. Mackinnon and her co-surgeons have
researched the use of redundant branches of
median nerve (branches to exor digitorum
supercialis, exor carpi radialis, and palmaris
longus) as transfers to ulnar nerve and radial
nerve (Ray and Mackinnon 2011; Mackinnon
et al. 2007). The nerve transfers are usually com-
bined with tendon transfers to produce a collective
effect. Most of their patients had good to excellent
motor function after reconstruction (Ray and
Mackinnon 2011), but as the majority of the
patients were adults, there is insufcient data to
recommend the use of nerve transfers in pediatric
patients.
In a child with prolonged radial nerve palsy
who has failed nerve repair or grafting or who has
had no neurologic return more than 1 year after
injury, tendon transfers must be considered
because prognosis for nerve recovery is poor.
Timing for tendon transfers is controversial, and
26 Radial Nerve Injury
some advocate for bypassing nerve grafting or
transfers if nerve gaps are more than 5 cm in
order to avoid scarring after a failed nerve proce-
dure (Ingari and Green 2010). In the unfortunate
situation where nerve and tendon procedures have
failed, wrist arthrodesis can serve as a nal sal-
vage option; however, this is rarely used in
children.
Ideal prerequisites for successful tendon trans-
fers, elucidated by Jones, Brand, Boyes, Steindler,
Bunnell, and others, include supple joints with full
range of motion, adequate donor tendon strength
and excursion, intact donor muscle innervation,
straight line of pull, synergism of transferred ten-
dons, and adequate soft tissue coverage with min-
imal scar tissue (Ingari and Green 2010). The
three important aspects of radial nerve function
to address are wrist extension, nger extension,
and thumb extension. Isolated PIN injury spares a
radial wrist extensor, so only thumb and nger
transfers are necessary. Proximal radial nerve
injuries that denervate triceps also require resto-
ration of elbow extension.
Although a comprehensive review of tendon
transfers for radial nerve palsy is beyond the scope
of this chapter, some of the historically successful
procedures are noted here. Donor tendons com-
monly utilized are pronator teres (PT), exor carpi
radialis (FCR), palmaris longus (PL), exor
digitorum supercialis (FDS), and occasionally
exor carpi ulnaris (FCU). Restoration of decits
involves extensor carpi radialis brevis and longus
(ECRB and ECRL) for wrist extension, extensor
digitorum communis (EDC) for nger extension,
and extensor pollicis longus (EPL) for thumb
extension. PT is generally accepted as a good
donor to ECRB for wrist extension. FCU was
used previously for transfer to EDC to provide
nger extension but is now usually preserved
because it serves as a strong wrist exor. Starr
and Brand substituted FCR and Boyes utilized
FDS as transfers to EDC, and both tendons have
shown good outcomes for nger extension. PL
can be rerouted to EPL to supply thumb extension
(Ingari and Green 2010). Numerous combinations
and alternative transfers have been reported, but
the above procedures typically provide successful
583
restoration of muscle function in radial nerve
palsy in children.
Complications and Management
Failure of a nerve repair, graft, or transfer to
restore sensorimotor function is a result that war-
rants tendon transfers or alternative salvage pro-
cedures, as noted above. The foremost
complication to avoid during the treatment of
radial nerve palsy in a child, however, is
arthrobrosis. Unresolved joint stiffness or con-
tracture can severely limit the potential for success
with nerve procedures and also precludes the use
of tendon transfers, leaving arthrodesis as the
main remaining option (Ingari and Green 2010).
Adhesions around tendons and neurovascular
structures of the upper extremity can become
chronically restrictive, so tenolysis of sclerotic
structures should be considered to ameliorate tis-
sue excursion.
All nerve transections result in neuromas, but
only some are symptomatic. Painful neuromas can
result in persistent paresthesias and allodynia in
patients with radial nerve injury. These can also
occur at donor nerve sites such as the sural nerve
(Staniforth and Fisher 1978). They can be
addressed with desensitization therapy, local
anesthetic injections, sympathectomy, or sharp
surgical transection and burial within muscle
followed by tendon transfers (Birch 2010).
When nerve or tendon transfers are completed,
motor decits can occur in the donor tissues, for
instance, wrist exion weakness when improperly
matched tendon transfers are completed (Ingari
and Green 2010). This can usually be managed
nonoperatively with strengthening during therapy,
but rebalancing tendon forces with additional
transfers may be needed. Another potential com-
plication is skin breakdown, which can occur if
adequate soft tissue coverage is not provided after
repair. The use of bone shortening, Z-plasties, and
other ap options for skin coverage can help
reduce tension on the skin closure (Birch 2010).
Other complications include hematomas, deep
vein thromboses, and lymphedema.
584
Summary
Children with radial nerve palsies that arise from a
spectrum of etiologies, usually traumatic, can be
managed at the outset with splinting, therapy, and
observation for return of neurologic activity, pro-
vided that penetrating trauma or open fracture is
absent. Patients without functional recovery in
36 months deserve consideration for nerve
exploration with neurolysis or primary repair, or
secondary procedures such as nerve grafting or
transfer. Prognosis for recovery generally deteri-
orates with more sophisticated nerve procedures,
so tendon transfers are a good option when joints
are supple and scar tissue is not restrictive. With
all treatment options, children tend to regain
motor and sensory function of the radial nerve
better than adults.
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 Part VIII
Brachial Plexus Palsy
 Neonatal Brachial Plexus Palsy
27
Charles T. Mehlman

Contents Abstract
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 590 The most common nerve injury in children is
neonatal brachial plexus palsy (NBPP). NBPP
History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 590
Earliest History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 590
is associated with signicant upper extremity
Kaiser Wilhelm II . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 593 impairment and carries with it quality of life
impact on the child as well as the entire family.
Epidemiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 594
Incidence . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 594 The occurrence of NBPP in the United States is
Risk Factors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 594 close to 1.5 per 1,000 live births. Thirty to forty
Classication Systems and Outcome Measures . . . . . 596 percent of these children will suffer permanent
Conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 601 upper extremity functional insufciencies.
NBPP is not a new nding. In the mid-1700s,
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 602
a physician named William Smellie wrote
about a child that presented face-rst for deliv-
ery. Smellie carefully delivered the child using
forceps and noted that the long time spent in
the birth canal had rendered the childs arms
paralyzed for several days. By the mid-1800s,
NBPP had become known as obstetrical palsy,
relating the palsy to delivery. In the late 1800s,
a physician named Wilhelm Heinrich Erb
posed a signicant breakthrough when he
described the C5C6 junction as a common
area of injury for NBPP patients. This chapter
reviews what might be accepted as the high
points of the history and epidemiology of
NBPP. Understanding the past allows us to
appreciate where we are in the present, and it
gives us greater ability to focus on the future.
Great strides have been made in just the last
decade. There have been signicant advances
C.T. Mehlman
made in virtually all facets of this injury,
Cincinnati Childrens Hospital Medical Center Brachial
Plexus Center, Cincinnati, OH, USA
e-mail: charles.mehlman@cchmc.org

# Springer Science+Business Media New York 2015 589

J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_27
590
Fig. 1 Hector strikes
Teucer with a jagged rock in
the region of his left clavicle
understanding its incidence and risk factors,
basic science insight to bone and muscle
growth abnormalities following nerve injury,
as well as both primary and secondary surgical
reconstructive treatment approaches. The
multidimensional physical and psychological
challenges of this patient population will con-
tinue to merit coordinated multispecialty care
for some time to come.
Introduction
Neonatal brachial plexus palsy (NBPP) is the
most common nerve injury in children, and it is
associated with signicant upper extremity
impairment (Reading et al. 2012; Weekley
et al. 2012; Nikolaou et al. 2011, 2014; Louden
et al. 2013; Mehlman et al. 2011). NBPP has a
signicant health-related quality of life impact on
the child as well as a psychological and quality of
life impact on their entire family (Akel et al. 2013;
Oskay et al. 2012; Firat et al. 2012; Karadavut and
Uneri 2011; Alyanak et al. 2013). Its story is a
rather rich and ancient one that ows all the way
up to the modern epidemiologic understanding of
the injury. The incidence of NBPP in the United
States is about 1.5 per 1,000 live births, and 3040
% of these children will suffer permanent upper
extremity functional decits (Pondaag et al. 2004)
(Foad et al. 2009). This chapter will present an
overview of the history and epidemiology of neo-
natal brachial plexus palsy.
C.T. Mehlman
History
Earliest History
Some consider the nearly 3,000-year-old story that
is Homers Iliad to contain the two earliest written
descriptions of brachial plexus injury, albeit in
young adults (Aydn et al. 2004, Plast Recon
Surg). The two accounts involve battles between
Hector and Teucer and then Achilles and Hector. In
the rst, Hector strikes Teucer over his left clavicle
with a jagged stone which causes Teucer to drop his
bow and arrow due to arm weakness (Fig. 1). Later
in the story, Achilles uses a spear to strike Hector in
the region of his right clavicle which makes Hec-
tors arm droop and drop his spear (Fig. 2).
The physician named William Smellie
(16971763) was a Scottish-born obstetrician
who practiced and taught in London from 1739
to 1759 (Roberts et al. 2010; Fig. 3). He became
famous for many things including obstetrical for-
ceps and a special breech delivery technique.
Smellie is commonly credited with the earliest
known English language description of brachial
plexus injury (McGillicuddy 2011). In his book he
describes the following events:
In the year 1746, about nine oclock in the morning,
I was called by a gentleman who had formerly
attended my lectures, to a woman in labour, and
found the childs face presenting. . . [using forceps
for delivery] I pulled gently, moving the head from
ear to ear, until it was brought lower down into the
pelvis, then . . .I turned the chin and anterior part of
27 Neonatal Brachial Plexus Palsy
Fig. 2 Achilles uses his spear to strike Hector in the region
of his right clavicle
Fig. 3 William Smellie (16971763)
the neck forwards, from the lower part of the
ischium to the space below the pubes, so the fore-
head was at the same time turned from the left
ischium to the lower part of the sacrum and coccyx.
Lastly, I moved the handles towards the pubes, and
delivered the woman of child, whole face was
swelled, and whole head was compressed . . . the
long compression had rendered the arms paralyzed
for several days, though this misfortune was soon
remedied by friction and embrocations. (Smellie
1784)
591
Fig. 4 Guillaume Benjamin Amand Duchenne
(18061875)
The large number of cadaveric specimens uti-
lized by Smellie, as well as his contemporary
William Hunter (known as the father of modern
surgery), has drawn renewed attention in recent
years. Theories related both to body snatching
schemes and outright murder have been advanced
by the New Zealand medical historian Don
C. Shelton (2010, 2012).
In 1872 (over 100 years after Smellies death),
the French physician Guillaume Benjamin
Amand Duchenne coined the term obstetrical
palsy and established its etiology as a neurogenic
one (Schmitt et al. 2008; Fig. 4). This obstetrical
term is now considered obsolete by many, and
more accurate terms such as NBPP are preferred
(Phua et al. 2012). In 1877 the German physician
Wilhelm Heinrich Erb offered an important ana-
tomical breakthrough when he described the
C5C6 junction (aka Erbs point) as a common
area of injury for brachial plexus palsy patients
(Watt et al. 2007; Schmitt et al. 2008; Tubbs
et al. 2007; Fig. 5). These two physicians have
become linked by the hyphenated eponym of
Erb-Duchenne brachial plexus palsy (Mehlman
2009).
Augusta Klumpke (18591927) was born in
San Francisco but later moved to Europe where
she completed her medical education in Paris and
592
Fig. 5 Wilhelm Heinrich Erb (18401921)
Fig. 6 Augusta Klumpke (18591927)
went on to break a major gender barrier when she
became the rst female intern in Paris (really a
residency by modern standards) (Fig. 6; Satran
1974; Shoja and Tubbs 2007; Ulgen et al. 2008).
While still a medical student, Klumpke was
inspired by the published work of Erb and under-
took brachial plexus-focused research of her own
(Shoja and Tubbs 2007). In her paper (published
in 1885), she elegantly made the case that the
oculopupillary phenomenon (aka Horner syn-
drome or Bernard-Horner syndrome) was
C.T. Mehlman
Fig. 7 James Warren Sever (18781964)
indicative of proximal nerve root injury to the
lower plexus, what she termed radicular paraly-
ses (Ulgen et al. 2008). Her assertion was con-
trary to prevailing opinion at the time that all
brachial plexus injuries gave rise to this ocular
nding. During her internship, she married one
of her former professors, French neurologist
Joseph Jules Dejerine, and thus, literature refer-
ences often list her as Dejerine-Klumpke (Shoja
and Tubbs 2007; Ulgen et al. 2008).
James Warren Sever (18781964) (Fig. 7) and
Joseph Battiato LEpiscopo (18901947) (Fig. 8)
contributed important secondary reconstructive
principles aimed at improving upper extremity
function of NBPP patients (Sever 1916, 1918;
LEpiscopo 1934, 1939). Sever focused on surgi-
cal release of contracted (dysplastic?) (Nikolaou
et al. 2011) structures, while LEpiscopo added
muscle transfers aimed at maintaining active
external rotation. The work of Sever (1916) can
also be pointed to as a watershed moment in the
history of NBPP due to its size (36-page mono-
graph) and its comprehensiveness (he reviewed
the worlds literature from the mid-1700s on),
and it was widely read as it clearly inspired
LEpiscopo and many, many others (Mehlman
2007). These two gures remain linked by the
hyphenated eponym: Sever-LEpiscopo
procedure.
27 Neonatal Brachial Plexus Palsy
Fig. 8 Joseph Battiato LEpiscopo (18901947)
Fig. 9 Algimantas Otonas Narakas (19271993)
Algimantas Otonas Narakas (19271993)
pioneered brachial plexus surgical repair tech-
niques both before and after the introduction of
the surgical microscope (Fig. 9; Egloff 1995;
Taleb et al. 2013). He was born in Lithuania but
went on to become a Swiss citizen and practice his
entire surgical career in Switzerland (Egloff and
593
Bonnard 1995). His eponymical contributions
include the Narakas classication (which groups
NBPP patients into four disease severity groups
based on the number of involved nerve roots) and
the Narakas meeting, traditionally referred to as
the Narakas Club (recognized as the preeminent
every other year brachial plexus meeting on the
planet) (Al-Qattan et al. 2009; Palazzi et al. 1999;
Taleb et al. 2013).
Kaiser Wilhelm II
The impact of neonatal brachial plexus palsy on
world history has been frequently discussed as it
relates to Kaiser Wilhelm II (18591941), the
notorious Emperor of Germany in World War I
(Fig 10; Jacoby 2008). Kaiser Wilhelm II was the
son of Princess Victoria (eldest daughter of Queen
Victoria) and Kaiser Wilhelm I. The primiparous
19-year-old mother-to-be had been in labor for at
least 9 h when Queen Victorias personal physi-
cian Sir James Clark diagnosed the babys breech
presentation. Urgent arrangements were made for
Professor Eduard Arnold Martin (a prominent
German obstetrician) to take over responsibility
for the delivery. Martin used chloroform as well as
the breech delivery technique described by
Smellie nearly 100 years earlier to successfully
deliver the baby (Ober 1992). Within several days
it was ascertained that the babys left arm was
abnormally weak, and subsequently it did not
grow properly (Jain et al. 2005).
Kaiser Wilhelm II blamed the lifelong disabil-
ity of his left arm on the failure of the
English physician (Sir James Clark) to
properly respond during his birth. As fate would
have it, there was also an English physician
involved with the care of his father, Kaiser
Wilhelm I, near the time of his death. This led to
Kaiser Wilhelm II stating years later, An English
doctor killed my father, and an English doctor
crippled my arm; and all this I owe to my mother
who would not have Germans around her (Jain
et al. 2005). This has led some historians to pro-
pose that this solidied a deep-seeded hate for the
English and thus helped shape world history
(Ober 1992).
594
Fig. 10 Kaiser Wilhelm II (18591941)
Epidemiology
Incidence
Early efforts at studying the incidence of NBPP in
the United States were limited by the information
systems of the day. In addition to this, most
reviews were also focused on a single institution
or metropolitan area (Foad et al. 2008). For
instance, a 1975 publication from the University
of California reviewed over 19,000 newborn
infants and identied only 11 babies with brachial
plexus palsy, yielding an incidence of 0.57 per
C.T. Mehlman
1,000 live births (Specht 1975). A ten-year anal-
ysis (19721982) of data from the Kaiser Foun-
dation Hospital in San Francisco identied
61 babies with birth palsy out of a cohort of over
30,000 deliveries for an incidence of 2.0 per 1,000
live births (Greenwald 1984). At Johns Hopkins
an 11-year (19932004) review revealed a sur-
prisingly high 5.8 per 1,000 live birth rate
(135 palsies among 23,273 births) (Gurewitsch
2006).
A 23-year (19802002) review of records at
the University of Mississippi produced 85 NBPP
cases from over 89,000 vaginal deliveries (inci-
dence 1.0 per 1,000 live births) (Chauhan
et al. 2005). These same authors offered provoc-
ative evidence-based extrapolation of their data in
that they estimated that the average obstetrician
(typically 140 deliveries per yr for ACOG fel-
lows) should encounter NBPP once every
7 years and a permanent plexus injury once
every 71 years (Chauhan et al. 2005). A 1999
publication of computerized database analysis of
over 300 hospitals across California found an
incidence of 1.5 per 1,000 live births (1,611
palsies in over one million deliveries) (Gilbert
et al. 1999). The rst large US national database
study of NBPP included over 17,000 palsy
patients from a pool of more than 11 million
births, yielding an incidence of 1.5 per 1,000
live births (Foad et al. 2008). Interestingly, 54 %
of the babies with NBPP did not have known risk
factors (Foad et al. 2008).
Risk Factors
Multiple risk factors have been suggested as pre-
dictive of NBPP, but numerous studies have
pointed to shoulder dystocia and macrosomia
(usually dened as birth weight >4.5 kg) as
being exceptionally strong ones (Foad
et al. 2008; Mollberg et al. 2005; Chauhan
et al. 2005). In an American national database
study, shoulder dystocia and macrosomia were
associated, respectively, with a 100 times and
14 times higher risk of neonatal plexus palsy
(Fig. 11; Foad et al. 2008). It would then seem to
simply be necessary to pre-identify macrosomic
27 Neonatal Brachial Plexus Palsy
Fig. 11 Shoulder dystocia.
(a) Baby higher in the birth
canal. (b) Shoulder dystocia
has occurred. Smellies
illustration from 1780, also
illustrating application of
Smellie forceps
infants (perhaps by ultrasound) in order to identify
those at increased risk for shoulder dystocia and
thus at greater risk for NBPP. But in reality ultra-
sound is an imperfect predictor of macrosomia
(Sacks and Chen 2000; Mehta et al. 2005;
Goetzinger et al. 2014; Nguyen et al. 2013),
macrosomia is an imperfect predictor of shoulder
dystocia (Chauhan et al. 2006; Gherman
et al. 2006; Alsunnari 2005), and shoulder dysto-
cia is an imperfect predictor of NBPP (Chauhan
et al. 2007; Christoffersson et al. 2003).
Other contemporary facts regarding shoulder
dystocia muddy the water even further. It has been
shown that there was a tenfold increase in the rate
of shoulder dystocia in recent decades (Dandolu
et al. 2005), and surprisingly there are mixed
results at best regarding the effectiveness of
obstetrical educational programs aimed at manag-
ing shoulder dystocia (Walsh 2011; Inglis
et al. 2011). Many obstetricians consider the
only reliable predictor of shoulder dystocia to be
a prior maternal history of shoulder dystocia
(Bingham et al. 2010; Ouzounian et al. 2012).
On top of all of this, rates of NBPP have remained
stubbornly steady over the course of decades
despite the above noted increase in shoulder dys-
tocia rates as well as substantial increases in
Cesarean section rates in the United States
(Walsh et al. 2011; Graham 1997). This has led
numerous authors to point to the shortcomings of
595
commonly accepted risk factors (Backe 2008;
Gurewitsch 2006; Sandmire and DeMott 2005)
and others to assert that one or more known risk
factors are present in only a minority (46 %) of
newborn babies with NBPP (Foad et al. 2008).
This previous discussion shows that as clear as
it is in many cases that traction-related birth
trauma seems to be the explanation for the neona-
tal brachial plexus injury, in other instances, it is
equally mysterious as to exactly how or precisely
when the injury occurred. This controversy
regarding the mechanism of injury is not at all
new. James Warren Sever conducted extensive
infantile cadaver experiments and concluded:
. . .that traction and forcible separation of the head
and shoulder puts the upper cords, the fth and sixth
cervical roots of the brachial plexus, under danger-
ous tension. This tension is so great that the two
upper cords stand out like violin strings. Any sud-
den force applied with the head bent to the side and
the shoulder held would without question injure
these cords . . . One thing impressed me, and that
was the evident vulnerability of the upper cords of
the plexus under any degree of traction, and I was
surprised that the paralysis was not of much more
frequent occurrence. (Sever 1916)
But in Severs same 1916 report of 470 NBPP
patients, there is a solid 7 % who were born
following apparently normal labors (Sever
1916). Many others have also traditionally
interpreted William Smellies 1746 birth palsy
596
description (cited earlier in this chapter) as sup-
portive of an intrauterine etiology.
This provocative issue has been taken on in
recent years by multiple authors at a variety of
centers (Jennett et al. 1992; Sandmire and DeMott
2000; Gonik et al. 2000). In 1992 Jennett
et al. concluded that the data are strongly sug-
gestive that intrauterine maladaptation may play a
role in brachial plexus impairment (Jennett
et al. 1992). Bernard Goniks classic paper
published in 2000 used mathematical modeling
to contrast endogenous forces (uterine and mater-
nal expulsive efforts) to exogenous forces (clini-
cian applied forces), concluding that the
endogenous forces were four to nine times greater
than clinician-related forces (Gonik et al. 2000).
This line of research drew attention to potential
differences between difcult and seemingly
uncomplicated labors.
Gurewitsch and her colleagues looked at this
question from the perspective of plexus palsies
that occurred with and without shoulder dystocia
and concluded that non-shoulder dystocia-associ-
ated NBPP was likely to be a mechanically dis-
tinct entity (Gurewitsch 2006). They tracked
13 separate risk factors and found that 11 % of
shoulder dystocia-associated brachial plexus
palsy patients had no identiable risk factors as
compared to 30 % of non-shoulder dystocia
plexus palsy patients. These risk factors ranged
from well-known ones such as macrosomia and
instrumented delivery to somewhat less well-
known risk factors such as long second stage of
labor and excessive maternal weight gain
(Gurewitsch 2006). It thus becomes increasingly
clear that alternate mechanism of injury explana-
tions need to be entertained.
A growing number of authors have drawn
attention to intended and unintended outcomes
associated with oxytocin augmentation of labor
(Wei et al. 2010; Mori et al. 2011; Bugg
et al. 2011; Mehlman et al. 2012; Costley and
East 2012; Miller 2009; Doyle et al. 2011;
Ouzounian et al. 2005). High- and low-dose
approaches to oxytocin administration exist, and
tachysystole (the term used for uterine hypersti-
mulation) occurs more frequently with high-dose
regimens (Wei et al. 2010). In a case-control study
C.T. Mehlman
of 52 mothers and their NBPP babies versus
132 mothers and their unaffected babies, oxytocin
administration and tachysystole were statistically
signicant risk factors for NBPP (respective odds
ratios of 2.5 and 3.7) (Mehlman et al. 2012). It
would seem plausible that a child traversing the
birth canal with a leading head and trailing shoul-
der (or perhaps other positions as well) who expe-
riences signicant uterine forces might very well
sustain NBPP.
Classification Systems and Outcome
Measures
In order to have hope regarding scientic progress
in the clinical care of NBPP patients, there must
be valid and reliable tools on both the front end
of the disease (e.g., disease severity measures,
age-specic rating scales) and the back end of
the disease (e.g., health-related quality of life,
functional outcomes) (Chang et al. 2013). Validity
addresses the issue of whether the instrument
(questionnaire, rating scale, etc.) measures what
it is intended to measure, and reliability speaks to
its ability to yield appropriate reproducible mea-
surements. Collectively these are referred to as
psychometric properties. Some of these NBPP
tools have yet to undergo formal reliability testing
while a number of researchers at different neona-
tal brachial plexus centers have performed impor-
tant reliability and validity studies for others. New
NBPP-specic instruments also continue to
evolve (Ho et al. 2012). This section will focus
on the most widely used and most widely studied
NBPP tools that are currently available.
The classic NBPP disease severity measure is
the Narakas classication. In 1987 Algimantas
Narakas proposed that based on physical exami-
nation at 23 weeks of age, patients could be
practically divided into four groups (Narakas
1987). The original classication recognized
groups 1 and 2 as classic Erb-Duchenne palsy
(C5 and C6) and extended Erb-Duchenne (C5,
C6, and C7), while groups 3 and 4 were total
plexus palsies without and with the presence of
Horner syndrome. The very presence of Horner
syndrome (aka Klumpkes sign as she was the rst
27 Neonatal Brachial Plexus Palsy 597

Table 1 Modified Narakas classification

Group Name Roots Key ndings
1 Typical C5, Wrist extension
Erb-Duchenne C6 often weak
palsy Hand not affected
2 Extended C5, Same as
Erb-Duchenne C6, 1 + active elbow
palsy C7 extension not as
strong and
denite wrist drop
2-A early recovery wrist extension against gravity
(23 weeks to 2 months)
2-B no early recovery wrist extension against
gravity (>2 months)
Fig. 12 Clinical photo of an infant with left Narakas 3 Total palsy C5, Wrist exed, hand
4 neonatal brachial plexus palsy without Horner C6, tightly closed
sign C7,
C8,
to describe the association between brachial T1
4 Total palsy C5, Horner sign
plexus injury and these oculopupillary ndings)
with Horner C6, indicates
has been shown to have independent unfavorable sign C7, preganglionic T1
prognostic value (see Fig. 12 baby photo) C8, injury
(Al-Qattan et al. 2000). These Narakas groupings T1
have been shown to have prognostic power, with
dramatically lower full recovery rates for Narakas
3 and 4 patients (Narakas 1987; Sibinski and data from ve distinct muscle groups, one of
Synder 2007; Foad et al. 2009). which is elbow exion (see Table 2). Prior to the
Al-Qattan and his Saudi Arabian colleagues Toronto Test Score, many brachial plexus sur-
have recently reviewed over 500 of their NBPP geons used just the presence or absence of elbow
patients and produced a useful modication of the exion at a particular age (e.g., 3 months of age)
Narakas classication (see Table 1) (Al-Qattan as their main criterion for recommending nerve
et al. 2009). They found that Narakas group reconstruction surgery. The Toronto Test Score
2 patients who recovered their wrist extension effectively changed this to multidimensional deci-
early (between 2 and 3 weeks of age and 2 m of sion making. The original article showed that the
age) had better rates of spontaneous recovery than Toronto Test Score at 3 months of age correctly
those who did not (Al-Qattan et al. 2009). For predicted the childs clinical status at 1 year of age
instance, 76 % of Narakas 2-A patients recovered 95 % of the time, while similar use of only elbow
shoulder abduction, while only 18 % of Narakas exion was predictive in only 13 % of cases
2-B patients did the same. It can be argued that the (Michelow 1994).
validity of the Narakas classication system (and Two other published reports have focused on
now the modied Narakas classication) is self- the Toronto Test Score. In 2003 the Boston Chil-
apparent, but formal reliability studies have yet to drens group assessed the reliability of the scoring
be published. system and found interobserver reliability in the
Disease severity is also commonly assessed by good range (mean 0.51) and intraobserver reliabil-
the Toronto Test Score, a tool aimed at assessing ity in the excellent range (mean 0.73) (Bae
children less than 1 year of age. This scale was et al. 2003). In 2008 these same authors compared
introduced in 1994 by brachial plexus specialists the patient-derived pediatric outcomes data col-
at the Hospital for Sick Children in Toronto, lection instrument, also known as the PODCI
Ontario (Michelow 1994). The Toronto Test (a health-related quality of life instrument), to
Score combines physical examination-derived the physician-derived Toronto Test Score. They
598
Table 2 Toronto Test Score
Observationa Grade Score
No joint movement 0 0
Flicker of movement 0+ 0.3
Less than half range 1 0.6
Half range of movement 1 1.0
More than half range 1+ 1.3
Good but not full range 2 1.6
Full range of movement 2 2.0
a
Active motion against gravity
found the strongest correlation between the
Toronto Test Score and the PODCI global func-
tion scores in 610-year-old patients (Bae
et al. 2008).
At the 1972 French congress of orthopedics,
Jean Mallet presented a method for measuring
upper extremity function (strongly weighted
toward the shoulder) of NBPP patients (Mallet
1972; Gilbert and Pivato 2005). Classically this
was a ve-category system with a perfect score of
25 points being achieved by getting a full ve
points for global abduction, global external rota-
tion, hand to neck, hand to spine, and hand to
mouth. This system became widely accepted and
has been used extensively now for decades.
Although often applied to younger children, the
scale is considered ideal for children 34 years of
age and older as proper cooperation is important
with the Mallet classication.
Psychometric properties of the Mallet classi-
cation have been studied at two different centers.
In Boston the reliability (intraobserver and
interobserver) of the Mallet was shown by Bae
et al. to be in the excellent range with kappa values
that exceeded 0.75 (excellent dened as
0.610.80) (Bae et al. 2003). Researchers from
the Netherlands have independently reported sim-
ilar excellent agreement (mean kappa 0.65)
with the Mallet classication (van der Sluijs
et al. 2006). In order to improve assessment of
internal rotation and midline function, Scott
Kozin has made an important modication to the
Mallet by adding the sixth category of internal
rotation, thus increasing a perfect score to
30 points (see Fig. 13; Kozin 2011). The reliabil-
ity of this modication of the Mallet score has not
yet been published.
C.T. Mehlman
Motion Sub-score
THUMB EXT
WRIST EXT
ELBOW EXT
ELBOW FLEX
FINGER EXT
Highest score 10
Lowest score 0
Health-related quality of life instruments are
also important. Researchers at the Shriners Hos-
pitals for ChildrenNorthern California studied
the pediatric outcomes data collection instrument
(PODCI) in 23 of their NBPP patients who were
candidates for secondary shoulder reconstruction
surgery (Huffman et al. 2005). They found that
their NBPP patients scored signicantly lower on
the PODCI as compared to normative pediatric
controls. The Boston Childrens group compared
PODCI scores to the Mallet classication,
Toronto Test Score, and Active Movement Scale
in 150 of their plexus patients between 2 and
10 years of age (Bae et al. 2008; Michelow
1994; Gurewitsch 2006; Alsunnari 2005; Graham
1997).
A variety of imaging techniques have been
studied regarding NBPP. It is important to identify
root avulsions in infants considered to be surgical
candidates as this clearly inuences reconstruc-
tive tactics. The Toronto group has studied the
predictive value of computed tomographic
(CT) myelography in 63 infants with NBPP.
They concluded that root avulsions were best
predicted by the presence of pseudomeningoceles
without evidence of traversing rootlets as this
nding demonstrated a 74 % positive predictive
value (likelihood ratio of 18.5) (Chow et al. 2000).
Martijn Malessy and his group from Leiden
University in the Netherlands have also recently
published their ndings related to CT
myelography. They studied a group of 124 infants
who were surgical candidates (118 cephalic deliv-
eries and 6 breech deliveries) and found that 56 %
of cephalically delivered patients had root avul-
sions, while 100 % of breech babies had the same
nding (Steens et al. 2011). The root avulsion
27 Neonatal Brachial Plexus Palsy
Fig. 13 Modied Mallet classication, including the sixth dimension added by Scott Kozin
kappa value for interobserver agreement between
their two neuroradiologists was 0.78 (excellent
range), and for detection of pseudomeningoceles,
it reached the almost perfect range (kappa
0.84) (Steens et al. 2011). Malessys group
considered a striking nding to be that in
about 15 % of patients, CT myelography identi-
ed avulsions of C7 or C8 that were not suspected
from clinical examination (Steens et al. 2011).
Thus, despite the acknowledged drawbacks of
CT myelography (e.g., invasive procedure, radia-
tion exposure), it remains the imaging procedure
of choice in these infants as magnetic resonance
imaging (MRI) and magnetic resonance
myelography have not outperformed it (Steens
et al. 2011; Medina et al. 2006).
Another area where radiographic imaging has
been studied is within the setting of secondary
shoulder deformity. An Amsterdam research
599
group led by Johannes van der Sluijs performed
MRI studies (1.5 T magnet) on 30 of their NBPP
patients (ages ranging from 3 months to 6.3 years)
and assessed the reliability of several shoulder
dysplasia measurement methods (van der Sluijs
et al. 2003). They concluded that a qualitative
glenoid classication (concave-at, convex, or
biconcave), an angular measurement of humeral
head subluxation, and the glenoid version angle
could all be measured with reasonable reliability
(van der Sluijs et al. 2003). Our Cincinnati group
has also studied the reliability of glenoid version
and glenohumeral subluxation measurements in a
group of 25 plexus patients (average age 4 years
and 4 months) who underwent MRI studies (mix-
ture of 1.5 and 3.0 T magnets) (see Fig. 14;
Lippert et al. 2012). Excellent interrater and
intrarater reliability was demonstrated but with
measurement errors in the ve range for both
600
Fig. 14 Glenoid version
and glenohumeral
subluxation measurement.
(a) Glenoid version
angle alpha 90 . (b)
Glenohumeral subluxation
is calculated as percent of
the humeral head anterior to
the scapular line (aka
PHHA). PHHA AB/
AC  100 %
(+5 for version and 5 % for subluxation)
(Lippert et al. 2012).
Much attention has been focused on the
glenoid side of the shoulder joint. Cincinnati bra-
chial plexus researchers have recently introduced
a new measurement that assesses the shoulder
deformity that occurs on the other side of the
glenoid (Reading et al. 2012). Humeral head trac-
ings were performed on the affected and unaf-
fected shoulders of 32 NBPP patients (average
age 2.9 years). The most cephalad transverse cut
of the humeral head where the biceps tendon was
still identied within the bicipital groove was used
for these tracings. The area of the anterior half of
each humeral head was then divided by the area of
the posterior half to calculate what has been
termed the skewness ratio (see Fig. 15; Reading
et al. 2012). Fair to good reliability for the mea-
surements was shown using the intraclass corre-
lation coefcient (aka the ICC, which is used for
continuous data and is analogous to kappa in that
it controls for chance agreement between raters).
The skewness ratio was also shown to be
C.T. Mehlman
responsive to change in that statistically signi-
cant improvements were documented following
arthroscopic anterior release and tendon transfer
surgery (Reading et al. 2012).
The disadvantages of MRI for imaging chil-
dren with NBPP include its high cost and the
highly likely need for either sedation or a true
anesthetic. As a result some authors prefer ultra-
sound imaging to diagnose and follow shoulder
dysplasia in their brachial plexus patients
(Saifuddin et al. 2002). Texas Scottish Rite
researchers analyzed the interrater and intrarater
reliability of this imaging approach in thirty of
their NBPP patients under the age of 1 year
(Vathana et al. 2007). In this age group there is
incomplete ossication of the humeral head. They
reported ICCs that were all in the excellent range
(0.8750.91), indicating the merit of this
approach (Vathana et al. 2007).
Three-dimensional motion analysis of children
has mainly been used in the setting of cerebral
palsy patients and in elite baseball pitchers
(Dreher et al. 2012; Nissen et al. 2009). Michele
27 Neonatal Brachial Plexus Palsy 601

Fig. 15 Skewness ratio.

(a) Bilateral humeral head
tracings made at the
appropriate level (highest
transverse cut with biceps
tendon within bicipital
groove). (b) Calculation of
skewness ratio using
anterior half divided by
posterior half

James and her fellow researchers at the Shriners

Hospitals for ChildrenNorthern California have
used such motion analysis in their NBPP patients.
These authors have shown both that the affected
shoulders of plexus patients are signicantly dif-
ferent from controls (Mosqueda et al. 2004) and
that the unaffected shoulder in such children is
also signicantly different from the affected
shoulder (Wang et al. 2007). Scott Kozin and his
coauthors have used such kinematic techniques to
quantify glenohumeral and scapulothoracic con-
tribution to arm elevation (Duff et al. 2007). Other
kinematic researchers have continued to rene
these measurement techniques in NBPP patients
(Mattson et al. 2012).
Conclusion
This chapter has reviewed what one might accept
as the high points of the history and epidemiology
of NBPP. Understanding the past allows appreci-
ation for where NBPP is in the present, and it
gives greater ability to focus on the future. Just
in the last decade, signicant advances have been
made in virtually all facets of this injury, under-
standing its incidence and risk factors, basic sci-
ence insight to bone and muscle growth
abnormalities following nerve injury, as well as
both primary and secondary surgical reconstruc-
tive treatment approaches. The multidimensional
602
physical and psychological challenges of this
patient population will continue to merit coordi-
nated multispecialty care for some time to come.
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 Microsurgery for Obstetrical Brachial
Plexus Palsy 28
Marc C. Swan and Howard M. Clarke

Contents Abstract
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 608 While the majority of obstetrical brachial
Historical Perspectives . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 608 plexus palsies may be managed
Epidemiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 608 nonoperatively, a noteworthy proportion of
Pathoanatomical Considerations . . . . . . . . . . . . . . . . . . 610 patients will necessitate microsurgical recon-
struction in order to optimize long-term func-
Clinical Assessment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 612
tional outcome. The indications for operative
Indications for Surgical Exploration . . . . . . . . . . . . . . 613 management, as well as the timing of surgical
Preoperative Investigations . . . . . . . . . . . . . . . . . . . . . . . . 617 intervention and the microsurgical techniques
Surgical Exploration and Reconstruction . . . . . . . . 618 employed, vary extensively between specialist
Intraoperative Setup . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 618 units. This is in part due to the anatomical
Bilateral Sural Nerve Harvest . . . . . . . . . . . . . . . . . . . . . . . . 619 complexity of the brachial plexus, an imperfect
Approach to the Brachial Plexus . . . . . . . . . . . . . . . . . . . . 619 understanding of the natural history of obstet-
The Role of Neurolysis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 622
Reconstruction with Sural Nerve Grafts . . . . . . . . . . . . . 623 rical brachial plexus palsy, and the broad clin-
Nerve Transfers . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 623 ical phenotype with which this challenging
Postoperative Care . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 625
condition presents.
Whereas surgical exploration of the child
Complications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 626 with a ail limb and an ipsilateral Horners
Functional Outcomes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 626 syndrome is without contention, those with
Future Developments . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 628 more subtle clinical signs represent a greater
management challenge. The importance of
Conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 628
treating these children within the context of a
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 629 specialist multidisciplinary team cannot be
overemphasized; indeed the physical and occu-
pational therapists play a fundamental role in
M.C. Swan (*) the assessment of these complex children. The
Nufeld Department of Surgical Sciences, University of indications for primary reconstructive surgery
Oxford, Oxford University Hospitals NHS Trust, will be examined together with the value of
John Radcliffe Hospital, Headington, Oxford, UK
specic radiologic and electrodiagnostic inves-
e-mail: marc.swan@nds.ox.ac.uk
tigations in guiding clinical decision making.
H.M. Clarke
The reconstructive priorities center on the res-
The Hospital for Sick Children and the Department of
Surgery, University of Toronto, Toronto, ON, Canada toration of hand function, elbow exion, and
e-mail: howard.clarke@utoronto.ca shoulder movement. The mainstay of
# Her Majesty the Queen in Right of United Kingdom 2015 607
Published by Springer Science+Business Media New York 2015. All rights reserved.
J.M. Abzug et al. (eds.), The Pediatric Upper Extremity, DOI 10.1007/978-1-4614-8515-5_28
608
achieving these goals is open surgical explora-
tion of the brachial plexus with neuroma exci-
sion and, wherever possible, sural nerve cable
grafting of the resultant defect. In limited cir-
cumstances neurolysis may be indicated. Intra-
plexal and extra-plexal motor nerve transfers
are also frequently performed when indicated
by the clinical motor examination and the anat-
omy of the plexus injury.
The surgical principles employed in microsur-
gical reconstruction of obstetrical brachial plexus
palsy will be detailed together with key technical
tips in the perioperative management of these
children. Parents must be adequately counseled
regarding the risks and realities of surgical inter-
vention, with appropriate management of paren-
tal expectations regarding likely functional goals
and the potential need for secondary surgery.
Introduction
Historical Perspectives
The concept of brachial plexus palsy arising as a
result of a traction injury during the birthing pro-
cess was rst mooted in the eighteenth century
(Smellie 1768). Advances in the eld of electro-
physiology enabled Duchenne to formally map
the muscles affected by obstetrical brachial plexus
palsy (Duchenne 1872). Erb was the rst to local-
ize the pathology to the upper cervical roots in the
palsy which later bore his eponym (Erb 1874); the
description of a clinicopathological variant
whereby the lower cervical roots were affected
soon followed (Klumpke 1885).
Kennedy provided the rst detailed operative
description of plexal neuroma excision with pri-
mary suture repair (without the aid of an operating
microscope) in a small cohort of patients with
upper trunk obstetrical brachial plexus lesions; ten-
sion was minimized by splinting the head to the
affected shoulder for a period of 2 weeks postop-
eratively (Kennedy 1903). Larger series of primary
plexus repairs followed (Wyeth and Sharpe 1917;
Taylor 1920), although subsequent concerns as to
the limited functional improvement following early
surgical repair, coupled with a substantial
M.C. Swan and H.M. Clarke
associated perioperative morbidity and mortality,
led to a trend towards nonoperative management
(Sever 1925; Jepson 1930). Surgical intervention
was chiey reserved for the correction of second-
ary soft tissue and bony deformities. Seddons dis-
appointing experience with autologous nerve cable
grafts in a small series of upper trunk reconstruc-
tions in adult brachial plexus traction injuries
reected the pessimism of earlier reports where
primary repair was performed (Seddon 1947).
In the 1970s, the advent of the operating micro-
scope facilitated markedly greater surgical preci-
sion and nesse in peripheral nerve surgery, which
led to a revolution in the surgical management of
obstetrical brachial plexus palsy (Millesi
et al. 1973; Gilbert et al. 1980). The trend towards
early intervention with neuroma excision and
autologous sural nerve interpositional cable grafts
was becoming established, with Gilbert recogniz-
ing the absence of recovery in bicep function as an
important prognostic indicator in children with
obstetrical brachial plexus palsy (Gilbert
et al. 1988, 1991). In parallel to the use of
interpositional nerve grafts, microsurgical tech-
niques expanded to include extra-plexal
neurotization from the intercostal nerves (Celli
et al. 1978), spinal accessory nerve (Allieu
et al. 1982), phrenic nerve (Gu and Ma 1996),
hypoglossal nerve (Malessy et al. 1999), and the
contralateral C7 nerve root (Gu et al. 1998).
Although beyond the scope of this chapter,
microsurgical techniques have also been utilized
in the secondary restoration of upper extremity
function by means of vascularized muscle trans-
fer; the gracilis muscle may be used to restore
elbow or hand function in global plexopathy,
being motored by either an intra-plexal or extra-
plexal donor (Ikuta et al. 1980; Chuang 1995).
The concept of augmenting brachial plexus func-
tion by means of a free vascularized nerve graft
may also have a role in selected cases (Taylor and
Ham 1976; Terzis and Kostopoulos 2010).
Epidemiology
The reported incidence of obstetrical brachial
plexus palsy varies between 0.5 and 3 cases per
28 Microsurgery for Obstetrical Brachial Plexus Palsy
1,000 live births (Specht 1975; Hardy 1981;
Levine et al. 1984; Sjoberg et al. 1988; Michelow
et al. 1994; Gilbert et al. 1999). Most authorities
agree that injury results from the birthing process
and that the likely mechanism is a forced increase
in the neck-shoulder angle, which results in lon-
gitudinal traction on the brachial plexus. Contro-
versy exists as to the relative origin of the forces
necessary to cause plexus injury whether from
intrinsic uterine contractions, iatrogenic manipu-
lations (including instrumentation), or external
compression from a hematoma or associated frac-
ture (Jennett et al. 1992; Borschel and Clarke
2009). It has been hypothesized that a clavicular
fracture imparts a protective effect on the under-
lying brachial plexus (Metaizeau et al. 1979);
however, a clavicular fracture has no prognostic
value in predicting the likelihood of spontaneous
neurologic recovery in newborns with obstetrical
brachial plexus palsy (Al-Qattan et al. 1994).
The risk factors for obstetrical brachial plexus
palsy are well understood and include maternal
gestational diabetes, a postdate pregnancy, use of
epidural anesthesia, shoulder dystocia, forceps
delivery, or vacuum extraction (Gherman
et al. 1999). The majority of obstetrical brachial
plexus injuries arise from a vertex vaginal deliv-
ery with an associated shoulder dystocia late in the
second stage of labor. The risk of shoulder dysto-
cia increases with fetal macrosomia: in children
weighing 2.53.5 kg, the incidence of shoulder
dystocia is 22 %; however, this increases to 74 %
when birth weight exceeds 4.5 kg (Gilbert
et al. 1999). Nonetheless, a quarter of brachial
plexus palsies occur in the absence of recorded
shoulder dystocia (Perlow et al. 1996). Other
independent risk factors include breech presenta-
tion, multiparity, and a history of a previous child
with obstetrical brachial plexus injury (Sjoberg
et al. 1988). The plexopathy resulting from breech
delivery tends to include avulsion injuries of the
upper roots (C5 and C6). Prematurity and intra-
uterine growth restriction are considered protec-
tive factors (Gilbert et al. 1999). Although
caesarian section signicantly reduces the risk of
plexus trauma, approximately 1 % of obstetrical
brachial plexus injuries occur following caesarian
section (Al-Qattan et al. 1996). In the context of
609
an emergency caesarian section, it is debated
whether the palsy arises from the initial attempt
at vaginal delivery or from the extraction of the
neonate from the uterus. It has been observed that
the incidence of obstetrical brachial plexus palsy
decreased over the twentieth century, a fact that
has been attributed to improved obstetric care
(Adler and Patterson 1967; Seddon 1975; Bennet
and Harrold 1976). More recently, Birch has
suggested that the incidence in the United King-
dom is rising, which may reect the trend towards
increasing birth weight (Gupta et al. 2000).
In the majority of cases (7085 %) of obstetri-
cal brachial plexus palsy, the clinical presentation
is that of an upper cervical root injury (i.e., an
Erb-Duchenne palsy), with fewer than 5 % of
cases being an isolated lower root (i.e., Klumpke
palsy) injury and the remaining 1025 % being
total plexus injury (Al-Rajeh et al. 1990; Laurent
and Lee 1994; Al-Qattan et al. 1995). Al-Qattan
argues that in the context of a breech presentation,
the majority of births are by cesarean section and,
in those whom do undergo a vaginal delivery, the
attention paid to preventing hyperabduction of the
shoulder renders the risk of a Klumpkes palsy
extremely low (less than 1 %) (Al-Qattan
et al. 1995). In unilateral lesions there is a prepon-
derance of right-sided cases (Hardy 1981; Sjoberg
et al. 1988). Bilateral lesions are observed in
approximately 5 % of cases and are more com-
monly seen in breech presentations (Al-Rajeh
et al. 1990).
Wide variation exists in the rate of complete,
spontaneous recovery following obstetrical bra-
chial plexus palsy, with confounding factors
including patient selection bias (as those with
mild symptoms may never be referred to a spe-
cialist unit) and the denition as to what consti-
tutes a complete recovery. The cited range of
complete functional recovery varies between
13 % and 95 % of patients (Wickstrom
et al. 1955; Gordon et al. 1973; Greenwald
et al. 1984; Piatt 1991). An early spontaneous
recovery is associated with an improved progno-
sis, with the Collaborative Perinatal Study
reporting that 93 % of patients who went on to
full spontaneous recovery had done so by
4 months of age (Gordon et al. 1973). Poor
610
Fig. 1 Schematic anatomy of the right brachial plexus (Marcus and Clarke 2008) (Courtesy of Quality Medical
Publishing)
prognostic indicators for spontaneous recovery
include a ail arm at birth, avulsion injuries,
lower plexus injuries, Horner syndrome, and
phrenic nerve palsy (Brown 1984; Narakas
1987; Jackson et al. 1988; Geutjens et al. 1996).
Pathoanatomical Considerations
The applied anatomy of the brachial plexus is
covered in chapter Supracondylar Humerus
Fracture. In spite of its apparent complexity, the
gross anatomy is remarkably consistent (Fig. 1),
being formed by the spinal roots of C5-T1, with
important variations including the prexed and
M.C. Swan and H.M. Clarke
postxed plexus with contributions from the
neighboring C4 and T2 nerve roots, respectively.
The plexus may occasionally be shifted by some
fraction of a complete segmental level. The roots
of the brachial plexus converge between the
anterior and middle scalene muscles to create the
trunks: the upper trunk being formed from C5 and
C6, the middle trunk from C7, and the lower trunk
from C8 and T1. Each trunk forms anterior and
posterior divisions behind the clavicle. These then
merge deep to the pectoralis minor muscle to
create the cords that are named according to
their relationship to the axillary artery: the lateral
cord is formed from the anterior divisions of the
upper and middle trunks, the medial cord is
the continuation of the anterior division of
28 Microsurgery for Obstetrical Brachial Plexus Palsy 611

the lower trunk, and the posterior cord is In axonotmesis (i.e., axonal damage with
formed by the union of all three posterior intact Schwann cell basal lamina), a partial
divisions. The proximal (i.e., shoulder girdle) spontaneous recovery might be expected.
muscles are innervated by branches of the roots, Neurotmesis may either represent a cord
trunks, and cords, while the muscles of the arm avulsion injury in the context of a
and forearm are supplied by the terminal branches preganglionic injury or complete disruption
of the brachial plexus: the median nerve (formed (rupture) of nerve continuity in a postgan-
from contributions from the lateral and medial glionic lesion; in the latter scenario, recovery
cords), the ulnar and musculocutaneous nerves is not possible without surgical intervention.
(terminal branches of the medial and lateral In practice, the picture is invariably more
cords), and the radial and axillary nerves (both complex, with multiple degrees of nerve
terminal branches of the posterior cord) (Marcus injury occurring within the same patient
and Clarke 2008). (Mackinnon and Dellon 1988). Indeed, the
A number of means of classifying the obstetri- classic nding of a neuroma-in-continuity
cal brachial plexus injury exist: has been described as a Sunderland VI
injury as it is typically a mixture of the
(a) Whether it lies in the supraclavicular (as in the other ve Sunderland types.
majority of cases) or infraclavicular plexus (d) Based on earlier work by Gilbert and Tassin,
(which may necessitate an alternate surgical Narakas classied obstetrical brachial plexus
approach). palsy into four groups, which reected the
(b) Whether the injury is preganglionic or post- histopathologic Sunderland classication.
ganglionic the latter being more common in Stratication is based on the neonatal clinical
the lower nerve roots due to them having less examination ndings at 24 weeks after birth,
robust connective soft tissue attachments to by which time neurapraxic injuries will have
the neural foramina. A preganglionic root begun to recover (Table 1) (Gilbert and Tassin
avulsion injury results in a permanent motor 1984; Narakas 1986, 1987).
and sensory decit in the corresponding der-
matome and is not currently amenable to pri-
mary surgical repair as the dorsal root
ganglion is avulsed from the spinal cord and
the ventral rootlets are separated from the Table 1 The Narakas classification of obstetrical birth
palsy (Narakas 1986, 1987) based on previous work by
anterior horn cells. This is readily evident Gilbert and Tassin (1984)
intraoperatively if the dorsal root ganglion
Approximate rate of
has retracted extraforaminally, although can
Affected nerve roots complete spontaneous
appear deceptively normal if it remains within Group and clinical ndings recovery (%)
the bony limits of the foramina. Postgangli- I C5, C6 90
onic tears are termed ruptures and are usually Paralysis of
amenable to surgical repair as the dorsal root shoulder and biceps
ganglion is preserved proximally (with the II C5, C6, C7 65
distal nerve undergoing Wallerian Paralysis of
degeneration). shoulder, biceps,
and forearm
(c) According to the description by Seddon of a extensors
nerve injury being either a neurapraxia, III C5, C6, C7, C8, T1 <50
axonotmesis, or neurotmesis (Seddon 1943). Flaccid limb
This classication was further expanded to IV C5, C6, C7, C8, T1 0
ve degrees of injury by Sunderland (1951). Flaccid limb and
Complete recovery would be expected from a Horner syndrome
neurapraxia as axonal integrity is not violated.
612
Clinical Assessment
An early referral to a specialist brachial plexus
multidisciplinary clinic is encouraged (Borschel
and Clarke 2009). The diagnosis of obstetrical
brachial plexus palsy is primarily one of clinical
acumen; not infrequently a child may need to
return for repeated assessment if compliance was
lacking at the initial consultation. The clinician
must exclude diagnoses that may potentially
mimic obstetrical brachial plexus palsy such as
underlying intracranial pathology (including cere-
bral palsy and brain tumors), a radial nerve palsy,
or a fracture or dislocation involving the shoulder
girdle or upper extremity (pseudoparalysis)
(Alsubhi et al. 2011). Plain radiographs should
be requested if there is suspicion of an associated
bony injury or joint dislocation. Soft tissue con-
tractures tend not to develop until some months
after birth; thus any limitation in the passive range
of motion of the upper extremity during neonatal
assessment should raise the suspicion of underly-
ing musculoskeletal pathology. For example,
injury to the proximal humeral epiphyses should
be suspected in a child when both active and
passive motion of the arm are equally restricted;
a plain radiograph should conrm the diagnosis
(Adler and Patterson 1967). A thorough birth
history including predisposing risk factors and
postnatal complications (including respiratory
distress, associated fractures, torticollis, or
Horners syndrome) is taken as detailed in
chapter Supracondylar Humerus Fracture.
Clinical assessment is best performed with the
infant placed on a clean sheet on a padded mat at
oor level (or low examination bench) as this facil-
itates efcient examination in a safe environment.
Clothing above the childs waist should be removed
and particular care taken to note the head posture;
children with obstetrical brachial plexus palsy tend
to look to the contralateral side. The presence of
torticollis should be noted; this may result from
birth trauma or as a secondary phenomenon due to
positioning of the child (invariably, but incorrectly,
nursed with the affected extremity uppermost). The
lower extremities must also be examined in order to
exclude a hemiparesis or neonatal tetraplegia.
M.C. Swan and H.M. Clarke
The posture of the upper extremity gives
invaluable information as to the level of the plexus
lesion. Shoulder adduction and internal rotation
with the elbow in extension, the forearm pronated,
and wrist/ngers held in exion (the waiters tip
position) is highly suggestive of an Erb-Duchenne
upper plexus (C5, C6
C7) injury. Recent evi-
dence indicates that the diagnostic specicity of
the waiters tip posture in infants is less than
was originally assumed, as the only absolute
requirement to produce a waiters tip posture
is an intact T1 nerve root (Fattah et al. 2012).
A ail limb, with the absence of any motor activ-
ity, implies a total plexus palsy (C5-C8, T1). Less
common phenotypes include a Klumpke lower
plexus (C8, T1) palsy (a accid hand in an other-
wise functioning upper extremity), an intermedi-
ate (C7, C8, T1) palsy (with an abducted shoulder,
exed elbow, and accid hand), and an isolated
C7 palsy (with the elbow held in a exed posture).
Physical signs that would support the diagnosis
of an upper plexus injury include winging of the
scapula (due to paralysis of the long thoracic
nerve of Bell; root value C5-C7), although assess-
ment of the scapulae in neonates is not possible. In
addition, paradoxical breathing between the chest
and abdomen could be the result of an associated
phrenic nerve injury (root value C3-C5; although
the phrenic nerve typically contributes to C5)
though this is impossible to detect clinically. An
ipsilateral Horner syndrome (characterized by
ptosis, miosis, anhidrosis, and enophthalmos)
can be seen on routine examination and indicates
disruption of T1 proximal to the sympathetic rami
communicantes (i.e., a preganglionic avulsion
injury) (Fig. 2).
A thorough motor examination of the upper
extremity is central to conrming the diagnosis,
prognosis, and subsequent treatment plan and
probably represents the most challenging element
of the examination. Traditionally, the Medical
Research Council muscle grading scale (Table 2)
has been used (Medical Research Council 1943);
however, its use in infants is limited on account of
their inability to fully cooperate with the exami-
nation, and thus the authors favor the validated
Active Movement Scale developed at The Hospi-
tal for Sick Children (Table 3) (Clarke and Curtis
28 Microsurgery for Obstetrical Brachial Plexus Palsy 613

Table 3 The Hospital for Sick Children Active Move-

ment Scale (Clarke and Curtis 1995)
Observation Muscle grade
Gravity eliminated
No contraction 0
Contraction, no motion 1
Motion  range 2
Motion > range 3
Full motion 4
Against gravity
Motion  range 5
Motion > range 6
Full motion 7

voluntary strength. It incorporates gravity as a

Fig. 2 Four-month-old child with left global brachial birth
palsy and Horners syndrome (Courtesy of Shriners Hos-
pital for Children, Philadelphia)
standard; movements are tested both against grav-
ity and with gravity eliminated in order to docu-
ment early recovery with precision. The Mallet
scale (Fig. 3), which was developed to provide a
quantiable assessment of shoulder function in
obstetrical brachial plexus palsy, is also widely
used although it demands a degree of patient
compliance that negates its routine use in children
under the age 3 years (Mallet 1972).

Table 2 Medical Research Council muscle grading scale Indications for Surgical Exploration
(Medical Research Council 1943)
Muscle It is generally accepted that in those children who
Observation grade meet the criteria for surgical intervention, treat-
No contraction 0 ment should not be delayed, as this is likely to
Flicker or trace of contraction 1 prejudice the maximal benet that could other-
Active movement, with gravity 2 wise be obtained. The challenge facing brachial
eliminated plexus surgeons has been to evolve the ability to
Active movement, against gravity 3 statistically prognosticate which children are most
Active movement, against gravity and 4 likely to benet from surgical intervention versus
resistance
those who are best managed expectantly. Clearly,
Normal power 5
surgical intervention should only be warranted if
the outcome after intervention is likely to result in
1995; Curtis et al. 2002). Fifteen cardinal motions enhanced long-term function compared to
of the upper extremity are assessed, including nonoperative management; such a judgment
those of the shoulder (abduction, exion, internal requires a sound understanding of the natural his-
rotation, and external rotation), elbow (exion tory of obstetrical brachial plexus palsy. Identi-
and extension), forearm (pronation and supina- cation is straightforward in the scenario of an
tion), and wrist, ngers, and thumb (exion and infant with a ail limb and an associated Horner
extension). The Active Movement Scale measures syndrome; equally, surgery is contraindicated in a
motion within the range of joint motion, not child with mild paralysis who recovers substantial
614 M.C. Swan and H.M. Clarke

Fig. 3 The Mallet method of assessing upper extremity function (Chung et al. 2012) (Courtesy of Elsevier Saunders)
28 Microsurgery for Obstetrical Brachial Plexus Palsy
motor function within the rst month as near
complete functional recovery would be antici-
pated (Al-Qattan et al. 2000). Clinical evidence
of an isolated T1 avulsion (i.e., a limp hand resting
in the intrinsic minus position) is a reliable indi-
cator for operative intervention within the rst
3 months. Despite accepting that the majority of
children with obstetrical brachial plexus palsy will
not require surgical exploration, there is currently
no universal consensus as to how to ascertain
which children will with reasonable certainty
require surgery.
Tassin noted that complete recovery of shoul-
der function at the age of 5 years (Grade V in the
Mallet classication system) was only seen in
those children with contraction of biceps and del-
toid muscles in the rst month and normal con-
traction of both muscles by the age of 3 months
(Tassin 1983). Gilbert and coworkers found test-
ing the deltoid problematic in neonates (due to
difculty in isolating its action from pectoralis
major activity) and therefore generated three
modied criteria for surgical intervention: a ail
arm with Horner syndrome, a complete C5C6
palsy without muscle contractility by 3 months
and a negative EMG (thus suggestive of a
root avulsion), and a C5C6 palsy with no biceps
activity at 3 months (Gilbert et al. 1988).
Failure of spontaneous return of elbow exion at
3 months of age is now the most widely
accepted criteria for surgical intervention in
infants with an upper plexus lesion (Gilbert and
Whitaker 1991; Gilbert 1995; Eng et al. 1996;
Gilbert et al. 2006).
Waters conrmed the ndings of Tassin and
Gilbert that infants in whom biceps function
recovered by 3 months of age progressed to
achieve normal neurologic function, while those
whose biceps recovery occurred late (i.e., from
4 to 5 months of age) had a worse long-term
recovery according to the Mallet classication
(Waters 1999). Those infants who had microsur-
gical brachial plexus reconstruction for absent
biceps recovery by 6 months of life had superior
function to those who experienced late
615
spontaneous biceps recovery, but their function
was not as good as those in whom elbow exion
recovered early.
However, Michelow et al. determined that
overreliance on a single functional parameter
alone at the age of 3 months would incorrectly
predict a poor recovery in 12.8 % of patients,
additionally implying that some children were
being reconstructed unnecessarily. This false-
positive rate could be reduced to 5.2 % if elbow
exion and elbow, wrist, thumb, and nger exten-
sion were incorporated into a combined test score.
The utility of adopting multiple muscle assess-
ments as an indicator for surgical intervention is
becoming increasingly accepted and should lead
to a reduction in unnecessary surgery, although a
more conservative threshold may result in some
children who would benet from surgery ulti-
mately being managed nonoperatively (Laurent
et al. 1993).
The importance of repeat assessment has been
promoted by Clarke and Curtis as a small propor-
tion of infants demonstrating evidence of return of
elbow function at 3 months may then fail to pro-
gress considerably with an ultimately suboptimal
recovery, thus endorsing the concept of
reassessment at 3-monthly intervals (Clarke and
Curtis 1995). The algorithm used for the assess-
ment and management of infants presenting with
obstetrical brachial plexus palsy at The Hospital
for Sick Children is detailed in Fig. 4. An initial
assessment is undertaken at 3 months using the
Active Movement Scale (Table 3); a converted
test score (the sum of the ve scores for elbow
exion and extension of the elbow, wrist, ngers
and thumb after conversion according to the
values in Table 4) less than 3.5 is strongly predic-
tive of a poor functional outcome and surgical
reconstruction is therefore recommended. Those
children with a test score of 3.5 or greater, with an
intact T1 and no evidence of a Horner syndrome,
are observed and undergo daily physical therapy
to maintain passive range of motion; they are
subsequently reassessed a 3-month interval. In
the scenario of a child who initially demonstrates
616 M.C. Swan and H.M. Clarke

Fig. 4 Flow diagram for

the assessment and
management of obstetrical
brachial plexus palsy at The
Hospital for Sick Children
(Courtesy of Dr. H. M.
Clarke, The Hospital for
Sick Children)

biceps function at the 3-month assessment, but
then fails to meaningfully progress by the
6-month assessment (i.e., similar test scores are
obtained), then it is likely that this heralds a poor
functional outcome and surgical intervention
should be considered.
At the 9-month assessment, the child under-
takes the cookie test in an attempt to identify
those in whom a good early recovery (i.e., a test
score of 3.5 or greater) failed to predict adequate
elbow exion by the end of the rst year (Clarke
and Curtis 1995; Curtis et al. 2002). If the child
fails to achieve greater than half the range of
elbow exion against gravity (i.e., an Active
Movement Score of at least 6), then exploration
of the brachial plexus is advocated (Fig. 5).
28 Microsurgery for Obstetrical Brachial Plexus Palsy
Table 4 The Hospital for Sick Children Active Move-
ment Scale conversion for use in calculating the interval
test score (Clarke and Curtis 1995)
Muscle grade (from Active Movement Converted
Scale) score
0 0
1 0.3
2 0.3
3 0.6
4 0.6
5 0.6
6 1.3
7 2.0
Fig. 5 Failed cookie test at 9 months of age: the child
fails to achieve greater than half the range of elbow exion
against gravity (Courtesy of Dr. H. M. Clarke, The Hospital
for Sick Children)
In practice, the test is performed with the child
either seated or standing with the elbow held
adducted against the trunk by the examiner to
negate the trumpet sign; a small cookie is placed
617
into the hand of the child, and they are encouraged
to eat the cookie by actively exing their elbow
against gravity. The test is considered void if there
is excessive neck exion (greater than 45 ).
Application of the test score should never sup-
plant sound clinical judgment; it is intended as a
guide to early treatment.
Preoperative Investigations
In those patients who require surgical exploration,
a preoperative diaphragmatic ultrasound is
performed to document phrenic nerve function
(Borschel and Clarke 2009). A chest radiograph
is recommended if considering extra-plexal
neurotization from the intercostal nerves (Slooff
et al. 2001). Additionally, a computed tomo-
graphic myelography is undertaken to determine
the probability of a root avulsion injury: the pres-
ence of a pseudomeningocele without demonstra-
ble ventral rootlets indicates (with a specicity of
0.98) that the nerve root is probably
avulsed (Chow et al. 2000). Pseudomeningoceles
indicate an underlying dural injury, and in
isolation, they are not absolute proof of a root
avulsion injury discontinuity of the rootlets
should be identied in both axial and coronal
planes to conrm the diagnosis of a preganglionic
injury. The necessity for general anesthesia,
intrathecal instillation of contrast medium, and
exposure to ionizing radiation has driven the
application of magnetic resonance imaging
(MRI) in the radiologic assessment of the
infant brachial plexus as it is noninvasive,
nonionizing, and can be performed under sedation
alone. However, MRI currently lacks the higher
spatial resolution of CT myelography in identify-
ing individual nerve roots (Caranci et al. 2013),
and the quality of the images appears to vary
widely across centers. Many specialist units do
not routinely image the brachial plexus save
for specic indications such as a suspected upper
root avulsion injury following a breech delivery
(Gilbert 1995; Al-Qattan 2004; Shenaq
et al. 2004).
618 M.C. Swan and H.M. Clarke

Electrodiagnostic studies have been used to Table 5 Preoperative planning checklist for brachial
provide information on the location, severity, plexus exploration and reconstruction with bilateral sural
nerve harvest
and extent of the obstetrical brachial plexus
palsy. Although more challenging to perform in Preoperative planning checklist
infants, nerve conduction studies (NCS) may help Operating table Table reversed in order that the
patient not resting on break in the
to differentiate between preganglionic and post- table
ganglionic plexus injuries. Motor and sensory Airway Nasal intubation secured with
nerve responses (conduction velocities and distal silk suture
latencies) vary signicantly with age and in neo- Transparent drape
nates are approximately 50 % the magnitude of Patient position
those seen in adults (Miller and Kuntz 1986). a. Bilateral sural a. Prone with abdominal
Electromyography (EMG) is not routinely used nerve harvest support
in the assessment of obstetrical brachial b. Brachial b. Supine with shoulder roll in
plexus exploration situ
plexus palsy; Gilbert et al. found that EMG
Tourniquet Sterile; placed on bilateral
ndings correlated poorly with prognosis, proximal thighs
although evidence of brillation on the back- Sterile Esmarch bandages
ground of absent voluntary motor unit activity Pressure care Egg crate mattress
was suggestive of a root avulsion injury (Gilbert All bony prominences padded
et al. 1988). Overall, there is reasonable agree- Temperature Rectal temperature probe
ment that electrodiagnostic studies are overly homeostasis Access to forced-air warming if
optimistic in the setting of obstetrical brachial required
plexus palsy and are therefore not used at The Fluid management Urinary catheter in situ
Hospital for Sick Children, Toronto (Vredeveld Total uid intake 4 ml/kg/h
Specic equipment
et al. 1999).
a. Sural nerve a. 0 4 mm endoscope
harvest
b. Assessment of b. Nerve stimulator
brachial plexus
Surgical Exploration c. Microsurgery c. Operating microscope and
and Reconstruction instruments
d. Nerve grafts d. Fibrin sealant
Intraoperative Setup Miscellaneous a. Ligaclip applicators
b. Book frozen section with a
neuropathologist
The working relationship between the surgeon,
c. 2-inch stockinette and
anesthesiologist, and theatre scrub staff is essen- safety pins for Velpeau splint
tial; the procedure is lengthy and complex, and
thus communication within the team is vital with
the perioperative safety of the child being of par-
amount importance. Factors that deserve specic forewarned that fresh specimens will be obtained
attention include the duration of the procedure, for frozen section and will require urgent
which is typically between 6 and 12 h, with the processing. The key considerations in the preoper-
attendant risks of patient hypothermia, ative planning are outlined in Table 5.
pressure area necrosis, and uid imbalance; the Following nasal intubation with an uncuffed
availability of only one extremity for intravenous endotracheal tube, the airway is secured by sutur-
access and noninvasive monitoring purposes ing the tube to the membranous nasal septum with
(as both lower extremities are usually prepped for a 3-0 silk suture to minimize the risk of inadver-
sural nerve harvest); and the need to avoid muscle tent extubation. The child is catheterized with a
paralysis if intraoperative nerve stimulation is to be size 8 Foley for the convenience of the team; it is
employed. The neuropathology laboratory is only rarely advised to treat low urine output with a
28 Microsurgery for Obstetrical Brachial Plexus Palsy
Fig. 6 Intraoperative
positioning of the infant for
a right brachial plexus
exploration: note the use of
a clear drape allows
visualization of the
endotracheal tube (Courtesy
of Dr. H. M. Clarke, The
Hospital for Sick Children)
uid bolus. Prophylactic intravenous cefazolin is
administered, with the dose repeated every 4 h for
the duration of the procedure. A rectal tempera-
ture probe is used to monitor core temperature
throughout the operation.
Bilateral Sural Nerve Harvest
In most situations the surgeon will be condent
that sural nerve grafting is necessary and the
procedure begins with the child in the prone
position in preparation for bilateral sural nerve
harvest. An abdominal bolster is used to facilitate
patient positioning with meticulous care taken to
pad all pressure areas appropriately. Endoscopic-
assisted sural nerve harvesting is used to mini-
mize donor site scarring (Capek et al. 1996). The
harvests are performed under tourniquet control
using three 2-cm incisions for access at the level
of the lateral malleolus, the distal belly of the
gastrocnemius muscle (anticipating the point at
which the nerve becomes subfascial in the
mid-calf), and the popliteal fossa. Endoscopic
visualization can facilitate identication of the
entire sural nerve from the most proximal origin
at the tibial nerve to the distal arborizations at the
lateral aspect of the foot, together with the
contributing lateral (peroneal) branch, which is
also harvested if present. Approximately 15 cm
of sural nerve can be harvested from each leg in a
10 kg infant. The nerves are marked with ink in
619
order to aid orientation and stored in a damp
sterile container in a refrigerator until later
required in the reconstruction. Sural nerve
harvesting in children leaves a measurable sen-
sory decit; however, this does not appear to
have signicant clinical implications (Lapid
et al. 2007).
Approach to the Brachial Plexus
On completion of the sural nerve harvest, the child
is turned supine and placed at the extreme head of
the table, close to the lateral edge, in order to
facilitate access for both the surgeon and assistant
(Fig. 6). A small padded roll is placed under the
scapulae in order to provide a modest degree of
neck extension; the head is turned fully away from
the affected side. The upper extremity is free
draped in order to facilitate observation of the
entire limb during intraoperative nerve stimula-
tion. Clear draping of the patients head enables
the anesthesiologist to continually visualize the
endotracheal tube.
The vast majority of infant plexus explorations
are performed via a supraclavicular approach in
the posterior triangle of the neck. A variety of
different incisions are used in order to access the
brachial plexus, with many surgeons favoring a
single transverse incision just above the clavicle
(Borrero 2001). Excellent exposure to the con-
tents of the posterior triangle of the neck is
620 M.C. Swan and H.M. Clarke

Table 6 A step-by-step approach to exploration of the brachial plexus

Surgical steps
Tattoo preoperative markings to facilitate subsequent wound closure
Elevate skin ap in subplatysmal plane and reect superolaterally
Divide clavicular head of the sternocleidomastoid and external jugular vein
Identify the cervical plexus and C4 nerve root; divide supraclavicular branches
Divide omohyoid muscle and reect Browns fat pad off the clavicle
Divide the transverse cervical and suprascapular artery and vein
Identify neuroma/plexus between anterior and middle scalene muscles
Identify phrenic nerve and perform neurolysis if required
Using the C4 marker, identify the C5 root and dissect proximally to the foramen to exclude a preganglionic avulsion
Sequentially identify and dissect the C6 root and foramina, assess for a preganglionic avulsion
Dissecting antegradely along the lateral border of the brachial plexus to identify the suprascapular nerve and the upper
and middle trunks
Identify the remaining nerve roots and foramina, taking care to protect the subclavian artery and parietal pleura, assess
for a preganglionic avulsion
Identify the lower trunk of the brachial plexus and its branches distally
Review the operative ndings
1. Which roots appear intact
2. The position and length of any neuromata that require excision
3. The position of distal plexus targets requiring nerve grafting
4. Length of sural nerve (
supraclavicular nerve) graft available
Perform intraoperative nerve stimulation to help differentiate between an intra-foraminal root avulsion and an intact
nerve root if required
Prepare for neuroma excision and stump sampling for frozen section
Calculate amount of sural nerve graft required to reconstruct resultant defect and consider options for intra-plexal or
extra-plexal neurotization
Cut grafts to length for proposed plexus reconstruction in a tension-free manner; prepare brin sealant
Ensure meticulous hemostasis within the wound bed; no further irrigation during gluing of grafts
Glue grafts in situ using a brin sealant; ensure optimal orientation using an operating microscope
Wound closure to include redraping of Browns fat pad, repair of omohyoid muscle, and reattachment of
sternocleidomastoid muscle
No surgical drain required
Skin closure in layers using absorbable sutures; inltrate local anesthesia
Simple wound dressing; application of Velpeau sling

afforded by a superiorly based V-shaped incision
with the medial limb running along the posterior
border of the sternocleidomastoid muscle and
then gently curving at its apex to create the lateral
limb which runs parallel to the superior border of
the clavicle (with the potential to extend into the
deltopectoral groove should this be necessary to
access the more distal plexus). A step-by-step
description of this surgical approach is listed in
Table 6. The tip and edges of the skin ap are
tattooed with surgical ink to facilitate accurate
wound alignment at the time of wound closure.
Subsequent dissection is performed under loupe
magnication in a subplatysmal plane with the
skin ap being anchored superolaterally beyond
the surgical eld by means of a hemostat. The
clavicular head of the sternocleidomastoid muscle
is divided near its origin to facilitate exposure; the
overlying external jugular vein is frequently
divided if it obstructs the surgical eld.
The branches of the cervical plexus are evident
at the lateral border of the sternocleidomastoid
muscle; the supraclavicular branches are divided
as they cross the clavicle (and may be used as
28 Microsurgery for Obstetrical Brachial Plexus Palsy
supplemental graft material), while the great
auricular and lesser occipital nerves are preserved.
Retrograde tracing of these branches enables
ascertainment of the C4 nerve root, which acts as
a sentinel for identication of the roots of the
brachial plexus. The omohyoid muscle is then
identied and divided at its tendinous midportion;
the divided ends are tagged with 40 polyglactin
sutures that are used to reect the muscle out of
the surgical eld and, later, to repair the muscle at
the time of wound closure. The omohyoid is a
useful landmark for the suprascapular notch and
thus the suprascapular nerve. Deep to omohyoid is
adipolymphatic tissue (the fat pad of Brown),
which is dissected free of the lateral border of
the sternocleidomastoid muscle and reected
superolaterally in order to access the plexus
proper. The transverse cervical artery and vein
(which lie centrally within the surgical eld) and
the suprascapular artery and vein (normally at the
superior border of the clavicle) are divided to
facilitate surgical access. Division of the clavicle
is not required as adequate exposure of the plexus
can be achieved with simple retraction; this also
avoids the presence of bony callus in the vicinity
of the reconstructed plexus and the potential risk
of bony instability.
At this stage of the dissection, the plexus is
usually quite evident as an off-white rm mass
that arises between the anterior and middle sca-
lene muscles, to which it is often tightly adherent.
The phrenic nerve is identied as the only nerve
that courses from lateral to medial as it descends
the neck; it is often heavily scarred to the anterior
surface of the neuroma. As the phrenic nerve
approaches the lateral edge of the anterior scalene,
the C5 spinal nerve root emerges; it commonly
makes a contribution to C5 (as evidenced by the
observation that the nerve is thicker proximal to
the C5 root and thinner distally), and its identity is
conrmed by means of a nerve stimulator and the
resultant diaphragmatic twitch that ensues. The
phrenic nerve is dissected free of the neuroma
(frequently necessitating division of the contribu-
tion to the C5 nerve root), and the scalene muscles
must also be liberated. Care is taken to preserve
the long thoracic nerve of Bell, which lies poste-
rior to the neuroma within the substance of the
621
middle scalene muscle, although contributions to
the long thoracic nerve may subsequently be
divided in order to resect the neuroma closer to
the foramen.
Having conrmed the location of the C5 root
either by its relationship to the C4 root
(ascertained by retrograde tracing of the
supraclavicular nerves) or by the direct contribu-
tion made by the phrenic nerve it is dissected
proximally to the bony foramen, often requiring
partial excision of the anterior and middle scalene
muscles to aid access. The C6 and C7 roots are
then dissected free in a similar manner, noting that
each successive foramen appears progressively
closer than the previous one. In the scenario of
an empty foramen, a root avulsion is probable,
which is proven by attempted identication of
the bulbous dorsal root ganglion (with subsequent
biopsy and histological conrmation).
The upper and middle trunks, which are fre-
quently matted within scar, should then be identi-
ed. Dissection along the lateral border of the
plexus in a proximal-to-distal fashion allows iden-
tication of the suprascapular nerve, which is
classically described as arising from the upper
trunk, but more commonly originates from the
posterior division of the upper trunk (Arad
et al. 2014). If the origin of the suprascapular
nerve arises proximal to the neuroma, then it is
preserved; however, it is frequently found to arise
from the neuroma itself, in which case it is divided
and the epineurium carefully tagged with a 60
polypropylene suture as an independent distal tar-
get for nerve grafting. If the dorsal scapular and
nerve to subclavius are found arising from neu-
roma, they are usually sacriced. Dissection con-
tinues distally, identifying the remaining branches
including the lateral pectoral nerve, until it pro-
ceeds beyond the neuroma and normal plexus is
identied. The lateral pectoral nerve most com-
monly arises from the anterior division of the
upper trunk proximal to the point of lateral cord
formation rather than the conventional description
of the origin being the lateral cord proper (Arad
et al. 2014).
Identication of the C8 and T1 roots is aided
by inferior retraction of the clavicle; care is taken
not to violate the dome of the parietal pleura,
622
which lies immediately deep to the T1 nerve root.
The roots are then traced proximally in order to
identify the respective foramina, which often
necessitates gentle release of the subclavian vein
as it passes above the lower trunk. The dissection
then proceeds distally with each branch of the
plexus being identied and preserved until normal
plexus is identied beyond the distal extent of the
neuroma.
The dorsal scapular artery is often divided to
improve surgical access and is also a useful ana-
tomical marker. It usually arises from the trans-
verse cervical artery, in which case it passes either
posterior or superior to the brachial plexus; how-
ever, when arising from the third part of the sub-
clavian artery, it most frequently passes between
the upper and middle trunks (Chaijaroonkhanarak
et al. 2014).
Following the initial dissection, the objective is
to have identied the extent of the plexal neuroma
and to have macroscopic exposure of normal
plexus distally. Proximally, the diagnosis of a
preganglionic avulsion injury is irrefutable in the
context of an empty foramen, with nerve root
laments and the dorsal root ganglion visible dis-
tal to the neural foramen. A root that has been
avulsed from the cord surface but remains within
the intervertebral foramen may appear normal on
gross dissection and represents the greatest diag-
nostic challenge encountered during surgery.
While a laminectomy would allow direct expo-
sure of the proximal rootlets, this approach is
relatively contraindicated in infants due to an
unacceptable risk of cervical instability. There-
fore, indirect evidence must be evaluated from
a variety of sources including preoperative clini-
cal examination (motor ndings and the presence
of a persistent Horner sign) and radiological
assessment (via CT myelography or MRI).
Intraoperative use of a nerve stimulator is useful;
direct stimulation of the root is performed and the
motor response in the limb is noted. An avulsed
root (whether extra- or intra-foraminal) cannot
conduct distally and is therefore incapable of
eliciting a muscle contraction.
Having delineated the extent of the neuroma, it
is then transected through its midportion using
scissors. The distal elements of the plexus are
M.C. Swan and H.M. Clarke
divided past the neuroma at the point where the
nerve feels supple and fascicular. The distal-most
elements of the resected neuroma are inked for
orientation and sent for frozen section in order to
identify the quality and axonal topography of the
stumps. Resection of the proximal stumps is
performed in a similar manner in order to reach
healthy neural tissue; the proximal margins are
also inked and sent for histologic evaluation.
Neural sections are stained with toluidine blue
and are evaluated by a neuropathologist to deter-
mine whether or not the proximal and distal
stumps are free from neuroma and thus represent
adequate sources or targets for reinnervation. His-
tologic examination is superior to visual inspec-
tion alone (Murji et al. 2008). Scarring associated
with residual neuroma will prevent axonal regen-
eration, thus necessitating additional resection.
A proximal stump specimen may have mini-
mal scarring, a good fascicular pattern, and no
evidence of dorsal root ganglion cells, but be
incapable of axonal regeneration due to the pres-
ence of an occult preganglionic intra-foraminal
rupture. This emphasizes the importance of a
holistic assessment of the clinical, radiologic,
and intraoperative ndings.
The Role of Neurolysis
Neurolysis has been proposed as a technique to
augment residual conduction within a neuroma-
in-continuity. An external neurolysis (i.e., an
epifascicular epineurectomy) is usually performed
in preference to an internal neurolysis (i.e.,
interfascicular epineurectomy).
The commonest surgical nding in obstetrical
brachial plexus palsy is neuroma-in-continuity;
however, the existence of physical continuity
does not necessarily imply physiologic continuity
(i.e., the existence of sufcient functioning
regenerated axons within the substance of the
neuroma). Chen et al. have shown that neuromas
contain copious collagen with sporadic nerve
bers enveloped by immature myelin sheaths;
notwithstanding that a substantial proportion of
the regenerated nerve bers crossed the neuroma,
there was no correlation between the
28 Microsurgery for Obstetrical Brachial Plexus Palsy
intraoperative neurophysiologic conductivity of
the neuroma and the percentage of regenerating
nerve bers across it (Chen et al. 2008).
The value of intraoperative neurophysiologic
testing remains unproven, and although the tech-
nique has not been universally adopted, some
centers perform intraoperative electrodiagnostic
studies and favor preservation of a conducting
neuroma-in-continuity if there is less than a
50 % drop in amplitude in the compound motor
action potential (CMAP) across the lesion
(Shenaq et al. 1998). In such a scenario, a
neurolysis would be performed and nerve conduc-
tion augmented where necessary by means of
fascicular grafts across the neuroma. When there
is greater than a 50 % reduction in CMAP ampli-
tude, then formal neuroma resection would be
undertaken.
Laurent et al. reported that the results for sural
nerve grafting were dramatically better than those
in patients who underwent neurolysis (Laurent
et al. 1993). Borrero also found the results of
neurolysis disappointing (Borrero 2001). Indeed,
neurolysis has been abandoned at The Hospital for
Sick Children in favor of neuroma resection and
grafting as neuroma resection is not considered
detrimental and it maximizes the opportunity for
maximal functional recovery (Kawabata
et al. 1987; Gilbert 1995; Capek et al. 1998;
Birch 2002). Nonetheless, a role for neurolysis
may exist in certain scenarios, such as a predom-
inantly upper trunk injury with less marked C8
and T1 involvement, where the lower trunk may
exhibit normal fascicular architecture and strong
muscle contraction on nerve stimulation after
release from a penumbra of scar.
Reconstruction with Sural Nerve Grafts
Following neuroma resection and histologic con-
rmation of adequate proximal and distal stumps
that are free from neuroma, a considerable gap is
created that must be bridged with cable grafts of
sural nerve (and supraclavicular branches of cer-
vical plexus if required). Priority must be given to
reinnervation of hand function, followed by res-
toration of biceps function, then shoulder stability,
623
and nally elbow and wrist extension. The sur-
geon measures the cumulative distance between
the proximal and distal stumps to be reconstructed
in order to create a bespoke reconstructive plan
making the most efcient use of the available graft
material. The typical defect is in the order of
2.54.5 cm, which, with up to 30 cm of sural
nerve being harvested, typically enables 612
cables of sural graft to be available. The prefer-
ence is for anatomical grafting (i.e., from the
original root to the originally intended target);
however, in the event of a proximal stump being
unavailable (i.e., due to a preganglionic avulsion
injury), an alternative source of axons must be
sought. Such extra-anatomical sources can arise
either from within the plexus (i.e., intra-plexal
neurotization) or from outside the plexus (i.e.,
extra-plexal neurotization).
Sural nerve grafts are inset in the reversed
orientation to eliminate axonal drop-off from
side branches. Anatomical grafts are arranged to
take advantage of the gross internal topography of
the proximal stumps; thus, for example, the most
cephalad portion of the C5 root would be grafted
to the suprascapular nerve stump, the posterior
aspect to the posterior division of the upper
trunk, and the anterior aspect to the anterior divi-
sion of the upper trunk. Grafting is undertaken
from deep to supercial in the wound in order to
avoid inadvertent disruption of a previously sited
graft and is done so with the aid of the operating
microscope. Sutureless coaptations are performed
by means of brin glue (Tisseel, Baxter
Healthcare) (Fig. 7). This has numerous advan-
tages including the ability to perform an intra-
foraminal coaptation and the ability to precisely
align the graft with the native plexus while the
brin sets. Some units perform coaptations in a
tension-free fashion with either 90 or 100 nylon
(Shenaq et al. 1998).
Nerve Transfers
Nerve transfer is a surgical technique whereby a
physiologically active nerve (selected to minimize
donor morbidity) is intentionally divided and
transferred to a nonfunctioning nerve in order to
624 M.C. Swan and H.M. Clarke

Fig. 7 Reconstruction of a
right Erb-Duchenne palsy
with anatomical sural nerve
grafts from the C5 to C7
roots to the suprascapular
nerve, anterior and posterior
divisions of the upper trunk,
and the middle trunk. The
use of brin glue obviates
the need for sutures
(Courtesy of Dr. H. M.
Clarke, The Hospital for
Sick Children)

reactivate paralyzed muscle function. Transfers

may be classied as being extra-plexal, intra-
plexal, or close-target nerve transfers. Nerve
transfers are indicated in the scenario of multiple
root avulsions where there are insufcient donor
axons available from the proximal stumps or
when there is inadequate graft material to com-
plete the desired reconstruction.
The most commonly performed extra-plexal
transfer is from the distal part of the spinal acces-
sory nerve to the suprascapular nerve (Allieu
et al. 1982). The spinal accessory nerve is located
on the deep surface of the trapezius muscle, which
can be readily accessed via the existing
supraclavicular exposure. The nerve is traced dis-
tally into the upper back beyond the proximal
branches serving the superior and middle bers
of trapezius in order to prevent the morbidity
associated with a drooped shoulder (Fig. 8).
Fig. 8 Schematic representation of an extra-plexal distal
With grossly limited donor axons, anatomical spinal accessory nerve transfer to the suprascapular nerve:
grafting might not be possible; the best available the proximal bers innervating the superior and middle
proximal root is transferred to the lower trunk in aspect of the trapezius muscle have been preserved, thus
order to prioritize reinnervation of the hand, preventing a drooped shoulder (Borschel and Clarke 2009)
(Courtesy of Lippincott Williams & Wilkins)
biceps function is motored by an extra-plexal
neurotization of the intercostal nerves to the
musculocutaneous nerve (Hattori et al. 1997; may be considered in the rare circumstance of a
Kawabata et al. 2001), while shoulder stability is ve-root (total) avulsion when there is dearth of
afforded by an accessory nerve transfer to the proximal stump axons (Gu et al. 1998). Other
suprascapular nerve. A contralateral C7 transfer extra-plexal donor nerves include the phrenic
28 Microsurgery for Obstetrical Brachial Plexus Palsy 625

Fig. 9 Nine-month-old
with harvest of three
intercostal nerves prior to
extra-plexal transfer
(Courtesy of Shriners
Hospital for Children,
Philadelphia)

(Gu and Ma 1996) and hypoglossal nerves

(Malessy et al. 1999).
Intra-plexal nerve transfer may be considered
in cases of non-global root avulsion, whereby at
least a single spinal root has sustained a rupture
injury and is therefore available for transfer
however, not to its original target (in which case
that would represent an anatomical reconstruc-
tion) but rather to a functionally more critical
target such as elbow exion (C6) or hand function
(lower trunk).
Although proximal (i.e., extra-plexal and intra-
plexal) nerve transfers represent the majority of
nerve transfers performed to reconstruct the bra-
chial plexus, the concept of a close-target nerve
transfer has developed as coaptation occurs at a
more distal site (i.e., closer to the neuromuscular
junction), thus enabling a more rapid recovery of Fig. 10 The Velpeau sling is inexpensive and securely
maintains the reconstructed extremity in full internal rota-
motor function. Examples include intercostal tion with the elbow xed at 90 of exion (Borschel and
nerve transfer to the musculocutaneous nerve to Clarke 2009) (Courtesy of Lippincott Williams & Wilkins)
enable elbow exion (Fig. 9) or transfer of the
long head of triceps branch of the radial nerve to
2 in. stockinette material (Fig. 10) (Gilchrist
the axillary nerve in Erb-Duchenne palsy to aug-
1967). Patients typically spend two nights in hos-
ment shoulder abduction (Jerome 2011).
pital postoperatively (Table 7). The patient returns
for a wound check and removal of the Velpeau
sling after 3 weeks, and the upper extremity is
Postoperative Care allowed to move freely. Passive range of motion
exercises is recommenced after the fth postoper-
A simple dressing is applied to the supraclavicular ative week.
incision, while the reconstructed upper extremity Restoration of preoperative movement is seen by
is immobilized for a period of 3 weeks with a 36 months postoperatively, and by 69 months,
Velpeau sling created from a single length of appreciable additional recovery is expected
626 M.C. Swan and H.M. Clarke

Table 7 Postoperative protocol following brachial plexus Table 8 Complications following brachial plexus explo-
reconstruction ration and reconstruction (La Scala et al. 2003)
Postoperative protocol Complication (%) Prevention or management
Inpatient stay 48 h Accidental extubation Prevent by suturing the
Type of immobilization Velpeau sling (2.9 %) nasotracheal tube to
Duration of immobilization 3 weeks membranous nasal septum
Postoperative wound check At discharge and at Monitor airway through a
3 weeks transparent drape
Recommence passive range of 5 weeks Postoperative uid May require treatment with
motion exercises overload (8.1 %) and diuretics or positive
pulmonary edema pressure ventilation
Clinical review in Annually until
(1.7 %) Prevent by restricting total
multidisciplinary clinic skeletal maturity
uid intake to 4 ml/kg/h
Wound infection (2.3 %) Prevent desiccation of soft
tissues during long
(Capek et al. 1998). Further recovery, as assessed by operative procedure
Prophylactic IV cefazolin
clinical examination, is anticipated until the fourth
every 4 h
postoperative year by which time the functional Thorough wound irrigation
improvement has inevitably plateaued. and meticulous hemostasis
Electrodiagnostic studies are not routinely before commencing nerve
performed postoperatively. Children are reviewed grafting
on an annual basis until skeletal maturity in order to Maintain patient
temperature homeostasis
monitor for soft tissue contractures and to consider throughout procedure
the need for secondary procedures. monitor for hypothermia
via rectal probe
Respiratory Conrm phrenic nerve
complications function preoperatively by
Complications ultrasound
1. Hemidiaphragm Identify and protect phrenic
A complication rate of 33.5 % has been paralysis (6.4 %) nerve intraoperatively;
conrm with nerve
documented in one retrospective series of 173 bra- stimulator if necessary
chial plexus reconstructions (La Scala et al. 2003). 2. Basal atelectasis
There were no fatalities. The complications, (2.9 %)
together with their management, are summarized 3. Pleural effusion
in Table 8 with some of the common surgical (1.7 %)
pitfalls (and pearls for prevention) listed in 4. Pneumothorax Perform submerged
(1.7 %) valsalva maneuver at time
Table 9. of wound closure to
identify a pleural breach
Chylothorax (0.6 %) Beware of thoracic duct
injury in left-sided plexus
Functional Outcomes explorations
Use ligaclips or suture ties
(not bipolar diathermy) in
Making direct comparisons between outcome the event of a chyle leak
data from different units is hindered by the
wide variability in the underlying pathology of
obstetrical brachial plexus palsy, different
methods of evaluating and classifying the Gilbert reported on 436 patients who
underlying severity of the condition, and underwent surgical reconstruction for obstetrical
signicant variance in the management brachial plexus palsy; follow-up was for a mini-
paradigms used. mum of 4 years and included those patients who
28 Microsurgery for Obstetrical Brachial Plexus Palsy
Table 9 Potential surgical pitfalls during brachial plexus reconstruction and suggested pearls for prevention
Potential pitfall
1. Poor access to surgical eld
2. Unable to identify C5 nerve root
3. Unable to differentiate between an intra-foraminal
preganglionic avulsion and a functional nerve root
4. Insufcient length of sural nerve graft for number of
computations required
had undergone secondary reconstructive proce-
dures (Gilbert 1995; Gilbert et al. 2006). Assess-
ment of restoration of function utilized the Mallet
scale; for C5C6 lesions, 80 % of patients
achieved good or excellent shoulder function,
while for C5C7 lesions, 61 % attained good or
excellent shoulder function. For C5-T1 lesions,
25 % recovered useful hand function. Similarly,
in a series of 282 infants undergoing brachial
plexus reconstruction at the Texas Childrens
Hospital, after a mean follow-up of 5 years, over-
all 75 % of patients had a good to excellent result
(based on the Mallet scale) after primary and
secondary reconstructive surgery (Shenaq
et al. 2005).
Results from The Hospital for Sick Children,
Toronto, of 108 patients with long-term follow-up
(for a minimum of 4 years) have been reported;
16 underwent neurolysis of conducting neuromas-
in-continuity, while 92 (48 with Erb-Duchenne
627
Pearls for prevention
a. Utilize V rather than linear incision
b. Divide clavicular head of SCM and omohyoid muscle
c. Divide EJV, suprascapular vessels, and transverse cervical
vessels
d. Anterior and middle scalene muscles may be resected as
necessary to allow improved access to nerve roots and
foramina
e. Downward retraction on clavicle obviates need for
osteotomy
a. Retrograde dissection of the supraclavicular braches of the
cervical plexus identies the C4 root which orientates the
location of C5
b. The phrenic nerve normally contributes bers to C5
a. T1 avulsions associated with persistent Horner syndrome
b. Radiologic nding in an avulsion of a pseudomeningocele
with absent nerve rootlets
c. Absence of muscle activity with direct intraoperatively
nerve root stimulation in preganglionic injuries
d. Histologic conrmation of an extra-foraminal dorsal root
ganglion in an avulsion injury
a. Employ extra-plexal neurotization of suprascapular nerve
(spinal accessory nerve) or musculocutaneous nerve
(intercostal nerves) to free sural nerve for key functional
target: lower trunk hand function
b. Consider harvest of the supraclavicular nerve, medial
cutaneous nerves of the arm and forearm, or the saphenous
nerve if additional graft if required
palsy and 44 with total plexus palsy) had neuroma
resection and nerve grafting (Lin et al. 2009). It
was concluded that early functional improve-
ments following neurolysis in Erb-Duchenne
palsy were not sustained over time; however, neu-
roma resection with nerve grafting produced sig-
nicant functional improvement for both
Erb-Duchenne and total plexus palsy, thus
supporting the view that neurolysis should be
abandoned in favor of neuroma resection and
nerve grafting.
Interestingly, in a series of 78 infants with
obstetrical brachial plexus palsy treated at the
Chang Gung Memorial Hospital, Taipei, with a
minimum of 4 years follow-up, those with
Erb-Duchenne palsy (n 34) who underwent
reconstruction with nerve grafting had consis-
tently better results when the target was more
proximal (i.e., the upper trunk) than distal (i.e.,
the musculocutaneous or axillary nerves)
628
(Chuang et al. 2005). It was speculated that this
might reect the length of graft required to reach
the more distal targets. In those patients with
mixed rupture and avulsion lesions (n 44),
65 % of those who required a spinal accessory
nerve to suprascapular nerve transfer achieved
good shoulder function. In infants with two or
more root avulsions, who were reconstructed
with an intercostal nerve to musculocutaneous
nerve transfer, 65 % attained good elbow exion,
while intra-plexal neurotization of C8 from either
C5 or C6 realized good nger exion in 60 % of
cases and fair exion in 28 % of cases. Of note, in
the ten patients who were reconstructed beyond a
year of age, no improvement was observed in
hand function, although all demonstrated
improved shoulder and elbow function.
The late consequences of obstetrical brachial
plexus palsy in both treated and untreated patients
are well documented (Adler and Patterson 1967).
These include hypoplasia of the upper extremity,
internal rotation contractures of the shoulder, ex-
ion contractures of the elbow, supination contrac-
tures of the forearm, dislocations of the radial
head, ulnar deviation of the wrist, a variety of
digital contractures, and signicant psychosocial
sequelae. Secondary reconstruction of these
deformities utilizes a host of techniques including
tendon transfer, tenodesis, capsulodesis, arthrod-
esis, and osteotomy; the goals are specic to each
level of plexus involvement: for upper plexus
injuries, restoration of shoulder abduction (del-
toid) and elbow exion (biceps) is critical, while
in middle plexus injuries, elbow extension (tri-
ceps) is the priority; nally, for lower plexus inju-
ries, it is recovery of nger exion and thumb
extension that is most important (Zancolli and
Zancolli 1988).
Future Developments
The ability to reimplant avulsed nerve roots into
the spinal cord medulla has hitherto rendered
extremely disappointing functional results,
although the possibility of improvement exists.
This area is the subject of considerable research
and raises the future prospect of early postnatal
M.C. Swan and H.M. Clarke
ventral root reimplantation via a dorsal
hemilaminectomy approach (Su et al. 2013).
Other potential areas of enquiry include the appli-
cation of the da Vinci telerobotic system to facil-
itate minimally invasive brachial plexus repair
with the benets of dampening tremor, motion
scaling, and improved ergonomics (Garcia
et al. 2012).
Much research has focused on the use of nerve
conduits and cadaveric allografts in brachial
plexus reconstruction in order to avoid the donor
site morbidity associated with sural nerve harvest
and to limit the potential reconstructive compro-
mises that one is currently required to make on
account of the restricted amount of available graft
material. Although evidence from current basic
science research fails to support their use in the
considerable anatomical defects seen in children
with obstetrical brachial plexus palsy, encourag-
ing clinical reports are beginning to emerge in the
context of adult traumatic brachial plexus injuries
both with bioabsorbable nerve conduits (Wolfe
et al. 2012) and cryopreserved cadaveric allo-
grafts (Squintani et al. 2013).
An improved understanding of the complex
changes in acute gene expression that are precip-
itated following traumatic ventral root avulsion
may open potential therapeutic channels that can
be exploited in a attempt to reduce motor neuron
degeneration in the early neonatal period in chil-
dren with obstetrical brachial plexus palsy
(Risling et al. 2011). Furthermore, as the inuence
of neurotrophic factors on motoneuron survival
after traumatic injuries is better appreciated, fur-
ther therapeutic targets may become available
(Chu and Wu 2009).
Conclusion
Brachial plexus birth palsy is a rare obstetric com-
plication, from which the majority of infants will
recover with nonoperative management and have
useful functional results. Patients should be man-
aged in a multidisciplinary team environment,
with the key principles of management being
early diagnosis, early assessment of function,
and early surgical intervention where indicated.
28 Microsurgery for Obstetrical Brachial Plexus Palsy
Surgical management consists of neuroma exci-
sion and sural nerve grafting, with nerve intra-
plexal or extra-plexal nerve transfers as required.
Outcomes continue to improve for 3 or more years
postoperatively.
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 Glenohumeral Joint Secondary
Procedures for Obstetrical Brachial 29
Plexus Birth Palsy

Roger Cornwall

Contents Abstract
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 634 Brachial plexus birth palsy leaves permanent
neurological decits in 2040 % of cases, lead-
Pathoanatomy and Applied Anatomy . . . . . . . . . . . . 634
ing to secondary muscle and joint deformities,
Assessment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 635 even following surgical repair of the injured
Signs and Symptoms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 635 nerves. These secondary musculoskeletal
Imaging and Other Diagnostic Studies . . . . . . . . . . . . . . 637
problems in the shoulder are the most common
Nonoperative Management . . . . . . . . . . . . . . . . . . . . . . . . 637 reasons for surgical intervention following
Operative Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 639 BPBP. Early attention to these complications
Contracture Release/Reduction . . . . . . . . . . . . . . . . . . . . . . 639 of the neurological injury with interventions
Muscle Transfers . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 643
aimed at restoring normal muscle length and
Osteotomies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 645
balance at the shoulder can allow improved
Preferred Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 647 growth and development of the glenohumeral
Outcomes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 648 joint. The internal rotation contracture can be
Management of Complications . . . . . . . . . . . . . . . . . . . . 648 treated with many different techniques of
release reported as successful. Active abduc-
Future Research . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 648
tion and external rotation can be improved with
Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 649 transfer of the latissimus and teres major mus-
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 649 cles to the posterior rotator cuff. Function can
be reliably improved following contracture
release and tendon transfers to augment shoul-
der strength, but normal function cannot be
restored. The contracture and deformity at the
shoulder is multiplanar and complex, and con-
tinued research into the biological and biome-
chanical pathophysiology of the contractures
and dysplasia will open the door to novel pre-
vention and treatment strategies in the coming
years.

R. Cornwall
Cincinnati Childrens Hospital, Cincinnati, OH, USA
e-mail: roger.cornwall@cchmc.org

# Springer Science+Business Media New York 2015 633

J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_29
634
Introduction
Brachial plexus birth palsy (BPBP) occurs in
approximately 1.5 per 1,000 live births (Foad
et al. 2008) and is presumed to result from traction
to the brachial plexus during delivery. The natural
history of BPBP is less favorable than historically
believed, with 2040 % of children experiencing
incomplete neurological recovery (Pondaag
et al. 2004; Foad et al. 2009). In these children
with persistent paralysis, secondary contractures
can occur, most notably shoulder internal rotation
contractures and elbow exion contractures
(Waters 2005). These contractures signicantly
impair function and quality of life (Huffman
et al. 2005; Bae et al. 2008, 2009) and are the
most common reason for surgery following
BPBP. Thus, historically, surgery for the
so-called secondary problems at the shoulder
have focused on restoring passive range of motion
by releasing contractures and improving active
range of motion by way of muscle transfers.
These palliative interventions were typically
performed only after optimal nerve reconstructive
surgery had been exhausted or after spontaneous
recovery had plateaued.
However, the landscape has changed dramati-
cally in recent years, spurred primarily by two
quantum leaps in the understanding of the prob-
lem. First, the contracture at the shoulder has been
clearly shown to be associated with progressive
skeletal dysplasia of the glenohumeral joint
(Waters et al. 1998). This dysplasia begins with
increased retroversion of the glenoid and leads to
complete posterior dislocation of the
glenohumeral joint with humeral head attening,
loss of glenoid concavity, and pseudoglenoid
formation. Second, this dysplasia occurs much
earlier than previously thought, with nearly 10 %
of infants progressing to glenohumeral disloca-
tion in the rst year, even as early as 3 months
(Moukoko et al. 2004; Dahlin et al. 2007). Thus, it
is not unusual to be faced with a dislocated
shoulder that needs to be addressed with second-
ary procedures even before the child is old
enough to determine if primary nerve surgery
is necessary.
R. Cornwall
Recognition of the early development of
glenohumeral dysplasia is of critical importance
in the management of the shoulder following
BPBP, as early treatment of the glenohumeral
joint deformity can alter the long-term course of
shoulder development and function. Several
series have demonstrated remodeling of the
glenohumeral dysplasia following release of the
internal rotation contracture with or without mus-
cle transfers to augment external rotation strength
(El-Gammal et al. 2006; Pearl et al. 2006; Waters
and Bae 2009; Kozin et al. 2010). Even humeral
head deformity has been shown to remodel fol-
lowing restoration of appropriate glenohumeral
alignment (Reading et al. 2012). However, it is
still unknown beyond what age remodeling is no
longer possible or the severity of glenohumeral
deformity that can remodel. Nonetheless, the pos-
sibility of restoring normal skeletal structure with
early surgery underscores the importance of early
detection of glenohumeral dysplasia. Therefore,
although this chapter will discuss interventions
designed to address the secondary musculoskele-
tal complications of a primarily neurologic injury,
it does not imply that such complications are of
secondary importance or that they should be con-
sidered only after the neurological injury is
addressed. The surgeon caring for the child with
BPBP must keep a holistic view of the complex
interplay between the neurologic injury and the
developing musculoskeletal system in order to
prioritize interventions to maximize long-term
function and development.
Pathoanatomy and Applied Anatomy
The most widely accepted mechanism for the
development of the shoulder internal rotation con-
tracture is muscle imbalance between functioning
internal rotators and paralyzed external rotators,
leading to static internal rotation joint posturing
and ultimate joint contracture (Waters 2005; Pearl
2009). This theory is supported by a magnetic
resonance imaging (MRI) study in BPBP patients
demonstrating a correlation between the degree of
shoulder joint contracture and the ratio of cross-
sectional area between internal rotator and
29 Glenohumeral Joint Secondary Procedures for Obstetrical Brachial Plexus Birth Palsy
external rotator muscles (Waters et al. 2009). Con-
versely, however, other MRI studies have demon-
strated the degree of contracture to correlate only
with atrophy of the subscapularis, an internal rota-
tor (Poyhia et al. 2005; Hogendoorn et al. 2010).
Furthermore, simple muscle imbalance cannot
explain paradoxical contractures, where the joint
becomes tight in the direction of the paralyzed
muscles. The most notably paradoxical contrac-
ture is the elbow exion contracture that occurs in
the setting of initial elbow exor paralysis
(Ballinger and Hoffer 1994; Herring 2002; Poyhia
et al. 2010). In fact, the elbow exion contracture
has been shown to correlate on MRI only with
atrophy of the brachialis (Poyhia et al. 2007). At
the shoulder, one such paradoxical contracture is
the glenohumeral abduction contracture that
occurs following paralysis of the shoulder abduc-
tors (Waters et al. 1998; Gu et al. 2000). Because
patients with this contracture cannot fully adduct
the glenohumeral joint, adducting the arm to the
side requires rotation of the scapula on the chest
wall (Waters and Bae 2006). The resulting supe-
rior protrusion of the superior-medial angle of the
scapula has been termed the Putti sign, after its
initial description in 1932 (Putti 1932). Further-
more, motion analysis studies have demonstrated
a greater than normal contribution of
scapulothoracic motion to global shoulder
motion, suggesting a functional ankylosis of the
glenohumeral joint in all directions, consistent
with contractures of all the denervated rotator
cuff muscles.
Recent ndings in an animal model of BPBP
implicate impaired growth and development of
neonatally denervated muscle in contracture path-
ogenesis (Nikolaou et al. 2011, 2013; Weekley
et al. 2012). Without normal neonatal innervation,
muscles fail to grow sufciently to keep up with
skeletal growth and contractures occur. Although
this impaired muscle growth affects the entire
denervated rotator cuff, the shoulder is positioned
in internal rotation by the functioning pectoralis
major muscle and other internal rotators, leading
to posterior extrusion and dysplasia of the
glenohumeral joint. The interplay between dener-
vation, muscle atrophy, and musculoskeletal
growth is likely complex, and the molecular and
635
cellular mechanisms of contracture development
are poorly understood. Nonetheless, the shoulder
contracture is more complex than a unidirectional
internal rotation contracture, and the pathogenesis
is more complex than a simple lack of mechanical
stretch of the muscles or joint capsule. It is likely
that a combination of contractures from impaired
muscle growth and postural forces from muscle
imbalance contribute to the pathoanatomy of
glenohumeral dysplasia, as supported by a recent
computational model (Crouch et al. 2014).
Assessment
Signs and Symptoms
The physical examination of the child with resid-
ual dysfunction from BPBP is critical to clinical
decision-making and revolves primarily around
passive and active range of motion. The assess-
ment of passive shoulder range of motion is
important, especially the passive external rotation
in adduction, as loss of this motion correlates with
the development of glenohumeral dysplasia
(Kozin 2004) and can be used as a screening tool
for infantile dysplasia and dislocation (Moukoko
et al. 2004). Additional signs of glenohumeral
dislocation include a deep axilla, asymmetric
skin folds, an apparently shortened humeral seg-
ment, and posteriorly palpable humeral head.
Thus, the physical examination ndings of
glenohumeral dysplasia are not dissimilar to
those of developmental dysplasia of the hip. Addi-
tionally, passive range of motion should be
assessed in other shoulder motions, including
abduction, internal rotation, and adduction.
Assessment of these motions requires careful sta-
bilization of the scapula, as scapular winging can
compensate for reduced glenohumeral motions in
these directions.
Residual paralysis of muscles about the shoul-
der girdle is difcult to quantitatively assess using
scales such as the British Medical Research Coun-
cil scale used for muscle strength grading in
adults. The Hospital for Sick Children Active
Movement Scale semiquantitatively assesses spe-
cic shoulder motions (abduction, external
636 R. Cornwall

Modified Mallet classification

(grade I = no function, Grade V = normal function)
Grade I Grade II Grade III Grade IV Grade V

Global Not No
Normal
abduction testable function

<30 30 to 90 >90

Global external Not No

Normal
rotation testable function <0 0 to 20 >20

Not No
Hand to neck Normal
testable function
Not possible Difficult Easy

Hand on spine Not No Normal

testable function

Not possible S1 T12

Hand to mouth Not No Normal

testable function
Marked Partial <40 of
trumpet sign trumpet sign abduction

Not No
Internal rotation
testable function
Cannot Can touch with Palm on belly,
touch wrist flexion no wrist flexion

Fig. 1 Modied Mallet Score (Courtesy of Shriners Hospital for Children, Philadelphia)

rotation, etc.) with and without gravity, although
the use of this system is limited by the
confounding effects of joint contractures (Curtis
et al. 2002). The most widely used scale of global
shoulder function in this condition is the Mallet
scale, recently modied as summarized in Fig. 1.
Six tasks are graded on a scale of 15, with 1 indi-
cating no function in that task and 5 indicating
normal function. The scale has been validated and
has demonstrated substantial intra- and
interobserver reliability (Bae et al. 2003).
However, the six tasks involve global shoulder
motion, and the contributions of individual com-
ponents of the shoulder girdle are difcult to
assess. For instance, the abduction component of
the Mallet scale is unable to separate
glenohumeral from scapulothoracic movement.
Scapulothoracic movement has been recently
demonstrated to contribute signicantly more to
shoulder abduction in this condition when com-
pared to uninjured contralateral control limbs
(Russo et al. 2013). Nonetheless, the Mallet
29 Glenohumeral Joint Secondary Procedures for Obstetrical Brachial Plexus Birth Palsy
scale allows an assessment of overall shoulder
function and is sensitive to changes over time
and following intervention.
Additional assessment of shoulder and upper
extremity function can take into account patient
and family reports of function. The Pediatric Out-
comes Data Collection Instrument (PODCI) is a
tool that measures function and quality of life in
several domains and has been validated in the
BPBP population (Dedini et al. 2008). The use
of this measure can help to guide long-term func-
tional goals and track improvements over time.
Imaging and Other Diagnostic Studies
The complex three-dimensional anatomy of the
shoulder girdle, combined with the largely carti-
laginous nature of the glenohumeral joint in
young children, limits the utility of plain radio-
graphs in the assessment of the shoulder joint
following BPBP. The most important information
to be gained from imaging the shoulder includes
the shape of the glenoid and the position of the
humeral head relative to the glenoid. The progres-
sion of glenohumeral dysplasia has been well
described (Waters et al. 1998) and tends to pro-
gress from increased glenoid retroversion to com-
plete posterior dislocation of the humeral head
and severe dysplasia of the glenoid.
Pseudoglenoid formation and humeral head at-
tening further complicate the dysplasia. These
deformities are most pronounced in the axial
plane, requiring imaging in this plane.
Ultrasonography has been widely used for
assessment of hip dysplasia in the infant and can
be easily applied to assessment of glenohumeral
dysplasia in the same age group (Moukoko
et al. 2004). Using a posterior axial approach
with a linear array transducer, the humeral head
and posterior glenoid can be routinely visualized
in children up to 1518 months of age. The rela-
tive position of the humeral head and glenoid can
be assessed, allowing assessment of glenohumeral
subluxation and dislocation. Ultrasonography has
two distinct advantages over MRI. First, it does
not require sedation or anesthesia, as is typically
required for MRI in the infant. Second, it can be a
637
dynamic study, assessing the relative position of
the humeral head and glenoid throughout a range
of glenohumeral motion. However, the use of
ultrasound to assess the shape of the glenoid has
not yet been proven reliable. Nonetheless, ultra-
sonography can be used as a screening tool to
detect infantile glenohumeral dislocation.
MRI provides the most comprehensive evalu-
ation of the immature glenohumeral joint follow-
ing BPBP. The cartilaginous glenoid, humeral
head, and labrum can be visualized, allowing
quantitative measurement of glenoid retroversion
and percent posterior subluxation of the humeral
head (Fig. 2) (Waters et al. 1998). In addition, the
muscles of the shoulder girdle can be visualized,
allowing at least a qualitative evaluation of muscle
atrophy. The disadvantage of this imaging modal-
ity is the need for sedation or general anesthesia to
allow a motionless study in the young child. Com-
puted tomography (CT) can be utilized in the
older child with an ossied humeral head and
glenoid, often obviating the need for sedation or
anesthesia, although ionizing radiation is used.
Nonoperative Management
Nonoperative treatment in the form of occupa-
tional and physical therapy remains the corner-
stone of initial management of residual shoulder
dysfunction and serves as the only treatment
required for a majority of children. Goals of ther-
apy involve maintenance of passive joint range
motion while awaiting neurological recovery,
together with functional rehabilitation following
neurological recovery and assistance with motor
skill development.
As passive external rotation in adduction cor-
relates with glenohumeral dysplasia, maintenance
of this motion is especially important in therapy.
The brachium is stabilized against the chest wall,
and the shoulder is passively externally rotated
using the exed elbow as a lever arm. Exercises
are performed several times with each diaper
change, using basic principles of passive
stretching. Additional passive motion in the
glenohumeral joint is important but difcult to
maintain. The abduction contracture is
638
Fig. 2 (a) Glenoid
retroversion measurements
(Glenoid
version [alpha]-90 ) and
(b) humeral head
subluxation measurements
(PHHA AB/AC  100
%) (From Lippert
et al. (2012))
particularly challenging to stretch given the dif-
culty of adequately stabilizing the scapula. Joint
mobilization techniques can be used to maintain a
supple glenohumeral joint despite scapular
hypermobility, but such techniques can be dif-
cult for parents and caregivers to perform at home.
Aside from maintaining passive range of
motion, motor reeducation is vitally important in
therapy. Because of the initial weakness of the
deltoid and supraspinatus, abduction attempts
recruit scapulothoracic movement. Scapular tap-
ing may assist with reacquisition of glenohumeral
motion by partially inhibiting compensatory
scapulothoracic movement. Similarly, the trumpet
sign (abduction of the shoulder during hand-to-
mouth movement) may initially be due to weak-
ness of external rotation and elbow exion but
may become habitual despite improved muscle
strength. Therefore, therapy is necessary to opti-
mize the pattern of movement, not just the
strength of individual muscles.
Botulinum toxin A injection has become an
increasingly accepted adjunct to physical and occu-
pational therapy in other pediatric neurological
disorders, and it may have a role in BPBP. Brachial
R. Cornwall
plexus injury, as a peripheral nerve injury, does not
result in muscle spasticity. However, botulinum
toxin has been used to facilitate motor learning in
recovering muscles following BPBP by temporar-
ily weakening antagonists. A series of eight chil-
dren underwent botulinum toxin injections into the
triceps, pectoralis major, and/or latissimus dorsi
muscles, with immediate and 4-month improve-
ments in function of the opposing reinnervating
muscles (DeMatteo et al. 2006). Similarly, four
patients with biceps-triceps co-contraction demon-
strated improved active elbow exion 18 months
following a single botulinum toxin injection into
the triceps muscle (Heise et al. 2005). In addition,
botulinum toxin injection in the internal rotators
may ease the passive stretching of external rotation
in adduction by weakening the resistance and dis-
comfort associated with these stretching exercises
in the young child. Such a strategy has been used in
infants with posteriorly subluxated shoulders,
allowing successful reduction that was maintained
at 1 year in 24 of 35 patients in one series (Ezaki
et al. 2010). The indications for botulinum toxin
injection in the setting of BPBP require further
elucidation.
29 Glenohumeral Joint Secondary Procedures for Obstetrical Brachial Plexus Birth Palsy
Operative Treatment
Surgical treatment of the secondary shoulder dys-
function following BPBP aims to accomplish
three goals: (1) restoration of passive motion by
contracture release, (2) realignment of the dys-
plastic glenohumeral joint, and (3) augmentation
of muscle power in the weak domains of shoulder
movement. If these goals cannot be accomplished,
palliative surgery in the form of humeral
osteotomy can improve global shoulder function
without addressing the glenohumeral joint defor-
mity and dysfunction.
A variety of procedures have been described
for surgical release of the shoulder internal rota-
tion contracture and augmentation of external
rotation strength. These procedures, modied
over many decades, typically involve lengthening
or sectioning internal rotators, such as the
subscapularis and/or pectoralis major, and transfer
of functioning muscles, such as the latissimus
dorsi and/or teres major to the posterior or
posterosuperior rotator cuff, to augment external
rotation and abduction function. Many earlier
series have demonstrated short- and medium-
term gains in global shoulder function but with
results that tend to deteriorate over time, poten-
tially due to a historical lack of awareness of
glenohumeral dysplasia.
The increasing awareness of glenohumeral dys-
plasia over the past decade has created an opportu-
nity to evaluate the effects of these surgical
procedures on glenohumeral dysplasia progres-
sion. In 2005, Waters and Bae reported a series of
25 children who underwent latissimus dorsi and
teres major tendon transfers with or without length-
ening of the subscapularis or pectoralis major
(Waters and Bae 2005). At 2-year follow-up, a
ve-point improvement in global shoulder function
on the Mallet scale was noted, in keeping with
previous reports, but only modest improvements
were seen in glenoid retroversion and
glenohumeral subluxation on MRI or CT imaging.
The authors concluded that muscle rebalancing
surgery only halted the progression of dysplasia
but did not allow substantial remodeling. Similarly,
Kozin et al. in 2006 described a complete lack of
639
glenohumeral remodeling at 1-year follow-up in
23 children treated with a similar combination of
procedures (Kozin et al. 2006). However, that same
year, a series of 33 children was reported describ-
ing 2-year follow-up of arthroscopic release of the
subscapularis with or without latissimus dorsi
transfer (Pearl et al. 2006). Of 15 children for
whom preoperative and follow-up MRI scans
were available, 12 showed substantial
glenohumeral remodeling. Also published in
2006 was a retrospective study of 109 patients
who underwent open subscapularis release and
teres major transfer (El-Gammal et al. 2006).
Follow-up CT scans available at least 1 year post-
operatively in 39 patients demonstrated glenoid
retroversion that correlated positively with age at
surgery, with normal glenoid retroversion in chil-
dren operated prior to 4 years of age and no
glenohumeral subluxation in children operated by
2 years of age. These two reports, in contrast to the
prior two, suggested that remodeling may indeed
be possible.
Validation of remodeling potential has now
been provided by a number of recent reports.
Waters and Bae recently reported a series of
23 patients who underwent subscapularis/
pectoralis major lengthenings and latissimus
dorsi/teres major transfers but with the addition of
open glenohumeral reduction (Waters and Bae
2009). In these patients, 83 % demonstrated
glenohumeral remodeling at 2 years, with signi-
cant improvements in both glenoid retroversion
and glenohumeral subluxation. Similarly, Kozin
et al. adopted the technique of arthroscopic
subscapularis release with or without external rota-
tion tendon transfers and reported a series of
44 children with signicant remodeling on 1-year
follow-up MRI (Kozin et al. 2010). These reports
draw attention to strategic adaptations in surgical
technique that place increased importance on
obtaining appropriate glenohumeral articular align-
ment in addition to extra-articular muscle balance.
Contracture Release/Reduction
The treatment of the shoulder internal rotation
contracture following BPBP has been modied
640
over the years (Fairbank 1913; Sever 1916;
LEpiscopo 1934; Hoffer et al. 1978; Pearl and
Edgerton 1998; Waters and Bae 2005; Newman
et al. 2006; Pearl et al. 2006; Pedowitz
et al. 2007). Contemporary techniques for treat-
ment of the shoulder internal rotation contracture
include (1) open or arthroscopic sectioning or
lengthening of the subscapularis and/or pectoralis
major tendons with or without release of the ante-
rior glenohumeral joint capsule. The relative
advantages and disadvantages of open versus
arthroscopic release have been debated, but no
clear winner has emerged. Similarly, no clear indi-
cations exist to select one technique over the other,
as each technique has been reported to successfully
improve passive external rotation. The true differ-
ences may be borne out with longer follow-up
evaluation of glenohumeral alignment and
remodeling, as some techniques of contracture
release may allow better articular realignment
than others. Nonetheless, surgical treatment of the
internal rotation contracture should be considered
when (1) the internal rotation contracture pro-
gresses to less than 20 of passive external rotation
in adduction despite appropriate nonoperative
means and/or (2) glenohumeral dislocation or pro-
gressive glenohumeral dysplasia is documented by
axial imaging. Internal rotation contracture release
should be used with caution in patients who have
substantial functional decits in midline function,
as many reports have demonstrated a risk of wors-
ening internal rotation function following internal
rotation contracture release (van der Sluijs
et al. 2004; Kambhampati et al. 2006; Kozin
et al. 2006; Newman et al. 2006; Pearl et al. 2006).
Techniques
Subscapularis Slide
Surgical release of the subscapularis from its ori-
gin on the anterior surface of the scapula has been
used for decades, with its proponents citing a low
rate of loss of internal rotation function, since the
insertion of the subscapularis is left intact (Carlioz
and Brahimi 1971). However, opponents cite a
high rate of contracture recurrence. The scapula
is approached through a longitudinal incision
along the lateral border of the scapula beginning
R. Cornwall
superiorly from the posterior axillary fold. The
lateral inferior angle of the scapula is retracted
laterally from the wound, and the subscapularis
is elevated extraperiosteally from its anterior sur-
face beginning inferiorly and progressing superi-
orly. Adequate release requires elevation of the
superior muscle belly deep in the wound, and this
portion is performed by palpating tight bands as the
shoulder is passively externally rotated. The release
is considered sufcient when the shoulder has
6080 of passive external rotation in adduction.
The wound is closed with absorbable sutures, and
the arm is positioned in a shoulder spica cast in
external rotation and 2030 of abduction. This
procedure can also be accomplished through a
longitudinal incision at the medial border of the
scapula, releasing the subscapularis beginning
medially and progressing laterally.
Arthroscopic Partial Subscapularis
Tenotomy
The patient is positioned in the lateral decubitus
position. Care must be taken during positioning to
protect the contralateral brachial plexus with an
axillary roll and to protect all bony prominences
with adequate padding. The shoulder is examined
under anesthesia to conrm the preoperative inter-
nal rotation contracture. In the case of an infantile
dislocation, the shoulder is imaged ultrasonogra-
phically under anesthesia using the posterior axial
view described by Moukoko et al. (2004). The
arthroscope (1.9 mm for infants, 2.7 mm for
older children) is inserted through a standard pos-
terior portal. If the joint is dislocated, the portal
entry is located more medially than typical, with a
more laterally directed trajectory of the arthro-
scope. Care must be taken to avoid injury to the
soft, unossied humeral head, and familiarity with
shoulder arthroscopy is essential. Following joint
inspection and identication of the intra-articular
subscapularis tendon, an up-biting basket resector
is inserted through an anterior portal, and the
superior 23 mm of subscapularis tendon is sec-
tioned. The shoulder is then passively externally
rotated in adduction to spread the cut bers, slid-
ing them along the intact bers (Fig. 3). The
release is considered successful if at least 60 of
passive external rotation in adduction can be
29 Glenohumeral Joint Secondary Procedures for Obstetrical Brachial Plexus Birth Palsy
Fig. 3 Surgical technique of arthroscopic partial subscapularis release, as viewed from the posterior portal in a left
shoulder
Fig. 4 Intraoperative
photographs demonstrating
maximum passive external
rotation in adduction before
(a) and after (b)
arthroscopic partial
subscapularis release
achieved (Fig. 4). If necessary, additional bers of
the subscapularis are sectioned until the desired
passive external rotation is achieved, but neither
the joint capsule nor the entire intra-articular
subscapularis tendon is sectioned. In the case of
an infantile dislocation, the shoulder is again
imaged with a sterilely draped ultrasound probe
to conrm glenohumeral reduction in external
rotation. Following successful release, the ten-
dons of the latissimus and teres major muscles
are transferred if indicated through a trans-
verse axillary approach to the posterosuperior
rotator cuff, as described below (Waters 2005).
Postoperatively, the shoulder is immobilized in
a shoulder spica cast in adduction and external
rotation following a release alone or in external
rotation and abduction following a release and
tendon transfers. The cast is removed at 4 weeks
postoperatively, and occupational therapy is
begun. No brace is used following cast removal,
unless the contracture begins to recur.
Open Subscapularis Release
The subscapularis can be lengthened or partially
released at its insertion through open approaches
as well. An anterior deltopectoral approach grants
access to the extra-articular portion of the
641
subscapularis tendon and has been used for
z-lengthenings of this tendon (van der Sluijs
et al. 2004). However, residual weakness of internal
rotation commonly complicated such a lengthening
procedure, and it has fallen out of favor. The
subscapularis can also be approached through a
transverse axillary incision used for the latissimus
and teres major tendon transfer as described below.
Once the latissimus tendon is harvested from the
humeral shaft for transfer, the joint capsule can be
seen in the deep portion of the wound. An
anteroposterior incision in the inferior capsule
allows direct inspection of the glenohumeral joint.
The intra-articular, superior portion of the
subscapularis tendon can be released with or without
the anterior joint capsule. The glenohumeral joint
can be anteriorly translated for reduction of a poste-
rior dislocation under direct visualization. As this
approach is typically used simultaneously with mus-
cle transfers, the shoulder spica cast applied postop-
eratively is typically placed with the arm in
abduction and external rotation.
Release of Other Structures
Other structures have been implicated in contrac-
ture pathophysiology, and several authors have
advocated for release of these structures as well.
642
The pectoralis major tendon may be tight, restricting
passive external rotation in abduction, and this ten-
don can be z-lengthened through an anterior
deltopectoral approach or axillary approach as
described above. Additionally, the corocohumeral
ligament and coracoid have been found by some to
be impediments to reduction of the dislocated and
dysplastic glenohumeral joint, and these structures
can be released or removed through an anterior
approach. In a long-standing dislocation, the anterior
joint capsule may require sectioning, and others
have advocated reeng of the posterior joint capsule
as well (Waters and Bae 2009). However, unlike
adhesive capsulitis in the adult shoulder, the joint
capsule is not routinely viewed as the primary struc-
ture responsible for the contracture.
Abduction Contracture Release
Only very recently have surgeons begun to
address the glenohumeral abduction contracture
that has been recognized for decades (Putti 1932).
Several techniques have been reported, including
partial deltoid release, subscapularis origin
release, and acromion osteotomy, but published
clinical studies are lacking.
Shoulder internal rotation contracture release
Preoperative planning
OR table: Standard table or beach chair if preferred for
arthroscopy
Position/positioning aids: Lateral decubitus in bean bag
with axillary roll for subscapularis slide; lateral decubitus
or beach chair for arthroscopic release, based on
preference
Fluoroscopy location: No uoroscopy, but portable
ultrasonography unit placed in front of patient to allow
visualization from a posterior transverse approach to
conrm reduction in the setting of an infantile
glenohumeral dislocation
Equipment: Standard soft tissue surgical equipment.
Small joint 1.9 mm or 2.7 mm arthroscope, small joint
instruments
Tourniquet (sterile/nonsterile): N/A
Subscapularis slide for shoulder internal rotation
contracture
Surgical steps
A longitudinal incision is made along the lateral border of
the scapula beginning superiorly from the posterior
axillary fold
(continued)
R. Cornwall
Subscapularis slide for shoulder internal rotation
contracture
Surgical steps
The lateral inferior angle of the scapula is retracted
laterally from the wound, and the subscapularis is
elevated extraperiosteally from its anterior surface
beginning inferiorly and progressing superiorly
Adequate release requires elevation of the superior
muscle belly deep in the wound, and this portion is
performed by palpating tight bands as the shoulder is
passively externally rotated
The release is considered sufcient when the
shoulder has 6080 of passive external rotation in
adduction
The wound is closed with absorbable sutures
Arthroscopic partial subscapularis tenotomy for shoulder
internal rotation contracture
Surgical steps
The arthroscope (1.9 mm for infants, 2.7 mm for older
children) is inserted through a standard posterior portal. If
the joint is dislocated, the portal entry is located more
medially than typical, with a more laterally directed
trajectory of the arthroscope
Following joint inspection and identication of
the intra-articular subscapularis tendon, an up-biting
basket resector is inserted through an anterior portal and the
superior 23 mm of subscapularis tendon is sectioned
The shoulder is then passively externally rotated in
adduction to spread the cut bers, sliding them along the
intact bers
The release is considered successful if at least
60 of passive external rotation in adduction can be
achieved
If necessary, additional bers of the subscapularis are
sectioned until the desired passive external rotation is
achieved, but neither the joint capsule nor the entire intra-
articular subscapularis tendon is sectioned
In the case of an infantile dislocation, the shoulder is
again imaged with a sterilely draped ultrasound probe to
conrm glenohumeral reduction in external rotation
The portals are closed with Steri-Strips or absorbable
sutures
Shoulder internal rotation contracture release
Postoperative protocol
Type of immobilization: Shoulder spica cast in 3040 of
abduction and 4060 of external rotation
Length of immobilization: 4 weeks
Rehab protocol: Preoperative therapy is restarted after
removal of cast with emphasis on passive external
rotation in adduction. Consider external rotation splint a
night for early recurrence of contracture
29 Glenohumeral Joint Secondary Procedures for Obstetrical Brachial Plexus Birth Palsy
Muscle Transfers
Persistent weakness of abduction and external rota-
tion is very common following incompletely
resolved BPBP. If nerve surgery (nerve grafting,
nerve transfers) has not been performed or has not
restored adequate power in the C5 distribution,
secondary muscle transfers can reliably improve
global shoulder function in abduction and external
rotation. Such procedures have been described for
decades and have evolved over time. The basic
principles remain the same, however. An abun-
dance of adductors and internal rotators that remain
innervated provides an opportunity to restore bal-
ance about the glenohumeral joint by transferring
one or more of these functioning muscles to the
posterior humerus or rotator cuff to allow them to
function as external rotators. The most commonly
transferred muscles are the latissimus dorsi and
teres major. The relative improvement in abduction
versus external rotation can be selected by the loca-
tion to which the muscles are transferred. If
improved external rotation in adduction is desired,
the tendons can be inserted on the posterior humeral
shaft, as described by LEpiscopo (1934). If the goal
is to improve abduction as well as external rotation
in abduction (hand-to-neck on the Mallet scale),
then the tendons can be transferred to the
posterosuperior rotator cuff as described by Hoffer
and Phipps (1998). Therefore, the exact technique
of the operation can be varied based on the indica-
tion. It is important to note that improving external
rotation in adduction should not sacrice functional
internal rotation in adduction. Loss of midline func-
tion from internal rotation weakness is more func-
tionally disabling than a lack of external rotation in
adduction. Therefore, a contraindication to external
rotation tendon transfer is a preexisting inability to
internally rotate to the sagittal midline, as such a
surgery would worsen that function further.
Techniques
Latissimus Dorsi/Teres Major Transfer
The patient is carefully positioned in the lateral
decubitus position, with the affected arm up. The
entire shoulder girdle is prepped to allow posi-
tioning of the scapula. The internal rotation
643
contracture of the glenohumeral joint is released
if necessary as described above. The limb is posi-
tioned over the head in maximum abduction. This
position can cause distortion of the surgical anat-
omy from what a shoulder surgeon is used to,
requiring care in the learning curve of this
approach. A transverse axillary incision is used
for the tendon transfers, centering the incision
over the posterior axillary fold. This approach
hides the incision in the skin creases of the axilla,
leading to a cosmetically subtle scar. The
latissimus tendon inserts into the skin of the axilla,
so these supercial bers must be transected in
order to expose the latissimus and teres major
tendons deeper in the wound. The anterior surface
of the latissimus tendon is easily identied and is
traced toward its insertion in the humerus. Inter-
nally rotating the shoulder in abduction will bring
the footprint of the latissimus tendon into better
view. The radial nerve lies immediately anterior to
the tendon insertion and must be retracted with
care. The axillary nerve resides just proximal and
deep to the tendinous insertions. The superior and
inferior margins of the tendon insertion are dis-
sected free, taking care to maintain hemostasis at
the superior (deep) margin where abundant ves-
sels exist. The posterior surface of the teres major
tendon is then identied anterior to the long head
of the triceps, tracing the teres major tendon to its
insertion on the humerus conjoined with the
latissimus tendon. Once a circumferential expo-
sure of the conjoined tendon on the humerus has
been achieved, the tendon is released either
sharply or with electrocautery. If electrocautery
is used, a shielded tip and caution are necessary
to avoid injury to the radial nerve anteriorly. Once
the tendon is released from the humerus, the ten-
don end is tagged with sutures and retracted from
the wound. The muscles are freed from surround-
ing tissues to the level of the pedicle in order to
allow adequate excursion for transfer.
The interval between the deltoid and triceps
(long head) is then identied. It may help to
abduct the shoulder to only 90 for this step, as
the deltoid and triceps bers are collinear in full
abduction, obfuscating the interval. Through this
interval, deeper dissection will identify the poste-
rior rotator cuff. The cuff should be debrided of
644
bursal tissue until tendon bers of the
infraspinatus can be visualized. The latissimus
and teres major tendons are then transferred pos-
terior to the long head of the triceps and sutured
directly to the infraspinatus/supraspinatus tendons
with nonabsorbable sutures with the shoulder
positioned in maximum abduction and external
rotation. If the goal is to improve external rotation
without improving abduction, the tendons can
instead be inserted more inferiorly onto the
humeral shaft through the same interval with
suture anchors. The tendons of the latissimus
and teres major should reach easily with a straight
line of pull if adequately released. Partial release
of the most supercial bers of the triceps long
head can improve the line of pull of the transferred
tendons coursing over the triceps. After the
wound is closed, the shoulder is immobilized in
a shoulder spica cast in abduction and external
rotation for 4 weeks before therapy is begun.
Muscle transfers for glenohumeral joint dysplasia
Preoperative planning
OR table: Standard table
Position/positioning aids: Lateral decubitus with bean
bag an axillary roll
Fluoroscopy location: N/A
Equipment: Standard soft tissue instruments. Suture
anchors in case of insertion of tendon transfers in
metaphysis
Tourniquet (sterile/nonsterile): N/A
Latissimus dorsi/teres major transfer for glenohumeral
joint dysplasia
Surgical steps
A transverse axillary incision is used, centering the
incision over the posterior axillary fold
The latissimus tendon inserts into the skin of the axilla, so
these supercial bers must be transected in order to
expose the latissimus and teres major tendons deeper in
the wound
The anterior surface of the latissimus tendon is easily
identied and is traced toward its insertion in the
humerus. Internally rotating the shoulder in abduction
will bring the footprint of the latissimus tendon into better
view. The radial nerve lies immediately anterior to the
tendon insertion and must be retracted with care
The superior and inferior margins of the tendon insertion
are dissected free, taking care to maintain hemostasis at
the superior (deep) margin where abundant vessels exist
(continued)
R. Cornwall
Latissimus dorsi/teres major transfer for glenohumeral
joint dysplasia
Surgical steps
The posterior surface of the teres major tendon is then
identied anterior to the long head of the triceps, tracing
the teres major tendon to its insertion on the humerus
conjoined with the latissimus tendon
Once a circumferential exposure of the conjoined tendon
on the humerus has been achieved, the tendon is released
either sharply or with electrocautery. If electrocautery is
used, a shielded tip and caution are necessary to avoid
injury to the radial nerve anteriorly
Once the tendon is released from the humerus, the tendon
end is tagged with sutures and retracted from the wound.
The muscles are freed from surrounding tissues to the
level of the pedicle in order to allow adequate excursion
for transfer
The interval between the deltoid and triceps (long head)
is then identied. It may help to abduct the shoulder to
only 90 for this step, as the deltoid and triceps bers are
collinear in full abduction, obfuscating the interval
Through this interval, deeper dissection will identify the
posterior rotator cuff. The cuff should be debrided of
bursal tissue until tendon bers of the infraspinatus can
be visualized
The latissimus and teres major tendons are then
transferred posterior to the long head of the triceps and
sutured directly to the infraspinatus/supraspinatus
tendons with nonabsorbable suture with the shoulder
positioned in maximum abduction and external rotation.
If the goal is to improve external rotation without
improving abduction, the tendons can instead be inserted
more inferiorly onto the humeral shaft through the same
interval with suture anchors
The wound is closed with absorbable sutures
Latissimus dorsi/teres major transfer for glenohumeral
joint dysplasia
Postoperative protocol
Type of immobilization: Shoulder spica cast with 90 of
abduction and 90 of external rotation
Length of immobilization: 4 weeks
Rehab protocol: Re-initiation of preoperative rehab
protocol after cast removal, with emphasis on active
elevation and external rotation. Electrical stimulation can
be used to facilitate recruitment of transferred tendons
Trapezius Transfer
Partial trapezius transfer can be used if abduction
and/or external rotation strength remains weak
and the latissimus and teres major muscles are
unsuitable for transfers, such as in severe global
BPBP with residual paralysis of the latissimus
29 Glenohumeral Joint Secondary Procedures for Obstetrical Brachial Plexus Birth Palsy
(Elhassan et al. 2012). The lower trapezius can be
transferred to the infraspinatus, as both muscles
have nearly identical line of pull and excursion.
Through two longitudinal incisions over the
medial and lateral ends of the spine of the scapula,
the lower trapezius can be released from its inser-
tion on the scapular spine and sutured into the
underlying infraspinatus. This transfer cannot be
performed if the spinal accessory nerve has been
used as a nerve transfer to the suprascapular nerve,
and debate currently exists as to which transfer
(nerve vs. muscle) most reliably restores external
rotation strength. Abduction can be improved
with a transfer of the upper trapezius to the del-
toid, as described for adult brachial plexus injuries
with a ail shoulder. This transfer can be
performed even following a spinal accessory to
suprascapular nerve transfer as long as the spinal
accessory nerve branches to the upper trapezius
were preserved in the nerve transfer. Through a
transverse incision along the spine of the scapula
and coursing laterally over the acromion, the
upper trapezius is released from the scapular
spine, acromion, and distal clavicle. The perios-
teal footprint of the insertion is tubularized to
prevent re-adhesion onto the bone of the
acromion. The tubularized trapezius insertion is
then woven into the middle deltoid distal to the
acromion, with the anterior and posterior deltoid
elevated from their origins and sutured to the
supercial surface of the trapezius muscle. Alter-
natively, the insertion of the trapezius onto the
acromion can be detached with a fragment of
bone which is then inserted into the humerus just
distal to the deltoid insertion using
interfragmentary screws. The arm is then
immobilized in a shoulder spica cast in abduction
for 4 weeks before therapy has begun. As this
procedure is typically performed in the most
severe cases, the gain in abduction is far less
than that seen with latissimus and teres major
tendon transfers.
Osteotomies
External rotation osteotomy of the humerus was a
commonly used technique to improve external
645
rotation function prior to the surge in popularity
of soft tissue and articular procedures for the
shoulder internal rotation contracture. Osteotomy
still has a role in the treatment of these
children, most often in the setting of
advanced glenohumeral deformity, or in a child
without sufcient power in muscles to be
transferred. A series of 27 patients who were
followed for at least 2 years after humeral
external rotation osteotomy demonstrated a
5-point improvement in global shoulder function
on the Mallet score with few complications
(Waters and Bae 2006). The authors
conclude that the treatment is effective for chil-
dren with advanced dysplasia that precludes ten-
don transfer surgery. However, it is unknown
what degree of dysplasia actually precludes
successful tendon transfer surgery. A recent
study found anterior attening of the humeral
head to remodel following arthroscopic partial
subscapularis release and latissimus/teres
major tendon transfers, suggesting that humeral
head attening alone should not cause one to
abandon muscle transfers and proceed to
humeral osteotomy (Reading et al. 2012).
Moreover, the results of humeral osteotomy tend
to worsen over time, as a series of 17 children
followed for at least 8 years after low
humeral osteotomy demonstrated a progressive
loss of abduction during the follow-up period
(Al-Qattan et al. 2009). This loss of abduction
may not be a failure of the surgical realignment
but rather a failure of the osteotomy to address the
progression of glenohumeral deformity. It is
therefore imperative to seize the opportunity to
restore glenohumeral alignment and muscle bal-
ance about the shoulder with early dysplasia
detection and intervention.
In light of the importance of restoring
glenohumeral alignment, severe glenohumeral
deformity may be salvageable with glenoid
osteotomy (Dodwell et al. 2012). Early results of
this procedure, analogous to periacetabular
osteotomies for hip dysplasia, demonstrate acute
improvements in glenohumeral dysplasia param-
eters and shoulder function, but potential compli-
cations and long-term outcomes remain to be fully
elucidated.
646
Techniques
Humeral Osteotomy
The patient is positioned in a beach chair or
semirecumbent position to allow exposure of the
shoulder and arm while retaining the ability to
palpate the landmarks of the torso and face
through the drapes. The humeral shaft can be
approached through a deltopectoral incision ante-
riorly or a more cosmetically preferable medial
incision. The deltopectoral approach will require
deeper dissection proximally, as most patients for
whom this procedure is indicated will have a
posteriorly dislocated humeral head. The medial
incision requires careful retraction of the
neurovascular structures in order to expose the
humerus. Once the humeral shaft is exposed
using either approach, a 6-hole plate (usually a
3.5 mm dynamic compression plate) is positioned
over the shaft of the humerus, centered just distal
to the deltoid insertion. The proximal holes are
drilled and measured. The plate is then removed,
and a transverse osteotomy is performed with
careful protection of the soft tissues, especially
the radial nerve posteriorly. The plate is then
afxed to the proximal fragment using the previ-
ously drilled holes, and the distal fragment is
rotated externally approximately 60 . The plate
is provisionally clamped to the distal fragment,
and the shoulder is taken through a range of
motion to ensure that the hand can reach the
mouth and the top of the head without losing the
ability to reach the midline of the abdomen. Once
an adequate position is conrmed, the remaining
screw holes are drilled, and screws are placed in
the distal holes. A sling and swathe is used for
immobilization as would be used for a surgically
treated humeral shaft fracture. Therapy is begun in
earnest once bony union is achieved.
Humeral osteotomy for severe glenohumeral joint
dysplasia
Preoperative planning
OR table: Standard table with hand table extension or
beach chair
Position/positioning aids: Supine with arm of hand table
or upright in beach chair. Regardless of position chosen,
the location of the face and belly must be discernable
(continued)
R. Cornwall
Humeral osteotomy for severe glenohumeral joint
dysplasia
Preoperative planning
through the drapes to ensure that the rotated humerus will
allow the hand to reach the face and belly
Fluoroscopy location: From ipsilateral side
Equipment: Sagittal saw and appropriately sized blade.
Plate and screw set appropriate for the size of humeral
shaft, given the age of the child at the time of operation:
typically 3.5 mm or 4.5 mm locking small fragment plates
Tourniquet (sterile/nonsterile): N/A
Humeral osteotomy for severe glenohumeral joint
dysplasia
Surgical steps
The humeral shaft can be approached through a
deltopectoral incision anteriorly or a more cosmetically
preferable medial incision. The deltopectoral approach
will require deeper dissection proximally, as most
patients for whom this procedure is indicated will have a
posteriorly dislocated humeral head. The medial incision
requires careful retraction of the neurovascular structures
in order to expose the humerus
Once the humeral shaft is exposed using either approach,
a 6-hole plate (usually a 3.5 mm dynamic compression
plate) is positioned over the shaft of the humerus,
centered just distal to the deltoid insertion
The proximal holes are drilled and measured
The plate is then removed and a transverse osteotomy is
performed with careful protection of the soft tissues,
especially the radial nerve posteriorly
The plate is then afxed to the proximal fragment using
the previously drilled holes, and the distal fragment is
rotated externally approximately 60
The plate is provisionally clamped to the distal fragment
and the shoulder is taken through a range of motion to
ensure that the hand can reach the mouth and the top of
the head without losing the ability to reach the midline of
the abdomen
Once an adequate position is conrmed, the remaining
screw holes are drilled and screws are placed in the distal
holes
The wound is closed with absorbable sutures
Humeral osteotomy for severe glenohumeral joint
dysplasia
Postoperative protocol
Type of immobilization: Sling and swathe
Length of immobilization: Until bony union typically
4 weeks
Rehab protocol: Preoperative therapy is resumed after
bony union, with emphasis on functional goals involving
external rotation
Return to sport protocol: Following bony union
29 Glenohumeral Joint Secondary Procedures for Obstetrical Brachial Plexus Birth Palsy
Preferred Treatment
Central to preferred treatment algorithms is the
early detection of glenohumeral dysplasia and
early intervention to restore skeletal alignment,
function, and muscle balance. The historical sec-
ondary distinction of muscle and joint surgeries
implicates that these procedures should follow
any nerve surgery performed. However,
glenohumeral joint dysplasia can require attention
in the early months of infancy, even before con-
sideration is given to nerve surgery. Thus, pre-
ferred treatments hinge on early detection of the
so-called secondary problems. Physical and/or
occupational therapy is begun within the rst
month of life, and serial examinations in the ensu-
ing months are used to detect muscle functional
recovery as well as any development of internal
rotation contractures. Progressive worsening of
internal rotation contractures, or any contracture
with less than 20 of passive external rotation in
adduction, prompts an ultrasound. If the ultra-
sound is normal or demonstrates a posteriorly
subluxated humeral head that can be reduced in
external rotation, efforts to restore or maintain
passive external rotation are redoubled with ther-
apy or with botulinum toxin injections if the ther-
apy is hindered by resistance from strong
pectoralis major contraction. If, however, the
ultrasound demonstrates that the glenohumeral
joint is completely dislocated, an MRI is
performed to better evaluate the nature of the
glenohumeral deformity, and the child is indicated
for operative reduction.
Fig. 5 (a) Maximum
passive internal rotation
following complete
subscapularis release and
external rotation tendon
transfers. (b) Internal
rotation osteotomy of the
humerus was required to
restore middling function
647
If operative reduction of an infantile disloca-
tion is required, an arthroscopic release of the
superior subscapularis is performed, with an ultra-
sound used intraoperatively to conrm reduction.
If necessary, capsular release may be added if
partial subscapularis release alone does not restore
adequate passive external rotation to reduce the
joint. In no instance is complete subscapularis
release performed. A high rate of redislocation
of infantile dislocations has been observed with
release, reduction, and casting alone, so if a child
requires surgery in infancy to reduce a dislocation,
latissimus/teres major transfer is performed at the
same surgery in order to provide immediate pos-
terior stability to the humeral head. It is unknown
why some children progress to dislocation very
rapidly in infancy, but it is speculated that those
children will not reliably recover active external
rotation and abduction strength if treated with a
release and reduction alone. Conversely, combin-
ing arthroscopic partial subscapularis release,
ultrasound-conrmed reduction, and tendon
transfers can lead to improvements in function
and substantial glenohumeral remodeling (Fig. 5).
Arthroscopic partial release of the
subscapularis for internal rotation contractures is
preferred in older children as well, combining the
release with a latissimus/teres major transfer if
concomitant weakness of abduction and external
rotation is present as well. Alternatively, a transfer
of the spinal accessory nerve to the suprascapular
nerve can be combined with a transfer of the
triceps long head branch of the radial nerve to
the deltoid branch of the axillary nerve in the
young child (<1824 months of age) in lieu of
648
the tendon transfers. The relative indications and
merits of muscle versus nerve transfer remain to
be elucidated. However, if restoration of the bal-
ance of forces about the glenohumeral joint is the
goal, it should be kept in mind that such balance
will not be achieved until several months follow-
ing nerve transfer, given axonal regeneration time
to the infraspinatus. Therefore, to buy time,
botulinum toxin injection in the pectoralis major
and/or subscapularis may temporarily avoid per-
sistent internal rotation imbalance while awaiting
infraspinatus reinnervation.
Humeral external rotation osteotomy is
reserved for children with severe glenohumeral
deformity and age that precludes substantial
glenohumeral remodeling, along with function-
ally limiting decits in external rotation. This
osteotomy is not performed in children who lack
midline internal rotation function, as such func-
tion will be further worsened by an external rota-
tion osteotomy. In these cases, consideration is
given to glenoid osteotomy to restore a fulcrum
for glenohumeral motion, combined with tendon
transfers to power such motion. Again, the role for
glenoid osteotomy remains unclear. Similarly, an
algorithm for surgical treatment of the abduction
contracture has not yet been adopted, as surgical
techniques and outcomes have yet to be fully
dened in clinical studies.
Outcomes
Many series have reported predictable improve-
ments in passive external rotation, active abduc-
tion and external rotation, Mallet scores, and
patient-reported upper extremity function follow-
ing a variety of combinations of the surgical pro-
cedures described in this chapter (Louden
et al. 2013). In general, improvements of 35
points on the aggregate Mallet score are common,
but the nal function is far from normal. If a
Mallet score of 4 out of 5 in most domains is
considered a success, then only 80 % function in
the affected limb is being restored. Nonetheless,
the improvements in upper extremity function and
quality of life according to the patients may
exceed the improvements in physician-reported
R. Cornwall
measures (Dedini et al. 2008), and the impact
these procedures have on children should not be
underestimated. Furthermore, the early detection
and treatment of glenohumeral dysplasia and dis-
location can allow formation of a relatively nor-
mal glenohumeral joint, perhaps leading to
sustained gains in function for many years into
adulthood.
Management of Complications
Despite advances in the understanding of and
treatments for the shoulder dysfunction following
BPBP, surgical treatment of the internal rotation
contracture is not without complications, espe-
cially with regard to achieving optimal balance
between internal and external rotation. Several
recent series of a variety of open and arthroscopic
techniques have called attention to the postopera-
tive loss of active internal rotation function (van
der Sluijs et al. 2004; Kambhampati et al. 2006;
Kozin et al. 2006; Newman et al. 2006; Pearl
et al. 2006). The resulting severe, functionally
disturbing external rotation contracture can
require internal rotation humeral osteotomy to
restore midline function (Fig. 6) (van der Sluijs
et al. 2004). For this reason, consideration should
be given to only partial release of the subscapularis
tendon or to less aggressive musculotendinous
lengthening. Conversely, the internal rotation con-
tracture may recur following release and tendon
transfers. A recent report describes revision
subscapularis lengthening with transfer of the
lower trapezius muscle to the infraspinatus tendon
with improvements in active external rotation.
Future Research
Recent years have seen a renewed enthusiasm for
research regarding the secondary shoulder prob-
lems in BPBP. Scientic inquiry in animal models
and humans will elucidate the underlying perturba-
tions of muscle development that result from dener-
vation during a critical window of postnatal muscle
development. Such knowledge may lead to medi-
cal intervention to preserve muscle development
29 Glenohumeral Joint Secondary Procedures for Obstetrical Brachial Plexus Birth Palsy
Fig. 6 (a) Complete dislocation of the glenohumeral joint
with severe glenoid dysplasia in a 6-month-old child with
BPBP. (b) Nearly complete remodeling of the
glenohumeral joint 2 years after arthroscopic partial
while awaiting reinnervation, preventing contrac-
tures, and obviating the need for many of the sur-
geries described in this chapter. Until then,
innovative surgical approaches to glenohumeral
deformity will expand the number of children that
can progress to adulthood with improved
glenohumeral alignment. Motion analysis will
help us understand the kinematics of abnormal
shoulder motion in these children, helping to
guide nonoperative and operative therapies to opti-
mize function. Perhaps most importantly, clinical
research increasingly using patient-reported out-
comes will keep the focus on what is done for the
children, not just what is done to the children.
Summary
The secondary musculoskeletal problems in the
shoulder are the most common reasons for surgi-
cal intervention following BPBP. Early attention
to these complications of the neurological injury
can allow restoration of normal growth and devel-
opment of the glenohumeral joint. Function can
be reliably improved following contracture
release and tendon transfers to augment shoulder
strength, but normal function cannot be restored.
Continued research into the biological and biome-
chanical pathophysiology of the contractures and
dysplasia will open the door to novel prevention
and treatment strategies in the coming years.
649
subscapularis release, ultrasound-conrmed glenohumeral
reduction, and latissimus dorsi/teres major tendon transfers
to the posterior superior rotator cuff
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 Secondary Procedures About
the Elbow, Forearm, Wrist, and Hand 30
Scott H. Kozin, Dan A. Zlotolow, and Joshua M. Abzug

Contents Abstract
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 653 Secondary procedures about the elbow, fore-
arm, wrist, and hand following brachial plexus
Elbow . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 654
Elbow Flexion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 654
injury are commonplace. Unbalanced and/or
Elbow Extension . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 660 inadequate recovery leads to impairment
about these joints. Each respective joint has
Forearm . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 666
Technique: Pronator Teres Tendon Rerouting . . . . . . . 667 importance during the performance of activi-
Technique: Biceps Tendon Rerouting . . . . . . . . . . . . . . . 668 ties of daily living and promoting independent
Technique: Osteotomy of Radius and/or Ulna . . . . . . 670 function. In addition, each joint has unique
Technique: One-Bone Forearm . . . . . . . . . . . . . . . . . . . . . . 673 challenges during formulation of a reconstruc-
Wrist and Hand . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 676 tive treatment plan. The mainstays of manage-
Technique: Flexor Digitorum Supercialis ment are tendon transfer, osteotomy, and
Tendon Transfer . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 676
arthrodesis. This chapter will discuss the par-
Rehabilitation and Outcome . . . . . . . . . . . . . . . . . . . . . . . 680 ticulars of managing impairment about the
Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 681 elbow, forearm, wrist, and hand. The indica-
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 681
tions for treatment and the variety of surgical
options will be discussed in detail. Authors
preferred techniques will be highlighted as
good judgment comes from experience and
experience comes from bad judgment.

Introduction

Residual brachial plexus injuries result in upper

limb impairment. The limited motion can affect
the shoulder, elbow, forearm, wrist, and/or hand.
S.H. Kozin (*) D.A. Zlotolow Muscle imbalance and reinnervation often result
Shriners Hospitals for Children, Philadelphia, PA, USA
in contracture over time. The shoulder is a prime
e-mail: skozin@shrinenet.org; dzlotolow@yahoo.com
example of this occurrence and has been
J.M. Abzug
discussed in the preceding chapter. This chapter
University of Maryland School of Medicine, Baltimore,
MD, USA will focus on the elbow, forearm, wrist, and hand
e-mail: Jabzug@umoa.umm.edu with an emphasis on diagnosis and treatment.
# Springer Science+Business Media New York 2015 653
J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_30
654
Many of the preferred procedures have developed
over time with ongoing modications based on
clinical experience (both good and bad). The sur-
gical tenets of tendon transfer, osteotomy, and
fusion are readily applicable to children and adults
with residual brachial plexus palsy. This chapter
will focus on children; however, the principles are
similar in adults. The main difference lies in the
reinnervation issues associated with the pediatric
muscles that result in contracture formation
(Nikolaou et al. 2011).
Secondary brachial plexus surgery
Nonoperative management
Indications Contraindications
Minimal functional Motivated patient with available
decits motors for transfer
Stiff joints
Unavailable motors
for transfer
Secondary brachial plexus surgery
Physical/occupational therapy recommendations
Maintain supple range of motion
Alleviate contracture
Maximize independence
Participate in the decision-making process
Elbow
Elbow exion is motored by the biceps, brachialis,
and brachioradialis muscles. Innervation is primar-
ily from the C5 and C6 nerve roots. Elbow exten-
sion is driven by the triceps and anconeus muscles.
Innervation is mainly C7, although the lower trunk
can contribute to triceps innervation (a.k.a. lower
trunk triceps) (Kozin 2007a).
Elbow Flexion
Since the most common brachial plexus injury
affects C5 and C6 (a.k.a. Erbs palsy), lack of
elbow exion is the principal problem. Failure to
regain elbow exion via spontaneous recovery of
nerve reconstruction (nerve graft or nerve trans-
fer) requires treatment. The loss of elbow exion
S.H. Kozin et al.
yields substantial impairment and difculties with
numerous activities of daily living, such as feed-
ing and grooming.
Tendon transfer is the primary method to
restore elbow exion. There are a variety of poten-
tial donors, including the exor-pronator mass
(Steindler), triceps, latissimus dorsi, pectoralis
major, and triceps muscle (Kozin 2002; Chuang
et al. 1993). There are advantages and disadvan-
tages to each of these transfers. The exor-
pronator transfer is relatively straightforward;
however, this transfer provides limited and weak
elbow exion to about 90 . In addition, the
patients must ex the ngers to bend the elbow
(Steindler effect). This limits the usability of the
transfer. The triceps to biceps transfer is effective
in restoring elbow exion, but the donor decit is
substantial. The loss of elbow extension leads to a
decreased available workspace and elbow exion
contracture over time. The pectoralis major trans-
fer can be unipolar or bipolar. The unipolar trans-
fer detaches the insertion and transfers the tendon
to the biceps using an intervening graft. This
transfer if often ineffective and induces a concom-
itant internal rotation force at the shoulder. The
bipolar transfer detaches both the origin and inser-
tion and rotates the entire muscle into the arm.
This transfer is effective, but the scarring and
disgurement are considerable. The latissimus
dorsi muscle is our preferred donor for tendon
transfer (Zancolli and Mitre 1973; Kozin 2002).
The latissimus dorsi innervation is from the
thoracodorsal nerve with a large C7 component.
Therefore, the muscle is often available in C5 and
C6 injuries. Furthermore, the loss of the latissimus
dorsi has negligible effect on function, especially
if the teres major is working.
Technique: Bipolar Latissimus Dorsi
Transfer
Bipolar latissimus dorsi transfer
Preoperative planning
OR table: regular
Position/positioning aids: lateral decubitus with bean bag
Fluoroscopy location: none
Equipment: standard
Tourniquet: none
30 Secondary Procedures About the Elbow, Forearm, Wrist, and Hand
Fig. 1 An eighteen-month-old with infraclavicular bra-
chial plexus injury, absent elbow exion, and insufcient
soft tissue along the anterior arm. The latissimus dorsi
muscle harvested on the thoracodorsal neurovascular ped-
icle prior to transfer beneath the pectoralis major muscle
(Courtesy of Shriners Hospital for Children, Philadelphia)
Bipolar latissimus dorsi transfer is performed
with the patient in the lateral decubitus position.
The arm and torso are prepped and draped to the
midline for adequate exposure. The ipsilateral
thigh is also prepared to allow for harvesting
fascia lata autograft. For allograft, an Achilles
tendon or posterior tibialis tendon is procured. A
long linear incision is performed along the poste-
rior axillary line extending from the insertion of
the latissimus dorsi muscle along the muscle belly
toward the iliac crest. A large skin ap is elevated
over the posterior aspect of the latissimus all the
way to the midline. The lateral border of the
muscle is identied and separated from the under-
lying serratus anterior muscle. The muscle with
the thoracodorsal fascia is harvested from distal to
proximal. The thoracodorsal neurovascular pedi-
cle is isolated between the proximal 1/3 and distal
2/3 s on the undersurface of the latissimus. Metic-
ulous hemostasis is attained throughout this dis-
section using electrocautery. The entire latissimus
dorsi muscle is harvested on the thoracodorsal
neurovascular pedicle (Fig. 1). Mobilization of
the vascular pedicle to the subscapular artery is
performed, with ligation of the arterial branch
(lateral thoracic artery) to the serratus muscle.
The tendinous insertion of the latissimus ten-
don into the humerus is divided. A separated
655
Fig. 2 The latissimus dorsi muscle passed under pectoralis
major and into arm for restoration of elbow exion (Cour-
tesy of Shriners Hospital for Children, Philadelphia)
anterior deltopectoral approach is then performed,
and the latissimus dorsi muscle and pedicle are
passed from posterior to anterior into this interval
(Fig. 2). The thoracodorsal vessels must avoid
torsion during passage. A large subcutaneous pas-
sage is tunneled from the deltopectoral interval to
the antecubital fossa and the biceps tendon is
isolated. The origin of the latissimus dorsi is
attached to the biceps tendon utilizing a weave
technique. Augmentation with fascia lata auto-
graft or allograft strengthens the repair. The
antecubital incision is closed before setting
nal tension at the anterior shoulder level. The
latissimus tendon is attached to the coracoid or
acromion, which should yield a 30 tenodesis
effect at the elbow. Suction drains are placed
into the back wound. The arm is immobilized
to the chest wall for 6 weeks with the elbow at
90 . Protective extension block splinting of the
elbow is continued until 3 months after surgery
(Fig. 3).
Bipolar latissimus dorsi transfer
Surgical steps
Long linear incision is performed along the posterior
axillary line
Identify lateral border of the latissimus
Separate from the underlying serratus anterior muscle
Isolate thoracodorsal neurovascular pedicle
(continued)
656 S.H. Kozin et al.

Bipolar latissimus dorsi transfer

Potential pitfalls and preventions
Potential pitfall Pearls for prevention
Pitfall #1 Difcultly Separate the serratus
isolating the neurovascular anterior from the
pedicle latissimus dorsi muscle
Pitfall #2 Inadequate Dissect proximal to the
pedicle length subscapular artery; ligate
the lateral thoracic artery
Pitfall #3 Suboptimum Augmentation with
attachment to the biceps autograft or allograft
tendon
Pitfall #4 Postoperative Avoid removing drains
seroma in the back too early; wait until
patient is ambulating
Pitfall #5 Difculty with Enlist an experienced
ring transfer therapist; try alternative
methods such as
biofeedback

Fig. 3 Postoperative right elbow exion against gravity
with well-healed posterior axillary incision (Courtesy of
Shriners Hospital for Children, Philadelphia)
Bipolar latissimus dorsi transfer
Surgical steps
Mobilization of the vascular pedicle to the subscapular
artery and ligate lateral thoracic artery
Perform deltopectoral approach and antecubital approach
Isolate the coracoid, acromion, and biceps tendon
Create large subcutaneous tunnel between deltopectoral
and antecubital incisions
Release origin and insertion of the latissimus muscle
Pass the muscle from back to front without kinking the
neurovascular pedicle
Attach origin of the latissimus dorsi to the biceps tendon.
Augment with fascia lata autograft or allograft
Attach the latissimus tendon to the coracoid or acromion
Bipolar latissimus dorsi transfer
Postoperative protocol
Suction drains are placed into the back wound
Arm immobilized to the chest wall for 6 weeks with the
elbow at 90
Tendon transfer ring and reeducation instituted
Protective extension block splinting of the elbow is
continued until 3 months after surgery
Free-Tissue Transfer
Free-tissue transfer is considered when there are
no available local donors (Chuang et al. 1993,
1996; Manktelow 1986). The primarily transfer-
rable muscle is the gracilis based upon the obtu-
rator neurovascular supply (Manktelow 1986).
The motor nerve to empower the free-muscle
transfer is either the spinal accessory or intercostal
nerves. This surgical procedure requires adequate
training and skill in microvascular techniques.
Technique: Free Gracilis Muscle Transfer
Free gracilis muscle transfer
Preoperative planning
OR table: regular
Position/positioning aids: standard, prep the contralateral
donor thigh and leg along with the recipient arm and
hemithorax
Fluoroscopy location: none
Equipment: intraoperative and postoperative Doppler
(Cook-Swartz Doppler), nerve stimulator (checkpoint
stimulator), microscope, microsurgical instrumentation,
brin glue
Tourniquet: none
The surgery can be performed with one or two
surgical teams. The gracilis muscle is harvested
from the contralateral thigh as the pedicle will
orient better than an ipsilateral harvest (Fig. 4).
The gracilis is isolated posterior to the adductor
30 Secondary Procedures About the Elbow, Forearm, Wrist, and Hand 657

Fig. 4 An eight-year-old
child with brachial plexus
birth palsy status post
exploration and grafting as
an infant with persistent
lack of right elbow exion.
Left gracilis harvest
incision diagrammed
(Courtesy of Shriners
Hospital for Children,
Philadelphia)

magnus and longus muscles via a long medial

incision from the pubis to the distal aspect of the
thigh. The obturator neurovascular pedicle is iso-
lated in the proximal 1/3 of the muscle and care-
fully traced in a proximal direction to increase the
length of the pedicle (Fig. 5). The dominant arterial
supply is provided by a branch from the profunda
femoris. There are consistently two veins, a single
artery, and a single nerve. The tendinous origin is
isolated proximal to the neurovascular pedicle. The
distal dissection is straightforward and the gracilis
is isolated as distal as possible. The tendinous
insertion is released from the pes anserinus. Fre-
quently, a skin paddle is harvested with the muscle
to enhance donor site coverage and facilitate ap
monitoring after surgery.
The upper limb is prepared by isolating the
nerve(s) to power the gracilis muscle and nding
an appropriate artery for inow and veins for out-
ow. The spinal accessory nerve is isolated via a
supraclavicular brachial plexus approach. The most
facile technique requires isolation of the trapezius
muscle at its attachment into the acromion. Dissec-
tion proceeds across the muscle better in a slow and Fig. 5 Left gracilis muscle isolated and pedicle extended
deliberate fashion. The spinal accessory nerve and in a proximal direction (Courtesy of Shriners Hospital for
its adjacent artery are encountered coursing along Children, Philadelphia)
the trapezius muscle. Electrical stimulation with a
biphasic stimulator (Checkpoint device, Check- The intercostal nerves are harvested via an
point Surgical LLC, Cleveland, Ohio, USA) is inframammary incision that extends from the
helpful. There are branches of the cervical plexus costochondral junction to the posterior axillary
in the vicinity that will not stimulate muscle con- fold (Kozin 2007a; Krakauer and Wood 1994;
traction and should not be confused with the spinal Malessy and Thomeer 1998). The skin along with
accessory nerve. the pectoralis major and pectoralis minor is elevated
658 S.H. Kozin et al.

Fig. 6 Gracilis muscle

innervated via intercostal
nerve transfers (blue
background) (Courtesy of
Shriners Hospital for
Children, Philadelphia)

in a cephalad direction to expose the underlying

ribs. Three intercostal nerves are preferred to
power the gracilis muscle (Fig. 6). The nerves are
harvested by reecting the intercostal muscle in an
inferior direction. A two-pronged hook is carefully
placed on the rib and retracted in cephalad direction.
The underlying intercostal nerve is isolated and a
vessel loop placed around the nerve. The nerve is
then painstakingly dissected from costochondral
junction to the posterior axillary fold. Meticulous
technique is necessary to avoid entering the lung
pleura. This process is repeated three times.
The donor artery and veins must also be iden-
tied and prepared. The choice varies with the size
of the patient, the diameter of the vessels, the
presence or absence of previous surgery, and the
preference of the surgeon. A frequent selection is Fig. 7 Gracilis muscle anastomosis via end to side to
brachial artery and vein (Courtesy of Shriners Hospital
end to side anastomosis to the brachial artery and for Children, Philadelphia)
end to end anastomosis to cephalic vein branches
(Fig. 7). Microsurgical expertise is a perquisite, the tendon passed to the elbow to provide some
especially in children with small caliper vessels. stability to muscle. Full attention is then directed to
The arm is further prepared by isolation of the microsurgical arterial and venous anastomosis. The
attachment site for the gracilis origin and inser- arterial repair is completed rst to minimize the
tion. The origin is either the clavicle or the ischemia time. Subsequently, the two venous anas-
acromion. The insertion site is usually any tomosis repairs are completed. After successful
remaining biceps tendon or directly into the anastomoses, attention is directed to nerve coapta-
coronoid if the biceps is completely absent. tion using suture and/or brin glue (Tisseel, Baxter
Once the donor and recipient sites are prepared, Pharmaceuticals, Deereld, Illinois, USA). Dopp-
the gracilis pedicle is ligated and the muscle ler cuffs (Cook Catheters, Cook Medical Inc.,
transported into the arm. The origin is secured and Bloomington, Indiana, USA) are placed around
30 Secondary Procedures About the Elbow, Forearm, Wrist, and Hand 659

the artery and a vein for postoperative monitoring. Free gracilis muscle transfer
Lastly, the gracilis tendon is woven into the biceps Postoperative protocol
tendon or secured through a drill hole in the Suction drains are placed into the thigh wound
coronoid. Tension is set such that there is no tension Arm immobilized to the chest wall for 6 weeks with the
across the neurovascular repair and the elbow rests elbow at 90
in about 30 of exion. Protective extension block splinting of the elbow is
The wounds are all closed with suction drains continued until reinnervation
Tendon transfer ring and reeducation instituted once
placed into the thigh. The arm is immobilized with
reinnervation is evident. If the intercostal nerves are
the arm at the side and elbow in 90 of exion. utilized, coughing often activates the muscle
The patient is transferred to the intensive care unit
for ap monitoring via the skin paddle and Dopp-
ler catheters. Routine anticoagulation other than a
daily aspirin is not recommended. The Doppler Free gracilis muscle transfer
cuffs are removed 5 days after surgery. The wires Potential pitfalls and preventions
Potential pitfall Pearls for prevention
can be cut rather than pulled, which may result in
Pitfall #1 Neurovascular Trace pedicle in proximal
vascular disruption.
pedicle too short direction to ensure
The arm is immobilized for 6 weeks and gentle adequate length
passive range of motion started. The terminal 30 Pitfall #2 Difculty with Critical step in setup.
of elbow extension is not allowed until signs of anastomosis Make sure the recipient
reinnervation and recruitment become evident. vessels are adequately
prepared
Appropriate therapy is mandatory to achieve a
Pitfall #3 Failure of Be prepared to try again
successful outcome. anastomosis and have emergency OR
team available
Free gracilis muscle transfer Pitfall #4 Donor difculty Resides directly on top of
identication of spinal the trapezius muscle;
Surgical steps
accessory stimulator is helpful
Long linear incision along the medial aspect of the donor
Pitfall #5 Donor difculty Small rents in the parietal
thigh
intercostal nerve harvest pleura do not require chest
Identify the gracilis from the pubis to the distal aspect of can result in tube placement; large rents
the thigh pneumothorax require chest tube
Isolate the obturator neurovascular pedicle in the
proximal 1/3 of the muscle
Trace neurovascular pedicle in proximal direction to
increase the length of the pedicle
Release the tendinous insertion from the pes anserinus
Double Free-Tissue Transfer
Prepare the recipient upper limb In extremely desperate cases with ail limbs,
Select and isolate the donor nerve(s), artery, and veins. In double free-muscle transfers are a valiant attempt
a similar fashion, isolate the eventual origin and insertion to restore marginal function (Doi et al. 2000).
sites The double Doi is performed in two stages. In
Ligate the obturator neurovascular pedicle and transport the rst operation, nger extension and elbow ex-
the muscle into the arm
ion are restored by a single free-muscle transfer
Stabilize the gracilis muscle in the arm by passing the
tendon to the elbow
innervated by the spinal accessory nerve. Finger
Complete arterial repair to minimize the ischemia time exion is restored by a second free muscle inner-
Complete two venous anastomoses to allow for venous vated by the fth and sixth intercostal nerves. The
egress third and fourth intercostal nerves are coapted to the
Coapt obturator nerve to donor nerve(s) motor branch of the triceps muscle to restore elbow
Secure gracilis origin to clavicle or coracoid and extension and to stabilize the elbow against the
complete distal weave to the biceps tendon exion moment generated by the rst muscle
Apply Doppler cuffs and close transfer.
660
Elbow Extension
Decient elbow extension removes an antagonist
to elbow exion and inhibits certain activities of
daily living. The lack of an antagonist to elbow
exion results in an elbow contracture due to the
unimpeded forces in the elbow exors. Certain
activities of daily living can be accomplished by
gravity-assisted elbow extension. In contrast,
elbow extension against gravity is required for
overhead activities and pushing up from a chair.
In patients that are upper extremity weight bearers
(e.g., paraplegia or crutch walkers), the loss of
strong active elbow extension is especially prob-
lematic. There are a limited number of donors for
elbow extension. Options include the posterior
deltoid, latissimus dorsi, and biceps (Kozin
2007b). Free-muscle transfer is another possibil-
ity. The choice of donor is confounded by trying
to avoid those muscles that have been
reinnervated. Reinnervated muscle is often
weaker and loses some of its physiologic proper-
ties (excursion and cross-sectional area). In addi-
tion, mass reinnervation can result in the inability
to selectively re an individual muscle. A careful
physical examination is necessary to ensure inde-
pendent ring. On occasion, dynamic electromy-
ography can facilitate the decision-making
process (Hutchinson et al. 2008).
The two main donors are the biceps and the
posterior deltoid muscles (Mulcahey et al. 2003;
LeClerq et al. 2008). Each procedure has its own
nuances. The biceps to triceps is easier and has a
less rigid postoperative rehabilitation. The poste-
rior deltoid transfer requires an intervening graft
that complicates the technique and adds greater
postoperative restrictions.
Technique: Biceps to Triceps Transfer
Operative Prerequisites
Active brachialis and supinator muscles are
requirements to biceps transfer to maintain
elbow exion and forearm supination (Kozin
2003; Kuz et al. 1999). The evaluation of their
presence requires a vigilant physical examination
of elbow exion and forearm supination strength.
The brachialis and supinator muscles can be
S.H. Kozin et al.
assessed independent of the biceps muscle. Effort-
less forearm supination without resistance will
cause supinator function that is palpable along
the proximal radius. Likewise, powerless elbow
exion causes palpable brachialis contraction
along the anterior humerus deep to the biceps
muscle. Equivocal cases require supplementary
evaluation to guarantee adequate supinator and
brachialis muscle activity. Injection of the biceps
muscle with a local anesthetic (e.g., bupivacaine)
induces temporary paralysis. Subsequently, the
examiner can assess independent assessment of
brachialis and supinator function via elbow ex-
ion and forearm supination, respectively.
A supple elbow with near complete range of
motion is also necessary. Patients with an elbow
contracture require resolution of the contracture
prior to surgery. Therapy and/or serial casting can
lessen the contracture. Surgery is deferred until
the contracture is less than 20 . A greater contrac-
ture negates the biceps tendon from reaching the
olecranon at surgery.
Technique: Biceps to Triceps Transfer
Biceps to triceps transfer
Preoperative planning
OR table: regular
Position/positioning aids: supine, meticulous padding of
all bony prominences
Fluoroscopy location: none
Equipment: standard
Tourniquet: sterile
The patient is placed supine on the operating
room table. The entire extremity is prepped and
draped. A sterile circular tourniquet is applied
(HemaClear, OHK Medical Devices, Grandville,
Michigan, USA) that exsanguinates during appli-
cation. An S-shaped incision is made along the
medial arm, across the antecubital fossa, and over
the brachioradialis muscle belly. Skin aps are
elevated and the biceps tendon is traced to its
insertion into the radial tuberosity. There are
large crossing veins that require ligation. The
lacertus brosus can be incorporated into the
transfer or left behind (Kozin 2003; Kuz
et al. 1999; Revol et al. 1999; Kozin et al. 2010).
The tendon is released directly from the radial
30 Secondary Procedures About the Elbow, Forearm, Wrist, and Hand 661

Fig. 8 An eighteen-year-
old female with lack of
elbow exion necessitating
biceps to triceps transfer.
Biceps harvested from
radial tuberosity and
proximal dissection
performed to improve
excursion (Courtesy of
Shriners Hospital for
Children, Philadelphia)

Fig. 9 Biceps passed along

the medial side of the arm to
the posterior incision
beneath the ulnar nerve
(Courtesy of Shriners
Hospital for Children,
Philadelphia)

tuberosity to maximize length. The tendon and
muscle belly are mobilized proximal into the
arm to maximize excursion and to improve line
of pull (Fig. 8). The musculocutaneous and lateral
antebrachial nerves must be protected.
Only the medial side of the arm, the median
nerve, the brachial artery, and the ulnar nerve are
isolated. A posterior incision is made around the
olecranon and extended in a proximal direction
along the triceps tendon. A subcutaneous tunnel is
created between the medial brachium and
posterior incision for tendon passage (Fig. 9).
The tendon can be passed over the median and
ulnar nerves; however, the advance in
neuroregeneration has changed our practice. The
tendon is passed over the median nerve, but under
the ulnar nerve to avoid any iatrogenic ulnar nerve
compression (Fig. 10). A Krackow suture is
placed in the tendon with the two suture ends
left long.
The triceps tendon is split and the tip of the
olecranon is exposed. A large bore blind bone
662
Fig. 10 Biceps passed
beneath the ulnar nerve
(Courtesy of Shriners
Hospital for Children,
Philadelphia)
Fig. 11 Biceps tendon passed though triceps tendon prior
to docking into olecranon (Courtesy of Shriners Hospital
for Children, Philadelphia)
tunnel is made in the olecranon for the acceptance
of the biceps tendon. The tendon is passed
through the medial leaet of the triceps tendon
in preparation of docking into the bone tunnel
(Fig. 11). The tendon is docked using two poste-
rior unicortical holes and suture retrievers (Suture
S.H. Kozin et al.
Fig. 12 Suture retrievers to facilitate docking biceps ten-
don into osseous tunnel (Courtesy of Shriners Hospital for
Children, Philadelphia)
retriever, Smith Nephew, Andover, Massachu-
setts, USA) (Kozin and Zlotolow 2012)
(Fig. 12). The elbow is placed in full extension
and the two ends are drawn into the bone tunnel
using suture retrievers (Fig. 13). The sutures are
tied over the posterior olecranon cortex.
30 Secondary Procedures About the Elbow, Forearm, Wrist, and Hand 663

Biceps to triceps transfer

Postoperative protocol
Elbow extension cast for 1 week
Gradually mobilize the elbow and initiate transfer
training at 15 degrees per week, using a dial-hinge brace,
Bledsoe brace (Bledsoe Brace Systems, Grand Prairie,
Texas, USA)
Tendon transfer ring and reeducation instituted
Protective splinting of the elbow is continued until
3 months after surgery

Biceps to triceps transfer

Potential pitfalls and preventions
Potential pitfall Pearls for prevention
Pitfall #1 Bleeding Large crossing veins require
during biceps harvest careful ligation during
dissection
Pitfall #2 Inadequate Resolve any elbow
biceps length to reach contracture prior to surgery
olecranon
Pitfall #3 Difculty Try alternative methods, such
ring transfer after as biofeedback
surgery
Pitfall #4 Difculty Be patient; avoid passive
Fig. 13 Biceps tendon docked into olecranon (Courtesy regaining elbow elbow exion until 3 months
of Shriners Hospital for Children, Philadelphia) exion following surgery

Technique: Posterior Deltoid Transfer

Additional suturing is performed during the clo-
sure of the triceps split with incorporation of the
biceps tendon. Following closure, a well-padded
long-arm cast is applied with the elbow in exten-
sion. The wrist is included within the cast and the
hand position depends upon concomitant proce-
dures performed for hand function.
Biceps to triceps transfer
Surgical steps
S-shaped incision is made along the medial arm, across
the antecubital fossa, and over the brachioradialis muscle
belly
The biceps tendon traced to insertion into the radial
tuberosity, released, and mobilized in a proximal
direction
Posterior incision around the olecranon and extended in a
proximal direction, the triceps tendon is split and the tip
of the olecranon is exposed
The biceps tendon passed from anterior to posterior via
spacious tunnel that runs over median nerve and deep to
ulnar nerve
The biceps tendon passes through triceps leaet and is
secured within osseous tunnel using a docking technique
Operative Prerequisites
The posterior deltoid must have adequate strength
to motor elbow extension. Shoulder extension and
palpation best assess posterior deltoid strength and
turgor. The examination can be performed with the
patient seated, although placing the patient prone
provides a more reliable assessment. Similar to
biceps to triceps transfer, a supple elbow with
near complete range of motion is also necessary.
Patients with an elbow contracture require resolu-
tion of the contracture prior to surgery.
Posterior deltoid transfer
Preoperative planning
OR table: regular
Position/positioning aids: supine with bump under
shoulder, meticulous padding of all bony prominences.
Can also position in lateral decubitus position if
performing isolated posterior deltoid transfer
Fluoroscopy location: none
Equipment: standard, allograft
Tourniquet: none
664
Fig. 14 A ve-year-old with right brachial plexus birth
palsy and lack of elbow extension. Incisions for deltoid to
triceps transfer (Courtesy of Shriners Hospital for Chil-
dren, Philadelphia)
The patient is positioned in the lateral
decubitus position with the affected side up
(LeClerq et al. 2008; Kozin 2007b). An axillary
roll is applied. All bony prominences are padded.
Prior to prepping and draping, Marcaine with
epinephrine is instilled in the planned incision
sites. The upper extremity and hemithorax are
then prepped and draped in usual sterile fashion.
A gently curved incision is made over the deltoid
muscle (Fig. 14). Skin aps are elevated. The
axillary nerve is identied entering the deltoid
muscle (Fig. 15). The posterior half of the deltoid
is selected for transfer (Fig. 16). A periosteal
sleeve is elevated with the posterior deltoid. Prox-
imal dissection is performed to improve the excur-
sion and line of pull.
A distal incision is then made through the distal
third of the triceps tendon. The triceps tendon is
identied. The ulnar nerve is isolated and
retracted out of harms way.
S.H. Kozin et al.
Fig. 15 Axillary nerve delineated and protected (Courtesy
of Shriners Hospital for Children, Philadelphia)
A subcutaneous tunnel is then created between
these two incisions using sequential hemostats
(Fig. 17). An allograft is favored to bride the
distance between the deltoid muscle and triceps
tendon. The allograft is thawed and the proximal
end and sutured to the posterior deltoid and under-
lying periosteum (Fig. 18). The remaining tendon
is then passed through the subcutaneous tunnel to
the distal incision (Fig. 19). The elbow is placed in
full extension and the allograft woven into the
triceps tendon and/or bone for additional augmen-
tation (Fig. 20).
Following rm xation, all wounds are irri-
gated and closed in layered fashion. The patient
is placed in a long-arm cast and shoulder spica
cast. The shoulder spica cast is applied with the
elbow straight and the shoulder in 90 of abduc-
tion. This position is maintained for 6 weeks
followed by a transition to a thoracolumbosacral
orthosis and attached hinged brace that was fabri-
cated prior to surgery. Therapy involves tendon
transfer training and a gradual allowance of elbow
exion and lessening of shoulder abduction. The
process is arduous and must be governed by an
experienced therapist. Expedited elbow exion or
30 Secondary Procedures About the Elbow, Forearm, Wrist, and Hand
Fig. 16 Posterior half of deltoid isolated (Courtesy of
Shriners Hospital for Children, Philadelphia)
Fig. 17 Large subcutaneous tunnel between incisions
(Courtesy of Shriners Hospital for Children, Philadelphia)
665
Fig. 18 Allograft secured to deltoid muscle (Courtesy of
Shriners Hospital for Children, Philadelphia)
shoulder adduction will result in attenuation of the
transfer (Fridn et al. 2000).
Posterior deltoid transfer
Surgical steps
Curved incision is made over the deltoid muscle
The axillary nerve is identied entering the deltoid
muscle
Posterior half of the deltoid isolated with a periosteal
sleeve
Distal incision about the distal third of the triceps tendon
Subcutaneous tunnel is then created between the two
incisions
Gap between posterior deltoid and olecranon bridged
with allograft or autograft
Elbow placed in full extension and the autograft/allograft
woven into the triceps tendon and/or bone
Posterior deltoid transfer
Postoperative protocol
Long-arm cast with elbow in extension attached to a
shoulder spica cast with shoulder abducted to 90
Transitioned to the thoracolumbosacral orthosis at
6 weeks and tendon transfer training initiated
Tendon transfer ring and reeducation instituted
(continued)
666 S.H. Kozin et al.

Fig. 20 Allograft secured to triceps with elbow in full

extension (Courtesy of Shriners Hospital for Children,
Philadelphia)

Fig. 19 Allograft passed to distal incision and triceps Posterior deltoid transfer
tendon (Courtesy of Shriners Hospital for Children,
Potential pitfalls and preventions
Philadelphia)
Potential pitfall Pearls for prevention
Pitfall #4 Attenuation of Avoid rapid allowance of
tendon transfer shoulder adduction or
elbow exion
Posterior deltoid transfer Pitfall #5 Difculty with Enlist an experienced
ring transfer therapist; try alternative
Postoperative protocol
methods such as
Process is arduous and must be governed by an biofeedback
experienced therapist
Protective extension block splinting of the elbow is
continued until 3 months after surgery

Forearm
Posterior deltoid transfer
Potential pitfalls and preventions The forearm is the forgotten joint with regard to
Potential pitfall Pearls for prevention movement and balance. The forearm is articulated
Pitfall #1 Injury to Identify axillary nerve at its proximal and distal ends (proximal and distal
axillary nerve entering posterior deltoid radioulnar joints). The movement is balanced by
Pitfall #2 Inadequate girth Choose posterior half two muscles that supinate (biceps and supinator)
of deltoid instead of posterior third
and two muscles that pronate (pronator teres and
Pitfall #3 Suboptimum Augment with autograft or
attachment to deltoid allograft pronator quadratus) (Kozin 2002a). The biceps
muscle and supinator muscles are primarily innervated
(continued) from C5 and C6. In contrast, the pronator teres is
30 Secondary Procedures About the Elbow, Forearm, Wrist, and Hand
predominately a C7 muscle and the pronator
quadratus is a C8 muscle. Hence, the injury
and/or subsequent fragmentary recovery can lead
to muscle imbalance. For example, global injuries
with recovery of only upper trunk lead to severe
supination deformities.
The deformity is initially supple, but over time
becomes xed. When deciding whether treatment
is warranted, the surgeon must weigh the pros and
cons of treatment. A therapists input is often
benecial in the decision-making process. Activ-
ities that require supination include turning a door
knob, carrying a tray, feeding ones self, dispens-
ing soap, catching a ball, and perineal care. Activ-
ities that require pronation include typing on a
keyboard, performing tabletop functions, and
holding onto bicycle handle bars. Most surgical
procedures to enhance rotation in one direction
will sacrice some opposite rotation. Therefore,
ample thought is necessary prior to proceeding
with surgical intervention.
Prevention is worth a pound of cure and
stretching/splinting of the unbalanced forearm is
mandatory. The goal is to prevent a xed forearm
contracture. For children with C5C6 injuries,
passive elbow exion and forearm supination are
part of the stretching paradigm. Once recovery
has plateaued, surgical options to rebalance
the forearm are considered. A supple imbalance
is amenable to tendon transfer, while a
xed deformity requires interosseous membrane
release to regain passive motion or osteotomy to
realign the forearm into a more functional
position.
A subtle loss of supination or pronation does
not warrant surgical treatment. The small lack of
rotation is often more bothersome to the parent
than the child. In addition, full forearm rotation is
unnecessary for routine activities of daily living.
Considerable loss of supination or pronation is
worthy of surgical consideration; however, the
inevitable loss of the opposite movement
needs to be taken into consideration. For the
child with a supple loss of forearm rotation,
there are a variety of donors for tendon transfer.
The pronator teres and the brachioradialis are
viable donors that yield a supination moment.
Supination transfers are rarely performed in the
667
brachial plexus population as pronation loss
would negatively affect many activities of daily
living. The pronator teres rerouting has the longest
track record for improving supination (Amrani
et al. 2009).
Technique: Pronator Teres Tendon
Rerouting
The patient is placed supine on the operating room
table. The entire extremity is prepped and draped.
A sterile circular tourniquet is applied
(HemaClear, OHK Medical Devices, Grandville,
Michigan, USA) that exsanguinates during appli-
cation. A longitudinal incision is made over the
radial aspect of the forearm. The supercial
branches of the lateral antebrachial cutaneous
nerves are mobilized. The radial sensory nerve is
identied emanating from the brachioradialis
muscle-tendon. The nerve is traced in a proximal
direction and the brachioradialis muscle is ele-
vated. Just proximal to this point, the pronator
teres tendinous attachment into the radius is
identied.
The pronator muscle is released and dissected
in a proximal direction to facilitate rerouting. A
spacious window is made in the interosseous
membrane to accommodate the transfer. The pro-
nator teres tendon is lengthened in a Z fashion
(Amrani et al. 2009). The distal part of the tendon
is rerouted from dorsal to volar through the win-
dow in the interosseous membrane. The distal part
is then sutured back to the proximal part under
slight tension. Alternatively, the pronator teres
tendon is detached and is transferred through the
interosseous membrane, around the radius, and
reattached to its former insertion site with trans-
osseous sutures or suture anchors with the forearm
in supination.
Inadequate pronation is an indication to con-
sider surgical intervention. A supple loss of pro-
nation is amenable to tendon transfer. The
principal donor muscle-tendon is the biceps ten-
don rerouted around the radius (Kozin 2006;
Kozin and Zlotolow 2012). This converts the
biceps from a forearm supinator into a forearm
pronator.
668
Fig. 21 A six-year-old female with residual brachial
plexus palsy and supple supination posturing of her left
arm. Skin incision for biceps rerouting (Courtesy of
Shriners Hospital for Children, Philadelphia)
Technique: Biceps Tendon Rerouting
Biceps tendon rerouting
Preoperative planning
OR table: regular
Position/positioning aids: supine
Fluoroscopy location: none
Equipment: standard
Tourniquet: sterile
The patient is placed supine on the operating
room table. The entire extremity is prepped and
draped. A sterile circular tourniquet is applied
(HemaClear, OHK Medical Devices, Grandville,
Michigan, USA) that exsanguinates during applica-
tion. An S-shaped incision is made along the medial
arm, across the antecubital fossa, and over the
brachioradialis muscle belly (Fig. 21). The
antebrachial cutaneous nerve is identied lateral to
the biceps tendon and protected (Fig. 22). The
biceps tendon is traced to its insertion into the radial
tuberosity (Fig. 23). There are large crossing veins
that require ligation. The lacertus brosus is incised
from the biceps tendon. The entire length of the
biceps tendon is isolated. A long Z-plasty is
performed with one limb left attached to the radius
and the other attached to the muscle (Fig. 24). The
limb attached to the radius is rerouted around the
radius through the interosseous space to yield a
pronation force. A curved clamp, such as a
S.H. Kozin et al.
Fig. 22 Isolation of the biceps tendon and lacertus
brosus. The antebrachial cutaneous nerve just lateral to
the tendon (Courtesy of Shriners Hospital for Children,
Philadelphia)
Castaneda pediatric clamp (Pilling Surgical, North
Carolina, USA), facilitates tendon passage (Fig. 25)
(Kozin 2006). The posterior interosseous nerve
must be protected to prevent iatrogenic injury.
The elbow is positioned in 90 of exion with
the forearm in pronation. The rerouted distal ten-
don is woven back through the proximal tendon
that remained attached to the biceps muscle
(Fig. 26). The subcutaneous tissue and skin are
closed in routine fashion. A long-arm cast is
applied with the elbow in 90 of exion and the
forearm in pronation for 5 weeks.
Biceps tendon rerouting
Surgical steps
Long S-shaped incision, across the antecubital fossa
Isolate the entire length of the biceps tendon
Perform long Z-plasty with one limb left attached to the
radius and the other attached to the muscle
Pass limb attached to the radius around the radius through
the interosseous space to yield a pronation force
Secure the rerouted distal tendon back to the proximal
tendon attached to the biceps muscle with forearm in
pronation
Biceps tendon rerouting
Postoperative protocol
Long-arm cast is applied with the elbow in 90 of exion
and the forearm in pronation for 5 weeks
Tendon transfer ring and reeducation instituted
Protective elbow and forearm splinting until 3 months
after surgery
30 Secondary Procedures About the Elbow, Forearm, Wrist, and Hand 669

Fig. 23 Biceps tendon

traced to its insertion into
radial tuberosity (Courtesy
of Shriners Hospital for
Children, Philadelphia)

Fig. 24 Z-plasty of the entire biceps tendon with long Fig. 25 A curved clamp (Castaneda pediatric clamp,
proximal and distal limbs (Courtesy of Shriners Hospital Pilling Surgical, North Carolina, USA) facilitates tendon
for Children, Philadelphia) rerouting around the radius (Courtesy of Shriners Hospital
for Children, Philadelphia)

Biceps tendon rerouting

Potential pitfalls and preventions
Potential pitfall Pearls for prevention Biceps tendon rerouting
Pitfall #1 Inadvertent injury Identify the nerve before Potential pitfalls and preventions
to lateral antebrachial isolating the biceps Potential pitfall Pearls for prevention
cutaneous nerve tendon Pitfall #5 Difculty with Try alternative methods
Pitfall #2 Inadequate biceps Dissect entire biceps ring transfer such as biofeedback
tendon length length before Z-plasty
Pitfall #3 Difculty passing Curved clamp facilitates
the distal tendon around the tendon passage Rehabilitation
radius
Immediate nger motion is encouraged to
Pitfall #4 Injury to the Be wary of the nerve
posterior interosseous during tendon passage decrease swelling. The cast is removed 5 weeks
nerve after surgery, and a long-arm splint is fabricated
(continued) that duplicates the casted position. Active elbow
670
Fig. 26 Distal limb repaired back to proximal limb using a
tendon weave augmented with nonabsorbable suture
(Courtesy of Shriners Hospital for Children, Philadelphia)
exion is started, which promotes biceps activity
across the elbow and forearm. Tendon transfer
training is instituted. Instructing the child to per-
form supination helps stimulate biceps activity
and yields forearm pronation. Relearning occurs
over time. A protective splint is worn for 8 weeks
after surgery. Passive forearm supination or elbow
extension motion is avoided until 3 months after
surgery.
A xed forearm deformity is more difcult to
manage. Therapy and static progressive devices
can be utilized with a goal of regaining passive
motion. There are a few different surgical options
to address the xed forearm to therapy (Hankins
et al. 2006). Release of the interosseous mem-
brane can gain some forearm rotation. Osteotomy
of the radius and/or ulna can reposition the fore-
arm into a more functional position. In general,
osteotomy of a single bone can rotate the forearm
about 45 , while osteotomy of both bones can
correct 100 . In severe deformities, the only reli-
able surgical technique is making a one-bone
forearm (Kozin 2006). This procedure consis-
tently realigns the forearm and negates the possi-
bility of recurrence secondary to persistent
imbalance. Over time, the one-bone forearm has
become our preferred procedure for severe defor-
mities in the brachial plexus population. The
transposition of the radius onto the ulna and the
use of the remaining proximal radius as an onlay
graft are preferred.
S.H. Kozin et al.
Fig. 27 An eight-year-old male with residual brachial
plexus palsy and xed 90 supination contracture of his
right arm (Courtesy of Shriners Hospital for Children,
Philadelphia)
Technique: Osteotomy of Radius
and/or Ulna
Osteotomy of radius and/or ulna
Preoperative planning
OR table: regular
Position/positioning aids: supine
Fluoroscopy: yes
Equipment: standard with varying plate sizes
Tourniquet: sterile
The patient is placed supine on the operating
room table. The entire extremity is prepped and
draped (Fig. 27). A sterile circular tourniquet is
applied (HemaClear, OHK Medical Devices,
Grandville, Michigan, USA) that exsanguinates
during application. A separate incision is used
for each osteotomy (Fig. 28). Fixation depends
upon the size of the bone. Low-contact dynamic
30 Secondary Procedures About the Elbow, Forearm, Wrist, and Hand
Fig. 28 Six- or eight-hole 2.4 mm titanium low-contact
dynamic compression plates (Synthes USA, Paoli, PA) are
used with incisions long enough to accommodate plates
(Courtesy of Shriners Hospital for Children, Philadelphia)
compression plates are preferred (Synthes USA,
Paoli, PA). The length of the incision used is equal
to the length of the desired plate. The proximal
ulna is approached between the exor carpi
ulnaris and extensor carpi ulnaris muscles. The
periosteum is preserved, except at the osteotomy
site. The plate is contoured to t the dorsal aspect
of the ulna. Three screws are placed in the prox-
imal portion of the plate. The proposed osteotomy
site is marked between the third and fourth screw
hole. The current alignment of the ulna is marked
with electrocautery or Kirschner wire to gauge the
amount of rotation achieved after osteotomy. The
plate is removed and the periosteum is incised at
the proposed osteotomy site. Retractors are placed
around the ulna and the bone is cut with a thin
sagittal saw under irrigation to limit thermal
necrosis (Fig. 29). The ulna is rotated with a
bone reduction clamp to improve pronation.
671
Fig. 29 Four screws are placed in the proximal portion of
the plate and ulna. The proposed osteotomy site is marked
between the third and fourth screw holes, and a Kirschner
wire is used to estimate rotational correction (Courtesy of
Shriners Hospital for Children, Philadelphia)
Once adequate rotation has been achieved, the
distal portion of the plate is secured using a
dynamic compression technique (Fig. 30).
The distal third of the radius is approached via
a dorsal or radial approach between the extensor
carpi radialis brevis and the extensor pollicis
longus tendons or between the brachioradialis
and extensor carpi radialis longus tendons. The
periosteum is preserved and an appropriately
sized plate is contoured to the radius. Three
screws are placed in the proximal portion of the
plate. The proposed osteotomy site is marked
between the third and fourth screw hole. The
periosteum is elevated around the radius at the
proposed osteotomy site. Retractors are placed
around the ulna and the bone is cut with a sagittal
saw under irrigation to limit thermal necrosis. The
radius is rotated with a bone reduction clamp to
672
Fig. 30 Fixation of the ulna after adequate correction
using dynamic compression technique (Courtesy of
Shriners Hospital for Children, Philadelphia)
improve pronation. Once adequate rotation has
been achieved, the distal portion of the plate is
secured using a dynamic compression technique
(Fig. 31).
If both bones have been cut, the rotation is
increased by going back and forth between
osteotomy sites using a bone reduction clamp for
provisional xation (Hankins et al. 2006). Once
adequate pronation has been achieved, the plates
are secured with bicortical screw xation
(Fig. 32).
Closure involves re-approximation of the sub-
cutaneous tissue and skin. The fascia is not closed.
A plaster splint is applied for 2 weeks rather than a
cast, and the child is monitored overnight for
neurovascular problems. The splint is changed to
a long-arm berglass cast 2 weeks after surgery.
Six weeks after surgery, the cast is removed and a
splint is fabricated. The protective splint is used
until the x-rays reveal crossing trabeculae at the
osteotomy site(s) (Fig. 33).
S.H. Kozin et al.
Fig. 31 Fixation of the radius with plate and screw con-
struct (Courtesy of Shriners Hospital for Children,
Philadelphia)
Osteotomy of radius and/or ulna
Surgical steps
Separate incision is used for each osteotomy
Proximal ulna is approached between the exor carpi
ulnaris and extensor carpi ulnaris muscles
Distal third of the radius is approached via a dorsal or
radial approach
Plate is contoured to t the bone and bicortical xation is
obtained proximal to the proposed osteotomy with three
screws
Plate is removed and the bone is cut with a sagittal saw
Plate is reapplied to the proximal bone and the distal bone
is rotated to improve pronation
Provisional xation obtained with a bone reduction
clamp between the plate and bone
Once adequate rotation has been achieved, the distal
portion of the plate is secured using a dynamic
compression and bicortical screw xation
Osteotomy of radius and/or ulna
Postoperative protocol
Long-arm plaster splint is applied for 2 weeks and a child
is monitored overnight for neurovascular problems
(continued)
30 Secondary Procedures About the Elbow, Forearm, Wrist, and Hand 673

Osteotomy of radius and/or ulna Osteotomy of radius and/or ulna

Postoperative protocol Potential pitfalls and preventions
Long-arm berglass cast 2 weeks after surgery Potential pitfall
Cast is removed 6 weeks after surgery and a splint is Pitfall #1 Inadequate
fabricated rotation after osteotomy of
Protective splinting until x-rays reveal crossing a single bone
trabeculae at the osteotomy site(s) Pitfall #2 Compartment
syndrome
Pitfall #3 Delayed union
Pitfall #4 Hardware
breakage
Pearls for prevention
Release interosseous
membrane or cut other
bones
Avoid closing the fascia
and immediate casting
Avoid bone necrosis, and
preserve periosteum;
ample xation
Prolong protection until
union is evident

Technique: One-Bone Forearm

One-bone forearm
Preoperative planning
OR table: regular
Position/positioning aids: supine
Fluoroscopy: yes
Equipment: standard with varying plate sizes
Tourniquet: sterile

The patient is placed supine on the operating

Fig. 32 Adequate forearm pronation after osteotomies of
the radius and ulna (Courtesy of Shriners Hospital for
Children, Philadelphia)
room table (Fig. 34). The entire extremity is
prepped and draped. A sterile circular tourniquet
is applied (HemaClear, OHK Medical Devices,
Grandville, Michigan, USA) that exsanguinates
during application. A curvilinear incision is
made along the distal radius and proximal ulna.
The volar forearm fascia is incised along the
length of the skin incision. The entire contents of
the volar forearm are identied (Fig. 35). The
radius is exposed via a trans-exor carpi radialis

Fig. 33 Postoperative
x-rays demonstrating union
across the ulna and radius
(Courtesy of Shriners
Hospital for Children,
Philadelphia)
674 S.H. Kozin et al.

incision. The median nerve is identied and
protected. The ulna is isolated deep to the exor
carpi ulnaris and ulnar neurovascular bundle. In
patients with substantial denervation, the forearm
Fig. 34 An eight-year-old male with residual right global
brachial plexus birth palsy and xed forearm supination
deformity (Courtesy of Shriners Hospital for Children,
Philadelphia)
muscles have mainly been replaced by fat with
only a few strands of viable muscle present.
The osteotomies are planned with the radius
osteotomy one to two centimeters distal to the
ulnar osteotomy. Proximal to the proposed ulnar
osteotomy site, an appropriated sized dynamic
compression plate is secured with three or four
bicortical screws. The anterior interosseous nerve
and vessels are dissected and elevated from the
volar interosseous membrane. The intervening
membrane is incised to allow the radius to be
positioned on top of the proximal ulna. The plate
is then removed and the ulnar and radial
osteotomies are performed with a ne-bladed sag-
ittal saw. The distal ulna can be excised or simply
mobilized out of harms way.
The radius is manually mobilized toward the
proximal ulna (Fig. 36). The plate is reapplied to
the ulna. Once the bones are coapted, the radius is
rotated into the desired position. The reduction is
held with a bone reduction clamp and the position
is carefully assessed. Once the position is deemed
acceptable, the plate is secured to the distal radius
using dynamic compression and bicortical screw
xation (Fig. 37). The remaining proximal radius
segment is used as onlay graft across the
osteotomy site. The radius is decorticated and
afxed to the construct using an intraosseous
suture or compression screw. The subcutaneous
tissue and skin are closed and a long-arm splint is
placed. The splint is changed to a long-arm cast

Fig. 35 Curvilinear
incision and identication
of the entire contents of the
volar forearm (Courtesy of
Shriners Hospital for
Children, Philadelphia)
30 Secondary Procedures About the Elbow, Forearm, Wrist, and Hand
2 weeks after surgery. The long-arm cast is worn
for an additional 4 weeks. Radiographs are taken
at a 2-week follow-up to ensure alignment and
repeated at 6 weeks to ensure the healing of the
osteotomy site. A protective splint is then fabri-
cated for another 6 weeks. Digital range of motion
exercises are started immediately after surgery.
Elbow motion is begun at 6 weeks following the
removal of the long-arm cast. The amount of
correction achieved is often remarkable (Fig. 38).
Fig. 36 The radius is cut distal to the ulna and manually
mobilized toward the proximal ulna (Courtesy of Shriners
Hospital for Children, Philadelphia)
Fig. 37 The forearm is
rotated into the desired
position and the ulna
secured to the distal radius
using dynamic compression
(Courtesy of Shriners
Hospital for Children,
Philadelphia)
675
One-bone forearm
Surgical steps
Curvilinear incision is made along the distal radius and
proximal ulna
Isolate radius is exposed via a trans-exor carpi radialis
incision
Mobilize the entire volar forearm compartment
The ulna is isolated deep to the exor carpi ulnaris and
ulnar neurovascular bundle
Plan osteotomies with the radius osteotomy one to two
centimeters distal to the ulnar osteotomy
Excise intervening interosseous membrane
Proximal to the proposed ulnar osteotomy site, secure an
appropriately sized dynamic compression with three or
four bicortical screws
Cut the ulnar and radial osteotomies with a ne-bladed
sagittal saw
Mobilize the radius toward the proximal ulna
Rotate the radius into the desired position and afx to the
end of the ulna using dynamic compression and bicortical
screw xation
Secure the proximal radius segment as onlay graft
bridging the osteotomy site
One-bone forearm
Postoperative protocol
Long-arm plaster splint is applied for 6 weeks and a child
is monitored overnight for neurovascular problems
Long-arm berglass cast 2 weeks after surgery
Cast is removed 6 weeks after surgery and a splint is
fabricated
Protective splinting until x-rays reveal crossing
trabeculae at the osteotomy site(s)
676 S.H. Kozin et al.

for maximum grip. In children without grip, active

wrist extension provides tenodesis for grip and for
lateral pinch. The second most important task is
lateral pinch, which affords the ability to perform
numerous activities of daily living. Most activities
that we do every day can be accomplished with
lateral pinch. The third essential motion is grasp,
which allows us to hold objects. The fourth and
last movement is digital opening for object acqui-
sition. The reason to place this function last on the
ladder is that wrist exion yields passive digital
opening, which is often adequate for object pro-
curement. In addition, synchronous digital open-
ing is difcult to achieve via surgery as
interphalangeal joint extension is primarily an
intrinsic function and metacarpophalangeal joint
extension is mainly an extrinsic function. Restor-
ing both movements by tendon transfer(s) is a
difcult task.
The selection of available and appropriate
donors for tendon transfer is crucial in children
with residual brachial plexus palsy. As stated pre-
Fig. 38 Final position of right forearm after osteotomy viously, try to avoid reinnervated muscles.
site (Courtesy of Shriners Hospital for Children, Equally as important, we make every attempt to
Philadelphia) select a synchronous muscle as postoperative
compliance in therapy is limited by age and cog-
One-bone forearm
nition. Therefore, nger exors for wrist exten-
Potential pitfalls and preventions sion are preferred as well as a wrist extensor for
Potential pitfall Pearls for prevention nger exion. The main donors are the
Pitfall #1 Avoid closing the fascia and exor digitorum supercialis and extensor carpi
Compartment immediate casting radialis longus, respectively. When these
syndrome tendons are unavailable, a search for alternative
Pitfall #2 Delayed Avoid bone necrosis, and donors based upon the established principles of
union preserve the periosteum; ample
xation; onlay graft
availability, expendability, synchrony, and
Pitfall #3 Hardware Prolong protection until union is excursion.
breakage evident

Technique: Flexor Digitorum

Superficialis Tendon Transfer
Wrist and Hand

The management of the wrist and hand in brachial Flexor digitorum supercialis tendon transfer
plexus palsy follows a similar algorithm to Preoperative planning
tetraplegia (Kozin 2007b). The terms hierarchy OR table: regular
of hand function and the reconstruction ladder Position/positioning aids: supine
are used interchangeably. The primary important Fluoroscopy location: none
movement is wrist extension, which aligns the Equipment: standard
Tourniquet: sterile
nger exors along Blixs length-tension curve
30 Secondary Procedures About the Elbow, Forearm, Wrist, and Hand 677

The patient is placed supine on the operating

room table. The entire extremity is prepped and
draped. A sterile circular tourniquet is applied
(HemaClear, OHK Medical Devices, Grandville,
Michigan, USA) that exsanguinates during appli-
cation. Initial attention is directed at exor
digitorum supercialis (FDS) harvest. A trans-
verse or longitudinal incision is made at the base
of the long and/or ring ngers. The FDS tendons
are isolated from the exor digitorum profundus
tendon. The tendon is tagged with a long
nonabsorbable suture to allow retrievability if
the tendon gets entangled within the carpal tunnel
during passage. An oblique incision is then made
over the distal third of the forearm, and the long
and ring nger FDS tendons are isolated. The
tendons are then cut distal to the suture at the
base of the ngers. An Alice clamp (Pilling Sur-
gical, North Carolina, USA) is applied to the
tendons in the forearm and rolled in a proximal
direction. This maneuver passes the tendons
through the carpal tunnel into the forearm incision
(Fig. 39). Fig. 39 Harvest of the exor digitorum supercialis ten-
The route of passage is dependent upon the don (Courtesy of Shriners Hospital for Children,
patient. The tendons can be passed around the Philadelphia)
radial side of the forearm to enhance pronation,
around the ulnar side to improve supination, or
through the interosseous membrane to
address only wrist extension. The passage was
through the interosseous membrane that
requires a spacious window in the membrane
proximal to the pronator quadratus and adjacent
to the anterior interosseous neurovascular bundle
(Fig. 40).
The tendons are retrieved via a dorsal incision
at the base of the long nger metacarpal (Fig. 41).
On occasion, an additional proximal incision is
necessary to shuttle the tendons. Passing the ten-
dons around the long nger metacarpal is pre-
ferred. One tendon is passed in a radial to ulnar
direction and the other in an opposite direction.
The wrist is then placed in the desired amount of
wrist extension based upon the preoperative
examination of digital opening and closing. The
tendons are woven back to themselves and to each
other to obtain a robust repair. The wrist should
Fig. 40 Passage of the exor digitorum supercialis ten-
rest in the desired position once the tendon repair don through the interosseous membrane (Courtesy of
is performed. The subcutaneous tissue and skin Shriners Hospital for Children, Philadelphia)
678 S.H. Kozin et al.

Fig. 41 Flexor digitorum

supercialis tendon
retrieved on the dorsum of
the wrist prior to passing the
tendon around the long
nger metacarpal (Courtesy
of Shriners Hospital for
Children, Philadelphia)

are closed. A long-arm cast is applied with the Flexor digitorum supercialis tendon transfer
wrist in extension and the elbow exed to 90 . Potential pitfalls and preventions
The cast is removed 3 weeks following surgery Potential pitfall Pearls for prevention
and a short-arm splint is fabricated. Tendon trans- Pitfall #1 The exor Make sure the fascia in the
fer training is initiated, although the synergism of digitorum supercialis palm and forearm has
the transfer negates the necessity of substantial tendon will not pass been released tendon
through carpal tunnel
relearning. The splint is discontinued 8 weeks
Pitfall #2 Difculty Use a curved clap with
from surgery. passing the tendon around small radius of curvature
the long nger metacarpal
Flexor digitorum supercialis tendon transfer Pitfall #3 Difculty Make large window in
Surgical steps passing through the membrane prior to
Harvest the exor digitorum supercialis tendons interosseous membrane passage
via transverse incision at the base of the long and ring Pitfall #4 Insufcient Redo tendon coaptation
ngers resting tenodesis tighter
Retrieve the tendons into volar distal third forearm via an Pitfall #5 Limited Advance rehabilitation to
oblique excursion of tendon encourage gliding
Route the tendons to the dorsum of the wrist either transfer
through the interosseous membrane or around the
forearm (radial or ulnar)
Pass the tendons around the long nger metacarpal. One
Technique: Extensor Carpi Radialis
tendon is passed in a radial to ulnar direction and the
other in an opposite direction Tendon Transfer
Secure the tendons back to themselves and to each other
while maintaining the wrist in extension Extensor carpi radialis tendon transfer
Preoperative planning
OR table: regular
Flexor digitorum supercialis tendon transfer Position/positioning aids: supine
Postoperative protocol Fluoroscopy location: none
Long-arm cast is applied with the elbow in 90 of exion, Equipment: standard
forearm in neutral, and wrist in extension for 3 weeks Tourniquet: sterile
Tendon transfer ring and reeducation instituted
Protective elbow and forearm splinting until 8 weeks The patient is placed supine on the operating
after surgery room table. The entire extremity is prepped and
30 Secondary Procedures About the Elbow, Forearm, Wrist, and Hand
Fig. 42 Harvest of the
extensor carpi radialis
longus (and brachioradialis)
via longitudinal radial
incision (Courtesy of
Shriners Hospital for
Children, Philadelphia)
draped. A sterile circular tourniquet is applied
(HemaClear, OHK Medical Devices, Grandville,
Michigan, USA) that exsanguinates during appli-
cation. A longitudinal radial incision is made from
the styloid to the proximal third of the forearm
(Fig. 42). The lateral antebrachial cutaneous and
radial sensory nerves are protected. Distal to the
rst compartment, the extensor carpi radialis
longus (ECRL) and extensor carpi radialis brevis
(ECRB) tendons are identied. In the proximal
part of the incisions, the ECRL and ECRB ten-
dons are also isolated. The ECRL tendon is then
cut as distal as possible. An Alice clamp (Pilling
Surgical, North Carolina, USA) is applied to the
ECRB tendon in the proximal forearm and rolled
in a proximal direction. This maneuver passes the
tendons under the rst dorsal compartment.
The volar portion of the skin ap is mobilized.
The fascia is incised along the exor carpi radialis
tendon. The underlying median nerve and exor
digitorum supercialis tendons are mobilized.
The deep exor digitorum profundus tendons are
isolated. The tendons are sutured together
(en masse) to set the desired nger cascade during
grasp. The radial artery and its venae comitantes
are elevated from the distal radius.
The ECRL tendon is passed deep to the radial
artery and woven through the exor digitorum
profundus tendons using a Pulvertaft weave
(Fig. 43). Tension is adjusted until there is nger
exion during wrist extension and tenodesis
679
opening during wrist exion. The subcutaneous
tissue and skin are closed. A short- or long-arm
cast is applied depending upon the age of the
patient and the strength of the repair. The wrist is
in slight extension and the elbow exed to 90 .
The ngers are slightly exed and this position is
maintained with a small roll of Webril cotton cast
padding. Early mobilization can be started on
postoperative day one as long as the tendons are
of adequate caliper and the transfer site is rmly
repaired. The Webril is removed and the patient is
instructed to bend his or her ngers. The response
is often overwhelming as nger exion is readily
initiated. The cast is removed 3 weeks following
surgery and a short-arm splint is fabricated. Ten-
don transfer training is initiated, although the syn-
ergism of the transfer negates the need of
extensive relearning. The splint is discontinued
8 weeks from surgery.
Extensor carpi radialis tendon transfer
Surgical steps
Longitudinal radial incision from the styloid to the
proximal third of the forearm
Isolate the extensor carpi radialis longus distal to the rst
compartment and in the proximal forearm
Cut the extensor carpi radialis longus and pull beneath the
rst compartment into the proximal forearm
Isolate and synchronize the exor digitorum profundus
tendons
(continued)
680 S.H. Kozin et al.

Fig. 43 Extensor carpi

radialis longus tendon
woven through the exor
digitorum profundus
tendons (Courtesy of
Shriners Hospital for
Children, Philadelphia)

Extensor carpi radialis tendon transfer Extensor carpi radialis tendon transfer
Surgical steps Potential pitfalls and preventions
Secure the rerouted distal tendon back to the proximal Potential pitfall Pearls for prevention
tendon attached to the biceps muscle with forearm in Pitfall #2 Poor Synchronize the exor
pronation synchronization of long digitorum profundus
Pass the extensor carpi radialis longus deep to radial nger exors tendons before tendon
artery and weave into the exor digitorum profundus transfer
tendons Pitfall #3 Limited Advance rehabilitation to
excursion of tendon encourage gliding
transfer
Extensor carpi radialis tendon transfer
Postoperative protocol
Long-arm cast is applied with the elbow in 90 of exion Rehabilitation and Outcome
and the wrist in slight extension. Fingers are slightly
exed and this position is maintained with a small roll of
Webril cotton cast padding The rehabilitation is critical to maximize the out-
Early mobilization can be started on postoperative day come. Communication between surgeon and ther-
one as long as the tendons are of adequate caliper and the apist is paramount for success. Communication
transfer site is rmly repaired
with the therapist about the surgery and antici-
Cast is removed 3 weeks following surgery and a short-
arm splint is fabricated
pated rehabilitation is mandatory. The main fac-
Protective wrist splinting until 8 weeks after surgery tors that govern the speed of the rehabilitation are
the age of the child, the size of the tendons, and the
strength of the repair. The size of the tendons
Extensor carpi radialis tendon transfer correlates with the strength of the repair. A simple
Potential pitfalls and preventions yet useful classication of tendon size has been
Potential pitfall Pearls for prevention devised (Table 1 (Kozin 2008)). Robust lin-
Pitfall #1 Inadvertent Careful dissection, identify guine tendons can be managed with early mobi-
injury to the lateral radial sensory nerve
antebrachial cutaneous between the brachioradialis
lization techniques to facilitate tendon gliding. In
nerve or radial sensory and extensor carpi radialis contrast, small vermicelli tendons require longer
nerve longus tendon mobilization, prolonged splinting, increased pro-
(continued) tection, and a slower rehabilitation. A skilled
30 Secondary Procedures About the Elbow, Forearm, Wrist, and Hand
Table 1 Grading scale of tendon size
Grade Implication
Linguine Early mobilization
Minimal concern for failure of repair
Active motion to prevent motion-
limiting scar
Spaghetti Intermediate rehabilitation
Vermicelli or Delayed rehabilitation
angel hair Substantial concern for repair
attenuation or failure
Protracted splint protection
Adapted from Kozin (2008)
therapist experienced in tendon transfer rehabili-
tation in children will maximize the outcome.
Early detection of impeding problems allows pos-
sible correction, while delayed recognition is dif-
cult to salvage.
The clinical outcome and effect on function are
discussed in detail within Outcome Measures.
The recognition that objective measurements and
legacy measures may not correlate with enhanced
function has resulted in a paradigm shift in out-
come assessments. The initiative toward patient-
or caregiver-reported outcomes has resulted in the
development of reliable subjective measures. For
children with brachial plexus palsy, Shriners Hos-
pitals for Children has invested millions of dollars
into patient-reported outcomes using the com-
puter adaptive test (CAT) approach (Mulcahey
et al. 2012, 2013). This methodology has been
shown to correlate with legacy measures, discrim-
inate among brachial plexus impairment levels,
possess minimal oor and ceiling effects, and be
less burdensome compared to previous measures.
CAT will change the manner in which we assess
outcome in the near future.
Summary
The management of the upper limb with residual
brachial plexus palsy is a challenging problem.
The patient as a whole needs to be considered
when formulating a treatment plan. Patients have
varying needs in life and individual requirements
for function. The shoulder, elbow, forearm, wrist,
681
and hand function as a coordinated unit. Each
individual joint is assessed for movement, con-
tracture, and function. A diligent and accurate
examination is mandatory to formulate an appro-
priate treatment plan. The pros and cons of treat-
ment at each joint are then assessed with regard to
potential gains and possible losses. The goal is to
obtain a balanced joint that affords the maximum
function and independence. The surgeon must be
keenly aware that enhanced movement in one
direction often leads to the loss of movement in
the other direction. The input of an astute therapist
is often invaluable in the decision-making pro-
cess. The patient and family must have realistic
expectations from any proposed intervention.
Once a treatment strategy has been established,
the surgery and therapy must be methodically
performed with attention to detail to maximize
patient outcome. Careful planning, implementa-
tion, and execution will result in a positive out-
come and a satised patient. Any blip or
breakdown along the way will result in a dissatis-
ed patient, family, therapist, and surgeon.
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 Traumatic Brachial Plexus Injury
in the Pediatric Population 31
Harvey Chim, Allen T. Bishop, Robert J. Spinner, and
Alexander Y. Shin

Contents Surgical Procedure: Intercostal Nerve Transfer

to Musculocutaneous Nerve . . . . . . . . . . . . . . . . . . . . . . . . . 699
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 684 Free Functioning Muscle Transfer (FFMT) . . . . . . . . . 701
Pathoanatomy and Applied Anatomy . . . . . . . . . . . . 685 Surgical Procedure: Double Free Functional
Gracilis Muscle Transfer . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 701
Assessment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 687
Signs and Symptoms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 687 Preferred Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 703
Imaging and Electrodiagnostic Studies . . . . . . . . . . . . . . 690 General Philosophy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 703
Injuries Associated with Traumatic Brachial Upper Trunk (C5-6 Injury) in Pediatric Patients
Plexus Injury . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 692 >4 Years . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 705
Classication . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 692 Pan-Plexus Injury in Pediatric Patients >4 Years . . . 706
Outcome Tools . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 693 Surgical Pitfalls and Prevention . . . . . . . . . . . . . . . . . . . 707
Differences Between Adult and Pediatric
Injuries . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 693 Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 707
Treatment Options . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 693 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 707
Nonoperative Management . . . . . . . . . . . . . . . . . . . . . . . . . . 694
Operative Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 695
Surgical Procedure: Spinal Accessory Nerve
(SAN) to Suprascapular Nerve (SSN) Transfer . . . . . 697
Surgical Procedure: Transfer of Triceps Motor
Branch to Axillary Nerve . . . . . . . . . . . . . . . . . . . . . . . . . . . . 698
Surgical Procedure: Ulnar Nerve Fascicular
Transfer to Biceps Motor Branch . . . . . . . . . . . . . . . . . . . . 698
Surgical Procedure: Median Nerve Fascicular
Transfer to Brachialis Motor Branch . . . . . . . . . . . . . . . . 699

Statement of Financial Disclosure: The authors declare

that they had no nancial interests or commercial
associations relevant to this study.
H. Chim A.T. Bishop R.J. Spinner A.Y. Shin (*)
Department of Orthopedic Surgery, Division of Hand
Surgery, Mayo Clinic, Rochester, MN, USA
e-mail: chim.harvey@mayo.edu; bishop.allen@mayo.edu;
spinner.robert@mayo.edu; shin.alexander@mayo.edu

# Springer Science+Business Media New York 2015 683

J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_31
684
Abstract
Traumatic brachial plexus injuries in children
are very rare. A particular characteristic of
pediatric patients is a high incidence of root
avulsions. Compared to adults, children also
have minimal deafferentation pain and a higher
incidence of associated skeletal injuries and
exhibit faster recovery. The approach to chil-
dren with traumatic brachial plexus injuries
can be divided into three groups based on
age. In very young children (<4 years of
age), management is focused on restoring and
maximizing hand function, similar to patients
with obstetric brachial plexus injuries. In chil-
dren more than 12 years of age, management is
similar to that in adult patients. For these
patients, the priorities for restoring function,
in order of importance, are elbow exion,
shoulder abduction and/or stability, hand sen-
sation, wrist extension and nger exion, wrist
exion and nger extension, and lastly, intrin-
sic hand function. This approach relies on max-
imizing function while prioritizing movements
that have the least distance for nerves to regen-
erate to target muscles. For children in between
4 and 12 years of age, treatment priorities are
controversial. In this chapter, the approach to
and workup of children with traumatic brachial
plexus injuries is described, as well as treat-
ment options such as nerve grafts, nerve trans-
fers, and free functioning muscle transfers.
Introduction
Traumatic brachial plexus injuries (BPI) present a
complex problem that leads to severe impairment,
disability, and hardship. Treatment is best pro-
vided through multidisciplinary management at
tertiary centers with experience in diagnosis, sur-
gical treatment, and rehabilitation. The number of
brachial plexus injuries in adults continues to rise
due to the prevalence of extreme sports and
increasing number of survivors of motor vehicle
accidents (Shin et al. 2005); hence, treatment pro-
tocols for adult patients are well established and
constantly evolving. However, the incidence of
H. Chim et al.
these injuries in the pediatric population is much
lower and the literature on pediatric traumatic
brachial plexus injuries correspondingly scarce.
Boome reported an overall incidence of 1.1 % of
pediatric out of all brachial plexus lesions in his
series 16 cases in 14 years (Boome 2000).
In adults, the most common cause of brachial
plexus injuries remains motor vehicle accidents
involving motorcycles or bicycles, leading to
around 70 % of these injuries (Narakas 1985). In
the pediatric population, these injuries are most
often caused by motor vehicle accidents involving
children as passengers or pedestrians. Data from
the National Pediatric Trauma Registry of the
United States (Dorsi et al. 2010) showed an inci-
dence of 0.1 % of traumatic brachial plexus inju-
ries in pediatric multitrauma patients. Common
associated injuries include head injuries (intracra-
nial bleeds, skull fractures), upper extremity vas-
cular injury, and fractures of the humerus, ribs,
clavicle, scapula, and spine.
A particular characteristic of pediatric patients
is a high incidence of root avulsions, comprising
two-thirds of patients in some series (Dumontier
and Gilbert 1990; El-Gammal et al. 2003). Com-
pared to adults, children also have minimal
deafferentation pain and a higher incidence of
associated skeletal injuries and exhibit faster
recovery.
The approach to children with traumatic bra-
chial plexus injuries can be divided into three
groups based on age. In this context, children
are dened as those patients with open growth
plates. In very young children (<4 years of age),
management is focused on restoring and maxi-
mizing hand function, similar to patients with
obstetric brachial plexus injuries (Waters 1999;
Terzis and Kokkalis 2008). In children more
than 12 years of age, management is similar to
that in adult patients. For these patients, the prior-
ities for restoring function, in order of importance,
are elbow exion, shoulder abduction and/or sta-
bility, hand sensation, wrist extension and nger
exion, wrist exion and nger extension, and
lastly, intrinsic hand function. This approach
relies on maximizing function while prioritizing
movements that have the least distance for nerves
to regenerate to target muscles. For children in
31 Traumatic Brachial Plexus Injury in the Pediatric Population
Fig. 1 Anatomy of the
brachial plexus, which is
broadly divided into roots,
trunks divisions, cords, and
branches. LSS lower
subscapular nerve, MABC
medial antebrachial
cutaneous nerve, MBC
medial brachial cutaneous
nerve, TD thoracodorsal
nerve, USS upper
subscapular nerve
(By permission of Mayo
Foundation for Medical
Education and Research,
All rights reserved)
between 4 and 12 years of age, treatment priorities
are controversial; however, the trend has been
for performing nerve transfers to restore
elbow exion and shoulder abduction, due to the
high incidence of root avulsions (Gilbert
et al. 2006).
Pathoanatomy and Applied Anatomy
The brachial plexus is derived from ve cervical
nerve roots, typically C5, C6, C7, C8, and T1
(Fig. 1). There may be contributions to the plexus
from C4; this is termed a prexed plexus, with
an incidence ranging from 28 % to 62 %. T2 may
also contribute to the plexus; this is termed a
postxed plexus, with an incidence ranging
from 16 % to 73 % (Kerr 1918). Each spinal
nerve root is formed by the conuence of the
ventral and dorsal nerve rootlets as they pass
through the spinal foramina. The dorsal root gan-
glion contains cell bodies of the sensory nerves
and lies within the connes of the spinal canal and
685
foramen. Hence, a preganglionic injury is dened
as one where individual spinal roots are avulsed
off the spinal cord, while a postganglionic injury
is one located distal to the dorsal root ganglion
(Fig. 2). Low-energy traction injuries may lead to
stretch injuries (Fig. 2c), with potential for spon-
taneous recovery. High-energy injuries are asso-
ciated with more severe damage to each nerve
root, which may lead to rupture of the postgangli-
onic segment, with no potential for recovery with-
out surgery. In the pediatric population,
preganglionic injuries are particularly common.
A preganglionic lesion has no possibility of spon-
taneous recovery hence, surgical reconstruction
is mandatory for recovery of meaningful upper
extremity function.
The C5 and C6 nerve roots merge to form the
upper trunk, C7 continues as the middle trunk, and
C8 and T1 combine to form the lower trunk. The
conuence of C5 and C6, termed Erbs point, is
also the spot where the suprascapular nerve arises.
Each trunk then divides into an anterior and pos-
terior division and passes deep to the clavicle. The
686
Fig. 2 Injury to the brachial plexus can cause different
injuries to each nerve root at preganglionic or postgangli-
onic level. (a) Normal spinal cord and roots; (b) Avulsion
injuries are preganglionic and cannot be repaired;
three posterior divisions merge to form the poste-
rior cord, while the anterior divisions of the upper
and middle trunks combine to form the lateral
cord. The anterior division from the lower trunk
continues as the medial cord. The lateral cord
divides into two terminal branches: the
musculocutaneous nerve and the lateral cord con-
tribution to the median nerve. The posterior cord
continues as the axillary and radial nerves, and the
medial cord contributes to the ulnar nerve and the
median nerve. The portion of the brachial plexus
formed by roots and trunks is located above the
clavicle and termed the supraclavicular plexus;
the portion formed by the divisions is found
behind the clavicle and termed the retroclavicular
plexus; the portion of the plexus formed by cords
and terminal branches is termed the
infraclavicular plexus.
A number of terminal branches emanate from
the roots, trunks, and cords. The C5 root has a
contribution to the phrenic nerve, dorsal scapular
nerve (rhomboids), and long thoracic nerve
(serratus anterior, also with contributions from
H. Chim et al.
(c) stretch injuries are postganglionic and have potential
for spontaneous recovery; (d) rupture injuries can be
repaired with surgery (By permission of Mayo Foundation
for Medical Education and Research, All rights reserved)
C6 and C7). The suprascapular nerve
(supraspinatus and infraspinatus) and nerve to
subclavius muscle originate from the upper
trunk. The lateral cord gives off the lateral pectoral
nerve, while the medial cord gives off the medial
pectoral nerve, medial brachial cutaneous nerve,
and medial antebrachial cutaneous nerve. The
posterior cord gives off the upper subscapular
nerve (subscapularis), thoracodorsal nerve
(latissimus dorsi), and lower subscapular nerve
(subscapularis, teres major). Through careful and
detailed testing of the function of muscles sup-
plied by individual terminal nerve branches, the
exact level of the injury to the brachial plexus can
be determined. The sympathetic ganglion for T1
is located close to the T1 root and provides sym-
pathetic innervation to the head and neck. Hence,
a preganglionic injury at T1 level manifests clin-
ically as Horners syndrome, characterized by
ptosis, miosis, and anhidrosis on the affected
side. In children, Horners syndrome can also
lead to heterochromia (difference in eye color
between both eyes). A lack of sympathetic
31 Traumatic Brachial Plexus Injury in the Pediatric Population
stimulation in childhood can interfere with mela-
nin pigmentation of the melanocytes in the super-
cial stroma of the iris.
Assessment
A complete history and physical examination,
together with imaging and electrodiagnostic
tools, allow localization of the level and severity
of the injury to the brachial plexus. The aim of the
preoperative workup is to determine the level of
the injury preganglionic (root level) or postgan-
glionic (trunk, division, cord, or branches) and
also the severity of the lesion (partial or complete)
for each component of the brachial plexus injured.
This allows prognostication of recovery and a
decision to be made for surgery. If no recovery is
observed within the rst 36 months, surgery is
indicated. A preganglionic injury at one or more
levels has little to no chance of recovery; hence,
earlier intervention may be indicated.
Signs and Symptoms
A history of the mechanism of injury can be
obtained from the patient or parents. High-energy
injuries, for example, from motor vehicle acci-
dents, have a lower chance of spontaneous recov-
ery than low-energy injuries. Sharp injuries from
lacerations should be explored acutely or sub-
acutely. Gunshot wounds, in contrast, should be
observed as many of these will exhibit spontane-
ous recovery over time.
As other injuries may be associated with the
injury to the brachial plexus, management of these
takes precedence in the multitrauma patient. Man-
agement follows acute trauma life support (ATLS)
principles, with attention to the airway, breathing
and circulation taking priority, followed by
treatment of other life- and limb-threatening inju-
ries. A detailed examination of the various com-
ponents of the brachial plexus should be
performed when the patient is stable and able to
cooperate with the examiner. The exam should be
recorded in a manner that allows for comparison
of dates (Fig. 3). Serial detailed examinations
687
help determine the presence or absence of nerve
recovery and prognosis for spontaneous
improvement.
In an older child, a detailed systematic exami-
nation of the brachial plexus may be possible,
together with documentation of muscle strength
following the modied grading system of the
British Medical Research Council (BMRC) for
adults (M0 to M5) (Mendel and Florence 1990;
Table 1). Note that a patient cannot have grade
3 power unless there is full active motion against
the existing passive range of motion.
A preganglionic lesion can be diagnosed
through the presence of Horners syndrome,
which suggests a root avulsion at the T1 level.
Additional muscles that are innervated close to the
spinal cord provide further evidence of a pregan-
glionic lesion. Paralysis of the serratus anterior
muscle, manifest through winging of the scapula
(may be very difcult to examine secondary to
paralysis of other periscapular muscles), suggests
a lesion proximal to the long thoracic nerve,
formed by the C5, C6, and C7 nerve roots. Atro-
phy of the rhomboids and parascapular muscles
also suggests a preganglionic lesion proximal to
the origin of the dorsal scapular nerve. Examina-
tion of individual sensory dermatomes may some-
times be unreliable due to overlap from other
nerves or anatomical variation.
Different patterns of injury may be predicted
based on the mechanism of injury. Upper brachial
plexus injuries (Fig. 4) occur in motorcyclists who
fall with the shoulder forced downward and the
head pushed to the other side. Lower brachial
plexus or pan-plexus injuries (Fig. 5) may occur
during fall from height through hyperabduction of
the injured upper extremity.
Examination of donor nerves should also be
performed if nerve transfer is contemplated as a
treatment option, such as the spinal accessory
nerve, nerve to triceps, and medial pectoral
nerve. Presence of a Tinels sign and tenderness
in the supraclavicular or infraclavicular area sug-
gests a postganglionic lesion, while absence of
these suggests a preganglionic lesion. An advanc-
ing Tinels sign is a prognosticator and suggests a
recovering lesion. Minimal preservation of move-
ment in tested muscle groups suggests a partial
688 H. Chim et al.

Fig. 3 The Mayo brachial plexus evaluation form. This allows complete assessment of the entire extent of the injury at a
glance (By permission of Mayo Foundation for Medical Education and Research, All rights reserved)

Table 1 BMRC scale for 0: No muscle contraction visible

muscle strength
1: Muscle contraction is visible but there is no movement
2: Active movement is possible with gravity eliminated
3: Active movement against gravity
4-: Active movement against gravity and slight resistance
4: Active movement against gravity and moderate resistance
4+: Active movement against gravity and strong resistance
5: Normal power

injury with a greater potential for recovery. Exam-
ination of stability, active, and passive range of
motion of all joints should also be assessed. Con-
comitant spinal cord injury should be ruled out by
performing a full neurological examination of the
upper and lower extremity, testing for power, sen-
sation, and reexes. Finally, a vascular exam
should be performed, as the subclavian or axillary
artery can be ruptured or damaged in substantial
injury to the brachial plexus.
31 Traumatic Brachial Plexus Injury in the Pediatric Population
Fig. 4 Patterns of brachial plexus injury are predictable.
Upper brachial plexus injuries occur when the shoulder is
forced downward and the head pushed to the opposite side,
Fig. 5 Patterns of brachial plexus injury are predictable.
Avulsion of the lower nerve roots with stretch and rupture
of the upper nerve roots occurs, in this case during a fall
from a tree, through catching a branch with the injured
In younger children and infants, evaluation of
single muscles is not easy and the patient often
not cooperative or able to tolerate a lengthy phys-
ical examination. The muscle grading system of
Gilbert and Tassin (1984) has been proposed
(M0 complete paralysis; M1 perceptible
contraction; M2 weak movements; M3 nor-
mal muscle); however, it is our practice to attempt
to use the BMRC grading as not to confuse muscle
689
for example, following a motorcycle crash (By permission
of Mayo Foundation for Medical Education and Research,
All rights reserved)
upper extremity causing hyperabduction and injury to the
plexus (By permission of Mayo Foundation for Medical
Education and Research, All rights reserved)
grading systems. The posture of the child can be
used to evaluate the level of injury. Paralysis of the
upper roots is suggested by the upper limb being
held in internal rotation and pronation, with
no abduction possible. Slight exion of the elbow
may suggest involvement of C5, C6, and C7, while
full extension of the elbow suggests involvement
of only C5 and C6. In complete involvement
of the brachial plexus, the entire upper extremity
690
Fig. 6 CT myelogram of patient with root avulsion on
right at C6 and C7 levels. (a) Large pseudomeningocele
extending into the right neural foramen at C7-T1 level on
is ail. Horners syndrome may also be observed
in a preganglionic lesion involving the C8-T1
roots.
Assessment of sensation in younger children
and infants is similarly difcult. Often, children
will only respond to testing with painful
stimuli. Tinels sign may be used to assess
for the level of the lesion and presence of nerve
regeneration. In addition, in children,
deafferentation pain is absent; hence, unlike in
adults, patients with preganglionic lesions will
seldom exhibit pain. Disturbances of the sympa-
thetic system may be observed in the immediate
period following injury, such as anhidrosis, cya-
nosis, and edema.
Imaging and Electrodiagnostic Studies
Radiographs of the cervical spine, shoulder, and
chest should be obtained as part of the workup.
Transverse fractures of the cervical vertebrae sug-
gest preganglionic injuries with root avulsion.
Fractures of the clavicle or ribs (rst or second)
may be associated with injuries to the brachial
plexus. Fractures of other ribs may preclude the
use of intercostal nerves as a donor nerve for nerve
transfer (Kovachevich et al. 2010). An elevated
hemidiaphragm suggests damage to the phrenic
nerve and a possible preganglionic injury.
H. Chim et al.
coronal view; (b) the same pseudomeningocele seen on
axial view (By permission of Mayo Foundation for Med-
ical Education and Research, All rights reserved)
Computed tomography (CT) myelography is
very useful in determining the presence of pregan-
glionic injury. This is usually performed at least
34 weeks after the injury. This delay allows
blood clot in the area of the avulsed cervical root
to resorb and for a pseudomeningocele to form.
CT myelography has been shown to have a diag-
nostic accuracy ranging from 70 % to 95 % for
detection of nerve root avulsion compared to plain
myelography alone (Carvalho et al. 1997; Doi
et al. 2002). Presence of a pseudomeningocele is
associated with preganglionic injury in 98 % of
cases (Nagano et al. 1989; Fig. 6).
Magnetic resonance imaging (MRI) is also
useful in evaluating patients with suspected root
avulsion (Fig. 7) and allows better visualization of
the entire brachial plexus. Large neuromas, abnor-
malities of the rootlets, inammation and edema,
as well as mass lesions can be visualized using
MRI. While CT myelography should still be con-
sidered the rst-line imaging modality in
suspected nerve root avulsion, MRI using an
overlapping coronal-oblique slice technique has
been shown to be as reliable as CT myelography
in detecting nerve root avulsion, with a diagnostic
accuracy of 93 % (Doi et al. 2002).
Electrodiagnostic studies are pivotal in localiz-
ing and determining severity of injury in the bra-
chial plexus. Baseline nerve conduction studies
(NCS) and electromyography (EMG) should be
31 Traumatic Brachial Plexus Injury in the Pediatric Population
Fig. 7 MRI of patient with pseudomeningoceles at right
C6, C7, and T1 levels. (a) Two right-sided cervical
pseudomeningoceles are seen on coronal view; (b) axial
performed 36 weeks after injury, after Wallerian
degeneration has occurred. Serial follow-up stud-
ies can then be performed every 610 weeks to
assess for recovery, complementing ndings on
physical examination for the purposes determin-
ing if there will be spontaneous recovery or if
surgical reconstruction is necessary.
Nerve conduction studies include testing of
motor and sensory nerves. Motor nerve testing is
useful in detecting more distal injuries and conduc-
tion blocks in incomplete injuries. In traumatic bra-
chial plexus injuries, amplitudes of compound
muscle action potentials (CMAPs) are in general
low. Sensory nerve testing is useful in determining
whether a root injury is pre- or postganglionic. In a
preganglionic injury, the dorsal root ganglion is
spared injury even though it is detached from the
spinal cord; hence, sensory nerve action potentials
(SNAPs) are preserved. However, the patient is
insensate in the associated sensory nerve distribution.
There is excellent correlation between C6 (supercial
radial nerve), C7 (median sensory to long digit), C8
(ulnar sensory nerve to small digit), and T1 (medial
antebrachial cutaneous nerve) nerve root levels and
individual peripheral sensory nerves in the upper
extremity, aiding in localizing the level of the lesions
in the brachial plexus. C5 and C6 innervate the lateral
antebrachial cutaneous nerve, and this can be tested
as part of sensory nerve evaluation for C5.
691
view of one pseudomeningocele (By permission of Mayo
Foundation for Medical Education and Research, All rights
reserved)
EMG provides the most reliable assay of motor
nerve injury. Fibrillation potentials and positive
sharp waves, indicative of denervation, can be
seen in proximal muscles as early as 1014 days
and in distal muscles in 36 weeks. Motor unit
analysis can determine the presence of injury in
individual muscles. Polyphasic motor units occur
in the presence of injury or pathology, while
nascent potentials indicate axonal regeneration.
Reduced recruitment of motor unit potentials can
be demonstrated immediately after injury. Testing
of individual muscles can help to distinguish pre-
ganglionic from postganglionic lesions. For
example, denervation of the paraspinal muscles,
rhomboids, and serratus anterior is a strong indi-
cator of a preganglionic lesion as these muscles
are innervated by branches from the cervical
roots. Unfortunately, EMG recovery does not
always equate with clinical recovery, and evi-
dence of clinical recovery may not be detected
through EMG in complete lesions, despite ongo-
ing regeneration, when target end organs are more
distal.
Intraoperative electrodiagnostic studies are
useful prior to making a denitive surgical deci-
sion. These include the use of nerve action poten-
tials (NAP), somatosensory and motor evoked
potentials (SSEPs and MEPs) and CMAPs. The
presence of a NAP distal to a lesion indicates
692
preserved axons in an incomplete lesion or signif-
icant regeneration, with a correspondingly better
prognosis (Kline and Happel 1993). Hence,
neurolysis alone without additional treatment
may be sufcient. The presence of an SSEP and
MEP suggests continuity between the peripheral
nervous system and the spinal cord via a dorsal
root and ventral root, respectively. Hence, an
SSEP and MEP is only present in postganglionic
lesions. As lesions are not necessarily all or none,
there may be situations where the SSEP is present
and MEP absent or vice versa. In such cases, the
viability of the root becomes difcult to ascertain,
and correlation with EMG, clinical ndings, and
radiographic ndings is necessary. CMAPs are
useful in partial lesions where the magnitude of
the lesion is proportional to the number of func-
tioning axons.
Injuries Associated with Traumatic
Brachial Plexus Injury
Brachial plexus injuries are often caused by high-
energy trauma; hence, there are often substantial
concomitant injuries. When a patient is evaluated
in the emergency room, standard ATLS principles
should be followed and the airway, breathing, and
circulation stabilized prior to a search for other
life- and limb-threatening injuries.
Injuries that should be evaluated include trau-
matic brain injuries and spinal cord injuries as
well as vascular injuries, ipsilateral upper extrem-
ity musculoskeletal injuries, scapulothoracic dis-
sociation, pneumothorax, hemothorax, and rib
fractures. Treatment of these injuries takes prece-
dence initially over the brachial plexus injury.
Classification
Brachial plexus injuries can be classied by the
cervical/thoracic nerves involved, level of the
injury, and severity of each nerve injury according
to the Seddon (1975) and Sunderland (1978)
classications.
Determination of the cervical/thoracic nerves
involved in the injury as well as level of the injury
H. Chim et al.
is based on clinical examination supplemented by
imaging studies, as described previously. The
level of the injury can be broadly divided into
preganglionic root, supraclavicular plexus (roots
and trunks), retroclavicular plexus (divisions),
and infraclavicular plexus (cords and branches).
Different authors have described various classi-
cations for the level of injury, pursuant on their
individual surgical strategies. Narakas (1981)
describes dividing the level of injury into ve
levels (supraganglionic root, infraganglionic spi-
nal nerve, infraganglionic trunk, and
retroclavicular and terminal branches). Chuang
(2010) alternatively divides brachial plexus inju-
ries into four levels (Level 1, preganglionic root
injury including spinal cord, rootlets, and root
injuries; Level 2, postganglionic spinal nerve
injury limiting the lesion to the interscalene
space and proximal to the suprascapular nerve;
Level 3, preclavicular and retroclavicular BPI
including trunks and divisions; Level
4, infraclavicular BPI including cords and termi-
nal branches proximal to the axillary fossa).
The severity of the injury to each nerve is
classied according to the Seddon/Sunderland
classication, with ve levels of nerve injury.
Seddon initially described three basic types of
peripheral nerve injury, which was expanded to
ve types by Sunderland. In neurapraxia (rst-
degree injury), a focal physiologic conduction
block exists at the site of nerve injury, with the
endoneurium, perineurium, and epineurium
remaining intact. This typically recovers sponta-
neously within days to weeks and does not require
surgical intervention. In axonotmesis (second- to
fourth-degree injury), the connective tissue frame-
work of the nerve is preserved, but the axon is
disrupted to varying extents, resulting in
Wallerian degeneration distal to the site of nerve
injury with a disruption of nerve conduction. In a
second-degree injury, the axon is disrupted, but
the endoneurium, perineurium, and epineurium
are preserved. Nerve regeneration and recovery
of motor and sensory function are expected
but may take months to years. In third-degree
injury, the axon and endoneurium are disrupted,
but the perineurium and epineurium are intact. In a
fourth-degree injury, the axon, endoneurium,
31 Traumatic Brachial Plexus Injury in the Pediatric Population
and perineurium are disrupted, while the
epineurium remains intact. In third- and fourth-
degree injuries, regeneration occurs variably or
may not occur at all; hence, these lesions often
require surgical intervention. In neurotmesis
(fth-degree injury), the nerve is completely
disrupted; hence, surgical intervention is neces-
sary. Fifth-degree injuries include preganglionic
avulsions and postganglionic ruptures of the bra-
chial plexus.
Outcome Tools
There is no clear consensus on the best instrument
to measure outcomes after brachial plexus recon-
struction in either the adult or pediatric patient.
The BMRC muscle strength scale is the most
common tool used, on a scale from M0 to M5.
However, this tool has a number of limitations
including its lack of precision and inter-rater reli-
ability as well as a wide range of strength covered
by grade of M4 (MacAvoy and Green 2007;
Shahgholi et al. 2012). The Disabilities of the
Arm, Shoulder and Hand (DASH) score is the
most validated measure of upper extremity func-
tion (Dowrick et al. 2005) and has been useful as
an outcome tool following brachial plexus recon-
struction; however, it is not validated for the pedi-
atric population.
A number of outcome tools are used to assess
outcomes following obstetric brachial plexus
injury and reconstruction, and there may be a
role to adopting them to infants or young children
following traumatic brachial plexus injury. The
most widely used tool is the Mallet classication
(Abzug and Kozin 2010), which measures integ-
rity of muscles innervated by the upper brachial
plexus. The arm is tested in ve different move-
ments: abduction, external rotation, hand behind
head, hand to back, and hand to mouth. Each
movement is classied from grade 0 to V. A recent
modication was proposed, the addition of a hand
to belly button category, which tests the childs
ability to reach to midline. Other tools used
include the Toronto Test Score (Bae et al. 2003),
Active Movement Scale, and Gilbert shoulder
classication.
693
Differences Between Adult
and Pediatric Injuries
In pediatric patients, surgical reconstruction
options are affected by the presence of open
growth plates and potential growth that may affect
the long-term results of certain procedures. The
most active physes in the upper extremity include
the proximal humerus, which accounts for 80 % of
longitudinal growth (Pritchett 1991) and the distal
radius. The length of the humerus grows approx-
imately 1.3 cm in boys and 1.2 cm in girls from
age seven until skeletal maturity, while the length
of the radius grows approximately 1.0 in boys and
0.9 cm in girls from age seven until skeletal matu-
rity (Pritchett 1988). Boys usually reach skeletal
maturity between 16 and 17 years old, while girls
reach skeletal maturity between 14 and
15 years old.
Therefore, in the pediatric population, the use
of functional free muscle transfer should be done
so with care as the transferred muscle may not
grow in proportion with the rest of the upper
extremity and lead to joint contractures. Addition-
ally, secondary procedures that cross joints, such
as tendon transfers, tenodesis, and joint fusions,
may lead to joint contractures and limitation of
function of the affected limb.
Treatment Options
Surgery has been shown to be benecial for
patients in traumatic brachial plexus injuries
with no hope for spontaneous recovery or in the
absence of clinical or electrodiagnostic evidence
of recovery. The mechanism of injury provides a
vital clue to decide the possibility for spontaneous
recovery. In sharp or blunt injuries causing lacer-
ations, all patients should undergo surgical explo-
ration as the possibility of spontaneous recovery is
low. In gunshot wounds, many patients will
recover spontaneously as the majority of injuries
are neuropraxic and caused by the shock wave
from the passage of the projectile. Hence,
nonoperative management is preferred
initially. In traction injuries, the indication and
694
timing for surgery are more controversial and
would depend on the type and exact mechanism
of injury. Early exploration of the brachial plexus
between 3 and 6 weeks should be performed
if there is a high suspicion of root avulsion.
In general, surgical exploration of the brachial
plexus should be performed by 6 months of
injury and is not performed more than 12 months
after the injury as results are poor, as the time for
nerve regeneration to the target muscle is greater
than the time of survival of the denervated motor
end plate.
Nonoperative Management
Indications/Contraindications
In patients waiting for surgery or those treated
conservatively, physical therapy is essential to
strengthen functioning muscles, maintain range
of motion, and prevent stiffness and joint contrac-
tures. Occupational therapy may be useful in
modication of the patients workplace setting
and home environment to improve the patients
functional ability and also in use of orthoses and
adaptations (Booney 1998). Specic indications
and contraindications for pure nonoperative
management of traumatic brachial plexus
injuries are listed below (Table 2). However,
rehabilitation has a role to play in all surgical
patients as well, both preoperatively and
postoperatively.
Table 2 Traumatic brachial plexus injuries: nonoperative
management
Indications Contraindications
Mechanism of injury: Mechanism of injury:
gunshot wounds, certain sharp lacerations, root
traction injuries avulsions
Patient expectations: Patient expectations:
unrealistic goals, agreeable with surgical
unwillingness to undergo reconstruction
surgery
Spontaneous ongoing Lack of improvement on
recovery in involved clinical and
elements electrophysiologic testing
H. Chim et al.
Techniques
Physical/Occupational Therapy
Recommendations
The main goals in treatment of traumatic brachial
plexus injury patients are prevention of secondary
deformities, maintenance of passive range of
motion, pain suppression, sensory rehabilitation
for recovery of somatosensory decits, treatment
of developmental disregard, and postoperative
care (Smania et al. 2012).
Due to the long time needed for reinnervation
of muscles following BPI, muscle atrophy will
lead to muscle imbalance, and subsequent second-
ary deformities of the upper extremity are com-
monplace. Hence, an important component of
rehabilitation consists of the prevention of joint
contractures.
Passive movements of the injured upper
extremity can be combined with an orthoses,
such as elbow and hand splints, to avoid joint
stiffness and to maintain range of motion. Bio-
feedback can be used to lessen cocontraction.
Botulinum toxin injections can be used for the
treatment of imbalanced muscles. It is particularly
important to avoid deformities such as internal
rotation of the shoulder, which markedly reduces
function and ability to care for ones self and may
also lead to glenohumeral dysplasia.
While neuropathic pain after BPI is a major
concern in adult patients, in the pediatric popula-
tion, deafferentation pain is often absent, despite
having a greater chance of preganglionic injury.
However, if this becomes an issue in older pediatric
patients, multidisciplinary management is manda-
tory for optimal patient care. Different modalities of
treatment are used, such as pharmacotherapy, phys-
ical therapy, transcutaneous electrical nerve stimu-
lation (TENS), and psychosocial intervention, all
playing important roles in pain management.
Following a peripheral nerve injury, patients
develop altered proles of neural impulses.
Hence, sensory reeducation is useful to reprogram
the brain through the use of cognitive learning
techniques and graded tactile stimuli to improve
tactile gnosis (Jerosch-Herold 2011). Exercises
including perception of different shapes and
31 Traumatic Brachial Plexus Injury in the Pediatric Population
textures as well as localization of stimuli help retrain
the brain to recognize sensory stimuli through cor-
tical plasticity. Developmental disregard, or behav-
ioral suppression of motor activity in the impaired
limb, has been treated through a targeted technique,
the constraint-induced movement therapy (CIMT)
(Fritz et al. 2012). This aims to increase use of the
impaired limb by limiting use of the non-affected
upper extremity. CIMT has been proposed to work
through changes in behavioral approach by the
patient with subsequent cortical reorganization in
the brain (Hoare et al. 2007).
Postoperative rehabilitation is key to achieving
good functional results. An example is passive
stretching of muscles to prevent secondary defor-
mities such as maintaining external rotation of the
shoulder to minimize glenohumeral joint defor-
mity. Following nerve transfers, induction exer-
cises are also used by patients (Terzis and
Kostopoulos 2007) to help the donor nerve to
re and reactivate the reinnervated muscle.
Outcomes
The literature is scarce on objective outcomes of
rehabilitation through physical and occupational
therapy. However, it is the experience of most
other brachial plexus surgeons that physical and
occupational therapies, both in the preoperative
and postoperative periods, are essential to opti-
mize outcomes following surgical reconstruction
of the brachial plexus.
Operative Management
Indications/Contraindications
Surgical management is indicated when sponta-
neous recovery is impossible, for example, in root
avulsions or in the absence of clinical and
electrodiagnostic evidence of recovery by
6 months after injury. There are few absolute
contraindications to surgical reconstruction of
the brachial plexus. These include the unwilling-
ness of the patient to undergo the surgical proce-
dure with subsequent prolonged rehabilitation or
unrealistic goals of the patient. Other contraindi-
cations include patients who exhibit spontaneous
695
ongoing recovery and those with life-threatening
conditions precluding surgery. Patients presenting
late (more than 12 months after injury) are not
candidates for primary plexus reconstruction but
may be candidates for free functioning muscle
transfer.
Timing of Surgery
Immediate exploration and repair is performed in
patients with a sharp open injury of the brachial
plexus. Surgery allows direct visualization and
repair of injured elements. For patients with blunt
injuries to the brachial plexus without chances of
recovery, surgery is performed about 34 weeks
after the injury to allow the zone of nerve injury to
delineate. In patients with a high suspicion of root
avulsion, surgery can be performed even earlier.
Delayed surgery is performed in patients with a
chance for spontaneous recovery. Exploration
may be delayed up to 6 months after the inciting
injury, particularly for patients where the mecha-
nism of injury suggests that spontaneous recovery
is possible, such as low velocity gunshot wounds
or closed traction injuries. Lack of progressive
recovery on clinical examination and electrophys-
iologic studies is an indication for surgery.
Finally, secondary reconstruction through pro-
cedures such as free functioning muscle transfer
(FFMT), tendon transfers, and adjunctive proce-
dures are performed for patients who present late
(more than 12 months after injury). In these
patients, primary plexus reconstruction is associ-
ated with poor results.
Primary Reconstruction
Primary reconstruction of the brachial plexus con-
sists of a number of surgeries and may consist of
direct nerve repair, neurolysis, nerve grafting, and
nerve transfers with or without free functioning
muscle transfer. This is performed either in an
immediate or delayed fashion. The exact series
of procedures performed may sometimes only be
decided after adequate exploration and exposure
of the brachial plexus to determine the injured
elements.
Direct repair of nerve ends may be performed
in an immediate fashion after sharp lacerations.
696
Neurolysis alone may be sufcient if the nerve is
in continuity and a nerve action potential (NAP)
is obtained (Kim et al. 2003). Nerve grafting is
indicated with ruptures or postganglionic neuro-
mas that do not conduct a NAP across the injured
segment. Typically, sural nerve graft is harvested
and used to bridge the gap as multiple cables.
Nerve transfer (neurotization) is indicated in
preganglionic injury/root avulsions or to acceler-
ate reinnervation of target muscles by shortening
the distance needed for the nerve to regenerate to
the motor end plate. A less important functional
nerve is sacriced and coapted to a more vital
denervated distal nerve. Common nerve transfers
used for shoulder abduction include spinal acces-
sory nerve or phrenic nerve to suprascapular nerve
as well as transfer of the triceps motor branch to
the axillary nerve. These nerve transfers have the
advantage of the donor and recipient nerves being
in close proximity, therefore obviating the need
for nerve grafting. Double and even triple nerve
transfers have been shown to improve shoulder
abduction compared to that achieved with a single
nerve transfer only (Cardenas-Mejia et al. 2008).
Nerve transfer for elbow exion can be
performed using either the intercostal nerves
with direct coaptation to the biceps motor branch
or the spinal accessory nerve with an
interpositional nerve graft (Songcharoen
et al. 1996). Patients with a history of rib fractures,
chest tube placement, or thoracotomy may not be
appropriate candidates for intercostal nerve trans-
fer as there is a possibility that the nerve may have
been damaged during these procedures. More
recently, the Oberlin transfer (Teboul
et al. 2004), consisting of transfer of a fascicle
from the ulnar nerve to motor branch of the
biceps, has been used with excellent results.
Intraoperative nerve stimulation is used to identify
the fascicles that stimulate wrist exion (exor
carpi ulnaris) and used as a donor for nerve trans-
fer. Double nerve transfers to restore elbow ex-
ion have been described, consisting of a fascicle
from the ulnar nerve to the motor branch of the
biceps and also a fascicle from the median nerve
to the motor branch to the brachialis (Mackinnon
et al. 2005). This has been shown to have
improved outcomes (Mackinnon et al. 2005;
H. Chim et al.
Liverneaux et al. 2006) in some studies; however,
other studies have shown similar outcomes to
single nerve transfer (Carlsen et al. 2011). These
common nerve transfers are further described in
detail subsequently.
Other donor nerves used include the medial
pectoral nerve, which can be transferred to the
musculocutaneous nerve or motor branch to
biceps. Contralateral C7 nerve root can also be
used for restoration of shoulder or median nerve
function and may be an attractive alternative for
the very young pediatric traumatic brachial plexus
group (age <4 years). However, outcomes are
poor in adult patients with risk of donor-site mor-
bidity, including permanent motor and sensory
loss (Sammer et al. 2012).
Surgical Approaches to the Brachial
Plexus for Primary Reconstruction
Except in patients where specic nerve transfers have
been selected as the treatment modality, the brachial
plexus should be exposed to identify the injured
elements that may be amenable to primary repair,
neurolysis, or nerve grafting. A supraclavicular
approach allows exposure of the nerves and trunks,
while an infraclavicular approach allows exposure of
the cords and terminal branches. Exposure of the
divisions can be exposed through either approach
and may also require a clavicular osteotomy.
A transverse incision is usually used approxi-
mately 2.5 cm cephalad to the clavicle. This is a
cosmetically appealing exposure that can be
extended by an incision following the sternoclei-
domastoid and the deltopectoral interval if needed
(Figs. 8 and 9). The infraclavicular incision begins
at the clavicular insertion of the sternoclei-
domastoid muscle, continues laterally toward the
coracoid process, and extends laterally to the
deltopectoral groove and the arm.
Following exposure of the injured elements,
intraoperative electrodiagnostic assessment is
performed routinely by our group, including the
use of somatosensory evoked potentials (SSEPs),
motor evoked potentials (MEPs), and nerve action
potentials (NAPs). SSEPs and MEPs test
the integrity of the intraforaminal and intraspinal
sensory and motor pathways and help test whether
31 Traumatic Brachial Plexus Injury in the Pediatric Population 697

Fig. 8 The brachial plexus

can be exposed through a
transverse incision
approximately 2.5 cm
cephalad to the clavicle.
This can be extended
cephalically through an
incision posterior to the
sternocleidomastoid muscle
and caudally through an
incision at the deltopectoral
interval, if needed
(By permission of Mayo
Foundation for Medical
Education and Research,
All rights reserved)

Table 3 Spinal accessory nerve (SAN) to suprascapular

nerve (SSN) transfer: case checklist
OR table: normal table
Position: supine with neck turned to contralateral side;
shoulder and neck elevated off table with bump
Equipment: nerve stimulator, intraoperative
electrophysiologic monitoring, operating microscope
Tourniquet: sterile for lower extremity
Precautions: avoid muscle relaxants, long-acting
paralytic agents, and agents depressing cortical responses
(inhalational agents should not be used until
neuromonitoring is completed)

to determine if the lesion has potential for sponta-

neous recovery (Kline and Happel 1993; Kline
and Hudson 1995).

Surgical Procedure: Spinal Accessory

Fig. 9 The entire brachial plexus can be explored through
this surgical approach (By permission of Mayo Foundation
for Medical Education and Research, All rights reserved)
the spinal nerves are in continuity with the spinal
cord. Hence, they are useful for testing the integ-
rity of a proximal nerve considered for nerve
grafting, as well as testing for preganglionic
injury. NAPs test for the presence of functioning
sensory and motor axons over a nerve segment
and are useful for testing a neuroma-in-continuity
Nerve (SAN) to Suprascapular Nerve
(SSN) Transfer
Preoperative Planning
Details of the surgical procedure should be
discussed with anesthesia (Table 3), in particular
the need for avoidance of muscle relaxants, long-
acting paralytic agents, and agents depressing
cortical responses. As the surgical procedure is
often a long one, patients should have a urinary
catheter inserted, with sequential compression
devices and adequate padding of bony
698 H. Chim et al.

prominences. A sterile tourniquet may be required

if sural nerve grafts need to be harvested. An
underbody warming device is benecial to keep
the patients body temperature normal. The entire
upper extremity is prepared and draped to allow
free movement of the entire arm; the ipsilateral
neck, mandible, hemithorax, axilla, and upper
extremity (with bilateral lower extremities if it is
anticipated that sural nerve grafts will be needed)
should be prepared and draped. A nerve stimula-
tor and electrodes required for intraoperative elec-
trophysiologic monitoring should be available, as
well as an operating microscope.

Positioning
The patient is placed in the supine position with
the neck turned to the contralateral side. The
shoulder and neck are elevated off the table with
a small midline bump.

Technique Fig. 10 Transfer of spinal accessory nerve to

See Fig. 10, Tables 4 and 5.
Surgical Procedure: Transfer of Triceps
Motor Branch to Axillary Nerve
suprascapular nerve for restoring shoulder abduction
(By permission of Mayo Foundation for Medical Educa-
tion and Research, All rights reserved)
Table 4 Spinal accessory nerve (SAN) to suprascapular
nerve (SSN) transfer: surgical steps

Preoperative Planning Transverse incision over distal clavicle

This is similar to that for SAN to SSN transfer,
including the case checklist.
Positioning
The patient is placed in a supine position with the
arm placed across the patients chest and held by
an assistant. A posterior approach to the arm
is used.
Technique
See Fig. 11, Tables 6 and 7.
Surgical Procedure: Ulnar Nerve
Fascicular Transfer to Biceps Motor
Branch
Preoperative Planning
This is similar to that for SAN to SSN transfer,
including the case checklist.
Identify SAN several centimeters above clavicle on
anterior surface of trapezius, conrm with electrical
stimulation
Dissect SAN as far distally as possible, preserve proximal
branch to upper portion of trapezius
Divide SAN distally
Identify SSN at suprascapular notch, divide superior
transverse ligament (optional, if area of injury is proximal
to suprascapular notch)
Dissect SSN as far proximally as possible to origin from
upper trunk
Divide SSN proximally
Suture SAN to SSN with 9/0 ethilon sutures, reinforce
transfer with brin glue
Close in layers
Positioning
The patient is placed in the supine position with
the arm outstretched on an arm table. This allows
access to medial aspect of the arm, where the
incision is made in the groove between the biceps
and triceps muscles.
31 Traumatic Brachial Plexus Injury in the Pediatric Population
Table 5 Spinal accessory nerve (SAN) to suprascapular
nerve (SSN) transfer: postoperative protocol
Type of immobilization: shoulder immobilization
Length of immobilization: 3 weeks
Rehabilitation protocol: physiotherapy started 3 weeks
postoperatively, targeted at maintaining passive motion
of all joints. Active muscle exercises started after signs of
motor reinnervation appear, focused on reeducation and
strengthening of muscles (typically 812 months
postsurgery)
Technique
See Fig. 12, Tables 8 and 9.
Surgical Procedure: Median Nerve
Fascicular Transfer to Brachialis
Motor Branch
Preoperative Planning
This is similar to that for SAN to SSN transfer,
including the case checklist.
Positioning
The patient is placed in the supine position with
the arm outstretched on an arm table. This allows
access to medial aspect of the arm, where the
incision is made in the groove between the biceps
and triceps muscles.
Technique
See Fig. 13, Tables 10 and 11.
Figure 14 shows a double nerve transfer for
restoration of elbow exion, consisting of transfer
of a fascicle from the ulnar nerve to the motor
branch of the biceps and also a fascicle from the
median nerve to the motor branch to the
brachialis.
Surgical Procedure: Intercostal Nerve
Transfer to Musculocutaneous Nerve
Preoperative Planning
This is similar to that for SAN to SSN transfer,
including the case checklist.
699
Positioning
The patient is placed in the supine position with
the arm outstretched on an arm table. This allows
access to medial aspect of the arm, where the
incision is made in the groove between the biceps
and triceps muscles.
Technique
See Tables 12 and 13. Intercostal nerves can be
transferred to the biceps branch (Fig. 15) or
directly to the musculocutaneous nerve (Fig. 16).
Outcomes of Nerve Transfers
The literature supports the effectiveness of nerve
transfers over nerve grafts in adults. However,
little is written about the outcomes of nerve trans-
fer in the pediatric traumatic plexus population. In
a systematic review of 31 studies comparing the
efcacy of nerve transfers and nerve grafting for
traumatic upper plexus palsy (Garg et al. 2011), it
was found that pooled international data strongly
favored dual nerve transfer over traditional nerve
grafting for restoration of shoulder and elbow
function. The study found that, for patients who
underwent nerve transfer for restoration of elbow
exion, 83 % and 96 % of 299 patients in total
achieved elbow exion strength of grade M4 or
greater and M3 or greater, respectively. In con-
trast, for patients who underwent nerve
grafts, only 56 % and 82 % of 57 patients in
total achieved elbow exion strength of grade
M4 or greater and M3 or greater, respectively.
Similarly, 74 % (total 54) of patients who
underwent dual nerve transfers for should func-
tion had shoulder abduction strength of grade M4
or greater compared to 35 % (total 57) of patients
who underwent single nerve transfer and 46 %
(total 28) of patients who underwent nerve
grafting.
Little and colleagues recently reported on the
outcomes of elbow exion with median and/or
ulnar nerve fascicle transfer in C5-6 and C5-7
palsies in 31 patients with neonatal brachial
plexus palsy (Little et al. 2014). Indications for
nerve transfer included root avulsion, dissociative
recovery, late presentation, and failed nerve graft
reconstruction. Concomitant procedures were
performed in 63 % of cases including long head
700
Fig. 11 Transfer of triceps branch to the axillary nerve for
restoring shoulder abduction. (a) Longitudinal incision
over posterior arm; (b) exposure of the nerve to triceps
and axillary nerve; (c) triceps branch (below) is transferred
of triceps to axillary nerve transfer, shoulder inter-
nal rotation contracture release, and tendon trans-
fer for external rotation; however, the results of
these procedures were not reported. The primary
outcome measure was postoperative elbow ex-
ion and supination as measured on the Active
H. Chim et al.
to the axillary nerve (above); (d) completion of nerve
transfer (By permission of Mayo Foundation for Medical
Education and Research, All rights reserved)
Movement Scale (AMS). Of the 31 patients,
27 (87 %) obtained functional elbow exion
(AMS  6), and 24 (77 %) had full exion recov-
ery (AMS 7). Of the 24 patients for whom
supination recovery was recorded, 5 (21 %)
obtained functional recovery (AMS  6).
31 Traumatic Brachial Plexus Injury in the Pediatric Population
Table 6 Transfer of triceps motor branch to axillary
nerve: surgical steps
Longitudinal incision on posterior arm from acromion to
midarm
Retract deltoid anteriorly
Identify axillary nerve in quadrilateral space
Mobilize axillary nerve proximally identify anterior
and posterior divisions. Separate anterior division from
posterior division and divide as proximal as possible
(alternatively, divide entire nerve and then separate
anterior division from posterior)
Open interval between long and lateral heads of triceps
Identify motor branch to long head of triceps, conrm
with electrical stimulation
Mobilize and divide triceps motor branch distally
Suture triceps motor branch to anterior division axillary
nerve with 9/0 ethilon sutures, reinforce transfer with
brin glue
Close in layers
Table 7 Transfer of triceps motor branch to axillary
nerve: postoperative protocol
Type of immobilization: shoulder immobilization
Length of immobilization: 3 weeks
Rehabilitation protocol: physiotherapy started 3 weeks
postoperatively, targeted at maintaining passive motion
of all joints. Active muscle exercises started after signs of
motor reinnervation appear, focused on reeducation and
strengthening of muscles
Free Functioning Muscle Transfer
(FFMT)
FFMT involves the transfer of a muscle from a
distant donor site to the upper extremity, with
microvascular coaptation of vessels and its
nerve, to replicate the function of a muscle in the
affected upper extremity. The muscle most com-
monly used for this purpose is the gracilis muscle,
which has the advantage of a long distal tendon
which reaches into the forearm for hand
reanimation, as well as a proximal location of its
nerve, which allows more rapid reinnervation of
the muscle following neurotization. The
thoracoacromial artery and cephalic vein are the
most common vessels used for anastomosis of the
functional muscle, followed by the thoracodorsal
artery and vein. FFMT was rst reported for
701
delayed reconstruction (>12 months after injury)
(Ikuta et al. 1979) or as a salvage procedure after
failed nerve reconstruction. However, it has been
used increasingly to provide reliable elbow ex-
ion where primary reconstruction with nerve
grafts or nerve transfers is not possible.
Doi et al. (2000) described a double free func-
tioning gracilis muscle transfer aimed at achieving
prehension in patients with pan-plexal injuries. In
stage I (Fig. 17), the rst FFMT is neurotized by
the spinal accessory nerve and used to restore
elbow exion and wrist or nger extension. In
stage II (Fig. 18), performed 68 weeks after the
initial surgery, the second FFMT is neurotized by
the fth and sixth intercostal nerves to restore
nger exion, together with the use of the third
and fourth intercostal nerves to neurotize the
motor branch of the triceps for elbow extension
and coaptation of the intercostal sensory rami to
the medial cord of the brachial plexus to restore
hand sensibility.
In the pediatric population, contractures of
joints have been noted following FFMT occurring
secondary to decreased growth of the FFMT in
comparison to the rest of the upper extremity.
Therefore, FFMT should be cautiously applied.
There are situations, however, where there are no
other alternative, and patients and parents need to
be counseled regarding its use.
Surgical Procedure: Double Free
Functional Gracilis Muscle Transfer
Preoperative Planning
This is similar to that for SAN to SSN transfer,
including the case checklist.
Positioning
The patient is placed in the supine position with
the leg abducted for harvest of the gracilis muscle.
Technique
See Tables 14 and 15.
Outcomes
Little data exists in the literature on outcomes of
FFMT in children. Zuker and Manktelow (2007)
702
Fig. 12 Transfer of ulnar nerve fascicle to biceps motor
branch (Oberlins method) for restoring elbow exion. (a)
Biceps motor branch is identied and dissected from
musculocutaneous nerve. (b) A nerve stimulator is used
to select an ulnar nerve fascicle responsible for wrist
Table 8 Ulnar nerve fascicular transfer to biceps motor
branch: surgical steps
Longitudinal medial skin incision in proximal arm
Identify musculocutaneous nerve and its three branches
(motor branch to the biceps, motor branch to brachialis,
lateral antebrachial cutaneous nerve)
Divide motor branch of biceps as far proximal as possible
Identify ulnar nerve, perform intraepineurial dissection
using loupes or operating microscope
Identify posteromedial fascicles innervating mostly
exor carpi ulnaris with aid of electrical stimulation
Divide one or two chosen fascicles under magnication
Suture ulnar nerve fascicles to biceps motor branch nerve
with 9/0 ethilon sutures, reinforce transfer with brin glue
Close in layers
reported muscle contracture as soon as 2 months
after surgery and grip strength reaching 25 % of
the normal side. Another series of four patients
(Bahm and Ocampo-Pavez 2008) reported FFMT
for delayed treatment of obstetric brachial plexus
H. Chim et al.
exion without affecting intrinsic hand function; this is
then isolated with internal neurolysis. (c) The selected
fascicle is divided and transferred to the biceps motor
branch (By permission of Mayo Foundation for Medical
Education and Research, All rights reserved)
Table 9 Ulnar nerve fascicular transfer to biceps motor
branch: postoperative protocol
Type of immobilization: shoulder immobilization
Length of immobilization: 3 weeks
Rehabilitation protocol: physiotherapy started 3 weeks
postoperatively, targeted at maintaining passive motion
of all joints. Active muscle exercises started after signs of
motor reinnervation appear, focused on reeducation and
strengthening of muscles
palsy in children between 6 and 13 years of age. In
this series with a 2-year follow-up, M3 grasp was
achieved in three out of four children. Further
studies are needed to assess long-term outcomes
of FFMT in children.
Secondary Reconstruction
Procedures such as tendon transfers, shoulder
arthrodesis, and wrist and hand arthrodesis can
improve function in the upper extremity or are
considered when there is no further recovery.
31 Traumatic Brachial Plexus Injury in the Pediatric Population
Fig. 13 Transfer of median nerve fascicle to brachialis
motor branch for restoring elbow exion. (a) Brachialis
motor branch is identied and dissected from the
musculocutaneous nerve. (b) A nerve stimulator is used
to select a median nerve fascicle responsible for wrist
Table 10 Median nerve fascicular transfer to brachialis
motor branch: surgical steps
Longitudinal medial skin incision in proximal arm
Identify musculocutaneous nerve and its three branches
(motor branch to the biceps, motor branch to brachialis,
lateral antebrachial cutaneous nerve)
Divide nerve to brachialis proximally (may require
careful dissection of brachialis branch of lateral
antebrachial cutaneous nerve)
Identify median nerve near brachial artery and vein,
perform intraepineurial dissection using loupes or
operating microscope
Identify fascicle innervating exor carpi radialis with aid
of electrical stimulation
Divide chosen fascicle under magnication
Suture median nerve fascicle to brachialis motor branch
nerve with 9/0 ethilon sutures, reinforce transfer with
brin glue
Close in layers
703
exion without affecting intrinsic hand function; this is
then isolated with internal neurolysis. (c) The selected
fascicle is divided and transferred to the brachialis motor
branch (By permission of Mayo Foundation for Medical
Education and Research, All rights reserved)
Table 11 Median nerve fascicular transfer to brachialis
motor branch: postoperative protocol
Type of immobilization: shoulder immobilization
Length of immobilization: 3 weeks
Rehabilitation protocol: physiotherapy started 3 weeks
postoperatively, targeted at maintaining passive motion
of all joints. Active muscle exercises started after signs of
motor reinnervation appear, focused on reeducation and
strengthening of muscles
Preferred Treatment
General Philosophy
We divide our approach to children with traumatic
brachial plexus injuries into three groups based on
age. In very young children (<4 years of age),
704 H. Chim et al.

Fig. 14 Ulnar nerve

fascicular transfer to the
biceps motor branch and
median nerve fascicular
transfer to the brachialis
motor branch are often
performed together
(By permission of Mayo
Foundation for Medical
Education and Research,
All rights reserved)

Table 12 Intercostal nerve transfer to musculocutaneous nerve: surgical steps

Inframammary incision extending from midaxillary line to costochondral junction for exposure of 3rd to 6th intercostal
nerves
Elevate subcutaneous tissue and pectoralis major and minor muscles, protect intercostobrachial nerve
Anterior surface of rib incised, periosteum circumferentially elevated while protecting underlying pleura
Elevate rib with umbilical tape to allow dissection of intercostal nerve
Periosteal sleeve of rib in midclavicular line incised, intercostal nerve (motor branch) identied with aid of nerve
stimulator and dissected
Intercostal nerve dissected to costochondral junction anteriorly and midaxillary or posterior axillary line posteriorly
Procedure repeated for other intercostal nerves
Each intercostal nerve transected distally and passed through serratus anterior muscle to axillary region
Longitudinal medial skin incision in proximal arm
Identify musculocutaneous nerve and its three branches (motor branch to the biceps, motor branch to brachialis, lateral
antebrachial cutaneous nerve)
Divide motor branch of biceps as far proximal as possible if planning transfer to biceps branch. This is performed if the
biceps branch has sufcient length for direct transfer; otherwise, intercostal nerve transfer to the musculocutaneous
nerve directly is chosen
Suture intercostal nerves to biceps motor branch or directly to musculocutaneous nerve with 9/0 ethilon sutures,
reinforce transfer with brin glue. Neurorrhaphy should be done with arm externally to 90 or to the patients own limit
of passive external rotation if less than 90 and abducted 90 to reduce tension on the repair
All wounds closed in layers

management is focused on restoring and maxi-
mizing hand function, similar to patients with
obstetric brachial plexus injuries. Subsequent pri-
orities include restoration of elbow and shoulder
function. The brachial plexus is explored and
nerve grafts performed from viable roots rst,
with nerve transfers if required. In pan-plexus
injuries, the priority is to restore hand function
through reinnervation of the medial cord through
nerve grafts if this is possible. In preganglionic
injuries, the only option may be nerve transfers.
In children greater than 12 years of age, man-
agement strategy is similar to adult patients. For
these patients, the priorities for restoring function,
in order of importance, are elbow exion, shoul-
der abduction and/or stability, hand sensation,
wrist extension and nger exion, wrist exion
and nger extension, and lastly, intrinsic hand
31 Traumatic Brachial Plexus Injury in the Pediatric Population
Table 13 Intercostal nerve transfer to musculocutaneous
nerve: postoperative protocol
Type of immobilization: shoulder immobilization
Length of immobilization: 3 weeks
Rehabilitation protocol: physiotherapy started 3 weeks
postoperatively, targeted at maintaining passive motion
of all joints. Active muscle exercises started after signs of
motor reinnervation appear, focused on reeducation and
strengthening of muscles. There will be a lifelong
limitation of abduction and external rotation to prevent
avulsing the intercostal nerve to biceps motor branch
(or musculocutaneous nerve), typically to 90 external
rotation and 90 abduction
Fig. 15 Intercostal nerve transfer to the biceps motor
branch for restoring elbow exion (By permission of
Mayo Foundation for Medical Education and Research,
All rights reserved)
function (which is often impossible to obtain).
This approach relies on maximizing function
while prioritizing movements that have the least
distance for nerves to regenerate to target muscles.
For children in between 4 and 12 years of age,
treatment is controversial due to a paucity of lit-
erature. Nerve transfers have been used predomi-
nantly to restore elbow exion and shoulder
705
Fig. 16 Intercostal nerve transfer to the
musculocutaneous nerve for restoring elbow exion
(By permission of Mayo Foundation for Medical Educa-
tion and Research, All rights reserved)
abduction due to the high incidence of root avul-
sions (Gilbert et al. 2006; Goubier et al. 2008;
Miller et al. 2013). Donor nerves that do not
work well in adults, for example, contralateral
C7, may result in better outcomes in the pediatric
age group due to their enhanced regenerative
capacity. In general, because of continued growth
during childhood, we try to avoid secondary
reconstructive procedures such as free functioning
muscle transfer, tenodesis, and joint fusion, which
may interfere with skeletal growth. FFMT may
result in contractures due to differential growth
between the functioning muscle and the child.
Upper Trunk (C5-6 Injury) in Pediatric
Patients >4 Years
In these patients, we would explore the brachial
plexus. In the presence of a functional C5 nerve
stump, nerve grafts to the posterior division of the
upper trunk and suprascapular nerve serve to
reinnervate the shoulder. In patients presenting
706
Fig. 17 Stage I of the double free gracilis muscle transfer,
neurotized by the spinal accessory nerve, aims to restore
elbow exion and wrist or nger extension (By permission
of Mayo Foundation for Medical Education and Research,
All rights reserved)
later (more than 69 months after injury) or with-
out a functional C5 nerve stump, our preference is
to perform double nerve transfer (SAN to SSN
and nerve to triceps to axillary nerve) for shoulder
function. For recovery of elbow function, we
would perform either a single or double nerve
transfers (ulnar nerve fascicle to biceps motor
branch and median nerve fascicle to brachialis
motor branch).
Pan-Plexus Injury in Pediatric Patients
>4 Years
In these patients, priority rests in restoring elbow
exion, then shoulder abduction. If exploration of
the supraclavicular plexus reveals functional
nerve stumps, nerve grafts are used to reinnervate
H. Chim et al.
Fig. 18 Stage II of the double free gracilis muscle transfer.
The 3rd and 4th intercostal nerves are used to neurotize the
motor branch of the triceps muscle to restore elbow exten-
sion, while the 5th and 6th intercostal nerves are used to
neurotize the gracilis to restore nger exion
(By permission of Mayo Foundation for Medical Educa-
tion and Research, All rights reserved)
the axillary and suprascapular nerves for shoulder
function. If additional roots are available, nerve
grafts are used to target elbow exion (via the
anterior division of the upper trunk). Use of con-
tralateral C7 may be an alternative to younger
patients and should be avoided in the older age
children. Spinal accessory and intercostal nerves
should also be considered in restoring shoulder
function and elbow exion. In older children
nearing skeletal maturity, FFMT neurotized by
the intercostal nerves is an option to restore
elbow exion or can be used as part of a double
transfer (Doi) to obtain elbow exion and nger
exion.
31 Traumatic Brachial Plexus Injury in the Pediatric Population
Table 14 Functional free muscle transfer: surgical steps
Longitudinal incision over pes anserine
Distal gracilis tendon identied and isolated
Distal medial thigh incision made to identify
myotendinous junction of gracilis
Anterior limb of elliptical incision around proximal skin
paddle made, fascia over adductor longus incised
Pedicle and obturator nerve identied in interval between
adductor longus and gracilis
Posterior limb of elliptical incision made, fascia over
adductor magnus incised
Resting tension of muscle marked with sutures at 5 cm
intervals
Distal tendon divided and passed to proximal wound
Proximal tendon released from pubic ramus
Vascular pedicle and nerve ligated and divided
Flap transferred to upper extremity, proximal tendon
secured to acromion/clavicle
Vascular anastomoses performed, typically to
thoracoacromial artery and cephalic vein
Suture of obturator nerve to donor nerve
Suture of distal tendon of functional muscle to target
muscle in proper tension
Close in layers
Table 15 Functional free muscle transfer: postoperative
protocol
Type of immobilization: shoulder immobilization in 30
of abduction and exion and 60 of internal rotation with
elbow in 100 of exion, wrist in neutral position, and
ngers in forced exion or extension, depending on
reconstruction
Length of immobilization: 4 weeks
Rehabilitation protocol: low-intensity electrical
stimulation starting 3rd postoperative week and
continuing until EMG-conrmed reinnervation noted in
muscles, then EMG biofeedback techniques to train
muscles to move elbow and ngers
Surgical Pitfalls and Prevention
Surgical pitfalls include those associated with sub-
stantial surgery. Each portion of the brachial plexus
reconstruction is complex and requires attention to
detail. The detailing of all the pitfalls and preven-
tion is beyond the scope of this chapter, as each type
of surgery has its own set of intraoperative and
postoperative complications.
707
Summary
Pediatric patients with traumatic brachial plexus inju-
ries are a rare patient population. Unique treatment is
required, customized to the patients age and growth
stage, as well as to the type of injury. In general, for
patients less than 4 years of age, the aim is to maxi-
mize hand function. For those between 4 and 12 years
of age, the literature at the moment does not provide
enough information to dictate the best modality or
course of treatment. For patients older than 12 years,
treatment follows that for the adult population.
Some nerve transfers inappropriate in adults
may be appropriate for children, for example,
use of contralateral C7 as a donor nerve, due to
the greater regenerative capacity of children.
There is a considerable need to obtain more out-
come data to determine the optimal treatment
regime for children. However, even with existing
data from the adult population on brachial plexus
reconstruction, selection of optimal treatment for
pediatric patients remains highly controversial.
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 Transverse Myelitis and Neuralgic
Amyotrophy 32
Allan Belzberg, Glendaliz Bosques, and Kelly Pham

Contents Abstract
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 712 Neuralgic amyotrophy (NA), a lower motor
neuron (LMN) lesion, presents a accid
Pathoanatomy and Applied Anatomy . . . . . . . . . . . . 713
Basic Spinal Cord Anatomy . . . . . . . . . . . . . . . . . . . . . . . . . 713
monoplegia of the upper extremity in children.
Basic Peripheral Nerve and Brachial Transverse myelitis (TM) may present with
Plexus Anatomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 713 either an LMN or an upper motor neuron
Inammatory and Autoimmune Etiology . . . . . . . . . . . 714 (UMN) injury, depending on areas affected on
Assessment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 715 the cord. Both entities are inammatory and
Signs and Symptoms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 715 autoimmune in nature. Diagnosis is with MRI
Imaging and Other Diagnostic Studies . . . . . . . . . . . . . . 719 and serological studies as well as nerve con-
Associated Injuries . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 721
Classication . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 721 duction studies (NCS) and electromyography
Outcome Tools . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 722 (EMG). Treatment varies slightly, but includes
Treatment Options . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 722
immunosuppression with steroids, replace-
Nonoperative Management . . . . . . . . . . . . . . . . . . . . . . . . . . 722 ment of antibodies with plasmapheresis in
Indications/Contraindications . . . . . . . . . . . . . . . . . . . . . . . . 724 addition to intravenous immunoglobulin
Rehabilitation Techniques . . . . . . . . . . . . . . . . . . . . . . . . . . . 724 (IVIG), immunosuppressant, and antineoplas-
Outcomes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 725
Operative Treatment of TM and NA . . . . . . . . . . . . . . . . . 726
tic agents. Further management includes reha-
Management of Complications . . . . . . . . . . . . . . . . . . . . . . 728 bilitation measures with stretching,
strengthening, range of motion, neuromuscular
Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 730
electrical stimulation, patient and family edu-
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 730 cation, and equipment evaluation. Children
with persistent LMN decits and limited
recovery (68 months after onset of disease)
A. Belzberg (*)
Department of Neurosurgery, The Johns Hopkins Hospital, may warrant surgical considerations for
Baltimore, MD, USA peripheral nerve surgery. This may include
e-mail: abelzbe1@jhmi.edu; ajbelzberg@gmail.com the use of nerve transfers. Secondary surgery,
G. Bosques including muscle and tendon transfers, can be
Childrens Memorial Hermann Hospital, The University of considered 12 years after disease onset, if
Texas Health Science Center at Houston (UTHealth)
persistent residual decits are present. Most
Medical School, Houston, TX, USA
e-mail: glendaliz.bosques@memorialhermann.org children with NA have good outcomes with
resolution of symptoms and improvement in
K. Pham
Johns Hopkins University, Baltimore, MD, USA function. Children with TM have a less favor-
e-mail: kdauer1@jhmi.edu able outcome with one third resolution rate.
# Springer Science+Business Media New York 2015 711
J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_32
712
Introduction
Neuralgic amyotrophy (NA), also known as
immune-mediated brachial plexopathy or brachial
plexitis, is characterized by an initial period of
neuropathic pain followed by paresis and pares-
thesias in a peripheral nerve distribution, com-
monly the brachial plexus, but can also involve
the lumbosacral plexus, phrenic nerve, recurrent
laryngeal nerve, cranial nerves, or even the distal
autonomic nervous system (van Alfen 2011).
There are two forms of NA: idiopathic neuralgic
amyotrophy (INA), also known as Parsonage-
Turner syndrome, and hereditary neuralgic
amyotrophy (HNA) (van Alfen and van Engelen
2006). Though the etiology is not completely
elucidated, an inammatory-immune-mediated
process is suspected for both. This theory is
supported by biopsies of affected brachial plexi
containing inammatory inltrates as well as the
commonly reported antecedent immunization or
infection, either bacterial or viral (Suarez
et al. 1996; van Alfen et al. 2000a). HNA, which
is autosomal dominant in inheritance, is linked to
a mutation in the SEPT9 gene on chromosome
17q25 (van Alfen 2007). Both forms of NA are
quite rare with the estimated overall incidence of
INA being 23/100,000 people/year (MacDonald
et al. 2000). The ratio of male to female is 1.8:1;
median age of onset has been reported to be
3 years of age. There is no predisposition to side
of the body involved. The epidemiological data on
INA in children is limited because of the clinical
similarities to poliomyelitis, resulting in
misdiagnosis. HNA, even rarer, has been
described in only approximately 200 families
worldwide (van Alfen 2007). With a complete
history, there is usually a parent with the diagnosis
or a history of similar symptoms; however there
are sporadic cases, though the incidence is
unknown. Onset of HNA is usually during the
second or third decade of life (van Alfen
et al. 2000b).
Transverse myelitis (TM), on the other hand, is
a spinal cord disorder characterized by abrupt
onset of motor, sensory, and autonomic distur-
bances thought to be secondary to a
A. Belzberg et al.
demyelinating lesion of the spinal cord across
one or multiple segments. It is generally classied
as idiopathic TM or disease-associated TM, which
is linked to connective tissue diseases such as
systemic lupus erythematosus (SLE) and
Sjgrens syndrome (SS), as well as other disor-
ders of the central nervous system such as multi-
ple sclerosis (MS), acute demyelinating
encephalomyelitis (ADEM), or neuromyelitis
optica (NMO). In some cases, initial presentation
may indicate TM, though patients progress to
develop optic neuropathy in the case of NMO or
patchy lesions throughout the central nervous sys-
tem over time and space as in the case of MS
(Borchers and Gershwin 2012). Diagnostic
criteria for idiopathic TM established by the
Transverse Myelitis Consortium Working Group
(TMCWG) include the presence of sensory, motor
or autonomic dysfunction attributable to the spi-
nal cord, bilateral signs or symptoms, clearly
dened sensory level, exclusion of compressive
etiology with inammation of the spinal cord
evidenced in the cerebrospinal uid (CSF),
serum or gadolinium enhancement on magnetic
resonance imaging (MRI), and progression to
nadir between 4 h and 21 days following the
onset of symptoms (Transverse Myelitis Consor-
tium Working Group 2002). Though the etiology
is unknown, it also is thought to be immune-
mediated as evidenced by the immune inltrates
in the spinal cord, cerebrospinal uid, and serum.
In the general population, the incidence of new
cases is 18/1,000,000, 20 % of which are in
children (Wolf et al. 2012). There is a bimodal
distribution with peaks in incidence in children
from birth to 2 years of age and 517 years of age
(Pidcock et al. 2007). There is conicting evi-
dence of female to male predominance in
children.
Usually an upper motor neuron lesion disorder,
a subgroup of children diagnosed with TM by
CSF, and MRI ndings have been identied as
having a distinct recovery pattern resulting in
lower motor neuron (LMN) injury (Sadowsky
et al. 2011). These children recover function in
all except for one extremity resulting in a accid
monoplegia. The upper extremity is usually
involved in a polio-like syndrome, though lower
32 Transverse Myelitis and Neuralgic Amyotrophy
Fig. 1 The spinal cord is
comprised of ventral and
dorsal white and grey
matter. The ventral grey
matter of the spinal cord
contains the alpha motor
neuron. (Reproduced with
kind permission from
Marion Murray and
Springer Science +
Business: Neuroscience in
Medicine, chapter Ulnar
Deciencies, 2008, page
209, Murray M, Figure 13)
extremity cases have also been reported (Liao
et al. 2007). Similar to NA, they develop weak-
ness and rapidly progressive atrophy. Some
patients may have intact sensory function. This
is thought to be secondary to either involvement
of the alpha motor neuron of the ventral spinal
cord or of the proximal nerve roots resulting in a
LMN type presentation and thus clinically similar
to NA (Sadowsky et al. 2011).
Pathoanatomy and Applied Anatomy
Basic Spinal Cord Anatomy
Patients with TM have spinal cord involvement,
which, depending on the extent of the demyelin-
ation, may include the alpha motor neuron. The
ventral grey matter of the spinal cord contains the
cell body of the alpha motor neuron, which exits
the spinal cord via the ventral root. See Fig. 1.
This ventral (motor) root connects with the dorsal
(sensory) root to form a spinal nerve root. NA
does not involve pathology of the spinal cord.
Basic Peripheral Nerve and Brachial
Plexus Anatomy
Once the ventral and dorsal nerve roots exit the
spinal cord, they form the spinal nerve roots, which
join in the periphery to form the plexus or continue
on as peripheral nerves. The majority of NA patients
713
present with brachial plexus involvement. However,
the lumbosacral plexus, phrenic nerve, recurrent
laryngeal nerve, cranial nerves, and the distal auto-
nomic nervous system may be involved, especially
in HNA. The brachial plexus, Fig. 2, formed from
the cervical spinal nerve roots C5, C6, C7, C8, and
T1, provides the sensory and motor innervation for
the upper extremity. It is located between the neck
and axilla, proximally between the anterior and mid-
dle scalene muscles and distally just posterior to the
clavicle and pectoralis muscles.
The plexus is divided into roots, trunks, divi-
sions, cords, and branches moving distally. The
nerve roots C5, C6, C7, C8, T1, and variably C4
and T2 descend from the spinal cord through the
neural foramen into the neck to form the plexus. C5
and C6 form the upper trunk, C7 the middle trunk,
and C8 and T1 the lower trunk. The trunks descend
and each branches into an anterior and a posterior
division. The anterior divisions of the upper and
middle trunks join to form the lateral cord. The
posterior divisions from the upper, middle, and
lower trunk join to form the posterior cord. The
anterior division from the lower trunk continues on
to form the medial cord. Off of the lateral cord
branch the lateral pectoral nerve and the
musculocutaneous nerve. It also joins the medial
cord to form the median nerve. The posterior cord
continues on to become the radial and axillary
nerves. The medial cord continues on to become
the ulnar nerve (Preston and Shapiro 2005).
On a more cellular level, these peripheral
nerves are made up of a layer of connective tissue
714
Nerve to Subclavius
Suprascapular
Medial Pectoral
Lateral Pectoral
Axillary
Musculocutaneous
Radial
Median
Ulnar
Medial Antebrachial Cutancous
(Medial cutaneous nerve to the forarm)
Fig. 2 Cervical and thoracic nerve roots form the brachial
plexus by branching into the upper, middle, and lower
trunks, then on to the anterior and posterior divisions,
which further merge to become the lateral, medial, and
posterior cords. The cords then branch into the peripheral
nerve distributions, the musculocutaneous, axillary, radial,
median, and ulnar nerves (Reproduced with kind
immediately surrounding the myelin sheath of an
axon, called the endoneurium. The endoneurium-
covered nerves are further grouped together and
covered by another layer of connective tissue, the
perineurium. The perineurium-covered nerves are
then grouped together and covered by a nal layer
of connective tissue, the epineurium, to form a
nerve.
Inflammatory and Autoimmune
Etiology
The pathophysiology of NA, though not
completely understood, is thought to be
A. Belzberg et al.
Dorsal Scapular
C4
C5
C6
C7
C8
T1
Long Thoracie
Upper Subscapular
Thoraeodorsal
(middle subscapular)
Lower Subscapular
Medial Brachial Cutaneous
(Medial cutaneous nerve to the arm)
permission from Marios Loukas and Springer Science +
Business Media: Surgical and Radiologic Anatomy, The
prexed and postxed brachial plexus: a review with sur-
gical implications, volume 32, issue 3, 2010, page
253, Pellerin M, Kimball Z, Tubbs RS, Nguyen S,
Matusz P, Cohen-Gadol AA, Loukas M)
inammatory and autoimmune in nature. There
may also be a mechanical component related to
local repetitive trauma.
On a cellular level, biopsies of affected bra-
chial plexi demonstrate mononuclear inamma-
tory inltrates, mainly T-lymphocytes,
surrounding the epineurial and endoneurial ves-
sels (Suarez et al. 1996). This inammatory inl-
tration causes patchy damage to the brachial
plexus and other nerves leading to the character-
istic presentation of patchy and severe neuro-
pathic pain followed by muscle paralysis.
It is often preceded by a viral or bacterial
infection, such as an upper respiratory infection.
A history of immunization, serum therapy,
32 Transverse Myelitis and Neuralgic Amyotrophy
pregnancy, childbirth, or surgery may also ante-
cede symptoms. With any of the aforementioned,
the body may produce an inappropriate immune-
mediated response against the brachial plexus
resulting in nerve inammation and subsequent
injury (van Alfen et al. 2000a).
In addition to inammatory and autoimmune
pathophysiology, both INA and HNA can be
related to local trauma to the neck involving the
brachial plexus. On a more cellular level, local
trauma can weaken the perineurium resulting in
focal damage to the individual fascicles of the
nerve subsequently resulting in the scattered pat-
tern of motor and sensory involvement (van Alfen
2011).
Similarly, idiopathic TM is thought to be auto-
immune in nature with multiple theories of path-
ophysiology, all of which culminate in the
demyelination and neuronal injury of the spinal
cord. Inltrates in the spinal cord in patients with
TM include monocytes and CD4+ and CD8+
lymphocytes, which are thought to lead to necro-
sis and cavitation of the spinal cord. The theory of
molecular mimicry is evidenced in the often pre-
ceding infection (Lymes disease, HIV, myco-
plasma, herpes virus, syphilis, and other central
nervous system infections) leading to anti-GM1
antibodies and the subsequent attack by the
bodys own immune system. The theory of
superantigens is that certain infections induce
T-cells against myelin causing destruction of the
spinal cord. There may also be humoral-mediated
dysregulation leading to increased IgE causing an
allergic response and further tissue destruction.
Finally, IL-6 released from astrocytes and
microglia bind to oligodendroglia and axons caus-
ing activation of nitric oxide synthetase leading to
tissue damage of the spinal cord (Wolf
et al. 2012).
In addition to the aforementioned, disease-
associated myelitis is found in conjunction with
a number of autoimmune diseases such as sarcoid-
osis, Behets disease, Sjgrens syndrome, con- et al. 2012). The most frequent initial symptoms
nective tissue disorders, and systemic lupus
erythematosus. There is also a spectrum of inam-
matory demyelinating disorders of which TM is a
part of, in addition to NMO, MS, and ADEM.
NMO is dened as transverse myelitis found in
715
conjunction with optic neuropathy. NMO is a
well-known autoimmune disorder and is associ-
ated with AQP4, aquaporin, antibodies. Acute
myelitis may also be the presenting symptom in
MS, and thus children may be initially diagnosed
with TM only to further go on to be diagnosed
with MS upon further imaging or another demye-
linating episode. Similar and yet distinct, there is
now a recurrent form of acute TM, making this
distinction sometimes difcult (Borchers and
Gershwin 2012).
Assessment
Signs and Symptoms
The majority of children with NA present after a
viral upper airway infection, osteomyelitis of the
shoulder or humerus (most commonly seen in
neonates), or vaccination. Two thirds of children
present with severe neuropathic pain in the shoul-
der or arm, which is consistent with adult presen-
tation. The other third present with painless
weakness and atrophy of the shoulder girdle or,
less often, more distal muscles of the upper
extremity (van Alfen et al. 2000a). If present, the
initial pain may last approximately 23 weeks
followed by patchy paralysis and atrophy of the
muscles innervated by the affected nerves (Tsairis
et al. 1972). One should consider other etiologies
such as tumor, which can inltrate local lymph
nodes resulting in compression of the brachial
plexus, direct inltration of the nerve by lym-
phoma or leukemia, as well as primary nerve
sheath tumors such as schwannomas, neuro-
bromas, or neurobrosarcomas. These usually
result in more insidious onset of pain, paresthe-
sias, and muscle atrophy, but should be consid-
ered in the differential diagnosis.
TM is also often preceded by infection but less
commonly following vaccination (Wolf
are fever; pain in the back, extremities, and abdo-
men; ascending numbness and weakness; walking
difculty; balance problems; and loss of bowel
and bladder control (urinary retention and/or con-
stipation). The distribution of these symptoms is
716
dependent on the spinal level of the lesion as well
as the area of the spinal cord involved. Cervical
cord lesions present with upper extremity as well
as lower extremity weakness and sensory
decits; lower thoracic and lumbar lesions result
in lower extremity weakness and numbness only.
Initial sensory loss and weakness are ascending
and may present similarly to Guillain-Barr
syndrome. If the posterior columns are involved,
the child may have ne motor dyscoordination
leading to ataxia (Borchers and Gershwin 2012;
Wolf et al. 2012). In the subgroup of children with
alpha motor neuron involvement or root level
involvement, the typical signs and symptoms
may be present initially, but are followed by
recovery of all except one limb, which remains
accid, atrophic, and areexic (Sadowsky
et al. 2011).
In the initial phase of TM, patients often pre-
sent in spinal shock with absent deep tendon
reexes (DTRs) and interruption of the sympa-
thetic ow through the spinal cord resulting in
bradycardia and hypotension. Spinal shock gen-
erally resolves in a period of days to 12 weeks
with return of DTRs and signs consistent with
UMN ndings including hyperreexia,
Babinskis reex, and increase in muscle tone.
With lesions above T6, there is a risk of auto-
nomic dysreexia. Other signs and symptoms
involve urinary retention secondary to disruption
of the connection between the pontine micturition
center and the sacral spinal cord. Constipation is
also a potential complication of neurogenic bowel
secondary to decreased motility.
Physical examination in a patient suspected to
have NA includes a complete generalized exami-
nation including overall appearance as well as
evaluation for hypotelorism or other facial dys-
morphic features, which are associated with HNA
(Jeannet et al. 2001).
Head, eyes, ears, nose, and throat evaluation
must be completed to look for signs of viral or
bacterial infection, which may precede the onset
of pain and weakness in NA and TM. In NA,
evaluate speech for possible dysphonia with
recurrent laryngeal nerve involvement. Palpation
of the neck to evaluate for mass lesions of the
brachial plexus is pertinent as well. This should
A. Belzberg et al.
be followed by a complete cardiovascular and
respiratory examination, as involvement of the
phrenic nerve may result in diaphragm paralysis
and result in paradoxical breathing patterns. Also
look for vasomotor symptoms such as edema, nail
and hair changes, and temperature dysregulation
(Fig. 3).
General examination should be followed by a
full neurologic and musculoskeletal examination.
Start the neurologic examination by testing the
function of the cranial nerves I-XII as NA may
affect these. One should then test sensation to light
touch and pinprick in both a dermatomal fashion
to rule out cervical cord or root pathology, but also
in the peripheral nerve distribution to evaluate for
plexus or peripheral nerve injury. DTRs and other
primitive reexes should be assessed to evaluate
for hyperreexia or hyporeexia to determine if
there is UMN or LMN involvement. Peripheral
nerve lesions resulting from NA should only be
associated with lower motor neuron ndings. In
TM, the majority of cases may have UMN signs,
though some cases may present with alpha motor
neuron involvement resulting in LMN exam nd-
ings (Fig. 4).
Manual muscle testing should be completed
and classied. One may use the Hospital for
Sick Children Active Movement Scale (Tables 1
and 2). Scoring ranges from 0 to 5 with 0 no
muscle contraction with gravity removed, 1
icker of movement with gravity removed, 2
less than 50 % range of motion (ROM) with
gravity removed, 3 greater than or equal to
50 % ROM with gravity removed, 4 full
ROM with gravity removed, and 5 less than
50 % ROM against gravity.
The musculoskeletal examination begins with
inspection of the child for edema, ecchymosis,
deformity, scar, rash, or atrophy. It is important
also to evaluate muscle bulk, scapular positioning,
arm positioning (abduction, adduction, internal
rotation, external rotation), and the childs posture
at rest and with movement. Palpation starts with
the bony structures including the sternal notch, the
sternoclavicular joint, along the clavicle to the
acromioclavicular joint, and on to the greater and
lesser tuberosities of the humerus. From here pal-
pate the coracoid process, the suprascapular fossa,
32 Transverse Myelitis and Neuralgic Amyotrophy 717

Fig. 3 Ten-year-old male with recurrent hereditary neu- weakness. (b) Typical facial features with considerable
ralgic amyotrophy (HNA) (Courtesy of Shriners Hospital hypotelorism. (c) Decreased shoulder abduction and wrist
for Children, Philadelphia). (a) Episode with right-sided drop. (d) Limited ability to grasp
718 A. Belzberg et al.

Fig. 4 A 3-year-old child

with residual accid
monoplegia of the left upper
extremity related to
transverse myelitis
involving the alpha motor
neuron of the ventral spinal
cord. Muscle atrophy and
circumferential and length
differences can be present.
This is postsurgical
intervention, as evidenced
by the surgical scar on the
anteromedial arm
(Reproduced with
permission from Sadowsky
et al. (2011))

Table 1 The active movement scale and myotomal distribution (Leis 2010)
Myotomal
Action Muscles distribution
Shoulder abduction Middle deltoid, supraspinatus C5, C6
Shoulder adduction Pectoralis major, latissimus dorsi, teres major, coracobrachialis, C5, C6, C7, C8, T1
infraspinatus, long head of the triceps, anterior and posterior deltoid
Shoulder exion Anterior deltoid, pectoralis major, biceps brachii, coracobrachialis C5, C6, C7, C8, T1
Shoulder external Infraspinatus, teres minor, posterior deltoid, supraspinatous C5, C6
rotation
Shoulder internal Subscapularis, pectoralis major, latissimus dorsi, anterior deltoid, teres C5, C6, C7, C8, T1
rotation major
Elbow exion Biceps brachii, brachialis, brachioradialis, pronator teres C5, C6, C7
Elbow extension Triceps, anconeus C6, C7, C8, T1
Forearm supination Biceps brachii, supinator C5, C6
Forearm pronation Pronator quadratus, pronator teres, exor carpi radialis C6, C7, C8, T1
Wrist exion Flexor carpi ulnaris, exor carpi radialis, palmaris longus, exor C6, C7, C8, T1
digitorum supercialis, exor digitorum profundus, exor pollicis
longus
Wrist extension Extensor carpi ulnaris, extensor carpi radialis longus, extensor carpi C6, C7, C8, T1
radialis brevis, extensor digitorum communis, extensor digiti minimi,
extensor indicis, extensor pollicis longus
Finger exion Flexor digitorum supercialis, exor digitorum profundus, C7, C8, T1
lumbricals, dorsal and palmar interossei, exor digiti minimi
Finger extension Extensor digitorum communis, extensor indicis proprius, extensor C7, C8
digiti minimi
Thumb exion Flexor pollicis brevis, exor pollicis longus, opponens pollicis, C8, T1
adductor pollicis
Thumb extension Extensor pollicis longus, extensor pollicis brevis, abductor pollicis C7, C8
longus

and along the spine of the scapula. To examine the (innervated by the long thoracic nerve) is likely
scapula, ask the child to ex both arms and push weak and/or atrophic, resulting in unopposed
against a wall. Evaluate scapular movement; if the middle trapezius (innervated by CN XI) action
scapula wings medially, then the serratus anterior (Fig. 5).
32 Transverse Myelitis and Neuralgic Amyotrophy
With abduction of the arm, a laterally deviating
scapula is indicative of upper trapezius weakness
and unopposed serratus anterior muscle. Special
musculoskeletal tests to evaluate supraspinatus
muscle impingement or bicipital tendinitis should
be considered to rule out other soft tissue-related
causes (Malanga and Nadler 2006). In addition to
the aforementioned musculoskeletal examination,
the Mallet classication, the Toronto Test Score,
and the Active Movement Scales (AMS), as men-
tioned above, may be used to measure active
movement of the upper extremity (Bae
et al. 2008).
Table 2 Hospital for sick children active movement scale
Muscle
grade Denition
0 No muscle contraction with gravity
removed
1 Flicker of movement with gravity removed
2 Less than 50 % range of motion (ROM)
with gravity removed
3 Greater than or equal to 50 % ROM with
gravity removed
4 Full ROM with gravity removed
5 Less than 50 % ROM against gravity
Fig. 5 Fourteen-year-old
female with neuralgic
amyotrophy affecting the
right long thoracic nerve
(Courtesy of Shriners
Hospital for Children,
Philadelphia). (a) Right
scapular winging
exacerbated by pushing
against wall. (b) Side view
with marked scapular
winging
719
Imaging and Other Diagnostic Studies
Diagnosis of NA is made with a combination of
blood work, imaging studies, and
electrodiagnostic studies, though it remains a
diagnosis of exclusion. Laboratory studies,
though unnecessary for diagnosis, may show
mildly elevated creatine kinase and elevated
liver function tests. Other signs of prior infection,
such as viral titers or antibodies, may be positive.
As in other autoimmune disorders,
antiganglioside antibodies may also be present in
the serum. Cerebrospinal uid (CSF) studies in
NA are generally normal, except for slightly
increased CSF protein (van Alfen 2007).
In addition to the aforementioned laboratory
studies, imaging studies may also contribute to
the diagnosis not only by ruling out other pathol-
ogies but also in ndings consistent with
NA. Imaging may begin with a basic chest X-ray
to evaluate for the presence of a mass lesion or for
elevation of the diaphragm as seen with phrenic
nerve involvement. For further evaluation, mag-
netic resonance neurography (MRN) of the neck
to evaluate the plexus directly and MRI of the
shoulder to evaluate the musculature are appropri-
ate. The MRN of the brachial plexus may
720
Fig. 6 T2-weighted imaging of the cervical spinal cord of
child with residual accid monoplegia after idiopathic TM
with cervical cord inammation involving the alpha motor
neuron. (a) Longitudinal involvement of the cervical spinal
demonstrate T2-signal enhancement or focal
thickening of the plexus (van Alfen and van
Engelen 2006). MRI of the neck and shoulder
may show denervation of affected muscles with
ndings of intramuscular edema, muscular atro-
phy, and fatty inltration. The most commonly
affected muscles seen on MRI are the
supraspinatus and the infraspinatus. Though it is
not completely dened, in the acute phase of
denervation, there may not be positive ndings
on MRI. The rst sign may be diffuse increased
signal related to edema seen on T2-weighted
images of involved musculature (Scalf
et al. 2007).
Diagnostic criteria for TM have been dened
by the TMCWG as described previously and
include imaging, CSF studies, and serological
studies. Serum evaluation includes mycoplasma
antibodies, West Nile Virus titers, Bartonella
henselae titers, and Lyme titers to evaluate for
infection. Rheumatological blood work should
include rheumatoid factor, antinuclear, anti-
double-stranded DNA, anti-single-stranded
DNA, anti-RNP, anti-smooth muscle, anti-SSA,
anti-SSB, and antiphospholipid antibodies to
evaluate for autoimmune disorders. Aquaporin-4
antibodies or NMO IgG should be evaluated for
the possibility of NMO-associated myelitis. CSF
studies should demonstrate pleocytosis or
increased IgG. CSF cultures should be sent to
rule out for infectious myelitis (Wolf et al. 2012).
MRI of the spine demonstrates pathology in
78 % of cases of clinical TM with lesions most
A. Belzberg et al.
cord from C3 through C7. (b) Involvement of the ventral
spinal cord and thus the alpha motor neuron (Reproduced
with permission from Sadowsky et al. (2011))
commonly in the cervicothoracic region of the
spinal cord (Fig. 6). Some patients have
multifocal lesions, though more commonly they
are singular lesions. Two thirds of lesions
involve the gray matter only and one third both
gray and white matter. The majority of
lesions span three or more segments of the spinal
cord, and in the acute phase, 19.162.5 % enhance
with gadolinium (Alper et al. 2011; Sellner
et al. 2009).
Electrodiagnostic studies such as nerve con-
duction studies (NCS) and electromyography
(EMG) may be useful in diagnosing NA, when
performed 1014 days after onset of symptoms to
avoid false negatives. NCS may show normal to
slightly prolonged conduction velocities with or
without decreased amplitude of the sensory nerve
action potentials (SNAP) and compound motor
action potentials (CMAP). EMG may show axo-
nal loss secondary to acute denervation with bril-
lations, positive sharp waves, and polyphasic
high-amplitude motor unit action potentials
(MUAP). There are rare instances, however, that
demonstrate a pattern of demyelination instead of
axonal loss (Vassallo et al. 2010). Since TM is
generally an UMN disease process, it usually
would not demonstrate any ndings on EMG.
However, in the subset of patients with accid
monoplegia resulting from damage to the alpha
motor neuron, NCS/EMG may demonstrate a
severe motor neuronopathy with acute and
chronic denervation ndings (Sadowsky
et al. 2011).
32 Transverse Myelitis and Neuralgic Amyotrophy
Associated Injuries
The initial pain associated with NA infrequently
persists, but a small subset of children may go on
to develop a chronic pain syndrome with either
neuropathic or musculoskeletal-type pain or a
combination of the two. The musculoskeletal
pain may be related to the atrophy and resultant
compensatory mechanisms and poor body
mechanics necessary for the child to function as
normally as possible (van Alfen 2007). The neu-
ropathic pain may be a continuation of the
pain at initial onset. In HNA, there is a subset
of patients that have a relapsing and remitting
disease course with exacerbations every 6
years resulting in pain and musculoskeletal
dysfunction.
TM-associated injuries in the acute phase of
the disease process may include loss of upper
airway patency and dysphagia secondary to cra-
nial nerve IX (glossopharyngeal nerve) involve-
ment in the innervation of the pharyngeal
muscles. Dysphagia may also be related to longi-
tudinally extensive lesions with extension into the
brainstem. If the spinal lesion is above C5, weak-
ness of the diaphragm may ensue, leading to
respiratory difculty. In the longer term, if the
lesion is above T6, there is a risk of autonomic
dysreexia (AD), which is a sympathetic dis-
charge resulting from a noxious stimuli below
the level of the injury (such as distended bladder
or stool impaction) and resulting in hypertension,
sweating, headache, goose bumps, and ushing.
This is a medical emergency and is treated by
removing the noxious stimulus and, if no
improvement, lowering the blood pressure with
pharmacological agents per published guidelines
(Consortium for Spinal Cord Medicine 2011;
Wolf et al. 2012).
Classification
NA is divided into two distinct categories: idio-
pathic and hereditary neuralgic amyotrophy. INA,
the more common of the two, presents, as men-
tioned above, with severe pain usually in the
shoulder followed by muscle paresis, atrophy,
721
and sensory decits. This is a singular episode
that resolves over the course of months to years.
HNA, an autosomal dominant form of NA
linked to a mutation in the SEPT9 gene on chro-
mosome 17q25, is set apart by its recurring attacks
of the characteristic acute severe pain. This is also
followed by muscle weakness, atrophy, and sen-
sory decits. It predominantly involves the bra-
chial plexus but may also affect the lumbosacral
plexus, cranial nerves, phrenic nerve, recurrent
laryngeal nerve, and the autonomic nervous sys-
tem or a combination. Any further differences are
discussed throughout the chapter.
TM is classied in a number of ways. It is
classied as disease-associated transverse myeli-
tis when found in conjunction with signs and
symptoms of MS, ADEM, NMO, and rheumato-
logic conditions such as SLE or antiphospholipid
antibody syndrome. Idiopathic TM is a diagnosis
of exclusion and accounts for the majority of
pediatric cases of TM (Wolf et al. 2012).
It can be further classied as acute partial TM
(APTM), acute complete TM (ACTM), and lon-
gitudinally extensive TM (LETM), based on MRI
ndings. APTM is dened by lesions of the spinal
cord on MRI that are asymmetric, patchy, and
span fewer than two vertebral segments with
resultant mild to moderate weakness, asymmetric
sensory loss, and possible bladder involvement.
These patients may go on to develop MS with
multiple lesions separated over time and space
throughout the central nervous system. ACTM
results in moderate to severe symmetric sensory
and motor decits secondary to lesions that span
the spinal cord. LETM is dened as a longitudi-
nally extensive lesion that spans at least three
vertebral segments. These patients, though at
very low risk for developing MS, have a high
possibility of progressing to NMO. Often the
rst lesion in NMO is a longitudinally extensive
spinal cord lesion; thus aquaporin-4 (or NMO
IgG) antibodies are important diagnostic tools in
these patients. Relapse of TM may be more com-
mon in APTM compared to ACTM as well as in
LETM with a diagnosis of NMO. Recurring TM is
very rare. Therefore patients should have a
reevaluation by an experienced neurologist to
assess for possibility of additional progression or
722 A. Belzberg et al.

Table 3 Upper extremity functional tests

Test Population Characteristics Study
SHUEE Congenital Video-based, evaluates spontaneous and on-demand activity Davids
hemiplegia et al. (2006)
MUUL Congenital Evaluates range of motion, target accuracy, uency and quality of Spirtos
hemiplegia reaching, grasping, pointing, manipulating, and releasing et al. (2011)
QUEST Congenital Evaluates dissociated movement, grasp, protective extension, and Thorley
hemiplegia weight-bearing et al. (2012)
AMS Brachial plexus Measure of joint movement in gravity-eliminated and against-gravity Curtis
birth palsy positions et al. (2002)
BBT Congenital Patient moves blocks from one side of box, over a divider to the other Sung
hemiplegia side while measuring the number of blocks lifted, carried, and released et al. (2005)
Brachial plexus in one minute Mulcahey
birth palsy et al. (2012)
These tests are used to evaluate the pediatric upper extremity function using a combination of range of motion, exercises
and functional tasks

diagnosis of MS, since this may impact function
and prognostication (Borchers and Gershwin
2012).
Outcome Tools
The Functional Independence Measure in Chil-
dren (WeeFIM ) and the Modied Rankin Scale
(MRS) are general functional measures used in
children to determine the level of independence
in daily life activities. The WeeFIM measures
self-care (eating, grooming, bathing and dress-
ing), sphincter control (toileting, bowel and blad-
der management), transfers (chair/wheelchair,
toilet, tub/shower), locomotion (walk, wheelchair,
crawl, stairs), communication (comprehension,
expression), and social cognition (social interac-
tion, problem solving, memory). WeeFIM levels
range from complete independence all the way to
total assistance on a seven-point scale. This is
used in children with NA and TM to determine
their general level of function as it pertains to their
daily life (Msall et al. 1994). The MRS, developed
for adults, may also be used as a general measure
of function and ranges from 0 no symptoms at
all; 1 no signicant disability despite symp-
toms, able to carry out all usual duties and activ-
ities; 2 slight disability, unable to carry out all
previous activities, but able to look after own
affairs without assistance; 3 moderate
disability, requiring some help, but able to walk
without assistance; 4 moderately severe disabil-
ity, unable to walk without assistance and unable
to attend to own bodily needs without assistance;
5 severe disability, bedridden, incontinent, and
requiring constant nursing care and attention; and
6 dead.
Measures of upper extremity function include
the Shriners Hospital for Children Upper Extrem-
ity Evaluation (SHUEE), the Melbourne Assess-
ment of Unilateral Upper Limb Function
(MUUL), the Quality of Upper Extremity Skills
Test (QUEST), the Active Movement Scale
(AMS), and the Box and Block Test (BBT). See
Table 3 and chapter Outcome Measures for
details on these functional measures.
Treatment Options
Nonoperative Management
Only anecdotal evidence for the treatment of NA
exists (Fig. 7). Nonoperative treatment measures
include high dose systemic corticosteroids, intra-
venous immunoglobulin (IVIG), and medical
management of pain (Moriguchi et al. 2011; van
Eijk et al. 2009; van Alfen 2009b). Systemic
corticosteroids and IVIG have demonstrated
some slowing of disease progression and
improvement in symptoms used either separately
32 Transverse Myelitis and Neuralgic Amyotrophy 723

Acute illness

TM, NA

Steroids

Upper Extremity
Improvement in signs
and symptoms
No improvement in
signs and symptoms
Dysfunction
NA TM

IVIG PLEX

Improvement in signs No improvement in

and symptoms signs and symptoms

TM, NA

Rehabilitation
Measures

3 month follow up

Improvement in signs No improvement in

and symptoms signs and symptoms

TM
MRI NA

Enhancement No enhancement

Rehabilitation
Further Neurology
measures
consultation for
steroids/PLEX/
immunosuppressants

6 month follow up

Improvement in signs No improvement in

and symptoms signs and symptoms

Rehabilitation Surgical
measures considerations

Fig. 7 Decision tree for the management of upper extremity dysfunction after transverse myelitis or neuralgic
amyotrophy

or together, but only in case reports and case series treated with gabapentin, carbamazepine, or
(Naito et al. 2012). Pain management acutely with amitriptyline.
a long-acting nonsteroidal anti-inammatory drug The American Academy of Neurologys
(NSAID) and a sustained release opiate are appro- evidence-based guideline for clinical evaluation
priate. During the subacute and chronic phases of and treatment of TM is the standard of practice.
treatment, persistent neuropathic pain may be Though there is insufcient evidence to support
724 A. Belzberg et al.

the use of corticosteroids, the rst-line treatment in which there is a team of physicians, nurses, and
of TM is high-dose methylprednisolone for 37 therapists working together towards the common
days. Intravenous methylprednisolone is started at goals of improvement in function for the patient.
30 mg/kg/day with a maximum dose of 1 g/day This team includes the physiatrist (rehabilitation
followed by a taper of 1 mg/kg/day over the physician), other medical specialists (such as a
course of 34 weeks (Scott et al. 2011). If no neurologist), rehabilitation nursing, physical ther-
improvement, or clinical worsening over apist, and occupational therapist. Other members
2448 h after initiating steroid treatment, general may include a behavioral psychologist and/or
practice will warrant consideration of plasmaphe- neuropsychologist. Rehabilitation begins in the
resis treatment. Plasmapheresis may be effective acute period of NA and TM with pain control
in patients with demyelinating diseases who have through the use of modalities and other techniques
failed treatment with high-dose corticosteroids and continues on through the progression of the
(Scott et al. 2011). Studies of immunosuppressive disease as the child develops muscle weakness
agents such as rituximab have shown the possi- and atrophy leading to disability. The goal of
bility of decreasing TM attacks. Use of antineo- rehabilitation as the disease progresses is to
plastic agents such as mitoxantrone has improve function in order to promote indepen-
insufcient evidence in decreasing attacks of dence and minimize medical and physical com-
TM. Other agents reported in the literature with plications which could be associated with the
insufcient evidence for efcacy include azathio- disease process. See Table 4 for the rehabilitation
prine, cyclophosphamide, and intravenous immu- team composition and breakdown of
noglobulin (IVIG) (Scott et al. 2011). responsibilities.
Occupational therapists play a pivotal role in
the rehabilitation of children with NA and TM
Indications/Contraindications
Table 4 Rehabilitation NA
Considering that there is such little literature to
support the use of the aforementioned in treatment Discipline Treatment
of NA, there are no specic indications or contra- Physiatrist Coordination of rehabilitation
needs
indications at this time. Further research must be
Neurologist Assistance in work-up and
completed. management of possible
Indications for the use of high-dose corticoste- inammatory components
roids in the treatment of TM include diagnosis leading to disability and
changes in function
based on the diagnostic criteria laid out by the
Occupational therapist Range of motion, stretching,
TMCWG. If there is no clinical improvement, or splinting, strengthening,
if there is clinical worsening 2448 h after initiat- NMES, adaptive equipment,
ing steroid treatment, this is an indication to treat desensitization, contrast
with plasmapheresis, though there is little evi- baths, ultrasound
dence to support its effectiveness (Scott Physical therapist Adaptive equipment,
functional mobility
et al. 2011). The use of immunosuppressants is evaluation, mechanics of
not dened as there is insufcient evidence to ambulation, and functional
support their efcacy and use in the treatment of mobility
TM (Scott et al. 2011). Behavioral Biofeedback, focused
psychology/ imagery, cognitive behavioral
neuropsychology therapy. Cognitive assessment
Rehabilitation nursing Coordination of basic medical
Rehabilitation Techniques needs
The rehabilitation team consists of a number of clinicians
The general rehabilitation approach for patients who all take part in treatment of the patient throughout their
with NA and TM is an interdisciplinary approach rehabilitation and with the common goals of the patient
32 Transverse Myelitis and Neuralgic Amyotrophy
with upper extremity involvement. In the acute
period, use of desensitization, contrast baths,
focused imagery, and cognitive behavioral ther-
apy may help with neuropathic pain. Supercial
as well as deep heat with ultrasound may help
with musculoskeletal pain. Once the child has
developed weakness and atrophy of the muscula-
ture, range of motion of the shoulder, stretching
and splinting are important to prevent joint con-
tractures. To improve weakness directly, use of
strengthening exercises and neuromuscular elec-
trical stimulation (NMES) may be useful. NMES
may be valuable, not only for active range of
motion but also for assisting in muscle contraction
and retraining. Biofeedback throughout the reha-
bilitation process may improve awareness of the
ongoing physiologic processes (Ramos and Zell
2000)
From a functional standpoint, the occupa-
tional therapist and physical therapist may per-
form an evaluation for adaptive equipment. In
those children with distal upper extremity weak-
ness, this includes a universal cuff, which uten-
sils can be attached to if the child has poor grip
strength. Foam tubing can be used on writing
and eating utensils to achieve easier grasp in
patients who have more but still limited grip
strength. Poor proximal upper extremity
strength may lead to difculties with ADLs.
Useful adaptive equipment including a reacher,
dressing stick, sock aide, long-handled sponge,
or shoe horn may be utilized to maximize inde-
pendence in daily activities. This may improve
the childs independence and may provide the
opportunity to perform age-appropriate activi-
ties such as feeding, grooming, bathing, dress-
ing, and school activities.
In addition to the equipment evaluation, the
physical therapist focuses on functional mobility
and proper body mechanics. This is very impor-
tant to prevent associated injuries to other muscles
because of compensatory techniques. This begins
with an evaluation of general mobility and gait
with a focus on body mechanics. This is also quite
important in the TM patient with motor decits in
the lower extremities for strengthening and
stretching to maintain range of motion as well as
gait training.
725
Outcomes
Outcomes of nonoperative treatment of NA
using measures of medical stability, function,
level of pain, or quality of life are not available.
The general trend of recovery in NA is 63 % of
children with full recovery, 25 % with
partial recovery, and 13 % that do not recover
(Host and Skov 2010). The aforementioned
upper extremity functional measures are used in
children with upper extremity dysfunction
secondary to cerebral palsy or brachial plexus
birth palsy, though they can be extrapolated to
be used in children with lower motor neuron
upper extremity dysfunction secondary to
NA. There is no data available in this population
of children with NA using these functional
measures.
Prognosis in TM generally follows the rule of
thirds, which states that approximately one third
of patients have a good outcome, one third have a
fair outcome, and one third have a poor outcome.
In children, complete recovery is achieved in
3350 % with poor outcomes in 1020 % of
cases (Wolf et al. 2012). Treatment outcome data
is not sufcient in children to make strong con-
clusions, but oral steroids have been shown to
result in better functional mobility outcomes. At
the same time, however, some patients who did
not receive treatment had better functional out-
comes for ADLs and functional mobility than
those who did undergo treatment. At follow up,
54 % of patients still experience dysesthesias and
75 % numbness. Urinary retention persists in
50 % of children at follow up, urgency in 68 %.
With regard to motor function, 52 % of children
who were non-ambulatory at the nadir of their
clinical course were able to ambulate with or
without an assistive device (Pidcock et al. 2007).
Overall, better outcomes are seen in children with
normal CSF white blood cells (WBC), diagnosis
within 7 days of the onset of symptoms, and lower
level of injury in the spinal cord. Poorer outcomes
are associated with age less than 3 years and
greater extent of lesion of the spinal cord (Pidcock
et al. 2007).
In our experience children with alpha motor
neuron involvement resulting in accid
726
monoplegia have minimal improvement of the
affected limb, despite aggressive nonoperative
management. Quick onset of muscle atrophy and
loss of bone mass is observed within the rst
month. In the growing child, similar to long-term
effects from poliomyelitis infection, this may
result in limb length discrepancy, osteoporosis
with high risk for fractures, high risk for neuro-
genic arthropathy (often seen in patients with
lower limb involvement), overuse of stronger
musculature, and muscular strain with
chronic pain.
Operative Treatment of TM and NA
A small subset of patients with TM and NA will
have permanent focal neurological dysfunction
due to injury within the spinal cord. When the
anterior horn is involved and there is loss of ante-
rior horn motor neurons, the resultant injury is a
LMN decit with extensive denervation of the
target muscle. There is severe atrophy and, given
the loss of the motor neuron cell body, no possi-
bility for nerve regeneration. Peripheral nerve sur-
geons encounter a similar scenario with traumatic
avulsion of nerves including elements of the bra-
chial plexus.
Novel therapeutic approaches are needed to
restore function in those patients facing permanent
disabilities. Traditionally, the nerve surgeon iso-
lates the site of injury, surgically resects the injured
segment, and restores continuity by end-to-end
repair or insertion of a nerve graft. If the point of
injury is in the spinal cord, such as seen in TM, at
the level of the motor neuron cell body, there are no
axons available to reinnervate the distal nerve with.
For NA, the injury can occur anywhere along the
peripheral nerve, and it may not be possible to
localize the injury to a particular segment.
Nerve transfers have demonstrated efcacy for
restoring function in the extremities after periph-
eral nerve injury (Pindrik et al. 2013). Nerve
transfers may be an option to restore function in
select patients who have isolated LMN decits
including those with TM and NA.
The aim of nerve transfer is to re-energize the
injured target nerve with axons from a
A. Belzberg et al.
Table 5 Preoperative planning for ulnar to biceps nerve
transfer
Ulnar to biceps nerve transfer
Preoperative planning
OR table: regular
Position/positioning aids: standard, prep of full
extremity
Fluoroscopy location: none
Equipment: Nerve stimulator, microscope,
microsurgical instrumentation, brin glue
Tourniquet: not used
functioning donor nerve. The recipient nerve
has crucial target muscle versus the donor nerve,
and there is also redundancy in the innervation of
the donor nerve muscle(s). The surgeon is rob-
bing Peter to pay Paul. In appropriately selected
cases, where there is sufcient redundancy that
there is no recognizable decit from taking the
donor fascicles, one may also transfer a single
fascicle rather than the entire nerve. The most
commonly performed nerve transfers include the
Oberlin procedure, that is, the transfer of an ulnar
nerve fascicle to the biceps branch of the
musculocutaneous nerve (Teboul et al. 2004).
Another nerve transfer technique involves using
a triceps nerve branch as a donor to the axillary
nerve for restoration of elbow exion and shoul-
der abduction (Bertelli et al. 2007). The preoper-
ative planning, surgical steps, postoperative
protocol, and potential pitfalls and prevention
are shown respectively in Tables 5, 6, 7, and 8).
See chapters Microsurgery for Obstetrical
Brachial Plexus Palsy and Traumatic Bra-
chial Plexus Injury in the Pediatric
Population for details of nerve transfers.
The advantages of distal fascicle transfers
include: nerve regeneration is closer to the target
muscle, improved direction of motor axons to the
target muscle, and direct transfer without the use
of interpositional nerve grafts. In comparison to
nerve repair at the point of proximal injury, the
point of coaptation in nerve transfer is usually
closer to the target muscle, and the expected
time for axonal regeneration to the motor endplate
is less. Distal transfers are also advantageous in
cases with a prolonged interval between injury
and surgery or when the level of injury is proximal
32 Transverse Myelitis and Neuralgic Amyotrophy 727

along the nerve or in the spinal cord (Dorsi and
Belzberg 2012).
The use of nerve transfer to manage patients
with TM was recently reported (Dorsi and
Belzberg 2012). The patient had recovered median
and ulnar function but lacked elbow exion and
Table 6 Surgical steps for ulnar to biceps nerve transfer
Ulnar to biceps nerve transfer
Surgical steps
Linear incision along the medial aspect of the arm
Identify the median, ulnar and musculocutaneous nerves
Isolate the biceps innervation and gain length with
internal neurolysis of the musculocutaneous nerve
Internal neurolysis of the ulnar nerve and identify a
fascicle that on stimulation activates exor carpi ulnaris
Divide the proximal portion of biceps innervation and
rotate distal portion towards the ulnar nerve
Divide the ulnar nerve fascicle to exor carpi ulnaris and
rotate the proximal portion towards the biceps nerve
Perform an end to end coaptation of the distal biceps
innervation to the proximal divided ulnar fascicle and
secure with brin glue
Close the wound
Table 7 Postoperative protocol for ulnar to biceps nerve
transfer
Ulnar to biceps nerve transfer
Postoperative protocol
Suction drains are rarely required
Arm immobilized at elbow for 34 weeks
Gentle range of motion exercises
Reeducation instituted once reinnervation evident
shoulder abduction. Transfers included ulnar and
median fascicle to biceps nerve and brachialis
nerve, respectively. In addition, the spinal acces-
sory nerve was transferred to the suprascapular
nerve. There was excellent recovery of elbow ex-
ion and less about the shoulder girdle.
Shorter durations of time between nerve injury
and surgery offer better chances for regeneration
and recovery of useful function (Pindrik
et al. 2013). Prolonged delay between the inciting
event and surgical intervention may result in
severe muscle atrophy and loss of support cells
in the distal nerve and/or muscle. Muscle bers
atrophy and scar 1218 months following dener-
vation and vacant endoneurial tubes degenerate
1824 months after Wallerian degeneration.
Prolonged denervation can prevent functional
recovery despite adequate nerve regeneration
across the area of injury following nerve transfer.
Persistent neurological decits amenable to
surgery will do best with the procedure performed
36 months after injury. Nerve transfers should be
attempted within 6 months after injury, or disease
onset, to offer the best chance of successful mus-
cle reinnervation (Pindrik et al. 2013). Therefore,
an early decision needs to be made regarding
likelihood that spontaneous will or will not
occur. Early changes on EMG reecting nerve
regeneration and muscle reinnervation include
the presence of nascent potentials. Imaging stud-
ies can demonstrate denervated muscle but have
not been useful to demonstrate nerve regeneration
(Pindrik and Belzberg 2014). Unfortunately, there

Table 8 Potential pitfall and prevention for ulnar to biceps nerve transfer
Ulnar to biceps nerve transfer
Potential pitfalls and preventions
Potential pitfall
Pitfall #1 Stimulation of musculocutaneous
nerve produces biceps activation
Pitfall #2 Difculty nding the biceps
innervation
Pitfall #3 Failure of tension free end to end
coaptation
Pitfall #4 Difculty identifying fascicle to
exor carpi ulnaris
Pearls for prevention
Abort the procedure and inform patient regeneration is likely
occurring
Trace the musculocutaneous nerve towards the axilla as the takeoff
can be proximal. There can be an anomalous anatomy of the lateral
cord and median/musculocutaneous nerves
Gain length by continuing the internal neurolysis of biceps branch
into musculocutaneous nerve
Separate the ulnar fascicles and lower the stimulation settings. Use a
fascicle that has strong muscle contractions, minimal nger
contractions, and is located medial in the nerve
728
is no current clinical methodology to adequately
predict the functional outcome for a patient 68
months into the diagnosis with TM with persistent
profound decits. Decision making as to surgical
intervention for nerve repair has to be individual-
ized and should include a multidisciplinary team.
In contrast to nerve reconstruction, surgical con-
sideration for muscle or tendon transfers can be
delayed until neurological recovery has plateaued.
By transferring the tendon of a functional muscle to
an alternate insertion, one can impact function in a
very predictable manner. Primary surgery usually
refers to a nerve repair with secondary surgery
referring to tendon or muscle transfers as well as
joint manipulations. The topic of secondary sur-
gery is covered elsewhere in this textbook in chap-
ter Glenohumeral Joint Secondary Procedures
for Obstetrical Brachial Plexus Birth Palsy. For
patient with TM or NA, it is critical to maintain
adequate passive range joint range of motion
throughout the recovery process. Stiff joints or
shortened tendons can limit the recovery and
options for secondary surgery.
Management of Complications
Systemic corticosteroids, sometimes used to treat
NA and TM, may result in growth disturbance,
weight gain, hyperglycemia, hypertension, cata-
racts, avascular necrosis, and osteoporosis in chil-
dren (Table 9). These complications occur more
frequently with prolonged use. Avoidance mea-
sures include limiting the time on steroids to as
low dose as possible and tapering the dosage as
quickly as possible while still avoiding adrenal
insufciency. Steroid-induced myopathy is
another adverse reaction that may lead to proxi-
mal weakness and worsening of functional
impairments. Monitoring for these side effects to
prevent further complications, such as fragility
fractures associated with osteoporosis caused by
steroids, is important. The physician should mon-
itor blood sugars, weight, and vision throughout
the treatment course. Also, the physician should
monitor for signs of avascular necrosis, which is
most common in the hip diagnosed by pain and
difculty walking (Kliegman et al. 2011).
A. Belzberg et al.
IVIG adverse effects include fever, headache,
myalgia, nausea, and vomiting, which may be
remedied by decreasing the rate of infusion.
More serious reactions include anaphylaxis, throm-
boembolic disorders, renal insufciency, and asep-
tic meningitis, which have been described in
patients with NA and TM being treated with
IVIG. This is managed by discontinuation of the
IVIG infusion (Kliegman et al. 2011).
Plasmapheresis complications include general
side effects such as paresthesias, muscle cramps,
nausea, vomiting, urticaria, and pruritus. More
severe complications include hypotension, bron-
chospasm, transfusion-related acute lung injury,
hypocalcemia, metabolic alkalosis, and coagula-
tion abnormalities. It may also deplete the childs
immunoglobulins putting them at substantial risk
for infection. Management of these complications
includes symptomatic treatment for the less severe
problems and discontinuing the treatment in the
more severe reactions such as the transfusion-
related acute lung injury (Kaplan and
Fridey 2012).
Immunosuppressants such as azathioprine can
cause rash, stomatitis, gastrointestinal distur-
bances, alopecia, and arthralgias, which may be
treated symptomatically. More severe complica-
tions include bone marrow suppression and hepa-
totoxicity. Suppression of the immune system
places the child at risk for infection. Antineoplas-
tic agents used include cyclophosphamide,
mitoxantrone, and rituximab. Used in the acute
phase of treatment, cyclophosphamide may cause
leukopenia and cardiomyopathy, which require
either adjustment of dose or discontinuation of
treatment. Other side effects include hemorrhagic
cystitis, nausea, and vomiting. In the long-term it
may lead to infertility, cardiomyopathy, secondary
malignancy, and leukoencephalopathy. The
milder side effects may be treated symptomati-
cally. The longer-term side effects limit the course
of cyclophosphamide use (Custer and Rau 2009).
Rehabilitative interventions may also result in
complications. These include pain, fragility frac-
tures, and tendinopathies. Pain is a prominent
feature of both TM and NA in the acute period,
but also may result from musculoskeletal imbal-
ance and poor biomechanics related to the more
32 Transverse Myelitis and Neuralgic Amyotrophy 729

Table 9 Management of complications of treatment

Treatment Common complications Management
IVIG Anaphylaxis, thromboembolic disorders, renal Stop the infusion
insufciency, and aseptic meningitis
Fever, headache, myalgia, nausea, and Slow the infusion
vomiting
Steroids Growth disturbance, weight gain, Taper
hyperglycemia, hypertension, cataracts,
avascular necrosis, and osteoporosis
Plasmapheresis Paresthesias, muscle cramps, nausea, Treat symptomatically
vomiting, urticaria and pruritus, infection
Hypotension, bronchospasm, transfusion- Stop treatment, if severe
related acute lung injury, hypocalcemia,
metabolic alkalosis, coagulation abnormalities
Cyclophosphamide Hemorrhagic cystitis, nausea, and vomiting Symptomatic treatment
Leukopenia and cardiomyopathy Decrease dosage or discontinue treatment
Long term includes infertility, Surveillance
cardiomyopathy, secondary malignancy, and
leukoencephalopathy
Rehabilitation Pain Ice, heat, ultrasound, desensitization,
antiepileptics, tricyclic antidepressants,
NSAIDs
Fragility fracture Evaluate bone density prior to aggressive
rehabilitative interventions. With occurrence,
stop therapies and consult orthopedics
Tendinopathies Decrease intensity of therapy, symptom
management with thermal modalities
Surgery Infection Culture the wound and blood for appropriate
use of antibiotics
Follow blood indicators ESR and C-reactive
protein
Pain Aggressive treatment of postoperative pain
Multidisciplinary approach to neuropathic
pain
Delayed presentation Very distal nerve transfer or use of free muscle
ap (gracilis) with nerve transfer
Treatments of TM and NA come along with complications, though some may be treatable whereas others may result in
discontinuation of treatment

subacute weakness and muscle atrophy.
Musculoskeletal-type pain may be treated with
NSAIDs and ice in the acute period. In the more
chronic period, supercial heat and deep heat with
ultrasound may be useful for pain management.
For painful dysesthesias, one can consider use of
neuropathic agents such as gabapentin or a tricy-
clic antidepressant such as amitriptyline. If the
pain is severe enough and/or chronic, one may
consider the use of opioids or more advanced
pain management strategies, though this does not
treat the source of the pain.
Fragility fractures are associated with disuse
osteoporosis, which is a prominent feature of
both NA and TM. It has not specically been
documented as a complication of rehabilitation
in NA, but in children with cerebral palsy and
with TM, the muscle disuse and reduced muscle
load on the bone contribute to fracture risk
(Sadowsky et al. 2011; Huh and Gordon 2013).
Other predisposing factors include the use of ste-
roids as glucocorticoids promote bone resorption
and reduce bone formation through both hor-
monal and cellular mechanisms. They also inhibit
730
intestinal calcium absorption and increase renal
calcium loss. Other medications that may be used
in the treatment of these patients which may con-
tribute to bone loss and fractures are immunosup-
pressants. First and foremost, optimal bone health
is highly recommended in this patient population.
Hydroxyvitamin D levels as well as calcium
intake should be assessed and deciency should
be treated with replacement doses of vitamin D3
and calcium, respectively. Patients may benet
from evaluation of bone mass with dual-energy
X-ray absorptiometry (DEXA) scan prior to
aggressive rehabilitation measures. If ndings
are consistent with osteoporosis or severe
osteopenia, the patient should be referred for
endocrinological evaluation regarding manage-
ment with possible bisphosphonates to maximize
bone density. If the child has a fragility fracture,
rehabilitative measures should be held until ortho-
pedic evaluation.
Overuse injuries such as tendinopathies may
occur during the course of rehabilitation, espe-
cially with the repetitive tasks that are performed
in strengthening exercises. Though previously
termed tendonitis, there is little to no inamma-
tion, and thus anti-inammatory medications are
not appropriate treatment. Treatment would
include a decrease in the intensity of therapy and
avoiding certain activities that are painful for the
child while maintaining range of motion and
preventing contractures. Symptomatic treatment
includes ice in the acute period followed by heat.
Summary
NA and TM, both thought to be autoimmune and
inammatory in nature, may result in upper
extremity monoplegia in children. NA, most
often affecting the brachial plexus, results in a
LMN syndrome and accidity. TM, on the other
hand, may lead to spastic or accid monoplegia
depending on the site of the lesion. Both may be
diagnosed with MRI and serological studies with
diagnosis also supported by NCS and EMG. Ini-
tial treatment of TM is with high-dose corticoste-
roids and, if no improvement, with
plasmapheresis or even immunosuppressants or
A. Belzberg et al.
antineoplastic agents. Steroids may also be used
for the initial treatment of NA, with little evidence
for management with IVIG. Rehabilitation
includes ROM, stretching, strengthening, neuro-
muscular electrical stimulation, techniques for
pain management, education, and equipment.
Outcomes may be measured with the WeeFIM
and the MRS for general function and the
SHUEE, MUUL, QUEST, AMS, and BBT for
more focused upper extremity outcome measure-
ment. Sixty-three percent of children with NA
fully recover, while 3350 % of children with
TM do. There is minimal outcomes data in NA,
but children with TM are noted to have signicant
motor recovery with ongoing bowel and bladder
dysfunction, especially during the rst 3 months.
For children not showing the expected recovery,
the aggressive use of nerve transfers provides an
alternative approach by providing a source of
motor axons. This can be done in collaboration
with muscle or tendon transfers to maximize the
unction. Future research is needed for treatment
including medical management, surgical manage-
ment, and rehabilitation strategies. Knowing
which patient will have a poor prognosis for spon-
taneous recovery versus those who would benet
from early surgical intervention with nerve repair
and possible tendon transfers remains a challenge.
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 Part IX
Tetraplegia
 Upper Limb Reconstruction in Persons
with Tetraplegia 33
Upper Extremity Reconstruction for Tetraplegia

Scott H. Kozin, Dan A. Zlotolow, and Joshua M. Abzug

Contents Rehabilitation and Outcome . . . . . . . . . . . . . . . . . . . . . . . 764

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 736 Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 765
High-Level Tetraplegia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 737 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 765
Technique: Intercostal-to-Phrenic
Nerve Transfer . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 737
Incomplete Tetraplegia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 741
Mid-level Cervical Tetraplegia . . . . . . . . . . . . . . . . . . . . 741
Classication . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 741
Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 742
Elbow . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 742
Forearm . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 748
Rehabilitation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 752
Wrist and Hand . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 755

Electronic supplementary material: The online version

of this chapter (doi:10.1007/978-1-4614-8515-5_33)
contains supplementary material, which is available
to authorized users. Videos can also be accessed at
http://www.springerimages.com/videos/978-1-4614-8513-1.
S.H. Kozin (*) D.A. Zlotolow
Shriners Hospitals for Children, Philadelphia, PA, USA
e-mail: skozin@shrinenet.org; dzlotolow@yahoo.com
J.M. Abzug
University of Maryland School of Medicine, Baltimore,
MD, USA
e-mail: Jabzug@umoa.umm.edu

# Springer Science+Business Media New York 2015 735

J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_33
736
Abstract
Tetraplegia is challenging to treat and requires
a team of competent and caring physicians
and nurses. The initial goal is to facilitate the
transition from able-bodied life to a life com-
plicated by spinal cord injury. The issues are
enormous and the task seems overwhelming
to the patient. Through a series of small steps,
the patient is often able to make the change-
over. However, complications are endemic
including depression, spasticity, autonomic
dysreexia, urinary retention, and decubiti.
As part of the rehabilitation, an assessment
of the upper extremities is integral. Many
patients can benet from tendon transfers to
improve motion and function. The potential to
restore elbow extension, wrist extension,
pinch, and grasp is critical for independence
and spontaneity. This chapter will discuss the
evaluation, treatment regimen, and surgical
techniques applicable to the person with spi-
nal cord injury.
Introduction
Spinal cord injury is a forever life-altering event
that drastically impacts the person and all his or
his caregivers, families, and friends. Paraplegia
and tetraplegia are both dramatic events, how-
ever; the loss of upper extremity function in
tetraplegia adds another dimension of impair-
ment, frustration, and dependence. In fact, per-
sons with tetraplegia state that any upgrading in
their hand function would result in an important
or very important improvement in quality of life
comparable to enhanced bladder and bowel func-
tion (Snoek et al. 2004). Therefore, the upper
extremity surgeon plays a pivotal role in the life
of a person with tetraplegia.
The treatment of persons with tetraplegia
requires a competent team of physicians, nurses,
and therapists. The physician team typically
includes a physiatrist, psychologist, upper extrem-
ity surgeon, spine surgeon, urologist, and plastic
surgeon. Each physician has an important role, but
all must function as part of the team. The physiatrist
S.H. Kozin et al.
is primarily responsible for the medical needs of
the patient including managing the uctuations
in blood pressure, treating the inevitable spastic-
ity, assessing the nutritional status, and oversee-
ing the progress in therapy. The psychologist is
required to evaluate and to determine the emo-
tional state of the patient with regard to his or her
support structure and coping mechanisms. The
upper extremity surgeons role is to discuss the
possibility of upper extremity reconstruction.
The timing and algorithm will be discussed later;
however, early introduction of the concept of upper
extremity reconstruction is valuable (Curtin
et al. 2005). The spine surgeon is obligated to
determine the stability of the spine and to assess
whether additional decompression or stabilization
is required. The urologist is responsible for deter-
mining the appropriate bladder and bowel manage-
ment program and to assess the patient for possible
surgical intervention(s) that will ease these neces-
sary activities of daily living. The plastic surgeons
role is primarily as a consultant to address and
manage any decubiti that developed.
In addition to the physicians, there are many
other pivotal roles in the treatment of persons with
tetraplegia. The nurses must possess an expertise in
the care of these patients to prevent demoralizing
complications. The respiratory therapist must dili-
gently monitor tracheotomy and pulmonary care to
prevent devastating and life-threatening complica-
tions. The social worker must be actively involved
in the transition from able-bodied life to a life that
requires support and innumerable modications to
the persons surroundings.
Upper limb reconstruction in persons with tetraplegia
Nonoperative management
Indications Contraindications
All persons with None, except for a really sick
tetraplegia patient who is unable to tolerate
any treatment
Contracture
prevention
Joint mobilization
Patient and
caregiver
education
Blood pressure
contracture
(continued)
33 Upper Limb Reconstruction in Persons with Tetraplegia
Upper limb reconstruction in persons with tetraplegia
Nonoperative management
Indications Contraindications
Decubiti
prevention
Psychology
support
Upper limb reconstruction in persons with tetraplegia:
physical/occupational therapy recommendations
Maintain supple range of motion
Prevent or alleviate contracture
Mobilize the patient slowly with awareness of potential
hypotension and hypertension issues
Minimize possibility of decubiti in bed and in wheelchair
Ascertain appropriate wheelchair seating and propulsion
process
High-Level Tetraplegia
High-level tetraplegia results in many particular
impediments to improve upper limb function.
The main obstructions involve the dependence
upon the respirator and the shoulder girdle. The
phrenic nerve arises from C3, C4, and C5 that
keep the diaphragm alive. The loss of diaphrag-
matic function is devastating as the person is
totally dependent upon the respirator 247. Any
malfunction or mucous plug is a dire emergency,
and imminent death is a real possibility. In
fact, the life span in persons with high-level
tetraplegia is roughly a decade, and pulmonary
complications contribute to their shortened life
span. There has been a variety of surgical
procedures to negate respiratory dependency.
The two options are pacing the phrenic nerve
or nerve transfer to the phrenic nerve (Yang
et al. 2011; Tibballs 1991). Phrenic nerve pacing
involves a pacemaker that stimulates the nerve
via a nerve cuff (Fig. 1). If the diaphragm does
not respond to stimulation, a nerve transfer may
be performed. The transfer can be an active
motor nerve, such as the spinal accessory nerve.
In addition, the transfer can be a donor nerve that
can be stimulated, such as the intercostal nerve
(s) below the level of injury (Krieger and Krieger
2000; Tibballs 1991) (Fig. 2). This surgery is
737
relatively new and innovative; however, the ben-
ets can be life changing with lessened ventilator
dependence.
Technique: Intercostal-to-Phrenic
Nerve Transfer
Intercostal-to-phrenic nerve transfer
Preoperative planning
OR table: regular
Position/positioning aids: lateral decubitus, meticulous
padding of all bony prominences, thoracic surgeon
Fluoroscopy location: none
Equipment: standard, chest tube, phrenic nerve
stimulator
Tourniquet: none
The patient is placed in lateral decubitus posi-
tion with the affected side up. A posterolateral
thoracotomy is performed at the 56 interspace
(Fig. 2). The lung is retracted to expose the
phrenic nerve, which is identied and stimulated
to conrm diaphragmatic paralysis. The phrenic
nerve is transected 5 cm proximal to its insertion
into the diaphragm (Fig. 3). The intercostal nerve
from the 56 interspace is identied from the
posterior axillary fold to the costochondral junc-
tion (Fig. 4). The nerve is cut at the costochondral
junction (Fig. 5). A 5 mm cuff of surrounding
muscular tissue is preserved around the nerve to
maintain its vascularity. The intercostal nerve is
directly repaired to the phrenic nerve without
tension (Fig. 6). The coaptation is performed
with sutures and/or brin glue.
The phrenic pacer electrode is then placed
around the phrenic nerve 1 cm distal to the coapta-
tion site (Fig. 7). The pacer line is tunneled to the
pacemakers receiver, which is implanted on the
anterior aspect of the chest wall or abdomen. A
chest tube is inserted followed by standard closure.
Following the surgery, the patient is admitted
to the intensive care unit for routine postoperative
care. The pacemaker is activated 3 months after
surgery, and the diaphragm is assessed for respon-
siveness. If the diaphragm is unresponsive, the
patient is returned to mechanical ventilation and
the diaphragm is reassessed at weekly intervals.
Once the diaphragm is found to be reactive, a
738
Fig. 1 A 6-year-old girl with a high-level tetraplegia,
ventilator dependency, and no function below the neck.
Electrodiagnostic studies found the phrenic nerve respon-
sive to stimulation and she was deemed a candidate for
phrenic nerve pacing (Courtesy of Shriners Hospital for
Children, Philadelphia). (a) Patient positioned supine on
pacing schedule is instituted. The duration of
pacing is increased as the parameters improve
(tidal volume, end tidal CO2, and patient comfort).
Intercostal-to-phrenic nerve transfer
Surgical steps
Posterolateral thoracotomy is performed at the 56
interspace
Phrenic nerve identied and traced toward the
diaphragm
Phrenic nerve transected 5 cm proximal to its insertion
into the diaphragm
Intercostal nerve with cuff of muscle isolated from
costochondral junction to posterior axillary fold
Intercostal nerve cut at the costochondral junction and
repaired end to end to the phrenic nerve
Phrenic pacer electrode is then placed around the
phrenic nerve 1 cm distal to the repair site
(continued)
S.H. Kozin et al.
operating table. Neck incision is for phrenic nerve isolation
and abdominal incision is for pacemaker insertion. (b)
Isolation of phrenic nerve isolation and electrode wrapped
around nerve. (c) Electrode secured around phrenic nerve.
(d) Pacemaker placed in abdominal pocket
Intercostal-to-phrenic nerve transfer
Pacer line is tunneled to the pacemakers receiver,
which is implanted on the anterior aspect of the chest wall
or abdomen
Chest tube and closure
Intercostal-to-phrenic nerve transfer
Postoperative protocol
Intensive care unit for routine postoperative care
Pacemaker is activated 3 months after surgery and the
diaphragm is assessed for responsiveness
Weekly assessments of the diaphragms
responsiveness
Once the diaphragm is found to be reactive, a pacing
schedule is instituted
Duration of pacing increased as parameters improve
(tidal volume, end tidal CO2, and patient comfort)
33 Upper Limb Reconstruction in Persons with Tetraplegia
Shoulder Girdle
The shoulder girdle instability plagues those
persons with high-level tetraplegia. The scapulo-
thoracic and glenohumeral joints lack stability
and motion. The loss of the parascapular stabi-
lizers including the rhomboids leads to fragrant
instability of the scapulothoracic joint that is
particularly problematic (Fig. 8). Decubiti can
develop between the scapula and wheelchair.
In addition, the total loss of proximal stability
Fig. 2 A 7-year-old girl with high-level tetraplegia and
ventilator dependency. Left thoracotomy performed for
exposure of phrenic nerve and harvest of intercostal nerve
(Courtesy of Shriners Hospital for Children, Philadelphia)
Fig. 3 Phrenic nerve
isolated and transected 5 cm
proximal to diaphragm
(Courtesy of Shriners
Hospital for Children,
Philadelphia)
739
negates any ability to position the hand in space.
Attempts to minimize the scapulothoracic joint
instability have resulted in mediocre results and
a fairly high complication rate (Pahys et al. 2009).
The unstable glenohumeral joint is yet another
obstacle for persons with high-level tetraplegia.
The lack of rotator cuff musculature and deltoid
function results in instability of the glenohumeral
joint with subluxation and possible pain. The sur-
gical options to restore stability are limited. The
absence of parascapular stabilizers negates the
possibility of shoulder arthrodesis as this proce-
dure would result in further scapulothoracic
instability. In addition, the only available donor
muscle for transfer is the trapezius and levator
scapula. In this scenario, the trapezius is trans-
ferred to the humeral head and the levator scap-
ula to the supraspinatus. This procedure will
lessen the humeral head subluxation but result
in minimal active shoulder motion. The best
treatment algorithm for this difcult problem
remains unsolved.
Functional Electrical Stimulation
Functional electrical stimulation (FES) for per-
sons with tetraplegia was here and gone. The sys-
tem provided substantial improvements for those
adults and children with high-level tetraplegia. FES
involved coordinated stimulation of innervated
muscles below the level of injury to result in
740
Fig. 4 Intercostal nerve isolated from costochondral junc-
tion to posterior axially fold with cuff of muscle (Courtesy
of Shriners Hospital for Children, Philadelphia)
Fig. 5 Intercostal nerve cut at costochondral junction
(Courtesy of Shriners Hospital for Children, Philadelphia)
S.H. Kozin et al.
Fig. 6 Intercostal and phrenic nerve repaired end to end
with brin glue (Courtesy of Shriners Hospital for Chil-
dren, Philadelphia)
Fig. 7 Stimulator electrode wrapped around the phrenic
nerve (Courtesy of Shriners Hospital for Children,
Philadelphia)
prehension. Lateral pinch and grasp were attainable
goals that resulted in an increase in function and
less dependence on caregivers (Kilgore et al. 1997).
For a variety of reasons, FES became unavailable
despite the initial promising reports. There are
substantiated rumors that a newer system will be
released with improved technology and more ver-
satility than the older system. Many persons with
33 Upper Limb Reconstruction in Persons with Tetraplegia
Fig. 8 High-level
tetraplegia with profound
instability of the
scapulothoracic joint
(Courtesy of Shriners
Hospital for Children,
Philadelphia)
high-level tetraplegia anxiously await the release
of such a device to improve function and enhance
independence in daily life.
Incomplete Tetraplegia
There are a variety of etiologies for incomplete
tetraplegia including trauma, transverse myelitis,
infection, and tumors. Incomplete tetraplegia typ-
ically results in an asymmetric lesion and differ-
ences in arm usage. The discrepancy in upper limb
usage and the frequent preservation of some lower
limb function confound the treatment algorithm.
A meticulous upper extremity assessment coupled
with time spent with an intuitive therapist is help-
ful when formulating a surgical plan. The surgical
goals in complete and incomplete are similar with
an emphasis on restoring upper limb function and
increasing spontaneity and independence.
Mid-level Cervical Tetraplegia
The most common level of cervical spinal cord
injury is C5C6. The preservation or recovery of
C5 and C6 nerve roots offers opportunity for
upper limb function. Nerve roots C5 and C6
motor the shoulder girdle, elbow exion, forearm
supination, and some wrist extension. These
741
motions stabilize the shoulder girdle and provide
a foundation for upper limb reconstruction. Lower
cervical spinal cord injury preserves additional
muscles and offers more reconstructive options.
The upper extremity classication of
tetraplegia combined with the hierarchy of hand
function or reconstructive ladder of hand func-
tion yields a relatively straightforward algorithm
for reconstruction.
Classification
The classication for upper extremity impairment
and surgical management has been well
established (Kozin 2007; Zlotolow 2011). The
goal of the international classication for surgery
of the hand in tetraplegia (ICSHT) is to guide
management (Table 1). The objective is to identify
those muscles distal to the elbow that are poten-
tially transferrable. The classication follows the
American Society for Spinal Cord Injury catalog-
ing but emphasizes muscle strength. Therefore,
each muscle below the elbow that is a grade 4 or
has greater strength adds additional gradation. For
example, if the brachioradialis is present and
strong, but no other muscles are of similar
strength, the ICSHT is grade I. The subsequent
muscle strength and gradation are based upon
primary nerve innervation. In other words, the
742 S.H. Kozin et al.

Table 1 International classification for surgery of the reach out into space. Daily activities for persons
hand in tetraplegia (ICSHT) with spinal cord injury are compromised such as
Muscle below pushing a wheelchair, transferring in and out of a
the elbow bed or a chair, and weight shifting for decubitus
Grade function Motor description
prevention. Restoration of elbow extension can
0 None Elbow exion and forearm
supination
have a dramatic increase in reachable workspace,
1 Brachioradialis Forearm stabilization with facilitate wheelchair propulsion (even popping
some supination/pronation wheelies), and enable transfers in and out of bed
2 Extensor carpi Wrist extension or chair. In addition, weight shifts are easier, which
radialis longus lessen the chances of decubiti development.
3 Extensor carpi Strong wrist extension Recent investigation into nerve transfers has
radialis brevis
generated promise; however, the current gold
4 Pronator teres Forearm pronation
standard is tendon transfer (Bertelli and Ghizoni
5 Flexor carpi Wrist exion
radialis 2013). The two main donors are the biceps and the
6 Extensor Finger extension posterior deltoid muscles (Mulcahey et al. 2003;
digitorum LeClerq et al. 2008). Each procedure has its own
communis nuances, positives, and negatives. The biceps-to-
7 Extensor Thumb extension triceps transfer is easier and has a less rigid post-
pollicis longus
operative rehabilitation. The posterior deltoid
8 Flexor Incomplete nger exion
digitorum
transfer requires an intervening graft that compli-
supercialis cates the technique and adds greater postoperative
9 Flexor Complete digital roll-up restrictions.
digitorum
profundus Technique: Biceps-to-Triceps Transfer
X Exceptions Variable

extensor carpi radialis longus is mainly a C6 mus-
cle. The extensor carpi radialis brevis is princi-
pally a C7 muscle as are the pronator teres, exor
carpi radialis, extensor digitorum communis,
extensor pollicis longus, and exor digitorum
supercialis. The exor digitorum profundus and
intrinsic muscles are principally innervated by C8
and T1 nerve roots. The addition of another strong
muscle adds a grade to the classication.
Treatment
Elbow
Persons with C5C6 spinal cord injury lack elbow
extension. The lack of elbow extension hampers
function. Decient elbow extension has multiple
negative ramications. Workable reach space is
dramatically decreased as the arm is unable to
Operative Prerequisites
Active brachialis and supinator muscles are
requirements to biceps transfer to maintain elbow
exion and forearm supination (Kozin 2003; Kuz
et al. 1999). The evaluation of their presence
requires a vigilant physical examination of
elbow exion and forearm supination strength.
The brachialis and supinator muscles can be
assessed independent of the biceps muscle. Effort-
less forearm supination without resistance will
cause supinator function that is palpable along
the proximal radius. Likewise, powerless elbow
exion causes palpable brachialis contraction
along the anterior humerus deep to the biceps
muscle. Equivocal cases require supplementary
evaluation to guarantee adequate supinator and
brachialis muscle activity. Injection of the biceps
muscle with a local anesthetic (e.g., bupivacaine)
induces temporary paralysis. Subsequently, the
examiner can assess independent assessment of
brachialis and supinator function via elbow ex-
ion and forearm supination, respectively.
33 Upper Limb Reconstruction in Persons with Tetraplegia 743

A supple elbow with near-complete range of

motion is also necessary. Patients with an elbow
contracture require resolution of the contracture
prior to surgery. Therapy and/or serial casting can
lessen the contracture. Surgery is deferred until
the contracture is less than 20 . A greater contrac-
ture negates the biceps tendon from reaching the
olecranon at surgery.

Biceps-to-triceps transfer
Preoperative planning
OR table: regular
Position/positioning aids: supine, meticulous padding
of all bony prominences
Fluoroscopy location: none
Equipment: standard Fig. 9 Eighteen-year-old female with spinal cord injury
Tourniquet: sterile and lacks elbow extension necessitating biceps-to-triceps
transfer. Biceps harvested from radial tuberosity and prox-
The patient is placed supine on the operating imal dissection performed to improve excursion (Courtesy
of Shriners Hospital for Children, Philadelphia)
room table. The entire extremity is prepped and
draped. A sterile circular tourniquet is applied
(HemaClear, OHK Medical Devices, Grandville,
Michigan, USA) that exsanguinates during appli-
cation. An S-shaped incision is made along the
medial arm, across the antecubital fossa, and over
the brachioradialis muscle belly. Skin aps are
elevated and the biceps tendon is traced to its
insertion into the radial tuberosity. There are
large crossing veins that require ligation. The
lacertus brosis can be incorporated into the trans-
fer or left behind (Kozin 2003; Kuz et al. 1999;
Revol et al. 1999; Kozin et al. 2010). The tendon
is released directly from the radial tuberosity to
maximize length. The tendon and muscle belly are
mobilized proximal into the arm to maximize
excursion and to improve line of pull (Fig. 9). Fig. 10 Biceps passed along the medial side of the arm to
The musculocutaneous and lateral antebrachial the posterior incision beneath the ulnar nerve (Courtesy of
nerves must be protected. Shriners Hospital for Children, Philadelphia)
Only the medial side of the arm, median nerve,
brachial artery, and ulnar nerves is isolated. A nerve but under the ulnar nerve to avoid any ulnar
posterior incision is made around the olecranon nerve iatrogenic compression (Fig. 11). A
and extended in a proximal direction along the Krackow suture is placed in the tendon with the
triceps tendon. A subcutaneous tunnel is created two suture ends left long.
between the medial brachium and posterior inci- The triceps tendon is split and the tip of the
sion for tendon passage (Fig. 10). The tendon was olecranon is exposed. A large-bore blind bone
passed over the median ulnar nerves, however; the tunnel is made in the olecranon for acceptance of
advances in neuroregeneration has changed our the biceps tendon. The tendon is passed through
practice. The tendons are passed over the median the medial leaet of the triceps tendon in
744
Fig. 11 Biceps passed
beneath the ulnar nerve
(Courtesy of Shriners
Hospital for Children,
Philadelphia)
Fig. 12 Biceps tendon passed through triceps tendon prior
to docking into olecranon (Courtesy of Shriners Hospital
for Children, Philadelphia)
preparation of docking into the bone tunnel
(Fig. 12). The tendon is docked using two poste-
rior unicortical holes and suture retrievers (suture
retriever, Smith and Nephew, Andover, Massa-
chusetts, USA) (Kozin and Zlotolow 2012)
(Fig. 13). The elbow is place in full extension
S.H. Kozin et al.
Fig. 13 Suture retrievers to facilitate docking biceps ten-
don into osseous tunnel (Courtesy of Shriners Hospital for
Children, Philadelphia)
and the two ends are drawn into the bone tunnel
using suture retrievers (Fig. 14). The sutures are
tied over the posterior olecranon cortex. Addi-
tional suturing is performed during closure of the
triceps split with incorporation of the biceps ten-
don. Following closure, a well-padded long arm
33 Upper Limb Reconstruction in Persons with Tetraplegia 745

Biceps-to-triceps transfer
Postoperative protocol
Elbow extension cast for 1 week
Gradually mobilize elbow and initiate transfer training
at 15 per week, using a dial-hinge brace Bledsoe brace
(Bledsoe Brace Systems, Grand Prairie, Texas, USA)
Tendon transfer ring and reeducation instituted
Protective splinting of the elbow is continued until
3 months after surgery

Biceps-to-triceps transfer
Potential pitfalls and preventions
Potential pitfall Pearls for prevention
Pitfall #1 Bleeding during Large crossing veins
biceps harvest require careful ligation
during dissection
Pitfall #2 Inadequate Resolve any elbow
biceps length to reach contracture prior to
olecranon surgery
Pitfall #3 Difculty ring Try alternative methods,
transfer after surgery such as biofeedback
Pitfall #4 Difculty Be patient, avoid passive
regaining elbow exion elbow exion until
3 months following
surgery

Fig. 14 Biceps tendon docked into olecranon (Courtesy

of Shriners Hospital for Children, Philadelphia)
cast is applied with the elbow in extension. The
wrist is included within the cast and the hand
position depends upon concomitant procedures
performed for hand function.
Biceps-to-triceps transfer
Surgical steps
S-shaped incision is made along the medial arm, across
the antecubital fossa, and over the brachioradialis muscle
belly
Biceps tendon traced to insertion into the radial
tuberosity, released, and mobilized in a proximal
direction
Posterior incision around the olecranon and extended
in a proximal direction, triceps tendon is split and the tip
of the olecranon is exposed
Biceps tendon passes from anterior to posterior via
spacious tunnel that runs over the median nerve and deep
to the ulnar nerve
Biceps tendon passes through the triceps leaet and
secured within the osseous tunnel using a docking
technique
Technique: Posterior Deltoid Transfer
Operative Prerequisites
The posterior deltoid must have adequate strength
for motor elbow extension. Shoulder extension
and palpation best assesses posterior deltoid
strength and turgor. The examination can be
performed with the patient seated, although plac-
ing the patient prone provides a more reliable
assessment. Similar to biceps-to-triceps transfer,
a supple elbow with near-complete range of
motion is also necessary. Patients with an elbow
contracture require resolution of the contracture
prior to surgery.
Posterior deltoid transfer
Preoperative planning
OR table: regular
Position/positioning aids: supine with bump under
shoulder, meticulous padding of all bony prominences.
(continued)
746 S.H. Kozin et al.

Fig. 16 Axillary nerve delineated and protected (Courtesy

of Shriners Hospital for Children, Philadelphia)

Fig. 15 Incisions for deltoid-to-triceps transfer (Courtesy

of Shriners Hospital for Children, Philadelphia)

Posterior deltoid transfer

Can also position in lateral decubitus position if
performing isolated posterior deltoid transfer
Fluoroscopy location: none
Equipment: standard, allograft
Tourniquet: none

The patient is positioned in the lateral decubitus

position with the affected side up (LeClerq
et al. 2008; Mulcahey et al. 2003). An axillary roll
is applied. All bony prominences are padded. Prior
to prepping and draping, Marcaine with epinephrine
is instilled in the planned incision sites. The upper
extremity and hemithorax are then prepped and
draped in usual sterile fashion. A gently curved
incision is made over the deltoid muscle (Fig. 15).
Skin aps are elevated. The axillary nerve is identi-
ed entering the deltoid muscle (Fig. 16). The pos-
terior half of the deltoid is selected for transfer
(Fig. 17). A periosteal sleeve is elevated with the
posterior deltoid. Proximal dissection is performed Fig. 17 Posterior half of deltoid isolated (Courtesy of
to improve the excursion and line of pull. Shriners Hospital for Children, Philadelphia)
33 Upper Limb Reconstruction in Persons with Tetraplegia
Fig. 18 Large subcutaneous tunnel between incisions
(Courtesy of Shriners Hospital for Children, Philadelphia)
A distal incision is then made through the distal
third of the triceps tendon. The triceps tendon is
identied. The ulnar nerve is isolated and
retracted out of harms way.
A subcutaneous tunnel is then created between
these two incisions using sequential hemostats
(Fig. 18). An allograft is favored to bride the
distance between the deltoid muscle and triceps
tendon. The allograft is thawed and the proximal
end and sutured to the posterior deltoid and under-
lying periosteum (Fig. 19). The remaining tendon
is then passed through the subcutaneous tunnel to
the distal incision (Fig. 20). The elbow is placed in
full extension and the allograft woven into the
triceps tendon and/or bone for additional augmen-
tation (Fig. 21).
Following rm xation, all wounds are
irrigated and closed in layered fashion.
The patient is placed in a long arm cast and shoul-
der spica cast. The shoulder spica cast is
applied with the elbow straight and the shoulder
in 90 of abduction. This position is maintained
for 6 weeks followed by a transition to a
747
Fig. 19 Allograft secured to deltoid muscle (Courtesy of
Shriners Hospital for Children, Philadelphia)
thoracolumbosacral orthosis and attached hinged
brace that was fabricated prior to surgery. Therapy
involves tendon transfer training and a gradual
allowance of elbow exion and lessening of
shoulder abduction. The process is arduous and
must be governed by an experienced therapist.
Expedited elbow exion or shoulder adduction
will result in attenuation of the transfer
(Fridn 2000).
Posterior deltoid transfer
Surgical steps
Curved incision is made over the deltoid muscle
Axillary nerve is identied entering the deltoid muscle
Posterior half of the deltoid isolated with a periosteal
sleeve
Distal incision about the distal third of the triceps
tendon
Subcutaneous tunnel is then created between the two
incisions
Gap between posterior deltoid and olecranon bridged
with allograft or autograft
Elbow placed in full extension and the autograft/
allograft woven into the triceps tendon and/or bone
748 S.H. Kozin et al.

Posterior deltoid transfer

Postoperative protocol
Long arm cast with elbow in extension attached to a
shoulder spica cast with shoulder abducted to 90
Transitioned to thoracolumbosacral orthosis at
6 weeks and tendon transfer training initiated
Tendon transfer ring and reeducation instituted
Process is arduous and must be governed by an
experienced therapist
Protective extension block splinting of the elbow is
continued until 3 months after surgery

Posterior deltoid transfer

Potential pitfalls and preventions
Potential pitfall Pearls for prevention
Pitfall #1 Injury to axillary Identify axillary nerve
nerve entering the posterior
deltoid
Pitfall #2 Inadequate girth Choose posterior half
of deltoid instead of posterior third
Pitfall #3 Suboptimum Augment with autograft or
attachment to the deltoid allograft
muscle
Pitfall #4 Attenuation of Avoid rapid allowance of
tendon transfer shoulder adduction or
elbow exion
Pitfall #5 Difculty with Enlist an experienced
ring transfer therapist, try alternative Fig. 20 Allograft passed to distal incision and triceps
methods such as tendon (Courtesy of Shriners Hospital for Children,
biofeedback Philadelphia)

Forearm deformity is initially supple, but as time passes,

The forearm is the forgotten joint with regard to
movement and balance. The forearm is articulated
at both its proximal and distal ends (proximal
and distal radioulnar joint joints). The strong
interosseous ligament tightly links the radius and
ulna and participates in load transfer (McGinley
et al. 2003).
Forearm rotation is balanced by two muscles
that supinate (biceps and supinator) and two mus-
cles that pronate (pronator teres and quadratus).
Supination is primarily controlled by C5 and C6
(biceps and supinator muscles). Pronation is prin-
cipally motored by C7 (pronator teres) and C8
(pronator quadratus). Mid-cervical tetraplegia fre-
quently results in loss of the pronators and pres-
ervation of the supinators. The resultant
imbalance will result in deformity over time. The
the contracture(s) becomes xed (Fig. 22).
When decided whether treatment is warranted,
the surgeon must weigh the advantages and dis-
advantages of treatment. A therapists insight is
helpful in the decision-making process. Activities
that require supination include turning a door
knob, carrying a tray, feeding ones self, and dis-
pensing soap. Activities that require pronation
include typing on a keyboard, performing tabletop
functions, and holding on to handle bars. The
surgical procedures to enhance rotation in one
direction will sacrice some degree of opposite
rotation. Therefore, ample thought is necessary
prior to proceeding with surgical intervention.
With reference to the forearm, an ounce of
prevention is worth a pound of cure. Stretching
and splinting of the unbalanced forearm is man-
datory to prevent contracture. The goal is to
33 Upper Limb Reconstruction in Persons with Tetraplegia
Fig. 21 Allograft secured to triceps with elbow in full
extension (Courtesy of Shriners Hospital for Children,
Philadelphia)
prevent a xed forearm contracture. A supple
imbalance is amenable to tendon transfer, while
a xed deformity requires intraoperative mem-
brane release to regain passive motion or
osteotomy to realign the forearm into a more
functional position. Persons with tetraplegia
and preservation of brachioradialis function can
sometimes utilize this muscle for forearm rota-
tion to minimize contracture formation. In other
words, these individuals will re their brachi-
oradialis to incite supination to neutral and then
utilize gravity to drop into pronation. Therefore,
the lack of brachioradialis innervation further
jeopardizes the forearm and predisposes the for-
gotten joint to contracture formation.
If the team and patient decide that additional
pronation is warranted, the surgery depends on
whether the position is supple or xed. The sup-
ple forearm can undergo tendon transfer to regain
pronation. Biceps rerouting is the workhorse pro-
cedure to gain pronation. If the deformity is
xed, the interosseous membrane can be released
to gain passive pronation at the time of biceps
rerouting; however, bony surgery is more
749
Fig. 22 Spinal cord injury at C5C6 with bilateral xed
supination forearm contractures (Courtesy of Shriners
Hospital for Children, Philadelphia)
reliable and preferred. The preferred technique
changes the forearm from a two-bone structure to
a one-bone structure (a.k.a. one-bone forearm)
and eliminates any forearm rotation. This proce-
dure is versatile and can reliably rotate the fore-
arm into the desired position without any
concern for recurrence.
Technique: Biceps Tendon Rerouting
Biceps tendon rerouting
Preoperative planning
OR table: regular
Position/positioning aids: supine
Fluoroscopy location: none
Equipment: standard
Tourniquet: sterile
The patient is placed supine on the operating
room table. The entire extremity is prepped and
draped. A sterile circular tourniquet is applied
(HemaClear, OHK Medical Devices, Grandville,
Michigan, USA) that exsanguinates during appli-
cation. An S-shaped incision is made along the
medial arm, across the antecubital fossa, and over
the brachioradialis muscle belly (Fig. 23).
750 S.H. Kozin et al.

Fig. 23 Six-year-old
female with supple
supination posturing of her
left arm. Skin incision for
biceps rerouting procedure
(Courtesy of Shriners
Hospital for Children,
Philadelphia)

Fig. 24 Isolation of the

biceps tendon and lacertus
brosis. Lateral
antebrachial cutaneous
nerve just lateral to the
tendon (Courtesy of
Shriners Hospital for
Children, Philadelphia)

The antebrachial cutaneous nerve is identied
lateral to the biceps tendon and protected
(Fig. 24). The biceps tendon is traced to its inser-
tion into the radial tuberosity (Fig. 25). There are
large crossing veins that require ligation. The
lacertus brosis is incised from the biceps tendon.
The entire length of the biceps tendon is isolated.
A long Z-plasty is performed with one limb left
attached to the radius and the other attached to the
muscle (Fig. 26). The limb attached to the radius is
rerouted around the radius through the inter-
osseous space to yield a pronation force. A curved
clamp, such as a Castameda pediatric clamp
(Pilling Surgical, North Carolina, USA), facili-
tates tendon passage (Fig. 27) (Kozin and
Zlotolow 2012). The posterior interosseous
nerve must be protected to prevent iatrogenic
injury.
The elbow is positioned in 90 of exion with
the forearm in pronation. The rerouted distal ten-
don is woven back through the proximal tendon
that remained attached to the biceps muscle
(Fig. 28). The subcutaneous tissue and skin are
closed in routine fashion. A long arm cast is
applied with the elbow in 90 of exion and the
forearm in pronation for 5 weeks.
33 Upper Limb Reconstruction in Persons with Tetraplegia 751

Fig. 25 Biceps tendon

traced to its insertion into
radial tuberosity (Courtesy
of Shriners Hospital for
Children, Philadelphia)

Fig. 26 Z-plasty of entire

biceps tendon with long
proximal and distal limbs
(Courtesy of Shriners
Hospital for Children,
Philadelphia)

Fig. 27 A curved clamp

(Castameda pediatric
clamp, Pilling Surgical,
North Carolina, USA)
facilitates tendon rerouting
around the radius (Courtesy
of Shriners Hospital for
Children, Philadelphia)
752 S.H. Kozin et al.

Fig. 28 Distal limb

repaired back to proximal
limb using a tendon weave
augmented with
nonabsorbable suture
(Courtesy of Shriners
Hospital for Children,
Philadelphia)

Biceps tendon rerouting Biceps tendon rerouting

Surgical steps Pitfall #5 Difculty with Try alternative methods
Long S-shaped incision, across the antecubital fossa ring transfer such as biofeedback
Isolate entire length of the biceps tendon
Perform long Z-plasty with one limb left attached to
the radius and the other attached to the muscle
Pass limb attached to the radius around the radius Rehabilitation
through the interosseous space to yield a pronation force
Secure rerouted distal tendon back to the proximal
tendon attached to the biceps muscle with forearm in
Immediate nger motion is encouraged to decrease
pronation swelling. The cast is removed 5 weeks after surgery
and a long arm splint is fabricated that duplicates
the casted position. Active elbow exion is started,
Biceps tendon rerouting which promotes biceps activity across the elbow
Postoperative protocol and forearm. Tendon transfer training is instituted.
Long arm cast is applied with the elbow in 90 of
Instructing the child to perform supination helps
exion and the forearm in pronation for 5 weeks
Tendon transfer ring and reeducation instituted
stimulate biceps activity and yields forearm prona-
Protective elbow and forearm splinting until 3 months
tion. Relearning occurs over time. A protective
after surgery splint is worn for 8 weeks after surgery. Passive
forearm supination or elbow extension motion is
avoided until 3 months after surgery.
Biceps tendon rerouting
Potential pitfalls and preventions Technique: One-Bone Forearm
Potential pitfall Pearls for prevention
Pitfall #1 Inadvertent injury Identify the nerve before One-bone forearm
to the lateral antebrachial isolating biceps tendon Preoperative planning
cutaneous nerve
OR table: regular
Pitfall #2 Inadequate biceps Dissect entire biceps
Position/positioning aids: supine
tendon length length before Z-plasty
Fluoroscopy: yes
Pitfall #3 Difculty passing Curved clamp facilitates
distal tendon around the tendon passage Equipment: standard with varying plate sizes
radius Tourniquet: sterile
Pitfall #4 Injury to the Be wary of the nerve
posterior interosseous nerve during tendon passage The patient is placed supine on the operating
(continued) room table (Fig. 29). The entire extremity is
33 Upper Limb Reconstruction in Persons with Tetraplegia
Fig. 29 Eight-year-old male with severe xed supination
contracture of his right forearm (Courtesy of Shriners Hos-
pital for Children, Philadelphia)
prepped and draped. A sterile circular tourniquet
is applied (HemaClear, OHK Medical Devices,
Grandville, Michigan, USA) that exsanguinates
during application. A curvilinear incision is
made along the distal radius and proximal ulna.
The volar forearm in the fascia is incised along the
length of the skin incision. The entire contents of
the volar forearm are identied (Fig. 30). The
radius is exposed via a trans-exor carpi radialis
incision. The median nerve is identied and
protected. The ulna is isolated deep to the exor
carpi ulnaris and ulnar neurovascular bundle. In
patients with substantial denervation, the forearm
muscles have manly been replaced by fat with
only a few strands of viable muscle present.
The osteotomies are planned with the radius
osteotomy 12cm distal to the ulnar osteotomy.
Proximal to the proposed ulnar osteotomy site,
an appropriated sized dynamic compression
plate is secured with three or four bicortical
screws. The anterior interosseous nerve and
753
vessels are dissected and elevated from the
volar interosseous membrane. The intervening
membrane is incised to allow the radius to be
positioned on top of the proximal ulna. The
plate is then removed and the ulnar and radial
osteotomies are performed with a ne-bladed
sagittal saw. The distal ulna can be excised or
simply mobilized out of harms way.
The radius is manually mobilized toward the
proximal ulna (Fig. 31). The plate is reapplied to
the ulna. Once the bones are coapted, the radius is
rotated into the desired position. The reduction is
held with a bone reduction clamp and the position
carefully assessed. Once the position is deemed
acceptable, the plate is secured to the distal radius
using dynamic compression and bicortical screw
xation (Fig. 32). The remaining proximal radius
segment is used as onlay graft across the
osteotomy site. The radius is decorticated and
afxed to the construct using an intraosseous
suture or compression screw. The subcutaneous
tissue and skin are closed and a long arm splint
was placed. The splint is changed to a long arm
cast 2 weeks after surgery. The long arm cast is
worn for an additional 4 weeks. Radiographs are
taken at a 2-week follow-up to ensure alignment
and repeated at 6 weeks to ensure healing of the
osteotomy site. A protective splint is then fabri-
cated for another 6 weeks. Digital range of motion
exercises are started immediately after surgery.
Elbow motion is begun at 6 weeks following
removal of the long arm cast. The amount of
correction achieved is often remarkable (Fig. 33).
One-bone forearm
Surgical steps
Curvilinear incision is made along the distal radius and
proximal ulna
Isolate radius is exposed via a trans-exor carpi
radialis incision
Mobilize entire volar forearm compartment
Isolate the ulna deep into the exor carpi ulnaris and
ulnar neurovascular bundle
Plan osteotomies with the radius osteotomy 12 cm
distal to the ulnar osteotomy
Excise intervening interosseous membrane
Proximal to the proposed ulnar osteotomy site secure
an appropriated sized dynamic compression with three or
four bicortical screws
(continued)
754 S.H. Kozin et al.

Fig. 30 Surgical exposure

of the volar forearm
compartment (Courtesy of
Shriners Hospital for
Children, Philadelphia)

Fig. 31 Radius and ulnar

are osteotomized with a
saw. The radius is cut distal
to the ulnar and transposed
on top of the ulna (Courtesy
of Shriners Hospital for
Children, Philadelphia)

One-bone forearm One-bone forearm

Cut the ulnar and radial osteotomies with a ne-bladed
sagittal saw
Mobilize the radius toward the proximal ulna
Rotate the radius into the desired position and afx to
the end of the ulna using dynamic compression and
bicortical screw xation
Secure the proximal radius segment as onlay graft
bridging the osteotomy site
Postoperative protocol
Long-arm plaster splint is applied for 2 weeks and
the child is monitored overnight for neurovascular problems
Long-arm berglass cast 2 weeks after surgery
Cast is removed 6 weeks after surgery and a splint
fabricated
Protective splinting until x-rays reveal crossing
trabeculae at the osteotomy site(s)
33 Upper Limb Reconstruction in Persons with Tetraplegia 755

Fig. 32 Plate and screw

xation from the proximal
ulna to the distal radius with
the remaining proximal
radius segment is used as
onlay graft across the
osteotomy site (Courtesy of
Shriners Hospital for
Children, Philadelphia)

One-bone forearm
Potential pitfalls and preventions
Potential pitfall Pearls for prevention
Pitfall #1 Avoid closing the fascia and
Compartment immediate casting
syndrome
Pitfall #2 Delayed Avoid bone necrosis, preserve
union periosteum, ample xation,
onlay graft
Pitfall #3 Hardware Prolonged protection until
breakage union is evident

Wrist and Hand

The management of the wrist and hand in

tetraplegia follows a similar algorithm to brachial
plexus palsy. The terms hierarchy of hand func-
tion or the reconstruction ladder are used inter-
changeably. The primary crucial movement is
wrist extension, which aligns the nger exors
along Blixs length-tension curve for maximum
Fig. 33 Postoperative correction with marked improve-
grip. In children without grip, active wrist exten- ment in forearm position (Courtesy of Shriners Hospital for
sion provides tenodesis for grip as the nger ex Children, Philadelphia)
into the palm and tenodesis for lateral pinch as the
thumb adducts toward the index. The second most pinch (Fig. 34). The third essential motion is
important movement or task is lateral pinch, grasp, which allows us to hold objects (Fig. 35).
which affords the ability to perform numerous The fourth and last movement is digital opening
activities of daily living. Most activities that we for object acquisition. The reason to place this
do every day can be accomplished with lateral function last on the ladder is that wrist exion
756 S.H. Kozin et al.

Fig. 34 Lateral pinch is the

prehension used for most
activities of daily living
(Courtesy of Shriners
Hospital for Children,
Philadelphia)

primarily an intrinsic function and metacarpo-

Fig. 35 Grasp is imperative for holding and retaining
objects within the hand (Courtesy of Shriners Hospital for
Children, Philadelphia)
yields passive digital opening, which is often ade-
quate for object procurement. In addition, syn-
chronous digital opening is difcult to achieve
via surgery as interphalangeal joint extension is
phalangeal joint extension is mainly an extrinsic
function. The only way the extrinsic system can
elicit interphalangeal joint extension is by limiting
metacarpophalangeal joint extension (e.g., a
Zancolli tenodesis of the exor digitorum
supercialis). This procedure can yield problems
such as limiting exor digitorum profundus
excursion and/or attenuating over time. Restoring
both metacarpophalangeal and interphalangeal
movements by tendon transfer(s) is a daunting
task replete with complications.
The selection of available and appropriate
donors for tendon transfer is crucial in persons
with tetraplegia. Try to select a synchronous mus-
cle as postoperative therapy is easier and tendon
retraining is minimized. Therefore, a wrist exten-
sor (extensor carpi radialis longus (ECRL)) for
nger exion is preferred. The main alternative
donors are the brachioradialis, which is often used
for wrist extensor or lateral pinch dependent upon
the persons function. When these donor tendons
are unavailable, search for alternative donors
based upon the established principles of availabil-
ity, expendability, synchrony, and excursion.
There are a myriad of possible surgical options
as more motors become available and expendable.
Convoluted procedures, however, should be
avoided as therapy is more difcult and compli-
cations are more prevalent.
33 Upper Limb Reconstruction in Persons with Tetraplegia 757

In patients that are ICSHT grade 0, there are no

tendon transfer options available as there are no
existing motors. In patients that are ICSHT grade
1, the brachioradialis can be transferred to the
extensor carpi radials brevis to achieve wrist
extension. At the same time, a tenodesis of the
exor pollicis longus to the radius enhances lat-
eral pinch and a split exor pollicis longus transfer
or interphalangeal arthrodesis stabilizes the
interphalangeal joint. In patients that are ICSHT
grade 2, wrist extension is present. Active lateral
pinch is obtained by transferring the brachioradialis
to the exor pollicis longus and stabilizing the
interphalangeal joint (split exor pollicis longus
transfer or arthrodesis).
In patients that are ICSHT grade 3, strong wrist
extension is present, and therefore, one of the
wrist extensor tendons is expendable. The exten-
sor carpi radials longus is transferred to the exor
profundus tendons deep to the radial artery via a
radial longitudinal incision. At the same time, the Fig. 36 An unstable and lax thumb carpometacarpal joint
brachioradialis is transferred to the exor pollicis may miss the index nger during attempted lateral pinch
(Courtesy of Shriners Hospital for Children, Philadelphia)
tendon and a split exor pollicis tendon transfer
performed. In patients that are ICSHT grade 4, the
pronator teres becomes innervated and potentially that are ICSHT grade 8 or grade 9 have some
available. The decision to utilize the pronator teres ability to grasp and hold items. The surgeon has
as a donor is a difcult one. The surgeon could the option(s) of enhancing grasp or attempting
transfer the pronator teres to the exor pollicis intrinsic reconstruction. These patients are treated
longus for lateral pinch. This strategy liberates with similar tenets as combined low median/low
the brachioradialis for another function such as ulnar nerve injuries. The objectives of restoring
thumb or nger extension. The difculty with thumb opposition and improving hand balance are
obtaining nger extension was discussed previ- realistic goals.
ously, and utilizing the pronator teres for this The thumb carpometacarpal (CMC) joint can
function is usually not recommended. be unstable without the support of the thenar
In patients that are ICSHT grade 5, the exor muscles. The thumb may miss the index nger
carpi radialis and active wrist exion are present. during attempted lateral pinch (Fig. 36). The pros
Active wrist exion provides distinct advantages and cons of CMC arthrodesis need to be contem-
to persons with tetraplegia. He or she can hook plated. Stabilization of the CMC joint provides a
objects by exing the wrist, and active wrist ex- foundation for stronger lateral pinch (Fig. 37). The
ion promotes digital extension that eases object position of arthrodesis, however, varies from the
acquisition. These benets preclude the exor abled-body person (45 of palmar abduction). Per-
carpi radialis from being expendable for tendon sons with tetraplegia usually lack intrinsic function
transfer. Patients that are ICSHT grade 5 or grade and metacarpophalangeal joint exion. The lack of
6 have the ability to extend the ngers and thumb, index metacarpophalangeal joint exion alters the
respectively. This further enhances hand function recommended position for CMC arthrodesis. Con-
especially since grasp and pinch are easily siderable less palmar abduction is necessary (about
obtained via transfer using the extensor carpi 20 ) for alignment between the thumb and radial
radialis and brachioradialis, respectively. Patients side of the index proximal interphalangeal joint.
758
Fig. 37 Thumb carpometacarpal arthrodesis improves
lateral pinch alignment but narrows the thumb index web
space (Courtesy of Shriners Hospital for Children,
Philadelphia)
Video 1 Active pinch
This position narrows the thumb index web space
and hampers acquisition of large objects such as
bottles or cans. Generally, in patients with ligamen-
tous laxity and very unstable CMC joints, arthrod-
esis is performed. In patients without ligamentous
laxity and some CMC joint stability, initial arthrod-
esis is not recommended. The patients are
counseled about the possibility of additional
S.H. Kozin et al.
surgery if adequate alignment cannot be achieved.
They often learn to quickly extend their wrist such
that the thumb icks into the appropriate position
for lateral pinch (Video 1).
Technique: Brachioradialis Transfer
to Flexor Pollicis Longus, Split Flexor
Pollicis Longus Transfer (ICSHT Grade 2)
Brachioradialis tendon transfer, split exor pollicis
longus
Preoperative planning
OR table: regular
Position/positioning aids: supine
Fluoroscopy location: none
Equipment: standard, Kirschner wire
Tourniquet: sterile
The patient is placed supine on the operating
room table. The entire extremity is prepped and
draped. A sterile circular tourniquet is applied
(HemaClear, OHK Medical Devices, Grandville,
Michigan, USA) that exsanguinates during applica-
tion. The split exor pollicis longus transfer is
performed rst. A mid-axial incision is made
along the radial side of the thumb. The dorsal ap
is elevated and the extensor pollicis longus tendon
identied. The volar ap is elevated with the
neurovascular bundle and the exor pollicis longus
isolated. The exor pollicis longus is split in the
midline (Fig. 38). The radial half traced to its inser-
tion into the distal phalanx and cut (Fig. 39). This
radial half is passed in a dorsal direction over the
interphalangeal joint axis (Fig. 40). The tendon is
then passed through the extensor pollicis longus and
back to itself. Tension is set so that the inter-
phalangeal joint rests in slight exion. A small
nonabsorbable suture is used to secure the repair
making sure the suture captures the exor pollicis
longus and the extensor pollicis longus tendons. A
0.45 Kirschner is driven from the thumb tip across
the interphalangeal joint for stabilization (Fig. 41).
The pin is cut short and a pin cap applied.
Next, a longitudinal radial incision is made
from the styloid to the proximal third of the fore-
arm. The lateral antebrachial cutaneous and radial
sensory nerves are protected. The brachioradialis
insertion into the radial styloid is isolated. The
tendon is released and the brachioradialis
33 Upper Limb Reconstruction in Persons with Tetraplegia
dissected form its investing fascia. The dissection
proceeds into the proximal 1/3 of the forearm
(Fig. 42). The radial sensory nerve and radial
artery must be protected throughout the dissec-
tion. The volar portion of the skin ap is mobi-
lized. The fascia is incised along the exor carpi
radialis tendon. The underlying median nerve and
exor digitorum supercialis tendons are mobi-
lized. The deep exor pollicis longus is isolated
Fig. 38 Radial mid-axial incision with exor pollicis
longus is split in the midline (Courtesy of Shriners Hospital
for Children, Philadelphia)
Fig. 39 Radial half of
exor pollicis longus cut at
its insertion (Courtesy of
Shriners Hospital for
Children, Philadelphia)
759
(Fig. 43). The radial artery and its venae
comitantes are elevated from the distal radius.
The brachioradialis tendon is passed deep to the
radial artery and woven through the exor pollicis
longus tendon using a Pulvertaft weave (Fig. 44).
Tension is adjusted until there is lateral pinch dur-
ing wrist extension and thumb opening during
wrist exion (Fig. 45). The subcutaneous tissue
and skin are closed. A long arm thumb spica cast
is applied with the wrist in extension and the elbow
exed to 90 . The thumb rests along the index
nger and the thumb tip is covered. Immobilization
time depends upon the strength of the repair and the
cooperation of the child. Typically, the cast is
removed 3 weeks following surgery and a short
arm thumb spica splint is fabricated. Tendon trans-
fer training is initiated and can be challenging. The
patient must learn how to stabilize the elbow and
re the brachioradialis at the same time. The splint
is discontinued 810 weeks from surgery.
Brachioradialis tendon transfer, split exor pollicis
longus
Surgical steps
Radial mid-lateral incision over the thumb
interphalangeal joint
Elevate dorsal and volar aps
Isolate extensor pollicis longus and exor pollicis
longus
Harvest radial half of exor pollicis longus from distal
phalanx
(continued)
760 S.H. Kozin et al.

Brachioradialis tendon transfer, split exor pollicis Brachioradialis tendon transfer, split exor pollicis
longus longus
Pass through extensor pollicis longus and back to Potential pitfalls and preventions
itself, secure with suture Potential pitfall Pearls for prevention
Longitudinal Kirschner wire xation from thumb tip Pitfall #1 Inadvertent Careful dissection, identify
across interphalangeal joint injury to lateral radial sensory nerve
Longitudinal radial incision from the styloid to the antebrachial cutaneous between brachioradialis and
proximal third of the forearm nerve or radial sensory extensor carpi radialis
Release the brachioradialis from the radial styloid and nerve longus tendon
dissect in a proximal direction into the proximal 1/3 of the Pitfall #2 Limited Proximal dissection to free
forearm intraoperative excursion all investing fascia
Isolate the exor pollicis longus in the volar forearm of the brachioradialis
compartment Pitfall #3 Limited Advance rehabilitation to
Transfer the brachioradialis to the exor pollicis excursion of tendon encourage gliding
longus transfer
Tension so that the thumb rests against the index nger
in with the wrist in extension
Technique: Extensor Carpi Radialis-to-
Brachioradialis tendon transfer, split exor pollicis Flexor Digitorum Profundus Tendon
longus Transfer (ICSHT Grade 2) (Video 2)
Postoperative protocol
Long arm thumb spica cast with the elbow in 90 of Extensor carpi radialis tendon transfer
exion, the wrist in extension, and the thumb resting Preoperative planning
against the index OR table: regular
Typical casting is for 3 weeks Position/positioning aids: supine
Cast is removed 3 weeks following surgery and a short Fluoroscopy location: none
arm splint is fabricated Equipment: standard
Thumb Kirschner wire removed 4 weeks after surgery Tourniquet: sterile
Protective wrist splinting until 8 weeks after surgery
The patient is placed supine on the operating
room table. The entire extremity is prepped and
draped. A sterile circular tourniquet is applied
(HemaClear, OHK Medical Devices, Grandville,
Michigan, USA) that exsanguinates during appli-
cation. A longitudinal radial incision is made from
the styloid to the proximal third of the forearm
(Fig. 46). The lateral antebrachial cutaneous and
radial sensory nerves are protected. Distal to the
rst compartment, the extensor carpi radialis
longus (ECRL) and extensor carpi radialis brevis
(ECRB) tendons are identied. In the proximal part
of the incisions, the ECRL and ECRB tendons are
also isolated. The ECRL tendon is then cut as distal
as possible. An Alice clamp (Pilling Surgical,
North Carolina, USA) is applied to the ECRB
tendon in the proximal forearm and rolled in a
Fig. 40 Radial half of exor pollicis longus in a dorsal
direction toward the extensor pollicis longus (Courtesy of proximal direction. This maneuver passes the ten-
Shriners Hospital for Children, Philadelphia) dons under the rst dorsal compartment.
33 Upper Limb Reconstruction in Persons with Tetraplegia 761

Fig. 41 Following split

exor pollicis longus to
extensor pollicis longus
tendon transfer, a 0.45
Kirschner is driven through
the thumb interphalangeal
(Courtesy of Shriners
Hospital for Children,
Philadelphia)

Fig. 42 Brachioradialis
tendon released and
mobilized into the proximal
1/3 of the forearm (Courtesy
of Shriners Hospital for
Children, Philadelphia)

Fig. 43 Flexor pollicis

longus tendon isolated in
volar compartment
(Courtesy of Shriners
Hospital for Children,
Philadelphia)
762 S.H. Kozin et al.

Fig. 44 Brachioradialis
tendon woven through the
exor pollicis longus
tendon (Courtesy of
Shriners Hospital for
Children, Philadelphia)

Fig. 45 Tendon transfer

tension is adjusted until
there is lateral pinch during
wrist extension (Courtesy of
Shriners Hospital for
Children, Philadelphia)

The volar portion of the skin ap is mobilized.

The fascia is incised along the exor carpi radialis
tendon. The underlying median nerve and exor
digitorum supercialis tendons are mobilized.
The deep exor digitorum profundus tendons are
isolated. The tendons are sutured together
(en masse) to set the desired nger cascade during
grasp. The radial artery and its venae comitantes
are elevated from the distal radius.
The ECRL tendon is passed deep to the radial
artery and woven through the exor digitorum
profundus tendons using a Pulvertaft weave
(Fig. 47). Tension is adjusted until there is nger
exion during wrist extension and tenodesis Video 2 Thumb ip
33 Upper Limb Reconstruction in Persons with Tetraplegia 763

Fig. 46 Harvest of the

extensor carpi radialis
longus (and brachioradialis)
via longitudinal radial
incision (Courtesy of
Shriners Hospital for
Children, Philadelphia)

Fig. 47 Extensor carpi

radialis longus tendon
woven through the exor
digitorum profundus
tendons (Courtesy of
Shriners Hospital for
Children, Philadelphia)

opening during wrist exion. The subcutaneous
tissue and skin are closed. A short or long arm cast
is applied depending upon the age of the patient
and the strength of the repair. The wrist is in
slight extension and the elbow exed to 90 .
The ngers are slightly exed and this position
is maintained with a small roll of Webril cotton
cast padding. Early mobilization can be started
on postoperative day one as long as the tendons
are of adequate caliper and the transfer site is
rmly repaired. The web roll is removed and
the patient is instructed to bend his or her ngers.
The response is often overwhelming as nger
exion is readily initiated. The cast is removed
3 weeks following surgery and a short arm splint
is fabricated. Tendon transfer training is initiated,
although the synergism of the transfer negates
the need of extensive relearning. The splint is
discontinued 8 weeks from surgery.
Extensor carpi radialis tendon transfer
Surgical steps
Longitudinal radial incision from the styloid to the
proximal third of the forearm
Isolate the extensor carpi radialis longus distal to the
rst compartment and in the proximal forearm
(continued)
764 S.H. Kozin et al.

Extensor carpi radialis tendon transfer Table 2 Grading scale of tendon size
Cut the extensor carpi radialis longus and pull beneath Grade Implication
the rst compartment into the proximal forearm Linguine Early mobilization
Isolate and synchronize the exor digitorum profundus Minimal concern for failure of repair
tendons Active motion to prevent motion
Secure rerouted distal tendon back to the proximal limiting scar
tendon attached to the biceps muscle with forearm in Spaghetti Intermediate rehabilitation
pronation
Vermicelli or Delayed rehabilitation
Pass the extensor carpi radialis longus deep to the Angel hair Substantial concern for repair
radial artery and weave into the exor digitorum
attenuation or failure
profundus tendons
Protracted splint protection
Adapted from Kozin (2008)
Extensor carpi radialis tendon transfer
Postoperative protocol
Long arm cast is applied with the elbow in 90 of
exion and the wrist in slight extension. Fingers are
slightly exed and this position is maintained with a the tendons correlates with the strength of the
small roll of Webril cotton cast padding repair. A simple yet useful classication of tendon
Early mobilization can be started on postoperative day size has been devised (Table 2) (Kozin 2008).
1 as long as the tendons are of adequate caliper and the
transfer site is rmly repaired Robust linguine tendons can be managed with
Cast is removed 3 weeks following surgery and a short early mobilization techniques to facilitate tendon
arm splint is fabricated gliding. In contrast, small vermicelli tendons
Protective wrist splinting until 8 weeks after surgery require longer mobilization, prolonged splinting,
increased protection, and a slower rehabilitation.
A skilled therapist experienced in tendon transfer
Extensor carpi radialis tendon transfer rehabilitation and children will maximize the out-
Potential pitfalls and preventions come. Early detection of impeding problems, such
Potential pitfall Pearls for prevention as adhesions, allows probable correction while
Pitfall #1 Inadvertent Careful dissection, identify delayed recognition is problematic to x.
injury to lateral radial sensory nerve
The clinical outcome and effect on function are
antebrachial cutaneous between brachioradialis
nerve or radial sensory and extensor carpi radialis discussed in Outcome Measures. Patients
nerve longus tendon with specic, realistic, functional goals will realize
Pitfall #2 Poor Synchronize the exor the best self-perceived outcomes. The recognition
synchronization of long digitorum profundus that objective measurements and legacy measures
nger exors tendons before tendon
transfer
may not correlate with improved function has
Pitfall #3 Limited Advance rehabilitation to resulted in a dramatic shift in outcome assessments.
excursion of tendon encourage gliding The initiative toward patient- or caregiver-reported
transfer outcomes (PROs) has resulted in the development
of reliable subjective measures. For persons with
spinal cord injury, Shriners Hospitals for Children
has invested millions of dollars into patient-
Rehabilitation and Outcome reported outcomes using the computer adaptive
test (CAT) platform (Mulcahey et al. 2012; Bent
The rehabilitation process is critical to maximize et al. 2013). This methodology has been shown to
the outcome following surgery. Dialogue between correlate with legacy measures, possess minimal
the surgeon and therapist is a prerequisite for suc- oor and ceiling effects, and be less burdensome
cess. The main factors that govern the speed of the compared to previous measures. CAT will change
rehabilitation are the age of the child, the size of the the manner in which we assess outcome in the near
tendons, and the strength of the repair. The size of future.
33 Upper Limb Reconstruction in Persons with Tetraplegia
Summary
The management of the upper limb tetraplegia
requires a multidisciplinary approach. The drastic
change from an able-bodied person to a person with
spinal cord injury is a game changer. Routine activ-
ities of daily living become challenging and frustrat-
ing. Independence is replaced by dependence.
Personal thoughts about bladder and bowel manage-
ment become an open dialogue. The determination
and motivation for enhanced upper extremity func-
tion becomes vital to increase independence and
spontaneity. The upper extremity surgeon and ther-
apist have pivotal roles in helping the patient. As
long as viable donor muscles are available, they
can formulate a reconstructive plan. Utilizing the
ICSHT and following guidelines regarding the
hierarchy of hand function, a surgical and rehabil-
itative plan can be devised. Effective execution of
this plan will result in a forever indebted patient
that functions more independently and is capable
of contributing to society. A successful outcome is
rewarding to the patient, surgeon, and therapist.
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 Part X
Neuromuscular Disorders
 Cerebral Palsy
34
Nina Lightdale-Miric and Carolien P. de Roode

Contents Surgical Approach . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 784

Wrist Flexor Releases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 784
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 770 Tendon Transfer for Wrist Extension . . . . . . . . . . . . . . . . 784
Pathoanatomy, Physiology, and Applied Flexor-Pronator Origin Muscle Slide . . . . . . . . . . . . . . . . 786
Anatomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 771 Arthrodesis/Chondrodesis and Proximal Row
Assessment of Cerebral Palsy of the Upper Carpectomy (PRC) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 786
Extremity . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 772 Thumb . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 789
Signs and Symptoms of Cerebral Palsy . . . . . . . . . . . . . 772 Classication . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 789
Electromyography (EMG) . . . . . . . . . . . . . . . . . . . . . . . . . . . 774 Preoperative Planning and Decision Making . . . . . . . . 789
Cerebral Palsy of the Upper Extremity Positioning . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 790
Treatment Options . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 774 Extrinsic Thumb Reconstruction
Nonoperative Management of Upper Extremity Surgical Approach . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 790
Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 774 Intrinsic Thumb Reconstruction
Surgical Approach . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 791
Operative Treatment of Cerebral Palsy . . . . . . . . . . 776 Thumb Joint Capsulodesis, Arthrodesis,
Indications/Contraindications . . . . . . . . . . . . . . . . . . . . . . . . 776 and Chondrodesis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 791
Surgical Procedures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 777
Finger Deformity in Cerebral Palsy . . . . . . . . . . . . . . . 792
Forearm Pronation Contracture . . . . . . . . . . . . . . . . . . 780 Finger Extrinsic Flexor Lengthenings . . . . . . . . . . . . . . . 794
Preoperative Planning . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 780 Flexor-Pronator Slide . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 795
Positioning . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 781 Flexor Digitorum Supercialis to Profundus
Surgical Approach . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 781 Transfer (STP) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 795
Technique: Forearm Pronation Finger Extension Transfers . . . . . . . . . . . . . . . . . . . . . . . . . . 795
Contracture Release . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 781 Lateral Band Rerouting . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 796
Central Slip Tenotomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 796
Wrist . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 783
Preoperative Planning . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 783 Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 796
Classication . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 783
Positioning . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 784 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 797

N. Lightdale-Miric (*)
Department Orthopaedics, Childrens Hospital of
Los Angeles, Los Angeles, CA, USA
e-mail: ninalightdale@gmail.com
C.P. de Roode
Division of Orthopaedic Surgery Childrens Bone and
Spine Surgery, University of Nevada School of Medicine,
Las Vegas, NV, USA
e-mail: cderoode@CBSOrtho.com

# Springer Science+Business Media New York 2015 769

J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_34
770 N. Lightdale-Miric and C.P. de Roode

of children with cerebral palsy of the upper

Abstract
extremity.
Cerebral palsy (CP) is a group of permanent,
but not unchanging disorders that affect the
development of limb function and posture.
Introduction
Nonprogressive disturbances that occur in the
immature central nervous system (CNS) cause
Cerebral palsy (CP) is a group of permanent, but
characteristic upper extremity motor and
not unchanging, disorders that involve abnormal
movement disorders and are often accompa-
development of movement, posture, and motor
nied by sensory, perception, cognition, com-
function, causing limb activity limitation. These
munication, and behavior disturbances, as well
nonprogressive disturbances occur in the devel-
as by seizure disorders and other syndromes.
oping or immature central nervous system (CNS)
As the child matures, the long bones will grow,
(Rosenbaum et al. 2006; Bax 1964; Bax et al.
but spastic muscle will contract and cause
2005). The characteristic motor and movement
imbalance across joints.
disorders of CP are often accompanied by sen-
Evaluation of the upper extremity in a child
sory, perception, cognition, communication, and
with CP must include multiple visits and
behavior disturbances, as well as by seizure dis-
incorporate input from the child, their guard-
orders and other syndromes. These associated
ians, and all treating medical and therapy
impairments can be equally or more disabling
caregivers. An individualized treatment plan
than the movement disorder itself. The severity
to improve the childs independence, mobil-
of the associated disorders varies greatly and is
ity, and communication should begin with the
related to the location and nature of the CNS
patient and caregiver goals and expectations
lesion. The origin of the CNS disturbance is not
and involve this multidisciplinary team.
always known. The lesion is often related to ische-
Nonoperative treatments can aid the child
mia and premature birth, though almost half of
considerably but require a commitment to
patients with CP were born near or full term.
therapy visits, home exercise plans, bracing,
Prevalence of CP has remained stable over the
and consistency.
past 40 years at 13/1,000 live births, despite
In the carefully selected patient, surgery
advances in obstetrics and neonatology. There
will provide discrete benets over nonopera-
are ongoing genetic studies looking at the genetic
tive treatments for hygiene, form, and func-
basis of cerebral palsy in those patients lacking a
tional improvement in the upper extremity in
history of CNS insult and prematurity (Clark and
children with cerebral palsy. Meticulous eval-
Hankins 2003; Lawson and Badawi 2003;
uation for established risk factors, functional
Smithers-Sheedy et al. 2013). Involvement of
testing, brace trials, selective botulinum toxin
the upper extremity in CP affects all aspects of a
injections, video analysis, EMG, and appropri-
patients life and function, ranging from personal
ate use of imaging studies can avoid com-
hygiene and social acceptance to activities of
plications and increase the rate of achieving
daily living (ADLs) such as feeding, dressing,
treatment goals. Ongoing large multicenter
and communication (Strauss et al. 2007; Kocher
clinical studies and centuries of published lit-
and Zurakowski 2004).
erature have described a variety of surgical
Approach to the patient with CP should be
procedures. Performing multiple procedures
multidisciplinary and include the patient and care-
at the same time to rebalance the entire
givers as well as pediatrician, therapists, and
extremity is advocated.
orthopedic surgeon. Neurologist, hand surgeon,
This chapter discusses currently accepted
neurosurgeon, and physiatrist also add invaluable
techniques for the evaluation and treatment
dimension to understanding each childs medical
34 Cerebral Palsy
needs, prognosis, and quality of life. Develop-
ment of a treatment plan should begin with the
patient and caregiver goals and expectations and
involve the entire team (Chin et al. 2005).
This chapter will describe how CP affects func-
tion and posture of the upper extremity, as well as
review currently accepted nonoperative and oper-
ative therapies available.
Pathoanatomy, Physiology,
and Applied Anatomy
Injury to the immature or developing CNS leads to
the musculoskeletal changes observed in CP. The
brain disturbance is stable and typically does not
advance unless it is due to an expanding mass.
Limb voluntary control, joint contractures, and dis-
use atrophy, however, can change with growth over
time. The severity of limb functional impairment
correlates with the CNS lesions in location and
magnitude (Krageloh-Mann and Horber 2007;
Horridge et al. 2011). MRI and ultrasonography
are the primary methods of neuroimaging in use to
evaluate and characterize the brain disturbance. It is
has been recommended to obtain imaging in all
patients, to aid in characterization of patients
based on their pattern of brain disturbance. The
information gained from neuroimaging will help
medical care providers anticipate the severity of the
movement disorder as well as associated disorders
(Horridge et al. 2011; Himmelmann and Uvebrant
2011; Rosenblum et al. 2006).
Physiology of the motor impairment in cere-
bral palsy involves aberrant muscle tone. The
brain disturbance in CP leads to loss of selective
motor control and muscle power resulting in an
imbalance between opposing muscle groups,
irregular forces across joints, and abnormal mus-
cle tone (Moberg 1976).
Muscle tone can be characterized as normal,
hypotonic, hypertonic, or rigid. It can be further
characterized by the nature of the tone as spastic,
dyskinetic, or ataxic. Dyskinetic tone can be further
differentiated into dystonia and choreoathetosis.
Most patients have mixed tone, exhibiting features
771
of weakness, dystonia, choreoathetosis, ataxia, and
spasticity. Typically, the child will have a predom-
inant type of tone or movement abnormality
(Rosenblum et al. 2006; Sanger et al. 2010).
Spasticity is velocity-dependent resistance to
stretch, though patients with spasticity may have
some increased tone at rest as well. Spastic mus-
cles have muscle spindle shortening and stiffness
and muscle tendon unit contractures (Chambers
et al. 2009; Wu et al. 2010). Spastic muscles can
be weak or strong. Spasticity leads to decreased
range of motion and over time joint contractures.
Dystonia in CP is a secondary dystonia. This is
important because secondary dystonias do not
respond similarly or reliably to therapies for pri-
mary dystonias. Denition of dystonia in CP is an
abnormal pattern of posture and/or movement,
which is involuntary, uncontrolled, and recurring.
It is predominantly hypokinetic with an increased
tone tendency (Monbaliu et al. 2010). The abnor-
mal postures are superimposed upon or substitute
for a voluntary movement. The movements may
be triggered by a specic posture. Dystonic pos-
tures are not seen during sleep (Sanger et al.
2010). This is an important distinction from cho-
rea, which is random and not triggered by volun-
tary movements. Cocontraction is considered a
feature of dystonia, but may not always be present
or responsible for the observed movement
(Malfait and Sanger 2007). Fortunately, cases in
which dystonia is the predominant tone are only
about 615 % of CP (Sanger et al. 2010).
Classication of CP is not universally agreed
upon. Classication is typically based on the pre-
dominant type of tone or movement disorder as
well as the anatomic distribution of body areas
affected. The terms diplegia and quadriplegia
have been used to refer to lower extremity
involvement and all four extremity involvement,
respectively. Hemiplegia refers to unilateral
involvement (Rosenblum et al. 2006). The upper
extremity is involved to varying degrees in the
majority of patients with CP. Classications have
been described for the wrist and ngers as well as
the thumb. These are included in their respective
sections below.
772 N. Lightdale-Miric and C.P. de Roode

Table 1 Modified Tardieu scale and modified Ashworth scale

Grade Modied Tardieu scale
0 No resistance to passive ROM
1 Slight resistance with no clear catch
2 Clear catch at precise angle, halting
passive ROM, followed by release
3 Fatigable clonus (<10s with resistance)
occurring at precise angle
4 Infatiguable clonus (>10s)
Modied Ashworth scale
No increase muscle to passive ROM
Slight increase in tone, catch, and release or minimal resistance
with ROM
Marked increase in muscle tone, catch in middle range and
resistance through remained of ROM, still easily moved
Considerable increase in muscle tone, passive movement is
difcult
Rigid in exion or extension

difcult later in the exam if the child becomes

Assessment of Cerebral Palsy
of the Upper Extremity
Signs and Symptoms of Cerebral Palsy
The most common position of the affected upper
limb in CP is one of shoulder internal rotation and
adduction, elbow exion, forearm pronation,
wrist exion and ulnar deviation, nger exion
or swan neck, and thumb in palm (Riad et al.
2007). The upper extremity in this position not
only looks palsied, but leads to impairment of
function and disabilities (Manske 1990). Disabil-
ity is more severe in those affected bilaterally.
Hemiplegic patients have difculties with biman-
ual tasks. The clinical examination to assess muscle
tone in patients with CP is critical to understanding
and predicting the outcomes of interventions.
Physical examination involves inspection, mea-
surement of strength and range of motion, assess-
ment of muscle tone, reexes, and functional
testing. A relaxed and cooperative child will render
the most reliable examination. Muscle tone, partic-
ularly spasticity, is exacerbated in an excited or
upset child. The key to obtaining the best exam is
having the child entertained or slightly distracted
by positive stimuli, such as a toy, tablet, or music.
Videotaping examinations, particularly those with
the skilled hand therapist, are helpful to the clini-
cian and for future comparisons of pre- and post-
intervention examinations (Leclercq 2003; Waters
et al. 2004; Carlson et al. 2009, 2012).
The examination begins by observing the child
in play and assessing active range of motion and
ability to perform functional tasks. This is more
uncooperative or overstimulated.
Range of motion of each joint should be
performed both actively and passively. Passive
ROM is evaluated both rapidly and slowly to
assess the changes related to spasticity. In spastic
CP, clonus and hyperreexia may also be present,
though clonus is not as common in the upper
extremity (Leclercq 2003).
Multiple grading systems exist for evaluating
and documenting spasticity. The Tardieu and
Ashworth scales are being the most well known,
and each has several modications. The authors
preferred method is a modication of the Tardieu
scale, recording the angle of catching at both
slow and fast velocities and describing the inten-
sity and duration of the muscle reex. The Tar-
dieu scales are the only grading systems that take
velocity into consideration (Scholtes et al. 2006)
(Table 1).
Dystonias are the more difcult type of tone for
the orthopedic surgeon to evaluate and treat, as it
does not allow for predictable outcomes to surgi-
cal interventions and in many cases is a contrain-
dication. However, dystonia often accompanies
spasticity to varying degrees. Cocontraction in
dystonia is not always apparent during initial
exam, particularly when spasticity predominates.
The practitioner is cautioned that interventions
may unmask dystonia with opposite results of
the intended procedure.
Multiple rating scales exist for dystonia and
have been tested for reliability and validity in
CP. None have been found superior. These include
the Barry-Albright Dystonia Scale, Burke-Fahn-
Marsden Scale, Burke-Fahn-Marsden Movement
34 Cerebral Palsy 773

Scale, and the Unied Dystonia Rating Scale Table 2 House functional assessment (adapted from
(Monbaliu et al. 2010). These scales are not com- House et al. 1981)
monly used by orthopedic surgeons in clinical Class Designation Activity level
practice. 0 Does not use Does not use
Strength is assessed using manual muscle test- 1 Poor passive Uses as a stabilizing weight
ing and compared bilaterally. Typical scales are assist
2 Fair passive Holds object placed into hand
05, 0-no contraction and 5-full range of motion
assist
against maximum resistance. Dynamometry may
3 Good passive Holds and stabilizes object
also be used (Crompton et al. 2007). assist for use by other hand
Selectivity of muscle or muscle groups is 4 Poor active Actively grasps object and
graded 02, where 0 is patterned movement, 1 is assist holds weakly
partially isolated movement, and 2 is completely 5 Fair active Actively grasps object and
isolated movement. assist stabilizes well
6 Good active Actively grasps object and
Reexes tested include deep tendon reexes of
assist manipulates against other
the biceps, brachioradialis, and triceps. Hoffmans hand
sign or increased nger exor reexes may be 7 Spontaneous Carries out bimanual
present and are consistent with an upper motor partial use activities easily and
neuron lesion. occasional spontaneous use
Associated injuries, such as sensory and 8 Spontaneous Uses hand independently
use complete
perception disturbances, are evaluated using
stereognosis testing. Stereognosis testing assesses
the perception/sensation impairment of the Table 3 Manual Ability Classification System (MACS)
extremity and is a valuable component to func- levels
tional testing and educational for patient and care- Level 1 Handles objects easily and successfully
givers. Gross and ne objects are placed into the Level 2 Handles most objects but with somewhat
patients hand, and patient is asked to identify the reduced quality and/or speed of achievement
object (Van Heest et al. 1993; Carlson and Brooks Level 3 Handles objects with difculty, needs help to
2009; Yekutiel et al. 1994; Goldner 1966; Gordon prepare and/or modify activities
and Duf 1999). Level IV Handles a limited selection easily managed
objects in adapted situations
Functional tests are many. Several have been
Level V Does not handle objects and has severely
validated in children as well as in the CP popula- limited ability to perform even simple
tion. These include: Manual Ability Classication actions
System (MACS) (Gong et al. 2010), the Shriners www.macs.nu
Hospital for Children Upper Extremity Evalua-
tion (SHUEE), the Jebsen-Taylor Hand Function
Test, House, and the Assisting Hand Assessment with cerebral palsy use their hands to handle
(AHA) (Davids et al. 2006). The House and AHA objects in daily activities and reects the childs
are specic to hemiplegia. Hemiplegic hand func- typical performance. It has been tested and vali-
tion can be documented in a busy upper extremity dated for use in children from 4 to 18 years of age.
clinic; using the House examination, see Table 2 It ascribes a level from I to Vof manual ability; see
(House et al. 1981; Morris et al. 2006; Van Heest Table 3 (Morris et al. 2006; Eliasson et al. 2006;
2003b; Bolanos et al. 1989). The AHA requires hrvall et al. 2014).
special training and more time to administer, but Outcome measures include the Canadian
also evaluates the extremity in usual situations of Occupational Performance Measure (COPM),
play and daily bimanual activities. which is validated for use in CP as well as pedi-
The Manual Ability Classication System atrics, and the Pediatric Outcomes Data Collec-
(MACS) was developed to describe how children tions Instrument (PODCI).
774
Electromyography (EMG)
Dynamic electromyography (EMG) is helpful in
determining the phasic nature of muscle spasticity
when entertaining the possibility of a muscle
transfer or release (Keenan et al. 1990). Though
some studies have shown that muscles can change
phase after transfer, it is our practice to attempt to
use in phase muscles for transfers when possible.
Transferring in phase muscle facilitates synergistic
movements and eases rehabilitation (Mowery
et al. 1985). EMG is also useful in determining if
cocontraction is present during specic tasks or
movements, particularly in patients with dystonia
(Malfait and Sanger 2007). Traditional EMG may
be used to determine underlying or resting muscle
activity. EMG can be performed using surface elec-
trodes or needle electrodes (Hoffer 1979, 1993a, b).
Cerebral Palsy of the Upper Extremity
Treatment Options
It cannot be overemphasized that the management
of patients with CP requires a multidisciplinary
team and must involve the patient and family/
caregivers in developing the childs individual-
ized treatment plan. Although they will be
discussed separately, nonoperative and operative
treatment is additive, and successful outcomes are
best achieved with integrated care approaches.
Nonoperative Management of Upper
Extremity Cerebral Palsy
Therapy
The importance of therapy in CP has been proven,
and patient outcomes regardless of the operative or
nonoperative intervention rely on adherence
to therapy protocols. Caregivers and patients
involvement in home exercise programs is critical
(Sakzewski et al. 2011, 2014; Scholtes et al. 2010).
Therapy is the mainstay of nonoperative manage-
ment, but is equally if not more important when
undertaken perioperatively (Speth et al. 2005).
Therapy modalities include strengthening,
stretching, functional tasking, ADLs, sensory
N. Lightdale-Miric and C.P. de Roode
education, and electrical stimulation. Therapists
use varying techniques including splinting, cast-
ing, ultrasound, and electrical stimulation
(Sakzewski et al. 2014).
Splinting and Casting
Splinting for functional assistance, such as wrist
stabilization in neutral position for hand function,
is common using both custom and prefabricated
orthoses. Splinting to prevent contractures is typ-
ically used at night and is directed to the most
common deformities, such as elbow exion, wrist
exion, nger exion, and thumb adduction
(Louwers et al. 2011; Imms 2011).
Serial casting is used for contractures. This
may or may not be accompanied by concomitant
botulinum toxin injections or other
chemodenervation. Serial casting is performed
by the physician or qualied therapist, and casts
are changed weekly for 36 weeks. Casting is
followed by therapy and night splinting to main-
tain the position attained.
Constraint-Induced Therapy
Constraint-induced therapies (CIT) stem from use
in stroke patients to reeducate and organize the
injured and healing brain. There is some contro-
versy in upper extremity CP management of the use
of CIT. Several studies have documented improved
extremity position and function in the CP popula-
tion (Crocker et al. 1997). Protocols for CIT vary
widely, but involve some form of constraint of the
better functioning upper extremity and intense ther-
apy and functional training of the involved extrem-
ity. This may or may not be followed by bimanual
training/therapies (Sakzewski et al. 2011, 2014;
Eliasson et al 2014; Tona and Schneck 1993; Pierce
et al. 2002).
Electrical stimulation (E-stim) paired with
splinting or bracing and therapy protocols have
shown some promise in patients with spasticity
and without static contractures (Ozer et al. 2006;
Carmick 1993). This involves supercial elec-
trodes applied to the weaker extensor muscles,
such as the extensor digitorum communis and
the triceps and placing the exors on mild stretch.
Protocols vary in frequency and duration, but are
typically performed at home daily for 412 weeks
34 Cerebral Palsy 775

and then placed on a maintenance schedule. E-stim Cerebral palsy of the upper extremity
can also be a useful adjunct to chemodenervation, Nonoperative management chemodenervation
using E-stim to the triceps following botulinum Indications Contraindications
toxin injections to the biceps and brachialis for Focal or multi-segmental Widespread tone ! high
elbow exor spasticity (Scheker et al. 2003; Ozer spasticity doses
et al. 2006; Wright and Granat 2000a, b). Dynamic contractures Severe static
contractures
Cerebral palsy of the upper extremity Dystonia History of allergic
Nonoperative management therapy reaction
Indications Contraindications
Muscle balance across Pain, recalcitrant contractures
joints, joint posture Pharmacologic Management
Activities of daily Poor voluntary control of the Systemic management of the movement disorders
living and extremity
independence in CP should also take into account the many
Pre- and postoperative Noncompliance, limited associated disorders, such as epilepsy and behavior
care access, socioeconomic issues disorders. The medications may interact or overlap.
A physiatrist, neurologist, or developmental pedi-
atrician typically oversees and manages these ther-
Chemodenervation apies. Medications include baclofen (oral or
Chemodenervation is commonly used in the treat- intrathecal), benzodiazepines such as diazepam
ment of focal spasticity. Phenol, ethanol, and bot- and clonazepam, dantrolene sodium, and tizanidine
ulinum toxins are the most commonly used. (Watanabe 2009; Francisco et al. 2009; Bonouvri
Injections are intramuscular and may be guided et al. 2013; Nogen 1976).
by ultrasound (US), electromyography (EMG), Many of these medications cause sedation,
electrostimulation, or surface anatomy (Elovic weakness, and central depression. Therefore, their
et al. 2009). Botulinum toxin injections have use is often limited in the higher functioning
been studied both in the upper and lower extrem- patients, who do not tolerate or appreciate these
ities in the treatment of muscle spasticity and side effects. The medications are more often used
abnormal muscle tone. It has been shown to in the more involved and refractory patients.
improve position and range of motion and some-
times function. The effect on the muscle is Cerebral palsy of the upper extremity
temporary, and the temporal effect of the improve- Nonoperative medical systemic management
ment has not been consistently quantied, but Indications Contraindications
ranges from 3 to 12 months duration (Wall et al. Nonfocal and severe spasticity Focal spasticity
or dyskinetic CP
1993; Wallen et al. 2007; Barber et al. 2013). The
Associated disorders, i.e., Minimal cognitive
use of chemodenervation in dystonia is encourag- seizure disorder impairment
ing, where surgical interventions are of limited use- High surgical risks No associated
fulness. The botulinum toxin injections may help in disorders
delaying surgical releases and/or predict the results
of a tendon or muscle release (Autti-Ramo
et al. 2000; Van Heest 1997; Speth et al. 2005; Cerebral palsy
Barrett 2011; Sakzewski 2010; Koman et al. 1990, Physical/occupational therapy recommendations
1993, 2003, 2010; Friedman et al. 2000; Corry Maintain supple range of motion
et al. 1997; Anakwenze et al. 2013). Botulinum Prevent contracture
toxin injections may be used as an adjunct to Maximize independence
surgical procedures, to aid in postoperative mus- Bimanual tasks
cle spasticity and pain following muscle/tendon Adjunct to all other nonoperative and operative
interventions
lengthenings or transfers (Yang et al. 2003).
776
Nonoperative Management Outcomes
Resistance exercise strengthening in the lower
extremities of children with CP was compared to
traditional therapies in a randomized trial. The
experimental group had muscle strength increases
up to 14 %, but did not demonstrate improved
mobility (Scholtes et al. 2010). The effects of
wearing a thumb and wrist brace on bimanual
activities in children with hemiplegia noted sig-
nicant improvement in spontaneous use and
bimanual activities (Louwers et al. 2011).
Constraint-induced therapy (CIT) compared with
bimanual training (BIM) did not demonstrate sig-
nicant differences, but did show that CIT yielded
greater unimanual improvement and BIM yielded
improved bimanual function; thus, perhaps both
therapies play a role in functional improvements
(Sakzewski et al. 2011; Scheker and Ozer 2003;
Law et al. 1991).
Short-term outcomes in a randomized trial
have shown botulinum toxin A added to an inten-
sive therapy regimen has improved functional
outcomes compared to intense therapy alone
(Speth et al. 2005). E-stim and dynamic bracing
combined was compared with either therapy alone
in a randomized trial. The combined therapy
yielded functional and postural improvement
over either therapy alone; however, after 2 months
results faded. The authors concluded that the ther-
apy had to remain ongoing to maintain benets
(Ozer et al. 2006; Scheker et al. 1999; Scheker and
Ozer 2003; Hines et al. 1993). Recently, a multi-
center prospective study compared therapy alone,
botulinum toxin, and surgery in children with
hemiplegia. The children in the surgical group
had greater improvement in upper limb position-
ing compared to the other groups (Van Heest
et al. 2013).
Operative Treatment of Cerebral Palsy
Indications/Contraindications
Extensive interviews of the child, family, thera-
pists, and other caring physicians are requisite to
avoid complications and navigate surgical
N. Lightdale-Miric and C.P. de Roode
decision making in children with cerebral palsy.
Multiple clinic visits as well as observing the
patient during activities (functional testing,
videotaping) and/or ambulation are necessary to
capture such a dynamic clinical exam (Van Heest
et al. 1999). Use of preoperative botulinum toxin
injection trials into spastic muscles and EMG may
help predict surgical outcomes and set expecta-
tions for surgical results. Ultimately, surgical indi-
cations and timing must be individualized to each
child and caregiver team. The importance of the
patients age and growth remaining cannot be
overemphasized when formulating the treatment
plan. Longitudinal growth will increase the likeli-
hood of contractures and recurrence of contrac-
tures postoperatively (Swanson 1960, 1964, 1968,
1982; Mital and Sakellarides 1981).
In the carefully selected patient, surgery will
provide discrete benets over nonoperative treat-
ments for hygiene, form, and functional improve-
ment in the upper extremity in children with
cerebral palsy (Waters and Van Heest 1998;
Stelling and Meyer 1959; Skold et al. 2007;
Roth et al. 1993; Pontn et al. 2011; Johnstone
et al. 2003; Dahlin et al. 1998). Ongoing large
multicenter clinical studies and centuries of
published literature have described a variety of
surgical procedures for improvement in the posi-
tion. Overwhelmingly, performing multiple pro-
cedures at the same time to rebalance the entire
extremity is advocated (Smitherman et al. 2011;
Samilson 1966; Samilson and Green 1972;
Nylander et al. 1999; Hoffer 1989, 1986;
Gelberman 1991; Eliasson et al. 1998). Below,
listed by anatomic location, are the indications,
contraindications, techniques, pitfalls, pearls, and
postoperative protocols for the most commonly
utilized procedures in reconstruction of the upper
extremity for children with cerebral palsy
(Zancolli and Zancolli 1981; van Munster
et al. 2007, 2009; Szabo and Gelberman 1985;
Samilson and Morris 1964; Pollock 1962;
Goldner 1955, 1961, 1974, 1979, 1983, 1987,
1988).
Dyskinetic movement disorders, continuous
spastic muscle tone, poor distal sensibility and
stereognosis, and limited voluntary control of the
34 Cerebral Palsy
upper extremity are relative contraindications to
soft tissue procedures. These ndings guide surgi-
cal decision making toward nonoperative treatment
and/or fusions and salvage procedures (Skoff and
Woodbury 1985; Manske and Strecker 1996; Lynn
et al. 2009; Lomita et al. 2010; Cooper 1952).
Surgical Procedures
Elbow
Preoperative Planning
The elbow positions the hand in space for func-
tion. If the hand cannot rest on the tabletop or
reach out for objects secondary to elbow exion
contracture, elbow contracture release may
increase upper extremity function (Morrey
et al. 1981). Children with cerebral palsy most
often demonstrate spasticity of their elbow exor
muscles (biceps, brachialis, and brachioradialis)
as well as weakness in their elbow extensors (tri-
ceps). This muscle imbalance can lead to exor
muscle and elbow joint contractures. With the
long bone growth of the humerus, elbow contrac-
tures can worsen during growth spurts and stabi-
lize with skeletal maturity. Finally, the elbow
crease can be a source of intertriginous infection
and skin breakdown. Elbow antecubital crease
hygiene is also an indication for surgery (Sherk
1977; Dy et al. 2013)
Preoperatively, the surgeon should determine
the structures responsible for the elbow contrac-
ture. Dynamic contractures, which correct with
passive stretch, can be improved with muscle or
tendon lengthening. Fixed exion contractures of
the elbow may require additional fasciotomies,
capsulotomies, myotomies, and skin releases
(Pletcher et al. 1976; Hotchkis 2011).
Upper extremity reconstruction in CP
Preoperative planning
OR table: regular
Position/positioning aids: supine/hand table
Fluoroscopy: required only for arthrodesis or osteotomy
Equipment: hand instrument tray
Tourniquet: sterile tourniquet upper arm
777
Surgical Approach
Elbow exion contractures are approached
through the antecubital fossae. The internervous
plane of dissection is between the biceps
(musculocutaneous n.) and brachioradialis
(radial n.). Across the antecubital fossae, the
biceps tendon lies lateral to the brachial artery
and the median nerve. In severe elbow exion
contractures, bowstringing of the neurovascular
structures can change the planes of dissection
and ultimately limit the elbow release. When com-
bined with forearm, wrist, or hand surgery, the
elbow contracture is addressed rst to allow for
improved positioning of the forearm for the more
distal procedures (Mital 1979).
Technique: Elbow Flexion Contracture
Release
The patient is placed supine on the regular OR
table with the entire upper extremity prepped,
draped, and outstretched on the hand table. If the
child has severe shoulder adduction contracture,
position the hand table where the elbow can be
accessed most comfortably. Tourniquet may be
used at the surgeons discretion. When used, a
sterile tourniquet is placed, the arm is
exsanguinated with an esmarch wrap with the
arm in extension, and the tourniquet is elevated.
Skin incisions can be a transverse antecubital
incision or a curvilinear incision or lazy S,
moving lateral proximal (to see the radial nerve)
and medial distal. In severe contractures, a well-
designed single or multiple Z-plasty may be nec-
essary to release antecubital skin pterygium
resulting from chronic xed contractures. All inci-
sion options are centered over the palpable
lacertus brosus and biceps tendon and extending
wide enough to visualize and protect the lateral
and medial neurovascular structures.
Once the skin is incised, antecubital veins are
mobilized and protected. The terminal branch of
the musculocutaneous nerve, the lateral
antebrachial cutaneous nerve, tracts just lateral to
the biceps tendon and is identied, protected, and
retracted. The lacertus brosus is identied and
divided, leaving the entire biceps tendon intact. If
the patient has a mild dynamic contracture,
778
fractional lengthening of the biceps may be
performed by dividing the tendinous portion
transversely at the musculotendinous junction,
keeping the muscle in continuity.
If the child has muscle contracture, joint
involvement, or moderate dynamic elbow spastic-
ity, then the biceps tendon is Z-lengthened. The
entire length of the biceps tendon is dissected and
visualized. A natural raphe exists splitting the
tendon. Open the raphe longitudinally, and cut
the medial half of the tendon proximally and the
lateral half distally. In a severe elbow contracture,
predict the expected lengthening needs for opti-
mized elbow position. In dynamic contractures,
limit lengthening to 2 cm to avoid extreme
weakening.
Through the arms of the biceps Z-plasty or
retracting the biceps if it is not lengthened, iden-
tify the median nerve and brachial artery and
protect them medially. The brachialis muscle is
then exposed. Dissect between the brachialis and
brachioradialis muscle to visualize the radial
nerve. A fractional lengthening of the brachialis
muscle is then performed based on severity of the
elbow involvement. The thickest part of the
brachialis tendon is lateral, right next to the radial
nerve. The tendon is divided transversely from
lateral to medial at the musculocutaneous junction
with two parallel incisions approximately one
centimeter apart (Manske et al. 2001).
Once the biceps and brachialis are lengthened,
assess the fascia over from the pronator mass and
extensor masses cross the elbow. In older patients,
this will thicken and be an additional obstacle to
elbow extension. The brachioradialis is then
exposed and evaluated. If you plan to use it as a
donor muscle for distal transfer, lengthening is not
recommended. If no transfer is planned, fractional
lengthening of the brachialis as it crosses the
elbow will allow additional elbow extension.
Complete brachialis myotomy is indicated
only in severe contractures for hygiene issues.
Performing the myotomy with a coated needle-
tip bovie may decrease postoperative bleeding. If
a substantial contracture is still present,
capsulotomy is performed. The anterior capsule
N. Lightdale-Miric and C.P. de Roode
can be released from the anterior humeral perios-
teum with an elevator while applying gentle
elbow extension force to facilitate capsular
release. Ultimately, the neurovascular bundles
medially and laterally will bowstring and may
limit the degrees of elbow extension obtainable.
In these cases, deate the tourniquet prior to
elbow casting, and place the elbow in a position
that ensures blood ow and capillary rell in the
hand. Translate the ends of the biceps tendon, and
repair them side by side, or weave them together
into the optimized lengthened position with 2-0
nonabsorbable suture.
The skin is then closed with absorbable deep
and subcutaneous suture. Consider placing a drain
if a myotomy was performed to prevent hema-
toma. A long arm cast or splint is applied. When
a mild dynamic elbow contracture release is
performed simultaneously with distal forearm,
wrist, and/or hand procedures, the distal short
arm cast will act like a weight, and patient can
begin gentle active exion of the well-repaired
biceps within a few days. If an extensive skin,
muscle, and/or joint contracture of the elbow has
been performed, use a cast or splint to maintain
elbow extension for a few weeks. If a forearm
procedure is performed, the elbow must be
immobilized with the forearm in the desired posi-
tion to prevent recurrence of forearm contractures.
See Fig. 1.
Elbow contracture release in CP
Surgical steps
Transverse, curvilinear, or Z-plasty skin incision
Identify and protect: antecubital veins, lateral
antebrachial cutaneous nerve, median nerve, brachial
artery, radial nerve
Transect the lacertus brosus
Fractional or Z-lengthen the biceps tendon
Fractional lengthening or myotomy of the brachialis
muscle
Expose and release the anterior capsule if needed
Release the fascia over the brachioradialis and exor-
pronator mass as they cross the elbow
Repair the biceps in a lengthened position if a Z-plasty
completed
Close the skin over a drain if myotomy of brachialis was
performed
34 Cerebral Palsy 779

Elbow exion contracture release in CP Surgical Pitfalls and Prevention

Postoperative protocol
Elbow exion contracture release surgery in cerebral
Dynamic contractures do not require casting or splinting
palsy
and can begin active range of motion immediately
Potential pitfalls and preventions
Plaster anterior splint or cast of elbow in maximized
extension for severe xed contractures with Potential pitfall Pearls for prevention
neurovascular checks Overlengthening the In dynamic contractures
If immobilized, keep in place x 46 weeks biceps tendon limit lengthening to <2 cm
May begin active and passive range of motion Long side-by-side repair
immediately following splint or cast removal or weave
Occupational or physical therapy protocols and home Postoperative hematoma Use of a coated needle-tip
exercise program to maintain results. Night splinting to bovie for myotomy
prevent recurrence Careful identication of
Activities of daily living training and return to sports at neurovascular structures
3 months and protection of
antecubital veins
(continued)

Fig. 1 (a) Preoperative photograph of elbow exion contracture. (b) Intraoperative positioning. (c) Immediate postop-
erative photograph. (d) Postoperative follow-up photograph
780
Elbow exion contracture release surgery in cerebral
palsy
Potential pitfalls and preventions
Potential pitfall Pearls for prevention
Neurovascular Limit retractors on
compromise from structures to avoid injury
bowstring and traction on
antecubital structures
Respect limit of
achievable elbow
extension with
neurovascular checks
postoperatively and splint
position adjustments
Musculocutaneous Neurectomy
Musculocutaneous neurectomy, or selective
peripheral neurotomy (SPN) for complete dener-
vation of the biceps and brachialis muscles
(Maarrawi et al. 2006; Sindou et al. 2007), is a
salvage procedure for elbow spastic deformity.
Active elbow exion is possible through the
remaining brachioradialis. It is contraindicated in
patients with functional elbows. Neurectomy
addresses only muscular spasticity and is rela-
tively contraindicated when xed contracture
exists or the elbow contracture must be
addressed in conjunction (Purohit et al. 1998).
Sensory decit in the lateral arm will result from
denervation of the lateral antebrachial cutaneous
nerve. Preoperative lidocaine block of the
musculocutaneous nerve or EMG can help
predict outcomes.
Technique: The patient is placed supine on the
operating table with the arm extended over a hand
board. The upper extremity and chest are
prepped with split drapes to midline to allow
access to the sterile axilla. A curvilinear incision
is made along the crease of the axilla. The fascia
over the brachial plexus cords and nerve branches
is opened, and the musculocutaneous nerve is
identied emanating from the lateral cord of the
brachial plexus next to the biceps tendon. The
nerve is followed distally toward its innervation
to the biceps muscle. The nerve is then transected,
and a one centimeter segment is removed. The
axillary incision is closed primarily. An adhesive
waterproof dressing or bulky shoulder wrap that
N. Lightdale-Miric and C.P. de Roode
incorporates the axilla is placed. The elbow is kept
clean and dry for 1014 days. No postoperative
immobilization is necessary.
Forearm Pronation Contracture
Preoperative Planning
The modern day workspace is predominately
tabletop with the forearm in pronation. Activities
of daily living such as hygiene, dressing, and
eating as well as most sports and instrument play
require forearm rotation into varying degrees of
supination. Spasticity of the exor-pronator mass
and pronator quadratus muscles as well as weak-
ness of the forearm supinators (biceps and supi-
nator muscles) positions the forearm in
hyperpronation. Operative treatment of forearm
dynamic and xed pronation contractures can
increase a childs independent and bimanual func-
tion (Gschwind and Tonkin 1992; Gschwind
2003; Colton et al. 1976).
On clinical examination or videotape func-
tional testing, the patient should be asked to
actively supinate. If the patient can actively supi-
nate to a functional position, no surgery is indi-
cated, and continued nonoperative strengthening
of the supinators should be pursued. If the child
cannot actively supinate but can be passively
supinated, then their forearm contracture is
dynamic, and pronator teres release or rerouting
is indicated (Manske and Strecker 1987). The
rerouting should only be performed if the pronator
teres is phasic with attempted supination on
dynamic EMG. If the pronator teres is continu-
ously active on EMG, release should be
performed (Strecker et al. 1988; de Roode
et al. 2010). If the child cannot actively or pas-
sively supinate, then the contracture is xed and a
pronator teres tenotomy and/or pronator
quadratus released, or forearm rotational correc-
tive osteotomies of both the radius and/or ulna
may be indicated (Sakellarides et al. 1981; Ezaki
and Oishi 2012).
Release of pronator muscles in combination
with a supinating wrist extension tendon transfer,
such as a exor carpi ulnaris (Green transfer) or
34 Cerebral Palsy
brachioradialis transfer, may result in overcorrec-
tion and decreased function. In addition, if the
pronator is released from its origin with a exor
origin slide, then it does not need to be released at
its insertion. Careful preoperative evaluation and
planning is required to prevent this complication
(Bunata 2006). Preoperative botulinum toxin
injection trial and/or EMG of the pronator teres
and quadratus muscles can be utilized to assess
individual muscle contribution and predict surgi-
cal outcomes. X-rays of the forearm are utilized to
assess radioulnar joint mechanical blocks to rota-
tion or if radial head dislocation, distal radioulnar
joint subluxation, or synostosis is suspected
(Ozkan et al. 2004; Cheema et al. 2006).
Positioning
The operating room setup, positioning, and prep-
aration are the same as that described for elbow
contracture release and most commonly part of
multiple procedures performed concurrently with
elbow, wrist, and hand reconstruction.
Surgical Approach
Most commonly, the pronator is approached at its
insertion on the midshaft of the radius through a
direct radial incision. If a concurrent forearm pro-
cedure such as a wrist and nger contracture
release is planned, surgical incisions may vary if
incisions for other planned procedures provide
access to the pronator teres and/or quadratus ori-
gins and insertions.
Technique: Forearm Pronation
Contracture Release
Topographically, the pronator teres can be pal-
pated as it inserts onto the radius as the most
supercial and lateral muscle of the exor-
pronator mass. Estimate the incision to access
the pronator insertion by tracing the muscle dis-
tally; this should bring you to a several centimeter
incision on approximately the midshaft of the
781
radius. Dissection through the skin will demon-
strate large forearm veins supercially than can be
dorsally mobilized or ligated. The lateral
antebrachial cutaneous nerve is identied on the
surface of the brachioradialis muscle and
protected laterally. The brachioradialis is swept
dorsally. Care should be taken to prevent retrac-
tors on the supercial radial nerve beneath the
brachioradialis. The long sweeping insertion the
pronator teres is found conuent with the perios-
teum of the midshaft of the radius. Dissect and
retract proximally to isolate the pronator muscle
and visualize its musculotendinous junction.
Tenotomy of the entire PT tendon is performed
under direct visualization. If the rerouting proce-
dure is planned, the PT insertion is lifted with a
slip of periosteum from the lateral radius. Once
the insertion has been lifted, dissection proximally
mobilizes the muscle to its neurovascular pedicle
to avoid kinking. Place stay sutures in the end of
the tendon. Use a suture passer, a right angle, or
curved Satinsky clamp (Pilling Surgical, North
Carolina, USA) to pass the tendon through the
interosseous membrane and around the dorsal
radius. By staying on the radius, the anterior
interosseous artery and nerve and radial artery
should not be at risk. Use suture anchors, or pass
the tendon on a free needle through bone tunnels
made with a K-wire or drill, at the level of the PT
insertion, and direct the tunnel from dorsal to
volar. Position the forearm in full supination.
Secure the tendon to the lateral dorsal radius
with nonabsorbable suture. Securing the
rererouted tendon may best be performed after
all other upper extremity procedures are com-
pleted so that inadvertent rupture of the repair
does not occur (Fig. 2). The forearm should still
be able to be passively pronated. The pronator
quadratus (PQ) should not be released concur-
rently with a rerouting, to avoid overcorrection
and loss of pronation.
If isolated pronator quadratus release is being
performed, the PQ release from the radial border
is performed through a radial styloid or volar
Henry approach.
After either PT/PQ release or PT re-rerouting,
perform primary closure, and an above-the-elbow
cast or sugar tong splint is placed to keep the
782
Fig. 2 (a) Exposure of pronator teres (PT) insertion. (b) Elevation of insertion in preparation for rerouting. (c) PT
insertion rerouted around the radius. (d) PT sewn through bone tunnels in the radius
forearm in supination for 46 weeks. Subse-
quently, refer to occupational therapy for remov-
able supination splinting for an additional 4 weeks
with place, and hold active and passive supination
and active pronation exercises. Encourage the
child to assume bimanual activities of daily living
and play that utilize supination and forearm
rotation.
Forearm contracture release in CP
Surgical steps
Curvilinear or oblique direct midshaft radial incision
along the PT. Distal radial incision or volar Henry
approach to access the PQ. Preoperatively consider all
incisions planned as the insertions for the PT and PQ may
be accessed through radial styloid or volar incisions made
for other concurrent procedures
Identify and protect the lateral antebrachial cutaneous
and supercial radial nerves lying anterior and beneath
the brachioradialis muscle, respectively
(continued)
N. Lightdale-Miric and C.P. de Roode
Forearm contracture release in CP
Surgical steps
Tenotomy or rerouting of the pronator teres muscle at its
insertion
During rerouting procedure, stay along the radius to
protect the interosseous ligament and neurovascular
structures. Secure the tendon to the dorsal lateral radius
through a bone tunnel with the forearm in supination
If necessary, extend the incision distally. Release the PQ
from its most radial attachment while actively supinating
the forearm
Forearm pronation release in CP
Postoperative protocol
Long arm cast in supination or initial sugar tong splint
46 weeks
Supination active place and hold
Begin bimanual activities after 6 weeks to encourage
supination
34 Cerebral Palsy
Forearm deformity correction in cerebral palsy
Potential pitfalls and preventions
Potential pitfall Pearls for prevention
Overcorrection Do not release all pronators
Recognize that FCU to ECRB is also
a supinating transfer
Careful pre-op evaluation of all
structures to predict outcomes
Undercorrection Identify possible joint mechanical
blocks to forearm rotation such as
radial head dislocation or synostosis
Avoid rerouting procedures in
patients with xed contractures
Wrist
Preoperative Planning
Wrist position in children with cerebral palsy is
intricately involved in nger deformity and over-
all hand function. Power grip and pinch cannot be
achieved through a exed wrist. Use of commu-
nication device or wheelchair can depend on wrist
position. Surgical correction of wrist deformity in
children with cerebral palsy can open the hand for
bimanual activities and increase function and
hand strength. However, due to the extrinsic n-
ger exors crossing the wrist and the almost uni-
form extensor weakness of the wrist and nger
extensors, overcorrection of wrist deformity can
actually worsen hand function. Therefore, surgery
for the treatment of wrist deformity should only be
performed after extensive evaluation of the indi-
vidual contribution of each wrist and nger exor
and extensor to predict the tenodesis effect after
correction of the wrist.
Deformity of the wrist is not only in the ex-
ion/extension plane but also in the radial/ulnar
deviation/coronal plane. Surgical correction of
lateral plane deviation can be achieved by
balancing the insertion location of tendons. Over-
time, bony deformity, degenerative changes, and
radiocarpal subluxation may occur. Joint resection
and salvage procedures can decrease pain
783
Table 4 Wrist and finger deformity: Zancollis
classification
Group 1 Active nger extension with <20o of wrist
exion
Group 2 Active nger extension with more than 20o
of wrist exion
Group 2a Active wrist extension with ngers exed
Group 2b No active wrist extension with ngers
exed
Group 3 Wrist and nger extension absent even
with full wrist exion
(Zancolli et al. 1983)
associated with deformity and arthritis. In addi-
tion, hyperexion causes difculty with volar
wrist crease hygiene and can yield skin break-
down and infection. Caregivers who dress and
clean children with severe wrist exion deformi-
ties are often satised with surgeries that place the
wrist in a neutral position (Van Heest and
Strothman 2009). Aesthetically, a hyperexed or
deformed wrist may be the most noticeable aspect
in a child with hemiplegia, and correction of wrist
position may increase the childs social con-
dence and use in bimanual tasks (Sakellarides
and Kirvin 1995).
A variety of procedures have been described to
correct wrist deformity in children with cerebral
palsy (Green 1942; Green and Banks 1962;
Carroll and Craig 1951). Reproducible and suc-
cessful outcomes depend on the surgeons careful
preoperative planning. Multiple clinical assess-
ments, goal-directed care, video or functional
analysis, interpretation of EMG, identication of
dystonia, as well as assessment of voluntary con-
trol of the limb and sensibility are all mandatory
prior to performing surgery for correction of wrist
deformity (Omer and Capen 1976).
Classification
While many classications of wrist deformity
have been described, the Zancollis classication
(Table 4) considers the composite effect of wrist
784
and nger tightness and can guide surgical man-
agement of wrist exion contractures in children
with cerebral palsy.
Positioning
The operating room setup, positioning, and prepa-
ration are the same as that described for elbow
contracture release and most commonly part of
multiple procedures performed at the same setting.
Fluoroscopy is used for bone procedures such as
arthrodesis and chondrodesis. Setup and trial imag-
ing of the wrist prior to prepping and draping is
helpful to plan location and position of the C-arm
as well as determine if large C-arm is needed to
visualize the entire distal forearm or mini C-arm is
adequate. If an elbow exion contracture release is
planned, this should be performed rst to allow
access to the forearm and wrist.
Surgical Approach
The wrist exors and extensors can be released,
lengthened, harvested, and transferred through
various approaches. Options include separate
small longitudinal, curvilinear, or transverse inci-
sions (large enough to protect nearby neuro-
vascular structures) or wide longitudinal forearm
incisions that expose the entire forearm anatomy.
Preoperative marking of all incisions for the fore-
arm, wrist, and ngers is imperative to prevent
small skin bridges between incisions.
Wrist Flexor Releases
In mild, dynamic wrist exion contractures, sim-
ple fractional or Z-lengthenings of the wrist
exors can be performed.
Technique: The supercial layer of the exor-
pronator mass can be approached through a long
ulnar border incision that is L shaped across the
wrist crease or through multiple smaller incisions
in the mid forearm. The fascia over each tendon is
kept with the volar skin to maintain the integrity of
N. Lightdale-Miric and C.P. de Roode
the volar forearm skin. The exor carpi ulnaris is a
workhorse tendon with relatively large power and
excursion. Harvesting the tendon from its inser-
tion on the pisiform or lengthening (fractional or
Z-lengthening) on the distal tendinous portion
requires careful protection of the ulnar artery and
nerve just deep to the tendon.
The exor carpi radialis can be fractionally
lengthened effectively within the large tendinous
portion of the muscle belly or Z-lengthened more
distally in the natural raphe of the tendon.
Overlengthening of tendons can be avoided by
placing the wrist in the desired extension position
and securing arms of the z at that level. Side-by-
side repair of the Z-lengthening or weave of the
tendon ends can be performed and secured with a
nonabsorbable stitch. The palmaris longus tendon
can be harvested for transfer, Z-lengthened, or
released with a tenotomy at the volar wrist crease.
Tendon Transfer for Wrist Extension
Wrist exion posturing that is passively correct-
able and more moderate (3045 ) can be managed
with tendon transfers (Beach et al. 1991). If the
wrist and nger extensors are weak, then the pow-
erful wrist exors will be primarily chosen for
donors. As these transfers also cross the wrist,
FCU or FCR transfers to the EDC can also
improve wrist extension. However, asking a ten-
don transfer to perform more than one task often
results in a disappointing outcome. If the nger
extensors are present and functioning, the FCU,
FCR, ECU, BR, PT, and PL are available for wrist
extension transfers. The recipient tendons are
either the ECRB or ECRL (Wenner and Johnson
1988). Due to its more radial insertion, insertion
into the ECRL will also correct coronal plane
deformity. If the ECU is contracted or spastic
and contributing to ulnar deviation of the wrist,
it should also be transferred more centrally. Pre-
operative serial clinical exams and EMG testing
for phasic, non-phasic, and continuous activity
will guide decision making (Kreulen and
Smeulders 2008). The ideal wrist transfer will
improve wrist and nger extension, permit nger
34 Cerebral Palsy
Fig. 3 The Green transfer is sutured with the wrist in 015 of exion. Photographs demonstrate the (a) harvest of FCU,
(b) Pulvertaft weave of FCU-ECRB, and (c) nal position after suture of the transfer against gravity
exion, decrease excessive palmar wrist exion,
and prevent wrist extension deformity. The FCU
to ECRB will be described below, but the princi-
ples of this technique can be applied to any num-
ber of transfers that have been described (Wolf
et al. 1998; Thometz and Tachdjian 1988;
Patterson et al. 2010; Carroll 1958).
Technique
The FCU is harvested through either a longitudi-
nal ulnar incision or through multiple small trans-
verse incisions. The muscular portion of the
tendon extends nearly to the wrist crease and
secures the tendon to the ulnar periosteum. This
makes harvest through small incisions challeng-
ing and frustrating. Through a long ulnar incision,
the sheath over the FCU is opened, and the FCU is
carefully circumferentially dissected from the
ulnar nerve and artery at its insertion into the
pisiform and cut transversely as distally as possi-
ble. The tendon is then dissected from its ulnar
periosteal attachments in a proximal direction
while protecting the ulnar neurovascular bundle.
A trial of passing the tendon around the ulnar
785
border and over the skin will demonstrate the
location of the dorsal incision.
Dorsally, an incision in line with the long n-
ger metacarpal is made several centimeters prox-
imal to Listers tubercle where the trial wrapping
allows several centimeters of overlap between the
FCU and wrist extensors. Using Listers tubercle
as an anatomic landmark, the EPL is located
within the third compartment, removed from its
sheath, and brought radial to the second compart-
ment. The second compartment is opened. The
ECRB and ECRL are separated. The wrist is
placed in exion, neutral, and extension, while
the position and tenodesis of the ngers is
reassessed. The FCU is passed from the volar
compartment through a capacious subcutaneous
tunnel around the ulnar border into the dorsal
wrist incision. The FCU is then woven into either
the ECRB or ECRL depending on the coronal
plane deformity. After the tendon is secured, the
proximal ECRB or ECRL may or may not be
transected. Wounds are closed, and the wrist is
held in maximal extension that allows nger exten-
sion with a splint or cast x 6 weeks (Fig. 3).
786
Flexor-Pronator Origin Muscle Slide
The exor-pronator origin muscle slide (Page
1923; Inglis and Cooper 1966, 1970; White
1972; El-Said 2001) elevates the origin and
advances distally the pronator teres and wrist
and digital exors. The exor-pronator mass that
originates from the medial epicondyle, therefore,
the slide can partially improve an elbow exion
contracture, as well. However, the exor-pronator
muscle slide is all or none, in that ne-tuning of connections may result in traumatic rupture and
the release is not possible. It is a technically
demanding procedure that requires experience.
The slide can cause excessive weakness of the
nger exors and overcorrection of pronation
deformity (Thevenin-Lemoine et al. 2013). The
exor origin slide may unmask weakness of wrist
extensors. Long bone growth and poor compli-
ance may result in contracture recurrence. It
should be reserved for patients with primary treat-
ment goals of hygiene and hand position, not
goals of increased usage.
Technique
A sterile tourniquet is placed on the upper arm.
The proximal skin incision is similar to an
extended cubital tunnel approach. The incision
starts on the medial arm approximately 5 cm prox-
imal to the medial epicondyle, curves anteriorly to
the medial epicondyle, and extends distally along
the ulnar border of the forearm to the wrist. Prox-
imally, the ulnar nerve is identied behind the
medial septum and traced distally to the cubital
tunnel. The medial intermuscular septum of the
arm is transected to allow anterior transposition of
the ulnar nerve at the elbow. The exor-pronator
muscle mass is dissected from the periosteum
over the medial epicondyle to the coronoid pro-
cess of the ulna. It is dissected en masse, with
release of the origins of the FCU and FDP from
the ulna and interosseous membrane. Care is
taken to protect the medial collateral ligament of
the elbow. Across the elbow, the ulnar nerve is
transposed anteriorly from the cubital tunnel, and
its branches to the FCU are identied and
protected. Connecting the release distally to prox-
imally, the entire mass is then lifted laterally and
N. Lightdale-Miric and C.P. de Roode
anteriorly. Moving laterally across the front of the
elbow, the lacertus brosus is divided, and the
median nerve and brachial artery are identied
and protected. The FDS and FPL origins are
released from the radius. Branches between the
ulnar artery and anterior interosseous artery are
maintained or carefully cauterized if necessary to
enhance mobilization. Branches from the anterior
interosseous nerve to the FPL and FDS are also
protected. Failure to recognize these deep arterial
postoperative bleeding. The elbow, wrist, and
digits are passively extended to stretch the wrist
capsular contracture and complete the release.
The brachioradialis can be reached most laterally
and released from the radius at its insertion if it has
not been released proximally as part of an elbow
exion contracture release. Protect the radial
artery and supercial radial nerve under the
brachioradialis during radial most dissection.
The ulnar nerve is transposed anteriorly before
closure. Tourniquet release and careful hemostasis
is essential after release and before closure.
Thrombin spray and a drain may be used to quell
bleeding associated with muscle dissection and
prevent postoperative hematoma. See Fig. 4.
The arm is immobilized in a splint for 6 weeks
with the elbow extended to 45 , the forearm supi-
nated, the wrist in maximal extension, and the
ngers in a resting posture. A removable splint is
continued for 4 additional weeks, allowing
removal for range of motion and therapy. After
2 months, splinting is converted for night use and
eventually discontinued unless there is a tendency
for recurrence of deformity.
Arthrodesis/Chondrodesis
and Proximal Row Carpectomy (PRC)
In the skeletally mature patient with weak wrist
extensors or rigid wrist exion contracture,
radiocarpal arthrodesis can provide hand position-
ing and improve volar wrist hygiene (Alexander
et al. 2000; Rayan and Young 1999). Less com-
monly, younger children who are not candidates
for soft tissue procedures or who have failed soft
34 Cerebral Palsy
Fig. 4 (a) Preoperative posture and intraoperative photographs of exor-pronator slide and thumb intrinsic release (b and c)
tissue procedures are candidates for radiocarpal
chondrodesis if they have enough remaining
growth. A concomitant proximal row carpectomy
will effectively lengthen the extrinsic nger and
wrist exors. The surgeons must trial and consider
the wrist fusion position with each patient to eval-
uate the resulting tenodesis effect on the nger
position. Most commonly neutral or mildly exed
position is preferred to augment digital extension.
Plates are most commonly utilized as they can
compress the radius to the carpal bones and
allow bicortical xation in the metacarpal and
radius. However, external xation techniques
have been described. With chondrodesis, remov-
able smooth K-wires are used to create the fusion
mass while preserving the physis (Fig. 5)
(Hargreaves et al. 2000).
Technique for arthrodesis: A longitudinal dor-
sal incision is made in line with the long nger
metacarpal. The EPL is identied behind Listers
787
tubercle, the sheath is opened, and the EPL is
rerouted radially. The second and fourth extensor
compartments are elevated subperiosteally expos-
ing the entire dorsal distal radius. The wrist cap-
sule is opened with a distally based ap. The
carpal bones are exposed. After the nger exten-
sors are protected, direct periosteal incision is
made over the long nger metacarpal. A rongeur,
osteotome, or burr is used to remove all cartilage
from the distal radius articular surface and carpal
bones. If the wrist is contracted greater than 45 ,
the proximal carpal row is removed, and the cap-
itate fused to the radius. On occasion, volar
release of any tight wrist exors may be necessary.
The plate is secured with the wrist in the desired
position. Compression and a capitate screw will
augment the fusion success rate. If thumb and
nger extensor transfers are also planned during
this procedure, they can be completed at this point
following plate xation. Wrapping the now latent
788
Fig. 5 Preoperative
photographs of wrist exion
deformity (a) at rest and (b)
in maximum extension
Fig. 6 (a) Preoperative radiographs in maximum extension. (b) Postoperative radiograph following wrist fusion
wrist extensors (ECU, ECRB, and ECRL) over
the plate but under any nger or thumb transfers
will protect them from friction and lessen the
chances of rupture (Fig. 6).
In skeletally immature patients, chondrodesis
or fusion of the carpal bones to the radial epiphy-
sis will allow the radial physis to continue to grow.
The technique is similar to arthrodesis with the
following exceptions. Exposure should be
extraperiosteal, paying particular attention not to
violate the periosteum near the physis. Smooth
pins and careful protection of the volar vascular
supply to the physis.
N. Lightdale-Miric and C.P. de Roode
Wrist arthrodesis for reconstruction for CP
Surgical steps
Dorsal longitudinal incision centered over long nger
metacarpal
Identify and protect neurovascular structures
Preparation of fusion mass with rongeur or burr
Stabilization with plate and screw construct
Lengthening or transfers
Cast 6 weeks
Postoperative care: Immobilization with cast
or splint for 46 weeks is necessary after all
wrist procedures. The position of the wrist and
ngers is individualized to limit tension on tendon
34 Cerebral Palsy 789

transfers, create stretch across lengthened tendons,

and allow bony healing or fusion. An interval cast Thumb
change may be indicated for wound evaluation,
especially in children who cannot effectively com- The adducted or thumb-in-palm deformity
municate and or have limited sensibility. Therapy severely restricts hand use for even the simplest
and night splinting are initiated, after discontinuing tasks. Treatment for thumb deformity can afford
immobilization. Therapy is directed at minimizing brace-free hand function, bimanual integration,
nger stiffness, promoting cortical remapping after and assisting hand use. While surgical treatment
tendon transfers, and incorporating activities of can improve function, enduring stabilization of
daily living. Home caregivers must integrate a the thumb in a child with cerebral palsy remains
home exercise program including active and passive one of the greatest challenges for even the most
range of motion and strengthening exercises daily. experienced surgeon. For soft tissue procedures,
Electrical stimulation and the use of intraoperative extrinsic and intrinsic muscles must be balanced
botulinum toxin in wrist tendon transfers have been against often severely attenuated joint stabilizers.
described to decrease postoperative pain, protect the Over time, the skin in the rst web space becomes
repair site, and facilitate rehabilitation. contracted and yields an additional deforming
force on the thumb (Smeulders et al. 2005; Inglis
Wrist reconstruction in CP and Cooper 1970; Gob 1972; Carlson 2002).
Postoperative protocol
Postoperative splint or cast with wrist and ngers in
positions appropriate for individualized procedures
46 weeks immobilization
Classification
Arthrodesis requires immobilization and x-rays to
demonstrate bony fusion at 6 and 12 weeks and therapy to The exact muscle forces causing the thumb-in-
prevent nger stiffness palm or spastic thumb deformity vary signi-
Tendon transfers and lengthenings require maintenance cantly from child to child. Overtime, joint con-
splinting, home exercise plan, training to use transfers tracture or laxity, skin tightness, and muscle
such as E-stim and biofeedback
balances can change. The House Classication
Brace-free extremity function is the ultimate goal
(House et al. 1981) of spastic thumb deformity
has proven the most reliable in identifying the
Cerebral palsy dominant contributors to types of thumb defor-
Potential pitfalls and preventions mity and guides surgical decision making
Potential pitfall Pearls for prevention (Table 5) (Sakellarides et al. 1995).
Overcorrection into wrist Preoperative planning
extension contracture including EMG,
assessment of childs
remaining long bone Preoperative Planning and Decision
growth, and presence of Making
dystonia
Failure to incorporate Preoperative and Decision making in the treatment of the thumb in
tenodesis effect resulting intraoperative testing.
cerebral palsy also requires signicant preoperative
in inability to open the Combine wrist exion
hand with the wrist in transfers with nger or evaluation and assessment of individualized patient
extension intrinsic muscle goals. Age of the child, available donor muscles,
lengthening joint hypermobility, and adduction severity of the
Neurovascular injury Longitudinal wide thumb deformity guide planning; see House Clas-
exposures. Multiple
transverse incisions only
sication, Table 5 (House et al. 1981). In addition
used by experienced to clinical exam and functional tests, botulism
surgeons toxin injections, EMG testings, and brace wear
790 N. Lightdale-Miric and C.P. de Roode

Table 5 Thumb classification and deforming factors, as extremity procedures. Fluoroscopy is used for
described by House et al. 1981 bone procedures such as arthrodesis and
Type I Simple metacarpal Adductor pollicis chondrodesis. Setup and trial imaging of the
adduction (AP) and rst dorsal hand prior to prep is helpful to plan location and
contracture interosseous (DI)
position of the C-arm as well as determine if large
Type II Metacarpal AP, rst DI, exor
adduction and pollicis brevis (FPB) C-arm is needed to visualize the entire distal fore-
metacarpal arm or mini C-arm is adequate.
phalangeal
(MP) exion
Interphalangeal Extrinsic Thumb Reconstruction
(IP) is mobile
Type III Metacarpal AP, rst DI, EPL
Surgical Approach
adduction, MP
hyperextension or Contracture and spasticity of the FPL against
instability weak or absent EPL, APL, and EPB muscles is
Type IV Metacarpal FPL +/ AP, rst the most common extrinsic cause of spastic
adduction, MP and DI, FPB
IP exion
thumb deformity. The FPL can be fractionally
or Z-lengthened in the forearm with other extrin-
sic nger procedures (Manske 1985). If the EPL
trials can be used to predict outcomes prior to is decient, then the PL to EPL transfer can be
surgical interventions. performed. The EPL acts as an adducting force,
A thumb adduction contracture is addressed due to its pull from ulnar to radial in the third
with soft tissue intrinsic and extrinsic exor and dorsal extensor compartment. The EPL can be
adductor lengthenings and releases (FPL, rst dor- transposed or rerouted through the rst dorsal
sal IO, abductor pollicis, opponens pollicis, FPB) extensor compartment to act more as an abduc-
paired with extensor and abductor strengthening tor. If the APL or EPB is decient, then FCR, PL,
transfers (FCR-APL, PL-APL, PL-EPB, and BR can be transferred to allow active thumb
BR-APL, BR-EPB, PL-EPL, BR-EPL, FCR-EPB) abduction and extension of the thumb across the
(McCue et al. 1970). If the joints are hypermobile CMC and MP joints (Rayan and Saccone 1996;
after rebalancing, then additional soft tissue stabi- Keats 1965). Tendon transfers from donors with
lization of the joints such as a capsulodesis or poor excursion or contractures will result in more
arthrodesis is added (Matev 1963, 1970). If the static results. Tendon transfers from the powerful
width of the rst web space is narrowed, then a or spastic FCR may result in overcorrection.
skin Z-plasty is performed as the approach to the
intrinsic releases. If dystonia exists, the thumb Technique: EPL Rerouting
remains adducted after previous efforts at soft tis- Identify the third extensor compartment at the
sue stabilization, and/or if the childs needs are level of Listers tubercle. Longitudinally open
purely hygiene related, then joint fusion at the the sheath over the entire EPL to avoid kinking.
CMC, MP, and/or IP may be indicated. Arthrodesis Extend the dorsal incision to expose the rst
or chondrodesis may be the index procedure if the dorsal compartment. Identify the APL and EPB
surgeon can predict that soft tissue stabilization is leaving a portion of the retinaculum to prevent
likely to fail and the childs functional and posi- bowstring. Dissect a long distally based slip of
tional goals can still be met (Silver et al. 1976; the APL, and wrap it around the EPL, and sew
Sakellarides et al. 1979; Chait et al. 1980). the slip back to itself to create a pulley. In addi-
tion, some authors advocate amputating the EPL
Positioning at the level of the MCP and securing the distal
portion to the extensor hood to create IP joint
The operating room setup, positioning, and prep- stability while reinserting the proximal EPL into
aration are the same as that described for all upper the dorsal extensor hood of the thumb metacarpal
34 Cerebral Palsy
for increased thumb abduction. All extrinsic inci-
sions are closed using inverted 3-absorbable
suture. A splint or cast is applied with the
thumb in abduction.
Intrinsic Thumb Reconstruction
Surgical Approach
Intrinsic thumb release can involve the FPL, the-
nar muscles (opponens pollicis, abductor pollicis
brevis, exor pollicis brevis), rst dorsal
interosseous and adductor pollicis, rst dorsal
extensor compartment muscles (abductor pollicis
longus, extensor pollicis brevis), and EPL (Tonkin
et al. 2008). The choice depends upon which
muscles are spastic and deforming. Joint and
bone procedures for stabilization of the thumb
include volar and dorsal approaches to the thumb
CMC, MCP, and IP joints. The incision(s) varies
with the proposed procedure (Smith 1982;
Mortens 1965; Hoffer 1983).
Technique
A four-ap or double opposing Z-plasty is com-
monly used to expose the intrinsic hand muscles
and release the rst web space skin contracture.
These aps can be separated or contiguous with a
thenar mid-palm curved crease incision to access
the thenar muscle origin or a dorsal thumb
S-shaped MCP joint exposure. The skin aps of
the Z-plasty must be thick and well vascularized
to prevent scar contracture of the web while
avoiding skeletonizing the neurovascular struc-
tures. Hypersensitivity of the rst web may per-
sist in children after extensive dissection or
traction of the digital nerves and prevent com-
fortable brace wear. The common artery between
the index and thumb will split more distally to the
nerve. Protecting the palmary neurovascular
structures, the fascia over the adductor can be
released on its palmar and dorsal surface. Dorsal
to the adductor, the rst dorsal interosseous can
be subperiosteally swept from its origin on the
second metacarpal with a freer or fractionally
lengthened at its musculotendinous junction.
Thumb MCP volar capsulodesis, ulnar collateral
ligament stabilization, or adductor insertion
791
procedures can also be performed through the
web space exposure.
In the thenar crease, the palmar fascia is divided,
and the supercial palmar arch and branching
median nerve are identied just distal to the carpal
tunnel. Deep to the arch, the long nger metacarpal
is identied and the origin of the adductor muscle.
These thenar and adductor muscles are released
from their origin, allowing the thumb to be brought
into abduction. Complete release of their origins is
necessary to prevent recurrent thumb adduction,
including the very distal transverse head origin of
the adductor and the very proximal limbs of the
opponens and short thumb exors.
Thumb-in-palm reconstruction for CP
Surgical steps
Fractional lengthening of FPL in the forearm if spastic
If EPL functional, perform rerouting procedure
If EPL decient, perform tendon transfer using BR, FCU,
or PL to the EPL, EPB, or APL
Four-ap or double opposing Z-plasty in the rst web
space with extension into thenar crease
Identify and protect neurovascular bundles to the thumb
and index ngers as well as mid-palmar arch, recurrent
motor branch, and median nerve in the carpal tunnel
Release the origin or insertion of the rst dorsal
interosseous +/ the FPB
Release the adductor pollicis origin subperiosteally from
the third metacarpal shaft and the thenar muscles from
their origin
+/ Release the A1 pulley of the FPL and perform a
capsulodesis
+/ Extend the incision dorsally over the MCP to
perform an arthrodesis or chondrodesis
Thumb Joint Capsulodesis,
Arthrodesis, and Chondrodesis
Surgical Approach
The thumb MCP joint is approached from the
volar side for a capsulodesis or volar plate
arthroplasty and from the dorsal side for an
arthrodesis or chondrodesis. The thumb IP joint
is approached dorsally through an H-shaped inci-
sion (Schuurman and Bos 1993; Kowalski and
Manske 1988; Filler et al. 1976).
792
Technique: Capsulodesis
The volar crease of the thumb MCP joint may be
incorporated in one of the rst web space Z-plasties.
The skin is divided, and the neurovascular structures
are protected, especially the crossing radial digital
nerve. The neurovascular structures may already be
well visualized from the thenar crease exposure.
The A1 pulley is divided and the FPL is pulled
radially. The volar plate and capsule are lifted from
the insertion and advanced to insert more distally or
imbricated with nonabsorbable suture to the sesa-
moid bones. The thumb MCP joint is pinned to hold
a position of 20 exion. Gentle extension of the
MCP joint should be met with resistance.
Technique: Thumb MCP Arthrodesis or
Chondrodesis
A curvilinear incision on the dorsal skin of the
thumb MCP joint may be incorporated in the rst
web space contracture Z-plasty release incision.
The interval between the adductor pollicis aponeu-
rosis and EPB is identied. The EPB and EPL are
dissected longitudinally until they can be trans-
posed over the radial aspect of the joint to expose
the entire dorsal capsule. A distally based capsular
ap is created to aid with closure. The ap is lifted
to expose the MCP joint. The UCL and RCL are
released, and the MCP joint is hyperexed to
expose the volar lip of the phalanx and the head
of the metacarpal. The volar capsule is peeled away
from the bone to expose the entire joint surface. Use
a rongeur to remove all cartilage from the metacar-
pal head, leaving as much metaphyseal bone as
possible. Complete removal of the volar condyles
is needed. In an arthrodesis, the proximal phalanx
base is cleared of all cartilage with a curette or
rongeur until bleeding metaphyseal bone is
circumferentially exposed. A ball and cup reamer
set (often used in the foot) can also be used. In a
chondrodesis, only the calcied center aspect of the
epiphysis is denuded of articular cartilage, and the
physis is carefully protected (Goldner et al. 1990).
Two smooth 0.45 or larger K-wires are placed
retrograde through the base of the proximal pha-
lanx out the condyles of the IP joint. Compression
is then placed across the MCP joint in a position of
1520 of exion, and the wires are passed retro-
grade into the metacarpal and carpal bones. The
N. Lightdale-Miric and C.P. de Roode
distally based capsule ap is then advanced and
repaired to the periosteum of the dorsal metacar-
pal with more compression. This provides a
smooth surface for the EPL, which is then brought
back dorsally. The EPB is repaired to the adductor
aponeurosis to keep the EPL centralized. The
thumb is immobilized for 68 weeks until bony
fusion is achieved on x-ray (Fig. 7).
Thumb-in-palm reconstruction in CP
Postoperative protocol
Thumb spica cast or splint immediately following
surgery with minimal circumferential dressings
6 weeks for tendon transfers and 68 weeks for
arthrodesis. Remove smooth pins with clinical and
radiographic union
Convert cast to removable thumb spica. Begin therapy
with pinch, grasp training
Biofeedback, E-stim, and occupational therapy with a
home exercise plan
Remove brace and begin ADL training
Surgical pitfalls and prevention
Thumb adduction contracture reconstruction in CP
Potential pitfalls and preventions
Potential pitfall Pearls for prevention
Thumb-in-palm Careful preoperative planning of
deformity recurrence soft tissue balancing across stable
joints
Identify dystonia and
hypermobility
Trials with botulinum toxin, EMG,
and brace wear to predict outcomes
Avoid rst web space skin
contracture with well-designed,
vascularized double opposing or
four-ap Z-plasty skin aps
Growth plate arrest Smooth wires across physis
Use uoroscopy to visualize the
epiphysis and physis
Overcorrection Avoid unmatched donor and
recipient tendon transfers
Finger Deformity in Cerebral Palsy
Mild nger exion contractures and deformity
will limit dexterity, grasp, and pinch function of
the hand. Severe nger deformity may result in
poor hand hygiene with the ngernails cutting
34 Cerebral Palsy
Fig. 7 Photographs of (a) preoperative position, (b and c)
intraoperative/immediate postoperative position, (d)
thumb MP arthrodesis intraoperative, and (e) follow-up
into the palm or yeast and bacterial skin infections
due to skin breakdown. The balance of tone, spas-
ticity, and contracture of the intrinsic and extrinsic
nger exors against the weakness of the nger
extensors most commonly create exion of the
metacarpophalangeal, proximal interphalangeal,
and distal interphalangeal joints of the ngers.
With the tenodesis effect, wrist exion allows
793
position following FDS, FDP, FCR, and FPL fractional
lengthening, PT release, 1st web release, thumb MP
arthrodesis, and EPL rerouting
the spastic contracted nger exors greater excur-
sion. However, strength and dexterity are worse
with wrist exion compared to extension. There-
fore, surgery to correct nger exion must be
combined with wrist and nger extension
balancing (Smith 1975).
When wrist extensors are weak, the child will
utilize the nger extensors to extend the wrist.
794
This overpull will hyperextend the PIP and atten-
uates the volar plate. Combined with spasticity
and contracture of the intrinsic muscle of the
hand (interossei and lumbricals), this results in
subluxation of the lateral bands and eventual
swan neck deformities of the ngers. If the lateral
bands drift too dorsal, attempted nger exion
results in snapping of the bands or frank inability
to close the hand for grasp. Surgery to correct
swan neck deformity crosses multiple, often
contracted, and stiff joints; involves balancing of
multiple tendon forces; and can be challenging
(Parry 1976).
Many procedures have been described for n-
ger deformity in cerebral palsy. In general, chil-
dren with tight exion of the PIP or DIP during
wrist extension may undergo FDS and/or FDP
fractional lengthenings. If the nger extensors
are weak, this can be paired with an FCU or BR
to EDC tendon transfer. Children with swan neck
deformities may require central slip tenotomy, PIP
tenodesis, palmar plate capsulodesis, lateral band
translocation, intrinsic origin releases, lateral
band reroutings, or spiral oblique retinacular lig-
ament (SORL) procedures (Saintyves et al. 2011).
Finally, patients with severe hypermobility or
hygiene-related issues may undergo PIP or DIP
arthrodesis. Flexor-pronator origin slides (see
above technique) and supercialis to profundus
(STP) transfers will release nger exion contrac-
tures and improve hand hygiene but result in
limited active digit function in most patients
(Matsuo et al. 1990, 2001).
Multiple clinical assessments, goal-directed
care, video or functional analysis, interpretation
of EMG, identication of dystonia, as well as
assessment of voluntary control of the ngers and
sensibility are all mandatory prior to performing
surgery for correction of nger deformity.
Stereognosis, proprioception, and temperature
should be tested preoperatively and are independent
predictors of outcome after surgical reconstruction
of the ngers in cerebral palsy. Using clinical exam
and testing will identify deforming structures and
muscles; however, much of this aspect of treatment
in children with CP is surgeon familiarity with a
technique and experience. The more commonly
utilized procedures will be described.
N. Lightdale-Miric and C.P. de Roode
Finger Extrinsic Flexor Lengthenings
Lengthening of the extrinsic nger exors is best
completed in the forearm away from the pulley
system. Supple wrist and nger joints are
required. Simultaneous procedures that augment
nger extension strength may be indicated. As the
deep nger exors share muscle bellies,
overlengthening or underlengthening one digit
can lead to loss of function in surrounding ngers
via quadrigia. Some degree of weakness will
result from lengthening, but this can be improved
with therapy and growth. The amount of neces-
sary lengthening increases which each Zancollis
classication of wrist and nger deformity
(Zancolli et al. 1983). This must be assessed
before and after the nal wrist procedure to incor-
porate tenodesis.
Technique
An approximately 5 cm midline longitudinal skin
incision is made on the volar forearm in line with
the long nger at the level of the musculo-
tendinous junction of the nger exors (middle
third of the forearm). Alternatively, the entire
volar forearm may already be exposed through a
single longitudinal ulnar-based concomitant pro-
cedures being performed. The palmaris longus is
identied, and the supercial fascia of the forearm
is incised. The exor supercialis is organized
with the long and ring nger tendons just superior
to the index and small ngers. Gentle traction on
each tendon and individual nger PIP joint
motion is completed to identify each tendon.
Each tendon is traced independently well within
the muscle belly and fractionally lengthened once
or twice one centimeter apart. The median nerve is
identied, and the ulnar artery and nerve are
protected. Sweeping the FDS and median nerve
aside, the relatively at musculotendinous portion
of the FDP is exposed. Again, each nger is
moved at the DIP, and gentle traction on the ten-
don bers is performed to identify each nger
prior to lengthening. Fractional lengthening is
completed once or twice one centimeter apart
depending on the release needs. Tenodesis and
wrist extension are performed to evaluate the cas-
cade and conrm all ngers are balanced. Gentle
34 Cerebral Palsy
traction on the ngers into extension will conrm
that release has improved the childs composite
wrist and nger motion. Forced extension of the
ngers will tear muscle and result in increased
weakening and scarring. Fractional lengthening
of FPL is often performed concurrently.
Flexor-Pronator Slide
(See description in Wrist section).
Flexor Digitorum Superficialis
to Profundus Transfer (STP)
The STP transfer is indicated in the patient with
severe clenched st deformity (Braun and Vise
1973; Braun et al. 1974). In this deformity, the
ngers are exed into the palm and cannot be
passively extended even with the wrist in exion.
Fractional lengthening of nger exors will not
provide enough length/correction to open the hand
in severe clenched st deformity. The patient will
likely also have a thumb-in-palm and wrist exion
deformities. Concurrent exor pollicis longus
(FPL) Z-lengthening and wrist arthrodesis proce-
dures are common. The STP transfer allows the
surgeon to set the nger exor tension similar to a
Z-lengthening. The STP transfer may unmask
intrinsic spasticity, which leads to intrinsic tightness
and swan neck deformity (Heijnen et al. 2008).
Technique
Setup is the same as for procedures described
above. Sterile tourniquet is applied. The approach
is through a distal volar forearm incision and
oblique extension across the volar wrist crease
into the palm. Palmaris longus tenotomy is
performed. The median nerve is visualized and
gently retracted. The ngers are placed into a
natural cascade. The profundus tendons are iden-
tied and sewn together proximal to the level of
the wrist crease. The supercialis tendons are
identied and sewn together as distal as possible.
The wrist exors are Z-lengthened or prepared for
extensor transfer. The FPL is Z-lengthened, and
both proximal and distal limbs are tagged with
795
suture. A carpal tunnel release is performed. The
profundus tendons are sharply divided proxi-
mally, and the supercialis tendons are divided
distally. The wrist and ngers are extended such
that the wrist is neutral and the ngers are exed
30 at the MP and IP joints. The distal limb of the
profundus tendons is then sewn in a side to side
repair to the supercialis tendons. The wrist is
taken through passive range of motion to evaluate
tenodesis; the ngers should ex into the palm
with maximum wrist extension, and ngers
should open to allow for release with wrist ex-
ion. The FPL is then repaired with the wrist in
extension and the thumb lightly abutting the index
nger at the proximal interphalangeal (PIP) joint
in lateral pinch. The patient is placed into a long
arm split cast or splint, extending to the thumb and
ngertips for 46 weeks. Position of the wrist
(neutral or extension) depends upon other proce-
dures performed and surgeon preference.
Finger Extension Transfers
If both the wrist and nger extensors are
nonfunctioning, a transfer to augment nger exten-
sion can act as a wrist and nger extensor as it
crosses the wrist. The FCU has the most excursion
and power; however, if it is unsuitable for transfer,
then alternate donors may be used in select patients.
As stated previously, asking a tendon transfer to
perform two tasks is often disappointing.
Technique
The FCU is harvested as described in the wrist
tendon transfer section. Dorsally, an incision in
line with the long nger metacarpal is made sev-
eral centimeters proximal to Listers tubercle.
Using Listers tubercle as an anatomic landmark,
the EPL is located within the third compartment,
removed from its sheath, brought radial to the
second compartment, or rerouted as described
previously. The fourth compartment is opened,
leaving the retinaculum over the wrist joint and
hand intact to avoid bowstringing. Using 20
nonabsorbable suture, all four-nger extensor ten-
dons are sewn together in a natural tenodesis
cascade. The FCU is woven through each tendon
796
at this level. The entire conglomerate is sutured.
Tension is set using passive wrist exion and
tenodesis to gauge tension. Once satised, stan-
dard closure is performed, and the child is casted
for 46 weeks with the wrist and digits in exten-
sion. The IP joints can be left out of the cast.
Following cast removal, therapy and hand inte-
gration is instituted with night bracing to maintain
nger extension.
Lateral Band Rerouting
Once the extrinsic nger exor and intrinsics have
been balanced, the swan neck deformity may be
correct. If not, lateral band rerouting, FDS
tenodesis, central slip tenotomy, and other
described reconstructions can be used to prevent
PIP hyperextension and improve grasp and grip
palsy. The lateral band has been displaced dorsally
and is ineffective in this position. Mobilizing the
lateral band in a volar direction and constructing a
constraint to keep it positioned is called lateral
band rerouting. Once the lateral band is passing
beneath the PIP axis, the PIP may have a resulting
mild exion contracture. Overcorrection may cre-
ate a boutonniere nger deformity or a oppy DIP
joint (de Bruin et al. 2010).
Technique
A midlateral incision is made on the nger
(Tonkin and Gschwind 1992). On the index nger,
the incision can be made along the ulnar side to
avoid scarring, which could interfere with pinch.
The lateral band is separated from the extensor
mechanism proximal and distal to the PIP joint.
The accessory collateral ligament and transverse
retinacular ligament of the lateral band are divided
to expose the palmar plate. A palmar plate
capsulodesis or advancement may be performed
at this time if indicated. The pulley mechanism is
opened over the middle phalanx, keeping A2 and
A4 untouched. The radial slip of the FDS is iden-
tied. The mobilized lateral band is brought palmar
and placed between the palmar plate and radial slip.
4-0 nonabsorbable sutures are placed to tack the
palmar plate and radial FDS forming a slip that
keeps the lateral band palmar with PIP motion.
N. Lightdale-Miric and C.P. de Roode
The tension is tested with tenodesis of the wrist
and MP joint. A smooth K-wire may be placed to
keep the pip in exion and DIP in neutral. When
the pin and cast are removed, a silver ring or gure-
of-eight splint is placed, and exercises started to
restore active grasp and pinch.
Central Slip Tenotomy
PIP hyperextension results in dorsal subluxation
of the lateral bands. Central slip tenotomy and
exion of the PIP joint force the lateral bands in
a volar direction. DIP extension is maintained
through the SORL and insertion of the lateral
bands distally. Joint degenerative disease and
stiffness are relative contraindications. Excellent
outcomes can be achieved (Carlson et al. 2007).
Once the volar plate is attenuated, correction is
much more difcult.
Technique
The nger is approached via a dorsal transverse
once centimeter proximal to the PIP joint. The
dorsal nger veins are mobilized and protected.
The lateral bands and central slip are all identied.
The central slip is divided transversely while care-
fully protecting the lateral bands. The PIP is exed,
and the release continues until the bands fall in a
volar direction about the PIP joint. The nger is
often pinned across the PIP joint. The skin is closed
with absorbable suture. The K-wire is removed at
4 weeks, and the patient is converted to silver ring
of gure-of-eight splints, and range of motion exer-
cises begin. Eventually, the splints are for nighttime
use only and ultimately discontinued altogether.
Summary
The treatment of upper extremity surgery in chil-
dren with cerebral palsy can be extremely reward-
ing. Most critical is the multidisciplinary
approach to the treatment plan, thoughtful
workup, and surgeons experience both with the
examination of patients with abnormal muscle
tone and the selection of the appropriate operative
procedure(s) to achieve success.
34 Cerebral Palsy
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 Arthrogryposis
Upper Extremity Reconstruction for
35
Arthrogryposis

Dan A. Zlotolow and Scott H. Kozin

Contents Abstract
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 803 The care of the child with arthrogryposis
Assessment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 804 requires a global understanding of that childs
Treatment Options . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 806
needs and abilities. The results of any interven-
Shoulder . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 808 tion at one joint in the arm can be inuenced
Elbow . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 810 not only by the condition of other joints in that
Forearm . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 819 limb, but in the lower extremities as well.
Wrist . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 822
The Thumb . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 825
Treatment options at the shoulder are limited,
The Fingers . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 833 with a humeral rotational osteotomy performed
most commonly to address an internal rotation
Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 836
contracture. The elbow is most often posi-
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 836 tioned in extension, often requiring a posterior
capsular release with triceps lengthening and
ulnar nerve transposition to gain passive
motion. Active elbow exion is more difcult
to achieve. At the wrist, the carpal wedge
osteotomy has recently been shown to have reli-
ably good results in correcting wrist exion con-
tractures (J Bone Joint Surg Am. 2013;95(20):
e150; J Hand Surg Am. 2013;38(2):26570).
Correction of thumb deformities can reliably
improve pinch and grasp, while correction of
camptodactyly remains unpredictable.

Introduction

Any child with two or more joint contractures at

birth can be considered as having the diagnosis
of arthrogryposis. Arthrogryposis is purely a
descriptive term and does not specify disease
D.A. Zlotolow (*) S.H. Kozin
Shriners Hospitals for Children, Philadelphia, PA, USA severity or causation. The term cannot be applied
e-mail: dzlotolow@yahoo.com; skozin@shrinenet.org to contractures occurring after birth from
# Springer Science+Business Media New York 2015 803
J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_35
804
Fig. 1 A child with
amyoplasia demonstrating
the characteristic facies with
a hemangioma over the
nasal bridge (a). The
shoulders are often
internally rotated with the
elbows in extension and the
wrists in exion (b)
(Courtesy of Shriners
Hospital for Children
Philadelphia)
posttraumatic, neurogenic, or other postnatal causes.
Arthrogryposis can be further subdivided into three
categories: amyoplasia (Fig. 1), distal arthro-
gryposis (DA) (Fig. 2), and syndromic. Amyo-
plasia has a wide range of presentations but
remains an idiopathic disorder with some com-
mon ndings. The distal arthrogryposis category
encompasses 11 discrete inheritable disorders,
each with variable penetrance within a similar
pattern of presentation (Table 1; Hall et al. 1982;
Bamshad et al. 1996). The most common and
therefore typical distal arthrogryposes are types
1, 2A, and 2B (DA1, DA2A, and DA2B), often
distinguishable from each other clinically by their
distinctive facies. The syndromic category
includes over 300 central nervous system and
neuromuscular disorders (Fahy and Hall 1990).
Amyoplasia is the single most common type of
arthrogryposis. There is no known cause, but the-
ories include intrauterine crowding and failure
of anterior horn cell development. Maternal
bicornuate uterus and oligohydramnios have
been historically thought to increase the risk of
amyoplasia, supporting intrauterine crowding as a
potential cause (Miller et al. 1979). More recent
investigations have challenged the commonly
held belief that maternal uterine factors are a com-
mon cause of arthrogryposis (Hall 2013; Fahy and
D.A. Zlotolow and S.H. Kozin
Hall 1990). Children with amyoplasia often have
characteristic facies, with hemangiomas over the
bridge of the nose and over the occiput, high
cheeks, and button noses (Hall et al. 1983). The
hemangiomas, often referred to as stork bites, can
range from small, at erythematous skin lesions to
large, raised ruddy to bluish tumors that can
extend through the skull into the cranium
(Fig. 1a). While most hemangiomas resolve or
diminish with time, raised lesions should be
imaged to evaluate for intracranial extension.
Neurosurgical consultation may be appropriate
to evaluate large lesions. Most children have aver-
age to above-average intelligence. Diminution of
the motor cortex may allow expansion of other
cortical centers during development, possibly
resulting in higher than average intelligence.
Assessment
A global assessment of the childs cognitive, social,
and physical abilities is required. In addition, hav-
ing an idea of the family support structure and
available resources can help develop the treatment
plan. Early interventions are most likely to make a
positive impact, so early assessment by a surgeon
familiar with these disorders is the key to long-term
35 Arthrogryposis 805

Fig. 2 The more common

types of distal
arthrogryposis include
Freeman-Sheldon (a),
Sheldon-Hall (b), Beals
contractural arachnodactyly
(c), and Escobar syndrome
(d) (Courtesy of Shriners
Hospital for Children
Philadelphia)

success. A thorough intake worksheet such as the
one included in this chapter (Fig. 3) can help to
obtain a more complete picture of the childs needs.
All of the joints of the upper limb should be exam-
ined for both active and passive motion. Watching
the child play with a variety of differently shaped
and sized toys is a very efcient way of judging the
childs function. If possible, observe how the child
eats nger foods or drinks from a bottle. There is no
substitute for spending a few minutes to play with
the child to gain a good understanding of who they
are as people and what they require as patients.
An examination while the child is under anes-
thesia, whenever possible, is extremely helpful for
determining the true extent of the contractures
without muscular resistance. Also, it is a great
opportunity to localize potential donor muscles
for muscle transfers such as the latissimus dorsi.
Percutaneous electrical stimulation with or without
ultrasound guidance can be used to determine the
suitability of the pectoralis major, the latissimus
dorsi, and the gracilis for muscle transfer. Children
with arthrogryposis will typically visit the operat-
ing room several times for either spine or upper or
806 D.A. Zlotolow and S.H. Kozin

Table 1 Distal arthrogryposis types

Distal arthrogryposis types
Distal arthrogryposis type 1
Distal arthrogryposis type 2A
Distal arthrogryposis type 2B
Distal arthrogryposis type 3
Distal arthrogryposis type 4
Distal arthrogryposis type 5
Distal arthrogryposis type 6
Distal arthrogryposis type 7
Distal arthrogryposis type 8
Distal arthrogryposis type 9
Distal arthrogryposis type 10
Adapted from Bamshad et al. (2009)
Other name(s) Label Characteristic ndings
Common or typical DA1 Camptodactyly, clasped thumb, clubfoot
Freeman-Sheldon DA2A Whistling face, camptodactyly, clasped thumb,
syndrome clubfoot, scoliosis
Sheldon-Hall syndrome DA2B Prominent nasolabial folds, downslanting
palpebral ssures, small mouth, camptodactyly,
clasped thumb, clubfoot
Gordon syndrome DA3 Short stature, cleft palate
Scoliosis DA4 Scoliosis, camptodactyly
Ophthalmoplegia, ptosis DA5 Ptosis, strabismus, restrictive lung disease
Sensorineural hearing DA6 Hearing loss, camptodactyly
loss
Trismus- DA7 Trismus, pseudocamptodactyly, short stature
pseudocamptodactyly
Autosomal dominant DA8 Multiple pterygia, camptodactyly, scoliosis,
multiple pterygium ptosis, downslanting palpebral ssures
syndrome, Escobar
Beals syndrome DA9 Camptodactyly, arachnodactyly, kinked upper
(congenital contractural earlobe, tall stature
arachnodactyly)
Congenital plantar DA10 Plantar contractures
contractures

lower extremity correction before the age at which
they become good candidates for muscle transfers
(usually 6 years old or older), and an intraoperative
assessment prior to that age is most often possible.
Treatment Options
The goal of any treatment plan for a child with
arthrogryposis is to achieve functional indepen-
dence for that child. For the lower extremities, the
goals are ambulation and sitting. Traditionally,
ambulation has trumped all other treatment
goals. In many centers, the lack of expertise and
familiarity with upper limb surgery relegates
upper limb function to an afterthought. This is a
disservice to these children and to the adults who
care for them. Basic upper limb function is as
important to the well-being of these children as
ambulation.
For the upper extremity, the primary goals are
to have one hand to eat and one to wipe. They
can be the same hand. The rate-limiting step in
optimizing the childs independence is always
nger and thumb motion. Independent self-
feeding requires at least some hand function.
The child must be able to acquire the food, grip
it rmly enough to bring it to their mouth, and
release it. Beyond that, the wrist and the elbow
combined must have enough extension to reach
the plate or the table and sufcient exion to allow
the hand to reach the mouth. Forearm and shoul-
der rotation need to allow the hand to face the
plate and then the mouth.
Perineal care can be performed through the
legs or around the back. Both require sufcient
hand function to hold the toilet paper rmly. Chil-
dren who wipe their buttocks through the legs
require wrist exion, elbow extension, and shoul-
der internal rotation. Children who wipe their
buttocks around the back require forearm supina-
tion, elbow extension, and shoulder extension.
The next priorities on the functional ladder are
bathing and dressing, followed by food prepara-
tion, driving, and other activities of daily living.
Recreational activities and sports participation
35 Arthrogryposis 807

Upper Extremity Arthrogryposis Assessment

Chief Complaint _____________________

History of Present Illness
Age _______mo/yr Hand dominance R/L/Amb/unknown Male/Female
Gestational complications oligohydramnios/bicornuate uterus/multiples(twin, triplet,quad)/other_____________
Delivery breech/vertex/shoulder/C-section/other_____________
Diagnosis on prenatal US No/Yes(______wks)
Family history None/Distal Arthrogryposis (type ______________)/club feet/camptodactyly/other_____________
Other family members affected are mother/father/brother(s) age _____/sister(s) age _____
/other ______________
Respiratory Status never supported/required intubation from ______ to ______/required tracheotomy from
_______ to ______
Contractures at birth
Shoulder mobile/adduction contracture/internal rotation contracture/other ____________________
Elbow mobile/flexion contracture/extension contracture
Forearm mobile/supination/pronation contracture
Wrist mobile/flexion contracture/extension contracture
Thumb mobile/thumb-in-palm fixed/thumb-in-palm correctable
Fingers mobile/flexion contracture/extension contracture
Functional Assessment
Feeding (R/L/Amb) unable/finger foods/regular utensils/modified utensils/feet/other_______________
Perineal Care (R/L/Amb) unable/through the legs/around the back/assistive devices only
Writing (R/L/Amb) easy/difficult/with modified pen/with assistive devices_________
Ambulation unable/single arm crutch (R/L)/bilateral crutches/AFO/AKFO/HKFO/other__________________
Splints
Elbow extension/flexion night/day/full-time
Wrist extension/flexion night/day/full-time
Hand extension/flexion night/day/full-time

PMHx ____________________________________________________________________
PSHx ____________________________________________________________________
Meds ____________________________________________________________________
Allergies ____________________________________________________________________
SocHx ____________________________________________________________________
ROS cardiac -/+________________________ pulmonary -/+_____________________
Other

Examination
General well appearing/agitated/cooperative/uncooperative stork bite (yes/no)
Ambulation normal/stiff legged/with single arm crutch (R/L)/with bilateral crutches/with AFO/with AKFO/scoots
only/other______
Primary prehension, right elbow/forearm/wrist/hand/passive fingers/active grasp/active pinch
Primary prehension, left
Right side
Shoulder abduction active _____/passive ______ external rotation active______/passive______
Elbow flexion active _____/passive ______ extension active _____/passive ______ Pterigium yes/no
Forearm supination active _____/passive ______ pronation active _____/passive ______
Wrist flexion active _____/passive ______ extension active _____/passive ______
Thumb CMC flexion active _____/passive ______ CMC extension active _____/passive ______
MP flexion active _____/passive ______ MP extension active _____/passive ______
Fingers MP flexion active _____/passive ______ MP extension active _____/passive ______
PIP flexion active _____/passive ______ PIP extension active _____/passive ______
Camptodactyly index_______ long_______ ring_______ small_______
Core muscle strength pectoralis ____/5 latissimus ____/5
Sensation intact U M R/diminished to light touch U M R
Skin intact/scar(s) over shoulder/elbow/forearm/wrist/hand/thumb/fingers
Vascularity cap refill brisk/delayed

Left side
Shoulder abduction active _____/passive ______ external rotation active______/passive______
Elbow flexion active _____/passive ______ extension active _____/passive ______ Pterigium yes/no
Forearm supination active _____/passive ______ pronation active _____/passive ______
Wrist flexion active _____/passive ______ extension active _____/passive ______
Thumb CMC flexion active _____/passive ______ CMC extension active _____/passive ______
MP flexion active _____/passive ______ MP extension active _____/passive ______
Fingers MP flexion active _____/passive ______ MP extension active _____/passive ______
PIP flexion active _____/passive ______ PIP extension active _____/passive ______
Camptodactyly index_______ long_______ ring_______ small_______
Core muscle strength pectoralis ____/5 latissimus ____/5
Sensation intact U M R/diminished to light touch U M R
Skin intact/scar(s) over shoulder/elbow/forearm/wrist/hand/thumb/fingers
Vascularity cap refill brisk/delayed

Fig. 3 An intake worksheet can be very helpful to get a thorough assessment of the child (Courtesy of Dan A. Zlotolow, MD)
808 D.A. Zlotolow and S.H. Kozin

may be achievable in children who are mildly course of therapy is recommended nonetheless. The
affected. deltoid, rotator cuff, pectoralis, latissimus dorsi, and
peri-scapular muscles may be absent. The trapezius
Arthrogryposis nonoperative management: stretching is often preserved. Pectoralis function is key to
and splinting
bimanual activities, allowing the child to press
Indications Contraindications
both hands together. Latissimus function adds
Shoulder adduction None
contracture shoulder extension and active adduction for holding
Shoulder internal rotation None objects against the side between the arm and thorax.
contracture Deltoid and rotator cuff function allows abduction
Elbow exion contracture None and forward exion.
Elbow extension Less than 30 of passive Surgical options are very limited. Patients who
contracture elbow exion have sufcient musculature around the shoulder
Forearm pronation None
to enable tendon or muscle transfers usually have
contracture
Wrist exion contracture None
adequate function to not require them. Those chil-
Wrist extension None
dren with poor function rarely have available
contracture donors. Capsular releases have also not been
Digital contractures None effective in this population. Internal rotation con-
tractures are best treated with a humeral external
rotation osteotomy. The medial approach requires
Arthrogryposis physical/occupational therapy
visualization and retraction of the median and
recommendations
ulnar nerves, but is more cosmetic and provides
Begin passive range of motion as soon as possible
Fit for splints as soon as able to t onto patients limb
a better bony surface for plate application than the
Avoid splinting during the day to encourage limb-cortical deltopectoral approach (Kozin 2007).
development For the child with a severe elbow extension
Nighttime hourglass splinting for elbow extension contracture, distinguishing between an internal
contractures rotation contracture at the elbow and a pronation
Nighttime composite nger, thumb, and wrist extension contracture at the forearm can be difcult. For
splints
these children, we will perform an elbow release
Avoid supination straps and shoulder taping since this is
ineffective
rst, followed by a concomitant distal humeral
rotational osteotomy if needed.
Relative contraindications to a humeral exter-
Arthrogryposis nal rotation osteotomy include (1) insufcient or
Common complication Management marginal internal rotation for perineal care and
Peri-implant fracture Revision or (2) absence of pectoralis function. Children who
immobilization
lack pectoralis function but retain triceps function
Elbow ligament varus/valgus Stop PROM
laxity
will use a crossover grasp pattern that requires
Recurrence of contracture Revision or acceptance
internal rotation.

Humeral External Rotation Osteotomy

Shoulder
Weakness in shoulder girdle musculature is difcult
to treat and is unfortunately very common in chil-
dren with amyoplasia. Patients often present with
severe internal rotation and adduction contractures.
These rarely respond to passive stretching, though a
Place the patient supine on a standard operating
room table with a hand table. Regional anesthesia
is helpful to control postoperative pain and as a
supplement to general anesthesia. The incision
line is drawn along the medial intermuscular sep-
tum at about the middle of the brachium.
Bupivacaine with epinephrine is injected subcuta-
neously to the length of the incision. The arm is
35 Arthrogryposis
Fig. 4 Intraoperative
photograph (a) and
uoroscopic image (b) of a
humeral rotational
osteotomy performed via a
medial approach (Courtesy
of Shriners Hospital for
Children Philadelphia)
prepared and draped to the axilla. A tourniquet is
not routinely used due to the proximal extent of
the dissection. If one is needed, a thin circular,
non-pneumatic tourniquet can allow sufcient
exposure (HemaClear, OHK Medical Devices,
Grandville, Michigan, USA).
Incise the skin and raise full-thickness subcu-
taneous aps anteriorly and posteriorly to the
level of the fascia. The medial brachial and
antebrachial cutaneous nerves are routinely
encountered and should be protected. The basilic
vein runs with the medial antebrachial cutaneous
nerve and should be preserved as well. Continue
the dissection between the median and ulnar
nerves along the intermuscular septum. The
humerus will be palpable before it is visible.
Strip enough of the triceps muscle off of the
bone to clear a footprint large enough for placing
a plate. Plate sizes run from 2.4 to 3.5 mm for this
operation and should be appropriate for the size of
the bone. Children with arthrogryposis often have
humeri that are smaller in caliber than expected
for an unaffected age-matched child. Remember
to gently precontour the plate so as to provide
compression on the far side of the bone. Forget-
ting to do so can lead to a delayed union.
Apply the plate provisionally to the proximal
osteotomy fragment as anterior as the soft tissues
will allow. Remove the plate. Split the periosteum
longitudinally and apply Holman retractors to
protect the surrounding neurovascular structures.
Perform a transverse osteotomy using a recipro-
cating saw while preserving the periosteal sleeve.
Reapply the plate to the proximal fragment. Place
a fracture reduction clamp around the distal
809
fragment and the plate. Externally rotate the arm
to the desired position of between neutral to 15 of
internal rotation. Provisionally clamp the distal
humeral fragment to the plate and reassess the
rotation of the arm. Sufcient internal rotation
should be preserved to allow for midline function
such as perineal care. Fix the plate to the distal
fragment and reassess one more time (Fig. 4a). If
the position of the arm is optimized, conrm
screw lengths and plate position using uoros-
copy (Fig. 4b).
Close the subcutaneous layer and the skin in
standard fashion. Apply a sterile dressing with the
elbow in 90 of exion. Apply a long arm splint
with a side bar to prevent elbow extension. If the
xation was felt to be tenuous or the child is very
active, a triangular wedge can be used to place the
arm in a gunslinger position (Fig. 5).
Remove the splint 46 weeks after surgery and
outt with a fracture orthosis (Fig. 6) if union has
not been achieved. Begin a passive range-of-
motion protocol while maintaining the orthosis
at all other times until union.
Humeral osteotomy preoperative planning
OR table: standard
Position/positioning aids: supine
Fluoroscopy: mini C-arm
Equipment: size-appropriate compression plate
Tourniquet (sterile/nonsterile): none or HemaClear
Humeral osteotomy surgical steps
Longitudinal medial incision on mid-brachium
Identify and protect medial brachial and antebrachial
cutaneous nerves
(continued)
810
Fig. 5 A triangular wedge can be made out of rolls of cast
padding to maintain the arm in a gunslinger position
(Adapted from Zlotolow and Thompson 2011)
Humeral osteotomy surgical steps
Follow medial intermuscular septum to bone between
ulnar and median nerves
Apply plate proximally as anterior as the anatomy will
allow
Remove plate and split the periosteum longitudinally
Perform a transverse osteotomy
Reapply plate proximally
Rotate humerus to desired position
Apply plate distally
Close wound
Apply long arm splint or cast
Humeral osteotomy postoperative protocol
Type of immobilization: long arm splint  body wrap
Length of immobilization: 46 weeks
Rehab protocol: nger mobility and contralateral arm
motion
Return to sport protocol: 3 months
Humeral osteotomy potential pitfalls and preventions
Potential pitfall Pearls for prevention
Nonunion Minimal periosteal stripping
Maintain periosteal cuff in continuity
Precontour plate to compress the far
side of the osteotomy
Overcorrection
(continued)
D.A. Zlotolow and S.H. Kozin
Fig. 6 A fracture orthosis can be used to protect the
osteotomy site until union is achieved (Courtesy of
Shriners Hospital for Children Philadelphia)
Humeral osteotomy potential pitfalls and preventions
Potential pitfall Pearls for prevention
Make sure hand can comfortably
reach midline before nalizing
rotation
Nerve injury Visualize the median and ulnar nerves
and dissect between them
Subperiosteal dissection at the
osteotomy site to protect radial nerve
Elbow
Active elbow extension is preserved in most chil-
dren with arthrogryposis. However, since most
children lack adequate elbow exion strength to
oppose the triceps, extension contractures are
common. If the child cannot ex the elbow more
than 30 , the true axis of rotation of the elbow
joint can be difcult to determine. In these chil-
dren, well-intentioned parents, therapists, and sur-
geons bend the elbow out of its plane of motion,
most commonly leading to valgus instability. Pas-
sive elbow motion exercises, static or dynamic
elbow splints, and serial casting in a child with
no passive exion are contraindicated.
If the child can ex the elbow beyond 30 ,
actively or passively, supervised range-of-motion
35 Arthrogryposis
Fig. 7 An hourglass splint can be used to help gain more elbow exion both before and after surgery provided the patient
has at least 60 of exion to begin with (Courtesy of Shriners Hospital for Children Philadelphia)
exercises can yield sufcient exion for the child
to reach their mouth. An hourglass splint (Fig. 7)
can be very helpful to augment and preserve the
gains made with therapy. The achievement of
passive hand to mouth motion can be a life-
changing milestone for these children. Feeding
and grooming become possible via pushing their
arm against their knee or other arm, a desk or
table, or by abducting the shoulder and allowing
gravity to ex the elbow (Fig. 8).
If passive motion cannot be achieved with
therapy or if therapy is not possible due to inade-
quate initial motion, surgical release is a good
option. The results of triceps lengthening, poste-
rior capsular release, and ulnar nerve transposition
have been promising (Van Heest et al. 2008). Loss
of terminal extension and triceps weakness are
811
possible, but most children achieve sufcient ex-
ion to feed themselves. The results are most pre-
dictable in children under the age of three, but
releases in older children are still possible with
variable outcomes (Zlotolow and Kozin 2012).
Because of the potential loss of elbow exten-
sion after a release, bilateral releases are discour-
aged. Begin with the arm most likely to have an
adequate hand for feeding (the dominant hand).
Do not release the nondominant side until the
child demonstrates at least the potential to carry
out perineal care with the exed arm. Remember
the doctrine one hand to wipe and one hand to
eat, but it can be the same hand.
Active elbow exion can be more difcult to
obtain than passive exion, and the functional
benets over passive exion are less clear.
812 D.A. Zlotolow and S.H. Kozin

Fig. 8 Children can use

passive exion of the elbow
to feed themselves by
pushing their forearm
against their knee (a), a
table (b), or their other arm
(c) or by abducting the
shoulder and allowing
gravity to ex the elbow (d)
(Courtesy of Shriners
Hospital for Children
Philadelphia)

On occasion, an elbow release alone will allow a from active elbow exion, there are several
previously restrained biceps or brachialis to ex options for achieving active exion. Poor triceps
the elbow. The new active exor function is function is a contraindication to any transfer for
typically a grade 1 or a weak 2 and rarely provides active elbow exion. Patients who have had a
enough active exion to truly enhance function. In previous triceps lengthening typically have
a child with passive motion who would benet compromised triceps strength and are usually not
35 Arthrogryposis
candidates for active elbow exion transfers.
Transfer of the entire triceps to the biceps has
proven to be a poor option for analogous reasons,
with many children developing elbow exion
contractures (Van Heest et al. 1998).
Transferring only the long head of the triceps
may avoid the exion contracture that results from
transferring the entire triceps. Proponents of this
technique have yet to demonstrate a substantial
improvement in function from this transfer
(Gogola 2010). More experience with this transfer
is needed before it can be recommended for
widespread use.
Other options for restoring active elbow ex-
ion are a Steindler exorplasty (Goldfarb et al.
2004), a bipolar latissimus dorsi transfer (Zancolli
and Mitre 1973), a uni- or bipolar pectoralis major
transfer (Clark 1946; Carroll and Kleinman 1979),
and a free muscle transfer (Kay et al. 2010). Few
children with arthrogryposis have a sufciently
strong latissimus dorsi for transfer, but when the
muscle is present, it is the best choice for restoring
active elbow exion. The pectoralis major transfer
has also been described, and the muscle is often
present. However, the possible functional loss and
cosmetic concerns make it a clear second choice
(Carroll and Kleinman 1979; Lahoti and Bell
2005). Transfer of only the sternocostal portion
of the pectoralis major may be a better option from
an appearance standpoint but may not be of
sufcient strength.
On the other side of the spectrum, elbow ex-
ion contractures are even more difcult to treat.
Children born with a exed elbow may benet
initially from a passive range-of-motion protocol.
Most children with elbow exion contractures
lack triceps function, and many are decient in
active exion as well. A pterygium across the
antecubital fossa often accompanies the exion
contracture (Fig. 9) and heralds a more recalcitrant
problem. Elbow exion contractures have not
responded reliably to surgical release, and there-
fore, the procedure is not recommended.
Elbow Extension Contracture Release
Place the patient supine on a standard operating
room table. Regional anesthesia is helpful to
813
Fig. 9 Children with elbow exion contractures often
have a pterygium over the antecubital fossa (Courtesy of
Shriners Hospital for Children Philadelphia)
control postoperative pain and as a supplement
to general anesthesia. Considerable time is spent
dening the anatomic landmarks of the elbow,
particularly if there is little to no elbow exion.
The olecranon is identied by following the sub-
cutaneous border of the ulna proximally along the
forearm to the elbow. Next, the medial and lateral
epicondyles are palpated to conrm the location
of the olecranon. If there is any doubt, uoro-
scopic images are obtained.
The incision line is drawn along the subcuta-
neous border of the ulna, curving ulnar to the
olecranon, then running along the posterior aspect
of the arm. The incision should run the entire
length of the triceps tendon, at least midway up
the brachium. Bupivacaine with epinephrine is
injected subcutaneously the length of the incision.
The arm is prepared and draped to the axilla. A
tourniquet is not routinely used due to the proxi-
mal extent of the dissection. If one is needed, a
thin circular, non-pneumatic tourniquet does
allow sufcient exposure (HemaClear, OHK
Medical Devices, Grandville, Michigan, USA).
Incise the skin and raise full-thickness subcu-
taneous aps medially and laterally past the level
of the epicondyles. The ulnar nerve can be identi-
ed just proximal to the cubital tunnel beneath a
thick fascio-tendinous band of the triceps that
inserts onto the medial epicondyle. Transpose
814 D.A. Zlotolow and S.H. Kozin

Fig. 10 Releasing an
elbow extension contracture
requires transposition of the
ulnar nerve (a), creation of a
V-shaped distally based
triceps tendon ap (b),
release of the posterior
capsule (c), elevation of the
medial head of the triceps
(d), and repair of the triceps
in a V to Y fashion (e)
(Courtesy of Shriners
Hospital for Children
Philadelphia)

the ulnar nerve anteriorly into a subcutaneous
pocket and secure the pocket closed by suturing
subcutaneous fat to the anterior aspect of the
medial epicondyle (Fig. 10a). Make sure that the
medial antebrachial cutaneous nerve and its
branches are not included in the sutured fat.
After transposition, conrm that the ulnar nerve
is not kinked or compressed anywhere along its
new course.
Incise the triceps tendon along its entire length
in a distally based V shape (Fig. 10b), leaving
enough tendon on the outside of the V to later
perform a V to Y repair. Elevate the tendon ap
off of the muscle using bipolar electrocautery to
35 Arthrogryposis
Fig. 11 A humeral
rotational osteotomy can be
performed at the same
setting as a posterior elbow
release since the exposure
has already been achieved
in performing the release.
After the osteotomy is
performed, the plates are
reapplied to the distal
fragment and a clamp is
used to secure the
osteotomy for assessment of
rotation (a). Final
uoroscopic images are
used to check screw lengths
and plate placement (b)
(Courtesy of Shriners
Hospital for Children
Philadelphia)
its insertion onto the olecranon. Release any
remaining triceps attachments to the olecranon
and medial and lateral epicondyles. Following
the olecranon down to the olecranon fossa, release
the fat pad and the capsule, keeping these in
continuity with the triceps muscle. Release the
capsule along the medial and lateral gutters as
distally as possible without destabilizing the
medial and lateral ligaments (Fig. 10c). Continue
incremental releases until the elbow can ex past
90 . The maximum that is typically achievable is
less than 120 . Do not force the elbow into ex-
ion, as a transphyseal fracture is easily created. If
there is nothing left to release posteriorly or medi-
ally and laterally along the elbow, but elbow ex-
ion is still inadequate to reach the mouth, consider
an anterior closing wedge humeral osteotomy just
proximal to the olecranon fossa.
To optimize triceps excursion, elevate the
medial head of the triceps off of the posterior
humerus extraperiosteally to the level of the spiral
groove (Fig. 10d). The radial nerve can be local-
ized and protected by following the lower lateral
brachial cutaneous nerve to the spiral groove
(Zlotolow et al. 2006).
Assess the level of external rotation of the
shoulder with the elbow at maximal exion. If
passive external rotation is not within 30 of neu-
tral, consider performing a concomitant humeral
rotational osteotomy (Zlotolow and Kozin 2012).
815
The exposure has already been accomplished by
elevation of the medial head of the triceps. Con-
tour medial and lateral column plates to t just
proximal to the distal humeral physis, staying out
of the olecranon fossa. Staggered plates of differ-
ent lengths are recommended to minimize the
potential for a stress riser at the end of the plates.
Apply the plates provisionally to the distal
osteotomy fragment. Remove the plates. Split
the periosteum longitudinally and apply Holman
retractors to protect the anterior neurovascular
structures. Perform a transverse osteotomy using
a reciprocating saw. Reapply the plates to the
distal fragment. Place a fracture reduction clamp
around the proximal fragment and the plates.
Externally rotate the arm to the desired position
of between neutral to 15 of internal rotation.
Provisionally clamp the proximal humeral frag-
ment to the plates and reassess the rotation of the
arm (Fig. 11a). Sufcient internal rotation should
be preserved to allow for midline function such as
perineal care. Fix the plates to the proximal frag-
ment and reassess one more time. If the position of
the arm is optimized, conrm screw lengths and
plate position using uoroscopy (Fig. 11b).
Alternative xation schemes include medial
and lateral crossing pins and a single posterior
plate. The results of medial and lateral plating
are superior to either of the alternatives in our
hands, but no consensus exists. Pin xation
816 D.A. Zlotolow and S.H. Kozin

requires prolonged immobilization of up to Elbow extension contracture release preoperative

6 weeks, increasing the risk of losing elbow planning
motion. Single plate xation is both less rigid OR table: standard
than dual plates but also results in a more pro- Position/positioning aids: supine with arm across chest
nounced stress riser. A postoperative fracture just on bolster
proximal to the single plate is a common Fluoroscopy: mini C-arm
complication. Equipment: size-appropriate compression plates for
medial and lateral columns if osteotomy required
With the arm in maximal exion, repair the
Tourniquet (sterile/nonsterile): none or HemaClear
triceps in a V to Y fashion using a braided
nonabsorbable suture (Fig. 10e). Elevation of the
medial head of the triceps to the spiral groove will Elbow extension contracture release surgical steps
allow the triceps muscle to reach all the way to the Longitudinal dorsal incision from proximal ulna to
olecranon in most cases, facilitating the repair. If proximal brachium
the length of the V is insufcient to allow for a Identify and transpose the ulnar nerve subcutaneously
Incise and elevate the triceps tendon in a distally based V
primary repair, autograft or allograft may be used
ap
to bridge the gap. A properly planned triceps Elevate the medial head of the triceps from its origin
tendon ap and the medial head elevation will Release the posterior capsule of the elbow until at least
avoid this complication. 90 of exion is achieved
Reassess the course of the ulnar nerve prior to Assess the need for a rotational osteotomy
closing the wound. Close the subcutaneous layer Afx medial and lateral column plates distal to the
and the skin in layers. Elbow extension can facil- planned osteotomy
itate closure by taking tension off of the wound. Perform a transverse osteotomy proximal to the
olecranon fossa
Apply a sterile dressing with the elbow in maxi-
Reapply plates distally
mal exion. Apply a long arm splint with a side
Rotate humerus to desired position
bar to prevent elbow extension. If the soft tissue
Apply plates proximally
envelope is too edematous to allow for positioning Repair the triceps in a V to Y fashion
the elbow in maximal elbow exion, ex the arm Close the wound
to within what the soft tissues will tolerate and Apply a long arm splint or cast
immobilize in that position. If the xation was felt
to be tenuous or the child is very active, a trian-
gular wedge can be positioned to place the arm in Elbow extension contracture release postoperative
protocol
a gunslinger position.
Type of immobilization: long arm splint  body wrap
If soft tissue edema prevented splinting of the
Length of immobilization: 2 weeks
elbow past 90 of exion, perform a splint change
Rehab protocol: hourglass splint for 6 weeks and
under sedation 1 week after surgery to recover the A/PROM
missing exion. Otherwise, remove the splint
2 weeks after surgery and outt with an hourglass
splint (Fig. 10). Begin a passive range-of-motion Elbow extension contracture release potential pitfalls and
preventions
protocol while maintaining the hourglass splint at
Potential pitfall Pearls for prevention
all other times for 6 weeks. Continue range-of-
Loss of passive Alternate exion and extension
motion exercises through the fourth postoperative extension splints
month while weaning the hourglass splint to Loss of active Elevate the medial head of the
nighttime and naps only. If elbow extension is extension triceps to maximize excursion
proving difcult to recover, alternate an elbow Inability to repair Make as long V in the triceps as
extension splint with the hourglass splint. triceps tendon possible
Radiographs should be used to conrm bony Elevate the medial head of the
triceps
union between 4 and 6 weeks after surgery if an
(continued)
osteotomy was performed.
35 Arthrogryposis 817

Fig. 12 The bipolar

latissimus dorsi muscle
transfer is an excellent
choice for providing active
elbow exion, provided the
muscle is expendable and of
adequate strength. The
muscle is isolated on its
pedicle (a) and transferred
via a tunnel towards the
coracoid (b). The pedicle is
checked throughout the
procedure to make sure it is
not kinked, rotated, or under
tension (c) (Courtesy of
Shriners Hospital for
Children Philadelphia)

Elbow extension contracture release potential pitfalls and
preventions
Potential pitfall Pearls for prevention
Peri-implant Stagger the plates to minimize the
fracture proximal stress riser
Bipolar Latissimus Dorsi Transfer
After the patient is under general anesthesia, place
the patient in a lateral decubitus position on a
beanbag. The eld should be clear from midline
on the back to the nipple line on the front and from
the base of the neck to the iliac crest. Make sure
that all of the bony prominences are well padded
and that there is an axillary roll in place. The
peroneal nerve should be free of compression at
the level of the bular head.
Begin with an incision in line with the lateral
border of the muscle, from the posterior axillary
fold to about the midpoint of the posterior iliac
crest. A skin paddle may be kept on the muscle for
postoperative monitoring and to facilitate closure
if there is good muscle bulk. Since children
with arthrogryposis generally have a smaller
latissimus dorsi and abundant skin and subcuta-
neous fat in the arm, a skin paddle is not generally
necessary. If a skin paddle is used, it should be
small enough to allow primary closure of the
harvest site.
Create full-thickness subcutaneous aps to the
level of the latissimus dorsi. Working from distal
to proximal and lateral to medial, free the muscle
from its attachments, maintaining meticulous
hemostasis (Fig. 12a). Hematomas can occur at
the harvest site if there is inadequate hemostasis.
Identify the thoracodorsal artery, vein, and nerve
as they emerge deep to the latissimus and trace it
back to the axillary artery and the brachial plexus.
Ligate the vascular branches to the serratus to gain
maximal length on the pedicle. Identify and pre-
serve the long thoracic nerve to the serratus run-
ning near the vascular branches. More proximally,
the circumex scapular artery and veins rarely
need to be ligated, but doing so can add a small
amount of length to the pedicle.
Once the pedicle is isolated, separate the
latissimus from the teres major and expose the
insertion. The axillary nerve and posterior circum-
ex humeral vessels are immediately deep to the
latissimus and serratus near their insertion onto
the humerus. Place a Penrose drain around the
latissimus insertion and provisionally close the
donor incision with a few staples or towel clamps
to prevent desiccation of the wound.
Make an incision in line with the conjoint
tendon at the anterior shoulder large enough to
pass the latissimus. Expose the coracoid. Make an
S-shaped incision at the antecubital fossa and
818
dissect down to the biceps tendon. Surprisingly,
most children will have a biceps tendon that exes
the elbow when pulled proximally. The biceps
muscle is often completely absent, replaced by
fascial-type tissue. Create a generous subcutane-
ous tunnel from the distal to the proximal arm
incision and pass a Penrose drain to mark the
tunnel.
Returning to the axillary dissection, create a
subcutaneous tunnel deep to the pectoralis major
tendon to the proximal arm incision. Again, mark
the path of the tunnel with a Penrose drain. Detach
the latissimus insertion off of the bone with a
periosteal cuff, taking care not to injure the adja-
cent posterior circumex humeral vessels. Evalu-
ate the length and freedom of movement of the
pedicle one nal time before passing the muscle.
Pass the latissimus into the proximal arm wound
(Fig. 12b). It is safer to pass the origin side rst
since the origin is further from the pedicle than the
insertion and is therefore less likely to put tension
on the pedicle.
Check the tension on the pedicle as the muscle
is being transferred (Fig. 12c). Provisionally place
the insertion over the coracoid and recheck the
tension on the pedicle. The muscle should be
vascularized and the pedicle should be loose with-
out having been rotated. If the muscle is question-
able or the pedicle is tight, return the latissimus to
the harvest wound and revisit the pedicle to
achieve more length. There may be a tethering
vessel or a traversing band of axillary fascia lim-
iting pedicle excursion. The tunnel position may
need to be revised to shorten the distance the
pedicle must travel.
Afx the insertion of the latissimus to the cor-
acoid using braided, nonabsorbable sutures in
older children or polydioxanone monolament
suture in skeletally immature children. Pass the
origin of the latissimus into the distal wound.
There should be more than adequate length to
reach the biceps tendon or the proximal ulna if
necessary. Remove any excess muscle, then
tubularize the latissimus and suture the end to
the biceps tendon or directly to the ulna just distal
to the coronoid. The tension should be set so that
the elbow is difcult to extend past 30 of exion.
D.A. Zlotolow and S.H. Kozin
Recheck the pedicle and reevaluate the condi-
tion of the muscle. If a skin paddle was used, the
paddle can be used to evaluate the vascularity of
the ap. Alternatively, a Cook-Swartz Doppler
ow monitor (Cook Medical, Cook Ireland Ltd,
Limerick, Ireland) can be used. However, if the
pedicle is loose and the muscle is well perfused in
the distal wound, there is little need for postoper-
ative monitoring.
Close the wounds in standard fashion with one
or two large drains in the back, maintaining the
elbow in exion during the closure. Apply a ster-
ile dressing and then splint the arm in 90 of
exion. Apply a sling to support the arm.
The splint can be removed and therapy initi-
ated 46 weeks after surgery depending on the
childs age, child and family compliance, and
quality of xation. A hinged elbow brace should
be used at all times except for bathing. Initially,
the brace should be locked to prevent extension
past 90 . Each week, the extension is enhanced by
15 as long as active exion is maintained. By
around 3 months postsurgery, full extension
should be achieved and the brace can be removed.
Begin strengthening exercises once motion is
restored.
Bipolar latissimus dorsi transfer preoperative planning
OR table: standard
Position/positioning aids: lateral decubitus/bean bag
Fluoroscopy: none
Equipment: Cook-Swartz Doppler (optional)
Tourniquet (sterile/nonsterile): none
Bipolar latissimus dorsi transfer surgical steps
Incision from posterior axillary fold to mid-iliac crest
Dissect out the latissimus dorsi from lateral and distal to
medial and proximal
Isolate the neurovascular pedicle to the axillary artery
Identify the insertion of the latissimus
Incision over the coracoid
Incision across the antecubital fossa
Create a subcutaneous tunnel from the coracoid to the
biceps tendon
Create a tunnel deep to the pectoralis major from the
neurovascular pedicle to the coracoid
Detach the insertion of the Latissimus from the humerus
Pass the latissimus through the rst tunnel to the coracoid
(continued)
35 Arthrogryposis 819

Bipolar latissimus dorsi transfer surgical steps malrotation. The risk of misdiagnosis is greatest
Afx the tendinous insertion to the coracoid when the elbow is contracted in full extension. In
Pass the latissimus through the second tunnel to the these children, surgeon-directed uoroscopic
biceps tendon images have been helpful in determining the posi-
Afx the latissimus to the biceps tendon, removing any tion of the shoulder and the forearm. An elbow
excess muscle release can also make forearm and shoulder rota-
Close the wounds over drains tion much easier to examine. If the malrotation
Apply a long arm splint
arises from the shoulder, a humeral rotational
osteotomy can be combined with the elbow
release as described above. If the malrotation is
Bipolar latissimus dorsi transfer postoperative protocol
at the forearm, then a separate surgical procedure
Type of immobilization: long arm splint  body wrap
is performed to correct the pronation if necessary.
Length of immobilization: 46 weeks
Pronation is problematic because the hand
Rehab protocol: nger mobility and contralateral arm
motion faces away from the child, particularly in children
with ulnar deviation and exion at the wrist. Feed-
ing, grooming, and perineal care can often be
Bipolar latissimus dorsi transfer potential pitfalls and improved by placing the forearm in neutral. Supi-
preventions nation straps and passive range-of-motion exer-
Potential pitfall Pearls for prevention
cises are the rst-line treatments to improve
Pedicle under tension Dissect and isolate pedicle to
supination. If these fail, surgical correction can
the axillary artery
Redirect tunnel to coracoid
be achieved by a radial osteotomy, an ulnar and
Muscle inadequate for Test the latissimus radial osteotomy, or a one-bone forearm. Consult a
function preoperatively or therapist who is familiar with the child to help
intraoperatively with a nerve determine the best resting position for the forearm.
stimulator For rotational correction below 45 , a radial
Test the muscle using the osteotomy is usually sufcient. An ulnar osteo-
cough test
tomy is added for corrections up to 90 . Beyond
Unable to pass muscle Make a medial incision down
to the biceps tendon the entire brachium to that, a one-bone forearm is more predictable
connect the proximal and and easier to accomplish. A one-bone forearm
distal arm incisions may also best serve patients who lack any active
or passive motion and are stuck in extreme
pronation.
Forearm
Radial Osteotomy With and Without
Contrary to what has traditionally been described, Ulnar Osteotomy
children with arthrogryposis typically do not have Place the patient supine on a standard operating
pronation contractures. Most children have inter- room table with an arm table. Regional anesthesia
nal rotation contractures at the shoulder, creating is helpful to control postoperative pain and as a
the illusion of pronation at the forearm. The fore- supplement to general anesthesia. Conrm that
arm is most often in neutral. However, since each the patients position at rest is truly in pronation.
child is unique, the rotation of the shoulder and the If the shoulder is internally rotated on examina-
rotation of the forearm should be assessed inde- tion, consider a humeral osteotomy rst, then
pendently and in concert to determine the best reassess the forearm position.
overall treatment plan. Mistakenly, forearm The arm is prepared and draped to the axilla.
osteotomies have been performed on children to The incision line is drawn along the exor carpi
correct shoulder rotation, and humeral osteo- radialis (FCR) tendon just proximal to the wrist
tomies have been performed to correct forearm crease. A tourniquet on the upper arm is routinely
820
used. Incise the skin and raise full-thickness sub-
cutaneous aps medially and laterally to the exor
carpi radialis. Mobilize the tendon and incise the
oor of the FCR sheath. Elevate the exor pollicis
longus (FPL) and the proximal portion of the
pronator quadratus to expose the distal radial
diaphysis. Provisionally apply a contoured
six-hole size-appropriate compression plate to
the distal aspect of the radius with the distal
3 holes. Make sure that the plate is proximal to
the volar metaphyseal are to avoid physeal
injury. Remove the plate.
Split the periosteum longitudinally and apply
Holman retractors to protect the local soft tis-
sues. Perform a transverse osteotomy using a
reciprocating saw. Reapply the plate to the distal
fragment. Place a fracture reduction clamp
around the proximal fragment and the plate.
Supinate the radius to between neutral and 30
of pronation. Provisionally clamp the proximal
fragment to the plate and reassess the rotation of
the forearm. The hand should ideally be able to
reach both the mouth and the perineum. If the
child has insufcient shoulder rotation, elbow
movement, and wrist motion to achieve both
tasks, then determine which function is best
left to the contralateral hand, and optimize the
opposite function for the surgical arm. Fix the
plate to the proximal fragment and reassess one
more time.
If the radial osteotomy was insufcient to pro-
vide the desired amount of rotation correction, a
proximal ulnar osteotomy can be added. Incise the
subcutaneous border of the ulna distal to the level
of the coronoid. Insert the proximal 3 screws of a
six-hole plate. Remove the plate.
Split the periosteum longitudinally and apply
Holman retractors to protect the local soft tissues.
Perform a transverse osteotomy using a recipro-
cating saw. Reapply the plate to the proximal
fragment. Place a fracture reduction clamp around
the distal fragment and the plate. Supinate the
forearm to between neutral and 30 of pronation.
Provisionally clamp the distal fragment to the
plate and reassess the rotation of the forearm.
Fix the plate to the distal fragment and reassess
one more time. If the position of the arm is
D.A. Zlotolow and S.H. Kozin
optimized, conrm screw lengths and plate posi-
tion using uoroscopy.
Close the subcutaneous layer and the skin.
Apply a sterile dressing with the elbow in exion
and the forearm in the desired position. Apply a
sugar-tong splint or a long arm cast to prevent
forearm rotation.
Remove the splint 2 weeks after surgery and
convert to a long arm cast until union. Then begin
a passive range-of-motion protocol while maintain-
ing a Munster splint at all other times for an addi-
tional 6 weeks. Continue range-of-motion exercises
through the third postoperative month while
weaning the splint to nighttime and naps only.
Radial/ulnar osteotomy preoperative planning
OR table: standard
Position/positioning aids: supine
Fluoroscopy: mini C-arm
Equipment: size-appropriate compression plates
Tourniquet (sterile/nonsterile): proximal arm nonsterile
Radial/ulnar osteotomy surgical steps
Longitudinal FCR incision and approach
Apply plate to radius distally
Remove plate and split the periosteum longitudinally
Perform a transverse osteotomy
Reapply plate distally
Rotate radius to desired position
Apply plate proximally
If more correction needed, osteotomize the ulna
Close the wound
Radial/ulnar osteotomy postoperative protocol
Type of immobilization: long arm or sugar-tong splint
Length of immobilization: 46 weeks
Rehab protocol: nger mobility and contralateral arm
motion
Radial/ulnar osteotomy potential pitfalls and preventions
Pearls for
Potential pitfall prevention
Insufcient correction with Perform an ulnar
radial osteotomy osteotomy
Recurrence of deformity Create a one-bone
forearm
35 Arthrogryposis
Fig. 13 Creation of a one-bone forearm requires
osteotomies of the radius and ulna. The ulnar osteotomy
is made 1 cm proximal to the radial osteotomy (a) to allow
One-Bone Forearm
Place the patient supine on a standard operating
room table with an arm table. Regional anesthesia
is helpful to control postoperative pain and as a
supplement to general anesthesia. Conrm that
the patients position at rest is truly in pronation.
If the shoulder is internally rotated on examina-
tion, consider a humeral osteotomy rst, then
reassess the forearm position.
The arm is prepared and draped to the axilla. A
lazy S incision line is drawn along the FCR tendon
just proximal to the wrist crease, then curving
proximal across the volar forearm to end along
the volar ulna. A tourniquet on the upper arm is
routinely used. Incise the skin and raise full-
thickness subcutaneous aps medially and later-
ally to the exor fascia. Mobilize the FCR tendon
and incise the oor of the FCR sheath. Elevate the
exor pollicis longus (FPL) and the proximal
portion of the pronator quadratus to expose the
distal radial diaphysis. Use the same incision to
expose the ulna via the interval between the con-
tents of the ulnar and carpal tunnels. Elevate
enough exor digitorum profundus muscle origin
to visualize the ulna. Connect the ulnar and radial
exposures along the interosseous membrane, tak-
ing care not to damage the anterior interosseous
nerve and artery.
Provisionally apply a precontoured six-hole
size-appropriate compression plate to the distal
aspect of the radius with the distal three holes.
Make sure that the plate is proximal to the
821
for overlap of the cut bone ends once the distal radial
fragment is xed to the proximal ulnar fragment (b) (Cour-
tesy of Shriners Hospital for Children Philadelphia)
watershed line to avoid physeal injury. Remove
the plate.
Split the periosteum longitudinally and apply
Holman retractors to protect the local soft tissues.
Perform a transverse osteotomy just proximal to
the most proximal of the screw holes using a
reciprocating saw. In a similar manner, make a
transverse ulnar osteotomy 1 cm proximal to the
radial osteotomy (Fig. 13). The staggered
osteotomies create bony overlap to encourage
union. Reapply the plate to the distal fragment of
the radius. Place a fracture reduction clamp
around the proximal fragment of the ulna and the
plate. Supinate the radius to between neutral and
30 of pronation. Provisionally clamp the proxi-
mal ulnar fragment to the plate and reassess the
rotation of the forearm. The hand should ideally
be able to reach both the mouth and the perineum.
If the child has insufcient shoulder rotation,
elbow movement, and wrist motion to achieve
both, then determine which function is best left
to the contralateral hand, and optimize the oppo-
site function for the surgical arm. Fix the plate to
the proximal fragment and reassess one more
time. If the position of the arm is optimized,
conrm screw lengths and plate position using
uoroscopy.
Close the subcutaneous layer and the skin.
Apply a sterile dressing with the elbow in exion
and the forearm in the desired position. Apply a
sugar-tong splint or a long arm cast to prevent
forearm rotation.
822
Remove the splint 2 weeks after surgery and
convert to a long arm cast until union. Then begin
a passive range-of-motion protocol while
maintaining a Munster splint at all other times
for 6 weeks. Continue range-of-motion exercises
through the third postoperative month while
weaning the splint to nighttime and naps only.
One-bone forearm preoperative planning
OR table: standard
Position/positioning aids: supine
Fluoroscopy: mini C-arm
Equipment: size-appropriate compression plates
Tourniquet (sterile/nonsterile): proximal arm nonsterile
One-bone forearm surgical steps
Longitudinal FCR incision curving proximally to ulnar
forearm
Apply plate to radius distally
Remove plate and split the periosteum longitudinally
Perform a transverse osteotomy
Reapply plate distally
Perform a transverse ulnar osteotomy 1 cm proximal to
radial osteotomy
Place distal radius on proximal ulna
Rotate radius to desired position
Apply plate proximally on ulna
Close wound
One-bone forearm postoperative protocol
Type of immobilization: long arm or sugar-tong splint
Length of immobilization: 46 weeks
Rehab protocol: nger mobility and contralateral arm
motion
One-bone forearm potential pitfalls and preventions
Potential pitfall Pearls for prevention
Delayed union/ Stagger the osteotomies to create
nonunion bony overlap
Elbow instability Retain proximal radius
Wrist
The wrist position of children with arthrogryposis
varies dramatically, with some children even hav-
ing one wrist in exion and one in extension. In
general, however, most patients with distal
D.A. Zlotolow and S.H. Kozin
arthrogryposis tend to have wrist extension con-
tractures while patients with amyoplasia have
exion contractures. Extension contractures at the
wrist are well tolerated provided that the child has
sufcient nger extension to acquire and release
objects, ample forearm supination and elbow ex-
ion to get food to their mouths, and adequate fore-
arm supination, shoulder extension, and elbow
extension to be able to wipe their perineum from
behind. Wrist exion contractures can in some
cases enhance function if the conditions above are
not met. More than for any other joint in the upper
limb, the indications for altering a childs wrist
position require a thorough assessment of the
childs overall functional goals and limitations as
well as their active and passive motions about their
shoulder, elbow, forearm, and hand.
Initial treatment consists of passive motion
and splinting. Splinting during the day should
be avoided whenever possible to allow the child
to use their hands for play and to promote corti-
cal/muscular development, unless splinting
enhances function. Since surgical interventions
in the wrist and hand have not been demonstrated
to have better outcomes at a younger age, surgery
can be delayed until the childs functional needs
and capabilities are better dened (Foy et al.
2013; Van Heest and Rodriguez 2013). Surgery
should at least be delayed until the child fails to
progress with therapy alone, and perhaps even
after they are potty trained. Because wrist exion
is so critical to being able to perform perineal
care from the front, it is important for the child to
be able to wipe from around the back before
considering any procedure that limits wrist
exion.
Serial casting can improve passive wrist exten-
sion, but the gains are typically not maintained
(Smith and Drennan 2001). Extensor carpi ulnaris
(ECU) to extensor carpi radialis brevis (ECRB)
tendon transfers after serial casting may delay recur-
rence, but have not been demonstrated to maintain
the range of motion achieved with casting. The ECU
muscle is often affected, particularly if the extensor
digitorum communis (EDC) is not functional.
If the child has good nger extension and suf-
cient pronation and elbow exion to reach their
mouth without the need of wrist exion, and can
35 Arthrogryposis
Fig. 14 Children with limited wrist extension will use the
EDC to extend the wrist, leading to a zigzag deformity of
wrist exion, MP extension, and IP exion. These patients
are excellent candidates for a carpal wedge excision and
tendon transfer for wrist extension (2848082_18) (Cour-
tesy of Shriners Hospital for Children Philadelphia)
perform perineal care around the back, the most
reliable procedure to provide wrist extension is a
carpal wedge osteotomy with ECU to ECRB
transfer (Van Heest and Rodriguez 2013). In
cases where one hand is to be used for wiping
and the other for eating, the eating hand can be
considered for a carpal wedge osteotomy as long
as nger extension, forearm pronation, and elbow
exion are adequate. The maximal amount of
wrist extension that is desired is limited by the
ability of the EDC to extend the ngers (Fig. 14).
However, wrist extension past 30 , even if there is
good nger extension beyond that amount of wrist
extension, is not necessary.
Carpal Wedge Osteotomy
Place the patient supine on a standard operating
room table with an arm table. Regional anesthesia
is helpful to control postoperative pain and as a
823
supplement to general anesthesia. A tourniquet is
applied to the upper arm.
The arm is prepared and draped to the tourni-
quet. The incision line is drawn either longitudi-
nally or transversely across the dorsal wrist
(Fig. 15a). A tourniquet on the upper arm is rou-
tinely used. Incise the skin and raise full-thickness
subcutaneous aps. Beware the dorsal sensory
branches from the radial and ulnar nerves on either
side of the incision. Identify the ECU and follow it
out to its insertion at the base of the fth metacar-
pal. Release the sixth extensor compartment
(Fig. 15b). Make a separate incision just distal to
the mid-forearm over the ECU muscle, approxi-
mately at the level of the ECU musculotendinous
junction. Identify the ECU muscle and tendon. If
there is no ECU muscle or the excursion is very
limited, do not transfer the tendon. Release the
ECU from its insertion and retrieve the tendon
into the proximal wound. Divide any soft tissue
adhesions from the forearm fascia to the ECU that
may limit muscle excursion.
Park the muscle in the proximal wound and
return to the distal wound. Mobilize the extensor
pollicis longus (EPL) and elevate the second and
fourth extensor compartments and their contents off
of the wrist capsule and carpal bones. Divide the
midcarpal joint capsule longitudinally and extend
the incision along the capitate proximally and dis-
tally, staying out of the radiocarpal joint. Place a
Holman retractor deep to the capsule on the medial
and lateral carpal borders. In a child with minimal
carpal ossication, use a knife to incise a dorsally
and radially based carpal wedge. For older children,
use a reciprocating saw. The cut should correct both
wrist ulnar deviation and exion (Fig. 15c).
If there is any tension while placing the wrist in
extension, consider releasing or lengthening the
wrist exors. Palpate the exor compartment to
identify tight structures. Typically, the exor carpi
ulnaris (FCU) is the limiting structure. Make a
longitudinal incision centered over the tight
volar structures. If the tight exor tendon has no
proximal muscle attached, as is commonly
encountered in amyoplasia, divide the tendon. If
instead there is viable muscle present, lengthen
the tight tendons until the desired wrist extension
is achieved without tension.
824
Fig. 15 The carpal wedge osteotomy is ideal for
correcting exion as well as ulnar deviation wrist contrac-
tures (a). The ECU and ECRB tendons are identied (b). A
carpal wedge is excised centered over the midcarpal joint
with either a knife or a reciprocating saw (c). Kirschner
Fixation of the carpal wedge osteotomy can be
Kirschner wires (K-wires), transosseous sutures,
or a combination of both. Insert the K-wires
through the osteotomy in an antegrade direction
until they exit the distal hand (Fig. 15d). Likewise,
place the transosseous sutures before closing the
wedge (Fig. 15e). Close the wedge and drive the
K-wires retrograde across the osteotomy. Cross-
ing the radiocarpal joint with the K-wires may be
helpful to maintain the desired wrist posture. Tie
the transosseous sutures if used (Fig. 15f). Close
the capsule.
D.A. Zlotolow and S.H. Kozin
wires are advanced antegrade through the osteotomy site
(d) and transosseous sutures are placed (e). The osteotomy
is secured with a combination of sutures and K-wires (f).
The ECU is then transferred to the ECRB (g) (Courtesy of
Shriners Hospital for Children Philadelphia)
Identify the ECRB tendon and conrm that the
tendon glides across the retinaculum. If there is no
excursion, tenolyse the ECRB. Create a subcuta-
neous tunnel from the distal to the proximal
wound and retrieve the ECU to the distal wound.
The ECU can be transferred to the ECRB either
proximal or distal to the retinaculum (Fig. 15g). A
proximal transfer can help maintain wrist exten-
sion passively since the tendon mass may not pass
across the retinaculum. A distal transfer may
allow better exion, but will not provide a passive
block to exion. If the ECU is robust, good
35 Arthrogryposis 825

muscle excursion will allow for transfer distal to Carpal wedge osteotomy surgical steps
the retinaculum. If the ECU has limited excursion, Place 34 transosseous sutures through osteotomy site
transfer proximal to the retinaculum is Close down the osteotomy
recommended. The ECU can be woven into the Release tight volar structures as necessary
ECRB either with a single or multiple Pulvertaft Advance K-wires retrograde across osteotomy
weaves. A single weave is less bulky and has been Tie down transosseous sutures
shown to be equivalent in biomechanical testing Transfer ECU to ECRB
(Brown et al. 2010). Close wound
Close the wounds and apply a sterile dressing.
Deate the tourniquet and hold pressure for sev-
eral minutes. Apply a short or long arm splint or Carpal wedge osteotomy postoperative protocol
cast in maximal wrist extension. Type of immobilization: short or long arm splint or cast
If a splint was used, convert to a cast after Length of immobilization: 46 weeks
2 weeks. Remove the cast and the pins 46 Rehab protocol: nger mobility and contralateral arm
motion
weeks after surgery depending on the age of the
child and the expected time to union. Children
younger than 4 typically heal in 4 weeks, whereas
Carpal wedge osteotomy potential pitfalls and
those older than 8 require 6 weeks. If the carpal preventions
bones have not yet ossied, radiographs are not Potential pitfall Pearls for prevention
helpful in determining union of the chondrodesis. Inadequate extension Release tight volar
Fabricate an orthoplast splint. The splint should gains after osteotomy structures
be worn at all times except for bathing and therapy Poor ECU muscle with Search for other muscle
35 times a day. Therapy consists of active and little excursion donors such as FCR, FCU,
or FDS
passive wrist extension and active wrist exion.
Inadequate nger Only extend the wrist to
Digital motion should be encouraged. Two to opening after carpal where nger opening is
three months after surgery, transition to just night- wedge adequate and not beyond
time splint use for another 34 months. Weight Child unable to perform Do not perform this
bearing is allowed at 3 months after surgery. perineal care after operation on a child who
osteotomy uses that hand to wipe
Carpal wedge osteotomy preoperative planning between the legs
OR table: standard Child unable to nger Do not perform this
feed after osteotomy operation on a child who
Position/positioning aids: supine
requires wrist exion to
Fluoroscopy: mini C-arm reach their mouth
Equipment: Kirschner wires, suture
Tourniquet (sterile/nonsterile): upper arm, nonsterile

The Thumb
Carpal wedge osteotomy surgical steps
Transverse ellipse of skin excised over dorsum of wrist Thumb function is often affected in children with
Identify and protect radial and ulnar cutaneous nerves arthrogryposis, but the presentation can vary dra-
Identify the insertion of the ECU tendon matically. The classic clasped thumb with rst
Make another incision over the ECU musculotendinous carpometacarpal (CMC) joint extension and
junction
metacarpophalangeal (MP) joint exion is more
Cut the ECU at the insertion
commonly seen in distal arthrogryposis. In gen-
Mobilize the second and fourth extensor compartments
eral, the CMC joint follows the position of the
Expose the midcarpal joint
wrist; patients with wrist extension contractures
Excise a dorsal and radial closing carpal wedge
Place 2K-wires antegrade through osteotomy site
have a CMC joint extension posture. The MP joint
(continued)
can be passively correctable or contracted in
826 D.A. Zlotolow and S.H. Kozin

exion. Children with wrist exion contractures,
more commonly seen with amyoplasia, more
often have CMC joint exion contractures with
the MP joint in extension. Interphalangeal
(IP) joint motion is typically limited but most
often positioned in extension. A exion posture
at all three joints is less common (Table 2).
Initial treatment is passive range of motion
until the child is old enough to be t with a splint.
Splints are applied at night and during naps and
should attempt to expand the rst web space and
correct the posture of the thumb. The splints
should be designed to provide simultaneous cor-
rection of wrist and nger contractures as well.
Tremendous gains can be made with passive
motion and splinting, and the parents should be
Table 2 A classification of thumb contractures in children
with arthrogryposis. Subtype A is passively correctable
and B is not correctable
Joint position
Classication CMC MP
Type 1 Extended Flexed
Type 2 Flexed Extended
Type 3 Flexed Flexed
encouraged to be active participants in their
childs care. Gains can be seen up to approxi-
mately 5 years of age, but most children plateau
after their second birthday. For those children that
remain contracted after a supervised course of
therapy, surgical intervention can be considered.
A thumb in the palm is not a problem in and of
itself unless it gets in the way of nger exion.
Children with stiff ngers in extension may actu-
ally benet from having their thumb in the palm,
since the palm may be the only surface appropri-
ate for opposition. All surgical options should
seek to place the thumb in a position where it
can best provide pinch and/or grasp, regardless
of appearance. The surgical algorithm (Fig. 16)
depends on the posture of the CMC and MP joints,
as well as on the passive motion of the joints. If the
CMC joint is extended and the MP joint is exed
but passively correctable, a tendon transfer is
indicated provided that an expendable, functional
donor tendon is available. The preferred donor is
the extensor indicis proprius (EIP), but alternate
donors such as the palmaris longus, exor
digitorum supercialis (FDS), and brachioradialis
can also be used.

Fig. 16 Surgical algorithm

for thumb contractures in
children with
arthrogryposis. Subtype A
is passively correctable and
B is not correctable
(Courtesy of Dan
A. Zlotolow, MD)
35 Arthrogryposis
If the CMC joint is in extension and the MP
joint has a exion contracture that is not correct-
able, MP chondrodesis is the best option. Contra-
indications include a xed extension contracture
at the CMC joint that would preclude opposition
after the MP chondrodesis.
Children with a exion contracture at the CMC
joint with an MP joint extension posture should be
evaluated to ensure that the thumb motion is ade-
quate for some type of opposition pinch. The
thumb may oppose to one or several of the ngers
or in many cases only to the palm. The palm
pinch can be effective for some children, partic-
ularly if the ngers are stiff and xed in exten-
sion. Some children have neither nger nor
thumb active motion and do not use their hands
at all other than to push objects around. Patients
with no digital motion usually lack any active
motion in their upper extremity; however, FPL
function is typically preserved even in severely
affected children. If there is sufcient FPL func-
tion to provide a usable pinch, yet the thumb is in
a position where it opposes only to space, a
thumb reorientation osteotomy is recommended.
The goal of the osteotomy is to place the thumb
into a position where it can best take advantage
of the childs existing nger and thumb motion to
provide pinch and/or grasp. The reorientation
osteotomy typically results in a sock puppet--
type position for the thumb, but other positions
may be optimal depending on the childs needs
and abilities.
For both type 1 and type 2 arthrogrypotic
thumbs, the rst web space contracture should
be addressed at the same time as the chondrodesis
or reorientation osteotomy. Options for expanding
the rst web space include the 4-ap Z-plasty, the
Abdel Ghani dorsal rotation advancement ap
(Abdel Ghani 2006), and the index rotation (sti-
letto) ap (Ezaki and Oishi 2010). For mild web
space contractures with a passively extendable
MP joint, the 4-ap Z-plasty remains the most
reliable and simplest alternative (Fig. 17a). More
severe contractures with no or minimal MP joint
exion contractures require the Abdel Ghani ap
(Fig. 17b). The best ap for addressing xed MP
exion contractures is the stiletto ap (Fig. 17c).
827
When necessary, the stiletto ap can be extended
into an Abdel Ghani-type ap to expand a very
tight rst web space (Fig. 17d). Often, the rst
dorsal interosseous and adductor pollicis muscles
are either tight or have been replaced with brous
tissue and must be either lengthened or divided.
Thumb Reorientation Osteotomy
Place the patient supine on a standard operating
room table with an arm table. Regional anesthesia
is helpful to control postoperative pain and as a
supplement to general anesthesia. A tourniquet is
applied to the upper arm.
The arm is prepared and draped to the tourni-
quet. Begin with a stiletto ap if appropriate
(Fig. 17c). Mark out the radial apex of the index
nger proximal interphalangeal (PIP) joint crease
with a dot and the entire thumb MP joint crease
with a line. Connect the dot to the most ulnar
aspect of the line, then draw a divergent line
from the dot to the dorsal aspect of the index MP
joint. Both lines emanating from the dot should be
of equivalent lengths. Curving the more dorsal
line provides a better contour for the web space
along the thumb (Fig. 18a). Pinch the V-shaped
ap together to conrm that there will be suf-
cient skin remaining on the index nger to close
the donor site for the stiletto ap.
Exsanguinate the arm and inate the tourni-
quet. Incise the skin, keeping the stiletto ap full
thickness down to the fascial level. Identify and
preserve the dorsal sensory branch of the radial
digital nerve to the index nger and the princeps
pollicis artery and its branches deep to the subcu-
taneous fat. Incise across the thumb MP joint
crease with care to not injure the neurovascular
bundles to the thumb (Fig. 18b).
Identify the rst dorsal interosseous and the
adductor pollicis muscles in the rst web space.
If the muscles are present and of reasonable qual-
ity, try to preserve them by rst dividing their
overlying fascia (Fig. 18c) and then gently
stretching the muscle. If the muscles have mini-
mal to no excursion, incise the muscles to allow
the thumb to abduct.
Make a separate longitudinal incision at the
base of the thumb metacarpal between the
828
Fig. 17 Multiple ap options exist for widening the rst
web space. The 4-ap Z-plasty is reliable for mild contrac-
tures (a). More severe contractures may benet from a
dorsal advancement ap (b). If the thumb is clasped and
the rst web space is contracted, the index rotation (stiletto)
extensor pollicis brevis (EPB) and extensor
pollicis longus (EPL) tendons. Carry the dissec-
tion down to the periosteum between the two
tendons. Split the periosteum longitudinally and
retract the periosteum with two Holman retractors.
Be careful not to damage the physis at the base of
the metacarpal. Using a reciprocating saw, make
an incomplete transverse osteotomy just distal to
the physis. Referencing the thumbnail, make a
second cut to create a dorsal closing wedge at
the desired angle (Fig. 19a). Complete both cuts.
Pass two 0.035 in. or 0.045 in. K-wires
antegrade through the osteotomy site and down
D.A. Zlotolow and S.H. Kozin
ap is an excellent option (c). If the web space is still tight
after a stiletto ap, the ap can be extended into a dorsal
advancement ap (d) (Courtesy of Shriners Hospital for
Children Philadelphia)
the thumb, keeping the wires as far from each
other as possible at the osteotomy site so as to
maximize rotational control (Fig. 19b). Reduce
the osteotomy by extending, abducting, and
pronating the thumb. Advance the K-wires across
the osteotomy site in a retrograde fashion. Make
sure that the thumb is now in the optimal position
to oppose to the ngers. In most cases, the optimal
position for the thumb is in the sock puppet
position (Fig. 19c).
Wire placement can be conrmed either clin-
ically or with the use of uoroscopy. Cut the
wires outside of the skin. Rotate the stiletto ap
35 Arthrogryposis
Fig. 18 The index rotation ap is the workhorse ap for
the thumb in arthrogryposis. The limbs of the ap should
be of equal length with at most a 3:1 length to width ratio
(a). The MP exion crease is incised to create the space to
into the thumb MP crease and close the wounds
with absorbable suture. Apply a sterile dressing
and deate the tourniquet while holding pressure
over the wound. Apply a short arm thumb
spica cast.
Remove the cast 46 weeks after surgery,
depending on the age of the child. Conrm
union at the osteotomy site radiographically and
clinically (lack of tenderness) before removing the
wires. Fabricate an orthoplast thumb spica splint
to be worn for a month at all times except when
bathing or performing range-of-motion exercises.
Transition to nighttime-only wear for a subse-
quent 2 months.
829
inset the ap (b). Divide the fascia over the rst dorsal
interosseous and adductor pollicis to allow expansion of
the web space (c) (Courtesy of Shriners Hospital for Chil-
dren Philadelphia)
Thumb reorientation osteotomy preoperative planning
OR table: standard
Position/positioning aids: supine
Fluoroscopy: mini C-arm
Equipment: Kirschner wires
Tourniquet (sterile/nonsterile): upper arm, nonsterile
Thumb reorientation osteotomy surgical steps
Incise and elevate a stiletto ap
Divide the fascia over the adductor and rst dorsal
interosseous
Make a longitudinal incision over the base of the rst
metacarpal
(continued)
830
Fig. 19 If the thumb and ngers are mobile but are not in a
position to oppose to each other, a thumb reorientation
osteotomy should be considered. A skin ap, usually a
stiletto, is used to open the rst web space. The base of
the metacarpal is then osteotomized distal to the physis to
place the CMC joint into an extension posture (a). Two
Thumb reorientation osteotomy surgical steps
Perform a dorsal closing wedge osteotomy distal to the
physis
Advance 2K-wires antegrade through the osteotomy site
Pronate and extend the thumb to an optimal position
Advance the wires retrograde across the osteotomy site
Close the wounds
D.A. Zlotolow and S.H. Kozin
K-wires are driven antegrade through the osteotomy site
(b). The thumb position is optimized into pronation, exten-
sion, and abduction, and the osteotomy is secured with the
2K-wires. Closure of the skin ap completes the procedure
(c) (Courtesy of Shriners Hospital for Children
Philadelphia)
Thumb reorientation osteotomy postoperative
protocol
Type of immobilization: short arm thumb spica splint
or cast
Length of immobilization: 46 weeks
Rehab protocol: nger mobility and contralateral arm
motion
35 Arthrogryposis
Thumb reorientation osteotomy potential pitfalls and
preventions
Potential pitfall Pearls for prevention
Inadequate Inadequate adductor and rst
abduction dorsal interosseous release
achieved
Inadequate Release any tethering soft
rotation achieved tissues
Extend the stiletto into a dorsal
advancement ap
Shorten the metacarpal base
No thumb motion Make sure FPL working before
surgery
Do not open FPL sheath
Start motion as soon as osteotomy
healed
Tip necrosis of ap Keep the ap full thickness and
preserve subcutaneous
vasculature wherever possible
Avoid a length to width ratio of
greater than 3:1
Avoid a tight closure (skin graft if
necessary)
Fig. 20 For type 1B thumbs, an MP chondrodesis and
stiletto ap is a reliable option (a). After elevating the
stiletto ap, a separate incision is made over the thumb
MP joint. The joint is exposed between the EPL and EPB
tendons, and the collateral ligaments are divided (b). The
head of the metacarpal is removed with a reciprocating saw
831
Thumb Metacarpophalangeal
Chondrodesis
Place the patient supine on a standard operating
room table with an arm table. Regional anesthesia
is helpful to control postoperative pain and as a
supplement to general anesthesia. A tourniquet is
applied to the upper arm.
The arm is prepared and draped to the tourni-
quet. Begin with a stiletto ap if appropriate
(Fig. 18). Mark out the radial apex of the index
nger proximal interphalangeal (PIP) joint crease
with a dot and the entire thumb MP joint crease with
a line (Fig. 20a). Connect the dot to the most ulnar
aspect of the line, then draw a divergent line from
the dot to the dorsal aspect of the index MP joint.
Both lines emanating from the dot should be of
equivalent lengths. Curving the more dorsal line
provides a better contour for the web space along
the thumb. Pinch the V-shaped ap together to
conrm that there will be sufcient skin remaining
on the index nger to close the donor site.
(c) followed by resection of the articular surface of the
proximal phalanx (d). In children older than 3 years of
age, the ossic nucleus signals that the physis is near (e).
Fixation of the chondrodesis and closure of the skin ap
dramatically improves the appearance of the hand (f)
(Courtesy of Shriners Hospital for Children Philadelphia)
832
Exsanguinate the arm and inate the tourni-
quet. Incise the skin, keeping the stiletto ap full
thickness down to the fascial level. Identify and
preserve the dorsal sensory branch of the radial
digital nerve to the index nger and the princeps
pollicis artery and its branches deep to the subcu-
taneous fat. Incise across the thumb MP joint
crease with care to not injure the neurovascular
bundles to the thumb.
Identify the rst dorsal interosseous and the
adductor pollicis muscles in the rst web space.
If the muscles are present and of reasonable
quality, try to preserve them by rst dividing
only their overlying fascia and then gently
stretching the muscle. If the muscles have mini-
mal to no excursion, cut the muscles to allow the
thumb to abduct.
Make a separate longitudinal incision over the
thumb MP joint between the EPB and EPL ten-
dons. Carry the dissection down to the periosteum
between the two tendons. Split the capsule longi-
tudinally and cut the collateral ligaments
(Fig. 20b). Using a reciprocating saw, make a
transverse cut just proximal to the articular surface
of the metacarpal head (Fig. 20c). Using a knife
and a rongeur, resect the articular surface of the
proximal phalanx to the level of the epiphyseal
ossic nucleus but no further (Fig. 20d). If the
epiphysis has not begun to ossify, as is commonly
the case in children under three, resect only a
minimal amount of articular surface. Be careful
not to damage the physis at the base of the prox-
imal phalanx (Fig. 20e).
Pass one 0.045 in. K-wire antegrade through
the proximal phalanx and down the thumb.
Reduce the chondrodesis and advance the
K-wire across the joint in a retrograde fashion.
Make sure that the thumb is now in the optimal
position to oppose to the ngers (Fig. 20f). Repair
the extensor hood and joint capsule with slow-
absorbing suture. If the FPL is very tight, consider
making a separate incision in the volar forearm
and fractionally lengthening the FPL.
Wire placement can be conrmed either clini-
cally or with the use of uoroscopy. Cut the wire
outside of the skin. Rotate the stiletto ap into the
thumb MP crease and close the wounds with
D.A. Zlotolow and S.H. Kozin
absorbable suture. Apply a sterile dressing and
deate the tourniquet while holding pressure over
the wound. Apply a short arm thumb spica cast.
Remove the cast 46 weeks after surgery,
depending on the age of the child. Union of the
chondrodesis cannot be conrmed radiographi-
cally. The timing for removal of the wire is
based solely on clinical parameters. Once the
wire is removed, fabricate an orthoplast thumb
spica to be worn for a month at all times except
when bathing or performing range-of-motion
exercises. Transition to nighttime splint only for
a subsequent 2 months.
Thumb metacarpophalangeal joint chondrodesis
preoperative planning
OR table: standard
Position/positioning aids: supine
Fluoroscopy: mini C-arm
Equipment: Kirschner wires
Tourniquet (sterile/nonsterile): upper arm, nonsterile
Thumb metacarpophalangeal joint chondrodesis
surgical steps
Incise and elevate a stiletto ap
Divide the fascia over the adductor and rst dorsal
interosseous
Make a longitudinal incision over the rst
metacarpophalangeal joint
Split the extensor hood between the EPL and EPB
tendons
Expose the joint and cut the collateral ligaments
Excise the metacarpal head with a saw
Excise the base of the proximal phalanx with a knife
Advance 1 or 2K-wires antegrade through the fusion site
Pronate and extend the thumb to an optimal position
Advance the wires retrograde across the fusion site
Repair the extensor hood
Close the wounds
Thumb metacarpophalangeal joint chondrodesis
postoperative protocol
Type of immobilization: short arm thumb spica splint or
cast
Length of immobilization: 46 weeks
Rehab protocol: nger mobility and contralateral arm
motion
35 Arthrogryposis
Thumb metacarpophalangeal joint chondrodesis
potential pitfalls and preventions
Potential pitfall Pearls for prevention
Inadequate Inadequate adductor and rst
abduction dorsal interosseous release
achieved
Inadequate Release any tethering soft tissues
extension achieved volarly
Lengthen the FPL if tight
Excise more metacarpal
No thumb motion Make sure FPL working before
surgery
Do not open FPL sheath
Start motion as soon as osteotomy
healed
Tip necrosis of ap Keep the ap full thickness and
preserve subcutaneous
vasculature wherever possible
Avoid a length to width ratio of
greater than 3:1
Avoid a tight closure (skin graft if
necessary)
The Fingers
Camptodactyly is common and can be a dening
characteristic of specic syndromes such as Beals
(contractural arachnodactyly). The ngers can also
be rigid, xed in either exion or extension. Chil-
dren with amyoplasia with no muscle function in
the hands often present with symphalangism (Baek
and Lee 2012) in extension (Fig. 21). Rigid ngers
only function to point or push objects around a
surface. Tablet computers have given children
with rigid ngers new ways to express themselves,
learn, play, and communicate.
Other patients, particularly those with distal
arthrogryposis, may exhibit a PIP exion contrac-
ture (camptodactyly). Camptodactyly can inter-
fere with capturing objects in the hand, with
some children developing a compensatory pinch
and grasp pattern against the dorsum of the
contracted ngers (Fig. 22a).
Initial treatment is passive range of motion
until the child is old enough to be t with a splint.
Splints are applied at night and during naps and
should attempt to correct the MP joint and IP joint
833
Fig. 21 The typical position of the hand in a child with
amyoplasia who has no hand function. The ngers are stiff
and in near full extension. The thumb is a type 2B (Cour-
tesy of Shriners Hospital for Children Philadelphia)
postures. The splints should be designed to pro-
vide simultaneous correction of wrist and nger
contractures as well. Tremendous gains can be
made with passive motion and splinting, and the
parents should be encouraged to be active partic-
ipants in their childs care (Benson et al. 1994).
Gains can typically be seen up to about 5 years of
age, but most children plateau after their second
birthday. For those children that remain con-
tracted after a supervised course of therapy, surgi-
cal intervention can be considered.
Radiographs often demonstrate hypotrophic
condyles at the PIP joint (Fig. 22b). Children
with passively correctable camptodactyly are
more likely to have well-formed condyles than
those children with xed exion contractures.
The practical implication of the radiographic nd-
ings are not clear, but the collective wisdom is that
the more severe the condylar hypoplasia the less
correctable the deformity.
The results of camptodactyly release are
unpredictable, despite some favorable results in
the literature (Smith and Grobbelaar 1998;
Foucher et al. 2006; Koman et al. 1990). The
mainstay of surgical treatment is a volar Z-plasty
834
Fig. 22 Camptodactyly can interfere with function as
seen in this child who was unable to place objects in his
hand. His thumb opposed to the dorsum of his ring nger
(a). Radiographs demonstrated hypotrophic condyles at
the PIP joint (b). A thumb MP chondrodesis and stiletto
to augment the skin at the PIP joint. Often, there is
a tight pterygium keeping the PIP joint in exion
(Fig. 22c). This pterygium can also make it dif-
cult for the child to hold objects in the hand.
Occasionally, the skin overlying the PIP joint is
so tight that a full-thickness skin graft is needed in
addition to or in lieu of a Z-plasty.
If the child has FDS and FDP function, the FDS
can be divided to lessen the deforming force of the
FDS on the PIP joint (Fig. 22d). In children with a
passively correctable camptodactyly and good
FDS excursion, the FDS can be transferred to the
lateral band to augment the extensor mechanism.
The results of this FDS intrinsicplasty are likewise
unpredictable, with some children developing PIP
joint extension contractures that limits grasp.
Another option is to use the FDS to enhance wrist
extension, but this can only be done if there is
D.A. Zlotolow and S.H. Kozin
ap as well as camptodactyly releases of the long and ring
ngers were performed. The Z-plasties were made along
the lines of maximal tension (c) and the FDS was released
(d). Clinical appearance at follow-up was improved (e)
(Courtesy of Shriners Hospital for Children Philadelphia)
sufcient nger extension to allow capture and
release with the wrist in at least neutral extension.
The FDS can be transferred through the
interosseous membrane (IOM) to the ECRB ten-
don or around the long nger metacarpal. Only
augment active wrist extension in children who
are able to extend their ngers without the need
for wrist exion tenodesis.
Camptodactyly Release
Place the patient supine on a standard operating
room table with an arm table. Local anesthesia is
helpful to control postoperative pain and as a
supplement to general anesthesia. A tourniquet is
applied to the upper arm. Once the patient is
asleep, reexamine the ngers to determine if the
PIP joint itself is contracted, if the FDS is tight,
and if there is extensor mechanism tenodesis.
35 Arthrogryposis
With the wrist in exion and the MP joint in
exion, the remaining PIP exion contracture is
likely due to the joint. If the FDS is tight, the
contracture should worsen with wrist extension.
Worsening of the contracture with extension at the
MP joint can also signal FDS tightness but may be
due to tight palmar skin.
The arm is prepared and draped to the tourni-
quet. Begin by drawing a line along the ridge of
maximal skin tension along the palmar aspect of
the PIP joint. Flex the nger to identify the apices
of the PIP exion crease, and mark those. Draw
parallel lines from each of those apices to the line
drawn along the axis of tension, intersecting that
line at 60 angles. The Z should have equal length
segments. Adjustments can be made to accommo-
date for more oblique lines of tension or for a more
distal or proximal pterygium.
Inate the tourniquet. Incise the skin with care
not to injure the neurovascular bundles. Identify
the bundles and retract them gently out of harms
way. The nerves may be surprisingly supercial in
some patients and may be contributing to the
contracture. If the neurovascular bundles are
tight with maximal passive PIP joint extension,
do not attempt to gain further passive extension. If
the bundles can accommodate further PIP joint
extension, test for FDS tightness by extending
the MP joint. Since the pterygium has been
released with the incision, the only cause of PIP
joint exion beyond the intrinsic level of joint
contracture is the FDS. If the child has a function-
ing FDP, consider an FDS tenodesis just proximal
to the fourth annular pulley.
Transfer of the FDS to augment intrinsic func-
tion should be reserved for high-functioning
patients with good FDS excursion and without
an intrinsic PIP joint contracture. Both a Zancolli
lasso and a formal intrinsicplasty have been
described (Smith and Grobbelaar 1998; Foucher
et al. 2006; Koman et al. 1990).
A volar plate release can be considered for
intrinsic joint contractures, depending on the ten-
sion of the neurovascular bundles. Divide the
checkrein ligaments and the attachment of the
accessory collateral ligament to the volar plate.
Manipulation should be gentle to avoid a physeal
fracture at the base of the middle phalanx.
835
Application of a K-wire is optional to hold the
joint in maximal extension.
Close the wound with absorbable sutures and
apply a sterile dressing. Deate the tourniquet and
check the nger for adequate perfusion. The arter-
ies may be in spasm and capillary rell may be
delayed at rst. The usual tactics of warming the
room, removing the dressings, blocking the sym-
pathetic nerves with a lidocaine injection, remov-
ing the pin (if used) in that order, usually returns
adequate perfusion to the digit. Venous outow is
rarely an issue.
Once blood ow is conrmed, apply a long or
short arm cast or splint to maintain the ngers in
extension. If other concomitant procedures were
performed, adjust the postoperative immobiliza-
tion accordingly.
Remove the operative splint/cast after 23
weeks and remove the pin if used. Fit the patient
with an orthoplast splint to be worn full time except
for motion exercises and bathing for a further
6 weeks. Nighttime splinting can continue thereaf-
ter for as long as needed, usually another 2 months.
Camptodactyly release preoperative planning
OR table: standard
Position/positioning aids: supine
Fluoroscopy: mini C-arm
Equipment: Kirschner wires
Tourniquet (sterile/nonsterile): upper arm, nonsterile
Camptodactyly release surgical steps
Incise and elevate a volar Z-plasty along maximal
tension line
Assess FDS tightness
Tenotomize FDS if necessary and if FDP functional
Assess joint contracture and neurovascular pedicle
tension
Mobilize volar plate if needed
Pin the PIP joint in maximal extension if desired
Close the wound
Camptodactyly release postoperative protocol
Type of immobilization: short arm thumb spica splint or
cast
Length of immobilization: 23 weeks
Rehab protocol: nger mobility and contralateral arm
motion
836 D.A. Zlotolow and S.H. Kozin

Camptodactyly release potential pitfalls and preventions

Potential pitfall Pearls for prevention References
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losing gains made at surgery classication of the distal arthrogryposes. Am J Med
No nger motion Too much surgery for too little a Genet. 1996;65(4):27781.
nger Bamshad M, Van Heest AE, Pleasure D. Arthrogryposis: a
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Brown SHM, Hentzen ER, Kwan A, Ward SR, Fridn J,
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Lieber RL. Mechanical strength of the side-to-side
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Carroll RE, Kleinman WB. Pectoralis major transplanta-
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of the childs contractures, but each child can be Foucher G, Lora P, Khouri RK, Medina J, Pivato G.
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these children adapt quite well to their limita- cies: a treatment algorithm based on clinical examina-
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the potential benets in function outweigh the Surg 2010;14:121124.
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exorplasty for the arthrogrypotic elbow. J Hand Surg.
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their bets. Hall JG. Uterine structural anomalies and arthrogryposis
Assisting a child with the transition from death of an urban legend. Am J Med Genet Part A.
dependence to independence is tremendously 2013;161A:828.
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rewarding for the surgeon and therapist. As Ster- gryposes: delineation of new entities review and
ling Bunnell once said, to someone who has nosologic discussion. Am J Med Genet. 1982;11:
nothing, a little is a lot. (Shin 2006). 185239.
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Hall JG, Reed SD, Driscoll EP. Part I. Amyoplasia: a
common, sporadic condition with congenital contrac-
tures. Am J Med Genet. 1983;15(4):57190.
Kay S, Pinder R, Wiper J, Hart A, Jones F, Yates A.
Microvascular free functioning gracilis transfer with
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Recon Aesthetic Surg. 2010;63(7):11429.
Koman LA, Toby EB, Poehling GG. Congenital exion
deformities of the proximal interphalangeal joint in
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Kozin SH. Medial approach for humeral rotational
osteotomy in children with residual brachial plexus
birth palsy. Oper Tech Orthop. 2007;17:8893.
Lahoti O, Bell MJ. Transfer of pectoralis major in arthro-
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1998;23:106370.
Van Heest A, James MA, Lewica A, Anderson KA. Poste-
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Zancolli E, Mitre H. Latissimus dorsi transfer to restore
elbow exion. J Bone Joint Surg. 1973;55A:126575.
Zlotolow DA, Kozin SH. Posterior elbow release and
humeral osteotomy for patients with arthrogryposis.
J Hand Surg Am. 2012;37(5):107882.
Zlotolow DA, Thompson SJ. Handbook of splinting and
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Zlotolow DA, Catalano LW, Barron OA, Glickel SZ. Sur-
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Surg. 2006;14(13):75465.
 Inherited Muscle Disease
36
Diana X. Bharucha-Goebel and Carsten G. Bnnemann

Contents Abstract
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 840 The focus of this chapter will be to highlight
some of the inherited disorders of muscle
Neuromuscular Examination of a Weak
Infant or Child . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 840
that can be associated with upper extremity
involvement. Many of these disorders are
Diagnoses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 841 marked by predominantly proximal muscle
Congenital Onset . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 842
Childhood Onset . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 847 weakness which can affect arms and legs to
varying extents during the progression of the
Diagnostic Features in Inherited Muscle
Disease and Concepts of Upper Extremity
disease, but this chapter will attempt to high-
Involvement . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 850 light more specic patterns of muscle
Central Nervous System Involvement . . . . . . . . . . . . . . . 850 involvement where known for the given mus-
Muscle Diseases with Dropped Head Syndrome . . . . 850 cle disease. This chapter will also provide
Common Muscle Diseases with Spine
Involvement . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 850
information on the assessment of muscle
Scapular Winging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 851 in terms of functional assessments as well
Contractures and Arthrogryposis . . . . . . . . . . . . . . . . . . . . 852 as imaging modalities. While no single
Joint Hyperlaxity . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 852 approach should be universally applied
Early Upper Extremity Weakness . . . . . . . . . . . . . . . . . . . 854
Early Distal Muscle Involvement . . . . . . . . . . . . . . . . . . . . 854
when it comes to the management of such a
heterogeneous group of disorders, this chap-
Diagnostic Approaches . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 854
ter will provide some general principles
Management Approaches . . . . . . . . . . . . . . . . . . . . . . . . . . 855 regarding the management of patients with
Duchenne Muscular Dystrophy inherited muscle diseases as it pertains to
and Corticosteroids . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 857 upper extremity function. While this chapter
Enzyme Replacement Therapy: Pompe Disease . . . . 857 serves as a basic guide for clinicians as to the
Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 857 consideration and some management strate-
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 858
gies, it should not be used to substitute the
care of these patients in centers that have
familiarity and the multidisciplinary capabil-
D.X. Bharucha-Goebel (*) ity to provide proper diagnostic workup,
Neurology, Childrens National Medical Center &
evaluation, and subsequently management
National Institutes of Health, NINDS, Bethesda, MD, USA
e-mail: diana.bharucha@nih.gov of patients with various forms of inherited
muscle diseases.
C.G. Bnnemann
National Institutes of Health, NINDS, Bethesda, MD, USA
e-mail: carsten.bonnemann@nih.gov

# Springer Science+Business Media New York 2015 839

J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_36
840
Introduction
Inherited muscle disorders represent a broad group
of disorders with congenital, childhood, or adult
onset that in addition can have variability in disease
course from those with relative stability over long
periods of follow-up to those with improvement or
motor gains to those with progressive weakness
and functional deterioration. When evaluating a
patient with suspected neuromuscular weakness,
primary muscle disorders (myopathies and muscu-
lar dystrophies) tend to be considered in the setting
of predominantly proximal upper and lower
extremity weakness. There are also exceptions to
this general rule in that there are patients with more
distal muscle involvement or those with predomi-
nantly facial involvement. For the purpose of this
chapter, we will focus on how various muscle
diseases can affect the upper extremities. However,
this should be taken in the context of understanding
that most of the disorders described in this chapter
can have important aspects resulting from involve-
ment elsewhere, such as facial weakness, lower
extremity weakness, ocular involvement, respira-
tory involvement, cardiac involvement, occasion-
ally brain involvement, and also orthopedic
complications such as scoliosis and joint subluxa-
tion or dislocations. Some of the symptoms and
themes described in this chapter can also be seen in
or applied to other disorders of the neuromuscular
system including: neuromuscular junction disor-
ders, motor neuron disorders, and neuropathies.
However, for the purpose of this chapter, the
focus will be on inherited disorders of muscle.
Inherited disorders of muscle can be broadly
divided into two groups: myopathies and muscu-
lar dystrophies. It can then be subdivided by many
variables including age of onset, histopathological
features, and genetics.
Neuromuscular Examination of a Weak
Infant or Child
Before delving into the various forms of inherited
muscle disease, it is worthwhile to discuss the
neurological examination of a child with
D.X. Bharucha-Goebel and C.G. Bnnemann
weakness. Especially when evaluating an infant
or young child, it is very important to understand
the difference between low tone (hypotonia) and
weakness. In the infant, various maneuvers can be
utilized to assess tone. When evaluating an infant
with suspected muscle disease, it is important to
observe the infants posture and spontaneous as
well as elicited movements. Healthy full-term
infants tend to keep a exed posture of the limbs
and can maintain their arms and legs against grav-
ity. A child with hypotonia (which can be seen in
inherited disorders of muscle but also in central
nervous system disease) will keep the legs splayed
to the side in a frog-legged position with fewer
antigravity movements. In addition, they may
keep arms extended and at their sides with little
antigravity movements. With gentle stimulation, a
provider can also evaluate whether the infant is
able to generate antigravity movements in each
extremity and across various joints (e.g., isolate
whether movements coming from the shoulder,
elbow, or only ngers). Such maneuvers may
allow a better assessment of strength in the
extremities. In central nervous system causes of
hypotonia, deep tendon reexes may be relatively
lively, while they tend to be suppressed in muscle
disease. In a very weak patient, the only move-
ments seen may be some nger exion or exten-
sion, and this could be missed if careful
examination is not performed. The traction
response or pull to sit evaluates an infant or childs
ability to control their head when gently pulled up
from supine to sitting. In addition to assessing
head control, one can assess whether they can
engage the biceps to keep elbows slightly exed
and whether there is stability of the shoulders
when gently pulling on the arms for a pull to sit
maneuver and whether the legs are pulled up. One
can also get a sense of truncal tone by evaluating
the shoulder girdle with vertical suspension. This
is performed by placing hands under the axilla and
picking up the infant or child. A child who has
difculty supporting their shoulders or arms may
have increased slip through due to a combina-
tion of low tone and/or weakness. Both of these
maneuvers generally assess tone, but can also be
affected in patients with weakness. Increased head
lag and increased slip through are signs associated
36 Inherited Muscle Disease
with hypotonia regardless of the cause and are not
specic to muscle disease or to neuromuscular
disorders. The presence of these ndings in an
infant with suspected neuromuscular disease can
be helpful, but these ndings should always be
considered within the entire clinical context. In
older children (as can be seen in the infant exam),
tone can be assessed by passive movements of
extremities around a joint.
Weakness can be assessed in various ways. The
most common rating used by clinicians for assess-
ment of muscle strength is the Medical Research
Council (MRC) scale. This rates muscle power as
follows (Table 1):
The MRC is useful in children that are able to
cooperate with an examination and provide reli-
able effort. Thus this test is more helpful in chil-
dren over 4 or 5 years of age. The scale above is
also adapted by providers where ( ) and (+) signs
are used to further subdivide strength within a
given score in the 25 range.
While this chapter will not delve into all upper
extremity rating scales or functional rating scales
(see chapter Occupational Therapy Evaluation
and Treatment), it is useful to note a functional
rating scale that describes upper extremity func-
tion that has been developed for the evaluation of
boys with Duchenne muscular dystrophy. The
Brooke scale was designed to assess upper
extremity function with a score from 1 (least
severe) to 6 (most severe). This scale was
designed for progression of upper extremity
dysfunction in Duchenne muscular dystrophy
(Brooke et al. 1981). While it could theoretically
be applied to some of the other inherited
neuromuscular conditions, caution would need
to be used, as other disorders may have early
contractures that limit function thus rendering a
far lower score earlier in disease course.
See Table 2.
In addition to evaluating strength and tone, it is
also important to take note of muscle bulk.
Patients may have certain patterns of muscle atro-
phy or pseudohypertrophy that may be character-
istic of particular types of diseases. Further details
on the history and examination of patients with
neuromuscular disease will be provided later in
this chapter.
841
Table 1 MRC scale for evaluation of muscle strength
0 No contraction
1 Flicker or trace movement
2 Active movement with gravity eliminated
3 Active movement against gravity
4 Active movement against gravity and resistance
5 Normal power
Table 2 Brooke scale of upper extremity function
Grade Description
1 Starting with arms at the sides, the patient can
abduct the arms in a full circle until they touch
above the head
2 Can raise arms above head only by exing the
elbow (shortening the circumference of the
movement) or using accessory muscles
3 Cannot raise hands above head, but can raise an
8-oz glass of water to the mouth
4 Can raise hands to the mouth, but cannot raise
an 8-oz glass of water to the mouth
5 Cannot raise hands to the mouth, but can use
hands to hold a pen or pick up pennies from the
table
6 Cannot raise hands to the mouth and has no
useful function of hands
Diagnoses
This chapter is focusing on those disorders of
muscle and so will not address disorders of the
motor neuron, nerves, or neuromuscular junction
that can all affect upper extremity function. For
the purpose of clarity and focus, this chapter will
not cover mitochondrial disorders and will not
cover all metabolic disorders that can affect mus-
cle, but will focus on one (Pompe disease). The
inherited disorders of muscle that will be
discussed in this chapter will include congenital
muscular dystrophies, congenital myopathies,
myotonic dystrophy, dystrophinopathies, Emery-
Dreifuss muscular dystrophy, and limb-girdle
muscular dystrophies. In general, with the excep-
tion of myotonic dystrophy and Pompe disease,
most of the disorders discussed in this chapter are
caused by abnormalities in the contractile appara-
tus, nuclear membrane, connection between the
contractile apparatus and the sarcolemmal
842
membrane, membrane repair, and extracellular
matrix. See Fig. 1 which shows a diagram of
the proteins contributing to various inherited
muscle diseases. The disorders discussed in
this chapter will largely be pediatric-onset
disorders.
Congenital Onset
The congenital myopathies and congenital
muscular dystrophies are dened based upon
clinically apparent muscle weakness at birth or
in early infancy. While some patients may not be
recognized to be symptomatic until a later age, the
onset of disease is the one that begins prenatally or
in infancy. There can be varying degrees of sever-
ity from very severe prenatal onset to subtle motor
difculties.
Fig. 1 Diagram of the proteins near the Sarcolemnal
(Plasma) membrane (Seen ingray). Note, extracellular pro-
teins are seen above and intracellular proteins are seen
D.X. Bharucha-Goebel and C.G. Bnnemann
The congenital myopathies are a group of dis-
orders often with characteristic histopathological
ndings on muscle biopsy and typically marked
by a disease course that is relatively stable over
longer periods of follow-up. While historically
these disorders have been dened by the charac-
teristic histological ndings, it is important to note
that the absence of such ndings does not always
preclude some of these diagnoses. There can be a
range of histopathological features seen in these
disorders, and the variability in histological nd-
ings within particular disorders may partly be
explained by the age of biopsy and the site of
biopsy. Symptoms can often begin prenatally,
with ndings of polyhydramnios, decreased fetal
movement, and breech position. At birth, ndings
of arthrogryposis of the upper and lower extremity
may be seen, as well as hip dislocations
and osteopenia related to poor fetal movement,
below. The represent some (but not all) proteins that con-
tribute to various forms of inherited muscle disease.
36 Inherited Muscle Disease 843

Table 3 Congenital myopathies

Classical histological
Disease Gene Salient clinical features ndings
Nemaline ACTA1, TPM3, NEB, Range in severity Nemaline rods in
myopathy TPM2, TNNT1, CFL2, Classic form facial and axial muscle continuity with Z lines
KBTBD13, KBTBD5 weakness with onset in infancy or
childhood
Feeding difculties common
Severe respiratory involvement may
be seen
Central core RYR1 Variable severity (severe neonatal- to Classic cores areas
disease/RYR1 childhood-onset hypotonia and of clearing on oxidative
associated delayed motor milestones) stains (NADH)
myopathy Possible ophthalmoplegia May also see multi-
minicores
Severe respiratory involvement may Absence of cores does
be seen not preclude diagnosis
Risk of malignant hyperthermia
Sparing of rectus femoris muscle on
muscle imaging
Centronuclear MTM1 X-linked (severely affected males) Centrally placed nuclei
myopathy/ Severe neonatal hypotonia
myotubular Ophthalmoplegia
myopathy
Early respiratory failure/ventilator
dependence
Centronuclear BIN1, DNM2, RYR1 Severe neonatal hypotonia (variable) Centrally placed nuclei
myopathy
Congenital ber- TPM3 Hypotonia at birth or shortly after Type 1 ber hypotrophy
type disproportion ACTA1 Variable degree of weakness (12 % smaller than the
SEPN1 Static or may show improvement type 2 bers

sometimes leading to fractures under delivery.
Within this group of disorders, there is great clin-
ical variability, with many of the entities ranging
from a severe and even lethal neonatal form with
fetal akinesia to a later onset or milder form. At the
most severe end of the spectrum (of those who
survive in utero to delivery) are infants who have
trace to no movements of the extremities, severe
inability to feed, and respiratory failure. Patients
may have ophthalmoplegia, in particular in the
centronuclear myopathies (Pierson et al. 2005).
Some patients can have pronounced facial weak-
ness as well. CK levels are often normal or can be
mildly elevated. See Table 3 for a list of genetic
causes and classic histological features seen in
the congenital myopathies (Pierson et al. 2005;
Jungbluth et al. 2003; Goebel 2005). Overall,
patients with congenital myopathy tend to be sta-
ble over long periods of time and even make
motor gains. The predominant factors that corre-
late with mortality or morbidity in this group of
disorders are the existence of severe respiratory
failure and risk of aspiration (to food or
secretions).
The congenital muscular dystrophies are a
heterogeneous group of disorders where the mus-
cle reveals dystrophic or myopathic features with-
out clear structural changes consistent with
congenital myopathies. The main congenital mus-
cular dystrophies include the following disorders:
disorders of alpha-dystroglycan glycosylation
(which can have signicant brain involvement),
collagen VI disorders, and laminin 2 CMD. See
Table 4 for a list and summary of these disorders,
key clinical features, and genetic causes. Collagen
VI-related congenital muscular dystrophy has
more striking upper extremity involvement
(involving contractures and joint laxity) that will
 844

Table 4 Congenital muscular dystrophies

Salient neurological MRI brain ndings
Disease Gene symbol [protein product] Motor features Ocular features features (if applicable)
-Dystroglycanopathies
Walker-Warburg syndrome POMT1 [protein Severe hypotonia Poor visual Decreased alertness Agyria or severe
(WWS) O-mannosyltransferase 1] attention lissencephaly
POMT2 [protein Neonatal onset Retinal Severe intellectual Polymicrogyria
O-mannosyltransferase 2] dysgenesis disability
FKTN [fukutin] Absent psychomotor acquisition Microphthalmia White matter
dysmyelination or
cystic changes
LARGE Congenital Cerebellar/
[acetylglucosaminyltransferase- cataracts brainstem
like protein] hypoplasia
Hydrocephalus
Complete or partial
absent corpus
callosum
Muscle-eye-brain disease POMGnT1 Hypotonia at birth or 1st few Milder than Severe intellectual Variable (mild to
(MEB)/Fukuyama CMD months WWS disability severe changes)
(FCMD) POMT1 Ambulation may acquired Congenital Refractory epilepsy Frontoparietal
glaucoma pachygyria
POMT2 Progressive Behavioral problems Polymicrogyria
myopia
ISPD [isoprenoid synthase Retinal atrophy Joint contractures Cerebellar/
domain-containing protein brainstem
hypoplasia
FKRP Juvenile Cerebellar vermis
cataracts hypoplasia/
dysplasia/cysts
FKTN Rarely severe Periventricular white
matter changes
D.X. Bharucha-Goebel and C.G. Bnnemann
 36

CMD-MR (CMD with POMT1 Neonatal-onset hypotonia Ocular ndings Mild cognitive Isolated
mental retardation) rare/mild impairment microcephaly
Minor white matter
changes
CMD-no MR Fukutin Neonatal- or childhood-onset Normal or mild Normal or minimal Normal or mild
FKRP hypotonia or delayed motor impairment abnormality
development
Defects of extracellular matrix proteins
Inherited Muscle Disease

LAMA2-related CMD LAMA2 Neonatal-onset max motor ability N/A Can have neuropathy, Abnormal white
(MDC1A, merosin- sit and stand with support epilepsy, subclinical matter signal
decient CMD) [Laminin 2] cardiomyopathy Occipital pachygyria
Pontocerebellar
atrophy (rare)
COL6-related dystrophy COL6A1, COL6A2, COL6A3 Variable severity (ambulatory to N/A Distal joint laxity None
(Ullrich CMD and Bethlem [Collagen VI] difculty sitting and severe Proximal contractures
myopathy) respiratory failure) Keratosis pilaris
Defects of proteins of the endoplasmic reticulum
Rigid spine muscular SEPN1 Delayed walking N/A Rigid spine None
dystrophy [Selenoprotein] Axial weakness Restrictive lung disease
Slow progression
Defects of nuclear envelope proteins
Lamin A/C-related CMD LMNA Axial weakness N/A Early motor deterioration None
[Lamin A/C] Dropped head syndrome Spinal rigidity
posterior neck weakness
845
846
be further discussed in section Diagnostic
Features in Inherited Muscle Disease and
Concepts of Upper Extremity Involvement of
this chapter.
In general this group of disorders tends to be
more progressive in nature. The onset is typically
either prenatally, at birth, or within the rst year of
life. Clinical features include diffuse weakness,
contractures (either in infancy or some that
develop later in childhood), and potential central
nervous system involvement in some (e.g., devel-
opmental delays, seizures, spasticity). The maxi-
mum motor skill achieved can vary. In the more
severe of the congenital disorders of muscle,
ambulation may not be achieved, while in a
more moderate group ambulation may be
achieved and later lost. In those representing the
severe end of the spectrum, patients may also have
signicant upper extremity involvement and yet
the patients are signicantly dependent on upper
extremity function for tasks such as feeding, use
of wheelchair, writing, typing, and other activities
of daily living. Children may make motor gains
early in life, but will eventually show some
decline in function. The rate of decline and age
of decline vary by disease and also from patient to
patient within the same disease. The degree of
deterioration and degree of impairment also vary
greatly in this group of disorders. Pathophysio-
logically, subgroups of disorders in this category
are caused by abnormalities of the extracellular
matrix of the muscle cell membrane, intracellular
molecules, and inner nuclear matrix proteins. See
Table 4 which will review the genetic causes of
congenital muscular dystrophy (Sparks
et al. 2012; Godfrey et al. 2007). Several of the
alpha-dystroglycanopathies represent the more
severe end of the spectrum with neonatal onset
of severe weakness, cognitive impairment, visual
impairment, and CNS MRI changes. CMDs with
more striking and unique upper extremity
involvement include the collagen VI disorders
where there is distal hyperlaxity combined with
proximal upper extremity contractures that are
progressive in nature. This will be discussed fur-
ther in a later section of this chapter.
In addition to the abovementioned disorders,
myotonic dystrophy is a disorder that can have
D.X. Bharucha-Goebel and C.G. Bnnemann
congenital onset or later in life onset (as there is
anticipation and increased severity from one gen-
eration to the next). Myotonic dystrophy type
1 (DM1) and type 2 (DM2) are autosomal domi-
nant multisystem disorders. DM1 is caused by
expansion of a CTG trinucleotide repeat in the
noncoding region of the DMPK gene, with
increased repeats correlating to disease severity.
DM1 can affect skeletal muscle, smooth muscle,
cardiac muscle, as well as the eye, endocrine
system, and central nervous system (Bird 2013).
Clinical ndings and severity in DM1 are variable
from mild to severe, with three predominant phe-
notypes: mild, classic, and severe congenital. In
classic DM1, weakness and muscle wasting of
facial muscles (especially temporal wasting), atro-
phy of the forearm exor muscles, weakness of
long nger exors, and weakness of ankle
dorsiexors can be seen. Patients can have prom-
inent myotonia, but this is often not present on
examination of neonates with suspected congeni-
tal myotonic dystrophy. In its most severe form, in
those patients with congenital myotonic dystro-
phy, severe neonatal hypotonia and respiratory
failure with feeding difculties can be seen. In
patients with congenital DM1, weakness is more
diffuse, but most notably, there is very striking
facial weakness as compared to the typical or later
onset DM1. Respiratory failure and prolonged
ventilator dependence in patients with congenital
DM1 correlates with higher rates of mortality.
Patients with myotonic dystrophy, especially
those with the congenital form, often have intel-
lectual disability, personality disorder, cataracts,
and cardiac conduction decits. DM2 tends to be
milder and have a later onset in life. DM2 is
characterized by myotonia and muscle dysfunc-
tion, with onset in childhood or adult years, and is
less commonly associated with cardiac conduc-
tion defects, cataracts, or insulin insensitivity
(as is seen in DM1). The onset of myotonia
tends to be later in life, often not until the third
decade. Muscle weakness and pain may be epi-
sodic or uctuating, with predominant involve-
ment of neck exors, nger exors, elbow
extensors, and hip exors and extensors (Dalton
et al. 2013). Expansion of the CCTG repeat on the
CNBP (ZNF9) gene causes DM2.
36 Inherited Muscle Disease
Childhood Onset
In contrast to those disorders with onset perina-
tally or in infancy, there are certain muscle dis-
eases that more distinctly have onset during the
childhood, adolescent, or adult years. This
includes dystrophinopathies, myotonic dystrophy
(although this can also have a severe congenital
form as discussed above), Emery-Dreifuss mus-
cular dystrophy, FSHD, Pompe disease, and
limb-girdle muscular dystrophies (LGMDs).
While there are some exceptions, these are a
group of disorders predominantly affecting the
shoulder and pelvic girdle muscles with age of
onset in childhood, adolescence, or even adult-
hood. This group of disorders is marked by grad-
ual progression of weakness over time. Although
proximal muscles are affected rst, over time,
distal muscles can become involved. CK levels
generally tend to be elevated. Muscle biopsy
reveals dystrophic changes, and immunohisto-
chemical stains can be helpful in narrowing
down the diagnosis.
The dystrophinopathies (Duchenne and
Becker muscular dystrophy) are X-linked
inherited disorders of muscle characterized by
calf pseudohypertrophy; proximal weakness,
followed by progressive more generalized weak-
ness; and cardiomyopathy. Duchenne muscular
dystrophy (DMD) is a severe progressive disease
that affects 1:3,6006,000 live male births.
Patients typically have normal strength in infancy,
but may have some delays in motor milestones.
Typically, by about 34 years of age, it becomes
apparent that these boys deviate in development
compared to their peers. In general, boys with
DMD hit a period of plateau in motor develop-
ment then followed by decline in function. They
may have more difculty with trying to run and
jump. They also may have more frequent falls
than their peers and may tend to walk on their
toes due to tightness of the Achilles tendon. Boys
with DMD have notable calf pseudohypertrophy
and lordotic posture, and they tend to walk on
their toes and have a Gowers sign reective of
difculty arising from the oor due to proximal
lower extremity weakness. Weakness often begins
in the proximal muscles then distal muscles and
847
legs before arms. Weakness progresses over time,
and in later stages of the disease, patients can
have severe diffuse weakness including limited
movements of the hands or ngers. Contractures
can be seen over time due to weakness and immo-
bility. Becker muscular dystrophy (BMD) has an
incidence of about 1:30,000 males. Its onset can
be variable from childhood (usually later than in
Duchenne MD) to early adulthood. In general,
patients with BMD maintain ambulation beyond
16 years of age and some even into adulthood.
Some patients may lose ambulation by their late
teens or 20s, while others may continue walking
into their 50s or later. Despite the milder impair-
ment of motor function, patients with Becker
muscular dystrophy can have cardiomyopathy
that exists even when patients have little to no
skeletal muscle weakness. Thus, in contrast to
those patients with Duchenne, where cardiomy-
opathy occurs later in the disease progression, in
patients with Becker muscular dystrophy, the
degree of severity of cardiomyopathy or age of
onset of cardiomyopathy does not clearly corre-
late with motor severity and cannot therefore be
predicted based upon the degree of skeletal mus-
cle weakness. Therefore, close and regular cardiac
screening is imperative. Contractures of the upper
extremity are rare in BMD patients who are still
ambulant. However, contractures of the heel
cords, hamstrings, and hip exors can be
commonly seen.
The limb-girdle muscular dystrophies are a
rare group of disorders with an overall incidence
of 1 per 100,000 (Rosales and Tsao 2012). This
section will focus only on those LGMDs with
onset in childhood and adolescence. Overall, the
recessively inherited LGMDs tend to be more
likely to present in childhood. See Table 5 for
details on the subtypes, genetic causes, and salient
clinical features of various childhood-onset
LGMDs (Pegoraro 2012; Rosales and Tsao
2012). Of note, disorders that at the protein level
interfere with the dystrophin-associated glycopro-
tein complex and sarcolemmal membrane tend
to manifest in similar ways to those with
dystrophinopathies (Duchenne or Becker muscu-
lar dystrophy). These disorders include alpha-
dystroglycanopathies and sarcoglycanopathies.
848 D.X. Bharucha-Goebel and C.G. Bnnemann

Table 5 Common limb-girdle muscular dystrophies of childhooda

Onset CK Early
Disease Protein (gene) (average) range ndings Other signs Late ndings
Autosomal dominant
LGMD1B Lamin A/C (LMNA) Birth to Normal Proximal Arrhythmia and Mild
adult, or lower other cardiac contractures
50 % with mildly limb complications of elbows
childhood elevated weakness
onset
LGMD1C Caveolin (CAV3) 5 years 425 Toe Calf hypertrophy Proximal and
normal walking distal muscle
Muscle Rippling muscle weakness
cramps disease
Mild
proximal
weakness
Autosomal recessive
LGMD2C- Sarcoglycan (SGCG, 315 Mild to Proximal Calf hypertrophy Late
2F SGCA, SGCB, SGCD) years (8.5 very weakness contractures
years) elevated Scoliosis
Wheelchair
bound around
15 years old
LGMD2A Calpain-3(CAPN3) 815 Normal Difculty Scapular winging Multiple joint
years to running contractures
580 and (elbows and
of walking Achilles
normal tendon)
Stiff back Muscle
Scoliosis wasting (hip
Muscle biopsy extensors and
(lobulated bers) knee exors)
LGMD2G Telethonin (TCAP) 915 317 Difculty Cardiomyopathy Proximal and
years normal running, (50 %) distal leg
walking weakness
Foot drop Muscle biopsy Proximal arm
(rimmed weakness
vacuoles) Wheelchair
bound about
18 years from
onset
LGMD2I Fukutin-related protein 1.527 Normal Difculty Calf and tongue Wheelchair
(FKRP) years to very running, hypertrophy bound about
(11.5 elevated walking 25 years from
years) Proximal Early cardiac onset
arm more involvement
than leg
weakness
LGMD2K O-mannosyltransferase 1 13 years 2040 Difculty Calf and thigh Ambulatory
(POMT1) normal with stairs hypertrophy over longer
and periods of
walking follow-up
Mild Cognitive delay
weakness Elbow, spine, and
neck contractures
possible
(continued)
36 Inherited Muscle Disease 849

Table 5 (continued)
Onset CK Early
Disease Protein (gene) (average) range ndings Other signs Late ndings
LGMD2M Fukutin (FKTN) 4 450 Early Hypertrophy of
months4 normal muscle calves, thighs,
years weakness and triceps
Proximal
leg more
than arm
weakness
LGMD2N O-mannosyltransferase-2 18 months 450 Slow in Scapular winging
(POMT2) normal running Lordosis
and Intellectual
getting up disability
LGMD2O O-mannose B-1, 2-N- First to 450 Difculty Hypertrophy of
acetylglucosaminyl second normal rising calves and
transferase decade from quadriceps
(POMGnT1) sitting
Difculty Wasting of
with steps deltoids and
hamstrings
a
Due to variable disease severity and age of presentation, there is genetic overlap between the LGMD and CMD families

They tend to have involvement of proximal mus-
cles before distal, and legs before arms, with pro-
gression and eventual loss of independent
ambulation.
Emery-Dreifuss muscular dystrophy
(EDMD) is a form of muscular dystrophy charac-
terized by early contractures (often before there is
signicant weakness), slowly progressive muscle
wasting and weakness, and cardiac conduction
defects (Emery 2000; Gueneau et al. 2009). The
onset is typically in childhood, and progression of
muscle weakness is slow. X-linked EDMD is
caused by mutations in the emerin gene, and the
more common autosomal dominant or rarely auto-
somal recessive form of EDMD is caused by
mutations in the LMNA gene encoding the protein
lamin A/C. There are patients with the EDMD
phenotype without emerin or LMNA mutations
which led to the discovery of several other genes
that can yield an EDMD phenotype including:
SYNE1, SYNE2, and FHL1. The upper extremities
are particularly affected in this group of disorders.
Early contractures occur in the elbows and post-
cervical muscles as well as in the Achilles ten-
dons. Patients can also have spinal rigidity (see
further description of this in section Diagnostic
Features in Inherited Muscle Disease and Con-
cepts of Upper Extremity Involvement below).
Muscle weakness early in the disease course
tends to occur in a scapulo-humero-peroneal dis-
tribution. Due to the high risk of cardiac conduc-
tion abnormalities and even sudden cardiac
death, the risk of which may require the implan-
tation of an intracardiac debrillator device
(ICD), and it is imperative that these children be
referred to cardiology and maintain regular car-
diac follow-up. In suspected cases of EDMD,
cardiology referral should be made promptly,
even prior to diagnostic or genetic conrmation
of the disorder.
Pompe disease is also known as glycogen
storage disease type II or acid maltase deciency.
It is a disorder caused by a deciency in the
enzyme acid -glucosidase (GAA), leading to an
accumulation of glycogen in various tissues. This
accumulation leads to deleterious effects and
affects primarily the skeletal muscle, heart, liver,
and respiratory function. Age of onset varies from
an infantile form with onset within the rst year of
life to a later onset. Patients with the infantile form
can have symptom onset within the rst few
months of life characterized by hypotonia,
850
Table 6 Muscle disease with associated head drop
Muscular dystrophies Congenital myopathies
FSHD Nemaline myopathy
Laminopathy
SEPN1-related dystrophy
weakness, and cardiomyopathy. These patients
have progressive weakness and cardiorespiratory
failure and if untreated (see later section regarding
enzyme replacement therapy) typically do not
survive beyond 12 years of life. Adult patients
may have a less fulminant disease course: skeletal
muscle weakness is milder and there may not be
cardiac involvement. However, these patients are
still susceptible to respiratory failure that can exist
out of proportion to the degree of skeletal muscle
weakness. A later section in this chapter
will discuss the treatment of children with
Pompe disease; however, it is imperative to mon-
itor their cardiac, pulmonary, and, in the infantile-
onset Pompe disease patients, hepatic function
as well.
Diagnostic Features in Inherited
Muscle Disease and Concepts of Upper
Extremity Involvement
When evaluating an infant or child with suspected
muscle disease, the above list may be quite
exhaustive and elusive to those individuals not
trained in making the formal genetic or clinical
diagnosis of children with complex neuromuscu-
lar conditions. Another diagnostic approach
is to try and identify salient features that help
characterize certain conditions from the others.
The following subsections help to stratify
disorders based upon key distinguishing clinical
features.
Central Nervous System Involvement
In general, the presence of intellectual impair-
ment, brain MRI changes including structural
changes of the cortex, cerebellum, and brainstem,
or vision changes should prompt evaluation for
D.X. Bharucha-Goebel and C.G. Bnnemann
Myotonic dystrophies Metabolic myopathies
DM1 Pompe (acid maltase deciency)
DM2
suspected congenital muscular dystrophy, more
specically the alpha-dystroglycanopathies. In
addition, white matter changes can be seen in
patients with LAMA2-related congenital muscular
dystrophy, usually with normal cognitive func-
tion. Cognitive involvement can also be seen in
dystrophinopathies and in myotonic dystrophy.
Overall, in the remainder of the inherited muscle
diseases, children have normal to above normal
intelligence.
Muscle Diseases with Dropped Head
Syndrome
The term dropped head syndrome refers to
patients with neck extensor weakness leading to
difculty holding the head upright. Table 6 pro-
vides a list of disorders where more striking neck
extensor weakness can be seen earlier in disease
(Finsterer and Strobl 2011).
Common Muscle Diseases with Spine
Involvement
Varying degrees of spinal involvement from sco-
liosis to spinal rigidity can be seen in children
with neuromuscular disease (Finsterer and Strobl
2011). In some cases, this may be the salient
feature, and in others, spinal ndings may occur
later in the disease with progression of generalized
muscle weakness. While the table below lists sev-
eral conditions that can have more notable
lumbar lordosis, note that a hyperlordotic posture
is often a manifestation of truncal and proximal
weakness when standing and ambulating
and, therefore, can be seen more broadly in vari-
ous neuromuscular disorders. Table 7 highlights
the types of spinal involvement in various muscle
disorders (Fig. 2).
36 Inherited Muscle Disease 851

Table 7 Spine involvement in neuromuscular disease

Scoliosis Hyperlordosis Kyphosis Spinal rigiditya
Dystrophinopathy Dystrophinopathy Dystrophinopathy Laminopathy
FSHD Calpain Laminopathy Emery-Dreifuss
MD
Laminopathy (LMNA) Sarcoglycan DM1 FHL1
Ullrich CMD/Bethlem Laminopathy Nemaline myopathy SEPN1-related
myopathy (ACTA1) dystrophy
SEPN1-related dystrophy FSHD Dysferlin
Multi-minicore disease Central core disease/RYR1 associated Ullrich CMD
myopathy
DM1 Multi-minicore disease Multi-minicore
disease
DM1 Pompe disease
a
Rigid spine is dened by partial or complete restriction of vertebral segments to move against each other. Patients may
have difculty bending forward or rotating the spine. See Fig. 2

Fig. 2 Patient with spinal

rigidity seen when patient
asked to bend forward.

Scapular Winging rest or may be seen by maneuvers such as

Scapular winging is dened by spontaneous or
provoked protrusion of the shoulder blade from
the thoracic wall. Scapular winging when seen
in inherited muscle diseases is typically related
to weakness of the scapulothoracic muscles.
Scapular winging may be present with arms at
asking the patient to stretch arms out forward
with palms facing forward and pushing
against slight resistance (see Fig. 3). The follow-
ing disorders have been associated with
scapular winging: LGMD2A (calpain 3), facio-
scapulohumeral muscular dystrophy, beta- and
gamma-sarcoglycanopathy, Emery-Dreifuss MD,
852
Fig. 3 Scapular Winging
laminopathy (LMNA), scapuloperoneal muscular
dystrophy (FHL1), LGMD2I (FKRP), telethonin,
nemaline myopathy due to ACTA1 mutations,
and Pompe disease (Finsterer and Strobl 2011;
Pestronk 2013).
Contractures and Arthrogryposis
Contractures are a frequent orthopedic problem in
patients with inherited muscle disease. In some
diseases (collagen VI-related disorders and
Emery-Dreifuss MD), the contractures can be
even more debilitating than the weakness in
early stages of disease. Figure 4a, b demonstrates
the progression of contractures that can be seen,
with photos showing two different patients with
collagen VI-related dystrophy at an early stage
(Fig. 4a) and later stage (Fig. 4b) of disease.
Figure 4c highlights the long nger exor con-
tractures that are seen in patients with collagen
VI-related dystrophy. Table 8 highlights diseases
associated with prominent upper extremity con-
tractures earlier in disease course (note that in later
stages of disease, more diffuse contractures can be
seen in various neuromuscular disorders).
The presence of arthrogryposis (or contrac-
tures of two or more joints at birth), while not
specic to neuromuscular disease, can be seen in
certain neuromuscular disorders, typically as a
D.X. Bharucha-Goebel and C.G. Bnnemann
consequence of limited fetal movement. In gen-
eral, the incidence of arthrogryposis is about 1 in
3,000 children (Pestronk 2013). These joint con-
tractures tend to occur in distal muscles more than
proximal muscles and often involve a exed pos-
ture of a joint. This can be seen in congenital
myopathies (including but not limited to muta-
tions of: TPM3, TPM2, TNNT3, TNNI2, ACTA1,
NEB, RYR1, MTM1, BIN1, MYH2, MYH3,
MYH8), congenital muscular dystrophies, and
myotonic dystrophy. While it is beyond the
scope of this chapter, it is important to highlight
that there are also patients with arthrogryposis
multiplex congenita with more severe and exten-
sive involvement of multiple joint contractures at
birth, for which an underlying genetic etiology
may or may not be known.
Joint Hyperlaxity
In addition to contractures, children with inherited
muscle diseases are also at risk for joint
hyperlaxity, dislocations, or subluxations. The
dislocation and subluxation of joints can be seen
in the setting of laxity or due to weakness leading
to poor stabilization of given joints. Congenital
muscle diseases with features of decreased fetal
movement and weakness beginning in utero can
be associated with hip dislocations/hip dysplasia
36 Inherited Muscle Disease 853

Fig. 4 (a, b) Reveal prominent elbow exion contractures. (c) Finger exion contracture

Table 8 Contractures affecting upper extremity function Interestingly, in patients with collagen VI-related
and associate diseases disorders, patients can have striking hyperlaxity
Disorder Prominent areas of contractures earlier in disease in some joints while also dem-
EDMD (emerin or Elbow, cervical spine onstrating contractures in other joints. Later in the
lamin A/C) disease, there tends to be more progression of
Congenital Proximal contractures, rigid spine joint contractures, including in the hands (see
laminopathy
section above). Some disorders are associated
Collage VI-related Proximal contractures, long nger
disorders exor contractures, elbow with focal areas of joint hypermobility (such as
contractures the distal nger or hand joints), while others may
be associated with more generalized
hypermobility. Inherited muscle diseases that are
associated with joint hypermobility include colla-
at birth. Joint dislocation can also be seen in later gen VI-related muscular dystrophy, SEPN1-
stages of disease due to changes in posture and related myopathy, RYR1-related myopathy, and
severe scoliosis that can leave joints in extreme beta-sarcoglycanopathy/LGMD 2E (Voermans
nonphysiological extension or exion. Joint et al. 2009). While not the focus of this chapter,
hypermobility is dened as an abnormally patients with spinal muscular atrophy and with
increased active and/or passive range of motion certain inherited neuropathies may also have
in a joint (Voermans et al. 2009). See Fig. 5 dem- joint hypermobility and in the correct clinical
onstrating distal joint hyperlaxity that can be seen context should be considered in the differential
earlier in the disease course in a patient with diagnosis of a child with suspected neuromuscular
Ullrich congenital muscular dystrophy. disease and joint hypermobility.
854
Fig. 5 Joint hyperlaxity in
a patient with ullrich
congenital muscular
dystrophy
Early Upper Extremity Weakness
In general, many inherited muscle diseases
either involve the upper and lower extremity
muscles simultaneously (as can be seen in con-
genital myopathies) or may involve the lower
extremity muscles more severely or earlier
than the upper extremities (as can be seen in
dystrophinopathies, sarcoglycanopathies, alpha-
dystroglycanopathies, and several other limb-
girdle muscular dystrophies). The exception
to these situations are those disorders that
have preferential upper extremity involvement
early in disease, which should have a more
narrow differential diagnosis including: Emery-
Dreifuss muscular dystrophy, FKRP (LGMD2I),
and FSHD.
Early Distal Muscle Involvement
In general, in most inherited muscle diseases,
there tends to be more proximal muscle involve-
ment than distal muscle involvement early in dis-
ease course (note that in later stages of disease,
both proximal and distal muscles may be involved
with weakness and/or contractures). There are a
few exceptions, in which distal muscles are pref-
erentially involved more severely and/or earlier in
the disease course. Those which can involve distal
arm and leg muscles include nebulin (targets toe
and nger extensor muscles) and dynamin
2. Those disorders that predominantly involve
D.X. Bharucha-Goebel and C.G. Bnnemann
distal leg muscles rst include mutations of the
following: titin, MYH7, FHL1 (in the
scapuloperoneal presentation of reducing body
myopathy), dysferlin (LGMD2B), and anoctamin
5 (LGMD2L).
Diagnostic Approaches
Occasionally, infants or children with inherited
muscle disease will be referred for orthopedic
consultation before it is recognized that there
may be an underlying neuromuscular disorder.
Unexplained hip dislocation in a hypotonic neo-
nate should generally prompt further neurological
workup to determine central or peripheral causes
of hypotonia. When a neuromuscular disorder is
suspected, referral to a neuromuscular specialist is
recommended. Likewise, children with orthope-
dic issues including unexplained contractures,
joint laxity, spinal rigidity, or progressive scoliosis
especially in the setting of weakness should war-
rant evaluation by a neuromuscular specialist.
The workup of a child with suspected inherited
muscle disease always begins by paying careful
attention to the clinical features. A thorough clin-
ical history (including prenatal, birth, and devel-
opmental history) as well as a detailed
examination is the most important aspect of the
neuromuscular evaluation. Details that should be
elicited for any pertinent family history include
but are not limited to: weakness, motor develop-
mental delays, early loss of ambulation,
36 Inherited Muscle Disease
cardiomyopathy, arrhythmia, or ocular ndings.
Coexisting issues that children with congenital
muscle disorders can have on prenatal, birth, or
developmental history include (but are not limited
to) polyhydramnios in utero, decreased fetal
movement, breech position, hip dislocation at
birth, fractures at birth (due to osteopenia related
to decreased fetal movement in utero), high
arched palate, ptosis, respiratory difculties
(or even respiratory failure), feeding difculties,
weak cry, speech articulation difculties,
hypernasal quality speech, ne motor difculties
due to hand weakness or laxity, and nocturnal
hypoventilation. It is always helpful to ask fami-
lies when they (or the healthcare providers) rst
had a concern. It is also very useful to get a sense
of the time course: Is this a condition that is stable,
slowly progressive, rapidly deteriorating, or one
where the patient is making improvements?
The clinical examination can best be done by
observing the infant at rest and with activity or
observing the child in natural play even prior to
the neurological examination. Attention should
be given to resting posture, antigravity move-
ments, and quality of movements. In a child, it
is helpful to evaluate their gait, ability to stand on
toes and heels, ability to arise from supine to
standing, ability to run, and ability to climb
steps. In addition to strength testing, muscle
tone and muscle bulk should be evaluated.
Reexes may be normal, reduced, or absent in
disorders of muscle. Additional assessments
such as use of myometry assessments can pro-
vide more quantitative measures of strength in
cooperative patients. More detailed therapy
assessments of the upper extremity strength
and function will be covered in chapters Out-
come Measures and Occupational Therapy
Evaluation and Treatment.
When an inherited muscle disease is suspected,
additional workup is advised. Serum creatine
kinase level is almost always needed. In some
conditions such as Duchenne and Becker muscu-
lar dystrophy and many of the limb-girdle muscu-
lar dystrophies, the CK level is grossly elevated
up to 1050 times the normal. In general, the more
marked elevations in CK tend to occur in disor-
ders where there is a destructive or dystrophic
855
process of muscle. In congenital myopathies, the
CK may be normal or mildly elevated. In congen-
ital muscular dystrophies, the CK can be mildly or
moderately elevated. Typically, beyond this point,
more targeted diagnostic tests should often be
performed by or prescribed by a neuromuscular
specialist. These tests may include muscle imag-
ing (by muscle ultrasound or muscle MRI), brain
imaging, EMG/nerve conduction study, muscle
biopsy, skin biopsy, and targeted genetic testing.
These efforts and the workup in the search for a
diagnosis may seem long and tedious (for fami-
lies, the patients, and even the healthcare pro-
viders), but the end goal of establishing a
diagnosis has multiple benets for a patient and
their family including: a better understanding of
the disease process and prognosis, more appropri-
ate and targeted referral for screening for known
comorbid conditions (such as cardiomyopathy or
ocular involvement), genetic counseling as it per-
tains to recurrence risk for future pregnancies or
other family members, and most importantly, as
clinical trials become available, a conrmed diag-
nosis will allow patients to potentially be consid-
ered for participation in such trials. As mentioned
above, in the process of trying to diagnose a
patient with suspected inherited muscle disease,
it is important to have an assessment of cardiac
function (with echocardiogram and EKG) as some
of these disorders can be associated with cardio-
myopathy or severe conduction decits.
Management Approaches
The management of patients with inherited mus-
cle disorders starts with a multidisciplinary
approach to their care. Ideally, the patient will be
seen by a team of providers who are familiar with
the diagnosis and management of neuromuscular
disorders. This team may ideally include a neu-
rologist, pulmonologist, cardiologist, orthopedist,
physiatrist, genetics counselor, physical therapist,
occupational therapist, nutritionist, social worker,
and psychologist or psychiatrist. The multidis-
ciplinary approach will allow for effective com-
munication between services and also a
comprehensive approach to care of these patients.
856
There are many documents that exist on standards
of care, and providers should periodically review
the literature for standards of care parameters as it
pertains to certain groups of disorders.
Maintenance of function begins with keeping
active and maintaining mobility/range of motion.
Regardless of degree of weakness, some
stretching or range of motion exercises (per the
recommendation of a trained therapist) will be
benecial to a patient with an inherited muscle
disease. Prevention or management of areas of
tightness and contractures will be benecial to
maintaining function in the upper and lower
extremities. Some patients will benet from ther-
apy in or out of the school environment, and
others may only need some stretching regimen at
home. For those children who are able, it is impor-
tant for them to keep active. In general, weight
training or activities involving resistance should
be avoided (e.g., weight lifting) in children or
adults with inherited muscle diseases. These exer-
cises such as weight lifting that involve eccentric
contraction of muscle can actually be more harm-
ful to patients (especially those with dystrophic
muscle disorders), and therefore, it is important to
advise those patients and families to avoid such
forms of exercise. Mild to moderate aerobic con-
centric or isometric exercises as tolerated are per-
missible and in fact are benecial to overall
health. In addition, provided that it is performed
in a safe setting for the child with appropriate
adult supervision, swimming and/or aquatic ther-
apy is an excellent activity for children with
inherited muscle disorders. Management of con-
tractures when they exist may involve daily
stretching, ongoing outpatient therapy, bracing
(daytime or nighttime or both), serial casting,
and in some cases tendon release. Extreme caution
should be made with tendon release in certain
populations. For example, existing elbow exion
contractures in some patients may be functionally
advantageous to allow a weak patient to bring
food to his/her mouth and for certain ADLs. The
release of such contractures in a more severely
weak individual could affect that individuals abil-
ity to raise his/her hand to mouth or face and may
be an unacceptable loss of function. When
D.X. Bharucha-Goebel and C.G. Bnnemann
considering such management, it is helpful to
discuss these plans on a case-by-case basis with
the families, the patient, and the neuromuscular
team (including the neurologists/physiatrists and
therapists involved in a patients care). Botox is
generally not recommended in the neuromuscular
population due to irreversible effects on the neu-
romuscular junction with the risk of further weak-
ness in an already weak population. There may be
a few exceptions or cases where there is over
whelming spasticity and contractive due to central
nervous system involvement, and botox may be
bencial. This should be determind on a case-by-
case basis.
Many children with inherited muscle disease
will have respiratory involvement. In order to
maintain overall health, it is imperative that respi-
ratory care be optimized. This can be done by
regular follow-up and evaluation in pulmonology
and with a proactive approach to optimization of
pulmonary health. Children with neuromuscular
disease are at risk for issues related to poor clear-
ance of secretions (due to weak cough), sleep
apnea/nocturnal hypoventilation, and overall
respiratory insufciency. Factors that contribute
to these respiratory complications include dia-
phragmatic weakness, weakness of muscles used
for inspiration (intercostal and accessory mus-
cles), chest wall stiffness, scoliosis that affects
the dynamics of breathing and lung volumes,
and oropharyngeal weakness. With few excep-
tions, most children with inherited muscle dis-
eases will need to be seen by a pulmonologist.
Pulmonary assessments that are followed over
time may include PFTs (should be performed in
both the seated and supine positions) as well as
sleep studies. Management strategies include
chest PT, nebulizer treatments, cough assist, and
suctioning routines for times of illness or
increased secretions. Other devices that may be
used for optimizing clearance of secretions and
bronchial hygiene include intrapulmonary percus-
sive ventilation or IPV (delivered by a mouthpiece
in non-ventilated patients or through the endotra-
cheal tube in ventilated patients) and respiratory
vest (also known as Vest Airway Clearance, The
Vest, Theravest, etc.). In addition, Bipap
36 Inherited Muscle Disease
(or bilevel pressure ventilation) is often required
in patients with nocturnal hypoventilation. In later
stages of disease or as the respiratory failure
becomes more severe and prevalent, daytime ven-
tilator support may be needed with tracheostomy
and ventilator support or with a sip and puff ven-
tilator (noninvasive diurnal ventilator) support.
Seasonal inuenza vaccination and preventive
care are crucial. In a subset of very weak neonates
with pulmonary involvement, monthly Synagis
vaccination (for RSV) may also be needed.
As mentioned above, cardiac assessments are
imperative. Referral to cardiology early in the
workup is often helpful to determine at baseline
if there exists any cardiomyopathy or defects of
cardiac conduction. Depending on the nature of a
given disorder and extent of known cardiac
involvement, the frequency of intermittent cardiac
follow-up and surveillance can then be
established. In more advanced stages of disease,
more frequent follow-up will be needed.
While most of the inherited muscle diseases are
managed through maintenance of health and sup-
portive measures to preserve function, there are a
few disorders that warrant further discussion in
regard to treatment options. It is beyond this chap-
ter to provide a comprehensive review of the initi-
ation of these treatments (as this should be done by
a neuromuscular specialist who is familiar with
these disorders). However, it is important for
other providers to know that some of these condi-
tions do have therapies that can augment function.
Two that will be discussed here include Pompe
Disease and Duchenne muscular dystrophy.
Duchenne Muscular Dystrophy
and Corticosteroids
Glucocorticoids are the only medication currently
available that show a documented slowing in the
decline of function and a delay to the time of loss
of ambulation in patients with DMD. Based upon
standard of care documents in Duchenne muscu-
lar dystrophy, glucocorticoid therapy should be
considered in all patients with Duchenne muscu-
lar dystrophy. The timing of when to start is
857
somewhat controversial. At the minimum, gluco-
corticoid therapy should be offered to patients
with Duchenne muscular dystrophy around the
time of plateau or at the earliest sign of decline
in motor function (typically by 48 years of age).
Some providers may take a more aggressive
approach and start glucocorticoids earlier. How-
ever, it is not recommended for glucocorticoids to
be started very early when a child is still making
motor gains or under the age of 2 years old. With
the initiation of glucocorticoid therapy, there
tends to be an initial improvement in function as
well as a delay to the time of decline in function
and an overall prolonged period of independent
ambulation. Again, the initiation and management
of this needs to be done by those familiar with this
population, as there are many side effects that
need to be monitored including: weight gain,
blood pressure, mood, bone health, short stature,
among others.
Enzyme Replacement Therapy: Pompe
Disease
Enzyme replacement therapy (ERT) with
alglucosidase alfa (brand name Myozyme)
was FDA approved in 2006 for treatment of
patients with Pompe disease. In the infantile-onset
form of Pompe Disease, ERT effectively lengthens
survival, improves motor function, and improves
cardiac function. In 2010, the FDA granted
approval for a similar replacement therapy com-
pound, Lumizyme (alglucosidase alfa), to be used
for the treatment of late-onset Pompe disease.
Summary
The approach to patients with inherited muscle
disease is one that is done best with a multidis-
ciplinary approach. A partnership among various
care providers will ensure optimal communication
regarding the multifaceted patient care issues in
this medically complex population and will there-
fore lead to optimal care. The evaluation of
suspected inherited muscle disease can be quite
858
complex and is likely best done by a trained
specialist. However, recognition of these groups
of disorders as described in this chapter will hope-
fully allow various providers to effectively detect
patients who have subtle signs of a neuromuscu-
lar condition and refer them earlier for diagnostic
assessment and follow-up. In addition, having a
sense of the comorbidities associated with some
of these disorders, especially the risk for respira-
tory and cardiac complications, will help all pro-
viders to refer these patients early and often for
necessary assessments. While the focus of this
chapter was predominantly on the involvement
of the upper extremity in inherited muscle dis-
ease, it is of course always helpful to keep in
mind that many of these disorders can have
much more extensive involvement and pattern
of muscle weakness in both the upper and lower
extremities, the facial muscles, as well as other
coexisting conditions such as: ocular involve-
ment, respiratory involvement, cardiac involve-
ment, intellectual impairment, epilepsy, and also
orthopedic complications such as arthrogryposis,
scoliosis, joint subluxation, or dislocations. While
to date there are not many approved therapies in
these disorders, there is a great deal of research in
the eld. Small molecule compounds as well as
genetic-based therapies are being evaluated, and
hopefully, there will be great advances in this
eld in coming years. Some of the upcoming
approaches have great promise in animal models,
and the eld is on the brink of seeing an immense
advancement in therapeutic options in coming
years. In the interim, all care providers have
the ability and responsibility to impact in the
quality of life and in the overall health of
these patients. From optimizing motor function
through therapy and maintenance of activity to
improving comfort by managing contractures to
providing strategies for effective clearing of
secretions with illness, each member of the care
team is crucial to improving the quality and lon-
gevity of the lives of patients with neuromuscular
disease.
D.X. Bharucha-Goebel and C.G. Bnnemann
References
Bird TD. Myotonic dystrophy Type 1. 1999 (Updated
2013). In: Pagon RA, Adam MP, Bird TD, et al.,
editors. GeneReviews (Internet). Seattle: University of
Washington; 19932013. Available from: http://www.
ncbi.nlm.nih.gov/books/NBK1165/
Brooke MH, Griggs RC, Mendell JR, et al. Clinical trial in
Duchenne dystrophy. I. The design of the protocol.
Muscle Nerve. 1981;4:18697.
Dalton JC, Ranum LP, Day JW. Myotonic dystrophy Type
2. In: Pagon RA, Adam MP, Bird TD, et al., editors.
GeneReviews (Internet). Seattle: University of
Washington; 19932013. Available from: http://www.
ncbi.nlm.nih.gov/books/NBK1466/
Emery A. Emery-Dreifuss muscular dystrophy a 40 year
retrospective. Neuromuscul Disord. 2000;10:22832.
Finsterer J, Strobl W. Orthopaedic abnormalities in primary
myopathies. Acta Orthop Belg. 2011;77:56382.
Godfrey C, Clement E, Mein R, et al. Rening genotype-
phenotype correlations in muscular dystrophies with
defective glycosylation of dystroglycan. Brain.
2007;130:272535.
Goebel HH. Congenital myopathies in the new millen-
nium. J Child Neurol. 2005;20:94101.
Gueneau L, Bertrand AT, Jais JP, et al. Mutations of the
FHL1 gene cause Emery-Dreifuss muscular dystrophy.
Am J Hum Genet. 2009;85:33853.
Jungbluth H, Sewry CA, Muntoni F. Whats new in neuro-
muscular disorders? The congenital myopathies. Eur J
Paediatr Neurol. 2003;7(1):2330.
Pegoraro E, Hoffman EP. Limb-Girdle muscular dystrophy
overview. 2000 (Updated 2012). In: Pagon RA, Adam
MP, Bird TD, et al., editors. GeneReviews (Internet).
Seattle: University of Washington; 19932013. Available
from: http://www.ncbi.nlm.nih.gov/books/NBK1408/
Pestronk A. Neuromuscular Disease Center (Internet).
St. Louis. (cited October 2013). Available from:
http://www.neuromuscular.wustl.edu
Pierson CR, Tomczak K, Agrawal P, et al. X-linked
Myotubular and centronuclear myopathies. J
Neuropathol Exp Neurol. 2005;64:55564.
Rosales XQ, Tsao CY. Childhood onset of Limb-Girdle
muscular dystrophy. Pediatr Neurol. 2012;46:1323.
Sparks S, Quijano-Roy S, Harper A, et al. Congenital mus-
cular dystrophy overview. 2001 (Updated 2012). In:
Pagon RA, Adam MP, Bird TD, et al., editors.
GeneReviews (Internet). Seattle: University of
Washington; 19932013. Available from: http://www.
ncbi.nlm.nih.gov/books/NBK1291/
Voermans NC, Bnnemann CG, Hamel BC, et al. Joint
hypermobility as a distinctive feature in the differential
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Part XI
Trauma
 The Multiply Injured Child
37
Susan Scherl

Contents Abstract
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 861 Trauma is the leading cause of death and dis-
ability in children. Fractures are rarely the
Common Mechanisms of Injury . . . . . . . . . . . . . . . . . . . 863
Child Abuse . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 863
cause of mortality in multiply injured children
Falls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 864 but contribute signicantly to morbidity.
Motor Vehicles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 864 Regardless of the mechanism causing the mul-
Evaluation and Resuscitation . . . . . . . . . . . . . . . . . . . . . . 865 tiple injuries, the initial medical management
Initial Assessment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 865 focuses on the life-threatening, non-orthopedic
Fluid Replacement . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 866 injuries to stabilize the childs condition. Sub-
Secondary Assessment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 866 sequent optimal treatment of orthopedic inju-
Trauma Rating Systems . . . . . . . . . . . . . . . . . . . . . . . . . . . . 866 ries, in a timely fashion, decreases the burden
Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 867 of musculoskeletal disability.
Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 867
Computed Tomography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 867
Intravenous Pyelography (IVP) . . . . . . . . . . . . . . . . . . . . . . 868
Magnetic Resonance Imaging . . . . . . . . . . . . . . . . . . . . . . . 868
Introduction
Ultrasound . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 868
Trauma is the leading cause of death in the pedi-
Non-orthopedic Injuries . . . . . . . . . . . . . . . . . . . . . . . . . . . . 868
Head Injury . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 868 atric population and in fact is more common than
Abdominal Injury . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 869 all other causes combined (Arias et al. 2003;
Genitourinary Injury . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 869 American Academy of Pediatrics Section on
Fat and Pulmonary Embolism . . . . . . . . . . . . . . . . . . . . . . . 870 Orthopaedics et al. 2008). Further, for every
Orthopedic Injuries . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 870 death, there are 18 hospitalizations and 233 emer-
Timing of Fixation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 870 gency department visits (Burt and Fingerhut
Pelvic Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 870
Open Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 871
1998). In the United States, there are about 20
million injuries to children and adolescents requir-
Conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 872 ing medical attention annually, with costs of about
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 873 17 billion dollars (Danseco et al. 2000). A 1997
national pediatric inpatient database reported
84,000 orthopedic trauma admissions, costing
$932.8 million in hospital charges (Galano
S. Scherl
et al. 2005). Treatment of children with femoral
Orthopedic Surgery, The University of Nebraska, Omaha,
NE, USA fractures in the United States in 2000 resulted
e-mail: sscherl@unmc.edu in expenditures in excess of $222 million
# Springer Science+Business Media New York 2015 861
J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_38
862
(Loder et al. 2006). In 2003, the mean expenditure
per hospitalization for pediatric injury was
$28,137, with a median of $10,808 (Pressley
et al. 2007).
In 2012, Centers for Disease Control published
a 10-year longitudinal study of unintentional
injury deaths in children (Centers for Disease
Control and Prevention 2012). Thirty-seven
percent of total childhood deaths were secondary
to accidents, conrming that accidents remained
the leading cause of death through the course of
the study. However, the death rate decreased by
29 % between 2000 and 2009, from 15.5 to 11.0
per 100,000 population. There were 9,143 pediat-
ric trauma-related deaths in 2009.
Boys are about twice as likely to sustain a
traumatic injury as girls, and there is evidence
that a greater percentage of injuries in males
may require hospital admission (Schalamon
et al. 2003; Sullivan et al. 2003; Borse and Sleet
2009). Penetrating trauma is less common in chil-
dren than in adults. Blunt trauma secondary to child
abuse, vehicular accidents, and falls from a height
accounts for most polytrauma in children (Buckley
et al. 1994). Severe head injury and combined
head, chest, and abdominal trauma are the most
common causes of death in the pediatric trauma
population (Kim et al. 2000; Sharma et al. 2006).
As children enter adolescence, their mecha-
nisms of injury and injury patterns become more
similar to those of adults. Alcohol abuse is a factor
in about one third of accidents causing injuries in
teenagers (Maio et al. 1994). Orthopedic surgeons
treating teenagers who have been involved in
vehicular accidents need to maintain a high level
of suspicion for potential alcohol abuse in this age
group, as well as a low threshold for referring
adolescents to appropriate counseling to avoid
future accidents and injuries (Sindelar et al. 2004).
Fractures are common in multitrauma and
contribute signicantly to morbidity, but rarely
are the underlying cause of mortality (Colombani
et al. 1985; Buckley et al. 1994; Cramer 1995;
Sindelar et al. 2004). Extremity fractures have
been estimated to occur in up to 76 % of cases of
polytrauma (Schalamon et al. 2003). Femoral
shaft fractures are common; one study found
they accounted for 22 % of fractures associated
S. Scherl
Fig. 1 Waddells triad (Courtesy of Dan Zlotolow, MD)
with polytrauma, with 9 % open (Buckley
et al. 1994). Fractures of the spine, pelvis, and
scapula and clavicle were less common but, due to
associated non-orthopedic injuries, led to longer
hospital and intensive care unit stays and higher
mortality rates.
Fractures of the ipsilateral upper and lower
extremity or of the femur and pelvis are common
in children struck by motor vehicles (Brainard
et al. 1992). In one study, 58 % (87/149) of chil-
dren with femoral fractures due to MVAs were
noted to have associated injuries, including 14 %
with head injuries, 6 % with chest injuries, 5 %
with abdominal injuries, and 4 % with genitouri-
nary injuries (Jawadi and Letts 2003). The coex-
istence of a femoral fracture and a head injury
indicates substantial high-energy trauma and has
a more guarded prognosis than does either of these
injuries alone. In pediatric patients struck by a
motor vehicle, a constellation of injuries known
as Waddells triad occurs. This includes a femur
fracture sustained by the initial impact of the
bumper to the childs lower extremity, followed
by a thoracic or abdominal injury when the childs
upper body is impacted by the hood of the vehicle,
and nally, a head injury sustained when the child
is thrown to the ground after impact (Waddell and
Drucker 1971) (Fig. 1).
The American College of Surgeons has
established criteria for pediatric trauma centers,
very similar to those for adult trauma centers.
The key guidelines are rapid transport and treat-
ment by a dedicated multidisciplinary trauma
team, headed by a pediatric general surgeon, and
including a variety of other pediatric surgical
specialists. Advanced imaging, such as CT scan-
ning, and an operating room should also be avail-
able at all times. The basic premise underlying the
concept of the trauma center is that of the golden
37 The Multiply Injured Child
hour (Cowley 1976): that initiation of denitive
treatment within the rst hour after injury is crit-
ical in improving survival after polytrauma.
Numerous papers have supported the premise
that survival rates and outcomes for severely
injured and younger children are improved at a
pediatric trauma center compared to a community
hospital (Smith et al. 1990; Nakayama et al. 1992;
Hall et al. 1996; Potoka et al. 2000; Davis
et al. 2005; Odetola et al. 2005; Densmore
et al. 2006; MacKenzie et al. 2006; Stylianos
et al. 2006; Amini et al. 2011). However, such
centers are costly and difcult to establish and
maintain, and there are many regions without
one. Therefore, many pediatric trauma patients
are stabilized at other hospitals before transfer to
a pediatric trauma center or are treated denitively
at an adult trauma center. One European cohort
study comparing 2,961 pediatric polytrauma
patients to 21,435 adults found that the golden
hour for pediatric patients often expires in the
eld or during transfer between hospitals (Wyen
et al. 2010).
The use of a general trauma center for pediatric
trauma care may be a necessary and acceptable
alternative if it is not feasible to bring children
directly to a pediatric trauma center (Knudson
et al. 1992; Sanchez et al. 2001). Delayed transfer
and improved transfer coordination may also opti-
mize patient outcomes (Larson et al. 2004;
Sabharwal et al. 2007; Soundappan et al. 2007).
Common Mechanisms of Injury
Child Abuse
Child abuse is a societal problem that crosses all
socioeconomic and ethnic groups and is the most
common cause of traumatic death in infants and
toddlers. In 2011, there were 3.4 million reports of
child abuse, involving 6.2 million children. Sixty-
one percent of the reports were investigated, of
which 18.5 % were substantiated, for a victimiza-
tion rate of about 9.1 per 1,000 children. There
were 1,570 child abuse fatalities in 2011 (United
States Administration for Children, Youth, and
Families, Child Welfare Information Gateway
863
(U.S.) 2011). Non-accidental trauma has higher
mortality and morbidity than accidental trauma
(Roaten et al. 2006). This diagnosis must be
suspected in all cases of multiple injuries in chil-
dren younger than 2 years old if there is no obvi-
ous and/or witnessed plausible explanation of the
injuries. Abuse should be considered a possible
cause of injury in all young children with one or
more long-bone fractures in association with head
injury, or any other manifestations of physical
abuse, such as burns. All long-bone fractures in
non-ambulatory children should be considered
suspicious until proven otherwise. Pediatrician
condence in identifying these injuries remains
low (Trowbridge et al. 2005). Although the
metaphyseal corner fracture (sometimes called a
classic metaphyseal lesion, or CML) is the most
highly specic fracture for child abuse, it is rela-
tively rare. The most common extremity fracture
secondary to abuse is a single transverse fracture
of the femur or humerus (King et al. 1988). There
is no fracture that is pathognomonic of abuse;
child abuse is a clinical diagnosis made on the
basis of the patients entire clinical and social
picture. Orthopedic surgeons have difculty
distinguishing accidental from non-accidental
trauma on the basis of X-ray evaluation alone
(Lane and Dubowitz 2007). Although rib frac-
tures occur in only about 5 % of children with
multiple injuries from trauma of other causes, they
are more common in child abuse (Garcia
et al. 1990; Peclet et al. 1990). Whereas blunt
compressive trauma to the thorax from other
causes may result in lateral rib fractures, the rib
fractures seen in child abuse are often posterolat-
eral and adjacent to the transverse processes of the
thoracic spine, in three or four sequential ribs on
the childs left side (Kocher and Kasser 2000;
Barsness et al. 2003; Williams and Connolly
2004). As a result, posterior rib fractures are
very characteristic of child abuse.
A skeletal survey is routinely performed in
suspected cases of abuse. Some authors have
recommended a bone scan in conjunction with
the skeletal survey (Mandelstam et al. 2003),
although this recommendation is controversial
since the addition of a bone scan requires seda-
tion, elevates radiation exposure, and increases
864
cost (King et al. 1988; American Academy of
Pediatrics 2000). Increasingly, a follow-up skeletal
survey, performed several weeks after the index
procedure, is being recommended (Harlan et al.
2009; Harper et al. 2013).
Falls
Falls are a common primary mechanism of
multiple injuries in children (Greenberg 1978;
Rozycki and Maull 1991; Buckley et al. 1994;
Lallier et al. 1999; Wang et al. 2001). According
to a CDC report published in 2008, falls were the
leading cause of injury in children, accounting
for 2.8 million emergency department visits
(Borse and Sleet 2009). Falls occur more often
in younger children, causing 50 % of nonfatal
injuries in children under the age of 1 year (Borse
and Sleet 2009). Fractures from falls usually
result from direct impact, whereas internal inju-
ries are the result of deceleration forces present at
the time of landing. Many factors, including
body position at impact and composition of the
landing surface, affect injury severity after a fall
(Greenberg 1978). Injuries associated with falls
from heights include head injuries in 39 % of
children (Lallier et al. 1999), orthopedic injuries
in 3465 % (Lallier et al. 1999; Pitone and Attia
2006), and mortality in 5 % (Demetriades
et al. 2003).
Motor Vehicles
Accidents involving motor vehicles account for
many multiple system injuries in school-age chil-
dren and preadolescents. In 2009, 21,000 children
aged 14 years and younger were injured, and
318 were killed after being struck by motor vehi-
cles in the United States, an approximately 50 %
decrease since 2000 (United States National
Highway Trafc Safety Administration 2009).
For children riding in cars, there were 179,000
injuries and 1,314 deaths in this age group in
2009 (United States National Highway Trafc
Safety Administration 2009). The CDC reported
that between 2000 and 2006, transportation-
S. Scherl
related injuries accounted for 66 % of all accidental
deaths in children 019 years of age, corresponding
to a death rate of 9.8 per 100,000 (Borse and
Sleet 2009).
More than half of the children killed as passen-
gers were unrestrained at the time of the accident
(United States National Highway Trafc Safety
Administration 2009). Child safety seats are
effective when used properly: they reduce
the risk of fatal injury by 71 % for infants and
54 % for toddlers (United States National High-
way Trafc Safety Administration 2009). An
unrestrained child is three times more likely than
a restrained child to suffer an incapacitating
injury in a rollover accident and eight times
more likely in a side-impact accident (United
States National Highway Trafc Safety Adminis-
tration 2010b).
Noncompliance with car seat use contributes
signicantly to morbidity and mortality following
MVAs. One trauma centerreported that 80 % of
children treated there following MVAs were
unrestrained at the time of the accident (Thomp-
son et al. 2003). Vaca et al. (2002) noted that
many parents of young children were unaware of
basic safety information regarding child car seats
and airbags and that there are state laws mandating
the proper use of child seat restraints. Severe
injuries are higher for children in the front
seat (Brown et al. 2006). A study of pediatric
injuries sustained in MVAs showed higher mor-
tality, longer mean hospital stays, higher mean
hospital charges, more hospital admissions,
and more fractures, intra-abdominal injuries, and
head injuries in unrestrained passengers (Chan
et al. 2006).
Even with the appropriate use of car seats,
properly restrained children may be severely
injured. Zuckerbraun et al. (Zuckerbraun
et al. 2004) noted that younger children are more
prone to cervical spine injuries. Maintaining
proper padding in car seats can potentially
decrease the risk of head injury while children
are restrained (Kumaresan et al. 2002).
Most states require that infants and toddlers be
restrained in car seats when riding in a car, and it is
important to note that standard adult shoulder and
lap belts do not adequately restrain children who
37 The Multiply Injured Child 865

are too big for car seats and too small for the
standard restraints. Age- and size-appropriate car
seats and restraints are essential for child occupant
safety. The current recommendation from the
National Highway Trafc Safety Administration
is that children use car seats until the age of 4 and
booster seats from 4 to 8 (Administration 2010).
In addition, there is increasing public sentiment to
require seat belt use on school buses, a policy that
has been in place for physically disabled student
transport for some time. The safety of automobile
travel can be dramatically improved with appro-
priate parent education regarding child safety
seats and airbags and by enforcement of
current laws.
Pediatric bicyclists, as well as their adult coun-
terparts, are at risk of injury from motor vehicles.
All children (and adults) who ride a bicycle should
wear a helmet. Bicycle helmets are the most
important and cost-effective factor in preventing
bike-related injuries. Helmets confer a 6388 %
reduction in brain injury from a bicycle accident
(Gill 2012). One study estimated that universal
helmet usage would prevent 56,000 head injuries
per year at a cost savings of 1.3 billion dollars
(Schulman et al. 2002).

Evaluation and Resuscitation

Initial Assessment
Initial resuscitation of a multiply injured child
follows the Advanced Trauma Life Support
(ATLS) or Pediatric Advanced Life Support
(PALS) protocols and is essentially the same as
that of an adult patient (Armstrong 1992; Cramer
1995; Maksoud et al. 1995). Regardless of the
mechanism causing the multiple injuries, the
initial medical management focuses on the life-
threatening, non-orthopedic injuries to stabilize
the childs condition (Maksoud et al. 1995).
The primary survey is comprised of the
ABCs airway, breathing, circulation, disability
(neurologic), and exposure and screening
radiographs (cervical spine, chest, and pelvis).
Immediate establishment of an adequate airway
is the rst priority in the care of the pediatric
Fig. 2 Transport board use in children. (a) Adult back-
board with no cutout exes the c-spine in a pediatric
patient. (b) Pediatric backboard with cutout allows slight
extension of the c-spine. (c) An alternate to a pediatric
backboard is to add an additional board from the shoulders
distally to allow slight extension of the c-spine (Courtesy
of Dan Zlotolow, MD)
polytrauma patient. Special care should be taken
to protect the cervical spine, particularly during
transport of the patient. In children less than six
years of age, the head is disproportionately
large compared to the rest of the body. Because
of this, children in this age group require a spe-
cial transport board with a cutout for the occipital
area. This prevents potentially dangerous exion
of the cervical spine (Herzenberg et al. 1989)
(Fig. 2ac).
866
Fluid Replacement
Since hypovolemia is the most common cause of
shock in pediatric trauma patients, early and ade-
quate uid resuscitation is critical (Schafermeyer
1993). Hypovolemia can result in the triad of
death, consisting of acidosis, hypothermia, and
coagulopathy (Wetzel and Burns 2002). After the
airway and breathing have been stabilized, circula-
tion is assessed, and appropriate uid replacement is
begun, utilizing crystalloid solution administered
intravenously. If intravenous access is difcult, the
intraosseous administration route may be used. Guy
et al. (1993) reported no complications in 15 children
between the ages of 3 months and 10 years who
underwent tibial intraosseous infusion of colloid
solution, crystalloid solution, and blood. A rabbit
tibia model has also shown that intraosseous infu-
sion is well tolerated (Bielski et al. 1993).
The goal of uid resuscitation is to maintain
the childs blood pressure at a level adequate for
organ perfusion, while avoiding overhydration
that can lead to increased intracranial pressure,
internal uid shifts, or pulmonary edema. A uri-
nary catheter is essential to monitor urine output
and gauge organ perfusion, and a central venous
catheter may also be necessary.
Secondary Assessment
The secondary assessment begins with a full his-
tory and physical exam. Because most polytrauma
in children is the result of blunt trauma, a careful
abdominal exam is essential, as injury to the liver,
spleen, pancreas, and kidneys is common.
Abdominal ecchymosis is a marker of visceral or
spinal injury (Sivit et al. 1991; Campbell
et al. 2003). The entire spine should be examined,
beginning with the removal of the cervical collar
for palpation of the cervical spine for tenderness,
crepitus, or step-off. It is necessary for a member
of the team to stabilize the head while the collar is
off. After replacement of the collar, the patient
should be logrolled for assessment of the remain-
der of the spine and the skin and soft tissue of the
back. A rectal exam to assess the sphincter tone is
performed.
S. Scherl
The extremity exam is also part of the second-
ary assessment, and all limbs should be evaluated
for swelling, deformity, crepitus, and neurovascular
status. Splints applied in the eld should be
removed so that a thorough exam may be made.
The skin of an injured limb should be examined
carefully for the possibility of an open fracture. If
there are open wounds, they should be cleaned
and dressed, and tetanus prophylaxis and antibi-
otics initiated. Splints can be reapplied when the
exam is complete.
The pelvis should be carefully assessed, keep-
ing in mind that a pelvic fracture combined with
one or more other skeletal injuries can be associ-
ated with head and abdominal injuries (Vazquez
and Garcia 1993). If instability is noted with
compression of the iliac wings, a pelvic binder
can be applied. Ideally, the neurologic exam
should be performed prior to intubation or admin-
istration of any medications, but this is not always
possible. Any neurologic decit should be noted,
and if the patient is unconscious, uncooperative,
or unable to understand or comply with the exam-
ination, that should be noted as well.
It is important to remember that some injuries
may be missed on the primary and secondary
surveys, particularly in patients with a head injury.
A tertiary survey, in which a full history and
physical exam is performed again, 2448 h after
the initial assessment, is necessary to aid in the
diagnosis of these late-presenting injuries. In one
series of 149 pediatric polytrauma patients,
13 injuries were diagnosed an average of
15 days following injury, including ve fractures
(one involving the spine), four abdominal injuries,
two aneurysms, one head injury, and one facial
fracture (Letts et al. 2002). It is helpful to alert
families of the possibility of such delayed
diagnoses, so that they are not surprised in the
event of one and so that they can inform the
medical team of any emerging symptoms.
Trauma Rating Systems
Trauma rating systems perform two functions: to
assist in the triage of injured patients and as prog-
nostic indicators. Usually, the trauma rating is
37 The Multiply Injured Child
performed between the primary and secondary
surveys. Many different trauma rating systems
have been developed and are in use, each with
its own strengths and weaknesses. There are a
variety that have been validated for the pediatric
population (Tepas et al. 1988; Champion et al.
1989; Eichelberger et al. 1989; Aprahamian
et al. 1990; Pollack et al. 1996; Potoka et al.
2001; Schall et al. 2002; Slater et al. 2003;
Schluter et al. 2010; Borgman et al. 2011), but
the most commonly utilized are the Glasgow
Coma Scale (GCS), the Injury Severity Score
(ISS), and the Pediatric Trauma Score (PTS).
The Glasgow Coma Scale (GCS) is commonly
used for the assessment of traumatic brain injury.
It evaluates eye opening (14 points), motor func-
tion (16 points), and verbal function (15 points)
on a total scale of 315 points (Teasdale and
Jennett 1974). In verbal children, the GCS is a
useful guide for predicting early mortality and
later disability. A GCS score of less than 8 points
indicates a signicantly worse chance of survival
for these children than for those with a GCS of
more than 8. The GCS score can evolve with the
underlying neurologic injury, so it should be noted
on admission and again 1 h after the child arrives
at the hospital. Moreover, the evolving GCS can
have prognostic signicance: the 72-h GCS motor
response score has been noted to be very predic-
tive of later permanent disability as a sequel to the
head injury (Young et al. 1981; Michaud et al.
1992; Hannan et al. 2000).
Both the ISS and the PTS are valid and repro-
ducible and can be used in varied pediatric set-
tings. The choice of one or the other varies by
trauma center, but each allows an objective means
to assess mortality risk at the time of initial treat-
ment, as well as allowing some degree of predic-
tion of future disability (Ott et al. 2000; Yian
et al. 2000; Sullivan et al. 2003). The ISS is
based on the Abbreviated Injury Scale (AIS),
which assigns a grade of moderate, severe,
serious, critical, and fatal for each of the ve
major body systems. To determine the ISS, one
computes the sum of the squares of the highest
AIS grade in each of the three most severely
injured areas. Twenty-ve is the highest score
for any individual area, and 75 is therefore the
867
highest possible ISS score. However, it is an ordi-
nal, not a linear scale (i.e., a score of 40 is not twice
as bad as a score of 20). It has been found to be a
valid predictor of mortality, length of hospital stay,
and cost of care (Brazelton and Gozain 2012).
The PTS assigns each of six components a
score of +2 (minimal or no injury), +1 (minor or
potentially major injury), or 1 (major or poten-
tially life-threatening injury). The potential score
thus ranges from 6 to +12. The PTS has good
predictive value for injury severity, mortality, and
the need for transport to a pediatric trauma center;
however, it is a poor predictor of internal injury in
children with abdominal blunt trauma (Saladino
et al. 1991).
Imaging
Radiographs
Primary screening radiographs classically consist
of a cross-table lateral cervical spine, antero-
posterior chest, and anteroposterior pelvis (Rees
et al. 2001; Dormans 2002). Cervical spine clear-
ance may require multiple studies. Though the
lateral cervical radiograph will detect an injury,
if present, in 80 % of cases (Lee and Fleisher
2012), some centers perform a CT scan of the
cervical spine instead, particularly in cases of chil-
dren with neck pain, with traumatic brain injury
(TBI), or who have been drinking alcohol (Sanchez
et al. 2005). Magnetic resonance imaging (MRI) is
useful for cervical spine clearance in those who
have persistent neck pain or tenderness despite nor-
mal plain lms and CT and should be considered in
patients who are obtunded (Frank et al. 2002).
Computed Tomography
CT scans are commonly used in the evaluation of
the multiply injured child. CT of the head can
diagnose skull fractures and intracranial hemor-
rhage, and the study can easily be extended
distally to evaluate the cervical spine as well.
Potential intra-abdominal injury in pediatric
patients is also commonly diagnosed by CT scan.
868
CT scans and serial hematocrit levels are also
followed to determine whether or not visceral inju-
ries, such as hepatic and splenic lacerations, need to
be treated operatively (Hoffmann et al. 1992;
Roche et al. 1992; Campbell et al. 2003).
A CTscan of the abdomen can also be extended to
include the pelvis. There is evidence that CT is more
sensitive than plain radiography in screening for
pelvic fractures. In one study, a screening pelvic
radiograph demonstrated only 54 % of pelvic frac-
tures identied on CT scan (Guillamondegui et al.
2003). Pelvic CT scan also has the added benets of
providing more detail about fracture pattern than
plain X-rays, thus aiding in preoperative planning.
Intravenous Pyelography (IVP)
Anterior pelvic fractures are often associated with
urologic injury. Although CT, MRI, and U/S can
be useful in evaluating the kidneys, IVP still has a
role in imaging the bladder and urethra (Onuora
et al. 1993). Urethral injuries in particular are
difcult to fully delineate preoperatively, regard-
less of imaging modality (Andrich et al. 2003).
Magnetic Resonance Imaging
MRI is most commonly used to evaluate traumatic
brain and spinal cord injuries. Because the spinal
cord is less elastic than the growing spine, chil-
dren can sustain a spinal cord injury without an
evident spine fracture (Aufdermaur 1974; Evans
and Bethem 1989; Bosch et al. 2002). This is
referred to as spinal cord injury without radio-
graphic abnormality (SCIWORA) syndrome.
MRI is particularly valuable in demonstrating
the site and extent of spinal cord injury in
SCIWORA and in dening the level of injury to
the disks or vertebral apophyses, which may not
be obvious on conventional radiographs.
Ultrasound
Serial ultrasound evaluations can be used to mon-
itor the liver, spleen, pancreas, and kidney injury
S. Scherl
in children with multiple injuries (Buess et al.
1992; Hoffmann et al. 1992; Roche et al. 1992),
as U/S is an accurate means of detecting
hemopericardium and intraperitoneal uid fol-
lowing injury. The protocol most typically used
is called FAST (focused assessment with sonog-
raphy for trauma). A FAST scan rapidly evaluates
four areas: the right upper abdominal quadrant,
the left upper abdominal quadrant, the subxiphoid
area, and the pelvis. Assessment of the role of
FAST in the management of pediatric trauma
patients is ongoing (Coley et al. 2000; Holmes
et al. 2001; Stafford et al. 2002; Eppich and
Zonfrillo 2007; Holmes et al. 2007); therefore,
CT is still more commonly used for assessment
and monitoring of visceral injury in children sus-
taining multiple injuries. Comparisons of CT and
ultrasonography have demonstrated the superiority
of CT for diagnosing visceral injury in children
with polytrauma (Richardson et al. 1997;
Mutabagani et al. 1999; Coley et al. 2000; Suthers
et al. 2004), but there is evidence that hemodynam-
ically unstable children with a positive FAST
should be taken for laparotomy rather than for CT
scanning (Levy and Bachur 2008).
Non-orthopedic Injuries
Head Injury
Traumatic brain injury (TBI) is extremely com-
mon in the multiply injured child (Letts
et al. 2002; Schalamon et al. 2003). Though sever-
ity of the head injury is the principle determinant
of the overall morbidity and mortality in the set-
ting of pediatric polytrauma (Jakob et al. 2010),
children often make a signicant recovery from
even severe head trauma. The two factors that
have been linked to poorer long-term outcomes
in cases of TBI are low oxygen saturation levels at
presentation and persistently decreased GCS
score at 72 h. However, most children who sustain
a signicant TBI do exhibit some residual cogni-
tive impairment and behavior problems (Green-
span and MacKenzie 1994; Swift et al. 2003;
Hanten et al. 2004). Because substantial neuro-
logic recovery is expected, the management of
37 The Multiply Injured Child
musculoskeletal injuries in these children should
be based on the assumption that restoration of
cognitive and motor function will ensue.
It is typically not necessary to wait until a coma-
tose child is awake to undertake fracture xation, as
waiting may lead to fracture malunion, since frac-
tures tend to heal very rapidly in patients with TBI
(Zhao, Zhao et al. 2007). Moreover, motion at
fracture sites can lead to increased intracranial
pressure, so denitive fracture treatment can help
control intracranial pressure. If denitive stabiliza-
tion is not possible, interim stabilization with
splints, traction, or external xation may be used.
Head trauma can also lead to several other
musculoskeletal system sequelae, including
increased spasticity, contracture, and heterotopic
bone formation. Spasticity tends to occur quite
early after TBI. In the clinical scenario of TBI
and concomitant fracture, the pull of spastic mus-
cles can lead to fracture shortening and angula-
tion. Operative xation may be necessary for
acceptable reduction in these cases (Tolo 1983,
2000). Persistent spasticity leads to joint contrac-
ture. Contracture can develop very quickly, so
it is imperative to institute early preventative
stretching and splinting. Surgical release is some-
times necessary for persistent contractures.
Heterotopic bone formation is common in the
extremities of patients with TBI and persistent
coma, particularly around the hip and elbow
(Mital et al. 1987; Kluger et al. 2000). Surgical
incisions may exacerbate the problem (Keret
et al. 1990). Heterotopic bone that does not inter-
fere with range of motion or function typically is
observed, whereas symptomatic heterotopic ossi-
cation may be excised. The timing of excision is
controversial, as is the use of nonsteroidal medi-
cations or salicylates for initial prophylaxis. After
surgical excision, Mital et al. (1987) reported suc-
cess in preventing recurrence of heterotopic bone
formation by the use of salicylates at a dosage of
40 mg/kg/day in divided doses for 6 weeks post-
operatively. Though low-dose radiation therapy
has also been successfully used for prevention of
post-excision recurrence, there are now two case
reports in the literature of postradiation sarcoma
from doses as low as 700 cGy (Farris et al. 2012;
Mourad et al. 2012).
869
Abdominal Injury
Abdominal injuries are relatively common in
pediatric polytrauma patients, with an incidence
of 827 % (Dereeper et al. 1998; Letts
et al. 2002). Physical exam ndings indicative
of such injuries are abdominal distension, bruis-
ing, and tenderness. Initial evaluation is
usually performed by CT scan and less com-
monly by peritoneal lavage, ultrasound, or lapa-
roscopy. The classic lap belt triad, consisting
of abdominal bruising in association with the
spinal cord and abdominal viscera injury, is
rarely seen, and therefore, all children who
were injured while wearing a lap belt should
be examined carefully for abdominal injury
even in the absence of visible ecchymosis
(Tso et al. 1993; Campbell et al. 2003).
The presence of pelvic fractures also cor-
relates strongly with both intra-abdominal and
genitourinary injuries (Bond et al. 1991).
Hepatic and splenic injuries are the most common,
but about 22 % of cases of pediatric pancreatitis
are traumatic (Benia and Weizman 2003).
In children, most visceral lacerations are
treated nonoperatively. The hematocrit and
abdominal exams are followed closely, and serial
CT or ultrasound exams are often utilized
(Canarelli et al. 1991; Coburn et al. 1995; Uranus
and Pfeifer 2001; Cloutier et al. 2004; Cochran
et al. 2004; Leinwand et al. 2004; Tataria
et al. 2007). It is usually possible to perform
denitive operative xation of fractures in a
child who is under observation for an abdominal
viscera injury.
Genitourinary Injury
Though rare in isolation, genitourinary injuries
occur in 924 % of children with pelvic fra-
ctures (Torode and Zieg 1985; Silber et al. 2001;
Letts et al. 2002). In boys, anterior pelvic
ring injuries are typically associated with
bulbourethral injuries, though other areas of the
bladder and urethra may also be involved
(Onuora et al. 1993; Batislam et al. 1997).
Despite such injuries being less common
870
in girls, they are typically quite serious when
they do occur, with damage to the vagina
and rectum. Sequelae of these injuries include
strictures, incontinence, and difculty with
childbirth (Podesta and Jordan 2001; Rourke
et al. 2003). There is an increased rate of
cesarean section in young women who have
had a pelvic fracture (Copeland et al. 1997),
and girls with displaced pelvic fractures, partic-
ularly if there has been a permanent change
to the shape of the pelvic ring, should be
informed that vaginal delivery may be difcult
or impossible.
Fat and Pulmonary Embolism
Though hypoxemia can occur in the multiply
injured child, the full clinical presentation of
fat embolism and acute respiratory distress syn-
drome is rare (Limbird and Ruderman 1978;
Robinson 2001). When a child does present
with the symptoms of fat embolism (change in
mental status, hypoxemia, axillary petechiae,
and the presence of lung inltrates on chest
radiograph), the treatment is similar to that
for adults: endotracheal intubation, positive pres-
sure ventilation, and hydration with intravenous
uid. Other interventions that have been used in
adults, such as early fracture stabilization,
intravenous alcohol, and high-dose corticoste-
roids, have not been studied in children.
Deep venous thrombosis and pulmonary
thromboembolism in children is rare but has
been reported (Levy et al. 2004; Azu et al. 2005;
Babyn et al. 2005; Truitt et al. 2005; Cyr
et al. 2006). Risk factors for deep venous throm-
bosis and pulmonary thromboembolism in the
multiply injured child include age greater than
9, ISS greater than or equal to 25, and/or
GCS lower than or equal to 8 and the presence
of an indwelling central venous catheter
(Champion et al. 1989; Roche et al. 1992). The
role of prophylaxis for deep venous thrombosis
and pulmonary thromboembolism in pediatric
patients is unclear (Rohrer et al. 1996; Truitt
et al. 2005; Sandoval et al. 2008; Brandao
et al. 2011).
S. Scherl
Orthopedic Injuries
Timing of Fixation
Though fractures are common in pediatric trauma
patients, they are rarely life threatening. Initial man-
agement of fractures thus often consists simply of
splinting until the child is deemed systemically stable
enough for operative fracture xation if indicated.
Damage control orthopedics, in which temporary
external xation is utilized in the period between
presentation and denitive fracture xation, has
been well studied and accepted in the adult literature
(Scalea et al. 2000; Pape et al. 2002; Taeger
et al. 2005; Tuttle et al. 2009). The concept behind
damage control orthopedics is that early denitive
surgery is a signicant stressor that acts as a physio-
logical second hit to an already critically ill patient.
Delaying the second hit allows for some interim
recovery and decreases the incidence of acute respi-
ratory distress syndrome and multisystem organ
failure. Damage control orthopedics has not been
studied in children. There is one case series of three
patients with femur fractures, initially treated with
an external xator and subsequently revised to
submuscular plating (Mooney 2012).
Timing of denitive surgery has also not been
studied extensively. Loder (1987) reported shorter
hospital and intensive care unit stays and a shorter
time on ventilator assistance, in 78 children with
multiple injuries who underwent operative stabi-
lization of fractures within the rst 2 or 3 days
after injury. The cohort of patients treated with
immediate fracture stabilization also had fewer
complications than those who had surgical treat-
ment more than 72 h after injury. Another more
recent study (Loder et al. 2001) reported a trend
toward a higher rate of complications of immobi-
lization (including pulmonary complications) in
fractures treated late (after 72 h), but the differ-
ence did not reach statistical signicance.
Pelvic Fractures
Pelvic fractures have been reported in up to 7 % of
children referred to level 1 regional trauma centers
37 The Multiply Injured Child
(Smith et al. 2004; Vitale et al. 2005). Most of
these fractures are stable, though unstable patterns
have been reported in up to 30 % of cases (Blasier
et al. 2000). It is important to remember that
pelvic fractures are often associated with other
injuries. In one study of 166 children with pelvic
fractures, there was substantial head trauma in
39 %, chest trauma in 20 %, visceral or abdominal
injuries in 19 %, concomitant acetabular fractures
in 12 %, and a mortality rate of 3.6 % (Silber
et al. 2001). In another series, 62 % of children
(8/13) with pelvic fractures had other orthopedic
injuries (Spiguel et al. 2006). Anterior pelvic
ring fractures frequently cause urethral injuries
(Abou-Jaoude et al. 1996; Batislam et al. 1997;
Podesta and Jordan 2001; Rourke et al. 2003).
Mortality in children with pelvic fractures appears
to be caused more often by an associated head
injury rather than from abdominal or retroperito-
neal bleeding from the pelvic fracture or adjacent
viscera, though such hemorrhages can be sig-
nicant (Musemeche et al. 1987; Demetriades
et al. 2003). Non-orthopedic injuries associated
with pelvic fractures led to long-term morbidity
or mortality in 31 % (11/36) of patients in one
review of pediatric pelvic fractures (Garvin
et al. 1990).
In cases in which it is necessary to acutely
stabilize a fractured pelvis either to control bleed-
ing or facilitate care or transport of the child, a
pelvic binder or an external xator may be used
(Reff 1984; Taeger et al. 2005; Jakob et al. 2010).
The C-clamp is not typically utilized for the pedi-
atric population, though it has been reported to be
safe (Holt and Mencio 2003). In cases in which
operative treatment is necessary, rapid healing
with a low complication rate is expected
(Karunakar et al. 2005).
Open Fractures
In the setting of polytrauma, approximately 10 %
of fractures are open (Buckley et al. 1994;
Schalamon et al. 2003). Twenty-ve to fty
percent of patients with open fractures have asso-
ciated injuries of the head, chest, abdomen, or
other extremities (Schalamon et al. 2003). Open
871
fractures are classied by the system of Gustilo
(Gustilo and Anderson 1976; Gustilo et al. 1984),
which takes into account the size of the wound,
the degree of soft tissue damage and wound
contamination, and the presence or absence of an
associated vascular injury. A type I wound is less
than 1 cm in diameter and has minimal associated
soft tissue damage. A type II wound is greater than
1 cm in diameter and has some associated soft
tissue damage, but typically will not require skin
grafting or aps. Type III injuries are large
wounds with extensive soft tissue damage and
contamination and sometimes associated vascular
injury as well.
Initial emergency management of open
fractures includes application of a dressing, pro-
visional reduction and splinting, and administra-
tion of tetanus toxoid and antibiotics. The dose
of tetanus toxoid is 0.5 mL intramuscularly to be
given if the patients immunization status is
unknown or if it is more than 5 years since the
last dose. A typical antibiotic regimen is a rst-
generation cephalosporin (cefazolin 100 mg/kg/
day divided q 8 h, maximal daily dose 6 g) for all
type I and some type II injuries, the addition of
an aminoglycoside (gentamicin 57.5 mg/kg/day
divided q 8 h) for more extensive type II and all
type III injuries, and the addition of penicillin
(150,000 units/kg/day divided q 6 h, maximum
daily dose of 24 million units) for farm injuries,
gross contamination, and associated vascular
injuries (Lavelle et al. 2008). Antibiotics are
usually given for 48 h initially, with an additional
course for every surgery. Keep in mind that
evidence-based medicine does support the use
of a short course of a rst-generation cephalo-
sporin, but the data are inadequate to support the
other treatment of the limbs outlined above
(Hauser et al. 2006). Moreover, the guidelines
above were developed prior to the recent increase
in prevalence of community-acquired methicil-
lin-resistant Staphylococcus aureus (MRSA).
Clindamycin or vancomycin should be added to
the regimen if contamination with MRSA is
suspected.
Thorough irrigation and debridement are criti-
cal in the treatment of open fractures, but the
timing of surgery is controversial. One study
872
reported an overall infection rate of 12 % after
open long-bone fractures, with no difference in
infection rates between groups of patients treated
with irrigation and debridement within 6 h of
injury and those treated between 6 and 24 h
following injury (Skaggs et al. 2000). A large,
multicenter trial reported an infection rate of
3 %, with no difference between children treated
within 6 h and those after 6 h (Skaggs et al. 2005).
Though the study includes over 500 fractures,
there is no power analysis, so it is possible that a
difference exists but was not detected. An earlier
study of open tibia fractures reported signicantly
higher infection rates if debridement and irrigation
were performed more than 6 h after open fractures
in children (Kreder and Armstrong 1995).
Debridement includes excision of necrotic skin
and soft tissue, extension of the wound to expose
the fracture ends, cleaning of the bone, and irriga-
tion of all tissues. Although high-pressure lavage
systems have been used for irrigation, there have
been reports of complications, including acute
compartment syndrome, with their use (Lauber
et al. 2005; Silva and Bosch 2009). Recent
studies, including the multicenter, randomized,
blinded uid lavage of open wound (FLOW)
study, have found that low-pressure lavage is
safer and more effective than high-pressure lavage
(Owens et al. 2009; Investigators et al. 2011).
Lavage solutions were also studied. Normal
saline, without the addition of antibiotics or deter-
gent, was found to be safest and most effective.
Volume of irrigation is at the discretion of the
surgeon, but reasonable guidelines for children
are 39 L of normal saline solution for the lower
extremities and 26 L for the upper extremities.
Serial irrigation and debridements are
performed every 23 days until the soft tissue is
clean and viable. Small wounds can be closed by
delayed primary closure or by secondary inten-
tion. Larger wounds may require skin grafting or a
muscle ap. The use of a vacuum-assisted closure
device can aid in healing and decrease the need for
skin grafts and aps (Mooney et al. 2000; Caniano
et al. 2005).
The method of stabilization of open fractures
depends on the location of the fracture, fracture
pattern, the position and severity of the soft tissue
S. Scherl
injury, the age and size of the child, and the
presence of associated injuries. External xation,
elastic nails, k-wires and screws, and rigid internal
xation all may be useful, depending on the
circumstance. In any case, the goal is stable xation,
allowing for adequate care of the soft tissue injury.
Conclusion
Trauma is the leading cause of death and disability
in children. Fractures are rarely the cause of mor-
tality in multiply injured children but contribute
signicantly to morbidity. Regardless of the
mechanism causing the multiple injuries, the
initial medical management focuses on the
life-threatening, non-orthopedic injuries to stabi-
lize the childs condition (Maksoud et al. 1995).
Rapid assessment and treatment during the
golden hour decreases mortality. The American
College of Surgeons has established specic
criteria for pediatric trauma centers, which include
the same principles of rapid transport and rapid
treatment by an in-house surgical team as in adult
trauma centers. There is increasing evidence that
pediatric trauma centers do provide improved out-
comes for severely injured children, but there are
relatively few such centers, and many children
will be stabilized or treated denitively at adult
trauma centers.
Initial resuscitation follows the Advanced
Trauma Life Support (ATLS) or Pediatric
Advanced Life Support (PALS) protocols. The
primary survey is comprised of the ABCs
airway, breathing, circulation, disability (neuro-
logic), and exposure and screening radiographs
(cervical spine, chest, and pelvis). Hypovolemia is
the most common cause of shock in pediatric
trauma patients, so early and adequate uid resus-
citation is critical.
Trauma rating systems have two functions: to
aid in triage and to predict outcomes. There are
many rating systems, each with strengths and
weaknesses. Of the commonly used systems,
both the Injury Severity Score (ISS) and Glasgow
Coma Score (GCS) have predictive value for
prognosis. The secondary survey is a systematic
examination of the patient from head to toe.
37 The Multiply Injured Child
It includes a complete history, physical examina-
tion, focused radiographs, and adjunctive imaging
studies such as CT and MRI scans.
Head injury severity is the principle determi-
nant of morbidity and mortality in a multiply
injured child. Children often make substantial
recovery from even severe head trauma. Manage-
ment of orthopedic injuries in children with head
trauma should be based on the presumption of
full recovery from the head injury. Spasticity and
contracture are common sequelae of brain injury
and should be addressed early.
Although blunt abdominal trauma is often
treated nonoperatively in children, it is necessary
to diagnose it. There is an association between
pediatric pelvic fractures and both intra-
abdominal and genitourinary injuries. Most frac-
tures in multiply injured children can be splinted
initially and undergo denitive treatment urgently,
not emergently. Pelvic fractures in children can
typically be treated nonoperatively but may
require xation if the child is hemodynamically
unstable. Tetanus toxoid and antibiotics should be
provided for all open fractures, though routine
culture is unnecessary. The timely administration
of intravenous antibiotics and the appropriate irri-
gation and debridement are the most important
steps in the treatment of open fractures. There
are many options for stabilization of open frac-
tures. In each case, stabilization should be planned
to allow easy access for further treatment of the
soft tissue injury.
Although about 22 % of children who sustain
polytrauma have some residual disability, optimal
treatment of their orthopedic injuries in a timely
fashion decreases their burden of musculoskeletal
disability. The best predictor of long-term disability
is the Glasgow Outcome Scale from 6 weeks after
injury and later (van der Sluis et al. 1997).
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 Non-accidental Trauma
38
Richard M. Schwend

Contents Forearm Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 892

Hand Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 892
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 880
Historical . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 880 Differential Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 892
Terminology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 880
Reporting and Documentation . . . . . . . . . . . . . . . . . . . . 893
Epidemiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 880
Role of Suspected Child Abuse and Neglect
Risk Factors for Abuse . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 881 (SCAN) Team . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 893
Home and Family . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 881 Surgeons Role . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 894
Child . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 881
Prevention . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 895
The Medical Setting . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 881
Resources . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 895
History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 882
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 895
Physical Examination . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 883
Abusive Head Trauma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 883
Soft Tissue Injuries . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 884
Burns . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 884
Abdominal Trauma/Genital . . . . . . . . . . . . . . . . . . . . . . . . . . 885
Death . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 885
Skeletal Survey Screening . . . . . . . . . . . . . . . . . . . . . . . . . 885
Follow-Up Survey and Other Studies . . . . . . . . . . . . . . . 886
Variations in Skeletal Survey Screening . . . . . . . . . . . . . 887
Usefulness of the Skeletal Survey . . . . . . . . . . . . . . . . . . . 888
Summary of Recommendations for
Skeletal Survey . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 888
Dating Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 888
Fractures in Abuse . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 888
Rib Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 890
Classic Metaphyseal Lesion . . . . . . . . . . . . . . . . . . . . . . . . . 890
Other Related Injuries . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 890
Upper Extremity Injuries in Abuse . . . . . . . . . . . . . . . 890
Clavicle Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 891
Humerus Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 891

R.M. Schwend
Division of Orthopaedics, Childrens Mercy Hospital,
Kansas City, MO, USA
e-mail: rmschwend@cmh.edu

# Springer Science+Business Media New York 2015 879

J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_39
880 R.M. Schwend

inadequately handled by the physician because of

Abstract
hesitation to bring the case to the attention of the
Maltreatment of children may involve physi-
proper authorities continues to be very relevant
cal, emotional, or sexual abuse or neglect. It is
over 50 years later.
too common with an incidence of 9.1/1,000
children. Although there are family- and
child-related factors associated with abuse
Terminology
and any age child can be abused, it is the
younger infant and child who are most com-
Older terms for child maltreatment include
monly at risk. Early infancy, when crying
unrecognized trauma, battered child syn-
peaks, is a time of increased risk of abuse and
drome, physical abuse, or child abuse. Newer
especially abusive head trauma. The diagnosis
terminology includes child maltreatment,
of non-accidental trauma is based on a detailed
non-accidental injury (NAI), or non-accidental
history of the injury with documentation of the
trauma (NAT). Non-accidental trauma refers to
precise mechanism as reported by the care-
the observed physical injuries inicted from
giver. The injuries must have an explanation
child maltreatment. General pediatricians, general
with a plausible mechanism for how they
practitioners as well as specialists who care for
occurred. All states have reporting laws for
children must be mindful of the possibility of
suspected abuse and provide a degree of immu-
child maltreatment when evaluating and treating
nity for reports made in good faith. The mini-
children with a wide variety of conditions (Preer
mal standard of evaluation when abuse is
et al. 2013). Child abuse pediatrics involves the
suspected includes a properly done skeletal
care of children who are maltreated either from
survey for the 024-month-old child, dilated
physical, emotional, or sexual abuse or from
fundoscopic examination by an ophthalmolo-
neglect (Jenny 2007; Preer et al. 2013). Although
gist, and consultation by the child protective
the quality of the child maltreatment literature
services team. The surgeon may be asked to
continues to improve due to adherence to
testify as either a material witness or as an
evidence-based medicine principles and multicen-
expert witness and so should be well prepared
ter prospective networks, the absence of a gold
for either role.
standard test for abuse and the heterogeneity of
data confound interpreting the literature (Greeley
2012).
Introduction

Historical Epidemiology

In 1962 Kempe et al. rst described the battered
child syndrome as a triad of subdural hemor-
rhage, skeletal injuries, and bruises, generally
affecting children younger than age 3 years and
frequently leading to disability or death (Kempe
et al. 1962). Clinical manifestations include a
wide variety and constellation of signs of trauma
ranging from mild to severe and from neglect to
death. There is often a history of previous epi-
sodes and a clear discrepancy between what the
examination nds compared to the historical data
provided by the caregiver. Kempes statement that
it is frequently not recognized or, if diagnosed, is
Child maltreatment remains unacceptably com-
mon. The Childrens Bureau of the US Depart-
ment of Health and Human Services (HHS)
analyzes data from the National Child Abuse and
Neglect Data System, which is submitted by all
states in the USA, District of Columbia, and
Puerto Rico. In their report Child Maltreatment
2011 (http://www.acf.hhs.gov/programs/cb/
resource/child-maltreatment-2011), there were
676,569 reported cases of child abuse and neglect
(9.1/1,000 children). Child neglect was the most
common form of maltreatment (78.5 %), followed
by physical abuse (17.6 %) and sexual abuse
38 Non-accidental Trauma
(9.1 %). In 2011 there were 1,545 deaths
(2.1/100,000), 81.6 % occurring in children less
than 4 years of age. Birth to 1 year of age has the
highest rate of abuse (21.2/1,000).
Although there has been a recent decrease in
reported cases of child abuse, many experts in the
eld of child maltreatment consider this data to be
misleading (Preer et al. 2013). US Child Protec-
tive Services data on the national incidence of
substantiated physical abuse showed a marked
55 % decrease in incidence from 1992 to 2009.
The Childrens Bureau also reported a continued
drop in child abuse cases in 2011 compared to
2010; however, this may be related to wide vari-
ability in the quality of the data. During this period
of decline in substantiated child abuse cases
reported by the US Child Protective Services,
the Kids Inpatient Database, which is a sample
of discharges from US hospitals, noted an increase
from 1997 to 2009, due mainly to an increase in
serious cases of abuse, especially in infants
(Leventhal and Giather 2012). This shows the
importance of not using single-source data when
making policy and program decisions.
Risk Factors for Abuse
Home and Family
Households that are in turmoil have a variety of
stressors that put the child at risk. Leo Tolstoy
wrote in the beginning of Anna Karenina
Happy families are all alike; every unhappy fam-
ily is unhappy in its own way. If any of the many
necessary ingredients is missing, a family can
have toxic stress that affects its ability to cope.
Poverty is associated with chronic and toxic
stress to families and is a major determinant
of health disparities. Approximately 16 million
(22 %) children live below the federal poverty
level while over 32 million (45 %) of US
children live in low-income families. Children
are disproportionately affected by poverty, with
infants and toddlers even more likely to be
poor. Making the situation worse, this is at an
age that they are also most vulnerable to
child abuse.
881
Children normally have increased crying
within the rst 3 months of life. When families
are stressed and have marginal coping skills, this
normal crying behavior of a young infant can be
the tipping point resulting in abuse (Barr
et al. 2006). However, any person from any social
or economic background may abuse a child.
According to the Childrens Bureau 2011 report
(http://www.acf.hhs.gov/programs/cb/resource/
child-maltreatment-2011), 85 % of perpetrators
of abuse were age 2046 years, with 53.6 % being
women. Over 80 % of perpetrators were parents,
of which over 87 % were the biological parents.
However, the presence of a nonbiologically
related adult increased the risk of death by abuse
50 times higher than even a single-parent family
(Schnitzer 2005). Other risk factors for abuse
include a history of the parent being abused them-
selves as a child, home daycare, history of drug or
alcohol abuse, maternal depression, maternal or
paternal age under 20 years, and history of psy-
chiatric illness in the family.
Child
The younger the child, the more the risk for abuse.
Child abuse is higher in children who are victims
of recurrent non-accidental trauma, so it is espe-
cially important to diagnose the rst episode and
intervene appropriately (Deans et al. 2013). First-
born children, premature infants, children from
multiple births, stepchildren, and children with a
disability are more likely to be abused (Beals and
Tufts 1983). Marginally functionally disabled
children seem to be at greater risk for abuse than
severely affected children, likely because of
greater parental frustration (Benedict
et al. 1990). As children become more mobile
and are able to walk and therefore fall, the acci-
dental trauma becomes more likely as a mecha-
nism (Schwend et al. 2000).
The Medical Setting
A form of child maltreatment can occur when a
caregiver either falsies or causes a childs illness
882
that leads to unnecessary, harmful, or potentially
harmful medical diagnostic investigations or
treatment (Flaherty et al. 2013). This has recently
been termed caregiver-fabricated illness in a
child which replaces older terms of
Munchausen syndrome by proxy and child skull fractures, but rarely signicant head trauma
abuse in the medical setting. Although this con-
dition is relatively rare (approximately
1/100,000), it is estimated that professionals car-
ing for children will see at least one case during
their career. Several key features include: bizarre
signs or symptoms, a diagnosis that does not
match the objective ndings, the caregiver that
seems disappointed when informed that the child
does not have a particular condition or is improv-
ing, inconsistent reported histories, and the care-
giver that insists on invasive procedures or solicits
sympathy or donations for the child. Symptoms
and signs that involve the upper extremity may
include motor delay, weakness, food allergy and
rash, eczema, burns, lacerations, easy bruising or
bleeding, arthritis, arthralgia, or morning stiff-
ness. The denition and diagnosis of caregiver-
fabricated illness should focus on protection of the
child, rather on the motives of the offending care-
giver. Management should include evaluation of
medical records, communication among medical
professionals caring for the child, and a multidis-
ciplinary approach.
History
An accurate diagnosis of child maltreatment is
essential to protect the child (Preer et al. 2013).
The parents should be meticulously interviewed
about the injury mechanism and the details care-
fully documented. Short-distance falls in infants
are common by the time they are 5 months of age,
as this is the age when they are active and start to
roll off of elevated surfaces. Nearly 50 % of nor-
mal infants have fallen off an elevated surface
after 5 months of age; however, this rarely leads
to serious injury (Kravitz et al. 1969). Simple falls
from elevated surfaces such as a bed or sofa rarely
lead to serious injury and typically involve the
skull or upper extremity. However, when children
are bouncing on furniture and catapulting during
R.M. Schwend
play activity, this can more commonly lead to a
fracture, usually to the upper extremity
(Hennrikus and Shaw 2003). Stairway falls repre-
sent a higher-energy mechanism, especially when
a caregiver falls with the child, and can result in
or death occurs. The biomechanics of short-
distance falls has been investigated with an
anthropomorphic test device (Thompson
et al. 2013). The possibility of head, neck, or
extremity injury is related to the surface of impact,
with linoleum-surfaced concrete having more
potential for injury than wood or playground
foam. In this study, severe head and extremity
injuries were considered low probability to occur
by accident, although concussion and humerus
fractures were possible with short-distance falls.
However, even short-distance falls onto concrete
can lead to a more serious head injury (Thompson
et al. 2013). A previous history of minor injuries is
frequently noted in children that are later found to
be abused. The so-called sentinel injuries in
abused children that include bruising, intraoral
injury, or other injury were found in a total of
27.5 % of abused children and in none of control
children (Sheets et al. 2013). Most (95 %) of
sentinel injuries occurred before 7 months of
age, so medical personnel need to be very aware
of these types of injuries in the young infant.
The history that documents child abuse is
termed the investigative interview and is a team
endeavor, led by members of the child protective
services team and police when potential child
abuse is being investigated. The role of the sur-
geon is to obtain and document a detailed history
of the circumstances of the injury including spe-
cics about how the child was injured; who was
supervising the child; who witnessed the injury;
what the child was doing just before and after the
injury; how the child fell, from what height, and
onto what surface; and what the caregiver did in
response to the injury. The more detailed the his-
tory, the better the surgeon is able to assess the
extent of potential injuries, as well as determine if
the injury mechanism described by the parents ts
the childs injuries and is developmentally plausi-
ble. Open-ended questions are better than leading
questions. Inconsistencies are noted but should
38 Non-accidental Trauma
not be challenged early in the interview. Other
injuries that are noted are discussed after the end
of the interview, once the primary injury has been
discussed. A delay in seeking medical care is very
suggestive for child abuse. This is particularly true
with abusive head trauma (AHT), since the injury
is severe enough to develop immediate symptoms
that required immediate medical care. Since the
onset of symptoms with AHT is so prompt, the
caregiver who reportedly found the child is often
the suspect. Additional information about the
child and family should be obtained by detailed
review of the medical record, from the childs
primary care physician or from social workers
who have been involved with the family.
Physical Examination
A detailed physical examination is necessary to
assess the childs growth parameters for failure to
thrive, vital signs for physiologic disturbance, and
a head-to-toe evaluation to detect associated inju-
ries. Specic areas and injuries seen in child abuse
such as those seen in the skin, central nervous
system, abdomen, and genitalia should receive
particular attention. Bruises or other suspicious
lesions must be documented, best by photographs
by a professional medical photographer. Tender
areas may represent an acute fracture. Swollen
areas may represent either fresh or healing frac-
tures. These areas all need focused radiographic
examination with orthogonal views. 50 % of
abuse cases have prior evidence of abuse, so the
examiner must look not only for new injuries but
evidence of previous injury as well. The skeletal
survey is performed in a child under 2 years of age
when there is suspicion for abuse and should be
considered an extension of the physical
examination.
Abusive Head Trauma
Head trauma is the most common cause of death
in physically abused children. When an infant
presents with altered mental status, head trauma
should be suspected. The American Academy of
883
Pediatrics Committee on Child Abuse and
Neglect recommends the term abusive head
trauma (AHT) over the older terms that have
been used, including shaken baby syndrome
(SBS). Pediatric abusive head trauma is an injury
to the skull or intracranial contents of an infant or
young child, less than 5 years of age, due to
inicted blunt impact and/or violent shaking
(Parks et al. 2012; Christian et al. 2009). Skull
fractures and thin underlying subdural hemor-
rhage (SDH) are common ndings in minor trans-
lational impact forces of accidental head trauma in
the 02-year-old age group (Hudson and Kaplan
2006). However, diffuse brain injury with neuro-
logical impairment implies a severe injury mech-
anism either from a known accidental severe
acceleration, deceleration, and translation mecha-
nism such as from a motor vehicle accident or
from abusive head trauma (Thomas et al. 2013).
A recent systemic review and pooled analyses
have both conrmed the diagnostic accuracy of
subdural hemorrhages, cerebral ischemia, retinal
hemorrhages, skull fractures in the presence of
intracranial injury, rib fractures, seizures, apnea,
and no adequate history to explain the injury for
abusive head trauma (Piteau et al. 2012; Maguire
et al. 2011). Piteau concluded that abusive head
trauma occurred at a younger age, at a time when
the infant is most likely to reach a peak in normal
crying behavior at 3 months of age (Piteau
et al. 2012; Barr et al. 2006). At this age the
young infant has greater susceptibility to head
injury due to more delicate physiologic and ana-
tomic differences. If AHT is not recognized and
the child returns to the home, repeat abuse is
common (Gerber and Coffman 2007). Abusive
head trauma incidence is similar in military
populations compared to civilian populations but
is more likely in families of lower military pay
grades, infants with military mothers, and prema-
ture infants or those with birth defects (Gumbs
et al. 2013).
Interviews with perpetrators of AHT reveal a
surprisingly candid description of the event,
which is precipitated by crying, followed
by vigorous shaking of the infant without
contacting a surface, that results in the immediate
onset of symptoms (Biron and Shelton 2005;
884
Starling et al. 2004). A complete neurological
examination is necessary for an infant or child
suspected to have been abused. Infants with
AHT may have fever, decreased consciousness,
seizures, macrocephaly, bulging fontanelles, pare-
sis, and increased reexes. An ophthalmologist
should be consulted to perform a dilated
fundoscopic examination for retinal hemorrhages.
The young infants eye has a very soft sclera, and
the globe can more easily deform with violent
shaking, causing vitreoretinal traction and direct
hemorrhage in the retina and in the optic nerve
sheath (Wygnanski-Jaffe et al. 2006). Retinal
hemorrhages from abuse are numerous and bilat-
eral, multilayered, and more anterior. Long-term
disability after AHT is very common and can
include developmental delays, recurrent seizures,
visual impairment, attention decits, behavior or
intellectual dysfunction, feeding difculties, and
sensory and/or motor decits. Disability after
AHT can range in severity from mild, with learn-
ing disorders not appearing until after 5 years of
age, to immediate and severe.
Soft Tissue Injuries
Soft tissue injuries are the most common ndings
in child abuse (McMahon et al. 1995). Compared
to an active toddler, soft tissue lesions in a young
infant are unusual. The childs entire body should
be evaluated for soft tissue injuries which may
include swelling, bruising, scars, welts, abrasions,
lacerations, or other soft tissue injuries. Children
with physical abuse are likely to have bruising
with more involved sites, such as the buttocks or
genitalia, face, neck, trunk, or upper arms (Kemp
et al. 2013). Petechiae, linear bruises of distinct
patterns, clusters of bruises, additional injuries, or
a history of previous abuse are also more likely in
abused children. The lesion may resemble the
device that was used, including belt buckles,
ropes, switches, coat hangers, open or closed
hand, ironing cords, feet, or teeth. Any child
may have a number of bruises at various locations;
however, the overall location and appearance of
the bruising as well as the medical and social
history raise the suspicion for abuse. The color
R.M. Schwend
of a bruise is a very rough indicator of its age.
Deciding on the age of a lesion should be done
with much caution, since accuracy of diagnosis is
critical (Schwartz and Ricci 1996). The physician
should examine for all soft tissue lesions and
injuries before treating an acute fracture or injury.
Once a cast is applied, the necessary documenta-
tion and photographs can be impossible to obtain.
The examiner should be aware of other condi-
tions that may mislead the diagnosis of abuse.
Mongolian spots are normal dark blue pigmented
areas on the lower back, proximal to the buttocks,
seen most commonly in black or Asian infants.
Typically, they gradually resolve with growth.
Vietnamese and Mong children may receive cao
gio, a cultural practice of placing marks on the
back. Eczema, impetigo, vascular malformations,
Ehlers-Danlos syndrome, neurobromatosis, and
McCune-Albright syndrome may all have distinc-
tive skin lesions that can be found on the trunk and
extremities. Several underlying medical condi-
tions such as osteogenesis imperfecta can predis-
pose to bruising or bleeding and should be
inquired for before extensive laboratory studies
are ordered (Anderst et al. 2013; Carpenter
et al. 2013). If bruising is the only nding in a
child with possible abuse, consultation with a
hematologist may be needed to accurately evalu-
ate and diagnose a bleeding disorder. Factor XIII
deciency may present with unexplained bleeding
with an initial normal coagulation screen.
Burns
Burns are common in abused children, especially
under the age of 3 years. Scalds from either a spill
or an immersion are the most frequent type of
abusive burn. A burn from an accidental spill is
typically found on the trunk and upper extremities
near the shoulder and has a conguration of
owing water. In an accidental hot water immer-
sion, there can be an indistinct stocking glove
appearance of various depth and variability of
the margins, with involvement of the exion
creases as the child extends and attempts to pro-
vide him/herself support. A deliberate immersion
burn from being forced into hot water has a
38 Non-accidental Trauma
uniform depth and a well-demarcated water line,
with sparing of exion creases as the child
attempts to withdraw. Other types of intentional
burns include those from cigarettes or contact
with heated objects. Burns on the dorsum of the
hand are more suspicious for abuse than those on
the palmar side. Circumferential scars on the wrist
can be from rope burns and abrasions from
restraints.
Abdominal Trauma/Genital
While infants are at high risk for shaking and
abusive head trauma, toddlers get punched and
beaten, resulting in trauma to the less protected
abdomen, chest, and pelvis. Trauma to the abdo-
men is the second most common cause of death
from abuse, after AHT. Abdominal trauma from
abuse can be present with fractures, burns, and
head injury (Maguire et al. 2013). For the child
less than 4 years of age, child abuse is the leading
cause of a duodenal injury. A non-motor vehicle-
related duodenal trauma in a child under 5 years of
age is highly associated with abuse. Abusive
abdominal trauma is more likely to result in mor-
tality (Maguire et al. 2013). Occult abdominal
trauma is easy to miss and requires a high index
of suspicion with frequent serial examinations and
with computed tomography (CT) scanning.
Death
The death of an infant is a tragedy for the family
and society. It must be handled respectfully and
with the medical and legal systems appropriately
coordinated (Jenny and Isaac 2006). A forensic
team approach is needed in cases of death from
unknown causes (Dudley 2012). Sudden infant
death syndrome (SIDS), in which the child dies
during sleep and which remains unexplained after
appropriate investigation, falls under sudden
unexpected death in infancy (SUDI). SUDI may
be caused by spontaneous intracranial bleeds,
metabolic disease, overwhelming sepsis,
unsuspected cardiac conditions, accidental or
non-accidental suffocation, and fatal child abuse.
885
In a large referral trauma center, 50 % of
trauma-related deaths were from non-accidental
trauma (Larimer et al. 2013). These injuries in
NAT patients were more severe, with higher
Injury Severity Scores (ISS) and intensive care
unit (ICU) admissions. The majority of these
NAT children had polytrauma, with closed-head
injuries, extremity fractures, rib fractures, and
abdominal or thoracic trauma. Thus, prompt eval-
uation and treatment by a trauma team are essen-
tial to prevent unnecessary death or disability.
About one in ten infants seen in the emergency
room for a life-threatening event was later found
to be a victim of child abuse (Bonkowsky
et al. 2008). A fatally injured child from a reported
short-height fall at home or similar low-energy
mechanism must always receive expert postmor-
tem investigation for child abuse. Although most
fatal injuries in children are accidental, abusive
fatal injuries are more likely to have associated
subdural hematoma in 82 % and retinal hemor-
rhage in 50 % (Ortega et al. 2013).
Novel technologies have been used to investi-
gate suspected cases of abuse following a death.
Whole-body postmortem CT provides essential
information in detecting the unexpected death of
a child and can help direct autopsy dissection and
sampling (Proisy et al. 2013). The University of
New Mexico has a statewide centralized medical
examiner system and recently has been
performing full-body, high-resolution CT scans
of all deaths with unknown or questionable
causes. Full-body 3D virtual reconstructions
with laser scanning and digital texture photogra-
phy have been used to reconstruct and illustrate
the premorbid condition before death (Davy-Jow
et al. 2013). The appropriate forensic investiga-
tion for fatal child abuse requires a detailed high-
resolution skeletal survey, CT scan, forensic
autopsy, and osteologic investigation for fractures
(Mungan 2007).
Skeletal Survey Screening
Young children who are victims of child abuse
may have occult injuries not detected by the his-
tory or physical examination. Skeletal injuries are
886 R.M. Schwend

Fig. 1 27 Month female

seen acutely for fracture of
her right humerus after a
reported fall to the ground.
Because of a suspicious
injury mechanism and
social situation a skeletal
survey was performed.
There was evidence of
healing scapula fracture
indicating previous non
accidental trauma

rarely life threatening but are frequently a strong
indicator of abuse. If not detected, the child may
return to the abusive home environment and sub-
sequently return to the hospital severely injured or
dead. The younger the child, the more difcult it
may be to detect an occult or healing fracture.
Occult fractures are the most common form of
occult injury and may be detected in up to 1/3 of
children <2 years of age who are victims of phys-
ical abuse (Karmazyn et al. 2011). Since the nd-
ing of an occult injury may be an indicator of
abuse and an opportunity to protect the child
from further harm, the American Academy of
Pediatrics considers it mandatory to obtain a skel-
etal survey for any child under the age of 2 years
who is suspected to be the victim of physical
abuse (Section on Radiology AAP 2009). For
children over age 5 years, the screening skeletal
survey has little value. For children between the
ages of 2 and 5 years of age, decision making
for obtaining a skeletal survey should be based
on specic clinical indicators of abuse (Fig. 1).
The American College of Radiology has recom-
mended a standard skeletal survey imaging
protocol (Table 1) (ACR 2011). They have also
recommended high-detail imaging systems, with-
out an anti-scatter grid, to be used in suspected
abuse in infancy, with faster general purpose sys-
tems for the skull and lateral lumbar spine where
the tissue is thicker. The customary radiographic
protocol (minimum AP and lateral views) should
be used for imaging a specic location that is
clinically suspected of injury. Separate views of
the hands and feet to detect subtle digital injuries
are part of the standard skeletal survey, as
Table 1 Complete skeletal survey (ACR 2011)
Appendicular skeleton
Humeri (AP)
Forearms (AP)
Hands (PA)
Femurs (AP)
Lower legs (AP)
Feet (AP)
Axial Skelton
Thorax (AP, lateral, right and left obliques), to include
ribs, thoracic and upper lumbar spine
Pelvis (AP), to include the mid lumbar spine
Lumbosacral spine (lateral)
Cervical spine (lateral)
Skull (frontal and lateral)
Note: Oblique views of the thorax are recommended to
detect rib fractures. A four-view examination of the skull is
obtained when head injury is present
10.420.4 % of children with a positive skeletal
survey will have fractures of the hands, feet, or
spine (Lindberg et al. 2013; Kleinman et al. 2013).
Follow-Up Survey and Other Studies
A follow-up skeletal survey at 2 weeks increases
the yield, with 21.5 % of subjects having new
information noted on the follow-up skeletal sur-
vey (Fig. 2). Therefore, follow-up skeletal surveys
have become customary in child abuse protocols
(Harper et al. 2013). A 15-view limited follow-up
survey found new information in 38 % of studies,
with less radiation and without missing clinically
38 Non-accidental Trauma 887

Fig. 2 Two month infant

not using left upper
extremity. No specic
injury mechanism could be
elicited from parents. Initial
AP radiograph of the left
humerus did not show a
fracture (gure 1a). A
skeletal survey was
completed and the lateral
image showed a left
humerus midshaft fracture
(gure 1b). A repeat
skeletal survey two weeks
later showed the healing
fracture (gures 2c,d). Eye
exam and head CT were
normal

signicant new fractures (Harlan et al. 2009).
Because of the high morbidity and mortality risk
of missing child abuse, the follow-up skeletal
survey should be routinely performed (Singh
et al. 2012). Obtaining skeletal surveys on siblings
of abused children has also been suggested.
Although radiation exposure is a risk associated
with obtaining a skeletal survey, there are no
studies that specically describe the actual risks
from radiation exposure to children receiving
a skeletal survey (Karmazyn et al. 2011). Radio-
nuclide bone scans, which cost 6 times more
than a skeletal survey, have been used more as a
supplement to the radiographic skeletal survey,
rather than as the primary imaging modality
(Mandelstam et al. 2003). Magnetic resonance
imaging (MRI) or ultrasound examinations may
be used to supplement the skeletal survey in
cases of suspected physeal or soft tissue injury.
CT scans are used to evaluate for abdominal
injury or abusive head trauma. MRI provides
full assessment of the intracranial pathology in
child abuse in order to detect asymptomatic,
nonacute parenchymal brain lesions. Diffusion-
and susceptibility-weighted imaging is very
reliable for diagnosing hypoxic-ischemic brain
injury and parenchymal hemorrhage (Parizel
et al. 2003). MRI can also detect related spinal
cord pathology in the child with AHT, such as
ligamentous injury and intraspinal injuries such as
spinal cord injury without radiographic abnormal-
ity or proximal brachial plexus injuries, especially
at the root level.
Variations in Skeletal Survey Screening
A skeletal survey is useful to screen for occult
fractures in children suspected to be at high risk
for abuse. However, there are marked variations in
the use of the survey across childrens hospitals.
The controversy is in the interpretation of the
AAP guidelines, what the physician determines
to be a suspected case, the physician experience
level, the standard of care for the institution, and
the availability of child abuse specialists and pro-
grams. In a survey of 40 childrens hospitals,
screening was performed for 83 % of children
under the age of 2 years who were diagnosed
with physical abuse (Wood et al. 2012). The like-
lihood of screening varied from 55 % to 93 % and
was higher for those hospitals with a child abuse
program. In particular, the use of a skeletal survey
for children with traumatic brain injury varied
from 38 % to 88 % (mean 68 %) and for infants
with a femur fracture from 41 % to 94 %
888
(mean 77 %). This variation in the interpretation
and use of the skeletal survey has led to a
multidiscipline expert panel RAND/UCLA
appropriateness method development of recom-
mendations for obtaining a skeletal survey in
young children less than 2 years of age, presenting
with fractures (Wood et al. 2014).
Usefulness of the Skeletal Survey
A skeletal survey is very useful for detecting
occult fractures in infants less than 6 months of
age (Duffy and Squires 2011). Children with abu-
sive head trauma had a higher chance of an occult
fracture compared to other types of abuse injuries
(Day et al. 2006; Duffy and Squires 2011).
For children with isolated skull fractures but no
abusive head trauma, the skeletal survey found
additional fractures in only 6 %, typically in
premobile infants under 6 months of age (Laskey
et al. 2013). The older child with a simple linear
skull fracture is less likely to have a positive
skeletal survey. Laskey et al. found that the
majority of long falls had only a simple skull
fracture with a negative skeletal survey (Laskey
et al. 2013).
Summary of Recommendations
for Skeletal Survey
The skeletal survey should be thought of as a
supplement to the history and physical examina-
tion in the infant and young child under the age of
2 years who cannot otherwise give a history or
cooperate in a physical examination. A skeletal
survey is recommended for all infants under
1 year of age with a fracture, unless the fracture
is clearly in a mobile infant who is cruising and
could accidentally sustain a fracture such as a
distal radius and ulna fracture or a toddler tibia
or bula fracture and a simple skull fracture in an
infant older than 6 months of age from a plausible
mechanism or a plausible birth fracture such as the
clavicle. For children 1224 months of age, a
skeletal survey is necessary if there is a history
of abuse or domestic violence, there is a delay in
R.M. Schwend
seeking care for a painful injury, the history is not
consistent or plausible for the fracture, the fracture
was reportedly from a non-plausible mechanism
such as being hit by a toy, or there are suspicious
soft tissue injuries. For any child under the age of
2 years, all rib fractures, classic metaphyseal
lesions, complex skull fractures, humeral frac-
tures with physeal separation, or femoral diaphy-
seal fracture should receive a skeletal survey as
part of their evaluation (Wood et al. 2014).
Dating Fractures
New periosteal bone is a classic nding of a
healing fracture on a skeletal survey (Table 2).
The follow-up skeletal survey at 2 weeks can
give more precise information about dating of
the fracture (Section on Radiology AAP 2009).
Dating of a fracture remains an imprecise
endeavor. A recent review of 82 accidental long
bone fractures in 63 children younger than
72 months of age showed that the fracture could
be dated as acute if less than 1 week from the
fracture, recent if 835 days, or old if >36 days
(Prosser et al. 2012). Soft tissue swelling, perios-
teal reaction, soft callus, hard callus, bridging, and
remodeling were six key features that allowed
dating (Table 2).
Fractures in Abuse
Although any aged child can sustain an injury
from abuse, it is the child under age 2 years with-
out the verbal skills to tell their story, who is the
Table 2 Dating fractures (Kleinman 1987)
Peak times of appearance of radiographic
features Days
Resolution of soft tissue change 410
Earliest appearance of periosteal new bone 1014
formation
Loss of fracture line denition 1421
Soft callus 1421
Hard callus 2142
Remodeling 1 year
38 Non-accidental Trauma
Table 3 Important patterns of physical abuse (Hobbs
1989)
A single fracture with multiple bruises
Multiple fractures in different stages of healing, possibly
with no bruises or soft tissue injuries
Metaphyseal-epiphyseal injuries
Rib fractures
New periosteal bone
Skull fracture in association with intracranial injury
Fig. 3 Three month old female infant seen in emergency
department with a midshaft femur fracture. A routine skel-
etal survey showed multiple fractures including a classic
metaphyseal lesion of the distal radius (block arrow). This
is also termed corner fracture
most vulnerable to repeat abusive episodes. Up to
9,000 infants and children under 2 years of age
are hospitalized in the USA with a fracture
(Leventhal and Martin 2010). Approximately
20 % of these fracture cases are caused by abuse,
although the numbers are likely much higher
due to underrecognition and underreporting
(Ravichandiran et al. 2010).
Hobbs described six important patterns of frac-
ture from physical abuse (Table 3) (Hobbs 1989).
However, no fracture pattern or location is patho-
gnomonic of child abuse. Several fractures such as
metaphyseal corner fractures (classic metaphyseal
lesion) (Fig. 3), posterior rib fractures, and frac-
tures in various healing stages are so commonly
seen in non-accidental trauma that a high suspi-
cion for abuse is always warranted for these
889
fracture patterns (Baldwin and Scherl 2013). Pre-
diction of abuse is often compounded by method-
ology, small retrospective series, a wide variation
in ages of children, inclusion of all types of frac-
tures, and when the mechanism of motor vehicle
accident (MVA) is included in the analysis.
Although there may be an association with abuse
for a certain type of fracture, that association does
not prove that the injury was caused by abuse. For
most studies that show this association for a par-
ticular fracture type, the association is usually less
than 50 %. For fractures in general, features such
as young age, Medicaid insurance, nonwhite race,
male sex, and presence of additional injuries such
as traumatic brain injury had associations with
abuse (Leventhal and Thomas 1993; Leventhal
and Martin 2010; Lane and Rubin 2002; Skellern
and Wood 2000). For an active child who is capa-
ble of rough housing on furniture, the rate of
suspected abuse was only 5.5 % (Hennrikus and
Shaw 2003).
Age seems to be one of the strongest predictors
of abusive fractures. For children under age
3 years who present with any type of fracture
from any mechanism, the probability of abuse
ranges from 10.8 % to 22.4 % (Leventhal
et al. 2007, 2008; Leventhal and Martin 2010).
For isolated long bone fractures in children less
than 3 years of age, only 1 % of these fractures
were diagnosed by the treating physician to be
from child abuse (Taitz and Moran 2004). For
younger-aged children, the probability for abuse
dramatically increases. In infants (<1 year of age)
with any type of fracture, 26.3 % were reported to
child protective services (CPS), with abuse being
conrmed in 15.2 % of the total study group
(Skellern and Wood 2000). Leventhal found an
even higher risk (37.5 %) of abuse in infants
(Leventhal and Thomas 1993). Other studies
have found age <4 months (Skellern and Wood
2000) and age <24 months (Leventhal et al. 2008;
Leventhal and Martin 2010) to be associated with
an increased risk of abuse. As a general principle,
because of the higher association of abuse with
age under 1 year and the difculty in performing a
reliable physical examination on an infant in pain,
one cannot be faulted for obtaining a skeletal
survey on an infant who presents with a fracture
890
even if the suspicion for child abuse is low.
Although fractures in infants are highly associated
with abuse, this association is typically less than
50 %. While important to be mindful and even
suspicious about abuse in an infant, it is equally
important to not be judgmental.
Rib Fractures
Rib fractures are rarely from accidental trauma.
They can be very difcult to detect, despite often
being multiple. They occur posteriorly by a mech-
anism of anterior compression on the thoracic
cage that levers the posterior aspect of the rib
against the transverse process of the spine
(Hobbs 1989). First rib fractures that have been
reported in case series and case reports in infants
have almost always been attributed to abuse
(Melville et al. 2012). Lower rib fractures are typ-
ically posterior at the costovertebral articulation
and are caused by lateral gripping of the infant
with anterior posterior chest compression (Tsai
et al. 2012). Rib fractures can be very difcult to
visualize on routine chest radiographs and are
detected by careful radiographic study and skeletal
survey (Hobbs 1989). Cardiopulmonary resuscita-
tion does not typically cause rib fracture in the
young child. Since rib fractures are so rare in
accidental trauma under 2 years of age, all rib
fractures in this age group should receive a full
skeletal survey with follow-up survey. Kleinman
reported sternum fractures to be specic for abuse
(Kleinman 1998).
Classic Metaphyseal Lesion
Metaphyseal fractures adjacent to the physis are
classic for physical abuse in the young child
(Fig. 3) (Hobbs 1989). The classic corner fracture,
termed the classic metaphysical lesion (CML), is a
result of acceleration and deceleration of the infant
with resulting torsion and distraction to the end
of the bone. This pulling and twisting of the
metaphysis disrupts this weaker newly formed tra-
becular bone near the physeal cartilage, resulting in
the appearance of a corner fracture (Hobbs 1989).
R.M. Schwend
Common areas for the CML include the distal
radius and ulna, elbow, knee, and ankle. Kleinman
et al. in autopsy studies of abused infants reported
that bucket handle and corner fractures are full-
thickness metaphyseal fractures that extend through
the primary spongiosa of the bone, just proximal to
the zone of provisional calcication (Kleinman and
Marks 1995). Near the center of the physis, the
amount of metaphyseal bone is thin but is much
thicker at the periphery, resulting in a bucket
handle radiographic appearance. Although often
present in several locations, the presence of one
CML lesion on skeletal survey is highly suspicious
and specic for child abuse (Kleinman et al. 2011).
Fracture callous does not always appear early in a
CML, so dating the occurrence of the fracture by
skeletal survey is not reliable.
Other Related Injuries
Brachial plexus injuries caused by abuse have
been reported in young children (McMillan
et al. 2010). Spine fractures may be the only
indication of skeletal trauma and has an associa-
tion with intracranial injury (Barber et al. 2013).
Likewise, a child with a cervical spine fracture
may initially present with a defect in the upper or
lower extremity. When abuse is the cause of a
spine injury, it is commonly associated with spinal
cord injury without obvious radiographic abnor-
malities (SCIWORA). The skeletal survey should
always include a lateral of the cervical, thoracic,
and lumbar spines, since CT scan may also show a
fracture. However, if clinical or radiographic eval-
uation suggests a spine fracture, further imaging
with MRI should be obtained. If a child has AHT
and an MRI is being done to further evaluate the
brain, the MRI should include the entire spine
(Kemp et al. 2010).
Upper Extremity Injuries in Abuse
Upper extremity fractures have been associated
with abuse, typically in younger children. Frac-
tures from abuse may be in unusual locations such
as the distal clavicle, acromial tip, scapula,
38 Non-accidental Trauma
Fig. 4 Six week old infant
was taken to emergency
room by his mother after a
reported fall. He was noted
to have limited spontaneous
use of left upper extremity.
Radiograph showed an
acromion fracture (arrow).
Further evaluation of family
situation revealed that the
boyfriend had been
babysitting alone and tried
to quiet him when he was
crying
proximal humeral metaphysis, or distal humeral
physis (Fig. 4). The mechanism of fracture is from
violent blows or traction injuries and is suggestive
of abuse in young children.
Clavicle Fractures
Clavicle fractures are the most common birth
fractures and are related to a large infant, dystocia,
or a difcult delivery. Infants may normally have a
separate ossication center at the tip of the
acromion that resembles a fracture, so this must
be distinguished from the sharp demarcated edges
with callous seen in a healing fracture (Fig. 4).
The older the child, the more likely a clavicle
fracture is caused by an accidental injury. The
probability of abuse in a child less than 3 years
of age varied from 5.9 % to 20.7 % (Leventhal
et al. 2007, 2008) but was 28.1 % in children less
than 12 months of age (Leventhal et al. 2008). In
an infant under 10 days of age with a history
consistent with a birth injury, a skeletal survey is
not indicated. For the 1121-day-old infant with
possible birth injury, the need for a skeletal survey
is less clear. After 21 days of age, a fresh clavicle
fracture requires a skeletal survey. For children
from 12 to 23 months of age with a history of a
fall, the skeletal survey should be performed if
there is suspicion from the history or a
891
non-plausible mechanism. If a child 1 month to
2 years of age presents with a healing fracture
noted unexpectedly on a routine chest radiograph,
a skeletal survey should be done.
Humerus Fractures
The probability of abuse in children less than age
3 years with a humerus fracture of any type from
any etiology ranges from 9.3 % (Kowal-Vern
et al. 1992) to 18 % (Shaw et al. 1997) to 50 %
(Leventhal et al. 2008). The probability for abuse
in the child who presents with a diaphyseal
humerus fracture depends on whether the case
was reviewed by Child Protection Services, in
which 8.8 % were determined to be abuse related,
or based on physician evaluation in which 17.6 %
were abuse (Shaw et al. 1997). An age under
1218 months is associated with an increased
probability that the fracture will be from abuse
(Thomas and Roseneld 1991; Strait and Siegel
1995; Leventhal et al. 2008; Pandya and Baldwn
2010). However, in these same studies, a
supracondylar humerus fracture was associated
with a lesser probability of being caused by
child abuse. Other features, including more
proximal fractures, diaphyseal fractures, evidence
of prior trauma, suspicious history, or spiral or
oblique fractures, were associated with abuse
892
(Pandya and Baldwn 2010). For a spiral fracture
of the humerus, the plausible mechanism, the
history of other injuries, and the skeletal survey
are key to determining if this is abuse related
(Hobbs 1989).
The distal humerus epiphyseal fracture is
highly associated with child abuse. An infant
may present with a swollen elbow of unknown
cause or with a story that is not plausible. If this is
an acute or recent injury, the infant or young child
may be in much pain, and the elbow may be warm
and tender. If the injury has been present for more
than 1014 days, it may be less painful or pain-
free and have swelling related to the palpable
healing fracture, which by then should be visible
on a radiograph. The injury may be confused with
infection or if displaced, an elbow dislocation.
Radiographs may simply only show soft tissue
swelling. If displaced, the proximal radius and
ulna are not in line with the humerus. A skeletal
survey should be done in an infant with a swollen
elbow that is not an infection or in an infant with
any fracture of the humerus. Ultrasound may
show the nature of the displaced epiphysis from
the shaft of the humerus. Follow-up skeletal sur-
vey may show the subperiosteal reaction at 1014
days, typical for a healing fracture.
Forearm Fractures
Radius and ulna fractures are associated with
abuse particularly in children less than 1 year
of age (Leventhal et al. 2008). When looking at
< 3-year-old children, abuse is considerably less
likely, ranging from 7.5 % to 30 %, depending on
the study population (Leventhal et al. 2007,
2008). The skeletal survey was noted to have a
sensitivity of 75 % (CI 34.996.8) to detect a
radius fracture and 50 % (CI 15.784.3) to detect
an ulna fracture (Mandelstam et al. 2003). The
distal radius contributes 80 % of the growth of
the radius, so metabolic conditions such as vita-
min D deciency are frequently apparent early in
this location. Olecranon fractures are a common
feature of OI, and this diagnosis should be con-
sidered, especially if bilateral. A distal radius or
ulna buckle fracture from a fall onto an
R.M. Schwend
outstretched hand in a child who cruises or
walks does not require a skeletal survey if there
is no other suspicion for abuse (Wood et al. 2014).
Hand Fractures
In older children, hand fractures are extremely
common but are suspicious for abuse in infants.
In infants younger than 10 months of age, frac-
tures are typically torus fractures of the metacar-
pals or the proximal phalanges of the hand, as well
as of the feet (Nimkin et al. 1997). Skeletal survey,
particularly the oblique view, is necessary to detect
these fractures, as clinical signs are unusual.
Differential Diagnosis
While it is essential not to miss child abuse as the
underlying cause of a childs injuries, it is just as
important to remain objective, be open to alterna-
tive explanations, and make an accurate diagno-
sis. Inappropriately diagnosing child abuse is
harmful to the child and family, risks the parents
losing custody, and is a misuse of resources
(Schwend et al. 2000). Be wary of false allega-
tions, especially when there are custody and visi-
tation disputes or if the charges are coming from
the parent (Bernet 1993). Alternative diagnoses
are also brought up in custodial and criminal
cases, so diagnostic accuracy during the initial
evaluation is essential.
Injury to a developing bone of an infant leads
to subperiosteal hemorrhage, leading to
subperiosteal healing bone by 1014 days
(Hobbs 1989). There is a normal physiologic
diaphyseal reaction that is frequently present in
infants younger than 6 months of age and is sym-
metric and diaphyseal only on the long bones of
the extremities (Pergolizzi and Oestreich 1995).
Initial radiographs can be normal, with a skeletal
survey and follow-up survey being most impor-
tant. Besides trauma and infection, other condi-
tions such as Caffeys disease (infantile cortical
hyperostosis), vitamin C deciency, vitamin D
deciency, metastatic neuroblastoma, Langerhans
histiocytosis, vitamin A intoxication, leukemia,
38 Non-accidental Trauma
and various drugs are more rare causes of
subperiosteal bone formation than child abuse
(Hobbs 1989). Other causes of fracture include
osteogenesis imperfecta, rickets of prematurity,
disuse osteoporosis, copper deciency, and oste-
omyelitis (Hobbs 1989). Congenital syphilis is
rare but is increasing in prevalence. Congenital
syphilis readily mimics the fractures of child
abuse with diaphysitis, metaphysitis, and patho-
logic fractures in various stages of healing, mim-
icking child abuse (Lim et al. 1995). If the
skeleton otherwise looks normal radiographically,
this strongly excludes general conditions of
genetic, metabolic, or bone disease (Hobbs 1989).
Other conditions may resemble the classic
metaphyseal lesion. Apparent corner fractures of
the distal radius and ulna, proximal humerus, and
tibia are seen in spondylo-metaphyseal dysplasia
(Currarino et al. 2000). Classic CML lesions of
child abuse have been described after forced serial
casting for clubfoot and in patients with acute
lymphoblastic leukemia. Bony beaks are normal
metaphyseal variations seen in 25 % of infants and
toddlers, in both the upper and lower extremities,
and are seen at the distal radius and ulna, usually
bilaterally.
Osteogenesis imperfecta (OI) should be con-
sidered when a child with multiple fractures is
seen. A family history of OI helps to focus the
evaluation, but OI may occur by spontaneous
mutation, without obvious previous family his-
tory. OI has clinical features that are very different
from what is seen in child abuse. Greeley
reviewed their series of 68 children with OI,
with 72 % being diagnosed based on clinical
features alone (Greeley et al. 2013). Children
with more than two fractures (10 % of the group)
were typically diagnosed prenatally or as new-
borns. Multiple rib fractures or more than one
fracture did not occur after the newborn period
and so is more consistent with child abuse than IO
(Greeley et al. 2013). Although no fracture is
specic for OI, children with OI may have coxa
vara and bowing of other long bones, osteopenia,
and gracile bones. Olecranon fractures are com-
monly associated with osteogenesis imperfecta
and are sometimes bilateral. Children with OI
may easily bruise. Infants normally have blue
893
sclera, which can give a false impression of OI
when multiple fractures are also present (Marlowe
et al. 2002). Although OI should be able to be
made on clinical grounds, biochemical assay is
available from skin biopsy broblast cultures
assayed for procollagen levels and structural
abnormalities. However, in actual practice, when
assays are done in cases of suspected child abuse,
OI was found in only 6 of 48 children. In 5 of these
6, the diagnosis of OI could have been made from
the clinical features (Steiner et al. 1996). OI and
child abuse can coexist in the same child.
Premature infants are more susceptible to frac-
tures, due to their prematurity, nutrition, and
increased family stress. Underlying genetic, met-
abolic, and nutritional deciencies may also pre-
dispose to fracture. A premature infant with
multiple fractures should be investigated for oste-
oporosis and bone health, as well as for abuse.
Temporary brittle bone disease, although well
established in the literature, has insufcient scien-
tic evidence to be plausible, so this diagnosis
should be discouraged as an explanation in chil-
dren with multiple fractures (Jenny 2010).
Reporting and Documentation
Role of Suspected Child Abuse
and Neglect (SCAN) Team
The rst step in caring for the child suspected of
abuse is to provide a safe environment and an
objective diagnostic workup, typically done by
admitting the child to the hospital. All states
require reporting of not only conrmed abuse or
neglect but also if abuse is suspected or is a
possibility. Despite clear requirements and immu-
nity for reporting in good faith, reporting by phy-
sicians is not done in a fourth of cases of likely
abuse and three fourths of possible abuse. Failure
to report abuse places the surgeon at risk for
malpractice and the child at risk for repeat injury
and death. The orthopedic surgeon should place a
call to the SCAN team and allow them to assume
management of the child, including hospital
admission, reporting to protective services, a diag-
nostic workup, and follow-up care. The minimal
894
Fig. 5 Algorithm for Do injuries have plausible mechanism?
treating upper extremity Yes
injury
Are there other features of abuse?
(Table 3 important patterns of physical abuse present)?
(History of previous abuse?)
no
Low risk No survey
abuse
evaluation when abuse is suspected includes a
properly done skeletal survey, dilated fundoscopic
examination by an ophthalmologist, and consulta-
tion by the child protective services team.
Surgeons Role
Orthopedic surgeons as a group are knowledge-
able about the need to be mindful of the possibility
of child abuse and reporting responsibility; how-
ever, only 61 % of more experienced surgeons
stated that they would further inquire about what
caused the childs symptoms (Tenenbaum
et al. 2013). From a practical sense, there are
clinical pearls and pitfalls to be aware of when
treating young children whom may be at risk for
abuse (Fig. 5, Table 4). The surgeon should record
ndings in as much detail as possible and docu-
ment who is giving the history. No changes should
be made to the record if the parent or family
member wishes to change their story; rather, the
new revised story should be accurately
documented as a new note. The timing and exact
mechanism of injury, who was caring for the child
at the time of the injury, family member response
to the injury, and how soon care was sought
should be recorded. Chart notes must be detailed
and accurate as they may be presented as evidence
in court for custodial hearings or for a criminal
R.M. Schwend
No
SCAN/Opthalmology
yes SCAN Opthal
Is child under 2 years of age?
No Yes
If 2-5 years Obtain skeletal survey.
Skeletal survey If < 1 year of age obtain
If highly suspicious skeletal survey for most
any fracture
Table 4 Pearls and pitfalls of child abuse
Skeletal survey is justied for any child <2 years of age
with suspected abuse
Careful detailed interview, documentation, and precise
diagnosis
Report suspicions to hospital child protective services
team
Unexplained fractures are more likely to be abuse related
rather than from undiagnosed medical conditions
However, keep open mind about other conditions that
may be present or coexist with the injury
Be cautious of a young infant with bruises or fractures
Be aware if the injury mechanism described by caregiver
does not make biomechanical sense
Injury that happens in a home daycare setting
Previous court proceedings for abuse
Serious injury sustained from a reported short fall
Any child unexpected death
Rib fractures, multiple fractures in various stages of
healing, CML
trial. Inaccurate notes could place a child at risk
for returning to an abusive home.
If called to court, the surgeon may be placed in
the dual role of a material witness as well as an
expert witness. However, limits to the physicians
expertise need to be dened in advance of the
testimony. The orthopedic surgeon, if requested
to testify in court, should come thoroughly pre-
pared, with the knowledge of the case and the
medical record, and have a pretrial, preferably in
38 Non-accidental Trauma
person, interview with the attorney for the child. If
expert witness testimony is expected, the orthope-
dic surgeon should also come well prepared with
the knowledge of the current relevant child abuse
literature. The surgeon may be asked to give opin-
ion within reasonable medical certainty, which is as
certain as a competent physician would need to be
in recommending treatment for any medical condi-
tion (Chadwick 1990). Since the courtroom is unfa-
miliar to most physicians, the prosecuting attorney
should take the time to educate the surgeon on
proper preparation, dress, courtroom behavior and
etiquette, language, and pitfalls to avoid.
Prevention
Recurrence rates are high, so children previously
abused need special consideration. A prior court
appearance is a risk factor for further abuse.
Releasing the child to the custody of a family
relative is a common way to provide for needs of
the child and family; however, this can still place
the child at risk if the perpetrator violates a court
order and obtains unsupervised access to the child.
Pediatricians are well aware of the importance for
prevention of child abuse, with dedicated fellow-
ship training and certication available (Preer
et al. 2012). Many early intervention programs
are available to prevent child abuse. Early home
visits after birth for high-risk parents are available.
The American Academy of Pediatrics (AAP) has a
project called Practicing Safety that works to
decrease child abuse and neglect through anticipa-
tory guidance and early screening of children age
03 years (www.aap.org/practicing%20safety). A
hospital-based education program for parents of all
newborn infants was shown to signicantly reduce
the incidence of abusive head trauma in children
under 3 years of age (Dias et al. 2005).
Resources
Visual Diagnosis of Child Abuse on CD-EOM.
This is media from the American Academy of
Pediatrics 2008 (www.aap.org) and has more
than 300 diagnosis slides.
895
Child Abuse: Medical Diagnosis and Manage-
ment. Lecture series of topics in child abuse
(www.aap.org)
AAP Section on Child Abuse and Neglect
website: www.aap.org/sections/scan
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 Nail Bed Injuries
39
Ryan Katz

Contents Abstract
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 899 Restoration of the nail plate and reconstruction
of the nail bed should be part of any treatment
Anatomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 900
algorithm for ngertip injuries. When possible,
Pathoanatomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 900 all structures should be reconstructed or
Assessment of the Injured Fingertip . . . . . . . . . . . . . . 900 replaced to deliver optimal aesthetic and func-
tional result. When a childs nger is crushed
Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 901
The Avulsed Nail Plate . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 901 and the nail plate is injured, there is often an
The Seymour Fracture . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 902 associated injury to the underlying nail bed.
Operative Treatment of Nail Bed Injuries . . . . . . . . . . . 902 This chapter will discuss the various types of
Preferred Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 904 injuries that occur as well as their treatment
Subungual Hematoma (Small) . . . . . . . . . . . . . . . . . . . . . . . 904 options. Management of complications,
Subungual Hematoma (Large) . . . . . . . . . . . . . . . . . . . . . . . 905 including nail deformities (hook nail, raised
Nail Plate Avulsion (Partial) . . . . . . . . . . . . . . . . . . . . . . . . . 905
Nail Plate Avulsion (Partial: Seymour Fracture) . . . . 905 nail, and split nail), will also be presented.
Nail Plate Avulsion (Complete) . . . . . . . . . . . . . . . . . . . . . 905
Loss of Matrix Substance (Sterile Matrix) . . . . . . . . . . 905
Loss of Matrix Substance (Germinal Matrix) . . . . . . 906 Introduction
Surgical Pitfalls and Prevention . . . . . . . . . . . . . . . . . . . . . 906
Management of Complications . . . . . . . . . . . . . . . . . . . . . . 906
The importance of the ngertip is often
Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 907
overlooked and underappreciated. This unique
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 907 structure, rich with sensory end organs, serves as
a means through which we navigate our tactile
world. From both an aesthetic and functional
standpoint, the nail plate is a critical feature of
the human ngertip. The uninjured nail plate facil-
itates pinch, increases ngertip sensitivity, allows
scratch, and protects the digit from trauma (Tos
et al. 2012). An intact and non-diseased nail plate
can also be a marker of general health and proper
nutrition. Restoration of the nail plate and recon-
R. Katz
struction of the nail bed should therefore be part of
Union Memorial Hospital, The Curtis National Hand
Center, Baltimore, MD, USA any treatment algorithm for ngertip injuries.
e-mail: ryankatz1@gmail.com Knowing the above and given the fact that
# Springer Science+Business Media New York 2015 899
J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_40
900
ngertip injuries represent nearly two-thirds of
pediatric hand injuries (Miranda et al. 2012), the
burden of surgical need in the pediatric population
can be quite large. In fact, nail bed injuries make
up 1524 % of all ngertip injuries (Miranda
et al. 2012) and are the most common pediatric
hand injuries presenting to the emergency room
(Fairbairn 2012). In approximately 50 % of cases,
nger nail injuries are associated with distal
phalanx fractures (Tos et al. 2012).
Anatomy
Formulating a treatment plan for the injured nail
bed requires a thorough understanding of the anat-
omy of the perionychium. When possible, all
structures should be reconstructed or replaced to
deliver an optimal aesthetic and functional result.
The nail plate is bordered by the lateral nail folds
and covered proximally by the proximal nail
fold. The eponychium is the supercial dorsal
roof of the proximal nail fold. Distally and imme-
diately volar to the nail plate is the lymphatic rich
hyponychium. Removal of the nail plate reveals
the nail bed immediately underneath. The nail
bed consists of the germinal matrix proximally
and the sterile matrix distally. The germinal
matrix visualized under the clear intact nail
plate as a white half moon is called the lunula.
The germinal matrix produces ungual keratin and
is responsible for 90 % of the thickness of the nail
plate (Tos et al. 2012). The sterile matrix essen-
tially provides only plate adherence (Fassler
1996). The matrix tissue that forms the dorsal
roof of the proximal nail fold provides shine to
the nail plate (Fassler 1996).
Pathoanatomy
When the childs nger is crushed and the nail
plate is injured, there is often an associated injury
to the underlying nail bed. This can manifest as a
subungual hematoma. The nature of the nail bed
injury and possible underlying distal phalanx
involvement cannot be entirely determined by
the size of the hematoma alone, and the treating
R. Katz
physician is advised to thoroughly examine the
digit both clinically and radiographically. Due to
the cartilaginous and radiolucent nature of the
open physis, physeal fractures in the pediatric
patient, if not suspected, are sometimes missed.
These fractures, called Seymour fractures, can
have signicant negative sequelae ranging from
growth disturbance to nail plate deformity to
osteomyelitis and loss of the distal phalanx
(Fairbairn 2012).
When the nail bed is injured and not appropri-
ately repaired, scar can replace the unique tissues
described above. In such a setting, the function of
those tissues will be disrupted. Specically, scar
involving the matrix tissue that forms the dorsal
roof of the proximal nail fold can result in loss of
nail sheen and cause an opaque line to disrupt
an otherwise clear nail plate. An injury to the
germinal matrix will halt nail growth at the loca-
tion of scar and result in a split nail. Germinal
matrix injuries can also cause a nail plate ridge
or alter the nail plate thickness. Injuries to the
sterile matrix will result in loss of nail plate
adherence. Loss of the distal phalanx, which pro-
vides nail bed support, will result in a hook nail.
These deformities draw attention to the injured
digit and may place the digit at risk for repeated
trauma (e.g., nail plate ingrowth, nail plate catch-
ing on objects).
Assessment of the Injured Fingertip
Knowing the mechanism of injury will aid the
physician in treating the patient with the injured
ngertip. Radiographs should be performed for
most ngertip injuries presenting to the emer-
gency room. The child will often hold their hand
in a protected posture and not readily offer the
hand for examination. After a sensory exam has
been performed if possible given the childs age,
the examiner should anesthetize the digit (and/or
sedate the patient if needed) to allow for a thor-
ough examination of the injury. If the digit has
been caught in a door, there will be a signicant
force applied over a small area. The pressure
thus created can fracture or avulse the nail plate,
lacerate the underlying nail bed, and fracture or
39 Nail Bed Injuries
amputate the distal phalanx. In a scenario where
an amputation has not occurred, the child will
often present with a partially intact nail plate and
a visible subungual hematoma. A partially
avulsed nail plate or an intact nail plate with a
large subungual hematoma should be completely
removed to allow inspection of the underlying nail
bed. If the digit has the appearance of a mallet
deformity, the index of suspicion for a Seymour
fracture (physeal fracture) should be high. Radio-
graphs showing a widened physis or displacement
between the distal phalanx base and diaphysis will
conrm the diagnosis. Radiographic comparison
of the injured digit to uninjured digits on the same
hand can aid in the diagnosis of physeal injuries.
Sharp lacerations through the nail bed that do not
involve bone do not require removal of the nail
plate. In these scenarios, the nail plate can act as a
splint maintaining alignment of the injured nail
bed. Avulsive injuries to the nail plate and bed
routinely expose the underlying distal phalanx.
The depth of injury should be assessed and the
need for soft tissue coverage and nail bed recon-
struction determined.
Treatment
The Avulsed Nail Plate
When crushed, the nail plate often fails at the
plate/soft tissue interface. This is likely the result
of the different material properties of the nail plate
and the surrounding perionychium. As the germi-
nal matrix does not contribute much to the adhe-
sive properties of the nail bed, the proximal nail
overlying the germinal matrix will likely give way
prior to the distal nail. It is thus relatively common
to see partial nail avulsions where the proximal
nail plate has ruptured through the eponychial
fold. Radiographs should be performed to rule
out an underlying distal phalanx injury including
a Seymour fracture. If no fracture is identied, the
partial avulsion can be treated by leaving the distal
nail plate intact, trimming the avulsed proximal
nail, and applying a nonadhesive dressing.
Stenting the eponychial fold has been thought to
prevent the formation of synechiae (adhesions
901
between the eponychial fold and nail bed), but a
recent study demonstrating a higher rate of clinic
visits and a higher incidence of delayed wound
healing and infection in stented patients has called
into question this practice (Miranda et al. 2012). If
a nail bed laceration is identied after removing the
proximal nail plate, it should be repaired under
loupe magnication. This can be done with either
a ne absorbable suture or cyanoacrylate tissue glue.
Nail bed repair tips and tricks
Suture 5-0 or 6-0 Chromic; a spatulated
recommendations needle may help pass through the
nail bed tissue
Permanent sutures are to be
avoided as removal can be painful
Suturing Follow the curve of the needle
recommendations failure to do so will tear the nail
bed
Simple sutures (no mattress)
Single passes with the needle are
encouraged to avoid tearing the
nail bed
Linear lacerations Primary suture or glue repair
Stellate lacerations Glue repair may be easier and less
traumatic to the nail bed
Nail bed tissue loss Look at underside of nail plate. If
nail bed remnant is adherent to
removed nail plate, it can be used
as a graft
Split graft from intact nail bed
(from the same digit) can be used
in the acute setting for sterile
matrix loss
The cyanoacrylate has been shown to offer
signicant time savings with clinical outcomes
comparable to suture repair (Strauss et al. 2008).
If the nail plate is completely avulsed, any visible
nail bed laceration should be repaired with either a
ne absorbable suture or glue as described above.
If there is associated loss of matrix substance that
cannot be repaired primarily, the clinician should
rst examine the underside of the avulsed nail
plate. If nail bed portions are identied adherent
to the avulsed nail plate, the plate can be
repositioned on the nail bed as a composite
graft aligning the nail such that the missing
matrix segments are replaced in their anatomic
position (Tos et al. 2012). If no nail bed graft is
readily available to replace lost matrix substance,
902
the surgeon can harvest graft from either a spare
part, the injured digit, or a toe. Sterile matrix loss
can be replaced with split matrix grafts. A split
graft can be harvested from either intact sterile
matrix of the injured digit or of an adjacent digit.
Split grafts offer the advantage of replacing lost
tissue without signicant donor site morbidity or
donor nail deformity. The split graft is harvested
by removing the nail plate and shaving a portion
of the sterile matrix with a sharp no. 15 blade. The
graft should be thin enough so the blade can be
visualized through the graft. No repair of the
donor site is needed.
Split grafts however cannot be used to recon-
struct lost germinal matrix, which requires the
growth potential afforded only by a full thickness
nail bed graft (or vascularized toe ap). As would
be expected such a graft would cause an obvious
donor nail deformity, and thus, these grafts should
come from either a spare part (e.g., an amputated
digit) or a digit that is easily concealed (e.g., a
toe). Split and full thickness grafts can be applied
directly to the exposed distal phalanx (Brown
et al. 1999).
If the nail plate remains intact after a signicant
crush injury, the clinician will often see a
subungual hematoma. Invariably, the hematoma
is reective of an underlying nail bed injury. Con-
troversy exists as to how to best treat subungual
hematomas. If the hematoma is small and there is
no associated fracture, simple trephination of the
nail plate will allow hematoma evacuation, pres-
sure reduction, and pain relief. Trephination can
be performed with a heated paper clip or a battery-
powered cautery. If the hematoma is large (tradi-
tionally cited as >25 % of the surface of the nail
plate), some authors recommend nail plate
removal and direct repair of the nail bed lacera-
tion. This has not been shown to be better
however to simple trephination or observation
even when distal phalanx fractures are present
(Gellman 2009; Roser and Gellman 1999). In the
setting of contamination or if there is displace-
ment of the underlying distal phalanx fracture,
removal of the nail plate is indicated to allow
irrigation, debridement, and direct repair of
the nail bed and to assist with fracture reduction
as needed.
R. Katz
The Seymour Fracture
The distal phalanx physis in the skeletally imma-
ture patient represents a potential fault line that
can fail in the setting of crush or force loading
injuries. Given the radiolucent nature of the
physis, radiographic ndings of physeal fractures
can be subtle. The clinician must therefore have a
high index of suspicion for this particular fracture
pattern when evaluating the pediatric patient with
a ngertip injury (Abzug and Kozin 2013;
Krusche-Mandl et al. 2013). Clinically, the patient
will often present with a partial avulsion of the
proximal nail plate through the eponychial fold.
The digit may have a mallet appearance. Radio-
graphically, the physis will look wide compared to
physes of adjacent, uninjured digits, and there
may be displacement of the distal phalanx diaph-
ysis in the dorsopalmar plane (Fig. 1ad).
When the fracture is displaced, it often tears the
proximal nail bed and therefore becomes exposed
to the environment. It should thus be considered
an open fracture. The nail bed can become inter-
posed between the fracture fragments and prevent
reduction. Failure to recognize the injury pattern
and reduce the fracture in a timely manner can
result in permanent nail deformity, permanent
growth disturbance, and osteomyelitis. Removal
of the avulsed nail plate will immediately reveal
the associated nail bed injury. If the nail bed is
interposed between the fracture fragments, it
should be withdrawn. The fracture can then be
reduced and stabilized with a smooth Kirschner
wire. The nail bed laceration should then be
repaired.
Operative Treatment of Nail Bed
Injuries
Many of these procedures can be done in the
emergency room setting under local anesthesia
with IV sedation, especially in older children.
Lidocaine with epinephrine (1 % Lidocaine with
epinephrine 1:100,000) has been proven safe at
the digital level and may preclude the need for a
digital tourniquet (Lalonde 2011; Lalonde and
Martin 2013). The patient is placed supine with
39 Nail Bed Injuries
Fig. 1 Seymour fracture.
(a) Clinical appearance of
proximal nail bed entrapped
in fracture. (b) Clinical
appearance of proximal nail
bed freed from fracture. (c)
Radiograph demonstrating
classic Seymour fracture
through open physis. (d)
Radiograph demonstrating
reduction of fracture and
stabilization with a
percutaneous wire
the injured extremity on a hand table. After a
sensory examination has been performed in
age-appropriate children, the digit is anesthetized.
If epinephrine has not been used, a nger tourni-
quet should be applied to the base of the digit to
provide a bloodless operative eld. The nail plate
can be removed by passing a Freer Elevator
directly under the volar surface of the nail. Care
should be taken to angle the elevator toward the
nail plate and not toward the nail bed this will
minimize iatrogenic injury to the nail bed. The
lateral nail edges should be freed from the lateral
nail folds also with the elevator. Once the nail has
been mobilized from its attachments to the nail
bed and lateral nail folds, a small clamp should be
attached to the lateral aspect of the nail plate and
then turned multiple times like twisting spaghetti
on a fork. This maneuver will allow the nail to
roll out from under the dorsal nail fold. The under-
lying nail bed should now be visible (Fig. 2a).
Any gross contamination, bone devoid of an
adequate soft tissue envelope, or clearly necrotic
tissue should be debrided.
Bony stability should be performed prior to
nail bed repair. A mini C-arm is recommended to
assess the adequacy of the reduction and conrm
903
appropriate placement of hardware. Though a
20G needle can often be manually driven through
the distal phalanx to achieve bone stabilization,
this technique places the surgeons nondominant
hand at risk of injury if a slip occurs during inser-
tion. Instead of a needle, with a lumen, utilization
of a 0.02800 , 0.03500 , or 0.04500 smooth Kirschner
wire and wire driver is recommended. After
achieving fracture stabilization, the nail bed
laceration should be repaired anatomically. This
is done with a ne absorbable suture (6-0 Chromic
or 6-0 Vicryl Rapide) and facilitated by a semicir-
cle needle. Some surgeons also prefer a spatulated
needle. The curve of the needle must be followed
as the nail bed tissue is unforgiving and tears quite
easily (Fig. 2bd). The nail bed repair can also be
performed with a cyanoacrylate glue (Fig. 3ac).
This requires the matrix tissue to be well aligned
prior to glue application. If it is not, a few align-
ment sutures should be placed prior to glue appli-
cation. Placement of a stent is not necessary but
should be considered in the setting of an associ-
ated injury to the dorsal nail fold where scar
between the eponychium and underlying nail
plate can occur. The development of such
synechiae can disrupt future nail growth. If a
904
Fig. 2 Nail bed repair with suture. (a) A longitudinal
nail bed injury through the sterile matrix. (b) A demonstra-
tion of nail bed repair with one suture pass following the
curve of the needle. (c) A demonstration of nail bed repair
stent is used, it can be any nonstick space occu-
pying object (the avulsed nail plate, foil from the
suture pack, silicone sheet, Xeroform). The stent
can easily be secured by a gure of 8 suture
thrown between the eponychium and the
hyponychium utilizing absorbable suture. The
stent can be removed at 1 week or remain in
place until the new nail plate pushes it out.
Split thickness nail bed graft can be harvested
from the injured digit, a spare part amputated
digit, or a toe with a no. 15 or no. 11 blade after
removing the nail plate. The blade is placed at a
slight angle to the nail bed donor site and used to
shave off a thin portion of premeasured nail bed
graft. As it is being raised, the graft should be thin
enough to enable visibility of the underlying blade
through the graft. Given the obvious potential for
donor site morbidity, full thickness nail bed grafts
are ideally harvested from an amputated and
non-replantable spare part. These can be raised
directly with a scalpel and are used most often to
replace missing germinal matrix. If the wound is
R. Katz
with one suture pass following the curve of the needle. (d)
A demonstration of completing the suture by tying a square
knot without tearing the nail bed
not contaminated and there is no concern for
active infection, the split or full thickness graft
can be placed directly on the distal phalanx. The
grafts can be secured with absorbable suture or
glue. Protecting the graft with a stent (described
above) may minimize the potential for shear forces
and inadvertent trauma. The nger tourniquet must
be let down at the end of the procedure and the
viability of the ngertip documented. Forgetting to
remove a tourniquet will result in loss of the digit.
Preferred Treatment
Subungual Hematoma (Small)
With an intact nail plate, if no underlying fracture
is present or there is a non- to minimally displaced
distal phalanx fracture, the nail plate should be left
intact. A trephination is performed only if the
patient has signicant pain. The patient can be
managed with a tip protector splint or Stack
39 Nail Bed Injuries
Fig. 3 Nail bed repair with glue. (a) The authors
preferred method of delivering a small amount of glue in
a precise fashion. The internal vial is cracked by squeezing
as it normally would be. The glue is then accessed by
passing a 20G needle through the plastic external vial.
The glue is withdrawn into a 3 cc syringe. The needle is
then changed to a 25G needle to allow precision delivery.
(b) Demonstration of a small amount of glue being deliv-
ered through a 25G needle. (c) Demonstration of a small
amount of glue being delivered to a precise location
through a 25G needle
splint that immobilizes only the distal inter-
phalangeal joint. Care should be made to ensure
that the splint does not apply pressure on the
nail bed.
Subungual Hematoma (Large)
With an intact nail plate, if no underlying fracture
is present or there is a non- to minimally displaced
distal phalanx fracture, the nail plate should be left
intact. A trephination is performed only if the
patient has signicant pain. The patient can be
managed with a tip protector or Stack splint
that immobilizes only the distal interphalangeal
joint. If there is a displaced distal phalanx diaph-
yseal fracture, the nail plate should be removed.
This allows for hematoma decompression, can aid
in fracture reduction, and allows for direct repair
of the nail bed. Using multiplanar intraoperative
905
uoroscopy, the fracture is manually reduced and
pinned with a 0.02800 , 0.03500 , or 0.04500 smooth
Kirschner wire. The wire is left protruding from
the skin and protected with a Jurgan Ball. The nail
bed is then repaired with a 6-0 Chromic suture
under loupe magnication.
Nail Plate Avulsion (Partial)
If the nail plate has been partially avulsed, the
raised portion of the plate should be trimmed
away, leaving the adherent portion of the plate
intact. Any underlying nail bed laceration is then
assessed and repaired as described above.
Nail Plate Avulsion (Partial: Seymour
Fracture)
If radiographs demonstrate a wide physis or dis-
placement of the distal phalanx diaphysis in the
dorsopalmar plane, a Seymour fracture is
suspected. In this scenario, the entire nail plate is
removed with a Freer Elevator to allow adequate
exposure of this open fracture. The nail bed,
which can be interposed within the fracture frag-
ments, is liberated and the fracture reduced. The
fracture is stabilized with a smooth Kirschner wire
facilitated by multiplanar interoperative uoros-
copy (Fig. 1c, d). The nail bed repair is then
performed with a ne absorbable suture.
Nail Plate Avulsion (Complete)
This scenario offers a clear view of the underlying
injury. A nail bed injury is repaired with a ne
absorbable suture under loupe magnication and
tourniquet control. A stent will only be applied if
there has been an injury to the dorsal nail fold.
Loss of Matrix Substance (Sterile
Matrix)
Under tourniquet control after the nail plate has
been removed, the dimensions of the missing
906 R. Katz

matrix are measured. If enough nail bed remains fracture. If the fracture is physeal, it can be missed
on the injured digit, graft harvest from this site is radiographically. This can have potentially signif-
preferable. The great toe can also be used as a icant negative sequelae. Prevention of this pitfall
graft donor site if the injured digit cannot offer the comes from having a high index of suspicion
tissue needed. If the great toe is to be used, after a when treating the skeletally immature patient.
toe digital block, the nail plate can be partially Comparison of the injured phalangeal physis to
excised or partially lifted (removal of the entire the uninjured physes of adjacent digits can reveal
toe nail is not necessary). At either donor site, a widening or displacement that will allow the
no. 15 blade is used to shave a thin graft from the examiner to accurately make the diagnosis of
sterile matrix. The graft should be thin enough to physeal fracture.
allow visualization of the underlying blade
through the graft as it is being raised. The graft
is then inset using a ne absorbable suture or Management of Complications
cyanoacrylate glue. A stent is placed over the
graft (either avulsed nail or foil from a suture Nail plate deformity can be quite frustrating to the
pack) to minimize shear and inadvertent trauma. patient and, in addition to functional disturbance,
The stent is secured with a gure of 8 stitch. can be a cause of social and emotional distress.
The deformed nail draws unwanted attention. The
split, cracked, or raised nail can get caught on
Loss of Matrix Substance (Germinal objects and be a source of pain (Fig. 4). Manage-
Matrix) ment of the nail plate deformity requires under-
standing and, if possible, correction of the
Under tourniquet control after the nail plate has underlying pathology. A hook nail occurs due to
been removed, the dimensions of the missing loss of underlying phalangeal support of the nail
matrix are measured. If an amputated spare
part is available, a full thickness germinal matrix
graft is harvested directly off of the distal phalanx.
This can be facilitated by raising the eponychial
fold and suturing it back prior to graft harvest. If
no spare part is available, the graft may be
harvested from a toe; however, this will result in
a toe nail deformity. The patient should be fully
informed and should provide witnessed consent
prior to proceeding with a full thickness germinal
matrix nail bed graft from an uninjured digit. The
graft can be applied directly to the distal phalanx
and secured with either absorbable sutures or a
cyanoacrylate glue (Fig. 3ac). A stent is applied
to the reconstructed nail bed and secured with a
gure of 8 suture.

Surgical Pitfalls and Prevention

Failure to recognize the magnitude of injury is the

most common surgical pitfall associated with
Fig. 4 Split nail. Example of a split nail deformity after
ngertip injuries. In 50 % of patients with a nail trauma (Courtesy of The Curtis National Hand Center
bed injury, there is an underlying distal phalanx Image Library. #The Curtis National Hand Center)
39 Nail Bed Injuries
Fig. 5 Hook nail.
Example of a hook nail
deformity after loss of bony
support in the setting of a
partial ngertip amputation
bed (Fig. 5). This challenging deformity may
benet from a local or regional ap to provide
soft tissue augmentation of the distal aspect of the
nger to provide nail bed support. A split thick-
ness nail bed graft can then be applied directly to
the ap. A raised nail has as its etiology loss of
sterile matrix integrity. This can be managed by
nail plate removal followed by identication and
excision of the scarred portion of the sterile
matrix. A split thickness nail bed graft harvested
from the injured digit or the great toe should be
applied to the resected area to effectively recon-
struct an uninterrupted sterile matrix. A split nail
has as its etiology loss of germinal matrix integ-
rity. This can be managed by nail plate removal
followed by identication and excision of the
scarred portion of the germinal matrix. Raising
and suturing back the eponychial fold will facil-
itate working on the germinal matrix. A full
thickness nail bed graft harvested from an ampu-
tated spare part or a toe can be applied to the
resected area to effectively reconstruct an
uninterrupted germinal matrix. As this procedure
can cause signicant morbidity to the donor
digit, the patient with this nail deformity should
also be offered a nail plate ablation in lieu of
reconstruction. This can be done via surgical
excision of the germinal matrix or chemical abla-
tion with phenol.
907
Summary
The purpose of this chapter has been to introduce
the reader to the intricate, delicate, and unique
anatomy of the pediatric perionychium, nail bed,
and nail plate. Too often, ngertip and nail injuries
are either overlooked or undertreated. This results
in a predictable consequence of deformity, discom-
fort, or altered function. The treating physician is
therefore counseled to do all in their power to
recognize the pathoanatomy and treat appropri-
ately. Avoiding pitfalls such as hidden nail bed
lacerations and occult physeal fractures of the distal
phalanx is paramount to minimizing the potential
negative sequelae of these common injuries.
References
Abzug JM, Kozin SH. Seymour fractures. J Hand Surg
Am. 2013;38(11):226770.
Brown RE, Zook EG, Russell RC, Robert C. Fingertip
reconstruction with aps and nail bed grafts. J Hand
Surg. 1999;24(2):34551. doi:10.1053/jhsu.1999.0345.
Fairbairn N. No such thing as just a nail bed injury.
Pediatr Emerg Care. 2012;28(4):3635. doi:10.1097/
PEC.0b013e31824d9d57.
Fassler PR. Fingertip injuries: evaluation and treatment.
J Am Acad Orthop Surg. 1996;4(1):8492.
908
Gellman H. Fingertip-nail bed injuries in children:
current concepts and controversies of treatment.
J Craniofac Surg. 2009;20(4):10335. doi:10.1097/
SCS.0b013e3181abb1b5.
Krusche-Mandl I, Kttstorfer J, Thalhammer G, Aldrian S,
Erhart J, Platzer P. Seymour fractures: retrospective
analysis and therapeutic considerations. J Hand Surg
Am. 2013;38(2):25864.
Lalonde DH. Reconstruction of the hand with wide awake
surgery. Clin Plast Surg. 2011;38(4):7619.
Lalonde DH, Martin AL. Wide-awake exor tendon repair
and early tendon mobilization in zones 1 and
2. Hand Clin. 2013;29(2):20713. doi:10.1016/j.
hcl.2013.02.009.
R. Katz
Miranda BH, Vokshi I, Milroy CJ. Pediatric nailbed repair
study: nail replacement increases morbidity. Plast
Reconstr Surg. 2012;129(2):3946. doi:10.1097/
PRS.0b013e31823af1bb.
Roser SE, Gellman H. Comparison of nail bed repair
versus nail trephination for subungual hematomas in
children. J Hand Surg. 1999;24(6):116670.
Strauss EJ, Weil WM, Jordan C, Paksima N. A prospective,
randomized, controlled trial of 2-octylcyanoacrylate
versus suture repair for nail bed injuries. J Hand Surg.
2008;33(2):2503.
Tos P, Titolo P, Chirila NL, Catalano F, Artiaco S. Surgical
treatment of acute ngernail injuries. J Orthop Traumatol.
2012;13(2):5762. doi:10.1007/s10195-011-0161-z.
 Flexor Tendon Injuries
40
Steve K. Lee and Joseph J. Schreiber

Contents One- or Two-Stage Tendon Reconstruction . . . . . . 924

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 910 Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 925
Epidemiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 910 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 925
Pathoanatomy and Applied Anatomy . . . . . . . . . . . . 910
Assessment of Flexor Tendon Injuries . . . . . . . . . . . . 911
Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 911
Classication . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 911
Outcome Tools . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 912
Flexor Tendon Treatment Options . . . . . . . . . . . . . . . . 912
Nonoperative Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 912
Operative Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 912
Preoperative Planning . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 912
Surgical Procedure, Flexor Tendon Repair . . . . . . 913
Operative Technique . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 914
Zone I Repairs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 916
FPL Repair . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 916
Preferred Methods: Tendon Repair . . . . . . . . . . . . . . . 917
Rehabilitation After Repair . . . . . . . . . . . . . . . . . . . . . . . . 918
Preferred Methods: Postoperative Protocol . . . . . . 919
Results . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 920
Complications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 924

S.K. Lee (*) J.J. Schreiber

Hospital for Special Surgery, New York, NY, USA
e-mail: steve.kichul.lee@gmail.com; schreiberj@hss.edu

# Springer Science+Business Media New York 2015 909

J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_41
910
Abstract
Pediatric exor tendon injuries can be a chal-
lenge for the operating surgeon to manage
effectively. The pediatric population presents
with several unique considerations. Their
small size can make the technical aspects of
exor tendon repair more difcult, while their
young age can make compliance with diagnos-
tic exams and postoperative rehabilitation
less reliable. While this population does
present a unique set of challenges, successful
results can be reliably obtained. The pediatric
patient has several advantages over their adult
counterpart, as they tend to heal more rapidly,
have a superior local blood supply, and tend to
have a greater ability to remodel scars and
adhesions.
Introduction
Successful treatment of exor tendon injuries of
the hand can be one of the more vexing problems
that a hand surgeon faces in their practice.
Achieving an acceptable result in a compliant
adult patient is a challenge in and of itself.
Achieving such results in a child, especially one
less than 5 years of age, is an even more difcult
task. As is the case in other realms of pediatric
surgery, children are not merely smaller versions
of adults. Indeed, the anatomy is of a relatively
smaller size, but there is more to the issue
than just the physical differences, as the
pediatric patient presents a unique set of chal-
lenges regarding the preoperative diagnosis,
intraoperative techniques, and postoperative reha-
bilitation protocols.
Epidemiology
In regard to exor tendon injuries, a child is
dened by most authors as being under 15 years
of age (Al-Qattan 2013). Injuries to the exor
tendons are more commonly observed in males
and become progressively more rare with
S.K. Lee and J.J. Schreiber
decreasing age (Nietosvaara et al. 2007; Friedrich
and Baumel 2003). In Finland, Nietosvaara
et al. (2007) reported that the annual incidence
of exor tendon injury in the pediatric population
is 3.6 per 100,000. Flexor tendon injuries have
been described in newborns, with the mechanism
being a laceration during childbirth via Caesarean
section (Kavouksorian and Noone 1982). A more
frequently encountered mechanism is a traumatic
laceration caused by a knife or by a fall onto a
sharp object, such as broken glass (Havenhill and
Birnie 2005). Verdan Zone II lacerations are the
most common location. Outbreaks are also
known to occur, for instance, during Halloween
activities such as pumpkin carving that is
commonly performed in North America and in
Europe (Al-Qattan 2013).
Pathoanatomy and Applied Anatomy
From an anatomy standpoint, the main consider-
ation for exor tendon lacerations in children that
differs from those in an adult counterpart is the
issue of size. This results in an increased level of
technical difculty in applying the same repair
techniques to pediatric patients that have been
shown to be of superior quality in the adult pop-
ulation. One of the repairs that have recently
gained traction in the adult literature is the
cross-locked cruciateinterlocking horizontal
mattress repair (Croog et al. 2007; Lee et al.
2010; Vigler et al. 2008), which has been
shown to have excellent biomechanical charac-
teristics. However, there is simply not enough
room in the very young patients exor tendon
for such a repair. Al-Qattan (2013) has shown
that in children less than 2 years of age, the exor
digitorum profundus (FDP) is 23 mm wide
and 0.51 mm thick. These small sizes of
tendons present technical issues surgically.
Regarding pulley size and conguration, Flake
et al. (1990) studied cadaveric hand specimens
ranging in age from newborn to 15 years of age.
The authors showed that the size and locations of
the pulleys of the exor tendon sheath in the
growing hand closely correlate and are
40 Flexor Tendon Injuries
proportional with the anatomic characteristics of
the adult hand.
Assessment of Flexor Tendon Injuries
The assessment of exor tendon injuries is more
difcult than in adults, especially in the youngest
age group (<5 years). Late presentation tends to
be more common in children than in the adult
counterpart (Fitoussi et al. 1999). Kato et al.
(2002) reported that the mean time from injury
to presentation was 23 days in the child that is less
than 6 years of age. When examining the child, if
they are too young to comply with specic direc-
tions, much can be ascertained either by directly
observing the cascade of the digits or by atten-
tively examining the use of the digits while they
explore or play in the examination room. For this
reason, access to toys or stickers may be of value
when assessing the possibility of a exor tendon
injury. Observing the location of lacerations and
knowing the mechanism of injury aids in determin-
ing the likelihood that the child has suffered a
exor tendon injury. Compression of the forearm
can be used to generate passive exion excursion to
aid in the diagnosis of tendinous injury. Compres-
sion over the exor carpi radialis (FCR) generates
thumb exion, while compression ulnar to the FCR
causes digital exion. Wrist tenodesis may be used
as well. With wrist extension, digits should ex in
an anatomical cascade; if a digit or more lacking in
normal cascade, this is evidence of injury.
The most common associated injuries are dig-
ital nerve and artery damage. Laceration to both
neurovascular bundles is fortunately a rare asso-
ciation, but signs of a dysvascular nger would
result in the injury being a true surgical emer-
gency. Injury to a single digital neurovascular
bundle in association with a exor tendon lacer-
ation is very possible and requires exploration
of both bundles during tendon repair to address
injuries as necessary. When assessing the possi-
bility of a digital nerve or vascular injury,
particular attention is paid to skin color, temper-
ature, and capillary rell time. These signs,
along with observing for the presence of
911
anhidrosis, can give clues into a possible associ-
ated neurovascular disruption. Another sign that
can be used to diagnose a digital nerve injury is
the immersion test (Havenhill and Birnie 2005).
For this exam, the hand is immersed in room
temperature water until wrinkling of the skin
commences. Given that denervated skin does
not wrinkle, the absence of wrinkling in the
digit of interest can indicate a possible digital
nerve disruption.
Imaging
As with other trauma, standard radiographs
should be obtained when there is an open injury
and decreased movement in order to rule out a
fracture, dislocation, or foreign body. It should be
noted that while visualization of a foreign body
conrms its existence, the inverse is not true, as
lack of visualization does not rule out its presence.
Advanced imaging is generally not indicated,
as ultrasound has limited diagnostic accuracy
for tendon lacerations and magnetic resonance
imaging requires a general anesthetic in young
children (Havenhill and Birnie 2005).
Classification
Classication systems available for exor tendon
injuries include categorizing the injuries by ana-
tomic location, patient age, or digital damage. The
most commonly used classication system is
based on anatomic region of injury (Zones IV)
as originally described by Verdan (1964). Zone I
is dened as the zone distal to the exor digitorum
supercialis (FDS) insertion. By denition, Zone
1 injuries only involve the exor digitorum
profundus (FDP) tendon. Zone II extends from
the FDS insertion distally to the proximal aspect
of the A1 pulley. Zone III comprises the area
of the lumbrical origin and extends from the
proximal aspect of the pulley system to the distal
end of the transverse carpal ligament. Zone IV
denotes the exor tendons within the carpal tun-
nel. Finally, Zone V extends from proximal to the
912
transverse carpal ligament to the myotendinous
junction in the forearm.
Another classication system relevant to pedi-
atric exor tendon injuries is by the childs age.
Most authors split pediatric patients into three
groups based on age: <5 years, 610 years, and
1115 years (Al-Qattan 2013). Treatment princi-
ples, regarding both intraoperative techniques and
postoperative rehabilitation, are typically tailored
to the age of the child. The youngest group is
oftentimes the most challenging for both diagno-
sis and postoperative management.
There is also a classication system based
upon overall associated digital damage (Merle
and Dautel 1995). This classication was later
correlated with functional outcomes after exor
tendon repair (Courvoisier et al. 2009). Grade
1 represents a moderately scarred digit without
associated neurovascular injury. Grade 2 is a
severely scarred digit with only one neurovascular
bundle intact, with or without injuries to the
pulley system or an associated joint contracture.
Grade 3 represents a severely scarred digit with
either proximal vascular disruption or disruption
of both digital neurovascular bundles.
Outcome Tools
The usual outcome tools described in the literature
utilize varying measurements for assessing range
of motion and are shown in Tables 1 and 2. The
StricklandGlogovac (1980) score utilizes com-
bined degrees of exion at the proximal
interphalangeal (PIP) and distal interphalangeal
(DIP) joints, subtracts the extension lag, and
calculates motion and outcomes as a percentage
of a full and normal arc of motion. The total active
motion (TAM) score (American Society for Sur-
gery of the Hand 2000) calculates outcomes as a
percentage of normal range of motion and takes
into consideration metacarpophalangeal (MP),
PIP, and DIP joint active motion. The DIP joint
range of motion score (Moiemen and Elliot 2000)
focuses on active, isolated FDP motion. Finally,
Buck-Gramcko et al. (1976) devised a scoring
system comprised of three parts: (1) total active
motion at the MP, PIP, and DIP joints,
S.K. Lee and J.J. Schreiber
(2) extension decit, and (3) nail to distal palmar
crease distance. The outcome classication is
based on a composite result of a graded point
system for each individual component.
Flexor Tendon Treatment Options
Nonoperative Treatment
Unless the child is not medically t for surgery, or
unless the wound is too contaminated for tendon
repair, most of these injuries require surgery to repair
or reconstruct the tendon injury (Table 3). If a tendon
is left unrepaired, not only will the digit not actively
ex at the involved joints, but proximal muscle
atrophy and retardation of bone growth may occur
as sequelae (Bora 1970; Cunningham et al. 1985). If
there is a chronic FDP injury where primary repair
will not be possible and the FDS has adequate
strength to ex the PIP joint, then allowing the
patient to have an FDS nger is a reasonable option.
Operative Treatment
Surgery is indicated in the vast majority of exor
tendon laceration cases. If the child has incomplete
exion and pain during attempted exion, partial
laceration is possible and surgical exploration is
indicated. Partial laceration of <50 % does not
typically require repair, but warrants debridement
of the tendon aps to assure optimal gliding within
the pulley system. Conversely, laceration of >50 %
of the tendinous substance warrants surgical repair.
The typical scenario in Zone II is a complete
laceration of one or both of the exor tendons.
While the method of repair and the suture mate-
rial chosen is universally controversial, some
general themes do exist.
Preoperative Planning
In the preoperative planning stage, the surgeon
must be prepared for and the patient and parents
must be consented for all possibilities that could
be encountered (Table 4). The tendon may or
may not be amenable to primary repair,
40 Flexor Tendon Injuries 913

Table 1 Outcome tools for measurement of flexor tendon recovery

Score as a percent of normal
Joints Formula for percent of normal
Outcome tool measured motion Excellent Good Fair Poor
StricklandGlogovac PIP and Active PIP  DIP exion 85100 7084 5069 <50
score DIP extension lag  100/175
Total active motion MP, PIP, Active PIP  MP  DIP exion 100 >75 >50 <50
(TAM score) and DIP degrees from full  100/260
DIP ROM score DIP DIP range of motion  100/74 85100 7084 5069 049
(Moiemen-Elliot
2000)

Table 2 Buck-Gramcko scoring system for assessing Table 3 Flexor tendon injury, nonoperative management
digital range of motion
Flexor tendon injury nonoperative management
Units Points Indications Contraindications
Free nail palm crease distance 0.00.5 cm 6 Chronic FDP injury where FDS Repair can safely
measured from the free nail 0.61.5 cm 5 can ex the PIP joint be performed
margin to the distal palmar 1.62.5 cm 4 Medically too ill for surgery
crease
2.64.0 cm 3 Wound too contaminated for
4.16.0 cm 2 surgery
>6.0 cm 1
Total extension decit 0 30 3
(MPJ + PIP + DIP) 31 50 2
Table 4 Preoperative planning for pediatric flexor tendon
51 70 1
repair
>70 0
Modied total active motion >400 8 Flexor tendon injuries preoperative planning
(MPJ + 2  PIP + 3  DIP) >320 6 OR table: hand table >10 years, arm board 510 years,
OR table <5 years
>280 4
Position/positioning aids: supine
>240 2
Equipment: hand holder, tendon passer, microscope, and
>240 0
instruments
Classication
Tourniquet
Excellent 1617
Consented for tendon graft, silicone rod, tendon transfer
Very good 1415
Consented for nerve and artery repair versus
Good 1113 reconstruction
Fair 79
Poor 06

especially in cases when there is a delay from the
time of injury to surgical presentation. The
surgeon should account for the possibility of
requiring primary tendon grafting, a two-stage
tendon reconstruction, or a tendon transfer pro-
cedure. The surgeon should also plan for and
obtain consent for the possibility of repair or
reconstruction of a nerve or vascular structure.
Silicone tendon rods, an appropriate hand hold-
ing device, suture material, microscope, and
microscopic instruments should all be readily
available in the operating room.
Surgical Procedure, Flexor Tendon
Repair
The procedure is performed under general anes-
thesia with the patient in the supine position. A
hand table is typically used for a larger child,
while an arm board or just the operating room
table may be used for a smaller child. A small
arm tourniquet is applied and the extremity is
prepared and draped as per routine. Intravenous
antibiotics should be administered within 60 min
of the skin incision. Bruner incisions are preferred
914
over midaxial incisions, especially in infants and
children less than 5 years of age. Kavouksorian
and Noone (1982) reported that in younger patients,
midaxial incisions may migrate palmarward and
result in digital exion contractures. Both digital
neurovascular bundles should be surgically
explored. In contrast to the adult patient, it can be
very difcult to assure the integrity of neurovascular
structures based solely on the preoperative physical
examination of the pediatric patient.
The tendons are subsequently explored. Win-
dows can be made in the pulley system as neces-
sary. The site of pulley system windowing
should be made after careful assessment of the
probable repair location. The surgeon should not
open the pulley system in the same location for
every case. For instance, if the tendon laceration is
very distal, the A4 pulley may need to be
sacriced, but the A3 pulley may be preserved.
Venting of the entire A4 pulley, or part of the A2
pulley (up to 50 %), may be required and should
still allow for an acceptable result (Kwai Ben and
Elliot 1998; Tang 2013).
Operative Technique
Surgical repair techniques vary depending on the
author and center. The authors preferred suture
conguration repair method for both adults and
those older children with larger tendon sizes is the
cross-locked cruciateinterlocking horizontal
mattress (CLC-IHM) method. Both the core
component of the cross-locked cruciate and the
circumferential component of the interlocking
horizontal mattress have superior biomechanical
characteristics when compared to other com-
monly used methods (Figs. 1 and 2) (Croog
et al. 2007; Lee et al. 2010; Lee 2012; Vigler
et al. 2008). Unfortunately, there is not typically
enough space in the tendon for this repair tech-
nique in small children under the age of 10 years.
In children under 2 years of age, the FDP is
reported to measure 23 mm in width and
0.51 mm in thickness. For this age group, most
authors use a two-strand modied Kessler suture
conguration (Fig. 3) (Berndtsson and Ejeskar
1995; Elhassan et al. 2006; Fitoussi et al. 1999;
S.K. Lee and J.J. Schreiber
Grobbelaar and Hudson 1994; Herndon 1976;
Kato et al. 2002; OConnell et al. 1994). In children
over 5 years of age, a four-strand repair (Fig. 4) may
be feasible depending on the size of the child
(Elhassan et al. 2006; Navali and Rouhani 2008;
Nietosvaara et al. 2007). The rupture rates for four-
strand repairs have been shown to be lower than
those observed following two-strand repairs (Navali
and Rouhani 2008; Nietosvaara et al. 2007).
Al-Qattan advocates using three gure of 8
sutures for children regardless of age. He reported
higher tensile strength than the modied Kessler
conguration with this technique (Al-Qattan and
Al-Turaiki 2009). The chief disadvantage of the
gure of 8 repair is that the resulting repair site
is bulky and oftentimes too large to freely pass
underneath the exor sheath. Therefore, venting
of the exor pulley system is almost always
required. Most authors supplement the core suture
with a running circumferential suture, which is
still surgically practical in the pediatric patient
despite the small size of the tendons. Isolated
repair of the FDP, as compared to repair of both
the FDP and FDS tendons, is controversial in both
the adult and pediatric literature. Some authors
(Al-Qattan 2013; Fitoussi et al. 1999; Kato
et al. 2002) recommend only repairing the FDP
tendon injury, whereas other authors (Grobbelaar
and Hudson 1994) advocate repair of both. Pail-
lard and colleagues (Paillard et al. 2002) showed
that resection of one slip of the FDS tendon
decreased gliding resistance as compared to pul-
ley plasty in a cadaveric model. The subject of
repair of both slips of FDS tendon, versus one slip,
as compared to none is also controversial. Based
on the above study and clinical experience, it is
reasonable to repair one slip of FDS tendon
provided that the FDP tendon still glides easily
after repair. It is imperative that the tendons glide
with no interruption in the operating room. It is a
fallacy to think that the gliding will improve with
time and motion. It is safe to conclude that the
motion the surgeon achieves in the operating
room is the best that will be achieved
postoperatively.
In regard to the core suture purchase length, it
has been shown in adults that the optimal suture
purchase length biomechanically is between 7 and
40 Flexor Tendon Injuries 915

Fig. 1 Cross-locked cruciate repair (Lee et al. 2010). Core sutures placed in a locked fashion prevent trumpeting or
bulk formation at the repair site, theoretically decreasing friction and enhancing tendon gliding

Fig. 2 Interlocking horizontal mattress repair (Lee et al. 2010). Circumferential suture conguration

Fig. 3 Modied Kessler repair. Suture conguration for two-strand repair technique

Fig. 4 Strickland repair (Strickland 1995). Four-strand core suture repair technique
916
Fig. 5 Bunnell button
repair (Lee et al. 2011). A
two-strand Bunnell pullout
suture is passed through the
distal phalanx and tied over
a button on the dorsal nail
plate
10 mm (Lee et al. 2010; Tang et al. 2005). For
children, Navali and Rouhani (2008) recommend
the rule of a suture purchase length of 1.52.0
times the width of the tendon. In a series of
12 children with ages between 15 and 23 months,
Al-Qattan (2013) showed that the average width
of the FDP tendon in Zone II was 2.5
mm. Depending on patient size, the suture
purchase length should typically equal 3.54.5 mm
in this patient population. Navali and Rouhani
(2008) also advocated that this distance should not
exceed 5 mm.
Zone I Repairs
Elhassan et al. (2006) reported on the Bunnell
pullout repair (Bunnell 1948) tied over a button
on the nail plate (Fig. 5) in a series of 16 children.
One repair ruptured postoperatively, while the
remaining cases had a TAM (total active motion)
of 89 % of normal which is rated as excellent
(range 66100 %). Al-Qattan (2013) advocates
avoiding the possible complication of infection
and nail deformity associated with the dorsal
button technique by repairing the proximal tendon
to the short distal stump and reinforcing it with a
repair to the volar plate of the DIP at a point distal
to the joint. Special care is required to avoid
placing sutures deep into the physis, which may
result in a growth disturbance. Al-Qattan (2012)
reported on the use of this technique in a series of
10 children. There were no cases of ruptures or
growth plate injury, and all patients had excellent
results on the StricklandGlogovac (1980) score.
S.K. Lee and J.J. Schreiber
Using the MoiemenElliot (2000) scale, excellent
results were achieved in 5 children, good results in
1, and fair results in 4.
FPL Repair
Repair principles and outcomes are similar in the
exor pollicis longus (FPL) as compared to the
FDP tendons. Grobbelaar and Hudson (1995)
reported on nine children with FPL repairs
managed postoperatively with controlled mobi-
lization. Good to excellent results using Buck-
Gramcko scores were achieved in seven
patients, with no cases of tendon rupture
reported. Fitoussi et al. (2000) reported on
16 children with FPL repairs using a modied
Kessler suture repair technique. Buck-Gramcko
evaluation showed good to excellent results
in all but one patient who had a tendon rupture
post-repair. One third of the cases had at least
a 30 decrease in ROM as compared to the
normal side. The authors also showed that
digital nerve injury had no negative effect on
eventual outcome. Overall, the authors
had improved results with earlier mobilization.
Orhun et al. (1999) had excellent Buck-
Gramcko evaluations in 6 of 7 patients,
with one remaining good result in a series of
pediatric Zone II FPL repairs. In the setting
of a chronic FPL injury, good results have
been reported with a two-stage tendon transfer
utilizing a silicone tendon implant followed by a
transfer of the FDS of the ring nger (Yamamoto
and Fujita 2013).
40 Flexor Tendon Injuries
Table 5 Preferred management of Zone II injuries in
children under 5 years
Flexor tendon injuries surgical steps, Zone II <5 years
Bruner incisions
Identify location of tendon repair
Open appropriate area of pulleys
Avoid handling tendon surface
Hold tendon ends with 25 gauge needles
For <5 years, modied Kessler (Fig. 3) with 4-0
Fiberwire
Suture purchase of core suture: 4 mm
Interlocking horizontal mattress circumferential suture
(Fig. 2) with 6-0 or 7-0 Prolene
Circumferential suture with 6-0 or 7-0 Prolene
Repair 1 slip of FDS with gure of 8 suture, 4-0 to 6-0
Prolene
Vent pulleys as necessary
Prolene (Ethicon, Somerset, NJ, USA), Fiberwire (Arthrex,
Naples, FL, USA)
Table 6 Preferred management of Zone II injuries in
children under 610 years
Flexor tendon injuries surgical steps, Zone II,
610 years
Bruner incisions
Identify location of tendon repair
Open appropriate area of pulleys
Avoid handling tendon surface
Hold tendon ends with 25 gauge needles
Modied Kessler core suture with 4-0 Fiberwire
Possibly Strickland repair (Fig. 4) if anatomy permits
Suture purchase of core suture: 45 mm
Interlocking horizontal mattress suture with 6-0 Prolene
Repair 1 slip of FDS with gure of 8 suture, 4-0 to 6-0
Prolene
Vent pulleys as necessary
Preferred Methods: Tendon Repair
The authors preferred methods for managing
Zone II exor tendon injuries by age are shown
in Tables 5, 6, and 7. In all age groups, Bruner
incisions are utilized, the location of tendon repair
is identied, and appropriate windows are made in
the pulley system for access. In children <5
years, a modied Kessler (Fig. 3) suture with 4-0
Fiberwire (Arthrex, Naples, FL, USA) is
917
Table 7 Preferred management of Zone II injuries in
children >10 years (depending on size of patient)
Flexor tendon injuries surgical steps, Zone II, >10
years
Bruner incisions
Identify location of tendon repair
Open appropriate area of pulleys
Avoid handling tendon surface
Hold tendon ends with 25 gauge needles
Cross-locked cruciate (CLC) core suture (Fig. 1) with 3-0
to 4-0 Fiberwire
Modied Kessler (MK) and possibly Strickland if size
precludes CLC
Suture purchase of core suture: 6+ mm (up to 10 mm if
adult size)
Interlocking horizontal mattress circumferential suture
Circumferential suture with 6-0 Prolene
Repair 1 slip of FDS with gure of 8 suture, 4-0 to 6-0
Prolene
Vent pulleys as necessary
performed, with a purchase length determined by
tendon size. A circumferential suture with Prolene
(Ethicon, Somerset, NJ, USA) is then placed in an
interlocking horizontal mattress fashion (Fig. 2).
If the FDS tendons are involved, repair of one slip
is performed in a gure of 8 fashion. Venting
of the pulleys is performed where necessary to
optimize gliding. The core suture technique is
dependent upon the patients age and anatomy.
In children 610 years of age, a Strickland repair
(Fig. 4) is performed as permitted by the exor
tendon size, and core suture purchase length is
adjusted to tendon size. In older children (>10
years), a cross-locked cruciate core suture is pre-
ferred as size permits.
The authors preferred technique for managing
Zone I injuries is shown in Table 8. A Bunnell
stitch is performed with 2-0 to 4-0 Prolene
depending on the tendon size, which is passed
with a Keith needle through the distal phalanx
distal to the physis and tied over a button on the
nail plate (Fig. 5). Excessive pressure on the nail
plate is avoided, and the distal pulley system is
vented as needed to optimize gliding. If the patient
is over 10 years of age and the tendon size permits,
a volarly placed pullout suture is augmented with
a second suture on the dorsal aspect of the tendon
that is anchored to distal phalanx with two suture
918
Table 8 Preferred management of Zone I injuries in pedi-
atric patients
Flexor tendon injuries surgical steps, Zone I
Bruner incisions
Identify location of tendon repair
Open appropriate area of pulleys
Avoid handling tendon surface
Hold tendon ends with 25 gauge needles
Bunnell core suture (Fig. 5) with 2-0 to 4-0 Prolene
(based on size)
Anchor-button repair (Fig. 6) if >10 years and size
permits
Bunnell portion held over button on nail plate
Pass sutures distal to physis of distal phalanx
Avoid pressure of button on nail plate
Vent pulleys as necessary
anchors (Mitek Microx Quickanchors; DePuy
Mitek, Inc., Raynham, MA). This anchor-button
technique (Fig. 6) has been shown to closely
approximate native FDP tendonbone interface
ultimate strength, with a mean load to failure of
115 Newtons in a cadaveric model (Lee
et al. 2011).
Rehabilitation After Repair
Just as is the case with repair techniques, the
optimal postoperative rehabilitation protocol is
controversial. Teenagers can be treated similarly
to adults, with a protected mobilization or a
controlled active motion protocol. For children
between 5 and 10 years of age, most authors
recommend modied early mobilization with a
hand therapist with immobilization between ther-
apy sessions (Moehrlen et al. 2009). With the
stronger gure of 8 technique, Al-Qattan (2012)
allows protected mobilization without immobiliza-
tion between sessions. He reported no ruptures in a
series of 44 cases with Zone II repairs using this
postoperative protocol. Between sessions, patients
wear a dorsal block splint with the wrist exed 30
to preclude them from making a strong st.
The most difcult group to manage postopera-
tively is those children under 5 years of age.
Various techniques have been described in this
S.K. Lee and J.J. Schreiber
Table 9 Surgical pitfalls and prevention
Flexor tendon injuries potential pitfalls and preventions
Potential pitfall Pearls for prevention
Pitfall #1
Rupture 4-strand repair if anatomy allows
Immobilization with LAC if <5
years
Pitfall #2
Adhesions Do not immobilize in LAC >4 weeks
Do not excise FDS
Pitfall #3
Growth Avoid suture passage through physis
disturbance of P3
problematic age group to ensure compliance
with postoperative motion protocols. Some
authors advocate placing a pulp to palm suture to
ensure complete immobilization and compliance
(Kato et al. 2002; Kavouksorian and Noone 1982;
Zolotov 2008). Others have used an internal
profundus splint to prevent early rupture while
allowing early mobilization (Hester et al. 1984).
With this technique, an additional suture is placed
in the proximal aspect of the FDP tendon, which is
then passed through the pulley system and tied over
a dorsal button on the nail plate. The tension is
adjusted to produce slight bulking at the repair site.
During motion exercises, this prevents complete
extension and was effective in preventing ruptures
at the repair site in a series of 35 exor tendon
injuries. It does, however, require later removal.
Tuzuner et al. (2004) reported the use of botulinum
toxin in this age group to assist with compliance.
The authors injected the toxin in a targeted fashion
into the muscle belly specic to the repaired
tendon. Mean duration of effect was 6 weeks of
muscle relaxation during which passive exercises
were performed. Good to excellent results were
reported in their series of seven patients, and the
botulinum toxin appeared to be safe and effective
in this cohort of young children.
While various immobilization techniques have
been reported, most authors simply use a long arm
cast (LAC) (Berndtsson and Ejeskar 1995; Hern-
don 1976; Kato et al. 2002; OConnell et al.
1994). LAC immobilization should not exceed
4 weeks, as results deteriorate with a prolonged
period of immobilization (OConnell et al. 1994).
40 Flexor Tendon Injuries
Fig. 6 Anchor-button
repair (Lee et al. 2011). A
two-part repair consisting of
a two-strand locking
Krackow suture along the
dorsal aspect of the tendon
that is xed with two
retrograde anchors. This is
supplemented with a
two-strand Bunnell suture
along the volar aspect of the
tendon that is tied over a
dorsal button
In the LAC, the elbow should be exed to 120
with the forearm in supination, the wrist and
ngers in exion, and the palm lled with soft
Webril (Covidien, Manseld, MA, USA). It is
imperative that with any postoperative splint or
cast, there is nothing rm placed palmarly under
the repaired nger(s). This would cause an
increased force challenge on the repair site
when the nger is actively exed. It should
be expected that all patients will move their
digits postoperatively to some degree, whether
it is due to noncompliance in the awake patient
or alternatively due to unintended movement
during sleep. By placing only loose soft dress-
ings in the palm, it is assured that there will
be minimal resistance to digital exion. How-
ever, after the cast is removed, there still remains
a risk for rupture in this age group with activities
such as crawling or unintended falls. Zolotov
(2008) recommends splint usage between therapy
sessions for an additional 2 weeks after cast
removal.
Preferred Methods: Postoperative
Protocol
The preferred postoperative management protocol
for children under 5 years of age is shown in
Table 10. A long arm cast is placed with the
elbow in 120 of exion and the forearm
919
Table 10 Preferred postoperative management protocol
following flexor tendon repair in children under 5 years
Flexor tendon injuries postoperative protocol, <5 years
Type of immobilization: long arm cast with elbow at
120 , supinated
LAC: wrist in 1020 of degrees exion
LAC: metacarpophalangeal joints in maximal exion
LAC: no resistance to digital exion; soft gauze uffs
in palm
Length of immobilization: 3.5 weeks
Rehab protocol: dorsal block splint (DBS) placed at
3.5 weeks
DBS: wrist in 1020 of exion
DBS: metacarpophalangeal joints in maximal exion
Passive and controlled active motion at 3.5 weeks
Restricted activity at 12 weeks (no climbing or sports)
Unrestricted activity at 6 months
LAC long arm cast, DBS dorsal block splint
supinated. The wrist is placed in slight exion
with the MCP joints in maximal exion to
decrease tension across the repair and minimize
the patients ability to generate exion power.
The palm is well padded to restrict resistance.
After 3.5 weeks, a dorsal block splint is placed
with similar wrist and MCP positioning. Passive
and controlled active motion is initiated in a
supervised manner. At 12 weeks, nonresistance
activities are allowed, and at 6 months postop-
eratively full activities are resumed without
limitations.
920
Table 11 Postoperative protocol, >5 years. Depending
on individual maturity level, <5 years protocol may be
used as needed
Flexor tendon injuries postoperative protocol, >5 years
Type of immobilization: dorsal block splint (DBS)
DBS: wrist in 1020 of exion
DBS: metacarpophalangeal joints in maximal exion
DBS: no resistance to digital exion
Length of protection in DBS: 68 weeks
Passive motion protocol initiated at 45 days
postoperatively
Controlled active motion at 45 weeks
Restricted activity at 12 weeks (no climbing or sports)
Unrestricted activity at 6 months
DBS dorsal block splint
Patients over 5 years of age that are deemed
sufciently mature to remain compliant are man-
aged as summarized in Table 11. The initial
immobilization for this group is a dorsal block
splint for 68 weeks with the wrist in slight ex-
ion and MCP joints in maximal exion. Passive
motion is initiated early, typically at postopera-
tive day 4 or 5. Controlled active motion is
performed in a supervised fashion for 45
weeks, followed by initiation of restricted
activity at 12 weeks, and resumption of unrestrictive
activities at 6 months.
Results
Postoperative results seen in the pediatric popula-
tion tend to compare favorably with those of
adults. This may be due to the fact that children
in general tend to heal more rapidly, have a supe-
rior local blood supply, and tend to have a greater
ability to remodel adhesions and scars (Lundborg
1979). Some have also speculated that continued
longitudinal digital growth in children may result
in traction on the tendon and rupture of tendinous
adhesions, thereby facilitating smooth gliding
through the bro-osseous tunnel (Gilbert and
Masquelet 1998).
The number of clinical outcomes studies in the
literature involving children is far fewer than
those for adults. Some of the outcome reports
S.K. Lee and J.J. Schreiber
combine Zone I and II results together, some
separate by zone of injury, and many use different
measurement scores for assessing and evaluating
outcomes. This lack of homogeneity can make
direct comparisons of techniques difcult to inter-
pret. Despite this challenge, some themes do exist.
Larger outcome papers will be discussed in
chronological order and then themes stated. A
summary is provided in Table 12.
OConnell et al. (1994) reported outcomes of
95 digits with either a Zone I or Zone II exor
tendon repair. There was an average follow-up of
24 months (range 3144 months). Patients were
divided into cohorts by age for data analysis:
05 years, 610 years, and 1115 years.
Nonabsorbable suture was used for one of several
repair techniques; Kessler in 44 %, Bunnell in
22 %, modied Tajima in 26 %, and miscella-
neous in 8 % of patients. They found that all
FDP repairs in Zone I had excellent results. They
also found that Zone II repairs had comparable
function whether early passive motion was initi-
ated or whether the patient was immobilized post-
operatively for 34 weeks. However, the authors
found immobilization for more than 4 weeks led
to an appreciable deterioration in outcomes. No
signicant differences in outcomes based on
patient age were found. Flexor tendon repairs
that were associated with either a digital nerve
or volar plate laceration tended to have inferior
outcomes. With extended follow-up, the authors
also noted a modest improvement in motion
occurred after several years.
Grobbelaar and Hudson (1994) detailed repairs
on 53 exor tendon injuries in 38 children with a
mean age of 6.7 years. Repairs were performed
with a modied Kessler Mason Allen suture
(material not specied) and a 6-0 nylon running
circumferential suture. The authors used early
controlled motion as described by Kleinert post-
operatively. Eighty-two percent of patients
achieved either a good or excellent result. Repairs
of both the FDP and FDS tendons were better than
when an FDP repair alone was performed. Com-
plications included three tendon ruptures, all of
which had poor results. No patient in the cohort
required a subsequent tenolysis.
40 Flexor Tendon Injuries 921

Table 12 Summary of results for flexor tendon repair in pediatric patients

Method of Suture material
repair for for
Method of Suture material circumferential circumferential
Author Zone repair for core for core suture suture Results
Al-Qattan I 3 gure of 8 4-0 Prolene Running 5-0 Prolene No ruptures; 11 had
(2012) sutures circumferential excellent outcome,
suture 3 good, 8 fair
Al-Qattan I and 6 strands 5-0 Prolene Running 6-0 Prolene StricklandGlogovac:
(2011) II (3 separate circumferential excellent in 9, good in
gure of 8 suture used for 3. No ruptures. None
core sutures) children less needed tenolysis
than 2 years old
Al-Qattan II 6 strands Not specied Running 4-0 or 5-0 StricklandGlogovac:
(2011) (3 separate circumferential Prolene excellent in 46, good in
gure of 8 suture 8. No ruptures. None
core sutures) needed tenolysis
Elhassan I and Zone II : FDP Zone II: 4-0 Zone II: 6- Functional evaluation
et al. (2006) II repaired with braided suture running 0 monolament of all digits: good to
modied Zone I: 4-0 circumferential excellent
Kessler two monolament suture
or four strands suture
depending on
the size of
tendon and
two strands for
FDS
Zone I :
Bunnell
pullout repair
Moehrlen I and Modied 4-0 or 5-0 Running 5-0 or 6-0 Strickland
et al. (2009) II Kessler Prolene circumferential Prolene classication: 72.5 %
2 strands suture excellent, 20 % good,
7.5 % fair
OConnell, I and Several Nonabsorbable All FDP repairs in
Strickland II techniques: suture Zone I had excellent
et al. (1994) Kessler 44 %, results. Zone II repairs
Bunnell 22 %, with comparable
modied function if early
Tajima 26 %, passive motion or
miscellaneous immobilization for 34
8% weeks
Berndtsson II Modied 4-0 or 5-0 Sometimes 6-0 braided TAM with Strickland
and Ejeskar Kessler braided supplemented polyester formula was 77 %
(1995) polyester by running
circumferential
sutures
Grobbelaar II Modied Not specied Running 6-0 nylon 82 % good to excellent
and Hudson Kessler circumferential results
(1994) Mason-Allen suture
Kato II Modied 4-0 or 5-0 Running or 6-0, 7-0, or 8-0 Total active motion
et al. (2002) Kessler nylon interrupted nylon (TAM) was 155 ,
circumferential 89 %. 11 patients
suture excellent, 1 good result
(continued)
922 S.K. Lee and J.J. Schreiber

Table 12 (continued)
Method of Suture material
repair for for
Method of Suture material circumferential circumferential
Author Zone repair for core for core suture suture Results
Navali and II Two strands: 5-0 Prolene Both groups: 7-0 Prolene No ruptures in four-
Rouhani Tajima core locked running strand repairs, one in
(2008) Four strands: circumferential two strands; no
Tajima core sutures signicant difference
plus horizontal in active ROM
mattress between groups
(Strickland
repair)
Nietosvaara II Group 1: Group 1: 3-0 or Group 2: 6-0 Prolene Good and excellent
et al. (2007) modied four- 4-0 Tendoloop running results in all 45 ngers
or six-strand Group 2: 3-0 or circumferential (Buck-Gramcko), in
Lim core 4-0 Tycron suture 39 ngers (ASSH), in
suture Group 36 ngers (original
2: double Strickland), and in
Kessler 32 ngers (DIP ROM)

Berndtsson and Ejeskar (1995) reported results
of 46 Zone II FDP laceration repairs at a mean of
5.5 years postoperatively. Thirty-one of the
repairs were performed within 24 h while
15 were delayed. The FDP tendon was repaired
with a modied Kessler technique performed with
either a 4-0 or a 5-0 braided polyester, which was
sometimes supplemented by a running circum-
ferential braided polyester 6-0 suture. Associated
FDS tendon lacerations were repaired in all but
one patient; the technique utilized was not men-
tioned. Twenty-seven ngers followed a Kleinert
rehabilitation protocol while the remaining
19 were immobilized in plaster. Overall motion
was assessed with Strickland scores, which were
graded as good with an average of 77 % of normal
motion attained. Strickland scores tended to
correlate with the age of the child, as older chil-
dren tended to have more favorable outcomes.
Patients under the age of 4 attained 54 % of
normal motion, those aged 410 years attained
77 % of normal motion, while children over
10 years of age achieved 82 % of normal motion.
Although a power analysis was not reported, other
variables, such as postoperative regimen utilized,
associated injury to an FDS tendon or digital
nerve, delay in repair, or type of trauma, had no
effect on the nal results.
Kato et al. (2002) summarized results of
12 children younger than 6 years of age with
Zone II repairs, with a mean clinical follow-up
of 8 years. FDP tendons were repaired with a
modied Kessler technique using 4-0 or 5-0
nylon. Circumferential suture was performed
with either a running or interrupted suture using
a 6-0, 7-0, or 8-0 nylon. In nine patients with
concomitant FDP and FDS lacerations, the FDP
was repaired alone in seven while both the
FDP and FDS tendons were repaired in two. All
children were immobilized with a long arm cast
for 34 weeks postoperatively. In terms of com-
plications, there were no tendon ruptures reported,
two patients developed tendon adhesions, and one
patient required a subsequent tenolysis. The aver-
age StricklandGlogovac score was 89 % of
normal motion. Eleven patients had excellent
results, while the remaining patient had a good
result. Phalangeal length averaged 99 % of normal
at most recent follow-up. The authors concluded
that functional motion and near normal growth
can be attained in children less than 6 years of
age who undergo Zone II exor tendon repairs.
Elhassan et al. (2006) described clinical results
attained in 41 ngers with either Zone I or Zone II
injuries in patients between the ages of 2 and
14 years of age. Zone I injuries were managed
with a Bunnell pullout repair performed with a 4-0
monolament suture. Zone II injuries were
repaired with a modied Kessler technique
performed with a 4-0 braided suture for the FDP
40 Flexor Tendon Injuries
tendon, which was performed with either two or
four strands depending on the size of the tendon.
A circumferential running suture with 6-0 mono-
lament was also performed. The FDS tendon
was repaired with two strands with a 4-0 braided
suture (exact method not specied). Mean clinical
follow-up duration was 42 months. The authors
separated the cohort into 2 age groups: 07 years
of age and 815 years of age. Approximately half
of the patients had early controlled motion, while
the other half were immobilized. Functional eval-
uation with total active motion assessment dem-
onstrated either good or excellent results in all
patients. Zone I repairs had superior results as
compared to those performed in Zone II. Associ-
ated nerve injuries were a negative prognostic
factor, as isolated tendon repairs performed better
than if an associated nerve repair was performed.
Neither the patients age nor the postoperative
protocol had an inuence on the nal range of
motion achieved.
Nietosvaara et al. (2007) studied a series of
45 ngers with exor tendon injuries in children
15 years of age or younger with a mean follow-up
of 38 months. Repairs were performed with a
modied four- or six-strand Lim core suture (3-0
or 4-0 Tendoloop, Braun, Tuttlingen, Germany) in
14 ngers and with a double Kessler (3-0 or 4-0
Tycron, Tyco Health Care, Norwalk, CT) in six
ngers, all followed with a running 6-0 Prolene
circumferential suture. There were no ruptures in
the cohort of 33 patients with multi-strand
repairs who were managed with a postoperative
active motion protocol. Three of nine patients
who had a two-strand core suture repair had
a subsequent rupture within 1 month of the
index operation. The mean ROM of DIP joint
in Zones I and II as a percentage of the contra-
lateral normal side was 60 %, as compared
to 98 % for Zone III and V injuries. There was
a wide discrepancy in functional outcome
scores: good and excellent results were mea-
sured in 45 ngers using the Buck-Gramcko
score, 39 using the ASSH TAM score, 31 using
the ROM of DIP joints, and 38 with the
StricklandGlogovac score.
Navali and Rouhani (2008) compared 32 Zone
II exor tendon repairs in patients who were under
923
4 years of age, which were performed with either a
four-strand or two-strand repair. The two-strand
repairs were performed with a Tajima core suture
with 5-0 Prolene. The four-strand repairs were
performed with a Tajima core plus a horizontal
mattress suture with 5-0 Prolene (Strickland
repair). Both had a running locking circumferen-
tial suture with 7-0 Prolene. Mean follow-up was
11 months. There was no signicant difference in
active ROM (156 in two-strand repairs and 158
in four-strand repairs). There were no ruptures
observed following a four-strand repair, whereas
one patient with a two-strand repair subsequently
ruptured 3 weeks postoperatively.
Moehrlen et al. (2009) described 49 exor ten-
don repairs performed with a two-strand modied
Kessler technique using 4-0 or 5-0 Prolene. A
running circumferential suture was also placed
with either 5-0 or 6-0 Prolene. All patients had
immediate postoperative mobilization with the
protocol tailored to their age. No signicant dif-
ferences were found between the age groups stud-
ied. Despite only a two-strand repair and early
motion protocol, there were no subsequent
ruptures in their series. Outcomes using the
StricklandGlogovac score were 72.5 % excel-
lent, 20 % good, and 7.5 % fair. Median total
active motion was 92.6 %. Based on the results
attained, the authors advocated that early motion
could safely be initiated utilizing their age-
specic rehabilitation protocol.
Al-Qattan (2011a) reported on a six-strand
repair (three separate gure of 8 core sutures)
with 5-0 Prolene and running epitendinous suture
with 6-0 Prolene used in 12 ngers in children less
than 2 years of age. While only the FDP tendon
was repaired, the author stated that the six-strand
repair was bulky and subsequently required pulley
venting proximal to the repair in this young
cohort. Postoperatively, all patients were
immobilized for 3.5 weeks. In this series, there
were no subsequent ruptures, and no patient
required a tenolysis. The StricklandGlogovac
scores were excellent in nine patients and good
in the remaining three patients.
In a series of 54 Zone II exor tendon lacera-
tions occurring in children between the ages of
5 and 10 years, Al-Qattan (2011b) reported
924
outcomes following repairs with a six-strand core
suture technique (three separate gure of 8
sutures). A supplemental running epitendinous
suture was also performed. All patients initiated
early active mobilization immediately with no
splinting. After a mean follow-up period of
13 months, there were no subsequent ruptures,
and nal outcomes using the Strickland-
Glogovac criteria were excellent in 46 ngers
(85 %) and good in the remaining eight ngers
(15 %).
Al-Qattan (2012) reported on 22 digits in chil-
dren between 5 and 10 years of age who had a
Zone I FDP injury. The repair technique used was
performed with three separate gure of 8 sutures
using 4-0 Prolene, with a running circumferential
suture with 5-0 Prolene. All patients were managed
with an early active mobilization protocol. There
were no subsequent ruptures reported. All patients
had excellent outcomes using the Strickland and
Glogovac criteria which assess active motion at
both interphalangeal joints. However, using the
Moiemen and Elliot criteria on the basis of net
active motion of the DIP joint, 11 patients had
excellent outcomes, 3 had good outcomes, and
8 outcomes were graded as fair. Given the results,
the author advocates that the strength of this repair
allows early motion in this cohort.
Despite the disparities in the literature, some
common overall themes do exist. For the youngest
age group (<5 years of age), long arm cast immo-
bilization is most likely the safest postoperative
protocol, but the time of use should not exceed
4 weeks. Utilizing four or more strands for the
core suture is protective against early rupture and
should be used if the size of the patients anatomy
permits. The FDS may or may not be repaired
depending on size and gliding constraints
(Table 12).
Complications
Possible complications include postoperative ten-
don rupture, scarring, and growth disturbance.
Fitoussi et al. (1999) reported that postoperative
ruptures occurred at a higher rate in younger
S.K. Lee and J.J. Schreiber
Table 13 Management of complications
Flexor tendon injuries
Possible complication Management
Tendon rupture Re-repair
Scarring, adhesion Therapy, tenolysis (after
6 months)
Growth disturbance None
patients. In a series of 58 repairs in pediatric
patients under 15 years of age, 5 ruptures
occurred, resulting in a 9 % rupture rate. Four of
the ve ruptures that occurred were in patients
who were less than 5 years of age. As would be
expected, two-strand repairs have been shown to
have a higher rupture rate as compared to four-
strand repairs (Navali and Rouhani 2008;
Nietosvaara et al. 2007). Fortunately, adhesion
formation and joint stiffness are less commonly
encountered in the pediatric population as com-
pared to in adults. Al-Qattan (2013) states that
worse scarring occurs when the FDS is excised
and therefore recommends against excising
it. Another possible complication is growth arrest
of the distal phalanx after reinserting the FDP into
the distal phalanx (Yamazaki et al. 2011). To
avoid this complication, it is important to make
sure the suture is passed distal to the growth plate
(Table 13).
One- or Two-Stage Tendon
Reconstruction
When treatment is delayed and tendon ends can-
not be primarily repaired, then a one- or two-stage
tendon reconstruction may be employed, as is
performed in the adult patient. Single-stage
grafting is usually performed when the pulleys
(particularly A2 and A4) are intact and there is
full or near full passive range of motion of the PIP
and DIP joints. Alternatively, a two-stage recon-
struction is used if these criteria are not met.
Bora (1970) reported 17 of 20 cases being
successful with a single-stage grafting technique.
Yamazaki et al. (2011) presented seven cases of
single-stage tendon grafting. Postoperatively, a
modied Kleinert protocol of active extension
40 Flexor Tendon Injuries
and passive exion was used. The mean active
motion of the DIP joints was 49 , while the mean
motion of the PIP joints was 106 . Excellent
results were achieved in ve, good results in
one, and a fair result in one.
Courvoisier et al. (2009) reported outcomes on
20 children who had delayed management of
exor tendon injuries with a tendon graft. Eight
patients were managed with single-stage grafting
after a mean of 7.5 months, while 12 patients were
managed with two-stage grafting after a mean of
9 months. In their two-stage reconstruction cohort,
pulleys were usually reconstructed with a slip of
the FDS tendon left attached to its insertion site and
then passed through holes made in the ever pre-
sent rim of the remaining pulleys. The mean
Strickland index was 70 for single-stage grafting
and 66 following two-stage reconstruction. Given
the equivalent functional outcome scores between
the two groups, the authors advocate for single-
stage reconstruction when pulleys are intact and the
range of motion is complete. The most determinant
factor for functional outcome in this series was the
severity index of the initial injury according to the
Merle and Dautel grading system (1995).
Valenti and Gilbert (2000) reported on a series
of 26 children with two-stage tendon reconstruc-
tion. Outcomes were assessed with the Strickland
scoring system and results were excellent in 21 %,
good in 52 %, fair in 13 %, and poor in 13 %.
Older children (1015 years) tended to have supe-
rior results in this series, with 82 % of these
patients having good to excellent results, as com-
pared to 53 % good to excellent results attained in
younger children (13 years).
Summary
Successful treatment of exor tendon injuries in
the pediatric population can be a challenge to the
treating surgeon. While treatment principles and
outcomes in the older child (>10 years) more
closely approximate the adult counterpart, younger
children present a unique set of challenges and
considerations. Flexor tendon repair in the pediat-
ric patient can be a technically demanding
925
operation due to their relatively smaller anatomy.
Furthermore, this population presents with addi-
tional difculties throughout the course of treat-
ment. Flexor tendon injuries can be difcult to
ascertain through physical examination in the
young, uncooperative child, and delays in presen-
tation are not uncommon. These delays may result
in the need for grafting or more involved recon-
struction techniques. Once the tendon has been
appropriately reconstructed, the postoperative
rehabilitation course presents another challenge,
as an inability to fully cooperate contributes to the
increased incidence of secondary ruptures. With a
systematic approach to these injuries, along with
appropriate parent and patient counseling, the risk
of complications can be minimized while at the
same time maximizing the likelihood of a success-
ful outcome.
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 Extensor Tendon Injuries
41
Robert B. Carrigan

Contents Abstract
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 929 Hand injuries are prevalent in children of all
ages and have been estimated to occur at an
Anatomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 930
annual rate of 11.6 injuries per 1,000 popula-
Extensor Tendon Injuries . . . . . . . . . . . . . . . . . . . . . . . . . . 931 tion. Lacerations have been estimated to
Assessment of Injury . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 931 account for 1/3 of hand injuries presenting to
Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 932 the emergency room. Many of these hand lac-
Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 932 erations, may have tendon involvement. Fail-
Ultrasound . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 932
Magnetic Resonance Imaging . . . . . . . . . . . . . . . . . . . . . . . 932
ure to recognize, and appropriately treat these
Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 932 injuries may result in permanent disability.
Nonoperative Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 932 Knowledge of the anatomy of the extensor
Mallet Finger . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 932 mechanism and its correlation to hand function
Central Slip Injury . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 933
Operative Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 934
is critical in making an accurate diagnosis and
General Principles of Tendon Repair . . . . . . . . . . . . . . . . 934 treatment plan. Children present signicant
challenges in obtaining a thorough physical
Specic Injuries . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 934
Mallet Finger . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 934 examination. Therefore, the use of passive
Chronic Mallet Fingers . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 935 tests and critical observation skills may aid
Central Slip Injury . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 937 in the determination of the extent of injury.
Secondary Reconstruction . . . . . . . . . . . . . . . . . . . . . . . . . . . 937
Surgical treatment of extensor tendon injuries
Postoperative Treatment and Rehabilitation . . . . . . . . . 938
Outcomes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 939 in children mirrors those in adults; however,
rehabilitation techniques differ signicantly.
Pitfalls and Prevention of Complications . . . . . . . . . 939
Injury Recognition . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 939 Careful attention to detail can allow for near
Repair Rupture . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 939 complete recovery from these challenging
Infection . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 940 injuries.
Adhesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 940
Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 940
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 941 Introduction

Children are inquisitive by nature. They are con-

R.B. Carrigan sistently nding new ways to interact with their
Division of Orthopaedic Surgery, Childrens Hospital
environment. The hand of a child is a tool used for
of Philadelphia, Perelman School of Medicine at the
University of Pennsylvania, Philadelphia, PA, USA exploration and interaction. This may lead to the
e-mail: carriganr@email.chop.edu childs hand being injured as a result of these
# Springer Science+Business Media New York 2015 929
J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_42
930
interactions. Hand injuries in children have been
estimated to occur at an annual rate of 11.6 inju-
ries per 1,000 population (Shah et al. 2012).
Lacerations have been estimated to account for
1/3 of hand injuries presenting to the emergency
room (Bhende et al. 1993; Fetter-Zarzeka and
Joseph 2002). Within these hand lacerations,
many have tendon involvement. Failure to recog-
nize and appropriately treat these injuries may
result in permanent disability.
Anatomy
The anatomy of the extensor mechanism has been
well described (Wehb 1995). It is much more
sophisticated than that of the exor tendon
(Fig. 1). Extensor tendons can be grouped into
three categories: wrist extensors, digital exten-
sors, and thumb extensors and abductors.
Wrist extensors include the extensor carpi
ulnaris (ECU), the extensor carpi radialis brevis
(ECRB), and the extensor carpi radialis longus
(ECRL). Each of these extensors originates on
the lateral humerus and is responsible for wrist
extension and, in the case of the ECU, ulnar
Fig. 1 Diagram of the extensor mechanism (this was taken from Hand Clinics, if Springer has one please substitute)
R.B. Carrigan
deviation. The ECRL and ECRB are innervated
by the radial nerve and insert on the index and
middle nger metacarpals, respectively. The ECU
is innervated by the posterior interosseous nerve
(PIN) and inserts on the base of the small nger
metacarpal.
Digital extensors include the extensor
digitorum communis (EDC), the extensor indicis
proprius (EIP), and the extensor digiti minimi
(EDQ). The EDC has one common origin on the
lateral condyle of the humerus and several slips
(index, middle, ring, and small) which insert on
the extensor hood of each digit at the metacarpo-
phalangeal (MCP) joint. The EIP originates on
the lateral border of the ulna and inserts distally
on the extensor hood of the index nger just ulnar
to the insertion of the EDC insertion. The EDQ
originates on the proximal ulna and inserts on the
extensor hood of the small nger just ulnar to the
insertion of the EDC. Juncturae tendinum are
brous connections between the digital extensors
at the level of the metacarpals. These interconnec-
tions help to evenly distribute forces between the
ngers during digital extension. The digital exten-
sors are innervated by the posterior interosseous
nerve.
41 Extensor Tendon Injuries
Table 1 Extensor tendon compartments of the wrist
1. Abductor pollicis longus (APL) and extensor pollicis
brevis (EPB)
2. Extensor carpi radialis brevis (ECRB) and extensor
carpi radialis longus (ECRL)
3. Extensor pollicis longus (EPL)
4. Extensor digitorum communis (EDC) and extensor
indicis proprius (EIP)
5. Extensor digiti minimi (EDQ)
6. Extensor carpi ulnaris (ECU)
Thumb extensors include the extensor pollicis
longus (EPL), the extensor pollicis brevis (EPB),
and the abductor pollicis longus (APL). The EPL
originates on the interosseous membrane and ulna
and inserts on the distal phalanx of the thumb. It
extends the thumb at the interphalangeal (IP) and
metacarpal-phalangeal (MCP) joints, as well as
mildly adducts the thumb. The EPB originates
on the radius and interosseous membrane and
inserts on the proximal phalanx of the thumb. It
extends and abducts the thumb. The APL origi-
nates on the ulna and interosseous membrane and
inserts on the base of the thumb metacarpal. It
abducts the thumb. The thumb extensors are
innervated by the posterior interosseous nerve.
Each of the extensor tendons passes beneath
the extensor retinaculum at the level of the distal
radius and ulna. The extensor retinaculum is a
strong brous band that acts as the only pulley
of the extensor system. The extensor retinaculum
is septated into six compartments in which the
extensor tendons run (Table 1).
The digital extensor apparatus is a very intri-
cate and elegant system of interconnecting
extensor tendons, interossei, and lumbrical mus-
cles, which allow for the controlled exion and
extension of the digits. The digital extensor ten-
dons insert on the extensor hood at the level of the
MCP joint. Transverse bers form the sagittal
bands which stabilize and centralize the extensor
tendons over the MCP joint. The volar and dorsal
interossei originate on the metacarpal shafts and
insert on the dorsal hoods of the extensor mecha-
nism. They are responsible for adduction and
abduction of the digits, respectively, as well as
exion of the MCP joint and extension of the
distal interphalangeal (DIP) and proximal
931
interphalangeal (PIP) joints. The lumbrical muscles
insert on the extensor apparatus at the level of
the proximal phalanx. They are responsible for
MCP joint exion and PIP joint extension.
Extensor Tendon Injuries
Assessment of Injury
A complete and thorough history and physical
examination is an important rst step in appropri-
ately managing any upper extremity injury in
children. When obtaining a history, it is important
to note when the child was injured and what the
mechanism of injury was. Was the injury a sharp
laceration or was there a crush component? When
did the injury occur? Acute injuries may be
repaired primarily, whereas subacute or chronic
injuries may need to be reconstructed.
Examining an injured child may be quite dif-
cult, especially when the child is younger or badly
injured. Toddlers and infants cannot follow direc-
tions very well. Given this, the keys to a success-
ful examination are passive tests and
observations. Once the history has been obtained,
the examination begins with the inspection of the
limb. A lot of information can be obtained by
simply observing the child interacting with the
family. The skin should be inspected for any
lacerations or compromise. A normal digital rest-
ing cascade should be observed. Where possible,
active and passive extension of each digit should
be tested at each joint (DIP, PIP, and MCP).
Disruption of the insertion of the extensor
mechanism about the distal phalanx is a mallet
nger. Mallet ngers will present with a exion
deformity of the DIP joint and weakness of active
DIP extension. Injuries to the central slip insertion
on the middle phalanx may present with normal,
but painful, extension at the PIP joint. Elsons test
should be considered when a central slip injury is
suspected (Elson 1986). Elsons test places the
PIP joint in a 90 of exion, and the patient is
asked to extend the nger against resistance. A
positive test, for central slip disruption, causes the
DIP joint to go into rigid extension when the PIP
is extended (owing to the pull of the lateral bands
932
Table 2 Zones of extensor tendon injury
Zone I Disruption of the distal insertion
(mallet nger)
Zone II Disruption of the tendon over the
middle phalanx
Zone III Disruption over the PIP joint (central
slip/Boutonnire injury)
Zone IV Disruption over the proximal
phalanx
Zone V Disruption over the MCP joint
Zone VI Disruption over the metacarpal
Zone VII Disruption over the wrist
Zone VIII Disruption over the distal forearm
Zone IX Disruption at the muscle tendon
junction
on the distal terminal tendon). Sagittal bands
should be examined for subluxation. The MCP
joint should be actively extended and exed.
Difculty with active extension associated with a
clunk or snapping sensation over the hood of the
MCP joint should be suspicious for sagittal band
disruption. Radial-sided injury of the long nger
is the most commonly observed location for this
injury.
Extensor tendon injuries may be classied
based on their temporal relationship (acute versus
chronic), integrity of the soft tissue (open versus
closed), or anatomic location. Seven anatomic
zones have been described and are listed in
Table 2.
Imaging
Radiographs
Standard radiographs of the hand (three views
(AP/Lat/Oblique)) are useful in evaluating the
hand for bony injuries, as in the case of the bony
mallet nger.
Ultrasound
Ultrasound (U/S) can be useful to assess discon-
tinuity of a tendon in a child whose examination is
questionable. Ultrasound is a real-time study,
R.B. Carrigan
where the examination is performed while the
child is awake. There is no need for sedation and
the examination is performed in real time which
allows tendon movement to be visualized.
Magnetic Resonance Imaging
The use of magnetic resonance imaging (MRI)
allows for the visualization of tendon injury in
several different planes (sagittal, coronal, and
axial). MRI like ultrasound is useful in assessing
tendon injury when the examination is question-
able. It can help determine the degree of retraction
of a tendon, but unlike ultrasound younger chil-
dren will need sedation for completion of the
study.
Treatment
The treatment for extensor tendon injuries varies
depending on several factors including the mech-
anism of injury, age of the child, and temporal
relationship of the injury to treatment. A general
rule of thumb is that open injuries are treated
with open surgery and closed injuries are treated
closed with immobilization or splinting.
Nonoperative Treatment
Nonoperative management of extensor tendon
injuries in children is reserved for closed injuries
and for children who are old enough to comply
with the treatment protocols. In cases where
splinting may be appropriate in an older child, a
younger child may not be compliant enough and
operative intervention may be necessary.
Mallet Finger
Mallet nger is the disruption of the distal termi-
nal extensor tendon where it inserts on the distal
phalanx. These are typically closed injuries, most
often sustained via an axial load to the tip of the
nger, forcibly exing the distal phalanx
41 Extensor Tendon Injuries
disrupting the distal terminal extensor tendon.
Mallet ngers may injure the soft tissue only
or involve a portion of the distal phalanx
(bony mallet nger). In younger children injuries
to the distal phalanx are frequently open injuries
resulting from crushing mechanisms such as
getting a nger closed in a door. These crush
injuries may or may not involve the extensor
mechanism but careful consideration should
be made.
Treatment of closed soft tissue mallet ngers
consists of static extension splinting of the DIP
joint. The DIP extension splint is applied to the
DIP joint with the PIP joint left free (Bendre
et al. 2005). The splint is worn continuously
24 h a day for 6 weeks. The nger is then
reexamined for an extensor lag. If no lag is
present, the splint is worn for 2 more weeks only
at night. If no lag persists after a total of 8 weeks of
treatment, splinting is discontinued. If a lag is
present after 8 weeks, then splinting may be
continued for 46 more weeks (Fig. 2).
Fig. 2 (a) Injury
radiograph of bony mallet,
(b) clinical photo of mallet
nger after 6 weeks of static
extension splinting, (c)
radiograph of healing of
bony mallet nger 6 weeks,
(d) radiograph of healing
mallet nger 12 weeks
933
Failure to treat a mallet nger injury may result
in a swan neck deformity of the nger. Incompe-
tence of the distal insertion of the extensor tendon
causes a greater force transmission to the insertion
of the tendon along the central slip. This overpull
causes hyperextension of the PIP joint and
coupled with the exion deformity of the DIP
joint results in the swan neck deformity.
Central Slip Injury
Injuries to the PIP joint arising from blunt trauma
may cause rupture of the central slip insertion on
the dorsal lip of the middle phalanx. The injury
may be underwhelming at rst, often confused as
a sprain of the PIP joint. Loss of, or painful,
extension at the PIP joint should raise concern
for this injury. Elsons test should be used to test
the competence of the central slip insertion. If the
central slip is disrupted, the PIP joint should be
immobilized in extension for 6 weeks. The DIP
934
and MP joints should be left free, and active
motion about these joints should be encouraged
to prevent shortening of the oblique retinacular
ligament and volar migration of the lateral bands.
At 6 weeks the splint is removed and AROM
of the PIP joint is initiated. The patient is
followed until full range of motion of the digit
is obtained.
Failure to recognize and treat the acute central
slip injury may lead to the development of a
chronic boutonniere deformity. This is character-
ized by a exion posture of the PIP joint and
hyperextension posture of the DIP joint. Similar
to the case of an untreated mallet nger leading to
a swan neck deformity, the imbalance of force
transmission along the injured extensor mecha-
nism causes the deformity to progress. The
overpull along the terminal tendon insertion
causes hyperextension of the DIP joint and lateral
band volar migration causes a progressive PIP
exion deformity.
Operative Treatment
Once the decision to bring a patient to the operat-
ing room has been made, many variations in
anesthesia may be considered. The choice of
anesthesia depends on many factors including
patient age, medical comorbidities, and anesthesia
availability. More often, in younger children,
general anesthesia is preferred. For older children
regional anesthesia with sedation may be
considered.
The patient is positioned supine on the operat-
ing room (OR) table and is pulled over to the side
of the affected limb. A radiolucent hand table is
placed, with the midportion at the level of the
axilla. A padded tourniquet is applied to the
upper limb, and the table is turned 90 to place
the hand in the center of the operating room.
A variety of instruments can be used to help
with the surgical treatment of tendon injuries in
children. A basic hand tray should consist of small
tenotomy scissors, retractors, and self-retainers.
This basic set can be supplemented with tools
specically designed to aid in tendon retrieval
and repair. A malleable hand, tendon retriever,
R.B. Carrigan
and a pediatric feeding tube are tools that are
useful in tendon repair for both exor tendons
and extensor tendons.
General Principles of Tendon Repair
The surgical management of tendon injuries
in children mirrors that of adults. The injured
tendons should be appropriately exposed with
extensile incisions. The soft tissue and tendon
ends should be handled gently. Frayed ends of
the tendons should be debrided to stable margins
with minimal resection.
The type of suture repair in extensor tendon
injuries varies depending on several factors,
including the age of the child and location of the
injury. Almost uniformly braided nonabsorbable
sutures are preferred for extensor tendon repair.
On occasion, as in the case of pullout sutures, a
monolament suture may be considered. Tendon
injuries in younger children require smaller suture
such as 40 or 50, where older children can
accommodate larger suture such as 20 or 30.
Distal injuries where the tendons are at are
repaired with gure-of-eight sutures of braided
nonabsorbable suture. More proximal injuries,
where the tendon is more round, can be repaired
with core locking sutures and an epitendinous
suture where appropriate (Fig. 3). Many types of
core suture techniques as well as epitendinous
techniques have been described for repair of
exor tendon injuries; however, they are not as
well described in extensor tendon injuries. Still
the principles of extensor tendon repair and exor
tendon repair remain similar; a repair should be
strong enough to withstand forces during rehabil-
itation and tidy enough to glide smoothly, with the
latter more important in exor tendon injuries.
Specific Injuries
Mallet Finger
Specic instances exist where management of a
mallet nger via static extension splinting is not
appropriate. These include open lacerations,
displaced bony mallets, and children who cannot
41 Extensor Tendon Injuries
Fig. 3 (a) Clinical photograph of laceration of the dorsum of hand, (b) clinical photo of repaired extensor tendons to the
ring and small
tolerate extension splinting for 6 weeks. Open
lacerations of the DIP joint are treated like any
other tendon injury. The distal terminal tendon is
exposed by elevating the soft tissue off of the
extensor mechanism. The tendon ends are identi-
ed and repaired with one or two gure-of-eight
sutures of 40 braided nonabsorbable suture. A
trans-articular Kirschner (0.035 or 0.045 k) wire is
placed retrograde across the DIP joint to secure
the repair.
Bony mallet ngers are common occurrences.
External forces from an axial load or crush com-
ponent may cause disruption of the terminal
extensor tendon at the insertion on the distal pha-
lanx. The insertion of the extensor tendon is prox-
imal on the distal phalanx. A bony mallet nger in
the skeletally immature patients represents a
Salter-Harris III fracture of the distal phalanx
epiphysis. In rare cases crush injuries to the distal
phalanx may result in a dorsal dislocation of the
distal phalanx epiphysis (Waters and Benson
1993; Fig. 4).
Indications for surgical intervention of bony
mallet ngers include open injuries, displaced
fractures of the distal phalanx, and patients who
cannot comply with closed treatments. Extension
block pinning is a technique which is useful in the
reduction of the displaced bony mallet nger
935
(Hofmeister et al. 2003; Tetik and Gudemez
2002; Fig. 5). This is a percutaneous technique
in which a K-wire is placed dorsally along the
margin of the head of the middle phalanx, just
proximal to the displaced dorsal fracture of the
distal phalanx. The nger is exed and the K-wire
is inserted. This K-wire then blocks the distal
fragment from migrating proximally when the
distal phalanx is extended. When the distal pha-
lanx is extended, the fracture should reduce to the
blocked proximal fragment. A trans-articular
K-wire (0.035 or 0.045) is placed retrograde
across the DIP joint to secure the repair. Open
treatment of mallet ngers is not without peril;
complications including persistent extensor lag,
infection, and skin necrosis have been reported
as high as 41 % in some series (King et al. 2001).
Chronic Mallet Fingers
In children who either fail nonoperative treatment
of a mallet nger or present late, tenodermodesis
has been described as a technique to address
symptomatic chronic mallet ngers (Iselin
et al. 1977; Shin and Bae 2007). Typically
reserved for patients with extensor lags greater
that 45 , this technique involves an elliptical
936
Fig. 4 (a) Radiograph of closed epiphyseal dislocation of the distal phalanx, (b) intraoperative photo of open reduction of
epiphyseal dislocation (note the attachment of extensor mechanism)
Fig. 5 (ac) Diagram of dorsal blocking technique for
pinning of bony mallet nger
incision over the DIP joint where a 34 mm
section of skin and tendon is excised. Skin and
distal terminal tendon are then repaired with
R.B. Carrigan
interrupted full thickness (skin and tendon) sutures
with the DIP joint in extension. A trans-articular
K-wire is placed retrograde across the DIP joint for
4 weeks. Subsequently, the pin is removed and
therapy is initiated (Fig. 6). Kardestuncer, Bae,
and Waters reported on a series of 10 patients who
underwent this procedure and achieved improve-
ment of their extensor lag without the need of
further surgery or any effect on the remaining skel-
etal growth (Kardestuncer et al. 2008).
Although not reported for the treatment of
chronic mallet ngers in children, other tech-
niques have been reported in the treatment of
chronic mallet ngers in adults (Grundberg and
Reagan 1987; Houpt et al. 1993; Kanaya
et al. 2013). The Fowler tenotomy (release of
the central slip insertion) has been shown to
treat symptomatic chronic mallet ngers
in adults. The division of the central slip is
accomplished via a skin incision over the PIP
joint. Skin aps are developed and the extensor
tendon is divided transversely at the central slip
insertion, leaving the lateral bands in place.
The operation can be performed under local
anesthetic which allows the patient to actively
extend the nger to see if DIP extension is
improved. The skin is closed with interrupted
suture and the patient is referred to therapy.
Active range of motion exercises are promptly
initiated.
41 Extensor Tendon Injuries
Fig. 6 (a, b) Diagram of
tenodermodesis
Central Slip Injury
Lacerations of the central slip should be repaired
primarily. The skin is incised in a curvilinear fashion
incorporating the laceration. Skin aps are devel-
oped and the extensor tendon exposed. The tendon
ends are trimmed to healthy tissue and repaired with
a gure-of-eight suture of braided nonabsorbable
suture. In cases where the tendon insertion is
avulsed from the middle phalanx, a suture anchor
may be placed in the middle phalanx and the tendon
repaired directly to the bone (Fig. 7). The PIP joint
may be pinned in extension to protect the repair for
4 weeks. After pin removal therapy is initiated.
Secondary Reconstruction
In cases where direct or primary repair of the
tendon injury is not possible, secondary recon-
struction may be considered. Additionally, in
cases of signicant tendon loss or tendon shorten-
ing, primary repair may not be possible and alter-
native strategies may need consideration. Two
techniques to address tendon decits are tendon
grafting and tendon transfers.
937
Free grafting of tendon defects may be consid-
ered when large zones of injury are present and the
tendon ends cannot be approximated. Tendon
grafting is not typically performed as a primary
procedure but utilized as part of a staged recon-
structive plan. The choices of tendon grafts
include the palmaris longus and plantaris tendons.
Tendons should be harvested in a safe manner,
particularly in the case of the palmaris longus
tendon. Preoperatively, the patient should be tested
for the presence of the palmaris longus tendon,
which may be absent. Intraoperatively, the surgeon
should clearly visualize the tendon before
harvesting it, as not to confuse it with the median
nerve. Once the tendon graft has been harvested,
the graft should be sutured in place. This may be
done with an end-to-end repair or Pulvertaft weave
where appropriate.
In cases where free grafting of tendon defects is
not possible, one may consider tendon transfers
for restoration of the injured tendons function.
The classic treatment for reconstruction of the
ruptured EPL tendon that cannot be repaired
primarily is the EIP to EPL tendon transfer. The
technique is straightforward and offers good to
excellent results. The EPL tendon is exposed via
938 R.B. Carrigan

Fig. 7 (ad) Radiographs and clinical photographs of the repair of a central slip following resection of an
osteochondroma off of the dorsal lip of the middle phalanx

a dorsal incision along the wrist just distal to
Listers tubercle. The proximal portion of distal
extent of the EPL tendon is identied and freed of
any adhesions, until active IP thumb extension is
noted when the tendon stump is pulled. The EIP
tendon is harvested at its insertion along the MP
joint of the index nger via a transverse incision.
Through the incision made along Listers tuber-
cle, the EIP tendon is pulled proximally. The EIP
tendon is then rerouted along the thumb ray and
sutured to the distal portion of the EPL
tendon with the IP joint in full extension. The
EIP tendon may be sutured end to end or where
possible a Pulvertaft weave is preferred. This
strong repair may allow for early motion rehabil-
itative protocols to be initiated when appropriate
(Fig. 8).
Postoperative Treatment
and Rehabilitation
The vast majority of children treated operatively
for extensor tendon injuries are immobilized post-
operatively, most often in a cast. Children of all
ages are inherently unreliable, not because
they are bad, but because they are simply
children. Placement of a cast is the best
41 Extensor Tendon Injuries
Fig. 8 (a) Intraoperative photo of chronic tendon laceration of the ring and middle (12 weeks old), (b) photo of chronic
tendon laceration treated with EIP to EDC tendon transfer; note restoration of digital cascade
protection against tendon rupture, and further-
more, it keeps the surgical incision clean. It is
easier for a parent to manage than a removable
splint. The cast is placed from just past the tips
of the ngers to the forearm in older children.
Younger children (under the age of 4) are
placed in a long arm cast, as short arm casts
often fall off before they are scheduled to be
removed.
The patient is seen back in the ofce at 4 weeks
postoperatively and the cast is removed. The inci-
sions are inspected and referral to occupational
therapy is made. Gentle passive and active
range of motion exercises are initiated. Where
appropriate custom orthoplast splints are provided
for added protection. Strengthening is started once
a full range of motion is achieved. The patients are
followed until full function is restored, which
typically occurs 36 months from surgery.
Outcomes
The management of extensor tendon injuries in
children generally yields good to excellent results.
Fitoussi reported in 2007 a series of 50 patients
younger than 15 years of age treated for extensor
injuries. 98 % of the digits were rated as good or
excellent according to the total active motion
939
grading system, despite 22 % of the ngers
showing some extension lag or loss of exion at
the last follow-up (Fitoussi and Badina 2007).
Pitfalls and Prevention
of Complications
Injury Recognition
One of the difculties in treating childrens tendon
injuries is making an accurate diagnosis initially.
Young children are difcult to examine and cannot
comply with directions. In cases where there is
questionable evidence of tendon injury, it is
reasonable to consider exploration of the injured
area. One does not want to bring children unneces-
sarily to the operating room but the greater mistake
would be to have a tendon injury left untreated, as
this may lead to greater disability in the long term
and may lead to more surgery in the long run.
Repair Rupture
Tendon rupture is uncommon following extensor
tendon repair in children. If there is suspicion for
tendon rupture following surgery, the surgeon
may consider ultrasound or MRI to evaluate the
940 R.B. Carrigan

integrity of the repair. If radiographic assessment Callout boxes

is inconclusive and there is clinical concern for Mallet nger nonoperative treatment
rupture, exploration is warranted. Age appropriate for Age intolerant for
splinting splinting
Bony mallet with congruent Bony mallet with
Infection stable joint incongruent joint

Postoperative infection following tendon repair Central slip injury nonoperative treatment
may be encountered due to contamination at the Indications Contraindications
time of the initial injury. Sharp lacerations such as Closed injury Open injury
glass are more often clean and not sources Acute injury Chronic injury
of infection. Machine or blast injuries have a No secondary Presence of secondary
greater degree of contamination and greater zone deformity deformity
of injury and, in turn, a greater risk of infection.
Case checklist
Prevention of infection is the key in this setting.
Position supine with arm rotated in the center of the
Recognizing that primary tendon repair, at room
the initial sitting, may not be possible is important. Hand table
Repair may need to be delayed until the soft Hand instruments
tissue bed is cleared of contamination. In cases Padded arm tourniquet
where a postoperative infection is suspected, C-arm (uoroscopy)
prompt surgical debridement and initiation of anti- Hewson suture passer
biotic therapy may prevent tendon rupture and loss. Double-ended K-wires (0.035 and 0.045 diameter)
Suture anchors
Suture (braided nonabsorbable of varying size on a
Adhesions taper needle)
Tendon repair operative technique
Tendon adhesions are rarely encountered in children, Extensile incisions that incorporate the primary
particularly on the extensor surface. Children can laceration when present
achieve increases in range of motion up to Mallet ngers H- or T-shaped incision over the
dorsal aspect distal to interphalangeal joint
2 years following surgery. Tenolysis should be
Central slip insertion lazy S or curvilinear incision
reserved for those children who have signicant along the proximal and middle phalanges
decits in function and have plateaued in formal Proximal hand and wrist injuries longitudinal or
therapy. zigzag-type extensile incisions
Develop skin aps leaving as thick as possible
Expose tendon ends debriding nonviable tissue
Summary Approximate tendon ends
Repair tendon with braided nonabsorbable suture
Extensor tendon injuries while common in chil- Suture technique will vary based on the location of
repair
dren, present unique challenges for the treating
Distal atter tendons may be repaired with simple
surgeon. Prompt recognition of the injury, gure-of-eight sutures
coupled with timely surgery and diligent therapy Proximal more cord-like tendons may be repaired
can provide lasting results for the child. with core sutures of varying technique (Kessler, cruciate
locking, box stitch, etc.)
Callout boxes Repair is inspected for gaping
Mallet nger nonoperative treatment Where appropriate trans-articular K-wires are placed
Indications Contraindications to protect repair
Closed injury Open injury Skin is closed with absorbable suture
Acute injury Chronic injury Wounds are dressed and cast is applied
(continued) (continued)
41 Extensor Tendon Injuries
Tendon repair postoperative rehabilitation
Cast immobilization 4 weeks
Rehab initiated after cast is removed, splint
provided
Tendon gliding and scar massage
Active and passive ROM
Strengthening
Return to full activity by 8 weeks following cast
removal
References
Bendre AA, Hartigan BJ, Kalainov DM. Mallet nger.
J Am Acad Orthop Surg. 2005;13(5):33644.
Bhende MS, Dandrea LA, Davis HW. Hand injuries
in children presenting to a pediatric emergency
department. Ann Emerg Med. 1993;22(10):151923.
Elson RA. Rupture of the central slip of the extensor hood
of the nger. A test for early diagnosis. J Bone Joint
Surg Br. 1986;68:22931.
Fetter-Zarzeka A, Joseph MM. Hand and ngertip
injuries in children. Pediatr Emerg Care. 2002;18(5):
3415.
Fitoussi F, Badina A. Extensor tendon injuries in children.
J Pediatr Orthoped. 2007;27(8):8636.
Grundberg AB, Reagan DS. Central slip tenotomy for
chronic mallet nger deformity. J Hand Surg
Am. 1987;12:5457.
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Hofmeister EP, Mazurek MT, Shin AY, Bishop
AT. Extension block pinning for large mallet fractures.
J Hand Surg Am. 2003;28:4539.
Houpt P, Dijkstra R, StormVanLeeuwen JB. Fowlers
tenotomy for mallet deformity. J Hand Surg Br.
1993;18:499500.
Iselin F, Levame J, Godoy J. A simplied technique for
treating mallet ngers: tenodermodesis. J Hand Surg
Am. 1977;2:11821.
Kanaya K, Wada T, Yamashita T. The Thompson proce-
dure for chronic mallet nger deformity. J Hand Surg
Am. 2013;38(7):1295300.
Kardestuncer T, Bae DS, Waters PM. The results of tenoder-
modesis for severe chronic mallet nger deformity in
children. J Pediatr Orthoped. 2008;28(1):815.
King HJ, Shin SJ, Kang ES. Complications of operative
treatment for mallet fractures of the distal phalanx.
J Hand Surg Br. 2001;26(1):2831.
Shah SS, Rochette LM, Smith GA. Epidemiology of pedi-
atric hand injuries presenting to United States emer-
gency departments, 1990 to 2009. J Trauma Acute Care
Surg. 2012;72(6):168894.
Shin EK, Bae DS. Tenodermodesis for chronic mallet
nger deformities in children. Tech Hand Up Extrem
Surg. 2007;11(4):2625.
Tetik C, Gudemez E. Modication of the extension block
Kirschner wire technique for mallet fractures. Clin
Orthop. 2002;404:28490.
Waters PM, Benson LS. Dislocation of the distal phalanx
epiphysis in toddlers. J Hand Surg Am. 1993;18(4):5815.
Wehb MA. Anatomy of the extensor mechanism of the
hand and wrist. Hand Clin. 1995;11:3616.
 Amputations/Replantation
42
Daniel Calva, Harlan M. Starr, and James P. Higgins

Contents Abstract
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 943 Although amputations occur much less fre-
quently in children than in adults and adolescents,
Historical Perspective . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 944
amputation injuries in the pediatric population
Current Demographics and Epidemiology . . . . . . . 944 are associated with signicant physical disgure-
Applied Anatomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 945 ment, functional and psychosocial disability, and
a high nancial burden. Amputations of digits in
Amputation Assessment . . . . . . . . . . . . . . . . . . . . . . . . . . . . 945
children are a challenging problem for the pedi-
Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 947 atric hand surgeon due to the technical handling
Replantation of the Injured Part . . . . . . . . . . . . . . . . . . 947 of the fragile and diminutive anatomy. Obtaining
Indications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 947 an accurate history of the injury mechanism also
Contraindications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 948
Anesthesia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 949
provides the surgeon with information that may
Operative Techniques . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 949 affect surgical treatment.
Postoperative Care . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 952
Outcomes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 953
Preferred Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 954 Introduction
Complications and Management . . . . . . . . . . . . . . . . . . . . 955
When Replantation Is Not Possible: Reconstructive Most upper extremity amputations occur during
Options . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 957
the operation of industrial machines, farm equip-
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 957 ment, or power tools such as table saws, snow-
blowers, and lawn mowers. For this reason,
amputations occur much less frequently in chil-
dren than in adults and adolescents. In the pediat-
ric population, however, hand injuries represent a
signicant proportion of traumatic injuries, being
second in frequency only to injuries of the face.
Amputations of digits in children are a challeng-
ing problem for the pediatric hand surgeon due to
the technical handling of the fragile and diminu-
D. Calva (*) H.M. Starr J.P. Higgins tive anatomy, the bedside management of patient
The Curtis National Hand Center, MedStar Union
and family expectations, and the real and per-
Memorial Hospital, Baltimore, MD, USA
e-mail: dcalva@gmail.com; hmstarr3@gmail.com; ceived functional and social impact of surgical
jameshiggins10@hotmail.com failure.
# Springer Science Business Media New York (outside the USA) 2015 943
J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_43
944
This section will focus on pediatric amputation
and replantation. The most current achievements
and techniques in reconstructive surgery, rehabil-
itation strategies, and replantation research will be
described.
Historical Perspective
At the turn of the twentieth century, Dr. William
Halsted and others rst described experiments
related to limb replantation utilizing laboratory
canine models (Halsted 1922). In 1960, Jacobson
and Suarez demonstrated that by using a micro-
scope, it was possible for small vessels to remain
patent after anastomosis in vitro (Jacobson and
Suarez 1960). The following year, Kleinert suc-
cessfully demonstrated the feasibility of small
vessel anastomosis for salvage of four severely
injured upper extremities. The youngest patient
in the series was a 23-year-old with a near ampu-
tation at the wrist requiring repair of all exor
tendons, the median and ulnar nerves, and the
radial and ulnar arteries (Kleinert et al. 1963). In
the pediatric population, it was Malt and
McKhann who in 1962 rst successfully replanted
an upper extremity at the level of the mid-humerus
in a 12-year-old boy injured in a train accident
(Malt and McKhann 1964). In 1965, Komatsu and
Tamai were the rst to report the successful
replantation of an amputated thumb accomplished
by the anastomosis of 1 mm vessels utilizing a
microscope (Komatsu and Tamai 1968). How-
ever, in their report, credit is given to Kleinert
and Kasdan for having been the rst in the world
to replant an incompletely amputated thumb in
1962 (Kleinert et al. 1963). Soon after, many
surgeons worldwide published their experiences
with successful replantation of upper extremity
parts, in both adults and children, at levels ranging
from the shoulder to the thumb.
As microsurgery developed as a tool of the
reconstructive hand surgeon, pediatric microvas-
cular surgery received little attention, likely due to
the disproportionately small amount of patients
compared to the adult population. In the
mid-1980s, multiple surgeons reported the feasi-
bility and high success rates of replantation
D. Calva et al.
(6595 %) in children, which were comparable
to that of the adult population (McC et al. 1980;
Cheng et al. 1985). Today, it is standard of care to
attempt microsurgical replantation in pediatric
patients with upper extremity amputations meet-
ing the appropriate indications.
Current Demographics
and Epidemiology
Amputation injuries in the pediatric population
are associated with signicant physical disgure-
ment, functional and psychosocial disability, and
a high nancial burden. Hand surgeons apply
broader indications for pediatric digital replanta-
tion in order to avoid the detrimental psychosocial
and developmental consequences of amputation,
as well as optimize and restore maximal hand
function. However, epidemiologic and demo-
graphic data have been lacking compared to the
adult population.
Similar to adults, the most common mecha-
nisms of nger amputation in children include
sharp cutting or piercing injuries along with
amputations related to power tools. However,
mechanisms unique to children, such as amputa-
tions related to accidents involving home exercise
equipment, have also been reported in the litera-
ture. Benson et al. reported on 19 children with
hand injuries related to stationary bicycles nding
those injured by wheel spokes typically produced
repairable nerve and tendon lacerations, while
those injured by a crushing mechanism from the
chain and sprocket often produced severe injury
including amputations that were not salvageable
(Benson et al. 2000). Another series reviewing
treadmill injuries in the pediatric population
found this injury pattern most frequently included
partial or full-thickness burns to the volar aspect
of the hand (Carman and Chang 2001). More
proximal amputations are often the result of
motor vehicle accidents or pedestrian versus mov-
ing train injuries (Jaeger et al. 1981).
In 2011, Squitieri et al. published their ndings
on the patterns and disparities of surgical care in
pediatric digit amputations. From 2000 to 2006,
data were collected for health-care costs and
42 Amputations/Replantation
utilization using ICD-9-CM codes to identify digit
amputation and replantation in children. National
estimates were generated by weighted frequency
calculations. A regression model was used to
determine the inuence of demographic factors
on treatment. From the 1,321 records that met
the inclusion criteria, the rate of attempted digit
replantation in the pediatric population was 40 %.
Male children were most likely to be injured
(73 %) with single digit amputations occurring
most commonly (82 %), with the thumb being
the digit least commonly injured (15 %). Children
managed with attempted replantation were on
average younger and sustained less severe inju-
ries. The mechanism of injury in the replantation
group included a higher percentile of sharp cutting
or piercing injuries, while children that did not
undergo replantation had more frequently suf-
fered crushing injuries from motor vehicle acci-
dents or rearms. There were no statistically
signicant differences in hospital type or geo-
graphical region for children who underwent
replantation. Fifty-two percent of pediatric replan-
tations occurred at hospitals that perform 12
digital replants per year, and 26 % occurred at
hospitals that perform 5 or more per year. After
controlling for potential confounding variables,
African-American children, Hispanic children,
and the uninsured were less likely to undergo
replantation compared to Caucasian children or
those with insurance. Additionally, children with
multiple digit amputations and amputations of the
thumb were more likely to undergo replantation.
Another interesting nding was that girls and
children from higher socioeconomic households
were more likely to undergo replantation com-
pared to boys and those from lower socioeco-
nomic backgrounds (Squitieri et al. 2011).
Applied Anatomy
Surgical anatomy in pediatric replantation is anal-
ogous to adult replantation with the two caveats
that the physis should be identied and protected
in order to prevent future bony growth disturbance
and the small caliber of the vessels may increase
technical demand. It also may be more difcult to
945
assess the injury radiograph as the interpretation
may be limited by the progression of bony ossi-
cation of the hand. As with adult replantation,
tendons, nerves, arteries, and veins must be iden-
tied and meticulously prepared for successful
repair. Osseous shortening will aid in the ability
to perform primary microsurgical anastomosis by
relieving tension on the repair site. However,
careful attention should be taken to plan an osse-
ous shortening strategy that does not violate the
physis of the metacarpals or phalanges.
Amputation Assessment
Determining the mechanism of amputation pro-
vides insight to the potential damage of soft tissue
and underlying critical structures which may ulti-
mately affect the patients functional outcome fol-
lowing replantation. Baker and Kleinert reviewed
their experience with nger amputations in chil-
dren less than 3 years old, nding the most com-
mon mechanism of injury involved exercise
bicycles, followed by lawn mowers, doors or win-
dows, belts or pulleys, and fans (Baker and
Kleinert 1994). More severe mechanisms of
injury include meat grinders, explosives, guns,
recrackers, animal bites, and burns. In amputa-
tions resulting from avulsive or crushing mecha-
nisms, immediate and ultimate functional
replantation success is less likely (Buncke
et al. 2003) (Fig. 1).
Fig. 1 Multiple digit amputation caused by a young child
placing ngers in a paper shredder. A severe crushing and
avulsive mechanism of this sort may make replantation
impossible (Courtesy of Joshua Ratner, MD)
946
Obtaining an accurate history of the injury
mechanism also provides the surgeon with infor-
mation that may affect surgical treatment. Com-
partment syndrome should be considered
following crushing injuries to the hand or forearm,
and replantation should be accompanied by
appropriate emergent fasciotomies. In the case of
severe contamination, such as in farm injuries,
more aggressive debridement should be planned,
and broad-spectrum antibiotics, including penicil-
lin for clostridium coverage, should be started
immediately. Avulsion-type injuries are often
indicative of a larger zone of injury than is readily
apparent. This injury mechanism can involve dis-
ruption of the more proximal musculotendinous
junction and inict long segment vascular injury
requiring a vein graft for successful replantation
(Buncke et al. 2003).
As with any trauma, the ABCs (airway,
breathing, circulation) should be addressed rst
and only then should the focus be on the ampu-
tated part. Partington et al. showed that in a 7-year
period, with a total of 1,100 patients that were
referred for emergency microvascular surgery,
there was a 1 % incidence of occult injuries that
ultimately changed the original plan of care
(Partington et al. 1993). The pediatric patient,
unlike an adult, may be unable to provide a reli-
able history, and therefore witnesses (parents or
ambulance crew) become extremely important in
understanding the extent and timing of trauma.
Amputations of the hand are self-evident, but it
is also important to determine the level of injury to
suggest associated more proximal injuries. Deter-
mining the time of injury and the eld manage-
ment of the amputated part should provide the
surgeon with the assessment of the cold and
warm ischemia insult sustained by the amputated
part and the urgency of reperfusion. Digit replan-
tation is considered feasible if warm ischemia
time is limited to 612 h. Due to the lack of
ischemia-sensitive muscle, a well-preserved and
cooled digit, cold ischemia can likely be tolerated
for up to 24 h after amputation prior to replanta-
tion (Lin et al. 2010; Waikakul et al. 2000). Cases
of delayed digit replantation have reported even
after 33 h and 94 h of warm and cold ischemia,
respectively (Chiu and Chen 1984; Wei et al. 1988).
D. Calva et al.
Fig. 2 Amputated part wrapped in moist sterile gauze.
Subsequently, the part should be placed in a waterproof
bag and then in a bucket of ice (Courtesy of Joshua
M. Abzug, MD)
The limits of tolerated ischemia time in distal
amputations are thus poorly dened. Indeed, Lin
et al. studied ischemia time in 31 cases of hand
and nger replantation that exceeded 24 h and
found no correlation between ischemia time and
postoperative outcome (Lin et al. 2010).
Fingertips amputated distal to the lunula in the
pediatric population, typically age three or youn-
ger, can be replaced as a composite graft without a
vascular anastomosis as their survivability, and
outcomes have been reported as excellent (Shenaq
and Kattash 1998). Heistein et al. evaluated fac-
tors affecting composite graft survival in ngertip
amputations distal to the DIP joint. After
performing a multivariate analysis, smoking was
found to be the only signicant risk factor for graft
failure. Age older than 18 was initially thought to
lead to higher failure rates, but later this was found
to be closely linked to smoking. There was a trend
for higher graft failure in crush injuries compared
to avulsion or sharp amputations, but this did not
reach statistical signicance (Heistein and Cook
2003).
Innis provided a methodical description of the
proper handling of amputated parts. The ampu-
tated part should be wrapped in clean moist gauze,
placed in a bag, and sealed without any direct ice
contact (Fig. 2). The bag with the part should then
42 Amputations/Replantation
be placed in another container with ice. A bulky
pressure dressing should be applied to the injured
extremity to prevent hemorrhage, and the patient
should either be transferred to the emergency
department or a tertiary care institution (Innis
1995).
Bleeding vessels can be routinely controlled by
application of pressure and appropriate bandages.
Application of vessel clamps in the eld or emer-
gency department to control bleeding should be
avoided if possible, so that inadvertent injury to
the adjacent structure (i.e., nerves) can be
avoided, and more extensive arterial injury
prevented. Use of tourniquets in the eld or during
transport should be discouraged unless all
attempts at pressure dressing hemostasis have
been exhausted. The use of eld tourniquets
risks further nerve and muscle damage if eld
management is prolonged or performed by those
unfamiliar with tourniquet technique. Parts that
are nearly amputated but attached should be
placed into near anatomical alignment and
splinted. This will prevent further damage to the
part that might occur from kinking of remaining
vascular structures (Michalko and Bentz 2002).
Determining concomitant medical conditions
enables the surgeon to ascertain the risk of lengthy
or complex surgical interventions. If the patient
has not received a tetanus vaccination, it should be
provided in the emergency department. If rabies
transmission is a risk from a rabid animal bite, one
should start treatment immediately. The disease is
usually fatal, and for this reason, the child should
receive rabies immune globulin and then a series
of ve doses of rabies vaccine over the following
2 weeks.
Imaging
All hand injuries should be evaluated with three
views of the hand (PA, oblique, and true lateral).
Dedicated nger x-rays should be obtained for all
digits that are injured. Two views of the extremity
(AP and lateral) are needed if there is an injury to
the forearm or upper arm. If the mechanism of
injury is suspicious for a larger zone of injury,
then the adjacent joints should also be imaged.
947
The amputated part should be imaged to fully
understand the pattern of injury, extent of bone
loss, joint involvement, and comminution.
Radiographs of the pediatric hand are limited
by the ossication progression of the bones.
Understanding the normal ossication center
anatomy is important to accurately interpret a
pediatric hand radiograph. At birth, all carpal
bones are cartilaginous and thus unable to be
evaluated by radiographs. Ossication progresses
in a predictable manner with the capitate and
hamate ossifying within the rst 3 months
followed by the triquetrum and lunate within the
rst 24 years of life. By age 6, the trapezium,
trapezoid, and scaphoid have also typically ossi-
ed, while the pisiform ossies during puberty.
The epiphyses of the metacarpals and phalanges
become recognizable between 12 months and
3 years of life. The physis is located distally in
all metacarpals except the thumb and proximally
in all phalanges (Gilsanz and Ratib 2012). It is
important to locate, protect, and prevent any fur-
ther damage to the physis when planning a replan-
tation surgery in children.
Replantation of the Injured Part
Indications
In 1995 Tan and Teoh published their experience
from 8 children with 10 digits that were replanted
from 1993 to 1994. They suggested that replanta-
tion was always indicated in children because of
the possibility of obtaining good functional results
in young patients. They described an 11-year-old
boy with a complete avulsion amputation of the
right ring nger who regained 100 exion at the
PIP joint and 10 at the DIP joint with his physis
remaining open 5 months after replant. Another
8-year-old boy, 13 months after replantation of the
left index nger following a sharp crush injury at
the level of the DIP joint, had excellent function
with 40 exion of the joint (Tan and Teoh 1995).
Cheng et al. published their series of 44 amputated
digits that were replanted in children with long-
term follow-up of 915 years (Cheng et al. 1998).
While surgical success in replantation does not
948 D. Calva et al.

assure functional success, these studies support Contraindications

broadened indications for pediatric replantation
due to the greater healing potential and In children many authors have advocated
adaptability in this population (Shenaq and attempting replantation of any digit amputation.
Kattash 1998). However, relative contraindications are worthy of
Dautel classied ngertip amputations and consideration. Boulas proposed the following
proposed several indications for replantation in contraindications:
children by addressing three categories: mecha-
nism of injury, vascular anatomy, and level of Warm ischemia time more than 12 h for digits
amputation within the ngertip. Since the major- where muscle is absent and more than 6 h
ity of pediatric amputations are caused by crush or where muscle is present
avulsion injuries and sharp or clean-cut injures are Severely crushed parts or the mangled extrem-
rare, he felt that the mechanism of injury should ity where the majority of the tissue components
have no inuence on the decision to attempt are damaged
replantation. He proposed that it is best to evaluate Extensive avulsion injuries which are associ-
the part under the microscope in order to decide ated with extensive damage to vessels, nerves,
upon replantation or revision amputation. Micro- and musculotendinous elements
scope magnication and microvascular tech- Multilevel or segmental amputation injury of
niques have made replantation for even very the extremity
distal amputations feasible. There is often an arte- Severe major psychiatric disorders where the
rial branch that courses close to the midline within patient cannot comply with postoperative
the pulp of the digit, and this branch can typically instructions
be anastomosed. Lastly, Dautel noted that each Prior nonfunctional or limiting functional con-
level of ngertip amputation poses technical chal- ditions of the injured extremity
lenges that differ according to the zone of ampu- Damage to the part by inadequate transport
tation. He proposed four zones within the care, such as when the part is placed directly
ngertip, all distal to the DIP joint: on ice, causing freezing and formation of intra-
cellular ice crystals, capillary damage with
Zone 1: No bone fragment; distal tip pulp with micro thrombosis, and irreversible vasocon-
no vessels suitable for anastomosis. The part striction (Table 1).
may be replaced as a composite graft.
Zone 2: Amputation through the nail bed, pre-
serving at least one half of the sterile matrix. Table 1 Operative Indications & Contraindications for
The part may contain an artery that courses Pediatric Replantation
close to the midline within the pulp and it can Operative
be used for anastomosis. In this zone, there are Operative indications contraindications
often no dorsal veins available; venous drain- In general, pediatric digital Other life-
age can be controlled by either retrieving a amputations should undergo threatening injuries
palmar vein or controlled bleeding. attempt at replantation Severe
Zone 3: Similar to zone 2; however, the sterile contamination
Relative
matrix remaining on the digit is minimal or
contraindications
absent. Multilevel or
Zone 4: This zone is proximal to the nail fold, mangled
usually within or just proximal to the germinal amputation
matrix, and there usually is a dorsal vein that Extensive avulsion
may be dissected and repaired to improve injury
venous drainage (Dautel 2000; Dautel and Excessive warm
ischemia time
Barbary 2007).
42 Amputations/Replantation
There are two strict contraindications that are
proposed:
The extremely contaminated injury which
could place the patient at risk for sepsis
and/or necrotizing infection
Concomitant life-threatening injuries, where
the priority should always be to preserve life
and not limb (Boulas 1998)
Anesthesia
Most pediatric hand trauma cases, and especially
those that will endeavor replantation, should be
done under general anesthesia as they are partic-
ularly long procedures. An adjunct to general
anesthesia is the use of regional blocks which
can enhance vasodilatation via sympathetic
blockade. The procedure has proven to be safe
and effective in the pediatric population and in
the acute setting (Ivani and Mossetti 2008).
Operative Techniques
The sequence of repair of various structures
involved in replantation may vary according to a
surgeons preference, but general principles may
be observed. In general, skeletal shortening and
xation is performed rst, with other macroscopic
work, such as tendon repairs, prior to performing
more delicate microsurgery. Surgeons may prefer
alternative techniques. Kim et al. advocated rst
addressing skeletal xation, then arterial repair,
followed by venous repair, tendons, and lastly
coaptation of nerves (Kim et al. 2005) (Table 2).
Addressing multiple digit amputations in chil-
dren is technically challenging and time consum-
ing. Some surgeons advocate attempting to
replant all parts and restoring hand anatomy and
function as close as possible to its original state
(Kim et al. 2005). Others feel that the surgeon
should try to replant enough digits to restore two
main functions: pinch and grasp. These goals
mandate that at the very minimum, a thumb and
an opposing digit be replanted (Buncke et al. 1991;
Bennett 1975). This decision depends on the
949
Table 2 Preoperative Planning for Pediatric
Replantation
OR table: standard table with radiolucent hand table
attachment
Position: supine with arm on hand table
Fluoroscopy location: same side as hand table
Equipment/supplies: uoroscopy, operating
microscope, Doppler with sterile probe, microsurgical
instruments, wire driver, Kirschner wires, 40
nonabsorbable suture, 60 monolament, 100 nylon,
warm saline, papaverine, heparinized saline irrigation
with 24-gauge Angiocath, and arterial and venous
microsurgical vessel clamp
Tourniquet: nonsterile
condition of the amputated parts. In difcult situ-
ations, the surgeon may choose to borrow parts
from a non-salvageable digit for use in
reconstructing other digits. In cases of multiple
digit replants, Kim et al. advocate a structure-by-
structure replantation technique as opposed to
the less time-efcient digit-by-digit scheme. In
structure-by-structure replantation, skeletal xa-
tion is achieved in all digits before subsequently
addressing tendons, nerves, arteries, and veins in
that sequence (Kim et al. 2005).
Amputations at the level of the hand or wrist
tend to be avulsion- or crush-type injuries in the
pediatric population. A thorough debridement of
devitalized or contaminated tissue is important to
prevent subsequent infection, but the surgeon
should also attempt to preserve as much healthy
tissue as possible for reconstruction. As with dig-
ital amputations, bony xation of the involved
metacarpals, carpals, and radius and ulna should
be the rst step of the replant procedure. This can
be accomplished with Kirschner wires,
intraosseous wiring, screws, or plates (Kim
et al. 2005). As in adult replantation, bone loss
or skeletal shortening may require partial or total
wrist arthrodesis. However, particular effort is
made in avoiding loss or damage of physes to
preserve future limb growth.
For cases of forearm or upper arm amputation,
it is critical that the part be placed in an ice saline
solution for transport and that cold perfusion with
heparinized saline be considered when the part
arrives to the replant center (Tamai 1982). Addi-
tionally, due to the possibility of reperfusion
950
compartment syndrome, patients should undergo
fasciotomies with consideration given to serial
operative wound assessment to discern the viabil-
ity of the muscles and skin (Kim et al. 2005). The
limb should be revascularized as soon as possible
to prevent muscle death and minimize the risk of
subsequent infection (Saies et al. 1994; Taras
et al. 1991).
As in cases of adult major limb replantation,
consideration may be given to intraoperative arte-
rial shunting with specically designed catheters.
This enables rapid reestablishment of arterial sup-
ply to the part. This is considered if the presurgical
ischemia time is lengthy and the amputated part
contains signicant amounts of ischemia-sensitive
muscle. The shunt is typically placed prior to skel-
etal xation and arterial ow is reestablished. The
surgeon may elect to shunt a large vein and mini-
mize blood loss during this procedure. Shunt reper-
fusion is maintained for adequate duration to
enable the surgeon to safely begin tourniquet-
controlled replantation thereafter. The surgeon
may lengthen the shunt reperfusion period if the
previous ischemia time was lengthy.
During reperfusion of forearm or arm level
amputations, the anesthesiologist should carefully
monitor the patients hematocrit and administer
blood as needed (Buncke et al. 2003). Due to the
reperfusion of ischemic muscle, the venous return
at rst is dark and high in potassium due to muscle
breakdown. Waikakul et al. showed that the risk
for failed replantation is higher if the measured
potassium is greater than 6.5 meq/dL from the
venous return (Waikakul et al. 1998).
If the required shunt reperfusion period is
lengthy and the anatomy permits, the surgeon
may proceed with osseous xation, tendon repair,
and nerve repair with the shunt in place. When the
perfusion period is deemed adequate, the shunt
may be removed and arterial repair or reconstruc-
tion with grafts is then pursued rapidly.
Skeletal Fixation and Bone Shortening
For digits, bone shortening is an important
technique to minimize tension on the anasto-
mosis of arteries, veins, and nerves; however, in
children, one must not compromise the physis
(Urbaniak 1984). Popular stabilization techniques
D. Calva et al.
Fig. 3 Successful replantation of the long and ring ngers
using longitudinal K-wires for xation (Courtesy of
Shriners Hospital for Children, Philadelphia, PA)
for digits include intraosseous wires or Kirschner
wires (Fig. 3).
For ngertip amputations, Kirschner wires are
often too large and should not be used in infants,
toddlers, or young children. For these patients,
small needles, such as a 25- or a 27-gauge, can
be used for bone xation. The needles may be
inserted by hand, and not with a power drill.
This minimizes the transfer of heat that is gener-
ated by the drill, which can lead to necrosis of the
small bone fragment and thin surrounding tissues.
In replantation performed at the distal ngertip, a
single needle is sufcient, whereas more proxi-
mally two crossed needles will achieve xation
and prevent rotation (Dautel 2000; Dautel and
Barbary 2007).
When the injury is at the wrist, forearm, or
upper arm level, the radius, ulna, and humerus
can be shortened up to 3 or 4 cm without signif-
icant functional problems. In long bones, rigid
xation with plates provides the best stability
while again making sure not to injure the physis
with shortening or xation (Buncke et al. 2003).
Tendon Repair
Multiple tendon repair techniques have been
described throughout the literature, and the tech-
nique employed is often dependent on surgeon
42 Amputations/Replantation
preference and the zone of injury. Tendon repair
within the exor tendon sheath is performed with
greater surgical precision to minimize adhesion
formation. Increasing the number of strands
across the repair site and the addition of an
epitendinous stitch has been shown to improve
the repair strength and tendon gliding within the
sheath. Outside the exor sheath, the tendons may
be repaired with core sutures. Less precision
needs to be dedicated to avoiding bulk of the
repair site as it does not require passage through
the sheath. Within exor tendon zone 5, if the
tendons are avulsed from the musculotendinous
junction, primary tendon transfers should be con-
sidered (Kim et al. 2005). Buncke et al. preferred
to repair the tendons with a double-opposing
locking loop with or without an epitendinous
stitch, although for extensor tendons, they recog-
nized that if there is no length, a mattress repair is
adequate (Buncke et al. 2003).
Arterial Repair
In the setting of a sharp laceration or penetrating
injury, identication of vessel ends is often
accomplished near the traumatic wound. How-
ever, following avulsion-type injuries, damage of
a long vessel segment may frequently be encoun-
tered and require vein grafting for repair even after
bone shortening (Michalko and Bentz 2002;
Cheng et al. 1998).
For ngertip injuries, evaluation of the proxi-
mal stump and the distal part should be done
under a microscope in order to identify possible
vessels for anastomosis. If the distal vessel is
collapsed, one can milk the ngertip pulp and
rell the vessels for aid in identication. Exposure
of the proximal vessels is performed with
midaxial or volar zigzag incisions (Tsai
et al. 1989). Vessels are repaired under the micro-
scope with 9-0, 10-0, or 11-0 monolament
(Dautel 2000; Dautel and Barbary 2007).
Venous Repair
Identifying suitable dorsal veins for anastomosis
is technically challenging. By starting with the
arterial anastomosis, subsequent venous engorge-
ment can aid in the identication of dorsal veins.
If possible, at least two veins should be repaired
951
with studies showing that this technique will
reduce venous congestion and bleeding and
improve replant survival (Michalko and Bentz
2002; Cheng et al. 1998; Kim et al. 2005). Hattori
et al. published a case series of 64 nger replants,
where venous anastomosis was possible 84 % of
the time, and reported an overall survival rate of
86 %. They concluded that the most critical factor
in survival of the replant was the venous anasto-
mosis (Hattori et al. 2003). Venous repair in very
distal ngertip amputations is difcult due to the
lack of dorsal veins. Tsai et al. advocate
performing a venous anastomosis using a volar
vein within the pulp (Tsai et al. 1989). In the
absence of identiable volar or dorsal veins, n-
gertip replantation may require using postopera-
tive controlled bleeding to maintain digit viability
until small venous pathways are developed
(Dautel 2000; Dautel and Barbary 2007).
Nerve Repair
An important aspect of the functional outcome of
digital replantation is the recovery of sensation.
Excellent recovery of sensation has been demon-
strated in replantation of pediatric digits, further
supporting attempted replantation of all pediatric
amputations when feasible. For ngertip injuries,
identication of neural elements and repair can be
technically challenging and sometimes impossi-
ble. However, great sensory recovery can be
expected even without a nerve repair. Dautel eval-
uated recovery of sensation in eight pediatric
patients with ngertip replantations where nger-
tip level nerve repair was not performed. Good
return of sensation was found in this group despite
the lack of nerve repair with an average 2-point
discrimination of 4.6 mm at nal follow-up
(Dautel 2000; Dautel and Barbary 2007). Faivre
et al. also found that there is adjacent and sponta-
neous neurotization after replantation of ngertips
distal to the DIP joint in children (Faivre
et al. 2003).
For more proximal injuries, primary nerve
coaptation should be performed using an 8-0 or
9-0 microsuture. If excessive tension or a gap
exists, nerve grafting should be performed. Typi-
cal sites of donor nerves are the posterior
interosseous nerve, medial antebrachial cutaneous
952
Table 3 Surgical Steps for Pediatric Replantation
Begin preparation of amputated part prior to induction of
anesthesia
Identify amputated part neurovascular structures via
bilateral midaxial approach
Perform osseous shortening of amputated part
preserving physis
Place Kirschner wires in amputated bone perched at
osteosynthesis site
Place locking core suture in FDP tendon of amputated
part
Remove nail
Identify proximal neurovascular structures via bilateral
midaxial approach
Fixate amputated bony structures to proximal bone with
Kirschner wires
Perform a 4-strand core suture repair of the FDP tendon
only
Repair extensor tendon with a gure-of-eight stitch
Repair nerve using operating microscope
Repair at least 2 dorsal veins using operating microscope
if tourniquet time allows
Release tourniquet and resect artery until undamaged
lumen identied and inow conrmed
Perform artery anastomosis under microscope
Conrm arterial and venous repair patency
Loosely approximate skin or leave wounds open
nerve, or digital nerve from adjacent
non-salvageable digits (Kim et al. 2005). Other
microsurgeons have successfully utilized the lateral
femoral cutaneous nerve, supercial peroneal
nerve from the dorsum of the foot, and the sural
nerve for nerve grafts (Faivre et al. 2003; Buntic
et al. 2002). While autograft remains the gold stan-
dard for nerve reconstruction, nerve conduits and
nerve allograft have proven effective for
reconstructing peripheral digital nerve decits up
to 30 mm while avoiding donor site morbidity
(Taras et al. 2013; Lohmeyer et al. 2013) (Table 3).
Dressings and Splinting
The skin edges should be loosely approximated
following replantation and a nonadhesive dress-
ing applied. If the skin closure is tight, the inci-
sions should be left open to heal by secondary
intention. The surgeon should avoid constrictive
circumferential dressings. A dorsal splint should
be applied to immobilize the nger, hand, and
D. Calva et al.
wrist following digit replantation (Dautel 2000;
Dautel and Barbary 2007).
Postoperative Care
Although heparin, dextran, or aspirin are widely
used for postoperative anticoagulation, there is no
conclusive evidence that anticoagulation
improves success rates following replantation.
Nikolis et al. concluded that the routine use of
IV heparin (25100 units/kg/h) following digital
replantation was not warranted. In their retrospec-
tive review of 104 revascularized digits and
71 replantations over a two-year period, IV hepa-
rin was used 35 % of the time and was associated
with a fourfold increased risk of heparin-related
complication without improvement in success
rates. The authors concluded that the injury mech-
anism and surgical technique remained the most
important predictor of successful outcome
(Nikolis et al. 2011).
Dextran is a macromolecule composed of glu-
cose that when administered intravenously exerts
an antiplatelet and anti-brin activity. If dextran
(20 mL/kg/24 h) is employed postoperatively,
other intravenous uids should be adjusted, as
dextran serves as an intravascular volume
expander (Buncke et al. 2003). Aspirin is rou-
tinely used by microsurgeons following vessel
anastomosis to inhibit platelet aggregation.
While safe in the adult population, the use of
aspirin should be avoided in children due to the
association with Reyes syndrome. Several case
series have reported that localized heparin can
provide benet in the setting of venous conges-
tion, although stronger evidence has not been
reported (Buckley and Hammert 2011). Antibi-
otics should be given for a minimum of 5 days,
but length of therapy should be dictated by the
timing of wound closure and the surgeons con-
cern for risk of infection (Michalko and Bentz
2002). Chlorpromazine has been used in pediatric
replantation to minimize vasospasm and constric-
tion of peripheral arterioles that may be induced
by anxiety experienced by the hospitalized and
conned child (Urbaniak 1984).
42 Amputations/Replantation
The child should be admitted to a unit where
the nursing staff is familiar with replantation or
free ap monitoring. The child should remain on
bed rest with a urinary catheter in place for the rst
24 h. The temperature of the room should be
maintained at or above 72 F to minimize arterial
vasospasms. If the child does not tolerate the room
temperature, a heating blanket can be utilized to
keep the extremity warm to >72 F. Finally, atten-
tive monitoring of the replanted part by the nurs-
ing staff is of paramount importance. An hourly
physical examination of the part should include
capillary rell, warmth, color, and turgor.
Evaluating skin temperature and digital surface
acoustic Doppler evaluation should be part of the
postoperative hourly monitoring protocol. Lu
et al. showed that a drop of more than 2 C com-
pared to adjacent normal digits or a temperature
below 30 C was a sign of replant compromise
(Lu et al. 1984). Lindfors and Marttila used the
threshold of 32 C to alert them of the possibility
of replant compromise (Lindfors and Marttila
2012). Reagan et al., in a series of 111 patients
with 188 revascularized digits, found that temper-
ature monitoring was 100 % sensitive but only
61 % specic for vascular compromise of the
digit. However, when combined with clinical
data, temperature monitoring was highly effective
in detecting early vascular compromise, with a
sensitivity of 100 % and a specicity of 99 %
(Reagan et al. 1994).
Monitoring of the part should be performed
every hour for the rst 24 h and then every 2 h
for the next 24 h. Postoperative diet is restricted
from caffeine or chocolate to minimize vaso-
spasms. The child should be protected from
exposure to nicotine, usually in the form of second-
hand smoke from family members (Michalko and
Bentz 2002) (Table 4).
Outcomes
Baker et al. found the most important factors
predicting favorable results for replantation in
children are clean, sharp lacerations, age greater
than 9 years old, and weight greater than 11 kg
953
Table 4 Postoperative Protocol for Pediatric
Replantation
Apply above-elbow splint
Monitor in hospital for 5 days
Utilize dextran and keep extremity under airow heating
blanket
Cast applied at rst clinic visit
Kirschner wires and immobilization continued until
fracture union achieved
(Baker and Kleinert 1994). Avulsion injuries and
age less than 9 were the main factors that
predicted unfavorable outcomes (Kim
et al. 2005; Saies et al. 1994). Clinical series of
pediatric replantations have reported success rates
ranging between 25 % and 97 % (McC et al. 1980;
Cheng et al. 1998; Saies et al. 1994; Ikeda
et al. 1990) with the majority of replanted patients
(91 %) being satised with the aesthetic and func-
tional outcome (Kim et al. 1996). In a cohort of
thumb replants, Cheng et al. reported an 88 %
recovery of pinch strength and 79 % of grasp
strength compared to the normal thumb (Cheng
et al. 1998). For amputations at the ngertips, Shi
et al. reported normal pinch strength (Shi
et al. 2010).
Recovery of sensation in children has been
found to be faster than adults (Stevenson and
Zuker 1986). Studies have shown that digital
nerve recovery in children can be excellent, even
in the absence of nerve repair (Ikeda et al. 1990).
Cheng et al. found that recovery of sensation in
ngertip amputations was 88 %, with 2-point
discrimination of 4 mm (Cheng et al. 1998). In
contrast, Saies reported a cohort of similar
patients that demonstrated a mean 2-point recov-
ery of 8 mm (Saies et al. 1994). For amputations at
the ngertips, Shi et al. reported an overall 2-point
discrimination of 3.8 mm for patients that
underwent nerve coaptation and 4.4 mm for
those that did not undergo nerve repair (Shi
et al. 2010). Large series have reported the inci-
dence of cold intolerance to be as high as 40 %
(McC et al. 1980; Cheng et al. 1998).
Cheng et al. found a mean total active motion
of 130 in the thumb and 150 for all other ngers
954
(Cheng et al. 1998). Saies et al. reported 155 for
the thumb and 172 for all other ngers (Saies
et al. 1994). For amputations at the ngertips, Shi
et al. reported an 89 mean range of motion at the
DIP joint (Shi et al. 2010).
Multiple studies have demonstrated that longi-
tudinal growth is not statistically affected by the
type of injury, the number of anastomosed vessels,
or the ischemic time. Only when the growth plate
is directly damaged by the amputation pattern
does the digit experience growth disturbance
(Ikeda et al. 1990). Cheng et al. reported that
91 % of replants reached normal growth at skele-
tal maturity ,while others have reported up to 95 %
of replants reaching normal growth (Cheng
et al. 1998; Shi et al. 2010; Stevenson and Zuker
1986). However, if the physis is injured, skeletal
growth can be reduced to 8688.5 % compared to
the normal contralateral digit (Demiri et al. 1995;
Wolfram et al. 2008).
Amputations at the wrist or distal forearm level
risk injury to the distal radial or ulnar physis at the
time of the injury or reconstruction. There has
been minimal data published on functional out-
come or skeletal growth in children following
replantation at this level (Shi et al. 2010; Beris
et al. 1994; Plato et al. 1980).
Preferred Treatment
All digital amputations in children are approached
with the intention to replant if possible. Distal tip
amputations will be replaced as composite grafts
after defatting in children under 3 years of age.
Restoration of the tip contour is usually observed
either as a result of successful revascularization or
healing of the underlying tissue by secondary
intention.
Every effort is made to begin preparation of the
amputated part in the operating room prior to
commencement of anesthesia and operative care
of the patient. Structures are identied and
assessed. Osseous shortening is performed with-
out violating the physis. Kirschner wires are
placed in the bone and perched at the
D. Calva et al.
osteosynthesis site. Sutures are placed in the distal
FDP tendon. Finally, the nail plate is removed to
enable use of heparin pledgets or leeches postop-
eratively if venous congestion is encountered.
Rapid preoperative preparation in this manner
minimizes the total operative and anesthesia time
for the patient.
More proximal digital amputations are pursued
with structures repaired in the sequence used in
adult replantations (bone, tendon, nerve, artery,
and vein). Osseous xation is Kirschner wires
almost exclusively. Interosseous wires are
avoided because of the proximity of the physis at
the phalangeal bases and because the speed of
osseous healing in this population makes the use
of buried hardware unnecessary.
Tendon repairs are performed with FDP repairs
only as the additional repair of the FDS often leads
to unnecessary bulkiness in the setting of replan-
tation. In a single digit replantation case, the exor
tendon will be repaired utilizing a standard zone
2 technique using four locking core sutures with
4-0 suture material and a single knot. If multiple
digits are replanted, the tendons will be prepared
on each digit with core sutures placed before the
parts are put in position. Likewise, the profundus
tendons in the hand will have core sutures placed
during preparation. When the two parts are
brought together, the suture on the proximal ten-
don end is then passed through the distal part in a
locking horizontal mattress fashion and returned
to the tendon repair site. The suture in the distal
tendon is likewise passed through the proximal
tendon in a similar fashion and returned to the
repair site. This technique can be performed rap-
idly and yields two knots at the tenorrhaphy site
and 4 core sutures. If time permits, 6-0 monola-
ment epitendinous sutures are then added to the
repair. Extensor tendon repairs are performed
thereafter with 4-0 suture using gure-of-eight
suturing technique.
Proximal to the ngertip nerves are repaired
with the microscope preferably while still under
tourniquet control for best visualization. Osseous
shortening may enable primary neurorrhaphy.
Otherwise, posterior interosseous or medial
42 Amputations/Replantation
antebrachial cutaneous nerve grafts are used to
achieve tension-free nerve repairs.
After nerve repairs are completed, tourniquet
time is assessed. If there is remaining tourniquet
time available, dorsal venous repairs will be
meticulously performed in as many veins as pos-
sible. Double non-crushing clamps are left in
position with heparin irrigation within the lumen
of the completed venous repairs. The hand is then
supinated, the tourniquet released, and arterial
repairs performed.
The artery must be liberally resected proxi-
mally and distally until the lumen appears
undamaged under microscope assessment of the
intima. Reversed vein grafts are used liberally
from the forearm to achieve a tension-free repair
after resection of the damaged segment. When
vein grafts are employed, distal anastomoses are
performed rst since the unconstrained proximal
vein graft can provide many degrees of freedom
when trying to rotate and access the more difcult
distal anastomosis deep surface.
The arterial inow is conrmed by releasing
the clamp on the proximal vessel. If inadequate,
topical vasodilators such as papaverine and warm
saline are used to relieve vasospasm before
reassessment. If this fails to yield adequate inow,
the vessel may require more proximal resection.
After proximal ow is deemed adequate, the
proximal anastomosis is performed under double
clamp control. When complete, the arterial anas-
tomosis is released with removal of the clamp and
papaverine is applied. The venous anastomotic
clamps are usually left in place until a column of
venous blood is seen engorging the repaired veins
just distal to the clamps. The clamps are then
released and topical papaverine applied. The
digit is kept warm during this tenuous period and
the patency observed prior to closure.
Skin is often left completely unrepaired to
avoid vessel compression with postoperative
swelling. The wounds will heal remarkably well
regardless of very wide gaps remaining after com-
pletion of the case. Even in the setting of exposed
vessel and nerve repairs, suturing skin is avoided
as much as possible.
955
Postoperatively very large above-elbow splints
are used to minimize extremity motion in the
pediatric population. The patient is usually mon-
itored for 5 days prior to discharge from the hos-
pital. Dextran is routinely used for 35 days, and
the extremity is kept under an airow heating
blanket to minimize vasospasm.
Postoperative arterial thrombosis will result in
reexploration and arterial anastomosis revision.
Leech therapy is used to try to treat for venous
thrombosis with preserved arterial inow, taking
care to monitor the patients hematocrit and provide
antibiotic prophylaxis for leech therapy (Table 5).
Complications and Management
Following replantation, frequent vascular checks
should be performed by an experienced nursing
staff. Changes in color to a dark, mottled, purple
appearance likely indicate venous congestion
(Michalko and Bentz 2002). If congestion persists
after releasing the bandages, removal of the nail,
periungual incisions, heparin-soaked pledgets,
and medicinal leeches have all been found to
improve outow in the setting of venous insuf-
ciency (Batchelor et al. 1984; Gordon et al. 1985;
Han et al. 2000). Leeches are effective in relieving
venous congestion via two mechanisms. The
leeches will digest approximately 5 mL of blood
at each feeding and decompress the venous con-
gestion for a short period of time. Leech saliva
contains hidurin, a potent heparin analogue,
which serves to maintain bleeding from the
attachment site or nail bed after the leech is no
longer attached (Brody et al. 1989). If leeches are
utilized, one must also start empiric treatment
with a third-generation cephalosporin that treats
Aeromonas hydrophila, a bacteria present in the
gut of the leech. In addition, there is signicant
bleeding associated with replantation and leech
therapy, and the patients hematocrit should be
monitored daily with blood transfusions adminis-
tered as needed. Niibayashi et al. found that in
children who underwent replants, those that failed
had a mean hematocrit of 30 % and those that
956 D. Calva et al.

Table 5 Pearls & Pitfalls

Potential pitfall
#1 Inadequate tourniquet time
#2 Inability to perform tension-free
primary nerve or vessel repair
#3 Lack of adequate inow
#4 Lack of adequate outow
Pearls for prevention
1a: Complete preparation of amputated parts before inating tourniquet
1b: Sequence of repair is structure by structure and not digit by digit
1c: Budget time to at least complete osseous xation, tendon repair,
microscopic nerve repair, and identify vessels before tourniquet is released
2a: Shorten amputated phalanx up to half the length to allow easier primary
coaptation
2b: Consider crossing over less injured proximal artery or noncritical
digital nerve to less injured distal artery and critical digital nerve,
respectively
3a: Liberal use of warm saline irrigation, vessel dilation, heparinized saline
irrigation, and papaverine and lidocaine topical irrigation to relieve spasm
3b: Ensure vessel is resected proximally to healthy lumen
3c: Liberal use of reversed vein graft if proximal resection prevents
primary coaptation
4a: Repair 2 or more veins
4b: Do not close midaxial incision
4c: Remove nail and plan for use of heparin pledgets or leech therapy
4d: Externalize irreparable distal vein stump through wound
4e: If distal veins are inadequate, consider using one of two distal arterial
stumps as substitute for distal vein to allow egress of retrograde arterial
ow

survived had a mean hematocrit of 33 % Table 6 Complications of Replantation

(Niibayashi et al. 2000).
Common
A nger suffering from arterial insufciency complication Management
becomes pale, mottled, and cold and demonstrates Venous Release constrictive dressings and
poor turgor. The rst step in evaluation is to pro- congestion elevate
vide adequate analgesic to the patient and remove Remove nail
the splint and any constrictive dressing (McC Use of heparin pledgets and/or leech
et al. 1980; Michalko and Bentz 2002; Taras therapy
Arterial Release constrictive bandages
et al. 1991). The use and safety prole of strepto-
insufciency Ensure adequate analgesia
kinase, urokinase, and tPa in children has not been
Consider return to OR for revision of
well established. There are a few case reports, arterial anastomosis with
including a neonate, a 9-year-old, and a 13-year- interpositional vein graft
old, where these plasminogen activators were Infection Use of postoperative IV antibiotics in
used successfully to treat arterial thrombosis setting of contamination
(Theile and Coombs 1996; Atiyeh et al. 1999). Return to OR for meticulous
debridement
Arterial thrombosis is treated with surgical
Failure of Revise amputation sparing as much
evaluation and possible revision of the arterial replantation length as possible. For thumb
anastomosis. Arterial complications tend to have reconstruction, consider index
better outcomes with revision anastomosis com- pollicization or toe-to-thumb transfer
pared to venous complications, with an estimated
salvage rate up to 50 % in some series
(Goldner 1985). even with successful replantation. Crush or avul-
Infection is a substantial risk in replantation sion injuries have been found to have a higher
because of the tissue injury and contamination infection rate than sharp guillotine-type injuries
from the injury and the subnormal perfusion (Saies et al. 1994; Beris et al. 1994) (Table 6).
42 Amputations/Replantation
When Replantation Is Not Possible:
Reconstructive Options
Pollicization of the Index
Pollicization of the index nger is a reconstructive
option to consider when the amputated thumb is
unable to be replanted. First described by Buck-
Gramcko for the treatment of hypoplastic and
aplastic thumbs, the technique is now an accepted
option for restoration of opposition, pinch, and
grasp in the setting of traumatic thumb amputa-
tions (Buck-Gramcko 1971). If the index is not
available due to the traumatic event, pollicization
using the middle or ring nger, in whole or parts,
has been described (Weinzweig et al. 1995).
Toe-to-Thumb Transfer
In children, Kotkansalo et al. showed good
restoration of grip strength and high patient satis-
faction following toe-to-thumb reconstruction
(Kotkansalo et al. 2011). Rosson et al. reported
that when replantation is not possible, a toe-to-
thumb reconstruction functions as well as, or even
better than, a replanted thumb. Analyzing
384 thumb amputations, with 274 thumb replants
and 110 toe-to-thumb reconstructions, the authors
found that great toe transfer function was compa-
rable to thumb replants from sharp injuries, and
interphalangeal motion was signicantly better
than replantation of crush-type amputations. The
survival rate of the thumb replants was 85 %,
compared to 93 % of the toe-to-thumb reconstruc-
tions (Rosson et al. 2008). Although toe-to-thumb
transfer should be reserved for experienced sur-
geons, it provides a predictable salvage option
when replantation has failed or is not possible.
Prostheses
Replantation is not always feasible and upper
extremity prostheses continue to evolve. The Rev-
olutionizing Prosthetics program, which is part of
the Defense Advanced Research Projects Agency
(DARPA), has yielded several promising break-
throughs in prosthetic arms and digits. Forthcoming
prosthetics include technology with direct brain-
wave control, 22 degrees of freedom, and individ-
ually powered digits. Research and development is
also focused on multifunctional and partial hand
957
prostheses that can provide the user more freedom
and functionality (Kotkansalo et al. 2011).
Transplantation
Hand transplantation, although not yet performed in
the pediatric population, is a promising method of
restoring form and function following non-
replantable traumatic amputation. However, many
drawbacks, including chronic immunosuppression,
currently make this option unfeasible for children.
Conclusions
Advancements in microsurgical techniques, com-
bined with the adaptive and regenerative capabil-
ities unique to the pediatric population, make
replantation the treatment of choice for the major-
ity of digit, hand, and upper extremity amputa-
tions in children. The reconstructive hand surgeon
needs to be familiar with the evaluation, operative
techniques, and postoperative management of
replantation specic to the pediatric population
to restore viability and function.
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Waikakul S, Vanadurongwan V, Unnanuntana
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Waikakul S, Sakkarnkosol S, Vanadurongwan V,
Un-nanuntana A. Results of 1018 digital replantations
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Wei FC, Chang YL, Chen HC, Chuang CC. Three success-
ful digital replantations in a patient after 84, 86, and
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Weinzweig N, Chen L, Chen ZW. Pollicization of
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 Pediatric Phalanx Fractures:
Evaluation and Management 43
Ross Feller, Augusta Kluk, and Julia Katarincic

Contents Abstract
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 962 The hand is the major medium that children
utilize to experience their surrounding world,
Epidemiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 962
making it particularly vulnerable to injury. His-
Pathoanatomy and Applied Anatomy . . . . . . . . . . . . 963 torically, the incidence of pediatric hand inju-
Osseous Anatomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 964 ries followed a bimodal age distribution during
Physeal Anatomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 964
Soft Tissue Anatomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 965 the toddler/preschool years and another spike
Nail Anatomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 965 during adolescence. However, this trend may
Remodeling in Pediatric Phalanx Fractures . . . . . . 965
very well be changing with increased partici-
pation in youth athletics. A rm knowledge of
Assessment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 965
pediatric hand pathoanatomy and the intrica-
Treatment of Specic Fracture Types . . . . . . . . . . . . . 968 cies of fracture healing in the child are neces-
Distal Phalanx Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 968 sary in order to properly evaluate, diagnose,
Phalangeal Neck Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . 972
Condylar Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 974 and treat these injuries. The exuberance of
Shaft Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 975 pediatric fracture healing and the potential for
Proximal Phalanx Base Fractures . . . . . . . . . . . . . . . . . . . . 977 remodeling make nonoperative management
Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 977 the mainstay of treatment for most pediatric
phalanx fractures. However, rapid fracture
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 978
healing also results in a narrowed window for
treatment. Therefore, the physician must iden-
tify those fractures that require special scrutiny
and a low threshold for operative intervention.
This chapter seeks to provide hand surgeons
with a detailed overview of the epidemiology,
pathoanatomy, evaluation, and treatment of
specic phalanx fractures in the pediatric pop-
ulation. There is an emphasis on fracture sub-
types that can become particularly problematic
if the diagnosis is missed or initiation of treat-
R. Feller (*) A. Kluk J. Katarincic ment is delayed. These include Seymour
Department of Orthopedic Surgery, Brown University,
fractures, condylar fractures and other intra-
Rhode Island Hospital, Providence, RI, USA
e-mail: ross_feller@brown.edu; augusta.whitney@gmail. articular fractures, phalangeal neck fractures,
com; juliakatarincic@aol.com; julie_katarincic@brown.edu and fractures with resultant malrotation.
# Springer Science+Business Media New York 2015 961
J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_44
962
Although beyond the scope of this chapter, the
evaluation and treatment of associated soft tis-
sue injury deserve equal attention.
Introduction
The hand is the major medium that children utilize
to experience their surrounding world. Conse-
quently, the hand is the most commonly injured
part of the body in children (Hastings and
Simmons 1984). Younger children more fre-
quently suffer crushing injuries to the distal pha-
lanx, as can occur when getting stepped upon or
accidentally closed in a door. Older children and
adolescents begin to participate in aggressive con-
tact sports and frequently sustain injury to their
hands. As such, the most common bony nger
injuries in children are fractures of the distal pha-
lanx (e.g., tuft fracture), with fractures of the
base of the proximal phalanx representing the
second most common location (Mahabir
et al. 2001; Rajesh et al. 2001). Children have an
amazing ability to heal rapidly compared to the
adult population. This disparity in fracture healing
results from the robust pediatric periosteum pro-
viding both structural and osteogenic support, the
presence of physes, and the relative lack of med-
ical comorbidity compared to the adult popula-
tion. However, with this accelerated healing
potential comes a narrowed window to treat or
alter treatment of phalangeal fractures. Within a
week, the option of closed reduction may be lost.
If injuries are initially unrecognized or the incor-
rect treatment path is initiated, fracture malunion
is likely to start developing as early as 2 weeks
following the injury. By 3 weeks after the injury, a
signicant amount of healing is likely to have
taken place, even in the older adolescent popula-
tion. As such, emergency physicians and other
rst-line providers must be aware of the necessity
for swift referral to a hand surgeon for denitive
evaluation and prompt initiation of treatment.
The vast majority of nger injuries in children
are best managed nonoperatively. Fortunately,
residual stiffness is also much less of a concern
when treating pediatric hand injuries. Cast immo-
bilization of the nger, including adjacent ngers
R. Feller et al.
or even the entire hand for several weeks, can be
employed with less concern of chronic stiffness than
when treating hand injuries in adults (Cornwall and
Ricchetti 2006). However, one must be aware of
several classes of fractures that require increased
scrutiny of reduction and a lower threshold for
prompt surgical treatment in order to prevent
permanent deformity and dysfunction. These prob-
lematic injuries include fractures with resultant
malrotation, Seymour fractures, phalangeal neck
fractures, intercondylar fractures, and other
displaced intra-articular fractures (Waters 2010).
It is important to note that interphalangeal joint
dislocations are often seen in conjunction with
phalanx fractures. Associated soft tissue and
neurovascular injuries must always be assessed
for in conjunction with treatment of the bony
injury. The assessment of the nail and its associated
structures deserves close attention as well.
The following chapter will provide an over-
view of the epidemiology, pathoanatomy, and ini-
tial evaluation of pediatric phalanx fractures.
Detailed and current treatment strategies will be
presented for each type of phalanx fracture, includ-
ing the problematic subsets mentioned above.
Epidemiology
The high incidence of pediatric hand fractures is
secondary to the fact that a growing child uses his
or her hand as an instrument to explore the sur-
rounding world, making it very vulnerable to
injury. It is for this reason that hand fractures
make up approximately 25 % of all pediatric frac-
tures (Grad 1986). A biphasic age distribution
exists as a result of different injury mechanisms,
including crush injuries in toddlers and
schoolyard or athletic contact injuries in the
older child and adolescent population. The annual
incidence of pediatric phalanx fractures is quoted
as 26.4 fractures per 10,000 children and peaks at
age 13 (Worlock and Stower 1986). However, this
data is likely outdated, as it does not reect the
increasing percentage of participation in youth
athletics. In addition, progressively younger age
segments are starting to compete in both individ-
ual and team sports. As an example, new
43 Pediatric Phalanx Fractures: Evaluation and Management 963

Fig. 1 Distribution of hand

fractures by age. Note the
relative lack of bimodal age
distribution previously
described (From Chew
2012)

prospective data emerging from the UK suggests

that after the rst decade of life, the incidence of
hand fractures and other injuries may climb as
much as 20-fold from previously quoted rates
(Vadivelu et al. 2006). Historically, males
accounted for nearly two thirds of all phalanx
fractures. However, the sex distribution of injuries
may also be changing as more females are partic-
ipating in contact sports. Figure 1 demonstrates
the distribution of hand fractures by age group.
The most common phalanx fractures involve
the border digits, namely, the index and small
nger rays (Fig. 2). The distal phalanx is the most
common location for a non-physeal injury which
typically involves a crushing mechanism, and the
most common location for physeal injury is the
proximal phalanx. Physeal injuries are usually
Salter-Harris II or III fractures. Diaphyseal frac-
tures are more likely to occur in older populations,
whereas younger patients are more likely to incur
injury to the metaphysis and/or epiphysis. There is
no difference in the incidence between right and
left-sided hand injuries in children. Fig. 2 Distribution of hand fractures by bone. Note the
predominance of injury to the index and small nger rays
(From Chew 2012)
Pathoanatomy and Applied Anatomy
fracture of the bone before failure of ligaments
The anatomy and tissue composition of the pedi- and other soft tissues. A sound understanding of
atric hand differ considerably from the adult. The the pertinent anatomical bony and soft tissue
viscoelasticity of pediatric tissue results in structures is integral in order to perform an initial
964 R. Feller et al.

Fig. 3 Appearance (A) and Middle and distal phalanges

fusion (F) of secondary A 16-36 mos
centers of ossication in the F 14-16 yrs
pediatric hand (From
Bucholz et al. 2006)
Proximal phalanx
A 10-24 mos
F 14-16 yrs
Metacarpal head
A 12-27 mos
F 14-16 yrs
(small digit later)

Thumb metacarpal
A 24-36 mos
F 14-16 yrs

evaluation and develop an appropriate treatment
plan for any phalanx fracture or other pediatric
hand injury. One must also have an intimate
understanding of both physeal and metacarpo-
phalangeal/interphalangeal joint anatomy and the
locations of attachment of soft tissues, i.e., ten-
dons and ligaments. Particularly with distal pha-
lanx fractures, a rm knowledge of the nail and its
supporting structures is of the utmost importance.
Osseous Anatomy
Although potential epiphyses exist at both the
proximal and distal ends of the metacarpal and
phalanx, secondary centers of ossication develop
in the proximal end of the phalanx (i.e., the base)
and the distal end of the nger metacarpals (i.e.,
the head). This is in distinction to the thumb, in
which the secondary center of ossication arises
at the base of the metacarpal. Phalangeal physes in
the hand remain open in boys and girls until
approximately 16 and 14 years, respectively
(Hastings and Simmons 1984). Figure 3
demonstrates the appearance of secondary ossi-
cation centers in the male and female patient,
including the age at which fusion occurs to the
primary centers of ossication.
Physeal Anatomy
The zone of hypertrophy (zone III) of the physis is
an area where cellular hypertrophy results in a
relative deciency of extracellular matrix. It is
the weakest layer of the growth plate, and as a
result, Salter-Harris fractures tend to involve this
zone. In zones I and II that abut the metaphysis,
collagen provides a supportive framework, while
in zone IV, provisional calcication resists patho-
logic forces (Torre 1988). In addition, the topog-
raphy of the growth plate becomes more irregular
as the patient progresses toward skeletal maturity.
As a result, fractures may propagate across several
zones, which may account for the partial growth
arrest seen in some fractures (OBrien 1984).
However, the physis has an excellent healing
potential deriving from an exuberant blood supply
43 Pediatric Phalanx Fractures: Evaluation and Management
from both enchondral and periosteal vessels
that have been shown to survive even in the
event of signicant displacement (Katarincic
et al. 2011).
Soft Tissue Anatomy
The pediatric periosteum is a hearty structure
that provides a rigid support to reduction. In
addition, the periosteum delivers a healing poten-
tial as it is well vascularized and provides a pop-
ulation of cells that may eventually become
osteoblasts when fracture healing is required.
However, the thick pediatric periosteum may
also become entrapped in the fracture site,
resulting in an inability to obtain an adequate
reduction.
The origin and insertion of tendons and liga-
ments are of particular importance in creating the
fracture patterns commonly seen in the pediatric
patient. At the level of the metacarpophalangeal
joint, the collateral ligaments attach nearly
entirely at the epiphyses of the adjacent metacar-
pal and proximal phalanx. This leads to a high
incidence of Salter-Harris type II and III fractures,
as the stronger collateral ligament avulses the
epiphysis. The palmar plate follows a similar
epiphyseal attachment pattern. In distinction, the
collateral ligaments and the palmar plate of the
proximal and distal interphalangeal joints have a
more broad-based insertion, extending beyond the
epiphysis and attaching to the metaphysis of the
distal segment as well. Thus, the physis at these
locations are somewhat protected from the avul-
sion mechanisms to which the proximal phalan-
geal physis is exposed (Crick et al. 1988). As a
rule, the extensor tendons, including the central
slip and terminal extensor tendon, insert on the
epiphysis of the proximal and distal phalanges,
respectively. The exor digitorum profundus ten-
dons and exor pollicis longus insert on the
metaphysis of the terminal phalanx, while the
exor digitorum supercialis (FDS) tendons
insert over the central 60 % of the middle phalanx
(Graham and Hastings 2000). Figure 4 shows the
soft tissue anatomy surrounding the metacarpo-
phalangeal (MCP) and interphalangeal joints.
965
Nail Anatomy
Although nail and nail bed injuries are discussed
in a separate chapter, these structures are often
injured in conjunction with phalanx fractures,
most commonly in association with distal phalanx
or tuft fractures. Whether the mechanism involves
a crush, laceration, or avulsion injury, there can be
signicant damage to the nail organ. If left
untreated, these injuries can result in a cosmetic
deformity as well as an increased risk of infection,
altered growth resulting in pain, and hygiene
issues (Egol et al. 2010). Figure 5 demonstrates
the distal phalanx and nail complex anatomy.
Remodeling in Pediatric Phalanx
Fractures
The pediatric patient has an incredible ability to
remodel phalanx fractures as long as 2 years of
growth or more remains. Angulation in the plane
of joint motion (i.e., exion-extension) is the best
tolerated and can readily remodel up to 30 in
children younger than 10 years of age and up to
20 after 10 years of age (Egol et al. 2010). Defor-
mity in the coronal plane (i.e., abduction-
adduction) also has the potential for remodeling,
although less so than sagittal plane deformity.
Residual rotational deformity following reduction
has little potential for remodeling and is thus an
indication for operative intervention. In addition,
as fractures occur farther from the physis
(i.e., more distal in the phalanx), the capacity for
remodeling decreases substantially. For example,
phalangeal neck fractures have a much poorer
chance for obtaining acceptable alignment follow-
ing completion of remodeling and healing.
Assessment
When initially evaluating a patient, the physician
should also ascertain what treatments have
already been initiated, including application of
tourniquets in the eld, providing pain
medications, administration of local/systemic
antibiotics including tetanus prophylaxis, and
966
Fig. 4 (a) Soft tissue attachments about the MCP joint.
Note that the collateral ligaments and palmar plate insert on
the epiphyseal portion of the proximal phalanx. (b) Soft
tissue attachments about the proximal interphalangeal
(PIP) joint. (c) Soft tissue attachments about the distal
interphalangeal (DIP) joint. The collateral ligaments and
palmar plates of these joints originate on the epiphysis of
the proximal segment and have dual insertion on the
most importantly application of local, topical, or
regional anesthesia which may make the physical
examination difcult to interpret. Any contamina-
tion of the wounds, especially marine or barnyard
R. Feller et al.
epiphysis and metaphysis of the distal segment. The exten-
sor apparatus attachments across the PIP joint and the DIP
joint (i.e., central slip and terminal tendon) insert on the
dorsal aspect of the distal epiphyseal segment, respec-
tively. The exor digitorum supercialis (FDS) and exor
digitorum profundus (FDP) tendons insert onto the
metaphysis of the middle and distal phalanx, respectively
(From Graham and Hastings 2000)
contamination, should be determined. The history
should also include the age, hand dominance,
sport participation, and/or any avocations or jobs
that the child may have. In addition, any prior
43 Pediatric Phalanx Fractures: Evaluation and Management
Fig. 5 Nail plate and nail bed anatomy and relationship to surrounding bony and soft tissue structures
injuries and their treatments or surgeries, their past
medical history including allergies and medica-
tions, and their social history including who lives
with them in the house should be obtained.
Physical examination of the injured child can
be challenging. Children can be generally distrust-
ful of an adult trying to touch or evaluate their
painful, swollen nger. The white coat phenom-
enon can certainly play a role in the interaction
between the physician and an older child or ado-
lescent patient, and stranger anxiety or separation
anxiety can provide a difcult roadblock to accu-
rate evaluation and diagnosis. Thus, the evalua-
tion begins upon entering the exam room by
observation of the patient. The physician should
be aware of the use or nonuse of certain ngers or
the entire extremity. Other signs of injury poten-
tially indicating child abuse should be investi-
gated. These include bruising and/or fractures in
various stages of healing, suspicious abrasions, or
burns, among others. The interaction between the
child and their parents or guardians should also be
observed for warning signs of abuse. One should
observe the injured digit for swelling, ecchymo-
sis, deformity, and limited motion, all of which
may be indicative of a fracture. Both the resting
position of the digit and its position during active
and passive motion should be examined. Residual
malrotation of the digit must not be overlooked, as
it is an absolute indication for operative
967
management. Overlapping of ngers and an
asymmetrical cascade compared to the contralat-
eral hand are clues to injuries with a malrotation
component. If swelling makes rotational align-
ment difcult to appreciate, observation of the
position of the nail plates with respect to one
another can provide useful information. Angular
alignment is more easily appreciated.
It is crucial to assess the neurovascular status of
the involved digit. This holds true for all injury
mechanisms, including closed crush injuries. In
terms of vascular compromise, one can look at
capillary rell, differences in temperature
between hands, and frank duskiness or color
change of the pulp. If necessary, a Doppler exam
can be performed to establish the presence of
digital arterial pulses distal to the injury. Finally,
pulse oximetry monitors can be applied to the
ngertip and the waveforms monitored for evi-
dence of vascular continuity.
Sensation and motor function are much harder
to elicit in the young pediatric patient. Sharp ver-
sus dull and 2-point discrimination does not
become a reasonable measurement of nerve func-
tion until about 5 years of age (Rang et al. 2005).
In younger patients who cannot or will not ver-
balize sensory function, the wrinkle test can be
employed. This test is performed by submerging
the hand or digit in warm water for 5 minutes with
subsequent examination of the volar nger pulp
968
for wrinkling, indicative of retained innervation.
The resting position of the digit can also provide
important clues to the diagnosis, as in the case of a
bony mallet nger. Passive wrist motion and
observation of the tenodesis effect also provides
important information. Obviously, active motor
function and range of motion are a helpful adjunct
in the examination. However, depending on the
age and maturity of the patient, he or she may be
unwilling to cooperate with this aspect of the
exam. It is important to be patient and repeat the
necessary components of the exam until the nec-
essary information is obtained.
Imaging of the injured digit should begin with
true anteroposterior (AP) and lateral radiographs.
Oblique views can be helpful, particularly when
evaluating the proximal phalanges that overlay
each other on a lateral view making detailed
assessment difcult. One must evaluate every
digit on hand lms so as not to miss injuries of
adjacent digits or phalanges. It is also important to
obtain a true lateral of the affected digit in isola-
tion, particularly when intra-articular fractures are
present.
Treatment of Specific Fracture Types
Distal Phalanx Fractures
Injury to the ngertip in the pediatric population
occurs with great frequency, particularly during
the toddler years. Associated soft tissue injuries
must be identied and treated concurrently in
order to optimize both functional and cosmetic
outcomes. The nail and its specialized structures
are particularly vulnerable in injuries of the distal
phalanx. This section will provide a discussion of
the following fracture types: extra-articular or tuft
fractures, Seymour fractures, and bony mallet n-
ger injuries.
Extra-articular Distal Phalanx Fractures
Distal phalanx tuft fractures are most common in
toddlers due to crush injuries. Most require only
symptomatic relief with analgesics, protection,
and splinting for several weeks. Patients may dis-
continue splinting and return to activity when
R. Feller et al.
comfortable. The associated soft tissue and nail
bed damage resulting from a ngertip crush typi-
cally becomes the focus of treatment in these
injuries. An associated subungual hematoma
greater than 50 % of the nail surface area should
be treated with nail plate removal for hematoma
evacuation and nail bed repair. Trephination with
a sterile needle or pin may be attempted if the
subungual hematoma involves less than 50 % of
the nail bed, as this can provide improved pain
relief (Fig. 6).
Seymour Fractures
First described by Seymour in 1966, the classic
Seymour fracture is a juxtaepiphyseal fracture of
the terminal phalanx with an associated laceration
of the nail bed, exion deformity, and ungual
subluxation (Seymour 1966). If the physis is
involved, as is typically the case in the pediatric
population, usually these will be Salter-Harris
type I or II fractures (Krusche-Mandl
et al. 2013). The proximal edge of the nail is
avulsed from the eponychial fold and usually pre-
sents as an open fracture (Barton 1979; Al-Qattan
2001a).
On physical exam, the ngertip droops and
takes on the appearance of a mallet nger as a
result of the exion deformity typically produced
at the fracture site. Clues as to the presence of this
injury pattern include frank exposure of the prox-
imal nail plate or in more subtle cases bleeding
present near the eponychial fold. The nail bed
laceration is typically not visible; however, the
proximal edge of the nail plate will sit on top of
the eponychial fold (Fig. 7). It is important to look
for bleeding around the eponychial fold even
when the proximal nail plate remains within the
nail fold because the underlying nail bed can still
be torn, representing an open injury.
On a true lateral radiograph, there is often
dorsal physeal widening. The epiphysis typically
remains intact while the metaphysis is angulated.
This distal segment will be exed.
This fracture requires surgical treatment to
avoid complications such as infection and growth
arrest. The proximal edge of the lacerated nail
matrix or nail fold is typically incarcerated in the
physis. This tissue needs to be removed from the
43 Pediatric Phalanx Fractures: Evaluation and Management 969

Fig. 6 Classication of
extra-articular distal
phalanx fractures. (a)
Transverse diaphyseal
fracture. (b) Longitudinal
splitting fracture. (c)
Comminuted distal tuft
fracture (From Kozin and
Waters 2006)

Fig. 7 Seymour fracture.

(a) Clinical photo
demonstrating avulsion of
the proximal nail plate and
bed from the eponychial
fold. One must be aware
that the clinical presentation
and/or radiographs may be
subtle. (b) In this case,
radiographs demonstrate
mild dorsal displacement
through the distal phalanx
physis. (c) As will be
explained in further detail in
the operative section,
irrigation and debridement
with K-wire xation were
eventually performed
(From Yeh and Dodds
2009)

fracture site to allow fracture reduction to Surgical Technique

occur. The nail plate must be removed to allow
for fracture irrigation and debridement, access to
the incarcerated tissue, and soft tissue
repair and/or reconstruction. For preoperative
considerations, refer to Table 1 below. This
checklist may be applied to the vast majority of
pediatric phalanx fractures; however, any unique
elements for specic fracture types will be
identied.
The administration of intravenous antibiotics
should be performed prior to beginning the pro-
cedure. Following tourniquet ination, the nail
plate is removed. This is accomplished by rst
sliding a freer underneath the nail plate, then
attaching a Kelly clamp, and applying a twisting/
pulling motion. Once removed, the nail plate
should be placed in saline and cleaned for later
placement in the nail fold for fracture protection at
970
Table 1 Preoperative considerations pertaining to the
vast majority of pediatric hand fractures
Preoperative checklist
Positioning Patient should be positioned supine with
an arm board attached to the OR table
Fluoroscopy Mini C-arm should be placed at the end
of the arm table so that the C-arm can be
placed in a perpendicular position and
brought in over the arm board
Equipment Freer elevator, Kelly clamp, small
curettes, dental pick, K-wires, K-wire
driver, C-arm, chromic suture, wire
cutter, heavy needle driver, arm or
forearm tourniquet
the end of the procedure. For adequate visualiza-
tion, it is often necessary to make longitudinal
incisions at the proximal corners of the nail fold
to allow for elevation and retraction. The lacera-
tion through the nail bed should now be visible,
and the entrapped tissue may be removed.
Hyper-exing the distal fragment will aid in
exposure of the fracture site. Any gross debris
and/or hematoma should be removed in the frac-
ture site with the use of dental picks and small
curettes. The site must be copiously irrigated, as
this constitutes an open injury.
The fracture should be reduced and transxed
with a Kirschner (K-) wire. Depending on the size
of the child, a 0.35 or 0.45 in. K-wire can be used.
The K-wire can either be placed anterograde
through the fracture site out the distal extent of
the nger prior to fracture reduction and then
passed retrograde across the proximal fragment,
or the wire can be placed entirely in a retrograde
fashion following fracture reduction. Whichever
technique is utilized, the wire should be driven
through the proximal phalanx and across the DIP
joint so that it is held in extension. The wire
should end in the subchondral bone of the middle
phalanx. At that point, fracture reduction and pin
placement are conrmed with uoroscopy. The
K-wire can either be cut beneath the level of the
skin for later removal in the operating room
(OR) or the wire can be left outside the skin,
bent 90 , and cut.
R. Feller et al.
Table 2 Key steps to the surgical treatment of Seymour
fractures
IV antibiotics must be administered, if not already done
so prior to surgery
Nail plate is removed, cleaned, and saved
Nail bed laceration and fracture site are identied and
irrigated/debrided
Fracture is reduced and transxed with 0.35 or 0.45 mm
K-wire
Conrm nal reduction and xation (pin should end in
subchondral bone of middle phalanx and DIP should be
held in extension)
Repair nail bed laceration and additional incisions with
6-0 chromic suture
Bacitracin, soft gauze, and Coban dressing is applied
Attention should now be turned to the nail bed
laceration. If the laceration is distal to the
eponychial fold, it should be repaired using 6-0
chromic suture. The longitudinal incisions at the
proximal aspect of the nail fold should also be
repaired using chromic suture. The nail plate that
was removed at the beginning of the case should
now be cleaned, trimmed if needed, and replaced
beneath the eponychial fold to provide support for
the fracture as well as maintain an open
eponychial fold. A dressing of bacitracin, soft
gauze, and Coban is applied to the digit prior to
tourniquet deation. Table 2 summarizes the key
surgical steps in the treatment of Seymour
fractures.
Postoperative Protocol
Soft gauze dressing and Coban should be
applied to the digit.
Weekly lateral radiographs should be obtained
for the rst 2 weeks to monitor fracture
stability.
K-wire should be removed at 34 weeks either
in the ofce or the OR depending on whether
or not the wire was buried.
In older children, stack splint immobilization
should be continued for 2 weeks and at night
time for fracture protection.
Return to sports is allowed at 45 weeks
postoperatively.
Thorough irrigation and debridement along with
antibiotic therapy are used to avoid infection;
43 Pediatric Phalanx Fractures: Evaluation and Management
Table 3 Summary of bony mallet finger injuries
The vast majority of these injuries can be managed
nonoperatively
Surgical indications include fractures involving greater
than 30 % of the articular surface, joint subluxation, and
irreducible fractures
Casting and/or prophylactic pin placement may be
required due to issues with noncompliance
Extension block pinning is a useful technique
which can be employed in cases where closed
reduction is difcult to obtain due to persistent dorsal
displacement
Weekly lateral radiographs must be obtained to ensure
continued joint reduction
however, fracture site infection/osteomyelitis can
be a dreaded complication of these open fractures
(Ganayem and Edelson 2005). Premature growth
plate closure can occur secondary to infection or
direct injury (Al-Qattan 2001a; Engber and
Clancy 1978).
Boney Mallet Fractures
The pediatric equivalent of the adult mallet nger
is a Salter III or IV fracture of the distal phalanx.
This results from an avulsion injury involving the
insertion of the terminal extensor tendon. These
injuries are most commonly seen in the teenage
population and represent an intra-articular injury.
Typically, the mechanism of injury is an
axial load or exion force applied to an extended
ngertip. These injuries can generally be treated
nonoperatively with closed reduction and
splinting. Wehbe and Schneider published a
large review of adult and pediatric mallet nger
injuries and suggested the results were uniformly
good, with surgical treatment offering no advan-
tage over nonoperative treatment. They
recommended splinting for nearly all cases
(Webbe and Schneider 1984). The main indica-
tions for operative treatment include fractures
involving greater than 30 % of the articular sur-
face, any evidence of joint subluxation, and rarely
irreducible epiphyseal-physeal injuries (Graham
and Hastings 2000).
Unique to the treatment of pediatric mallet
nger injuries is the question of patient
971
compliance with splinting. Splinting a pediatric
nger can be difcult since the digits tend to be
short and plump. While older children may be
capable of understanding the importance of
being compliant with splinting, it may be difcult
for younger children to grasp this concept.
If nonoperative treatment is chosen for a
boney mallet fracture in children, compliance
should be checked early and often in the course
of treatment. If compliance is an issue, a
transarticular K-wire can be placed through the
DIP joint and the hand can be casted to protect the
wire from breakage.
For preoperative planning, please refer to
Table 1.
Surgical Technique
Fracture reduction is performed by extending the
DIP joint of the affected digit. Following conr-
mation of reduction with uoroscopy, a single
K-wire is placed in a retrograde fashion through
the ngertip. The wire should cross the DIP joint
and end in the subchondral bone of the middle
phalanx. If the dorsal fracture fragment remains
displaced after K-wire xation, extension block
pinning may be a useful technique. First, back the
K-wire across the DIP joint so that it may be
exed. While exed, place a second K-wire in a
retrograde oblique direction passing just dorsal to
the fracture fragment and into the middle phalanx.
The fracture fragment will now remain reduced
as the DIP joint is extended and the original
K-wire can be passed proximally along its original
path. Pins can be cut beneath the level of the
skin for future removal in the operating room or
bent and cut outside the skin for removal in the
ofce.
In adolescents, a soft dressing should be
applied to the digit. In younger patients, a cast
should be applied to protect the K-wire from
breaking. Weekly lateral radiographs should be
obtained for the rst 2 weeks to monitor joint
reduction. K-wire removal is performed between
3 and 4 weeks in the ofce or operating room
depending on whether or not the wire was buried.
Table 3 provides a summary of the major sur-
gical indications and treatment considerations
pertaining to pediatric bony mallet injuries.
972
Phalangeal Neck Fractures
Fractures of the neck of the proximal or middle
phalanx occur in children. These fractures are
often displaced with dorsal angulation or transla-
tion. The adjacent interphalangeal joint is often in
hyperextension with a bony block to exion cre-
ated by the bony spike of the volar cortex of the
proximal fragment. This bony spike and
hyperextended posture essentially obliterate the
subcondylar recess. These fractures can also
have rotation of the distal fragment or coronal
plane malalignment. As mentioned previously,
the remodeling that occurs in these two planes is
substantially less than in the sagittal plane, mak-
ing these fractures problematic to treat
nonoperatively (Cornwall and Waters 2004).
True AP and lateral radiographs of the digit are
essential to accurately evaluate these fractures. If
true lateral radiographs are not obtained, the
extent of displacement can be easily
underestimated. Furthermore, the fracture can be
confused with a distal physis (secondary ossica-
tion center).
If phalangeal neck fractures can be reduced and
remain aligned, nonoperative treatment with
splint or cast immobilization may be acceptable
(Puckett et al. 2012). However, these fractures
have a high propensity to re-displace and percu-
taneous pin xation is often used to maintain the
reduction during bony healing (Karl et al. 2012;
Paksima et al. 2012). If the fracture presents late
and closed reduction is unable to be achieved,
open reduction and xation must be performed
(Cornwall 2012).
For preoperative planning, please refer to
Table 1.
Surgical Approach
Closed reduction should be attempted by rec-
reating extension at the fracture site and then
exing the distal interphalangeal joint.
The reduction should be veried on
uoroscopy.
If the reduction is acceptable, 2 crossed
K-wires are placed in a retrograde fashion
from the radial and ulnar sides of the distal
fragment, across the fracture site. In younger
R. Feller et al.
patients, single wire placement may be suf-
cient (Fig. 8).
The K-wires can be cut beneath the level of the
skin and buried for later removal in the OR or
left protruding from the skin and bent 90 with
a heavy needle driver for later removal in the
ofce.
If the fracture reduction is not acceptable after
an attempt at closed reduction, an attempt at
percutaneous manipulation should be
performed using a K-wire placed through the
skin dorsally into the fracture site and used as a
joystick to reduce the distal fragment.
If percutaneous attempts at reduction are
unsuccessful, a longitudinal incision should
be made on the dorsum of the digit after exsan-
guination of the limb with an Esmarch bandage
and forearm/arm tourniquet ination.
Dissection through the subcutaneous tissue to
the level of the extensor mechanism should be
performed. The extensor tendon should be
divided sharply to expose the fracture site.
With the fracture site visualized, 2 K-wires
can be placed in an anterograde fashion across
the distal fragment in an oblique direction. The
fracture is then reduced and the K-wires are
driven retrograde across the fracture site into
the proximal fragment. Alternatively, the frac-
ture can be reduced and crossed K-wires driven
across the fracture in a retrograde fashion from
a percutaneous starting point.
Once the fracture is reduced and adequate x-
ation is veried on uoroscopy, the wound
should be irrigated and the extensor tendon
closed with interrupted 4-0 Vicryl sutures.
The skin should be closed with either absorb-
able or nonabsorbable suture. The younger the
age of the child, the more appealing
nonabsorbable suture is to eliminate the need
for suture removal in the ofce, which can be
traumatic for the pediatric patient (Table 4).
Phalangeal neck fractures are thought to have
little remodeling potential given their distance
from the physis (Barton 1979; Al-Qattan 2001b).
There have been case reports of remodeling in the
sagittal plane. A corrective osteotomy can be
43 Pediatric Phalanx Fractures: Evaluation and Management
Fig. 8 (a) Preoperative radiograph demonstrating a
displaced neck fracture of the middle nger proximal
phalanx. (b) Postoperative appearance following closed
reduction and percutaneous pinning. (c) Films at time
performed for malunited phalangeal neck frac-
tures; however, this poses the potential risk of
avascular necrosis of the condyles.
Complications
Phalangeal neck fractures are thought to have little
remodeling potential given their distance from the
973
of wire removal and (d) at nal follow-up. (e and f)
Full exion and extension were maintained (From
Al-Qattan 2001b)
physis (Barton 1979; Al-Qattan 2001b).
Malunion is a problematic complication seen
after closed treatment of these injuries. Corrective
osteotomy can be performed for malunited pha-
langeal neck fractures; however, this poses the
potential risk of avascular necrosis of the con-
dyles. Osteotomy can be a challenging
974
Table 4 Key steps in the surgical management of phalan-
geal neck fractures in children
Reduction is achieved by recreating hyperextension,
followed by exion of the distal fragment and DIP
Two crossing K-wires should be utilized for xation and
can be passed retrograde while the interphalangeal joint is
held in exion
Joysticking can be employed with difcult reductions
Open reduction and xation utilize a extensor tendon
splitting versus paratendinous approach
Anterograde wire placement followed by reduction and
retrograde passage versus primary retrograde passage
may be employed with open approaches
Extensor apparatus must be repaired following ORIF
(Open Reduction Internal Fixation)
Immobilization with forearm-based intrinsic-plus splint
or cast is recommended until K-wire removal
proposition given the distal location of the frac-
ture, leaving limited bone for xation. If new bone
has formed obliterating the subcondylar fossa, this
bone will need to be removed to restore exion.
Simmons and colleagues have described a tech-
nique to remove this excess bone from the
subcondylar fossa of phalangeal neck malunions.
They describe using a zigzag volar incision cen-
tered over the PIP joint to access the excess bone.
The boney block to exion is removed using
either a rongeur or burr until satisfactory exion
is restored to the PIP joint (Simmons and Peters
1987).
Nonunion with or without avascular necrosis is
also a rare complication. Al-Qattan published a
review of pediatric phalangeal neck fractures
complicated by nonunion with or without avascu-
lar necrosis (Al-Qattan 2010). He concluded that
nonunion without avascular necrosis is most often
seen in the thumb, resulting in instability requiring
bone grafting. In contrast, avascular necrosis is
typically seen in the small nger and results in
stiffness rather than joint instability (Fig. 9).
These patients were successfully managed
nonoperatively. They were able to identify reduc-
tion with K-wire xation by an unsupervised
junior surgeon as a risk factor for avascular necro-
sis. This may be related to excessive manipulation
and multiple passes of the K-wire to obtain ade-
quate reduction and xation. Limiting the number
of reduction attempts may be helpful in avoiding
R. Feller et al.
avascular necrosis as a complication when
treating these injuries.
Table 5 lists the potential pitfalls associated
with the management of phalangeal neck fractures
in children.
Condylar Fractures
Condylar fractures of the proximal and middle
phalanges are the result of either avulsive forces
of the collateral ligaments secondary to varus or
valgus stress or a combination of axial load and
shear across the joint surface. These fractures may
be unicondylar or bicondylar, and as with any
axial mechanism, there may be a signicant
amount of articular depression or displacement.
It is important to recognize upon initial evaluation
that most of these fractures will be inherently
unstable and require operative xation (Day and
Stern 2011). If nonoperative treatment is initiated,
weekly radiographs must be obtained in order to
ensure that there is no articular displacement or
angulation, as malunion will result in future
osteoarthrosis.
For details on preoperative planning, please
refer to Table 1.
Surgical Technique
A variety of approaches are utilized to treat these
fractures. A dorsal incision can be used to perform
a tendon splitting or peritendinous approach (i.e.,
between the extensor mechanism and the lateral
band). However, there are advocates of a lateral
approach in which the periosteum and extensor
mechanism are elevated en bloc. This is
recommended as a measure to avoid scarring and
adherence between these two layers as a result of
dissection (Shewring et al. 2013). Whichever
approach is used, it is imperative that exposure
provides adequate view of the joint surface.
Fixation may be achieved through the use of
K-wires or miniscrews. A single K-wire has been
shown to provide suboptimal stabilization with a
high rate of displacement during healing, whereas
two or more wires reliably provide adequate
stabilization and rotational control (Weiss and
Hastings 1993). However, multiple wires may
43 Pediatric Phalanx Fractures: Evaluation and Management
Fig. 9 Avascular necrosis of the small nger proximal
phalanx following reduction and xation of a displaced
proximal phalangeal neck fracture. Often these injuries are
Table 5 Complications associated with the management
of pediatric phalangeal neck fractures
Malunion May require corrective osteotomy,
particularly if a bony block to
exion exists
Avascular Most often seen in the small nger,
necrosis (AVN) results in signicant joint stiffness
Nonunion Most often seen in the thumb,
results in signicant joint instability.
Requires xation with bone grafting
Iatrogenic AVN Osteotomy and excessive
manipulation/multiple K-wire
passes may lead to avascular
necrosis of the condyles
not be possible in smaller fracture fragments and
comminution may result from multiple attempts at
placement or not having a predrilled hole, as is the
case with screws. Advocates of screw xation also
cite the inability of K-wires to provide compres-
sion across the fracture site. With that being said,
the use of two or more K-wires provides adequate
xation and leads to excellent results as long as
congruity of the articular surface is maintained
(Fig. 10).
975
characterized by joint stiffness rather than instability (From
Al-Qattan 2010)
Key concepts related to the surgical manage-
ment of condylar fractures in children are
presented in Table 6.
Shaft Fractures
Diaphyseal fractures of the proximal and middle
phalanges behave similarly in the child and adult.
They are less common than fractures at the prox-
imal or distal end of the phalanx. The character-
istic displacement in the proximal phalanx is
apex volar as a result of three distraction forces.
Extension of the distal fragment results from
the insertion of the central slip and the
dorsal course of the lateral band relative to
the fracture axis. Flexion of the proximal
fragment results from the action of the intrinsic
muscles on the MCP joint (Graham and Hastings
2000).
Most of these fractures can be managed
nonoperatively with closed reduction and
splinting in the intrinsic-plus position for 34
weeks. However, there are subsets of fractures
976
Fig. 10 (a) Unicondylar fracture of the ring nger prox-
imal phalanx in a 15-year-old male. (b and c) Open reduc-
tion and xation were achieved with the use of two K-wires
placed parallel to the articular surface, providing both
rotational control and a buttress to prevent subsidence.
that may require surgical intervention. These
include fractures with resultant malrotation,
unstable fracture patterns (short oblique or trans-
verse), and inadequate alignment following
closed reduction which may all require closed
R. Feller et al.
(d) Radiographs following fracture healing demonstrating
a well-maintained articular surface (Courtesy of Dr. Julia
Katarincic, Department of Orthopaedics, Brown Univer-
sity, Rhode Island Hospital)
versus open reduction and xation. In
children under ten years of age, 30 or less of
angulation is tolerated, whereas less than 20 of
angulation is tolerated in older children (Egol
et al. 2010).
43 Pediatric Phalanx Fractures: Evaluation and Management
Table 6 Key concepts related to the surgical management
of condylar fractures
Open reduction and xation will often be necessary to
achieve adequate articular reduction
An extensor tendon splitting versus peritendinous
approach may be utilized; however, one must ensure
adequate visualization of the joint surface
K-wires or miniscrews may be utilized for xation. If
possible, two K-wires should be placed parallel to the
articular surface to provide for both rotational control and
to act as a buttress to prevent subsidence of the fracture
condyle. As these are unstable injuries, wire should not
cross at the fracture site
Advocates of miniscrew xation cite the added ability to
provide compression across the fracture site
Proximal Phalanx Base Fractures
Fractures at the base of the proximal phalanx are
very common pediatric injuries and most typically
involve the small nger. In most series, they rep-
resent the most common fracture in the pediatric
hand (Graham and Hastings 2000). These injuries
result from a combination of axial load and rota-
tion or pure hyperextension. Historically, they are
termed extra-octave fractures because the typi-
cal abduction deformity would allow the child to
reach an additional octave on the piano if left to
heal unreduced. Most of these fractures are Salter
II injuries, but some can involve fractures through
the epiphysis as well. Fortunately, in most of these
cases, the portion of articular involvement is rel-
atively small and articular displacement following
reduction is rare. In addition, the potential for
remodeling is substantial secondary to their prox-
imity to the physis and the multiplanar motion of
the MCP joint. Growth arrest is an uncommon
complication.
For these multiple reasons, closed reduction
and casting is adequate treatment in the majority
of cases. Reduction is performed by exing the
MCP joint and applying a radial or ulnar force to
the distal fragment. Flexion of the MCP with
resulting ligamentotaxis aids in reduction. Opera-
tive management is indicated if the fracture
involves greater than 25 % of the articular surface
or if there is greater than 1.5 mm displacement of
the joint surface following reduction (Segmuller
and Schonenberger 1980). There have been
977
reports of failure of closed reduction secondary
to entrapment of soft tissue structures
within the fracture site, such as the exor tendon
or extensor hood (Cowen 1975; Nogueira
et al. 1999). Therefore, one must maintain a high
index of suspicion for this occurrence if multiple
attempts at closed reduction fail and be prepared
to utilize open reduction in those instances
(Fig. 11).
In cases where open reduction must be
employed, a dorsal or dorsolateral incision is uti-
lized. A tendon splitting versus paratendinous
approach may then be used depending on fracture
pattern and surgeon preference. Whatever
approach is used, soft tissue repair and recentra-
lization of the extensor mechanism is of vital
importance.
Table 7 provides a summary of the manage-
ment of pediatric proximal phalanx base fractures.
Summary
The hand is particularly vulnerable to injury in the
child as a direct result of its role as a tool to
explore the surrounding world. The classic
bimodal age distribution of these injuries may be
changing as participation in youth athletics con-
tinues to increase and progressively younger age
segments become involved. Pediatric hand anat-
omy and fracture healing differ considerably
from the adult. One must have an intimate under-
standing of these differences in order to effec-
tively diagnose and treat phalangeal fractures in
children. Although this chapter focused on frac-
ture management, it is vital that the physician
address soft tissue injury as well.
Assessment of the pediatric patient can be
challenging and examination requires keen obser-
vation and the ability to modify typical maneuvers
and tests in order to elicit key information. The
vast majority of pediatric phalanx fractures can be
managed nonoperatively, namely, with closed
reduction and immobilization. Compliance with
splinting is a denite concern particularly with
younger patients, and one should have a low
threshold to utilize casting. Fortunately, immobi-
lization results in less stiffness in the child and can
978
Fig. 11 (a) Salter-Harris II fracture of the middle and ring
nger proximal phalanx with multiple failed attempts at
closed reduction. (b) Intraoperative photograph
Table 7 Summary of concepts related to the management
of proximal phalanx base fractures
Most commonly involves the small nger
Flexion of the MCP with resulting ligamentotaxis will aid
in closed reduction, and the hand should be immobilized
in the intrinsic-plus position
Operative management is indicated in cases with residual
displacement greater than 1.5 mm or fractures involving
greater than 25 % of the articular surface
Failure of closed reduction may be related to entrapment
of a number of soft tissue structures
therefore be employed until the physician is con-
dent that complete fracture healing has occurred.
The supreme healing and remodeling potential
of fractures in this population poses the potential
threat of suboptimal outcomes if injuries are not
identied and treated in a timely fashion. Along
those same lines, throughout this chapter, there has
been an emphasis on injuries that deserve special
consideration and frequently surgical intervention
in order to achieve a successful outcome.
Despite their frequent occurrence, there is a
relative paucity of evidence to support current
approaches to treatment of phalanx fractures in
children. Prospective data obtained from random-
ized trials must be generated in order to advance
the understanding of these injuries and their ideal
treatment.
R. Feller et al.
demonstrating entrapment of the FDP tendon in the frac-
ture site (From Rodriguez-Vega 2013)
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 Metacarpal Fractures
44
Shannon Cassel and Apurva S. Shah

Contents Base of Thumb Fractures . . . . . . . . . . . . . . . . . . . . . . . . . 1000

Preferred Method . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1001
Introduction to Pediatric
Metacarpal Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 982 Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1003
Pathoanatomy and Applied Anatomy Relating References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1003
to Pediatric Metacarpal Fractures . . . . . . . . . . . . . . . 983
Assessment of Pediatric Metacarpal Fractures . 983
Associated Injuries . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 985
Classication . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 988
Outcome Tools . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 989
General Surgical Planning . . . . . . . . . . . . . . . . . . . . . . . . 989
Epiphyseal and Physeal Injuries, Metacarpal
Head Fractures, and Metacarpophalangeal
Joint Dislocations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 991
Preferred Method . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 992
Metacarpal Neck Fractures . . . . . . . . . . . . . . . . . . . . . . 993
Preferred Method . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 995
Metacarpal Shaft Fractures . . . . . . . . . . . . . . . . . . . . . . 995
Preferred Method . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 998
Metacarpal Base Fractures and Reverse
Bennett Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 998
Preferred Method . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1000

S. Cassel A.S. Shah (*)

Department of Orthopaedics and Rehabilitation,
University of Iowa Hospitals and Clinics, Iowa City,
IA, USA
e-mail: shannon-cassel@uiowa.edu;
apurva-shah@uiowa.edu

# Springer Science+Business Media New York 2015 981

J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_45
982 S. Cassel and A.S. Shah

Abstract
Pediatric metacarpal fractures are commonly
encountered injuries and range from
nondisplaced fractures treated best with cast-
ing in situ to complex open injuries with soft
tissue compromise requiring complex recon-
struction. This chapter will cover the basic
epidemiology of these fractures, clinical exam-
ination and diagnostic imaging pearls, and
appropriate indications for operative versus
conservative management of all metacarpal
fracture subtypes. Additionally, this chapter
will review, in brief, human bite injuries and
hand compartment syndrome as possible asso-
ciated injuries.

Introduction to Pediatric Metacarpal

Fractures

Metacarpal fractures are a common fracture in the

pediatric population. They are generally
observed in a bimodal fashion, affecting toddlers
and adolescents most frequently (Armstrong
et al. 2003). In a retrospective investigation of
all hand injuries presenting to a pediatric emer-
gency department, it was found that approxi-
mately 19 % of all pediatric hand injuries were
fractures. The small metacarpal was the most
frequently fractured bone (Fig. 1). This investi-
gation also found that boys were more likely than
girls to sustain hand injuries and that hand frac-
tures were often related to sporting injuries or
direct blows with a clenched st (Bhende
et al. 1993). Another study retrospectively
reviewed nearly 3,900 metacarpal fractures and
found the highest incidence of these injuries to
occur in males, 1029 years of age (2.5 %). The
most common mechanism in this group, as well
as children less than 9 years of age, was a fall
onto the hand, which accounted for approxi-
mately 39 % of all metacarpal fractures in chil-
dren less than 9 years of age (de Jonge
et al. 1994). A Canadian population-based
study found that the most common age for a
hand fracture was 14 years for males and
Fig. 1 PA radiograph of a small nger metacarpal neck
fracture in a 14-year-old boy after a fall onto the right hand
13 years for females but found that peak inci-
dence did not occur until 2024 years in males
and 8084 years in females (Feehan and Sheps
2006). Although incidence varies by geographic
region, metacarpal fractures appear to comprise
2040 % of pediatric hand fractures (Feehan and
Sheps 2006; Hastings and Simmons 1984). The
most commonly fractured metacarpal is the small
metacarpal (Rajesh et al. 2001; Worlock and
Stower 1986; Tables 1 and 2). Approximately
15 % of metacarpal fractures occur in patients
aged 014 years (Chung and Spilson 2001).
Unlike some other pediatric upper extremity
injuries, metacarpal fractures are frequently
treated similarly in children and adults, with a
few exceptions. This chapter will review
the anatomy related to pediatric metacarpal
fractures, classication of various fractures,
appropriate assessment, and recommended
treatments.
44 Metacarpal Fractures 983

Table 1 Distribution of pediatric hand fractures by digit younger children, fractures adjacent to the physis,
(Hastings and Simmons 1984) and when the deformity is in the plane of motion
Digit Estimated relative frequency of the sagittal plane in the hand. Children under age
involved fracture ten may remodel fractures up to 2030 in the
Thumb 20 % sagittal plane, while children older than age ten
Index 16 % may remodel fractures up to 1020 in the sagittal
Long 16 %
plane (Kozin and Waters 2010). Knowledge of the
Ring 18 %
physeal anatomy of the hand is crucial to under-
Small 30 %
standing the treatment and outcomes of pediatric
metacarpal fractures. The index, long, ring, and
small metacarpals have epiphyses at the distal
Table 2 Distribution of pediatric hand fractures by bone aspect of the bones; however, the thumb metacar-
(Hastings and Simmons 1984; Worlock and Stower 1986) pal has an epiphysis at the proximal end (Lindley
Estimated relative frequency of and Rulewicz 2006). Secondary ossication cen-
Bone involved fracture ters are located at the distal ends of the metacar-
Distal phalanx 19 % pals of the index, long, ring, and small ngers.
Middle phalanx 18 % The secondary ossication center for the thumb
Proximal 43 % metacarpal is located at the proximal end of the
phalanx bone (Lindley and Rulewicz 2006; Fig. 2). The
Metacarpal 20 %
nger metacarpal secondary ossication centers
Neck 65 %
appear at 1227 months of age and close in late
Shaft 8%
adolescence, at approximately aged 1415 years
Base 27 %
in girls and aged 1617 years in boys (Lindley and
Rulewicz 2006). The thumb metacarpal second-
ary ossication center appears at 2436 months of
Pathoanatomy and Applied Anatomy age and closes by 1416 years of age (Gruelick
Relating to Pediatric Metacarpal 1959; Stuart et al. 1962).
Fractures Physeal fractures are common in the pediatric
hand. The anatomy of the collateral ligaments
Metacarpal fractures arise from axial loads or leads to Salter-Harris II and III fractures of the
bending forces, typically sustained during sport- distal end of the metacarpals (Bogumill 1983).
ing injuries, falls onto the hand, or direct blows The ulnar and radial collateral ligaments originate
with a clenched st. Metacarpal fractures typically at the metacarpal epiphysis and insert on the prox-
result in apex dorsal angulation due to the line of imal phalangeal epiphysis (Bogumill 1983). Spe-
pull of the intrinsic muscles of the hand. The thick cic physeal fracture patterns involving the
periosteum surrounding pediatric metacarpals metacarpals will be discussed in subsequent
may minimize fracture displacement and facilitate sections.
closed reduction. In some cases, the periosteum
may also become entrapped in the fracture site
making anatomic reduction difcult or impossi- Assessment of Pediatric Metacarpal
ble. Specically in the metacarpals, the perios- Fractures
teum provides a smooth surface for tendon
gliding and speeds bone healing (Kozin and Clinical assessment of any pediatric injury can be
Waters 2010). challenging, particularly assessment of the pedi-
Pediatric fractures of all kinds are unique in atric hand. In addition to a thorough
their ability to remodel over time. Greater neurovascular examination, metacarpal injuries
remodeling potential has been observed in should always be evaluated for rotational
984 S. Cassel and A.S. Shah

Fig. 2 PA radiograph of
the left hand of a 6-year-old
girl demonstrating normal
ossication centers.
Secondary ossication
centers are located at the
distal ends of the
metacarpals of the index,
long, ring, and small
ngers. The secondary
ossication center for the
thumb metacarpal is located
at the proximal end of
the bone

deformity. Irreducible rotational deformity may

inuence treatment decisions and be an appro-
priate indication for operative treatment. To
assess rotational deformity, the digital cascade
is observed in exion. If the patient cannot com-
ply with active exion of the digits, tenodesis by
passive wrist extension can be used to evaluate
the digital cascade. The alignment of the nail
plates of the ngers should be examined with
the ngers exed and extended and compared
to the contralateral hand. Subtle changes of the
rotational alignment of the nail plates may also
indicate malrotation (Freeland and Lindley
2006; Figs. 3 and 4). Approximately 510 of
malrotation is typically evident on clinical
examination and may be enough to cause
dysfunction (Lindley and Rulewicz 2006).
All metacarpal fractures should be evaluated
with posteroanterior (PA), lateral, and oblique
lms of the hand. When obtaining an oblique
radiograph, it is important to recognize that posi-
tioning the hand in supination permits better
Fig. 3 Patient with a long nger metacarpal fracture and
visualization of the second and third metacarpals resultant malrotation (excessive pronation) of the long
and positioning the hand in pronation permits nger
44 Metacarpal Fractures
Fig. 4 Subtle representation of malrotation in patient with
ring nger metacarpal fracture and slight scissoring of the
ring nger under the long nger
better visualization of the fourth and fth
metacarpals. This is particularly important
because subtle fracture-dislocations of the
fourth and fth carpometacarpal joints are dif-
cult to detect on routine radiographs. When
reviewing hand lms, the phalangeal line test
can be used to identify subtle injuries (Campbell
1990). On a PA hand radiograph, the line drawn
from the center of the phalangeal neck through
the center of the phalangeal metaphysis at the
level of the physis should pass through the
center of the metacarpal or phalangeal
head regardless of joint exion. If this line is
disrupted, there is likely an injury present
(Campbell 1990; Fig. 5). It is important to rec-
ognize that subtle malrotation is not detectable
on radiographs. As a consequence, emphasis
on careful clinical examination cannot be
overstated.
Specialized radiographs may be helpful
in specic clinical scenarios. A Brewerton view
(Fig. 6) can be helpful in assessing metacarpal
head fractures or other articular injuries of
the metacarpophalangeal joint. In order to
obtain the Brewerton view, the dorsal aspect of
985
Fig. 5 Phalangeal line test demonstrating normal align-
ment of the thumb, index, and long digits with interruption
of the phalangeal line in the ring and small digits due to
metacarpal shaft fractures
the hand is placed against the cassette with
the metacarpophalangeal joints exed to
65 . The central beam is then angled 15
to the ulnar side of the hand (Lane 1977;
Fig. 7a, b).
Associated Injuries
In order to fully evaluate the patient with a meta-
carpal fracture, one must be cognizant of two
specically associated injuries human bite inju-
ries and hand compartment syndrome. Both of
these injuries can have devastating consequences
if the diagnosis is missed or delayed.
Human bite injuries are commonly associated
with metacarpal head fractures or metacarpal neck
fractures. Occasionally, bite injuries may not be
986
Fig. 6 Brewerton view of a Salter-Harris III fracture of the
long nger metacarpal head in a 13-year-old boy who fell
onto a clenched hand while snowboarding. The patient had
a 15 rotational deformity and was indicated for surgery
Fig. 7 (a, b) Photographs demonstrating the proper tech-
nique to obtain Brewerton view of the metacarpal head.
The dorsal aspect of the hand is placed against the cassette
S. Cassel and A.S. Shah
associated with any fracture. The incidence of
these injuries is not well known as patients may
only present these injuries if they are associated
with pain from a fracture or with a deep infection
(Shoji et al. 2013). Bite injuries may be incurred in
a variety of ways but commonly are due to a
clenched st contacting another persons mouth.
This so-called ght bite often involves a lacera-
tion through the skin at the level of the metacarpo-
phalangeal joint. In this situation, the laceration
occurs with the metacarpophalangeal joint in ex-
ion, but with extension of the joint, the skin lac-
eration may no longer align with injuries to the
deeper structures. Violation of the metacarpo-
phalangeal joint capsule is typically distal to the
skin wound, while any injury to the extensor
mechanism is usually located proximal to the
skin wound (Bunzli et al. 1998). These injuries
can lead to inoculation of the joint with oral bac-
teria resulting in complications such as septic
arthritis, tenosynovitis, or osteomyelitis. Patients
presenting with these injuries may not be forth-
coming with the details or etiology of the injury
(Shoji et al. 2013). For these reasons, all skin
lacerations near the metacarpophalangeal joint
should be considered ght bites until proven oth-
erwise. All of these injuries should also be evalu-
ated with radiographs. Radiographs may
with the metacarpophalangeal joints exed to 65 . The
central beam is then angled 15 to the ulnar side of the hand
44 Metacarpal Fractures 987

Fig. 8 (a, b) PA and oblique radiographs of a right ring demonstrate osteolysis of the metacarpal head suggestive
nger metacarpophalangeal joint ght bite in a patient who of osteomyelitis. The patient also had incompetence of the
presented 3 weeks after the original injury. Radiographs ring nger extensor mechanism

demonstrate an underlying fracture or foreign

body (possibly a retained tooth fragment) or, in
the case of delayed presentation, may show signs
of osteomyelitis and destruction of the metacarpal
head (Chadaev et al. 1996; Fig. 8a, b). Fight bite
injuries should be treated with urgent irrigation
and debridement of the wound and the metacarpo-
phalangeal joint. This can be performed in the
emergency room setting or in the operating
room. All patients should have an up-to-date tet-
anus vaccination and be placed on prophylactic
oral antibiotics for 35 days to cover Staphylo-
coccus, Streptococcus, Eikenella, Corynebacte-
rium, and anaerobes (Bunzli et al. 1998). If a
patient presents in a delayed fashion or with an
actively infected human bite, he or she should be
taken to the operating room for irrigation and
debridement (Fig. 9). In these cases, the wound
should be left open to drain, and consideration
should be given to treatment with a longer course
of oral antibiotics or admission to the hospital for
Fig. 9 Operative photograph during irrigation and
intravenous antibiotics (Bunzli et al. 1998). debridement of a 3-week-old ght bite. There was puru-
Hand compartment syndrome is a rare entity lence in the metacarpophalangeal joint and destruction of
but, when encountered, is a surgical emergency. two-thirds of the metacarpal head due to osteomyelitis
988
Table 3 Six compartments of the hand and their respec-
tive contents
Compartment Contents of compartment
Thenar Flexor pollicis brevis
Abductor pollicis brevis
Opponens pollicis
Hypothenar Abductor digiti minimi
Flexor digiti minimi brevis
Opponens digiti minimi
Adductor Adductor pollicis
Interossei 4 dorsal interossei
3 volar interossei
Carpal tunnel Median nerve
4 exor digitorum supercialis tendons
4 exor digitorum profundus tendons
Flexor pollicis longus tendon
Digit
Compartment syndrome may occur anytime that
there is a prolonged increase in interstitial tissue
pressures in a fascial compartment causing ische-
mia (Dolan et al. 2012). Causes of compartment
syndrome of the hand vary from crush injuries and
blunt trauma to inltration of intravenous substances
to a tight cast or splint. One should have a higher
index of suspicion for compartment syndrome in the
setting of high energy trauma, multiple metacarpal
shaft fractures, or carpometacarpal fracture-
dislocations (Leversedge et al. 2011). There are
six compartments of the hand thenar, hypothenar,
adductor, interosseus, carpal tunnel, and digits
(Leversedge et al. 2011; Table 3).
Compartment syndrome is largely a clinical
diagnosis and should be considered whenever
the patients pain appears out of proportion to
the injury. Other clinical signs and symptoms of
compartment syndrome include balloon hand
with tense swelling, paresthesias or progressive
neurologic decits, pain with passive stretch of
the ngers or interossei, pallor, or pulselessness
(Al-Qattan 2008; Leversedge et al. 2011; Table 4).
If a clinical diagnosis of compartment syn-
drome is not clear or cannot be obtained due to
the patients mental status, the interstitial pres-
sures may be measured directly. There is some
debate about what measurements should consti-
tute the diagnosis of compartment syndrome.
Many consider any interstitial pressure
S. Cassel and A.S. Shah
Table 4 Signs and symptoms of hand compartment
syndrome
Signs and symptoms of hand compartment syndrome
Pain out of proportion to injury
Tensely swollen hand/balloon hand
Pain with passive stretch
Paresthesias or progressive neurologic decit
Pallor
Pulselessness
measurement within 30 mmHg of the patients
diastolic blood pressure at the time of measure-
ment indicative of compartment syndrome
(Matsen et al. 1980).
Once the diagnosis of compartment syndrome
is established, the patient should be taken
emergently to the operating room for fasciotomy.
The thenar compartment may be released by a
longitudinal incision along the radial border of
the thenar eminence. The hypothenar compart-
ment is released in a similar fashion with a longi-
tudinal incision along the ulnar margin of the
hand. Cadaveric studies have shown that in over
half of the population, the thenar and hypothenar
compartments may be comprised of more than
one discreet compartment (DiFelice et al. 1998).
Release of the dorsal and volar interossei is
accomplished via two longitudinal incisions
along the dorsal second and fourth metacarpal
shafts (DiFelice et al. 1998). The adductor pollicis
can be released through the longitudinal incision
overlying the dorsal second metacarpal shaft or a
separate, dedicated incision over the dorsal rst
web space to ensure adequate decompression. The
carpal tunnel may also be surgically released.
Care must be taken during surgical dissection to
ensure all compartments are fully released. Frac-
tures may be treated surgically at the time of
fasciotomy or may be treated conservatively if
minimally displaced.
Classification
Metacarpal fractures are typically classied
according to the location of the fracture and fur-
ther described in terms of fracture displacement,
44 Metacarpal Fractures
Table 5 Classification of metacarpal fractures
Location of fracture Possible subclassication
Metacarpal head Intra-articular
fracture Open fracture/ght bite
Metacarpophalangeal fracture-
dislocation
Metacarpal neck
fracture
Metacarpal shaft
fracture
Metacarpal base Intra-articular
fracture Carpometacarpal fracture-
dislocation
Bennett fracture
Baby Bennett fracture
involvement of the physis, and intra-articular
extension (Table 5). Classication of metacarpal
fractures is largely the same between adults and
children. This chapter will discuss metacarpal
fractures and their management based on the loca-
tion of the fracture.
Outcome Tools
Primary outcome measures for pediatric metacar-
pal fractures include pain as measured on a visual
analog scale and metacarpophalangeal joint and
interphalangeal joint motion as measured with a
goniometer. There is no specic upper extremity
functional outcome measure which has been val-
idated for use in children. The American Acad-
emy of Orthopaedic Surgeons (AAOS) and the
Pediatric Orthopaedic Society of North America
(POSNA) developed the POSNA pediatric mus-
culoskeletal functional health questionnaire for
use in evaluating the global functional health of
children with musculoskeletal disorders. The
questionnaire focuses on upper extremity func-
tion, transfers and mobility, physical function
and sports, comfort, happiness and satisfaction,
and expectations for treatment (Daltroy
et al. 1998). This questionnaire has been studied
in children with and without musculoskeletal dis-
orders and has been validated for use in children
(Haynes and Sullivan 2001). Although the ques-
tionnaire encompasses upper extremity function
989
as a factor in global functionality, it is not suf-
cient for evaluating upper extremity function in
isolation. Future research may focus on the devel-
opment of a limb-specic outcome measure
which could be used to evaluate children.
General Surgical Planning
Many pediatric metacarpal fracture patterns can
be treated nonoperatively with reduction and
splinting or casting. However, approximately
1020 % of pediatric hand fractures do require
operative intervention (Campbell 1990). Given
the remodeling potential in children, there is
great variation in what is deemed acceptable frac-
ture positioning. In physeal fractures, most sur-
geons agree that up to 30 of sagittal plane
deformity is acceptable and will remodel in
young patients with signicant growth potential
(Pulvertaft 1966; Blount 1977). In metacarpal
neck fractures, 1550 of apex dorsal angulation
is acceptable, with greater deformity acceptable in
the more ulnar digits due to the corresponding
motion at the carpometacarpal joints (Bushnell
et al. 2008). Deformity is less well tolerated at
the metacarpal shaft, where more than 1015 of
apex dorsal angulation in the index and long n-
gers or 2535 in the ring and small ngers should
not be accepted. In contrast to sagittal plane defor-
mity, there is limited remodeling potential in the
axial plane. For this reason, clinically relevant
malrotation should be corrected by closed or
open reduction if deemed necessary. This is par-
ticularly important because, for every degree of
metacarpal shaft rotation, up to 5 of rotation may
be evident at the ngertip (Freeland and Lindley
2006).
When surgery is indicated, closed reduction
and percutaneous pinning of the fracture with
Kirschner wires is the most common treatment.
When planning a surgical treatment for a metacar-
pal fracture, there are many factors to consider.
In general, patients should be positioned supine
on a at bed with the operative extremity
extended on a hand table or diving board.
A pneumatic tourniquet is applied over a cotton
padding on the upper arm or proximal forearm.
990 S. Cassel and A.S. Shah

Fig. 10 Photograph
demonstrating the
placement of a non-sterile
tourniquet on the upper arm
of a patient indicated for
operative treatment. Note
that the tourniquet is well
padded with a soft roll to
prevent any soft tissue
injury

Fig. 11 Sterile cotton

stockinette is placed over
the operative arm, and a
sterile blue towel is
wrapped around the edge of
the tourniquet prior to
draping

A tourniquet, though not often needed for the
majority of these injuries, is helpful to have
available should an open reduction be required
(Figs. 10, 11, and 12).
Fluoroscopy is key to obtaining and
maintaining reduction during the case. In some
cases, the uoroscopic machine itself may serve
as the operating surface for the pediatric hand, and
images may be obtained by rotating the hand and
arm rather than rotating the c-arm. In adolescents
or young adults, these procedures often can be
carried out under regional anesthesia, but in youn-
ger children, general anesthesia is preferred. Most
cases require splinting or casting after the proce-
dure (while the child remains anesthetized) to
support the xation as well as to ensure patient
compliance. Young children may require
prolonged casting or long arm casting to ensure
compliance with activity restrictions. Careful
application of long arm casts in children, includ-
ing a supracondylar mold, can prevent cast migra-
tion and inadvertent loss of fracture xation or
reduction. Traditionally, there has been much
emphasis placed on position of the hand for splint
or cast immobilization with most metacarpal inju-
ries immobilized in the intrinsic plus position.
44 Metacarpal Fractures
Fig. 12 Operative arm extended over the hand table after
being properly prepped and draped
Concern remained that if the hand were
immobilized improperly, the patient may suffer
from residual stiffness or loss of motion. How-
ever, Tavassoli et al. treated metacarpal fractures
with immobilization in three different positions
for 5 weeks with no difference in motion, grip,
or fracture alignment at nal follow-up. In gen-
eral, metacarpal fractures may be immobilized in
the position which is most conducive to
maintaining fracture reduction without concern
about residual loss of motion (Tavassoli
et al. 2005). If, however, fracture reduction and
patient comfort are not concerns, the intrinsic plus
positioning is favored.
Epiphyseal and Physeal Injuries,
Metacarpal Head Fractures,
and Metacarpophalangeal
Joint Dislocations
Most of the metacarpal head fractures in children
represent Salter-Harris II or III fractures
(Bogumill 1983). The index and small metacar-
pals are most frequently involved (McElfresh
and Dobyns 1983). Metacarpal head fracture-
dislocations are also common. One must always
be wary of subtle articular compression fractures
991
when the metacarpal head is involved. These frac-
tures may be highly comminuted and can be dif-
cult to reconstruct operatively (Fig. 13a, b).
The Brewerton view is helpful for diagnosis and
for further evaluating these injuries (Fig. 6).
For nondisplaced fractures involving the artic-
ular surface of the metacarpal head, closed treat-
ment with casting in the intrinsic plus position for
4 weeks is appropriate. Surgery is indicated for
displaced fractures or any fracture with greater
than 1 mm of articular surface incongruity or
greater than 20 % articular involvement
(Weinstein and Hanel 2002). There are several
surgical options. The type of surgical treatment is
dictated by each fractures individual character-
istics. Options include open reduction
through a dorsal approach and xation with
countersunk interfragmentary screws, closed or
open reduction and internal xation with
smooth Kirschner wires, or open reduction and
internal xation with bioabsorbable screws or
sutures.
Metacarpal head fractures may be complicated
by avascular necrosis or growth arrest. There are
numerous case reports documenting growth arrest
with metacarpal shortening and symptomatic
avascular necrosis following a metacarpal head
fracture (Campbell 1990; Light and Ogden 1987;
McElfresh and Dobyns 1983). Light and Ogden
reported cases of avascular necrosis and metacar-
pal shortening following appropriate diagnosis
and treatment of metacarpal epiphyseal
fractures and hypothesized that the extent
of the physeal damage may not always be
evident radiographically or at the time of surgery
(Light and Ogden 1987). Causes of avascular
necrosis are not clear, but some hypothesize
that intra-articular pressure from a hemarthrosis
at the time of injury may contribute
(Campbell 1990).
Metacarpophalangeal dislocations usually
occurs due to hyperextension injuries. The hyper-
extension moment may disrupt the volar plate and
cause it to become interposed and trapped in the
joint by the accessory collateral ligaments. Irre-
ducible dislocations may show puckering of the
volar skin on clinical examination or incarceration
of the sesamoids in the metacarpophalangeal joint
992
Fig. 13 (a, b) PA and
oblique radiographs
demonstrating a long nger
metacarpal head and neck
fracture with depression of
the articular surface and a
ring nger metacarpal neck
fracture in a 17-year-
old male
on the lateral radiograph. Irreducible dislocations
require open reduction. Most irreducible disloca-
tions involve the index metacarpophalangeal
joint. In these situations, the exor tendons are
usually displaced in an ulnar direction with the
lumbrical entrapping the metacarpal head on the
radial side. Open reduction may be performed
via a volar or dorsal approach. If the volar
approach is used, one must take great care to
avoid iatrogenic injury to the radial digital
nerve, which is typically tented over the meta-
carpal head just beneath the skin. If the volar
plate is interposed in the joint, the A1 pulley
may require release in order to free the volar
plate (Campbell 1990; Gilbert 1985). More
rarely, irreducible dislocation of the small
metacarpophalangeal joint may be encountered.
Baldwin et al. described the entrapment
of the small metacarpal head by radial displace-
ment of the exor tendons and ulnar presence of
the abductor digiti minimi (Baldwin et al. 1967).
When performing open reduction of these inju-
ries, one must also be suspicious for and search
S. Cassel and A.S. Shah
for any osteochondral injury to the metacarpal
head as this should also be addressed at the time
of surgery (Light and Ogden 1987).
Preferred Method
For most pediatric metacarpal head fractures with
displacement or articular incongruity greater
than 1 mm, the authors prefer operative treatment
using a dorsal approach through a small curvi-
linear incision. The interval between the extensor
tendon and the ulnar sagittal band is incised,
leaving a small cuff for subsequent repair of the
sagittal band. A dorsal capsulotomy is made, and
care is taken to preserve any soft tissue attach-
ments to the metacarpal head fragments, partic-
ularly the collateral ligaments, in order to
preserve fragment vascularity. Fracture hema-
toma is evacuated and the joint is irrigated
(Fig. 14ac). Open reduction is then achieved
and secured using Kirschner wires, screws, or
suture. Smooth Kirschner wire xation is
44 Metacarpal Fractures
Fig. 14 (ac) Intraoperative photographs of a long nger metacarpal head fracture with comminution and incongruity of
the articular surface of the metacarpophalangeal joint
typically preferred in younger children, and
countersunk interfragmentary xation can be
used in older adolescents or young adults
(Fig. 15ac). After fracture stabilization is
achieved, a layered wound closure of the dorsal
capsule, sagittal band, and skin is performed. The
hand is then immobilized in a berglass mitten
cast for 4 weeks, followed by initiation of range
of motion exercises.
Metacarpal Neck Fractures
Metacarpal neck fractures are the most common
metacarpal fracture seen in children and adults
and often are caused by hitting an object with a
993
clenched st. These injuries have been coined,
boxers fractures, due to the common mecha-
nism. The fth metacarpal is the most common
site for these fractures (Rajesh et al. 2001). The
classic deformity is apex dorsal due to the pull of
the intrinsic muscles, which cross the metacarpo-
phalangeal joint volar to the axis of rotation
(Fig. 16ac). The ring and small nger metacar-
pals tolerate a larger amount of residual dorsal
angulation than the index and long metacarpals
due to compensatory motion at the
carpometacarpal joints of the ring and small n-
gers (Bennett 1982). Up to 50 of fracture angu-
lation is acceptable in the small nger
metacarpal; alternatively, only about 15 of
residual angulation is acceptable in the index
994 S. Cassel and A.S. Shah

Fig. 15 (ac) Postoperative radiographs demonstrating interfragmentary screw xation of the long nger metacarpal
head fracture and percutaneous pinning of the adjacent ring nger metacarpal neck fracture

Fig. 16 (ac) PA, oblique, and lateral radiographs of a small nger metacarpal neck fracture in 14-year-old boy after a
fall onto the right hand. Note the typical apex dorsal displacement

nger metacarpal neck. There is no compensa- Metacarpal neck fractures may be treated conser-
tion for the deformity in the axial plane, and the vatively with cast immobilization in the intrinsic
malrotation of digits is poorly tolerated by plus position if an acceptable reduction can be
patients (Lindley and Rulewicz 2006). achieved and maintained. The most commonly
44 Metacarpal Fractures
Fig. 17 Photograph
demonstrating the Jahss
maneuver for reduction of
metacarpal neck fractures.
The metacarpophalangeal
joint is exed to 90 and
upward pressure is applied
along the proximal phalanx.
Counterpressure can then
be applied along the dorsal
aspect of the proximal
metacarpal
used method for reducing these fractures was
described by Jahss in 1938 and involves exing
the metacarpophalangeal joint to 90 and appli-
cation of upward pressure along the proximal
phalanx. Counterpressure can then be applied
along the dorsal aspect of the proximal metacar-
pal (Fig. 17). Placing the metacarpophalangeal
joint at 90 relaxes the intrinsic muscles and
tightens the collateral ligaments (Jahss 1938).
Once reduction has been attempted, the clinical
exam should be repeated and new radiographs
should be obtained. Any residual rotational
deformity should not be tolerated due to limited
remodeling potential and should be an absolute
indication for operative treatment. If the residual
angulation is acceptable, the hand may be
immobilized in the intrinsic plus position for
4 weeks.
Surgical treatment is indicated in cases of resid-
ual or recurrent unacceptable deformity (unaccept-
able degree of angulation or rotational plane
deformity). Surgery for these injuriestypically
involves closed reduction and percutaneous xa-
tion. Fixation may be maintained using crossed
smooth Kirschner wires or transmetacarpal smooth
Kirschner wires. Following xation, patients
should continue cast immobilization in the intrinsic
plus position for 4 weeks.
995
Preferred Method
If surgery is indicated, the authors prefer to perform
closed reduction in the operating room with
crossed smooth Kirschner wires to maintain reduc-
tion. A closed reduction is obtained with the Jahss
maneuver. The diameter of the Kirschner wires
(0.035, 0.045, or 0.062 in.) is selected based on
size of the metacarpal and age of the patient. Cor-
rection of the angular deformity should be con-
rmed with uoroscopy. Appropriate rotational
alignment should be conrmed by carefully
observing the digital cascade following tenodesis
with passive wrist extension (Fig. 18ac). Patients
are then immobilized in a mitten cast for 34 weeks
in the intrinsic plus position. The pins and cast are
removed at 34 weeks and motion is initiated.
Metacarpal Shaft Fractures
The most common mechanism causing metacarpal
shaft fractures is a bending or torsional force
(Rajesh et al. 2001). Treatment is inuenced by
the number and position of the metacarpal shaft
fracture(s). Single metacarpal fractures can often be
closed reduced and treated conservatively with cast
immobilization in the intrinsic plus position. The
996
Fig. 18 (ac) PA, oblique, and lateral postoperative radiographs after closed reduction and percutaneous pinning of a
small nger metacarpal neck fracture
central rays of the hand are inherently more stable
due to the intermetacarpal ligaments and
carpometacarpal joints (Weinstein and Hanel
2002; Bushnell et al. 2008). Border rays may dem-
onstrate more instability and are more likely to
require surgical treatment (Seitz and Froimson
1988). In the presence of multiple metacarpal frac-
tures in the hand, instability is likely and opera-
tive treatment is often required (Fig. 19ac). If
conservative management is selected, close
follow-up is necessary with frequent radio-
graphic evaluation in the rst few weeks to
ensure that fracture reduction is maintained.
Indications for operative treatment include the
presence of an open fracture, multiple fractures of
the hand, irreducible fractures or unacceptable
angulation, unstable fractures, associated soft tis-
sue injury, rotational deformity, or excessive short-
ening of the metacarpal. Parameters for acceptable
angulation vary according to the involved metacar-
pal and the associated compensatory mobility at the
carpometacarpal joint. The ring and small meta-
carpals tolerate about 3040 of angulation;
whereas the index and middle ngers only
S. Cassel and A.S. Shah
tolerate 1020 of angulation (Lindley and
Rulewicz 2006). Shortening of any metacarpal
more than 25 mm is not acceptable. For every
2 mm of metacarpal shortening, there is approx-
imately 8 % loss of grip strength and up to 7 of
extensor lag (Freeland and Lindley 2006;
Strauch et al. 1998). In cases of malunion, an
osteotomy performed at the fracture site or at
the base of the metacarpal may be used to correct
deformity as vascularity is optimized in this area
(Campbell 1990; Freeland and Lindley 2006).
Surgery most often consists of closed reduction
and percutaneous pin xation using smooth
Kirschner wires; however, several other surgical
options are also acceptable. Surgical treatment of
metacarpal shaft fractures may also include open
or closed reduction with interfragmentary screws,
internal xation with plate and screws,
intramedullary xation, or external xation
(Fig. 20ac). Complications such as nonunion or
malunion are very rare in children but can occur
especially if the injury involved extensive soft
tissue damage or if the fracture was open (Ireland
and Taleisnik 1986).
44 Metacarpal Fractures 997

Fig. 19 (ac) PA, oblique, and lateral radiographs of shaft fractures and with involvement of border rays (such
midshaft fractures of the ring nger metacarpal and small as small nger metacarpal), the fractures are more likely to
nger metacarpal. In the presence of multiple metacarpal be unstable and require operative xation

Fig. 20 (ac) PA, oblique, and lateral postoperative radio- nger metacarpal and closed reduction percutaneous pin-
graphs demonstrating two different xation options open ning along the shaft of the small nger metacarpal
reduction internal xation with plate and screws of the ring
998
Preferred Method
If surgery is indicated, the authors prefer to per-
form closed reduction in the operating room with
crossed smooth Kirschner wires or
transmetacarpal wires to maintain reduction. Fix-
ation of the ring metacarpal can be particularly
challenging due to the small isthmus diameter.
The diameter of the Kirschner wires (0.035,
0.045, or 0.062 in.) is selected based on the size
of the metacarpal and age of the patient. Appro-
priate rotational alignment should be conrmed
by carefully observing the digital cascade fol-
lowing tenodesis with passive wrist extension.
Patients are then immobilized in a mitten cast
for 34 weeks in the intrinsic plus position. The
interphalangeal joints may be left free out of the
cast. The pins and cast are removed at 34 weeks
and motion is initiated. In the setting of multiple
metacarpal fractures, open reduction and internal
xation are often preferred. The metacarpal can
be approached dorsally through a longitudinal
incision. If adjacent metacarpals are fractured, a
single longitudinal incision between rays can be
utilized. Nonlocking modular hand plates should
be selected based on the size of the metacarpal.
At least four cortices of xation are
recommended on each side of the fracture.
Appropriate rotational alignment should be con-
rmed by carefully observing the digital cascade
following tenodesis with passive wrist extension.
Care should be taken to preserve the periosteum
for repair following fracture reduction and plat-
ing. Patients are then immobilized in a mitten
cast for 34 weeks with the metacarpophalangeal
joints in exion. The interphalangeal joints may
be left free out of the cast.
Metacarpal Base Fractures and Reverse
Bennett Fractures
Metacarpal base fractures, like metacarpal neck
fractures, most often involve the small digit meta-
carpal. Metacarpal base fractures make up about
1320 % of metacarpal fractures in children with
over half of these involving the small metacarpal
(Cornwall 2006). These injuries may be complex
S. Cassel and A.S. Shah
and can involve the articular surface or be
fracture-dislocations of the carpometacarpal
joint. Fracture-dislocation of the carpometacarpal
joint may be subtle, and dedicated radiographs
evaluating the congruity of the joint must be
included in the initial evaluation (Bushnell
et al. 2008). The fourth and fth carpometacarpal
joints may be assessed by a 30 pronated view.
The second and third carpometacarpal joints are
best evaluated with a 30 supinated view. Meta-
carpal base fractures are more often associated
with higher energy mechanisms, and one should
be cognizant of the possibility of hand compart-
ment syndrome or other concomitant injuries
(Fig. 21a, b).
Fractures of the small metacarpal base are also
known as baby Bennett or reverse Bennett frac-
tures (Fig. 22a, b). These fractures tend to be more
unstable due to the increased mobility of the
carpometacarpal joints of the fourth and fth
metacarpal and the deforming forces of the exten-
sor carpi ulnaris and the hypothenar muscles. The
extensor carpi ulnaris inserts in the dorsoulnar
aspect of the base of the fth metacarpal and
shortens and displaces the metacarpal shaft in an
ulnar direction. The intermetacarpal ligament
between the fourth and fth metacarpal bases
typically remains intact and stabilizes the radial
fragment of the fracture (Bushnell et al. 2008;
Mozaffarian et al. 2012).
Metacarpal base fractures may be treated
nonoperatively with cast immobilization for 34
weeks if the fracture is nondisplaced, in which the
carpometacarpal joint is reduced and stable, and if
there is no rotational deformity of the nger. The
reduction of carpometacarpal fracture dislocations
is achieved by longitudinal traction, volar directed
pressure at the level of the dislocation, and adjust-
ment of rotational deformity by exing the digit at
the metacarpophalangeal joint (Bushnell
et al. 2008). Frequently these injuries are unstable
or have a rotational component and are best
treated with operative xation. Surgical treatment
of metacarpal base fractures and carpometacarpal
fracture-dislocations involves closed or
open reduction and transmetacarpal pinning or
pinning obliquely through the base of the meta-
carpal into the carpal bones. The operative
44 Metacarpal Fractures 999

Fig. 21 (a, b) PA, oblique, and lateral radiographs demonstrating ring and small carpometacarpal dislocations with
dorsal displacement

Fig. 22 (a, b) PA and

oblique radiographs of a
fracture of the small nger
metacarpal base, also
known as a baby Bennett or
reverse Bennett fracture

xation of reverse Bennett fractures is challeng- 2 cm distal to the joint at a 2030 angle to the
ing due to the close proximity of the ulnar nerve. coronal plane and from 10 volar to dorsal to 20
Cadaveric studies have demonstrated that the dorsal to volar in the sagittal plane (Mozaffarian
safest position for pinning of these injuries is et al. 2012).
1000 S. Cassel and A.S. Shah

Preferred Method

When surgery is required for these injuries, the

authors prefer to perform closed reduction and
percutaneous pinning in the operating room
under uoroscopic guidance. A smooth Kirschner
wire placed transmetacarpal into the adjacent ring
metacarpal and a second smooth Kirschner wire
placed obliquely through the fracture and into
the carpus yield a stable construct (Fig. 23).
The diameter of the Kirschner wires (0.035,
0.045, or 0.062 in.) is selected based on the size
of the metacarpal and age of the patient. Appro-
priate rotational alignment should be conrmed
by carefully observing the digital cascade follow-
ing tenodesis with passive wrist extension.
Patients are then placed into a berglass mitten
cast with the metacarpophalangeal joints in ex-
ion. The interphalangeal joints may be left free out
of the cast. After 4 weeks of immobilization, the
wires are removed at the clinic and motion is
initiated.

Fig. 23 Postoperative radiograph demonstrating closed

Base of Thumb Fractures
Fractures of the base of the thumb metacarpal are
fairly common. In children less than 16 years,
22 % of hand fractures involve the thumb (Stanton
et al. 2007). These fractures are classied as
intra-articular or extra-articular. Within the cate-
gory of intra-articular, there are two main fracture
patterns Bennett fracture and Rolando fracture.
The deforming forces at play in these injuries are
crucial to understanding treatment. The abductor
pollicis longus pulls the metacarpal base into a
supinated position, while the adductor pollicis
displaces the metacarpal shaft into adduction.
Bennett fractures involve a fracture through the
base of the thumb metacarpal with a volar frag-
ment of the metacarpal base remaining attached to
the volar oblique ligament. The volar fragment is
usually still articulating with the trapezium (Bennett
1982). Rolando fractures also have the volar
fragment, but the metacarpal base is comminuted
and often has a Y-shaped fracture pattern.
reduction and percutaneous pinning of a baby Bennett
fracture. Kirschner wires are directed into the carpus to
form a more stable construct for healing the fracture
In children, fractures of the thumb metacarpal
base are often extra-articular and typically repre-
sent Salter-Harris type II fractures (Fig. 24a, b)
and, more infrequently, Salter-Harris type III frac-
tures (Grifths 1966). In Salter-Harris type II
fractures of the thumb metacarpal base, if the
distal fragment is displaced in a radial direction,
closed treatment is often successful. Up to 30 of
angulation is acceptable in these fractures due to
the remodeling potential of children and the
mobility of the carpometacarpal joint (Grifths
1966). If the distal fracture fragment is displaced
in an ulnar direction, closed reduction may prove
more difcult due to the presence of soft tissue in
the fracture site. Salter-Harris type III fractures
often require operative treatment to restore artic-
ular congruity (Campbell 1990).
44 Metacarpal Fractures
Fig. 24 (a, b) PA and
lateral radiographs of a
Salter-Harris II fracture of
the base of the thumb
metacarpal with radial
displacement
The thumb metacarpophalangeal joint is the
most common site of dislocation in the pediatric
hand (Fig. 25a, b). Classication by Farabeuf in
1876 divides these injuries into three groups
(1) simple incomplete dislocation, (2) simple
complete dislocation, and (3) complete complex
dislocation. In incomplete dislocations, the
volar plate is disrupted but the collateral liga-
ments remain intact. Most often these disloca-
tions can be closed reduced and immobilized.
Simple complete dislocations have the addi-
tional nding of disrupted collateral ligaments
but no interposition of the volar plate. Closed
reduction should be attempted using the maneu-
ver described by Farabeuf exion and adduc-
tion of the thumb metacarpal base then
hyperextension of the metacarpophalangeal
joint. Failure of closed reduction is an indica-
tion for operative treatment. Complete complex
dislocations have interposition of the volar plate
and sesamoids between the metacarpal head and
the base of the proximal phalanx and are
unlikely to be successfully closed reduced.
Operative reduction may be performed through
1001
a volar or dorsal approach (Campbell 1990;
Gilbert 1985; Farabeuf and Barnard 1901).
Preferred Method
If surgery is indicated for these injuries due to
unacceptable alignment or rotation or extension
of the fracture to the articular surface, the
authors prefer to perform closed reduction and
percutaneous pinning in the operating room
under uoroscopic guidance. Fixation with
smooth Kirschner wires is recommended with
the selection of the size of the wires (0.035,
0.045, or 0.062 in.) determined by the size of
the metacarpal and age of the patient. Care
should be taken to avoid xation across the
physis of the thumb metacarpal if possible to
prevent premature physeal closure and growth
arrest. Fixation across the physis is, in some
cases, unavoidable but should be minimized if
possible. Fixation may need to be extended
across the base of the metacarpal into adjacent
carpal bones (Fig. 26a, b). Patients should then
1002 S. Cassel and A.S. Shah

Fig. 25 (a, b) Two views

of a right thumb
metacarpophalangeal joint
dislocation in an 8-year-old
boy. A closed reduction was
achieved without difculty.
The child recovered full
function after a brief period
of splint immobilization

Fig. 26 (a, b)
Postoperative PA and lateral
radiographs of closed
reduction and percutaneous
pinning a Salter-Harris II
fracture of the base of the
thumb metacarpal
44 Metacarpal Fractures
be placed into a thumb spica cast for 34 weeks.
Wires may be removed at the clinic in 34
weeks and motion initiated.
Summary
Pediatric metacarpal fractures are common inju-
ries that are typically classied according to the
location of the fracture. The importance of the
clinical exam cannot be overstated. If the align-
ment is acceptable, many of these fractures can be
treated nonoperatively with immobilization for
4 weeks. In cases of malrotation, operative treat-
ment is recommended and closed reduction with
percutaneous pinning is usually successful. One
must always be watchful for hand compartment
syndrome and ght bites which can complicate
the treatment of these injuries.
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 Hand Dislocations
45
John Lubahn, Rey Ramirez, Raymond Metz, and
Patrick Emerson

Contents Outcome Tools . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1014

Treatment Options for Dorsal Dislocation of the
Metacarpophalangeal Joint Dislocations . . . . . . . . 1006 Proximal Interphalangeal Joint . . . . . . . . . . . . . . . . . . . . . 1014
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1006 Surgical Approach and Technique . . . . . . . . . . . . . . . . . 1017
Pathoanatomy and Applied Anatomy . . . . . . . . . . . . . . 1006 Preferred Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1018
Assessment of Metacarpophalangeal
Dislocations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1008 Dislocations of the Finger Distal
Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1008 Interphalangeal Joint and Thumb
Classication . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1009 Interphalangeal Joint . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1019
Outcome Tools . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1009 Assessment of Distal Interphalangeal Joint
Treatment Options for Dorsal Dislocation of the Dislocation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1019
Metacarpophalangeal Joint . . . . . . . . . . . . . . . . . . . . . . . . . 1010 Distal Interphalangeal Joint
Surgical Approach and Technique . . . . . . . . . . . . . . . . . 1011 Dislocation Treatment Options . . . . . . . . . . . . . . . . . . . . . 1019
Preferred Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1012 Complications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1020
Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1020
Complications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1012
Thumb Metacarpophalangeal Joint
Physical/Occupational Therapy Dislocations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1020
Recommendations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1012 Reduction Technique . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1020
Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1013 Physical Therapy Recommendations . . . . . . . . . . . . . . . 1021

Interphalangeal Joint Dislocations . . . . . . . . . . . . . . . 1013 Thumb Ulnar Collateral Ligament Injuries . . . . 1021
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1013 Pathoanatomy and Applied Anatomy . . . . . . . . . . . . . . 1022
Pathoanatomy and Applied Anatomy . . . . . . . . . . . . . . 1013 Assessment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1023
Assessment of Interphalangeal Dislocations . . . . . . . 1014 Outcome Tools . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1023
Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1014 Nonoperative Management . . . . . . . . . . . . . . . . . . . . . . . . . 1023
Classication . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1014 Outcomes of Nonoperative Treatment . . . . . . . . . . . . . 1024
Operative Treatment for Thumb UCL Injury . . . . . . 1025
Surgical Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1025
Technique . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1025
J. Lubahn (*) R. Ramirez Surgical Outcomes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1026
Hand, Microsurgery and Reconstructive Orthopedics, Erie, Preferred Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1026
PA, USA Management of Complications . . . . . . . . . . . . . . . . . . . . . 1027
e-mail: jdlubahn@jdlubahn.com Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1027
R. Metz References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1027
UPMC Hamot Medical Center, Erie, PA, USA
Core Orthopedics and Sports Medicine, Elk Grove Village,
IL, USA
e-mail: raymondjmetzjr@gmail.com
P. Emerson
UPMC Hamot Medical Center, Erie, PA, USA

# Springer Science+Business Media New York 2015 1005

J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_46
1006
Abstract
Finger dislocations are incredibly common
injuries. They are very common in the pediatric
athletic population. They are most frequently
reduced on the playing eld by athletic
trainers, but are also seen in the Emergency
Department. Most often, they are simple dislo-
cations which are easily reducible. Finger dis-
locations can occur anywhere along the digits:
the carpometacarpal joint, the metacarpo-
phalangeal joint, the proximal interphalangeal
joint, and the distal interphalangeal joint.
Not all dislocations are easily reducible,
and some need operative intervention to reduce
the joint. There are several structures specic
to each joint which can impede a stable reduc-
tion. A stable reduction is essential to proper
long term function of the digit and the hand as a
whole. Proper knowledge of both closed and
open methods of joint reduction are essential
for orthopaedists and hand surgeons alike.
Metacarpophalangeal Joint
Dislocations
Introduction
Metacarpophalangeal (MCP) joint dislocations of
the ngers are a relatively rare injury in the upper
extremity. Of those reported however, a signi-
cant subset of these injuries are found in the pedi-
atric population (Becton et al. 1975; Hunt
et al. 1967; Baldwin et al. 1967; Murphy and
Stark 1967). While several theories exist, many
feel that the ligamentous laxity of children is a
signicant contributing factor (Ogden 1982).
Despite this fact, the ligaments around the joint
are often stronger than the immature bone. Con-
sequently, bony avulsions and fractures are more
frequently seen than dislocations alone. When
dislocations are seen, they usually result from a
dorsally directed force against an immovable
object. This often occurs with hyperextension of
the digit from a fall on the outstretched extremity
of a toddler or a direct blow in adolescent athletic
competition. Among the nger MCP dislocations,
J. Lubahn et al.
Fig. 1 (Dinh 2009) Clinical photo demonstrating the sub-
cutaneous location of the metacarpal head in a dorsal
metacarpophalangeal dislocation. Note the position of the
radial digital nerve which can often be displaced volarly
just under the skin making it prone to injury with this
approach
the index is the most commonly affected followed
by the small nger (OBrien 1991). While volar
dislocations are not unheard of, they are an
exceedingly rare variant and are not routinely
encountered.
Pathoanatomy and Applied Anatomy
The metacarpophalangeal joint is supported by
many adjacent soft tissue structures including the
interosseous muscles, the collateral ligaments,
and the volar plate. Because of the unique condy-
loid articulation, signicant range of motion is
possible that is not afforded to the more distal
interphalangeal (IP) joints (Eaton 1971; Figs. 1
and 2). The collateral ligaments can be found
originating dorsal to the center of rotation on the
metacarpal head and inserting onto the base of the
proximal phalanx. Similarly, the accessory collat-
eral ligaments can be found originating just volar
to the center of rotation while inserting onto the
proximal phalanx and volar plate. The shape of
the metacarpal head in combination with the
eccentric origin of the ligaments contributes to a
cam effect resulting in relative laxity of the joint in
45 Hand Dislocations
Fig. 2 (Dinh 2009)
Schematic representation of
common obstacles to
reductions. The metacarpal
head is often trapped
between the exor tendons
ulnarly and lumbricals
radially. The natatory
ligament lies distally, while Natatory ligament
the supercial transverse
metacarpal ligament is
positioned proximally
Superficial transverse
metacarpal ligament
Flexor digitorum
profundus muscle
extension while tightening in exion. The palmar
plate is a rectangular brocartilaginous body that
can be found on the volar side of the joint. It has a
rm attachment near the proximal aspect of the
proximal phalanx and a looser membranous
attachment at the metacarpal metaphysis (Light
and Ogden 1988). The volar plate also lacks the
rm checkrein ligaments found distally in the
proximal interphalangeal (PIP) joints. This
accounts for the relative hyperextension seen at
this joint and subsequent disruption of the loose
proximal attachment of the volar plate. With dor-
sal dislocations, the volar plate is displaced dorsal
to the metacarpal head where it is still attached to
the proximal phalanx. The volar plates remain
attached to one another through the deep trans-
verse intermetacarpal ligament (al-Qattan and
Robertson 1993). Follow-up radiographs will
often demonstrate evidence of periosteal reaction
on the palmar distal metacarpal metaphysis
representing the torn attachment of the volar
plate (Green and Terry 1973).
Dislocations of the MCP joint can be divided
into complex or simple. In simple dislocations, the
volar plate remains volar or just distal to the
1007
Metacarpal head
Lumbrical
muscle
articular surface of the metacarpal head. The
term complex dislocation has been widely applied
to those injuries in which closed reduction is not
possible. Multiple authors have described this
complex lesion and the failure of closed
reduction (Baldwin et al. 1967; Burman 1953;
Hunt et al. 1967; McLaughlin 1965; Milch 1965;
Murphy and Stark 1967). This is most often due to
the trapped volar plate that becomes positioned
dorsal to the metacarpal head; however, several
structures can sesamoids ultimately be responsi-
ble for an inability to reduce the joint. The nata-
tory ligaments, palmar fascia, supercial
transverse metacarpal ligament, lumbricals, and
the exor tendons have all been reported as pos-
sible obstacles to joint reduction (Kaplan 1957;
Beatty et al. 1990; Plancher 2004). The exor
tendons and lumbricals are often found locked
dorsally behind the displaced metacarpal head.
Often the lumbrical can be found on the radial
side of the metacarpal and the exor tendons on
the ulnar side contributing to a noose-like tighten-
ing made worse with traction. The A1 pulley is
often still attached to the volar plate and pulls the
exor tendons dorsal to the metacarpal head.
1008 J. Lubahn et al.

Assessment of Metacarpophalangeal
Dislocations

Examination of a childs hand poses many chal-

lenges that are not present in the adult patient.
Pain, stranger anxiety, and lack of understanding
all contribute to the difculty of the hand exami-
nation in a child. Clinically, the nger appears
shortened, supinated, and angulated in an ulnar
direction when an MCP dislocation is present.
The skin may appear puckered volarly where the
metacarpal head has buttonholed through the
volar joint capsule. Careful examination of the
palm skin is necessary to ensure there is no evi-
dence of an open injury. Absent and painful
attempts at MCP range of motion are often pre-
sent. The metacarpophalangeal joint may exhibit
several different positions. The joint may appear
hyperextended as it does in a simple dislocation
(or subluxation), which often represents a tear of
the volar plate without dorsal displacement
behind the metacarpal head. Alternatively, the
proximal phalanx may be lying dorsal and parallel
to the metacarpal. This is classically the complex
dislocation in which closed reduction is not Fig. 3 (Plancher 2004) Radiograph demonstrating an
entrapped sesamoid in the metacarpophalangeal joint
possible.
Swelling, bruising, and pain are all frequent
ndings with both types of dislocations (Fig. 3).
Careful examination of the neurovascular the radial to the ulnar side of the nger, should be
status distal to the injury is equally important. met with some resistance. If not, and the pen
The clinician should assess for adequate glides back and forth over the ngertip as if over
blood ow by comparing the color, temperature, a smooth surface on the involved nger in com-
capillary rell, and skin turgor of the nger to an parison to the other ngers, the examiner can
uninjured digit. Palpation of the palm may reveal conclude that the sympathetic innervation to
a bony prominence representing the metacarpal smooth muscles responsible for skin turgor has
head. Decreased sensation to light touch and been disrupted and a nerve injury to that nger has
diminished two-point discrimination suggest occurred.
pressure on the digital nerves which may be
tented volarly over the metacarpal head. If a pedi-
atric patient is unable to participate in two-point Imaging
discriminatory testing, the wrinkle test may be
performed by submerging the hand in water A complete radiographic series, including
for several minutes if nerve dysfunction is posterior-anterior (PA) and lateral views (Fig. 4),
suspected. Absence of wrinkling is indicative of in combination with a detailed clinical examina-
nerve injury. A thorough sensory exam may be tion, should always be obtained on initial contact
limited by pain in the nger, but in with the patient. The dislocation can often be
operative cases, once the patient is asleep, rubbing difcult to interpret with a PA radiograph alone.
a sterile plastic marking pen back and forth, from With this view, the MCP joint may appear
45 Hand Dislocations
Fig. 4 (Plancher 2004)
Radiograph of a complex
metacarpophalangeal
dislocation. Note the almost
parallel relationship of the
metacarpal and proximal
phalanx (need a skeletally
immature patient here)
narrowed, distracted, angulated, or even normal in
appearance (Light and Ogden 1988). Lateral plain
lms can often be obscured by the overlapping
metacarpals. A line drawn straight down the shaft
of the proximal phalanx should always intersect
the metacarpal head. Failure of the line to intersect
the metacarpal head suggests subluxation or dis-
location (Campbell 1990). On the lateral view, the
proximal phalanx lying parallel to the metacarpal
shaft indicates a complex dislocation, while a
simple dislocation is inferred if the proximal pha-
lanx is oriented 90
to the metacarpal. In a child
older than 10 years of age, sesamoids that have
undergone ossication may also be observed in
the joint (Green and Terry 1973; Campbell 1990).
More advanced imaging including computed
tomography (CT) and magnetic resonance imag-
ing (MRI) is rarely necessary for the diagnosis of
these injuries. If the dislocation is associated with
a fracture of the proximal phalanx or metacarpal
head, a CT may permit better visualization of the
articular anatomy, especially if an intra-articular
fracture is suspected. These tests should only be
reserved for rare cases in which a complete under-
standing of the bony anatomy is not possible with
plain radiographs alone.
1009
Classification
Dislocations are described by the direction of the
distal part and the ability to achieve closed reduc-
tion. The term simple routinely refers to those
dislocations in which closed reduction is success-
ful, while complex dislocations require open reduc-
tion. A complex dislocation represents bayoneting
of the proximal phalanx on the metacarpal. Alter-
natively, a simple joint dislocation is demonstrated
by joint subluxation or perching of the proximal
phalanx on the metacarpal. Subluxation of the joint
can easily be converted to a complete (and com-
plex) dislocation by exaggeration of the hyperex-
tension or an incorrect reduction maneuver.
Outcome Tools
Successful management of these injuries in the pedi-
atric population should include full restoration of
strength and range of motion. Assessments of grip
strength, pinch strength, and range of motion for all
the joints of the nger are important to measure and
document. However, no outcome tool exists that is
specic for pediatric hand dislocations.
1010
Treatment Options for Dorsal
Dislocation of the
Metacarpophalangeal Joint
Nonoperative Management
Indications/Contraindications
An attempt at closed reduction should be made
prior to any surgical intervention. While the com-
plete dislocation is often irreducible by closed
means, the clinician may attempt closed reduction
of the joint to preclude the need for surgery. Mul-
tiple attempts at closed reduction should not be
performed as it can lead to damage of the growth
plate or rarely premature growth arrest of the
metacarpal physis (Green and Terry 1973)
(Table 1).
Techniques
Appropriate analgesia is important prior to any
attempt at a closed reduction. A digital nerve
block proximal to the metacarpophalangeal joint
with appropriate blocking of the dorsal and volar
nerves is preferred. Attempts at closed reduction
should focus on the type of dislocation. Simple
dislocations often will reduce with gentle traction
and volar translation. The translation is often much
more important than the traction. If a complete
dislocation is suspected, MCP joint hyperexten-
sion with distal and palmar translation of the prox-
imal phalanx should be attempted. Excessive
traction may result in a noose-like mechanism
of soft tissue surrounding the metacarpal head.
Hyperextension or traction alone may also convert
an otherwise simple dislocation into a complex
lesion (McLaughlin 1965; Campbell 1990).
Table 1
Metacarpophalangeal joint dislocations
Nonoperative treatment
Indications Contraindications
Successful Closed reduction unsuccessful
closed reduction Chronic dislocation (trial of closed
reduction should be attempted)
Fracture dislocation requiring open
treatment
Open dislocation
J. Lubahn et al.
Additionally described techniques involve injec-
tion of the joint with a local anesthetic or saline
in an attempt to deliver the palmar plate volar to the
metacarpal head (OBrien 1991).
If closed reduction is successful, the joint
should be immobilized in a dorsal blocking splint
with the joint in slight exion and the wrist in
either neutral or slight extension. Placement of
the wrist in exion may contribute to MCP exten-
sion as the extensor tendons are placed under
greater tension. A trial of 34 weeks of immobi-
lization should be followed with protected range
of motion. Buddy taping is a useful tool to prevent
additional instability while focusing on restora-
tion of joint motion.
Outcomes
Long-term outcome studies of this rare injury have
not been reported in the literature. While the adage
all things pediatric do well is not always true,
recognition of the injury pattern, appropriate reduc-
tion, and subsequent immobilization are all keys to
achieving a good outcome. Formal hand therapy is
rarely necessary as age-appropriate play activities
usually result in full and stable range of motion
with appropriate treatment. Long-term complica-
tions of these injuries are infrequent and are most
often the result of a delay in diagnosis or treatment.
While good outcomes have been reported in the
treatment of missed dislocations of greater than
3 months, results tend to deteriorate as time to
treatment increases (Barenfeld and Weseley 1972;
Murphy and Stark 1967).
Operative Management
Indications/Contraindications
See Table 2.
Surgical Procedure
Preoperative Planning
Prior to surgical reduction of the joint, the clini-
cian should obtain appropriate radiographic views
and document a detailed physical exam. A com-
plete understanding of the offending structures
and challenges of each approach is necessary
(Table 3).
45 Hand Dislocations
Table 2
Metacarpophalangeal joint dislocations
Operative treatment
Indications Contraindications
Closed reduction unsuccessful Closed reduction
successful
Chronic dislocation (trial of closed
reduction should be attempted)
Fracture dislocation requiring open
treatment
Open dislocations
Table 3
Open reduction for metacarpophalangeal joint
dislocation
Preoperative planning
OR table: radiolucent hand table
Position: supine
Fluoroscopy: brought in from end of hand table
Equipment: standard hand surgery set, Kirschner wire set
Tourniquet: nonsterile
Surgical Approach and Technique
Both dorsal and volar approaches have been
described for the irreducible dorsal MCP disloca-
tion. The volar approach provides superior visu-
alization of the metacarpal head and additional
structures (other than the volar plate) that may be
blocking the reduction (Gilbert 1985; Kaplan
1957; Light and Ogden 1988; McLaughlin 1965;
Green and Terry 1973; Barenfeld and Weseley
1972). If a volar approach is utilized, extreme
care must be taken when making the skin incision
to avoid injury to a digital nerve, in the case of the
index nger, the radial digital nerve. An oblique
Bruner-type incision should be centered over the
metacarpal head. In the case of an open injury, the
laceration is frequently extended to allow expo-
sure of the injury. Iatrogenic nerve injury has been
reported as the digital nerve is immediately
beneath the skin as it becomes tented volarly by
the displaced metacarpal head (Becton et al. 1975;
Green and Terry 1973). After identication of the
digital nerves and blunt soft tissue dissection, the
surgeon may incise the A1 pulley. With
1011
Table 4
Volar approach of dorsal dislocation of the
metacarpophalangeal joint
Surgical steps
Bruner incision centered on MCP head
Blunt dissection to identify digital nerves
Incise the A1 pulley
Extract volar plate from MCP joint
Examine joint for articular damage
Reduce joint
Determine stable arc for range of motion
Close skin
attachments on either side of the volar plate, tran-
section of the pulley may relax the tissue enough
to facilitate reduction. Prior to reduction, a careful
examination of the articular surface should be
performed looking for loose bodies or
osteochondral fragments. The metacarpal head
should be delivered dorsally with retraction of
the exor tendons and lumbrical. A skin hook
may be used to retrieve the dorsally displaced
volar plate. If reduction can still not be accom-
plished, some surgeons advocate a longitudinal
incision in the volar plate. This functions to relax
the accessory collateral ligaments that also share
an attachment on the volar plate. More chronic
dislocations may require release of the ulnar col-
lateral ligament to facilitate joint reduction. Once
the joint is reduced, the nger should be taken
through a gentle range of motion to determine a
stable arc of motion. Typically no additional clo-
sure other than the skin is needed (Table 4).
Several authors prefer a dorsal approach as it
has the advantages of avoiding potential damage
to the digital nerve and easier visualization of the
dorsally dislocated volar plate (Becton et al. 1975;
Bohart et al. 1982; McLaughlin 1965; Hunt
et al. 1967). A curvilinear midline longitudinal
incision is made centered over the metacarpo-
phalangeal joint. The extensor tendon is identied
and longitudinally split before a capsulotomy is
made into the joint. Successful reduction from the
dorsal approach often requires a longitudinal inci-
sion in the volar plate. Regardless of which
approach is preferred, hardware including
Kirschner wires is rarely necessary and may
1012
Table 5
Open reduction metacarpophalangeal joint postoperative
protocol
Dorsal blocking splint to ngertips for dorsal dislocation
Length of immobilization: 2 weeks followed by active
range of motion with a dorsal blocking splint for 2 weeks
Rehab protocol: buddy taping for 12 weeks. Encourage
early range of motion within the stable arc
Return to sport when range of motion returns and pain is
minimal
For very small children who require splinting, consider
placing a thermoplast splint directly on the skin to ensure
joint position and then overwrap it with cast material
cause additional damage to the immature growth
plate (Ogden 1982; Baldwin et al. 1967). A dual
approach incorporating both volar and dorsal
approaches may be needed for chronic disloca-
tions with a delay in diagnosis (Murphy and Stark
1967; Barenfeld and Weseley 1972; Table 5).
Preferred Treatment
If a complex dislocation is present, a volar
approach is preferred because all offending struc-
tures can be identied. Reduction of the volar
plate can often be accomplished with release of
the A1 pulley and a translation maneuver. If a
relaxing incision is used, repair of the volar plate
can easily be performed. While the dorsal
approach can be successful, one disadvantage is
the inability to release the volar structures, such as
the A1 pulley. Additional structures, such as the
palmar aponeurosis, may also prevent the meta-
carpal head from reducing (Kaplan 1957). Release
of these structures requires a volar approach.
Additionally, suture repair of the volar plate inci-
sion is not possible from the dorsal side alone
(Table 6).
Complications
Successful treatment of these complex injuries
require early recognition of the injury as well as
close followup after joint reduction. Several of the
most commonly encountered complications are
J. Lubahn et al.
Table 6
Metacarpophalangeal joint dislocation
Potential pitfalls and preventions
Pitfall Prevention
Conversion of simple Avoid excessive traction
to complex dislocation during closed reduction
Premature growth Avoid multiple forceful
arrest attempts at closed reduction
Chronic instability or Obtain good quality
subluxation radiographs to rule out
fracture dislocation
Missed fracture or Obtain good quality
osteochondral injury radiographs and carefully
inspect the articular surface
during open reduction
listed. As previously mentioned, improper reduc-
tion technique may convert a simple dislocation
on presentation to a complex one. This is often
seen when traction alone is used in an attempt to
close reduce a simple dislocation. Moreover,
repeated forceful reduction attempts may damage
the growth plate and lead to subsequent premature
growth arrest. Appropriate injury radiographs in
addition to post reduction images must always be
obtained to rule out additional subluxation, dislo-
cation, or fracture.
Missed or chronic dislocations may pose a
greater challenge. While successful reduction
has still been described as far out as 3 months,
these patients may suffer from decreased range of
motion and be left with a stable yet stiff nger
(Murphy and Stark 1967). Successful operative
treatment of chronic dislocations may require
both volar and dorsal surgical approaches to ade-
quately remove incarcerated structures and free up
scar tissue.
Physical/Occupational Therapy
Recommendations
Postoperative care should focus on early range of
motion, with prevention of hyperextension. This
can be accomplished with patient education alone
or in combination with a dorsal blocking splint.
Small osteochondral fractures and avulsions are
often present and can be treated conservatively
45 Hand Dislocations
with removal or anatomic reduction if soft tissue
attachment is still present. Larger fragments
involving the articular surface or growth plate
should be xed anatomically and may require
pin xation.
Summary
While metacarpophalangeal joint dislocations are
relatively rare, clinicians should be aware of
potential complications of these injuries and
appropriate treatment. Prompt and appropriate
treatment of these injuries often will result in no
long term complications
Interphalangeal Joint Dislocations
Introduction
Dislocations of the interphalangeal joints are
unusual injuries in children. Dislocations require
rupture of one or more of the restraining ligaments
of the joint. The ligaments in children are rela-
tively stronger than the surrounding bone, and
therefore, avulsions or physeal fractures are
more common than joint dislocations. An injury
type that may be more commonly seen is the volar
plate avulsion injury, which usually does not pre-
sent as a dislocation (Weber et al. 2009). How-
ever, this injury may be considered along with
interphalangeal joint dislocations because it
results from the same mechanism and is treated
in a similar manner. The usual mechanism of an
interphalangeal dislocation or volar plate avulsion
is a hyperextension or dorsally directed force.
This results in a dorsal dislocation. Volar disloca-
tions are extremely rare. More commonly, a rota-
tional torque may produce a volar rotatory
subluxation. Lateral dislocations are another rare
variant. Fracture dislocations of the proximal
interphalangeal joint are generally not seen in
children. These injuries result from the same
forces as dislocations and are much more likely
to cause a bony avulsion, with or without a joint
dislocation, in a child.
1013
Fig. 5 Diagram depicting the three sided box formed by
the accessory collateral ligaments and the volar plate. C:
proper collateral ligament A: accessory collateral ligament
(credit: Rey Ramirez)
Pathoanatomy and Applied Anatomy
The interphalangeal joints are supported by a
strong set of radial and ulnar collateral ligaments
and a volar plate that form a boxlike structure
along the three sides of the joint (Eaton 1971;
Fig. 5). The volar plate originates from the
metaphysis of the proximal bone to insert on the
epiphysis of the more distal bone and prevents
hyperextension. The collateral ligaments originate
from the collateral recesses of the phalangeal head
and insert on the distal metaphysis and epiphysis.
In addition to preventing varus or valgus motion,
the collateral ligaments span the physis, thereby
protecting it from injury. Furthermore, the blood
supply to the phalangeal condyles is present in
branches of the digital artery that travel with the
collateral ligaments.
The accessory collateral ligaments insert on the
volar plate to complete the formation of a three-
sided box. Dorsally the extensor tendon inserts
onto the epiphysis of the middle and distal pha-
langes and resists volarly directed forces. For a
dislocation to occur, at least two structures of this
three-sided box must be damaged. In the most
common dorsal dislocation, the volar plate rup-
tures distally and the collateral ligaments rupture
proximally to destabilize the joint. In volar dislo-
cations there is frequently injury to the extensor
1014
tendon (central slip), and these may be considered
as acute boutonniere-type injuries.
Assessment of Interphalangeal
Dislocations
The evaluation of a childs Digit is more difcult
than that of an adult. The child is frequently
frightened and unable to follow directions. Obser-
vation during play may allow the examiner to
detect an area that is being protected. Swelling is
frequently present. A neurovascular exam is
important to obtain. Capillary rell, color, temper-
ature, and skin turgor should be assessed. If there
is any concern for nerve injury and the child is
unable to provide an interactive exam, a wrinkle
test may be done as described earlier in the section
on MCP joint dislocations.
Imaging
Imaging of interphalangeal dislocations is fre-
quently diagnostic. Posteroanterior and lateral
radiographs are needed. Obtaining a true lateral
of the nger in a child may be difcult, especially
in younger children who are not cooperative. It is
common to recruit the parents to hold the digit in
the appropriate position if needed. The lack of
ossication can make interpretation of radio-
graphs challenging. Reference to radiographs of
the uninjured side or a radiographic atlas may be
necessary. As the joint may not be ossied, the
alignment of the bones should be compared to
detect dislocation. A line drawn down each pha-
lanx should intersect at the level of the joint. Care
must also be taken to not miss displaced epiphy-
seal fragments representing avulsion or even frac-
ture dislocation of the epiphysis (Fig. 6). There is
a limited role for advanced imaging including
MRI or CT.
Classification
Dislocations are classied based on the degree
and direction of displacement. It is standard to
J. Lubahn et al.
refer to the more distal bone in describing the
dislocation. For example, a dislocation of the
middle phalanx dorsal to the proximal phalanx is
a dorsal dislocation. Dislocations should be
thought of as complete or incomplete and simple
or complex. In incomplete injuries the base of the
phalanx remains perched on the head of the adja-
cent phalanx. In complete injuries the phalanges
are bayoneted, as the base has dislocated
completely and is located next to the head of the
adjacent phalanx. Incomplete and complete inju-
ries have also been described as type I and type II,
though this is a less useful classication and is not
commonly used. In this scheme, a type III injury
would be a fracture dislocation. Simple versus
complex dislocations describe whether the dislo-
cation is reducible by closed versus open means,
respectively. Avulsion fractures may be consid-
ered under the Salter-Harris classication and are
Salter-Harris type III injuries. Incomplete and
complete injuries have also been described as
Type I and Type II (Eaton 1971). Fracture-
dislocations have been referred to as type III.
This numerical system is less commonly used.
Outcome Tools
Outcomes after interphalangeal dislocation are
generally scored by range of motion, as a exion
contracture is the most common complication. To
date, there are no specic outcome scores to assess
nger dislocations.
Treatment Options for Dorsal
Dislocation of the
Proximal Interphalangeal Joint
Nonoperative Management
Indications/Contraindications
Nonoperative management is generally preferred
for these injuries. Patients may require operative
treatment for unsuccessful closed reduction
attempts. Chronic injuries may also require open
reduction with release of scarred or contracted
tissues. Fracture dislocations may require
45 Hand Dislocations 1015

Fig. 6 (a) AP and lateral of 16-year-old male with index postreduction of the index PIP joint. (c) 16-year-old male
PIP fracture dislocation. Note the small epiphyseal frag- 4 weeks status postreduction of the index PIP dislocation.
ment dorsally. (b) 16-year-old male 2 weeks status Note that the dorsal epiphyseal fragment has healed

operative treatment, depending on the fracture Table 7

present (Table 7). Proximal interphalangeal joint dislocations
Nonoperative treatment
Techniques Indications Contraindications
A digital block is the preferred anesthesia modal- Successful closed Closed reduction unsuccessful
ity. Incomplete dislocations will reduce with gen- reduction Chronic dislocation (trial of closed
tle traction and translation. Complete dislocations reduction should be attempted)
require recreation of the angulation of the injury Fracture dislocation requiring open
treatment
followed by translation of the base of the phalanx
Open dislocation
back onto the head of the adjacent phalanx.
1016
Excessive longitudinal traction should be avoided
as the tensioned collateral ligaments may become
trapped on the condyles. Furthermore, longitudi-
nal traction may pull the volar plate into the joint
and produce an irreducible dislocation. For exam-
ple, a dorsal PIP dislocation may be reduced by
hyperextension and pressure on the dorsum of the
middle phalanx. If there is concern about the volar
plate being entrapped, an intra-articular injection
of saline will insufate the joint and may push free
the offending structure. Postreduction radiographs
should be obtained in orthogonal views.
Following reduction, the joint must be
protected to prevent repeat displacement. Dorsal
dislocations may be placed in a dorsal blocking
splint. Volar dislocations should be immobilized
in extension. Early range of motion of dorsal
dislocations in children has been demonstrated
to be safe and effective (Paschos et al. 2014).
However, in the pediatric population, early range
of motion of the interphalangeal joints is not as
critical as in the adult. If there is concern about the
reliability and maturity of the child, the nger may
be safely immobilized for three to four weeks.
A technique that is useful for ensuring proper
positioning of a childs nger, which may be
difcult to ensure in a cast, is to place a small
aluminum or plastic splint onto the nger and then
place a covering cast. This method may be used
for a large variety of injuries and is especially
helpful for boutonnire or mallet type injuries.
Range of motion should begin at 34 weeks for
dorsal and lateral dislocations. Volar dislocations
should have immobilization of the proximal
interphalangeal joint for 6 weeks to allow healing
of the central slip.
Volar plate avulsion fractures are a unique
injury. These are most commonly Salter-Harris
type III fractures of the middle phalanx base.
Despite being physeal fractures, these rarely
require operative treatment. The joint is generally
stable throughout its range of motion. Early range
of motion with 1 week of buddy taping or a dorsal
splint may be used. Unstable injuries, which are
more common after a dislocation, may require a
slightly longer period of immobilization. Early
range of motion has been shown to be effective
even in the pediatric population (Weber
J. Lubahn et al.
et al. 2009; Rimmer and Burke 2009). Several
authors have cautioned against the danger of
overtreating these injuries by prolonged immo-
bilization (Cornwall 2012; Waters 2010). Some
caution should be taken to not mistake large frag-
ments for simple avulsions, as these will behave
more like adult fracture dislocations than pediatric
avulsions.
Outcomes
Good outcome data for dislocations in children is
lacking. Children generally regain stable range of
motion. Formal hand therapy is rarely necessary
as age-appropriate activities usually can accom-
plish the same goal. Volar plate avulsion fractures
frequently progress to nonunion, though this is of
little consequence as full, painless range of motion
generally results.
Operative Management
Indications/Contraindications
Operative treatment is needed for treatment of
complex interphalangeal dislocations. It must be
recognized that excessive traction may convert a
simple dislocation to a complex one. A complex
dislocation may have several etiologies including
(1) interposition of the volar plate between the
phalanges and (2) buttonholing of the phalangeal
head between the central slip and lateral band
(Fig. 7). Volar plate interposition is more com-
monly seen with dorsal dislocations and
buttonholing through the extensor mechanism is
seen in volar dislocations. Fracture dislocations of
the interphalangeal joints may also require open
reduction (Table 8).
Surgical Procedure
Preoperative Planning
Surgery for pediatric interphalangeal dislocations
is generally straightforward. The goal of surgery
is to remove the offending structure preventing
relocation. In dorsal dislocations this is typically
the volar plate. In volar or lateral dislocations, the
proximal phalanx head may be buttonholed
through the extensor mechanism. A dorsal or
volar approach may be used to address the volar
45 Hand Dislocations 1017

Fig. 7 Simple versus

complex dislocation. (a)
Figure A illustrates a simple
dislocation. Note that the
proximal phalanx is
perched on the metacarpal
head and that the volar plate
is torn at its origin. (b) is a
complex dislocation. The
volar plate has moved with
the proximal phalanx and is
interposed in the joint,
preventing relocation. Also,
in simple dislocations (a)
the joint appears
hyperextended, while in
complex dislocations, (b)
the bones are in bayonet
apposition

Table 8
Table 9
Proximal interphalangeal joint dislocations
Open reduction for proximal interphalangeal joint
Operative treatment dislocation
Indications Contraindications Preoperative planning
Closed reduction unsuccessful Closed reduction OR table: radiolucent hand table
successful
Position: supine
Chronic dislocation (trial of closed
Fluoroscopy: brought in from end of hand table
reduction should be attempted)
Equipment: standard hand surgery operating set, K-wire
Fracture dislocation requiring open
set
treatment
Tourniquet: nonsterile
Open dislocations

plate. In contrast, a dorsal approach is needed to
release the extensor mechanism. Hardware is gen-
erally not necessary (Table 9).
Surgical Approach and Technique
The entrapped volar plate may be approached
through a volar or dorsal approach. The volar
approach is through a standard Bruner-type inci-
sion centered over the PIP joint. The A3 or A5
pulley is incised to allow reection of the exor
tendon(s) and identication of the joint. The volar
plate is extracted using a small hook or forceps. If
there is difculty mobilizing the volar plate
extraction may be aided by splitting the volar
plate longitudinally with a knife and extracting
each half separately. The volar plate may then be
1018
repaired to its insertion with 4-0 braided strong
nonabsorbable suture to increase stability.
Alternatively, a small incision can be made
dorsolaterally centered between the central slip
and lateral band at the level of the PIP joint.
A Freer elevator can then be introduced through
a small capsulotomy into the joint. Using the tip of
the elevator, the surgeon then attempts to push
free the entrapped volar plate. This technique is
less traumatic than a volar incision. Intraoperative
uoroscopy should be used to conrm the
reduction.
Volar and lateral dislocations may have entrap-
ment of the phalangeal head by the extensor mech-
anism or collateral ligament. A curved dorsal
incision will allow complete visualization of the
tendons and joint. The head is then gently freed
from the offending structure and the joint is reduced.
Pin xation is generally not needed as the joint is
fairly stable once reduced. Longitudinal splits in the
extensor tendon can be repaired using 40 polypro-
pylene. In children, 40 plain gut may be used to
close the skin without requiring suture removal.
Early range of motion within several days of
surgery is encouraged for dorsal dislocations. The
need for protective splinting may be determined
by assessing joint stability during the operation. A
dorsal blocking splint may be used as needed.
With volar dislocations there is often injury to
the central slip. In this case, prolonged immobili-
zation of the PIP joint in extension for 6 weeks or
longer is often necessary to prevent a boutonniere
deformity (Tables 10, 11, 12 and 13).
Preferred Treatment
Most dislocations of the proximal interphalangeal
joint can be treated nonoperatively utilizing closed
reduction techniques. Complex dorsal dislocations
are approached through a volar incision, which
allows for identication of all offending structures,
reduction of the joint, and repair of the volar plate.
Volar dislocations are approached through a curved
dorsal incision. The central slip may be inspected
for injury, and if it is injured, a thermoplast splint or
cylinder cast, to maintain the PIP joint in extension,
is fashioned and used to prevent the development
J. Lubahn et al.
Table 10
Mini-open reduction of the proximal interphalangeal
joint
Surgical steps
Dorsolateral incision between central slip and extensor
Incise capsule
Insert small elevator into joint to remove volar plate
Reduce PIP joint
Table 11
Open reduction dorsal dislocation of the proximal
interphalangeal joint
Surgical steps
Bruner incision centered on PIP joint
Incise exor sheath over A3 pulley
Mobilize exor tendon to expose joint
Extract volar plate from PIP joint
Reduce PIP joint
Repair volar plate to insertion using 40 strong
nonabsorbable suture
Table 12
Open reduction of volar dislocation of the proximal
interphalangeal joint
Surgical steps
Dorsal incision centered over proximal phalanx head
Identify proximal phalanx and entrapping structures
(lateral band, collateral ligament)
Free proximal phalanx head and reduce joint
Table 13
Open reduction proximal interphalangeal joint
postoperative protocol
Dorsal blocking splint to ngertips for dorsal dislocation
Intrinsic plus splint followed by mallet splint for volar
dislocation or dorsal blocking splint for dorsal dislocation
Length of immobilization: 23 weeks for dorsal
dislocation, 6 weeks at PIP joint for volar dislocation
Rehab protocol: Dorsal dislocation: Initiate DIP joint
exion while preventing hyperextension after 12 weeks.
Volar dislocation: Begin DIP joint motion after 6 weeks
Return to sport when range of motion returns
For very small children who require splinting consider
placing a thermoplast splint directly on the skin to ensure
joint position, then overwrap with cast material.
Temporary k-wire xation of the DIP joint may also be
used
45 Hand Dislocations
Table 14
Proximal interphalangeal joint dislocation
Potential pitfalls and preventions
Pitfall Prevention
Conversion of simple to Avoid excessive traction
complex dislocation during closed reduction
attempts
Chronic boutonniere Splint PIP in extension for
deformity after volar 6 weeks to allow healing of
dislocation central slip
Chronic instability or Obtain good quality
subluxation radiographs to rule out
fracture dislocation
of a boutonniere deformity. Though children are
less prone to developing contracture at the PIP
joints, early range of motion may be allowed in a
compliant patient (Table 14).
Dislocations of the Finger Distal
Interphalangeal Joint and Thumb
Interphalangeal Joint
Dislocations of the distal interphalangeal (DIP) or
thumb interphalangeal (IP) joint are fairly uncom-
mon injuries. In children, these may be seen after a
crush injury, such as catching the nger while
closing a door. Most commonly these are open
injuries due to the tight skin envelope. The
supporting structures of the joint are similar to
that of the nger PIP joint. The joints are well
stabilized by the collateral ligaments and volar
plate. The short lever arm of the distal phalanx,
as well as the close insertion of the exor and
extensor tendons, imparts additional strength and
makes dislocation unlikely. Dislocations may be
dorsal, lateral, or volar. Volar dislocations fre-
quently have injury to the terminal extensor ten-
don and are therefore a mallet-type injury
equivalent.
Assessment of Distal Interphalangeal
Joint Dislocation
The skin envelope should be examined for breaks.
With a crush mechanism, injury to the nail plate or
1019
underlying bone should be assessed for.
Posteroanterior and lateral radiographs of the
digit should be obtained. There is a very limited
role for CT or MRI. Care must be taken not to miss
a fracture of the distal or middle phalanx, and the
integrity of the exor and extensor tendons should
be assessed.
Distal Interphalangeal Joint
Dislocation Treatment Options
Dislocations may generally be managed
nonoperatively. Open injuries may be treated
utilizing a digital block, followed by a thorough
irrigation and debridement. Reduction of the
joint is performed by traction and direct pressure
on the distal phalanx opposite the direction of
the dislocation. Postreduction radiographs
should be obtained to conrm a symmetric
reduction was obtained. The skin break may be
sutured or left open. In general, transverse
lacerations heal well without sutures,
whereas longitudinal and/or oblique lacerations
are best treated with reapproximation of the
tissue with one or two stitches. Absorbable
sutures such as 40 plain gut are preferred in
children.
The joint may be immobilized in a dorsal splint
for 23 weeks, followed by the initiation of range
of motion exercises. Lateral dislocations, with
injury to the collateral ligaments, may be
immobilized for 34 weeks. Chronic instability
is rare.
Surgery is rarely needed and is only necessary
if the joint is irreducible by closed means. Most
commonly the offending agent is an interposed
volar plate. Additional structures that may block
the reduction include the exor or extensor ten-
dons, the collateral ligaments, sesamoid bones, or
a fracture fragment. Irreducible dislocations
require surgery to remove or maneuver the
offending structure.
Volar dislocations are very rare. These are
essentially mallet injuries with concomitant injury
to the terminal extensor tendon and so should be
treated by splinting the DIP joint in full extension
for 6 weeks. If there is a break in the skin, the
1020
Table 15
Distal interphalangeal joint dislocation
Potential pitfalls and preventions
Pitfall Prevention
Missed fracture Obtain good quality
radiographs
Chronic mallet Splint DIP in extension for
deformity after volar 6 weeks to allow healing of
dislocation the terminal tendon
sutures on the dorsum of the DIP joint should be
passed deep down to the level of the bone. If
placed this way, the sutures will also grab and
reapproximate the extensor tendon
(dermatotenodesis, Brooks 1958). Consideration
can also be given to temporary k-wire xation of
the DIP joint to prevent movement during exten-
sor tendon healing. This is especially useful when
the joint is very unstable or when difculty is
anticipated in maintaining a proper splint (e.g.,
the young child) (Table 15).
Complications
Complications following PIP or DIP joint dislo-
cations are unusual in a child. The greatest con-
cerns are for persistent instability, stiffness, or
weakness. Instability is extremely rare and there
is scant literature on this topic in the pediatric
population. Treatment of PIP instability should
aim to preserve motion and growth. DIP instabil-
ity may be treated with arthrodesis of the joint.
Stiffness is also rare. Hand therapy including
exercises and splinting may be considered. Per-
sistent extensor weakness may require reconstruc-
tion of the central slip or terminal tendon
(Table 16).
Summary
PIP and DIP joint dislocations are relatively rare
injuries in the child. These injuries can generally
be treated nonoperatively. Attention should be
paid to preventing instability and preserving
motion.
J. Lubahn et al.
Table 16
Proximal or distal interphalangeal joint dislocation
Complications
Complication Management
PIP persistent Physis-preserving surgery to restore
instability stability (volar plate imbrication,
lateral band tenodesis)
Stiffness Therapy
DIP persistent Arthrodesis or chondrodesis
instability
Boutonnire Central slip reconstruction
deformity
Thumb Metacarpophalangeal Joint
Dislocations
As opposed to thumb interphalangeal (IP) joint
dislocations, a thumb metacarpophalangeal
(MCP) joint dislocation is exceedingly rare in
the pediatric patient (Fig. 8).
As with other MCP dislocations, it is important
to identify the dislocation and attempt a prompt
reduction. The most common mechanism of
thumb MCP dislocations is hyperextension of
the digit. This results in a rupture of the volar
plate, usually at the proximal aspect. The capsule
and collaterals can also be ruptured with a
corresponding higher energy hyperextension
force. Most dislocations can be reduced by closed
methods; however, complex dislocations have
been reported in the adult literature (Kozin 2006).
In a similar manner to nger MCP joints, the
thumb MCP can be reduced by the same methods.
Caution must be heeded to the noose effect of
soft tissue around the metacarpal head with
aggressive traction during reduction attempts.
Reduction Technique
The thumb metacarpal is placed into a position of
adduction and exion while placing direct pres-
sure on the base of the proximal phalanx. If there
is concern for exor pollicis longus (FPL) entrap-
ment, IP exion will relax the tension and assist in
the reduction. The proximal phalanx is then
reduced onto the metacarpal head and brought
45 Hand Dislocations
Fig. 8 (a) Posteroanterior and lateral radiograph of
6-year-old male with a right thumb metacarpophalangeal
joint dislocation. (b) Posteroanterior and lateral radiograph
of the 6-year-old male immediately following the closed
into exion. As with other dislocations, the stable
arc of motion, as well as joint stability, must be
assessed postreduction [Greens 6th ed.].
Open reduction of complex dislocations can be
accomplished via a dorsal approach exploiting the
interval between the extensor pollicis longus
(EPL) and extensor pollicis brevis (EPB). Alter-
natively, a volar approach can be used. This
allows inspection and repair of the volar plate.
During an open reduction, inspection to assess
injury should include the adductor aponeurosis
as well as the collateral ligaments. Failure to repair
an ulnar collateral ligament could result in insta-
bility of the thumb MCP joint.
Physical Therapy Recommendations
A thumb MCP dislocation should be treated in a
similar manner to a thumb UCL repair.
Nonoperative treatment involves protection of
the thumb MCP joint in a thumb spica cast for
1021
reduction of the thumb MCP dislocation. (c)
Posteroanterior and lateral radiograph of 6-year-old male
2 months status postreduction of thumb MCP dislocation
4 to 6 weeks. The important principle is immobili-
zation of the thumb MCP joint. The IP joint may be
left free to permit limited use of the hand. After
removing the cast, a removable splint is applied for
an additional 2 weeks while active range of motion
exercises are begun. Patients may wean from the
splint over the following weeks. Return to sport is
generally at 3 months following the injury, unless a
splint protecting the MCP joint is worn. Patients
treated surgically may begin earlier range of motion
exercises, focusing on exion and extension at the
MCP joint. Children however are unlikely to
develop signicant stiffness and so preference
may be given to immobilization for 4 to 6 weeks.
Thumb Ulnar Collateral Ligament
Injuries
The thumb MCP joint is largely analogous to the
nger MCP joints. Volar and dorsal dislocations
are treated similarly to the nger MCP joint. The
1022
Fig. 9 Stener lesion. (a) The normal ulnar collateral liga-
ment of the thumb is deep to the adductor aponeurosis. Part
of the aponeurosis is removed (cutout) to show the UCL
insertion. (b) With avulsion from its insertion and abduc-
tion of the thumb, the ligament with the attached fracture
principal difference of the thumb MCP joint is the
signicance of the adductor aponeurosis and ulnar
collateral ligament. The substantial valgus and
adduction forces on the thumb ray make the
ulnar collateral ligament prone to injury. These
forces are somewhat mitigated by the additional
support of the adductor aponeurosis, which is the
insertion of the adductor pollicis brevis muscle
onto the thumb proximal phalanx and thumb
extensor mechanism. The adductor aponeurosis
provides dynamic stability to the thumb. In the
case of a thumb ulnar collateral ligament (UCL)
injury, however, the adductor aponeurosis may
inhibit healing by interposing between the
detached UCL and its insertion. This is known
as the Stener lesion (Stener 1962) (Fig. 9).
Valgus stress at the thumb MCP joint may
cause (1) sprain or partial tear of the UCL;
(2) complete rupture, most commonly at the distal
J. Lubahn et al.
fragment is pulled proximal to the aponeurosis and moves
supercial. (c) With relocation of the joint, the aponeurosis
remains interposed between the fracture fragment and the
proximal phalanx, thus preventing healing from occurring
insertion; or (3) avulsion with a Salter-Harris
type I, II, or III fracture. The most common pattern
is a Salter-Harris type III fracture with a consider-
able amount of the articular surface often involved
(White 1986; Kozin 2006). Thumb MCP joint
ulnar collateral ligament injuries have also been
referred to as gamekeepers thumb or skiers
thumb.
Thumb UCL injuries are less common in chil-
dren than adults. When the child begins sporting
activities in adolescence, these injuries become
more common. The mechanism of injury is a
valgus or abduction force.
Pathoanatomy and Applied Anatomy
Support of the thumb MCP comes from the volar
plate and collateral ligaments. Additional volar
45 Hand Dislocations
support comes from the insertion of the thenar
muscles into the sesamoids, which are present
within the volar plate. The thumb MCP joint
volar plate somewhat weakened by a lack of
checkrein ligaments. Dynamic support against
varus or valgus stress is provided by the adductor
and abductor aponeuroses.
The Stener lesion results from interposition of
the adductor aponeurosis between the proximal
phalanx and the torn ligament or displaced frac-
ture (Fig. 9). This occurs after signicant angula-
tion of the thumb MCP joint occurs that allows the
torn distal extent of the ligament or bony fragment
to slide proximally. Once this occurs, the ligament
or bone will displace supercial to the aponeuro-
sis. The interposition of the aponeurosis will pre-
vent healing of the ligament back to the bone or
the fracture fragments to each other. Therefore,
open treatment is necessary to align the damaged
ends and allow healing.
Assessment
The usual history involves a description of a trau-
matic injury, which often occurs during a sporting
event. Activities, such as holding a pole during
skiing, place a signicant force at the thumb MCP
joint ulnar collateral ligament. The thumb should
be assessed for swelling, tenderness, ecchymosis,
and sensation. Tenderness is usually well local-
ized to the UCL, especially the distal insertion.
The presence of ecchymosis in the rst web space
is suggestive of a fracture or complete rupture.
Valgus stress at the MCP joint will cause pain
with a UCL injury. More importantly, increased
laxity and the lack of a rm end point are highly
suggestive of displaced fracture or complete UCL
tear. The contralateral MCP joint provides a good
basis for comparison, as laxity at the thumb MCP
joint is highly variable.
The joint should be stressed both in full exten-
sion and in 40
exion. The ulnar collateral liga-
ment is at maximal tension at the 40
exed
position and so this is probably the most important
position to test (Harley 2004). Care should also be
taken to keep the thumb in neutral pronation/supi-
nation during testing (Mayer et al. 2014).
1023
Recommended criteria to diagnose complete
ulnar collateral ligament injury are 30
of laxity
or an increase of 15
compared to the other side.
Lack of a rm endpoint is also suggestive of a
complete collateral ligament injury. Injection of
local anesthetic may be helpful for the patient to
allow their thumb to be stressed.
Radiographs, including a PA and lateral view,
are necessary to assess the joint alignment and for
the possibility of a fracture (Fig. 10). It is impor-
tant to remember that radiographs of the thumb
ray are perpendicular to those of the rest of the
hand and so a specic thumb series should be
ordered. Stress radiographs may show joint
space widening, especially when compared to
the contralateral hand. Assessment of a thumb
UCL injury is primarily clinical; however, the
use of ultrasound and use of MRI have both
been described (Hglund et al. 1995). MRI in
particular may be useful to diagnose the Stener
lesion, though it carries additional cost and may
require sedation in a child (Spaeth et al. 1993).
Diagnosis of the Stener lesion is both impor-
tant and challenging. There is no reliable physical
exam tool. Signicant consideration should be
given to ultrasound or MRI if there is a complete
tear and nonoperative treatment is selected by the
family.
Outcome Tools
There are no commonly used outcome tools spe-
cically designed for thumb UCL injuries. The
ability of the thumb MCP joint to resist valgus
stress is the most important factor, and therefore,
its competence should be assessed at each follow-
up visit.
Nonoperative Management
Indications
Nonoperative management of a thumb UCL
injury is reasonable with sprains, incomplete rup-
ture of the ligament, and nondisplaced fractures. If
there is complete tear or a displaced fracture,
preference should be given to operative treatment
1024
Fig. 10 Lateral and PA
views of the thumb
demonstrate a dorsal
dislocation of the thumb
metacarpophalangeal joint.
Note a single PA view of the
thumb on the right can have
a normal appearance. A full
series of thumb radiographs
should always be obtained
to conrm that there is not a Stener lesion. Oper-
ative treatment is also necessary to reduce
displaced articular fractures, which may involve
up to one-third of the joint. Open reduction and
internal xation is necessary to restore the integ-
rity of the thumb UCL and obtain a congruent
articular surface. Consideration should be given
to obtaining an ultrasound or MRI to rule out a
Stener lesion prior to undergoing nonoperative
treatment if a complete tear is suspected.
Nonoperative treatment involves protection of
the thumb MCP joint in a thumb spica cast for 46
weeks. The important principle is immobilization
of the thumb MCP joint. The IP joint may be left
free to permit limited use of the hand. After
removing the cast, a removable splint is applied
for an additional 2 weeks while active range of
motion exercises are begun. Patients may wean
from the splint over the following weeks. Return
to sport is generally at 3 months following the
injury, unless a splint protecting the MCP joint is
worn (Table 17).
J. Lubahn et al.
Table 17
Thumb ulnar collateral ligament injury
Nonoperative treatment
Indications Contraindications
Nondisplaced Displaced fracture
fracture
No evidence of Instability or lack of a rm end point
joint laxity with valgus stress of the thumb MCP
joint
Stener lesion on ultrasound/MRI
Outcomes of Nonoperative Treatment
There are no studies looking specically at ulnar
collateral ligament injuries in the pediatric popu-
lation. However, nonoperative management of
incomplete tears or nondisplaced avulsion frac-
tures of the thumb UCL is generally associated
with good healing rates and no residual disability
(Sollerman et al. 1991). Nonoperative manage-
ment has been described for complete tears and
displaced fractures. Published results are
45 Hand Dislocations
inconsistent, ranging from 100 % satisfaction
(Kuz et al. 1999) to persistent pain and disability
(Dinowitz et al. 1997). Nonunion rates of 25 %
may be seen with nonoperative management,
though this does not seem to correlate with poor
outcomes (Kuz et al. 1999).
Operative Treatment for Thumb
UCL Injury
Operative treatment for a thumb UCL injury is
indicated when complete tears or displaced frac-
tures are present. Complete tears may be diagnosed
on physical examination by increased laxity or the
lack of a rm end point with valgus stress. Frac-
tures should be evaluated for by obtaining radio-
graphs of the thumb ray. Surgery should be
considered for all tears unless there is strong evi-
dence that there is an incomplete rupture present or
a nondisplaced fracture is seen on radiographs.
Surgical Treatment
The goal of surgery is to anatomically reattach and
allow for healing of the ruptured ligament or
avulsed bony fragment. The UCL injury is gener-
ally distal; thus, it is on the side of the thumb
proximal phalanx. The surgical approach should
allow for identication of the injury as well as
visualization of the adductor aponeurosis, which
may be interposed between the ligament and bone
(the Stener lesion). A curved S-shaped incision
that begins on the dorsum of the thumb metacar-
pal, travels volarly at the MCP joint, and con-
tinues across the midaxial ulnar side of the
thumb proximal phalanx allows excellent visual-
ization. Care must be taken to identify and protect
branches of the dorsal radial sensory nerve, which
are typically noted on the dorsal aspect of the
incision. The adductor aponeurosis must be
divided to allow for visualization of the proximal
phalanx base. Transosseous sutures, wires,
screws, or suture anchors may all be used to obtain
xation. After repair of the ligament or fracture,
the adductor is repaired to restore its role as a
dynamic stabilizer of the joint (Table 18).
1025
Table 18
Surgical treatment for thumb UCL injury
Preoperative planning
OR table: radiolucent hand table preferred
Position: supine
Fluoroscopy: brought in from end of hand table
Equipment: standard hand surgery operating set, drill,
suture anchors, K-wires
Tourniquet: nonsterile
Technique
The ligament is approached through a dorsal inci-
sion. A gently curved incision is designed to curve
from the dorsum of the metacarpal to the midaxial
ulnar side of the thumb proximal phalanx. Care
must be taken when elevating the skin to protect
branches of the supercial radial nerve. The
adductor aponeurosis and extensor mechanism
are exposed. A Stener lesion, if present, may be
visualized as a knot of brinous tissue lying super-
cial to the aponeurosis. The aponeurosis is
sharply divided from the extensor mechanism at
its insertion to expose the underlying proximal
phalanx and MCP capsule. The ligament may be
identied at this step, if it was not previously
identied as a Stener lesion. If a fracture is pre-
sent, a longitudinal capsulotomy will be necessary
to allow visualization of the fracture and articular
surface. The joint is gently irrigated and debrided
to remove any hematoma and/or loose cartilagi-
nous pieces. If a midsubstance rupture of the
ligament is present, it may be repaired at this
point. Figure-of-eight nonabsorbable sutures are
placed utilizing a substantial size suture, such as
20 Fiberwire.
More commonly, the injury to the UCL is at its
insertion into the proximal phalanx base. If there
is no fracture present, the ligament is debrided and
reattached to its insertion. Transosseous sutures or
a suture anchor with 20 or 30 strong
nonabsorbable suture may be used. In children,
care should be taken to avoid injury to the proxi-
mal phalanx physis, regardless of the method cho-
sen. Small (~2.5mm) anchors may be used.
Intraoperative uoroscopy may be utilized to con-
rm the appropriate placement of all of the
1026
hardware not crossing the physis. Care should be
taken to ex the joint to 45
while tying the
sutures to prevent overtightening of the ligament.
The proper insertion of the ulnar collateral liga-
ment is on to the volar half of the proximal pha-
lanx. Dorsal or midaxial attachment of the
ligament is not anatomic and may cause joint
stiffness (Bean et al. 1999). If there is a fracture
present, it should be dbrided and the fragment
reduced. Fixation is chosen based upon the size of
the fragment. Options include Kirschner wire x-
ation, tension wire xation, suture anchors, or
mini-screw placement (Kozin and Bishop 1994).
For xation utilizing a Kirschner wire, a
double-ended wire is rst inserted antegrade
through the fracture site and out through the skin
on the radial side of the thumb. Another Kirschner
wire or small drill is then used to make a hole 1 cm
distal to the epiphysis. A strong nonabsorbable
suture is placed through the drill hole, crossed,
and passed through the ligament at its insertion to
the fragment. The fragment is reduced and the rst
wire is drilled back retrograde to spear the frag-
ment in its reduced position. The suture is then
tied to provide a tension band construct (Kozin
2006).
Closure requires careful repair of the adductor
aponeurosis incision made during approach. Sub-
sequently, the skin is closed with absorbable
suture. A thumb spica cast is applied that covers
the percutaneous wire, if present. The thumb
interphalangeal joint is not incorporated into the
cast in order to promote gliding of the extensor
mechanism. The Kirschner wire is removed
4 weeks following surgery and active range of
motion is initiated. A splint is fabricated to protect
the repair during activities and is weaned over the
next 4 weeks. Sports and activities that place
radial pressure on the tip of the thumb are avoided
for 3 months (Tables 19 and 20).
Surgical Outcomes
Near-complete return of range of motion and
strength may be expected after surgical treatment.
Suture anchor repair was reported to have a 7 %
loss of MCP motion, a 21 % loss of IP joint
J. Lubahn et al.
Table 19
Surgical treatment for thumb UCL injury
Surgical steps
Dorsal ! volar curved incision
Exposure of the adductor aponeurosis and extensor
mechanism
Divide adductor aponeurosis longitudinally ulnar to
the EPL tendon to expose metacarpophalangeal joint
Identify Stener lesion if present
Repair midsubstance rupture with nonabsorbable
suture (e.g., 20 braided polyester)
Fracture:
Debride fracture
Pass K-wire through fracture site and out
percutaneously
Pass nonabsorbable suture through drill hole in
proximal phalanx and ligament insertion on fragment
Reduce fracture and advance wire retrograde to x the
fragment
Tie suture in gure-of-8 pattern
Ligament avulsion from insertion:
Debride insertion site
Place suture anchor in volar half of proximal phalanx
Flex MCP joint to 45
to prevent overtightening
Repair ligament to insertion using locking stitch
Repair adductor aponeurosis to EPL
Thumb spica cast with IP joint free
Table 20
Surgical treatment for thumb UCL injury
Postoperative protocol
Thumb spica cast for 4 weeks
Removable thumb spica splint for weeks 58
Splint removed for range of motion exercises and
sedentary activity
Rehab protocol: encourage range of motion of thumb IP,
MCP, and CMC joints
Return to sport at 3 months
motion, and equivalent pinch and grip strength
when compared to the opposite hand (Kozin
1995).
Preferred Treatment
Operative treatment of most UCL injuries is
recommended to ensure reliable healing.
Nonoperative management may be considered
45 Hand Dislocations 1027

Table 21 Table 22
Thumb UCL injury Thumb UCL injury
Potential pitfalls and preventions Complications
Pitfall Prevention Complication Treatment
Chronic instability Avoid missing a Stener lesion Chronic instability Avoid missing a Stener lesion
Neuropraxia of the Identication and careful Neuropraxia of the Identication and careful
radial sensory nerve protection during the surgical radial sensory nerve protection during the surgical
approach approach
Overtightening of the Avoid inserting the ligament Overtightening of the Avoid inserting the ligament
ligament too distal or too dorsal. Tighten ligament too distal or too dorsal. Tighten
ligament with MCP in 45
of the ligament with MCP in 45

exion of exion

for injuries with no laxity and a rm end point

with valgus stress at the thumb MCP joint. Frac-
tures should be treated operatively unless they are
nondisplaced. Consideration should be given to
obtaining an MRI or ultrasound in order to rule
out a Stener lesion if nonoperative treatment is
being considered. Our preference is to proceed to
surgery if any doubt is present. We do not rou-
tinely obtain stress radiographs.
For ligamentous injuries, suture anchors pro-
vide reliable xation and may be easily placed
into the metaphysis under radiographic guidance
to avoid the physis. Fractures are reliably xed
using a Kirschner wire and tension band-type
construct (Table 21).
approach. The nerves can also be rapidly and
easily mobilized proximally and distally to pre-
vent a traction injury (Table 22).
Summary
Injuries of the thumb UCL are uncommon inju-
ries. They are often treated surgically to ensure
healing. Future research will enable better dis-
crimination of operative and nonoperative
candidates.

Management of Complications
Chronic instability of the UCL can be a challeng-
ing complication to deal with. Treatment is varied
depending on the chronicity of the lesion, the age
of the patient, the amount of pain present, and the
motion of the MCP joint. Late repair of the liga-
ment may be attempted, as reconstruction utiliz-
ing a tendon graft is difcult to perform in the
pediatric population. Given the large range of
motion of the CMC joint, arthrodesis or
chondrodesis provides a reliable way to restore
painless use of the thumb after any problem at the
thumb MCP joint.
Neuropraxias of the radial sensory nerve are
often self-limiting injuries. These injuries are best
managed by meticulous prevention. Care must be
taken to not divide the nerve branches during the
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 Carpal Fractures
46
Theresa O. Wyrick

Contents Abstract
Introduction to Carpal Fractures . . . . . . . . . . . . . . . . 1030 Fractures of the pediatric carpus are relatively
rare. The literature describing them consists
Pathoanatomy and Applied Anatomy Relating
to Carpal Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1030
mostly of case reports and case series, with
the majority of reports devoted to fractures of
Assessment of Carpal Fractures . . . . . . . . . . . . . . . . . 1031 the scaphoid in this population. A fair number
Signs and Symptoms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1031
Imaging and Other Diagnostic Studies . . . . . . . . . . . . . 1031 of series available report on the treatment of
Injuries Associated with Carpal Fractures . . . . . . . . . 1031 scaphoid nonunions in the pediatric popula-
Classication . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1032 tion, which is also a relatively rare entity. Due
Treatment Options . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1032 to the ossication of the pediatric carpus,
Nonoperative Management . . . . . . . . . . . . . . . . . . . . . . . . . 1032 throughout childhood and early adolescence,
Operative Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1033 a large portion of the pediatric carpus is carti-
Scaphoid Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1033
Capitate Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1035
laginous, which can make radiographic evalu-
Lunate Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1035 ation difcult. Therefore, a high index of
Trapezium and Trapezoid Fractures . . . . . . . . . . . . . . . . 1035 clinical suspicion for a carpal fracture in the
Hamate Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1035 pediatric population must be present on the
Triquetrum Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1035
Pisiform Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1036
part of the examining physician when radio-
Surgical Procedure: Operative Treatment of graphs of the immature carpus are negative for
the Acute Scaphoid Fracture . . . . . . . . . . . . . . . . . . . . . . . 1036 fracture, but the physical examination is sug-
Surgical Procedure: Operative Treatment of gestive of injury being present. Immobilization
the Scaphoid Fracture Nonunion . . . . . . . . . . . . . . . . . . . 1041
and careful follow-up with observation and
Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1045 repeat radiographs in 23 weeks are warranted
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1046 to aid in diagnosis.
Fractures of the scaphoid are the most com-
monly seen pediatric carpal fracture. This is
followed in incidence by fractures of the capi-
tate, with fractures of the remaining carpal
bones quite rare in isolation. These other carpal
fractures are usually nondisplaced and require
T.O. Wyrick only routine immobilization to obtain success-
Department of Orthopaedic Surgery, Arkansas Childrens
ful healing. However, in the case of a displaced
Hospital, University of Arkansas for Medical Sciences,
Little Rock, AR, USA carpal fracture, open reduction and internal
e-mail: wyricktheresao@uams.edu xation is warranted. Most often, temporary
# Springer Science+Business Media New York 2015 1029
J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_47
1030
Kirschner wire xation is sufcient. Failure to
diagnose or initiate appropriate treatment
promptly in the case of a scaphoid fracture
can result in nonunion. However, even with
full patient compliance, accurate and early
diagnosis, and appropriate treatment, nonunion
can occur. Fortunately, nonunion of the scaph-
oid is rare in the pediatric and adolescent pop-
ulation. Treatment of scaphoid nonunions
surgically with nonvascularized bone graft
from the distal radius yields union rates
approaching 100 % with few complications or
long-term problems.
Introduction to Carpal Fractures
Carpal fractures in the pediatric population are
relatively rare. However, there are an increasing
number of reports in the literature identifying
them and their associated injuries. This may be
due to an increase in incidence, but it may also be
due to an increased recognition on the part of the
treating physician (De Putter et al. 2011; Gholson
et al. 2011).
Pediatric carpal fractures are often not appreci-
ated on the initial radiographs due to the ossica-
tion process of the carpal bones and the inherent
difculty associated with interpreting wrist
radiographs in the skeletally immature popula-
tion. Repeat radiographs or more advanced imag-
ing modalities, such as magnetic resonance
imaging (MRI) or computed tomography (CT),
may be needed to conrm the evaluating physi-
cians suspicions of an occult carpal fracture in the
setting of normal radiographs. Most carpal frac-
tures in children heal uneventfully with simple
cast immobilization, and therefore, only a rela-
tively small number are treated with operative
intervention.
The most commonly fractured carpal bone
seen in the pediatric patient is the scaphoid.
Nae reported on 82 carpal fractures in children
with 71 of the fractures involving the scaphoid.
Distal pole fractures in the scaphoid were found to
be particularly common in children in this series
(Nae 1987).
T.O. Wyrick
Pathoanatomy and Applied Anatomy
Relating to Carpal Fractures
The carpus in the young child is mostly cartilag-
inous providing an inherent protection from frac-
ture. Falls onto the outstretched pediatric hand
more commonly result in distal radius fractures
than carpal fractures. Therefore, it is theorized that
a signicant amount of biomechanical force is
required to create a carpal fracture in this younger
population. As the carpal bones ossify, the injury
patterns transition to become more like what is
seen in the adult population. In the adolescent
patient with a traumatic wrist injury, adult
patterns of injury are more commonly seen
including fractures and ligamentous injuries
(Goddard 2010).
It is important to understand the typical ossi-
cation pattern of the carpus, as well as normal
variations, when evaluating the pediatric wrist
radiograph in the setting of an injury. The ossica-
tion center of the most commonly fractured
carpal bone, the scaphoid, rst appears in children
by age 5 or 6 years and may be bipartite in less
than 1 % of children (Doman and Marcus 1990).
The ossication of the scaphoid proceeds in a
distal to proximal direction, following the blood
supply. As the proximal pole is the last area to
ossify, wrist radiographs may appear to have
widening of the scapholunate interval in the setting
of no pathology or injury (Fig. 1). Ossication of
the scaphoid is generally complete by 1315 years
of age.
All of the carpal bones are completely cartilagi-
nous at birth. The ossication center of the capitate
is the rst to appear and is usually visible by the
third or fourth month of life. This is followed very
closely by the hamate ossic nucleus. The
triquetrum begins to ossify next between 7 months
and 2 years of age. Subsequently, the lunate
becomes visible between 3 and 4 years of age.
The trapezium, trapezoid, and scaphoid all begin
to ossify around the same age, between 3 and
6 years. The last ossic nucleus to appear is that of
the pisiform, around 812 years of age. Completion
of the carpus ossication process occurs between
8 and 12 years of age (Hsu and Light 2006).
46 Carpal Fractures
Fig. 1 PA radiograph of a skeletally immature wrist in a
10-year-old male patient demonstrating a scaphoid frac-
ture. The distance between the scaphoid and lunate is
apparently widened due to the proximal pole not yet
being ossied. The scapholunate ligament was found to
be intact and uninjured upon direct inspection at the time of
surgical treatment
Assessment of Carpal Fractures
Signs and Symptoms
Children generally present following a fall onto an
outstretched arm. Additionally, sports and other
extracurricular activities are common mecha-
nisms of injury. Children may complain of pain;
however, they may be more stoic than adults. A
hesitance to report an injury or pain to their par-
ents or other authority gures may be present as a
result of their desire to continue playing a sport.
A thorough evaluation of wrist and digital
range of motion is necessary as well as an evalu-
ation of the entire extremity in order to identify
any potential concomitant areas of injury. Swell-
ing and ecchymosis are usually present in the
1031
region of the carpus. Tenderness to palpation
overlying the injured area is common, and the
wrist range of motion may be limited due to
pain and/or swelling. It is important to perform a
thorough neurovascular exam in order to identify
any deciencies as children may not readily
describe neurologic complaints to the treating
physician due to unfamiliarity with the sensation,
a lack of recognition of its importance in the
evaluation, or difculty in describing it accurately.
Nae specically looked at clinical signs and
symptoms present in pediatric carpal fractures. In
these patients, the most common signs of a carpal
fracture included dorsal wrist swelling, tenderness
in the anatomical snuffbox and/or over the distal
radius, and painful extension of the wrist and/or
thumb (Nae 1987).
Imaging and Other Diagnostic Studies
Plain radiographic evaluation of potential carpal
fractures should include standard posteroanterior
(PA) and lateral views centered on the wrist. If
there is a high suspicion of a scaphoid fracture
based on physical examination, then the addition
of two other dedicated wrist views is indicated,
including an oblique view and a PA view of
the wrist in ulnar deviation. Initial radiographs can
be negative for any appreciable fracture; however,
an underlying fracture may be present. Therefore,
immobilization is reasonable as an initial treatment
form in patients who demonstrate clinical signs and
symptoms consistent with a fracture even in the
setting of normal initial radiographs. Reexamination
clinically in conjunction with repeat plain radio-
graphs should be performed 23 weeks following
the injury to look for a fracture when it may become
more obvious radiographically. MRI can be helpful
in diagnosing an occult carpal fracture as well,
particularly in the immature wrist (Nae 1987).
Injuries Associated with Carpal
Fractures
Distal radius fractures are the most common
injury associated with carpal fractures in the
1032
Fig. 2 A 14-year-old male fell onto an outstretched hand while playing basketball sustaining a displaced physeal fracture
of the distal radius with a minimally displaced scaphoid waist fracture
pediatric population (Gholson et al. 2011). The
combination of a scaphoid and distal radius frac-
ture is most common (Fig. 2); however, fractures
of the lunate and pisiform have also been reported
in association with distal radius fractures in chil-
dren (Anz et al. 2009).
In addition, fracture of the scaphoid has been
reported in association with a fracture of the cap-
itate, the so-called scaphocapitate syndrome
(Anderson 1987). Injuries to the scapholunate
and lunatotriquetral interosseous ligaments are
quite rare in the pediatric population (Zimmerman
and Weiland 1990).
Classification
Classication of pediatric scaphoid fractures is
most commonly based on the anatomic location
of the fracture, as in the adult population: avulsion
distal pole, transverse distal pole, waist and prox-
imal pole (Fig. 3) (Anz et al. 2009). Fractures are
further classied as displaced or undisplaced.
Additionally, scaphoid fractures in children can
be classied based on the amount of ossication
present. Type I fractures are purely chondral,
T.O. Wyrick
occurring in children less than 8 years of age.
The fracture line may be purely chondral or
involve part of the ossic nucleus. These chondral
fractures require MRI for diagnosis. Type II
fractures are osteochondral in nature and occur in
children 811 years of age. Lastly, type III fractures
are osseous in nature. These are the most common
type, occurring in children 12 years of age or older.
No classication system exists specic to fractures
of the other carpal bones in the pediatric population
(Anz et al. 2009).
Treatment Options
Nonoperative Management
Indications
Nonoperative management is the most commonly
utilized method of treatment of pediatric carpal
fractures. Observed immobilization is indicated in
patients with closed nondisplaced carpal injuries
without joint subluxation or dislocation. Most
carpal fractures in skeletally immature patients
require a short period of below the elbow immo-
bilization ranging from 4 to 6 weeks. In skeletally
46 Carpal Fractures 1033

Fig. 3 (a) A nearly skeletally mature 16-year-old male an open dorsal approach with a small headless compres-
with a proximal pole fracture of the scaphoid after a fall sion screw resulting in successful union
onto an outstretched hand. (b and c) Patient was treated via

Table 1 Noperative Treatment of Pediatric Carpal fracture. The scaphoid is the most commonly
Fractures fractured bone treated operatively in the pediatric
Indications Contraindications population. Specically for scaphoid fractures in
Undisplaced, stable Displaced, unstable, or pediatric patients at or near skeletal maturity,
fracture open fracture operative treatment should be considered if frac-
No joint subluxation or Joint subluxation or ture displacement is greater than 1 mm, the frac-
dislocation dislocation ture is comminuted or involves the proximal pole,
there has been a delay in diagnosis and initial
treatment, or the intrascaphoid angle is greater
mature patients and adolescents with carpal frac- than 45 on the lateral view (Anz et al. 2009).
tures, the immobilization time needed for bony
union is more similar to that seen in adults requir- Contraindications
ing between 6 and 12 weeks. It is important to remember that the large majority
of pediatric carpal fractures are treated
Contraindications nonoperatively with simple cast immobilization.
Nonoperative treatment is contraindicated in Closed, stable, undisplaced carpal fractures with-
open, displaced, or unstable carpal fractures or in out evidence of joint instability should be treated
the setting of joint subluxation or dislocation with cast immobilization and followed with rou-
(Table 1). tine radiographs to ensure displacement does not
occur. It should be noted that late displacement in
this setting is extremely rare (Table 2).
Operative Treatment

Indications Scaphoid Fractures

Indications for operative treatment of carpal frac-
tures in the pediatric population include open
fracture, displacement, fracture instability, associ-
ated joint subluxation or dislocation, associated
neurovascular compromise, associated compart-
ment syndrome, and established nonunion of the
Scaphoid fractures are the most commonly seen
fractured bone in the immature carpus
representing 2.9 % of hand and wrist fractures
seen in children (Light 2000). The incidence of
scaphoid fractures peaks around 15 years of age in
1034
Table 2 Operative Treatment of Pediatric Carpal
Fractures
Indications Contraindications
Displaced, unstable, or open Undisplaced, stable
fracture fracture
Joint subluxation or dislocation No joint subluxation
or dislocation
Failure to heal with adequate
period of immobilization
the skeletally immature population. The mecha-
nism of injury is usually a fall onto an outstretched
hand with the wrist extended. As previously men-
tioned, initial plain radiographs may not reveal a
fracture. In clinically suspicious cases, radio-
graphs should be repeated in 23 weeks while
immobilizing the patient in the interim. The frac-
ture can become more obvious radiographically at
this time. Occasionally, MRI is used to conrm
the diagnosis.
Historically, the most commonly fractured area
in the skeletally immature scaphoid is the distal
pole which is quite different from that seen in
adults with scaphoid fractures. This is likely due
to the eccentric ossication pattern seen in child-
hood. Adolescents, however, do commonly dem-
onstrate the classic mid-waist pattern of scaphoid
fracture as seen in adults. Historically, up to 87 %
of fractures in the pediatric scaphoid are
undisplaced and involve the distal pole (Wulff
and Schmidt 1998). These fractures are amenable
to successful nonoperative treatment with 46
weeks of cast immobilization. A short-arm
thumb spica cast is usually sufcient immobiliza-
tion in this setting to result in fracture healing.
Undisplaced waist fractures in this population
may require a slightly longer immobilization
period of 78 weeks to achieve healing (Elhassen
and Shin 2006).
Waters and Bae et al. found that male sex, high-
energy mechanisms of injury, closed physes, and
high body mass index in adolescents have been
shown to be associated with more adult injury
patterns including scaphoid waist and proximal
pole fractures. Treatment of acute fractures with
appropriate immobilization resulted in a 90 %
T.O. Wyrick
union rate in their retrospective analysis of
351 pediatric and adolescent scaphoid fractures.
Interestingly, they found that the most common
location for scaphoid fractures was the waist
(71 %) followed by the distal pole (23 %) and
the proximal pole (6 %). Chronic fractures,
displaced fractures, and proximal pole fractures
that were treated with cast immobilization alone
more often resulted in nonunion. Increased time to
union was seen in patients with older fractures,
displaced fractures, proximal pole fractures, and
fractures seen in the setting of osteonecrosis.
Encouragingly, the union rate following surgical
treatment of nonunions in their series was 96.5 %.
Increased time to union was seen in association
with open physes, use of bone graft, fracture dis-
placement, proximal pole fracture, and the type of
screw used for xation at the time of surgery
(Gholson et al. 2011).
Displaced fractures of the scaphoid in children
require open reduction with internal xation either
with k-wires or a headless compression screw.
Fortunately nonunion in the pediatric population
is rare if appropriate treatment is carried out
(DArienzo 2002). Nonunion is most commonly
seen in scaphoid waist fractures in patients
between the ages of 11 and 15 years. Many cases
of nonunion can be attributed to delay in or failure
to diagnose the fracture. In fact, acute scaphoid
fractures in children and adolescents that are
treated promptly with immobilization very rarely
result in nonunion (0.8 %) (Fabre et al. 2001).
Most of the reported cases of scaphoid nonunion
occur at the waist (Wulff and Schmidt 1998; Fabre
et al. 2001; Chloros et al. 2007).
In pediatric patients who present with a
nonunion of the scaphoid and have never had
treatment with immobilization, this is the
recommended rst course of action. One can
consider the use of a bone stimulator to help
with healing.
However, in pediatric scaphoid fracture
patients who have not gone on to achieve union
despite appropriate diagnosis and immobilization,
surgical treatment is warranted. Fortunately,
results of open reduction with internal xation
and bone grafting, usually nonvascularized, are
46 Carpal Fractures
uniformly good in this population with very good
rates of healing and low complication rates
(Mintzer and Waters 1999; Waters and Stewart
2002). There have been no reports of growth
disturbance of the scaphoid with this treatment.
Injuries of the less commonly fractured carpal
bones in the immature carpus will be discussed
briey. However, the discussion of the surgical
treatment in detail will be limited to the scaphoid
as this is the most commonly encountered fracture
requiring surgical intervention in the pediatric
population.
Capitate Fractures
The capitate is the second most commonly frac-
tured bone in the immature carpus. Fractures of
the capitate are extremely rare in isolation and are
usually undisplaced in this setting. Most com-
monly these fractures are amenable to simple
immobilization as treatment (Young 1986).
Capitate fractures are more commonly seen in
association with scaphoid fractures and likely
represent an injury on the spectrum of a trans-
scaphoid, trans-capitate perilunate-type injury.
In these scenarios, displaced fractures of the
capitate are treated with concomitant open
treatment of the scaphoid with simple pinning of
the capitate fracture. It is important to remember
that the retrograde blood supply in the
capitate makes displaced fractures of the
capitate susceptible to avascular necrosis of
the proximal pole, thus necessitating prompt
treatment.
Lunate Fractures
Isolated lunate fractures are extremely rare in the
pediatric population and only a few case reports
exist. Most of the lunate injuries seen are similar
to those seen in the adult population, including
isolated lunate dislocations or perilunate disloca-
tions with or without an associated distal
radius fracture (Sharma et al. 2007). These injury
patterns should be treated with urgent
1035
closed reduction and cast immobilization with
appropriate treatment of concomitant injuries as
indicated.
Trapezium and Trapezoid Fractures
Fractures of the trapezium and trapezoid are
extremely rare in the pediatric population. The
general mechanism of injury postulated remains
a fall onto an outstretched extended wrist. Most
are undisplaced and routine immobilization is the
recommended treatment.
Hamate Fractures
Hamate fractures in the skeletally immature patient
are also extremely rare. Direct trauma to the ulnar
aspect of the hand and crush injuries can result in
this fracture. The most commonly seen fractures
are fractures involving the hook of the hamate and
those associated with carpometacarpal fracture dis-
locations of the ring and small ngers. Hamate
hook fractures should be treated with simple immo-
bilization in the pediatric population, as they rarely
go on to nonunion in these patients. Displaced and
unstable carpometacarpal fracture dislocations
should be treated with reduction and xation
(Goddard 2010).
Triquetrum Fractures
Fractures of the triquetrum are also very rarely
seen in the pediatric population. The mechanism
of injury is generally a hyperextension force to the
wrist. These fractures occur most commonly
between the ages of 11 and 13 years (Letts and
Esser 1993). The majority of triquetrum fractures
can be described as subtle avulsion-type or
impingement-type fractures. Oblique radiographs
may be required to diagnose them, and therefore,
the diagnosis is often delayed or missed.
Tenderness over the triquetral point located
dorsally and just distal to the ulna should arouse
the suspicion of this fracture to the examiner.
1036
These injuries can be associated with soft tissue
injuries to the lunatotriquetral ligament or
the triangular brocartilage complex. Cast immo-
bilization for 34 weeks is recommended with
uneventful healing generally expected (Letts and
Esser 1993).
Pisiform Fractures
Pisiform fractures of the immature carpus are
extremely rare. When seen in isolation, they are
generally undisplaced and routine immobilization
is recommended. Pisiform fractures can be seen
along with other carpal bone fractures. There are a
few case reports of pisiform dislocations, some in
association with physeal fracture of the distal
radius (Ashkan et al. 1998; Mancini et al. 2005).
Dislocation of the pisiform can generally be
treated with closed reduction followed by cast
immobilization for 34 weeks and appropriate
treatment of other associated injuries.
Surgical Procedure: Operative
Treatment of the Acute Scaphoid
Fracture
Preoperative Planning
Plain radiographs should be examined for fracture
location, the presence of comminution and the
presence of other associated injuries to the distal
radius or other carpal bones. The location of the
fracture may determine which surgical approach
is used. Distal pole fractures are more easily
approached from volarly, while proximal pole
fractures are more easily stabilized from dorsally.
Evaluation of the size and skeletal maturity
level of the patient to determine choice of implant
(k-wires, absorbable pins, headless compression
screw) should be performed using plain radio-
graphs (Table 3).
Positioning
The patient is placed supine with a radiolucent
hand table attached on the operative side.
All bony prominences should be well padded.
T.O. Wyrick
Table 3 Operative Treatment of Acute Scaphoid
Fractures - Preoperative Planning
Supine position
Radiolucent hand table
Mini or standard c-arm
Nonsterile tourniquet
Special equipment: drill, k-wires, headless compression
screw of choice, all lengths and sizes available
The hand table should be centered using the
patients shoulder as a guide. A mini c-arm or
standard sized uoroscopy unit should be used
intraoperatively. A nonsterile pneumatic tourni-
quet is applied to the upper arm and the limb is
exsanguinated prior to ination.
Surgical Approaches
Dorsal Open Approach
Listers tubercle is palpated on the dorsal aspect of
the distal radius. A transverse or longitudinal inci-
sion is made at the level of the radiocarpal joint
just distal to Listers tubercle. Careful blunt dis-
section is used to expose the extensor tendons
distal to the true extensor retinaculum specically
identifying the extensor tendons of the second,
third, and fourth compartments. The interval
between the third and fourth compartments is
developed to expose the dorsal wrist capsule. A
small release of the distal aspect of the extensor
retinaculum over the third and fourth compart-
ments will help facilitate exposure and not result
in bowstringing of the tendons. Once the dorsal
wrist capsule is exposed, a limited arthrotomy is
performed. This can be oriented longitudinally,
obliquely, or transversely. Care is taken to avoid
injury to the scapholunate ligament during the
arthrotomy as it often has attachments to the
undersurface of the wrist capsule in this area.
With exion of the wrist, the proximal pole of
the scaphoid and the scapholunate ligament are
easily visualized and accessed.
Volar Open Approach
The two externally palpable landmarks of the
distal pole of the scaphoid and the exor carpi
46 Carpal Fractures
radialis (FCR) tendon are palpated on the volar
radial surface of the distal portion of the wrist. A
zigzag-type incision is used to cross the wrist
exion crease between these two landmarks and
end proximally on the radial side of the FCR
tendon. The distal pole of the scaphoid is easily
palpable and identiable in the distal aspect of the
incision. Once the distal pole is identied, it is
exposed sharply to isolate the entry point for
xation. Extreme extension of the thumb can
aide in exposure of the distal pole and facilitate
placement of xation in the optimal center-center
position of the scaphoid. A small portion of the
proximal radial edge of the trapezium can be
removed if needed to facilitate exposure to the
distal pole of the scaphoid. The exposure can be
extended proximally if visualization of the scaph-
oid waist is required by longitudinally incising a
portion of the radioscaphocapitate ligament deep
to the FCR tendon sheath. The incised ligament
must be preserved for meticulous repair following
xation of the scaphoid fracture.
Technique
After adequate surgical exposure is obtained
using one of the above surgical approaches, ana-
tomic reduction is obtained. In the displaced frac-
ture, the extended volar and dorsal approaches
may be used to ensure the fracture line is readily
visible. Reduction is facilitated by placing k-wires
in the distal fragment and the proximal fragment
oriented from dorsal to volar which can then be
used as joysticks to reduce the fracture. From the
dorsal approach, the scaphocapitate articulation is
easily visible and can aide in obtaining the proper
reduction, particularly in fractures with signicant
comminution at the waist. A large clamp can then
be used to clamp the joystick k-wires together to
hold the reduction, while a preliminary k-wire is
passed across the fracture site. This wire can pro-
vide temporary xation and prevent rotation of the
two fragments relative to one another during
placement of a headless compression screw. If
this derotational wire is used, it should be placed
in an area of the scaphoid that will still allow
enough room for placement of the center-center
guidewire and screw later. The appropriate drill
1037
Table 4 Operative Treatment of Acute Scaphoid
Fractures - Surgical Steps
Anatomically reduce the fracture under either direct
visualization or uoroscopic imaging
K-wires may be used as joysticks to facilitate reduction
Preliminarily stabilize the fracture fragments using a
0.045 k-wire
Advance xation to stabilize fracture this may include
single headless compression screw in the center-center
position or multiple k-wires
Table 5 Operative Treatment of Acute Scaphoid
Fractures - Postoperative Protocol
Postoperative week Treatment
Zero Short-arm thumb spica cast
Week 46 Short-arm thumb spica splint
with gentle AROM, no
strengthening
Week 68, with Discontinue immobilization,
radiographic healing initiate PROM and
strengthening, return to sports
if applicable
and measuring devices are then used based on
which headless screw system is being used.
Approximately 4 mm is routinely subtracted
from the measured length to ensure that the head-
less compression screw is not too long. The prox-
imal end of the headless compression screw
should be well buried beneath the cartilage sur-
face under direct visualization. Care should be
taken during advancement of the screw to ensure
it is not too long prior to fully seating the screw.
Therefore, frequent utilization of intraoperative
uoroscopic imaging is recommended during
this portion of the case. Multiple c-arm images
should be taken to ensure the screw is the appro-
priate length and is in the optimal center-center
position on the PA and lateral views (Table 4).
Postoperative Protocol
The postoperative protocol following surgical
treatment of the acute scaphoid fracture varies
widely depending on surgeon preference, patient
demands, and signs of radiographic healing.
Table 5 below outlines the authors standard post-
operative protocol in this setting.
1038
Preferred Treatment
Nonoperative treatment is the preferred rst line
of treatment in the undisplaced scaphoid fracture.
A short- or long-arm thumb spica cast is
recommended for at least 6 weeks or until clinical
and radiographic signs of union are present. Three
months or more of cast immobilization may be
necessary to achieve union. This treatment has
shown union rates of approximately 95 % regard-
less of fracture location (Gholson et al. 2011).
Nonoperative treatment for the acute displaced
scaphoid fracture is less successful with the best
union rate seen in fractures of the distal pole
(87 %). Union rates after nonoperative treatment
of acute displaced waist and proximal pole frac-
tures are less (22 % and 28 %, respectively)
(Gholson et al. 2011).
Fractures presenting for treatment greater than
6 weeks after the initial injury are referred to as
chronic fractures. Successful union following
nonoperative treatment of chronic nondisplaced
distal pole fractures approaches 95 %. However,
cast immobilization for chronic nondisplaced
waist and proximal pole fractures is less success-
ful with union rates of 43 % and 50 %, respec-
tively (Gholson et al. 2011).
Finally, the lowest success rates following
nonoperative treatment are seen in chronic
displaced fractures of the scaphoid. In this
setting, union rates for distal pole fractures are
30 %, while union rates for fractures of the
waist and proximal pole are only 2 % (Gholson
et al. 2011).
This leads to the development of a treatment
algorithm advocating cast immobilization for
acute nondisplaced fractures regardless of the
location, acute displaced fractures of the distal
pole, and chronic nondisplaced fractures of the
distal pole. Surgical treatment can be offered to
patients and families who present with chronic
nondisplaced fractures of the scaphoid waist and
proximal pole. Surgical treatment should be con-
sidered the primary treatment alternative for acute
and chronic displaced fractures of the waist and
proximal pole (Gholson et al. 2011).
Displaced scaphoid proximal pole and waist
fractures are treated using a limited dorsal
approach as described above with a headless
T.O. Wyrick
compression screw. The starting point for the
screw is found at the scaphoid insertion of the
scapholunate ligament with the wrist maximally
exed. The surgeons index nger can be placed
on the distal pole of the scaphoid on the volar
surface of the wrist as a point of reference for
passing the guidewire in the appropriate position.
In addition, the guidewire is aimed toward the
thumb metacarpal with regard to the radial-ulnar
orientation. The size of the screw is determined
preoperatively and intraoperatively based on the
size of the fracture fragment and the size of the
bone. Most headless compression screw systems
have micro, mini, and standard sizes. The micro-
size screw is usually used for younger pediatric
patients and for small proximal pole fracture frag-
ments in larger patients. The mini-size screw is
generally used for waist fractures in adolescent
patients. In the case of a very small proximal
pole fragment, k-wires are utilized. Care is taken
to avoid distal dissection to prevent iatrogenic
disruption of the retrograde blood supply to the
scaphoid and the development of subsequent
avascular necrosis.
Displaced distal pole scaphoid fractures are
addressed utilizing the volar approach as
described above ideally using a headless compres-
sion screw in older children and k-wires for very
small fracture fragments or in young children.
Waist fractures can also be addressed via a volar
approach (Fig. 4). It is slightly more difcult to get
the optimal center-center position for screw place-
ment from the volar approach in the authors
experience.
Surgical Pitfalls and Prevention
Damage to the retrograde vascular supply of the
scaphoid from the dorsal approach can lead to
avascular necrosis, a devastating complication.
Care should be taken when approaching the
scaphoid from the dorsal aspect to avoid dissec-
tion distal to the waist.
In addition, injury to the scapholunate ligament
should also be avoided from the dorsal approach.
It is most at risk during the dorsal capsulotomy.
Failure to achieve the center-center position
with xation can result in delayed union or
persistent nonunion and xation failure (Table 6).
46 Carpal Fractures 1039

Fig. 4 (a and b) A 10-year-

old male fell onto an
outstretched hand while
riding a dirt bike sustaining
a displaced scaphoid waist
fracture. (c and d) The
patient was treated with
open reduction via a volar
approach and placement of
a small headless
compression screw with
successful healing

Treatment-Specific Outcomes population, acute nondisplaced fractures had

It is important to have knowledge of the union
rates specic to timing of presentation and initia-
tion of treatment, fracture displacement, and frac-
ture location when treating scaphoid fractures.
Acute nondisplaced scaphoid fractures that are
treated with prompt immobilization in the pediat-
ric and adolescent population will usually go on to
heal uneventfully with a nonunion rate of 0.84 %
(Fabre et al. 2001; Gholson et al. 2011). In the
largest recent series looking specically at scaph-
oid fractures in the adolescent and pediatric
very high union rates with cast immobilization.
In this series, the authors studied 312 scaphoid
fractures, 222 acute (less than 6 weeks old) and
90 chronic (greater than 6 weeks old). Of the
222 acute fractures, 201 were casted initially and
of those 181 healed with the remaining 20 patients
requiring surgical treatment for persistent non-
union. Of the 90 chronic fractures, 77 were casted
initially with 59 of these patients failing to achieve
union and requiring surgical treatment. Increased
time to union was seen in patients with chronic
1040
Table 6 Operative Treatment of Acute Scaphoid
Fractures - Pitfalls and Prevention
Potential pitfall Pearls for prevention
Avascular necrosis of Do not dissect distal to
scaphoid scaphoid waist in dorsal
approach
Scapholunate ligament Perform dorsal capsulotomy
injury carefully, incising each layer
meticulously and slowly
Failure to achieve Obtain multiple still and live
center-center screw uoroscopic images in PA,
position PA in ulnar deviation, lateral
and oblique views
intraoperatively until optimal
guidewire position is
obtained
fractures, fractures with osteonecrosis, displaced
fractures, and proximally located fractures. Over-
all, lower union rates were seen in patients with
proximally located fractures, chronic fractures,
and displaced fractures in those patients treated
with cast immobilization alone (Gholson
et al. 2011).
Acute nondisplaced fractures of the distal pole
demonstrated a 99 % union rate. Acute
nondisplaced fractures of the scaphoid waist dem-
onstrated at 92 % union rate. Acute nondisplaced
fractures of the proximal pole demonstrated union
in 95 % of patients treated with cast immobiliza-
tion (Gholson et al. 2011).
When looking at acute displaced fractures, the
union rates in this large series were lower. Acute
displaced fractures of the distal pole reached
union with cast immobilization in 87 % of
patients. Acute displaced fractures of the waist
treated nonoperatively reached union in only
22 % of cases. Acute displaced fractures of the
proximal pole treated nonoperatively reached
radiographic union in 28 % (Gholson et al. 2011).
Chronic fractures in this same series were
dened as those presenting for treatment greater
than 6 weeks after the initial injury. The union
rates following nonoperative treatment of chronic
nondisplaced fractures of the distal pole, waist,
and proximal pole were 95 %, 43 %, and 50 %,
respectively (Gholson et al. 2011).
Chronic displaced fractures had by far the low-
est rates of union following cast immobilization as
T.O. Wyrick
expected. The union rates following cast
immobilization of chronic displaced fractures of
the distal pole, waist, and proximal pole were
30 %, 2 %, and 2 %, respectively (Gholson
et al. 2011).
Following surgical treatment of scaphoid frac-
tures, in this same large series, the union rate was
found to be 96.5 % with the union rate in the acute
fractures being 97.6 %. The patients with acute
fractures treated surgically received open reduc-
tion with headless screw xation via either a dor-
sal percutaneous approach if the fracture was
undisplaced or a volar open approach if the frac-
ture was displaced. Operative treatment for
chronic fractures included iliac crest structural
graft in the setting of a viable proximal pole and
a signicant humpback deformity or
vascularized bone graft from the distal radius if
osteonecrosis of the proximal pole was seen.
Three different types of headless compression
screws were utilized in these patients. In the
surgical cohort, proximal pole fractures took sig-
nicantly longer to heal than fractures of the waist
and distal pole. Chronic fractures healed slower in
the surgical cohort as well as seen by those
patients requiring bone graft taking ve additional
weeks to heal compared to those patients not
requiring bone graft. Fractures treated surgically
in patients with open physes required three
additional weeks to heal compared to those with
closed physes (Gholson et al. 2011).
Management of Complications
Persistent nonunion in the setting of appropriate
treatment is rare. Most studies report union rates
in the surgical treatment of nonunions in the
pediatric and adolescent populations near 100 %
(Fabre et al. 2001; Mintzer and Waters 1999).
Regarding operative treatment of scaphoid
nonunions, in the large series by Bae and
Waters and associates, the union rate following
surgery was 96.5 % (109 of 113) (Gholson
et al. 2011).
Continued observation and the consideration of
an external bone stimulator as treatment for non-
unions failing to heal radiographically within
6 months after surgical treatment are warranted in
this population. It should be noted that the safety
46 Carpal Fractures
Table 7 Operative Treatment of Scaphoid Nonunions -
Preoperative Planning
Supine position
Radiolucent hand table
Mini or standard c-arm
Nonsterile tourniquet
Special equipment: drill, saw with small saw blade
options, osteotomes, k-wires, headless compression
screw of choice, all lengths and sizes available, small
round burr
and effectiveness of bone stimulators in children is
not known, and some feel that these should not be
used in skeletally immature patients. Rarely,
revision with vascularized bone grafting is needed.
Surgical Procedure: Operative
Treatment of the Scaphoid Fracture
Nonunion
Preoperative Planning
Planning for treatment of a scaphoid nonunion
requires assessment of plain radiographs as well
as a CT scan to evaluate for cyst formation at the
fracture site, humpback deformity in the scaph-
oid, and subtle signs of osteophytes and/or
arthritic changes (Table 7). An MRI with contrast
is considered in the preoperative planning stage to
evaluate for avascular necrosis of the proximal
fragment. However, the results of healing in
surgical treatment of pediatric and adolescent
scaphoid nonunions with nonvascularized bone
graft approach 100 % in most studies
(Fabre et al. 2001; Mintzer and Waters 1999).
Therefore, vascularized bone graft is usually not
needed to achieve union, thus obviating the
need for MRI.
Positioning
The patient is placed in the supine position with a
radiolucent hand table. Rarely, iliac crest bone
grafting is indicated in the pediatric population
for scaphoid nonunion treatment as studies
have shown good results with distal radius bone
grafting in the setting where bone graft is
needed (Anz et al. 2009; Chloros et al. 2007;
Fabre et al. 2001; Henderson and Letts 2003).
1041
Therefore, there is generally no need to prepare
the iliac crest in the pediatric population.
Surgical Approaches
Dorsal Open Approach
Listers tubercle is palpated on the dorsal aspect of
the distal radius. A transverse or longitudinal inci-
sion is made at the level of the radiocarpal joint
just distal to Listers tubercle. Careful blunt dis-
section is used to expose the extensor tendons
distal to the true extensor retinaculum specically
identifying the extensor tendons of the second,
third, and fourth compartments. The interval
between the third and fourth compartments is
developed to expose the dorsal wrist capsule. A
small release of the distal aspect of the extensor
retinaculum over the third and fourth compart-
ments will help facilitate exposure and not result
in bowstringing of the tendons. Once the dorsal
wrist capsule is exposed, a limited arthrotomy is
performed. This can be oriented longitudinally,
obliquely, or transversely. Care is taken to avoid
injury to the scapholunate ligament during the
arthrotomy as it often has attachments to the
undersurface of the wrist capsule in this area.
With exion of the wrist, the proximal pole of
the scaphoid and the scapholunate ligament are
easily visualized and accessed.
Volar Open Approach
The two externally palpable landmarks of the
distal pole of the scaphoid and the exor carpi
radialis (FCR) tendon are palpated on the volar
radial surface of the distal portion of the wrist. A
zigzag-type incision is used to cross the wrist
exion crease between these two landmarks and
end proximally on the radial side of the FCR
tendon. The distal pole of the scaphoid is easily
palpable and identiable in the distal aspect of the
incision. Once the distal pole is identied, it is
exposed sharply to isolate the entry point for
xation. Extreme extension of the thumb can
aide in exposure of the distal pole and facilitate
placement of xation in the optimal center-center
position of the scaphoid. A small portion of the
proximal radial edge of the trapezium can be
removed if needed to facilitate exposure to the
1042
distal pole of the scaphoid. The exposure can be
extended proximally if visualization of the scaph-
oid waist is required by longitudinally incising a
portion of the radioscaphocapitate ligament deep
to the FCR tendon sheath. The incised ligament
must be preserved for meticulous repair following
xation of the scaphoid fracture.
Either the dorsal or the volar approach can be
extended proximally to obtain bone graft from the
distal radius.
Dorsal Approach Extension
By extending the skin incision proximally, the
distal radius can be easily accessed for obtaining
bone graft. Listers tubercle is easily palpated
proximal to the physis of the distal radius. An
incision is made in the periosteum covering
Listers tubercle, and the tubercle is sharply
exposed while protecting the adjacent extensor
tendons. A rongeur is used to remove Listers
tubercle and access the metaphyseal bone from
the distal radius. Using uoroscopic guidance
intraoperatively, it is imperative to ensure that
iatrogenic injury to the physis is avoided. A
corticotomy is then made in the distal radius
metaphysis and cancellous bone is removed for
graft utilizing curettes.
Volar Approach Extension
The skin incision is extended proximally along the
radial aspect of the FCR tendon sheath. Subse-
quently, the deeper aspect of the tissues is
accessed either by careful dissection between the
FCR and the radial artery or by opening the super-
cial and deep layers of the tendon sheath of the
FCR sharply. Once the FCR is mobilized, it is
retracted ulnarly and the radial artery is retracted
radially to expose the pronator quadratus muscle.
The pronator muscle is then incised along its
radial insertion into the radius, while ensuring
that the dissection stays proximal to the physis.
A small cortical window can be made to obtain
graft from the metaphyseal bone of the distal
radius if needed. If corticocancellous graft is nec-
essary to provide more structural support for cor-
rection of a humpback deformity, it can be
harvested from the same location using either
sharp osteotomes or an oscillating saw.
T.O. Wyrick
Alternatively, a larger corticocancellous graft of
volar and radial cortical bone with accompanying
cancellous bone can be harvested from the radial
most aspect of the distal radius. Rarely, some
surgeons will approach and utilize the iliac crest
for structural bone graft.
Technique
After adequate surgical exposure is obtained
using one of the above surgical approaches,
anatomic reduction is obtained. With the extended
volar and dorsal approaches, the fracture line is
readily visible. Reduction is facilitated by placing
k-wires in the distal fragment and the proximal
fragment oriented from dorsal to volar which can
then be used as joysticks to deliver the fracture
fragments for preparation. Utilize either a curette,
rongeur, burr, or a saw to freshen and atten the
nonunion site proximally and distally as needed.
Radiographic evaluation intraoperatively is criti-
cal in evaluating the reduction in nonunions of the
scaphoid, particularly in the setting of a exion or
humpback deformity of the scaphoid and/or if
extension of the lunate exists. A true lateral radio-
graphic image intraoperatively is necessary to
correct these patterns. In general, when the exion
deformity of the scaphoid has been adequately
corrected, the lunate will no longer be extended
and will be neutrally aligned over the radius.
When this has been achieved, it can be useful to
place a temporary pin from the distal radius into
the lunate to hold this position during the remain-
der of the procedure, a so-called radiolunate pin.
A large clamp can then be used to clamp the
joystick k-wires together to hold the reduction,
while a preliminary k-wire is passed across the
fracture site. This wire can provide temporary
xation and prevent rotation of the two fragments
relative to one another during placement of a
headless compression screw. If this derotational
wire is used, it should be placed in an area of the
scaphoid that will still allow enough room for
placement of the center-center guidewire and
screw later. A trimmed ruler is then used to mea-
sure and assess the amount of bone graft needed.
The bone graft is then harvested as described
above in the surgical approach section based on
the size needed, the approach used, and the
46 Carpal Fractures
Table 8 Operative Treatment of Scaphoid Nonunions -
Surgical Steps
Place k-wires to use as joystick to deliver and expose the
proximal and distal fragments
Utilize either a curette, rongeur, or a saw to freshen and
atten the nonunion site proximally and distally if needed
Correct humpback deformity
Consider temporary radiolunate pin to correct extension
deformity of the lunate if present
Preliminarily stabilize the construct with k-wires
Obtain center-center position with guidewire for headless
compression screw
Advance drill over guidewire
Bone graft options
If signicant humpback deformity is present, consider
corticocancellous autograft from volar distal radius
If nonunion present for less than 1 year and minimal
cystic changes at nonunion site and no humpback
deformity exists, consider placing cancellous autograft
through drill hole only just prior to passing screw
If signicant cystic changes at nonunion site, pack
cancellous autograft tightly at nonunion site and
throughout drill hole prior to screw placement
preference of the surgeon. The bone graft is then
placed in the defect present at the scaphoid non-
union site. The appropriate drill and measuring
devices are then used based on which headless
screw system is being used (Table 8). Approxi-
mately 4 mm is routinely subtracted from the
measured length to ensure that the headless com-
pression screw is not too long. Cancellous bone
graft can be packed into the drill hole as well prior
to placement of the screw. The proximal end of the
headless compression screw should be well buried
beneath the cartilage surface. Care should be
taken during advancement of the screw to ensure
it is not too long prior to fully seating the screw.
Therefore, frequent utilization of intraoperative
uoroscopic imaging is recommended during
this portion of the case. Multiple c-arm images
should be taken to ensure the screw is the appro-
priate length and is in the optimal center-center
position on the PA and lateral views (Fig. 5).
Postoperative Protocol
The postoperative protocol following surgical
treatment of the scaphoid nonunion varies widely
depending on surgeon preference, patient
demands, and signs of radiographic healing.
1043
Table 9 below outlines the authors standard post-
operative protocol in this setting.
Authors Preferred Treatment
A dorsal approach is utilized for any nonunion
without signicant humpback deformity, whereas
a volar approach is preferred if signicant hump-
back deformity is present.
A nonvascularized bone graft is generally used
in children and adolescents with union rates near
100 % with this technique in this population
(Fabre et al. 2001; Mintzer and Waters 1999).
This is in contrast to the treatment algorithm for
adults. A preoperative MRI is not obtained in the
pediatric and adolescent population. However,
careful evaluation of the blood supply to
the scaphoid intraoperatively and assessment
for punctate bleeding from the proximal pole in
performed. If no bleeding from the proximal
pole of the scaphoid is evident, the patient is
skeletally mature, and the nonunion has been
present for greater than 1 year, strong consider-
ation is given to using a vascularized bone graft.
In this setting preoperatively, a dorsal approach
is planned with preservation of the ability to
harvest a vascularized distal radius bone graft
if desired.
If there is minimal cystic change at the non-
union site, the nonunion site is found to have some
signicant brous stability at the time of surgery,
and/or the nonunion has been present for 1 year or
less, cancellous bone graft from the dorsal distal
radius is utilized and simply placed through the
drill hole made for the headless compression
screw.
However, if there is signicant resorption and
cystic change at the nonunion site, complete insta-
bility at the nonunion site and/or the nonunion has
been present for more than 1 year, the nonunion
site is formally taken down and freshened with a
saw. Both the nonunion site and the drill hole are
packed with cancellous graft from the dorsal distal
radius.
In the scenario of a signicant humpback
deformity, a volar approach is utilized and a
bicortical corticocancellous graft from the distal
radius is utilized to give further structural support
to the construct.
1044 T.O. Wyrick

Fig. 5 (ad) A 15-year-old male injured his wrist playing
football and was treated for a scaphoid waist fracture
acutely with open reduction via a volar approach and
headless screw xation. The lateral radiograph (b) and
CT (d) demonstrate less than optimal screw placement.
The patient failed to achieve union and had signicant
wrist pain 1 year after surgery. (e) and (f) The patient
underwent revision surgery via an extended dorsal
approach after removal of the screw with placement of
nonvascularized bone graft from the distal radius and a
headless compression screw in a more optimal position
resulting in pain resolution and bony union

Table 9 Operative Treatment of Scaphoid Nonunions - Surgical Pitfalls and Prevention

Postoperative Protocol Complications which can occur after surgical
Postoperative week Treatment treatment of scaphoid fractures and nonunions
Zero Short-arm thumb spica cast are highlighted in Table 11 below. During
Week 6 Removable thumb spica the dorsal surgical approach, dissection should
splint, gentle AROM be limited to the area proximal to the scaphoid
Week 12, radiographic Discontinue immobilization, waist to avoid iatrogenic injury to the retrograde
healing present initiate strengthening, PROM
blood supply of the scaphoid. If this is not done,
46 Carpal Fractures 1045

Table 10 Operative Treatment of Scaphoid Fractures -
Complications and Management
Common
complications Management
Nonunion Consider external bone
stimulator, revision with
vascularized bone grafting from
distal radius, persistent nonunion
rare with appropriate treatment
Loss of mobility Static progressive or dynamic
splinting to gain needed range of
motion, surgical treatment rarely
needed in this population
Osteonecrosis of the Vascularized bone grafting from
proximal pole the distal radius
Persistent push- Symptomatic treatment, activity
off pain avoidance
avascular necrosis of the proximal pole can
result. Care should also be taken during the
capsulotomy via the dorsal approach to avoid
iatrogenic injury to the scapholunate ligament
as it is often intimately attached to the undersur-
face of the dorsal capsule and subsequent
scapholunate instability can occur. Failure to
achieve the optimal center-center screw position
regardless of the approach can increase the
chances of fracture nonunion. Multiple still
and live uoroscopic images intraoperatively
should be obtained to ensure the guidewire
position is optimal prior to placement of the
screw. Lastly, in the case of signicant DISI
deformity in scaphoid nonunions, failure to cor-
rect the humpback deformity of the scaphoid and
the extension deformity of the lunate will lead to
malunion, persistent and progressive carpal insta-
bility patterns over time, and loss of wrist motion
(Table 11).
There are very few reports of osteonecrosis of
the scaphoid in the pediatric and adolescent
populations. Waters and Stewart reported a small
series of three adolescent male patients who
developed avascular necrosis of the
proximal pole and were successfully treated with
vascularized bone grafting (Waters and Stewart
2002). Larson, Light, and Ogden demonstrated
a single case of avascular necrosis in a 5-year-old
Table 11 Operative Treatment of Scaphoid Fractures -
Pitfalls and Prevention
Potential pitfall Pearls for prevention
Avascular necrosis of the Do not dissect distal to waist
proximal pole in dorsal approach
Injury to scapholunate Perform dorsal capsulotomy
ligament carefully, incising each layer
carefully and slowly
Failure to achieve Obtain multiple still and live
center-center screw uoroscopic images in PA,
position PA in ulnar deviation, lateral
and oblique views
intraoperatively until
optimal guidewire position
is obtained
Failure to fully correct Place temporary radiolunate
DISI deformity, pin with lunate in optimal
humpback deformity position neutrally aligned
over the distal radius on the
lateral image
boy following nonoperative treatment of a
scaphoid waist fracture (Larson et al. 1987).
Persistent loss of mobility and pain in the radial
side of the wrist with extension and axial loading of
the wrist are not uncommon complaints in adult
patients with scaphoid fractures that have gone on
to reach union successfully. Fortunately, in most
cases, pediatric and adolescent patients tend to
regain close to normal range of motion even after
surgical management and extended immobilization
in the case of scaphoid fractures and nonunions
(Anz et al. 2009; Henderson and Letts 2003).
Avoidance of push-ups and other offending activ-
ities as well as the use of supportive wrist tape or
splinting can help with these symptoms (Table 10).
Summary
Fractures of the pediatric carpus are relatively
uncommon. A relatively high index of suspicion
should exist on the part of the examining physician
in the setting of clinical evidence of a fracture and
negative radiographs owing to the large portion of
carpal bones that are unossied during skeletal
immaturity and the tendency for undisplaced
fractures to be difcult to visualize initially.
1046
Most fractures are undisplaced and therefore tend
to heal uneventfully with a relatively short period
of immobilization. Displaced fractures may require
open reduction and internal xation utilizing
k-wires or a headless compression screw. Long-
term complications are relatively rare. Scaphoid
fractures can progress to nonunion, occurring
most commonly in the setting of missed or delayed
diagnosis or failure to initiate or comply with
prompt and appropriate treatment. Most agree that
scaphoid nonunions requiring operative treatment
can be successfully treated with open reduction and
nonvascularized bone grafting from the distal
radius with few complications and union rates
approaching 100 %.
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 Distal Radius Fractures
47
Ahmed Bazzi, Brett Shannon, and Paul Sponseller

Contents Nonoperative Management of Fractures of

the Distal Radius . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1054
Introduction to Fractures of the Distal Discussion: Nonoperative Management of
Radius . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1048 Physeal Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1055
Pathoanatomy and Applied Anatomy Relating Technique: Closed Reduction
to Fractures of the Distal Radius . . . . . . . . . . . . . . . . . 1048 of Physeal Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1055
Discussion: Nonoperative Management of
Assessment of Fractures of the Distal Torus Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1055
Radius . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1050 Discussion: Nonoperative Management of
Signs and Symptoms of Fractures Greenstick Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1057
of the Distal Radius . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1050 Technique: Closed Reduction
Fractures of the Distal Radius Imaging and Other of Greenstick Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1059
Diagnostic Studies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1051 Discussion: Management of Complete
Injuries Associated with Fractures Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1059
of the Distal Radius . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1051 Technique: Closed Reduction
Fractures of the Distal Radius Classication . . . . . . . 1052 of Complete Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1060
Fractures of the Distal Radius Outcome Tools . . . . . 1053 Technique: Splint Immobilization . . . . . . . . . . . . . . . . . . 1061
Technique: Cast Immobilization . . . . . . . . . . . . . . . . . . . 1061
Fractures of the Distal Radius
Treatment Options . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1053 Operative Treatment of Fractures of the Distal
Radius . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1062
Indications/Contraindications . . . . . . . . . . . . . . . . . . . . . . 1062
Surgical Procedure . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1062
Electronic supplementary material: The online version
of this chapter (doi:10.1007/978-1-4614-8515-5_48) Open Reduction of Irreducible Fractures . . . . . . . 1063
contains supplementary material, which is available Treatment-Specic Outcomes of Percutaneous
to authorized users. Videos can also be accessed at Pinning of Distal Radius Fractures . . . . . . . . . . . . . . 1063
http://www.springerimages.com/videos/978-1-4614-8513-1. Preferred Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1065
A. Bazzi (*) Summary and Future Directions . . . . . . . . . . . . . . . . . 1065
Pediatric Orthopedic Surgery, Childrens Hospital of
Michigan, Detroit, MI, USA References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1066
e-mail: abazzi4@dmc.org; ahmedb521@hotmail.com
B. Shannon
Johns Hopkins University School of Medicine, Baltimore,
MD, USA
e-mail: brettshannon@jhmi.edu
P. Sponseller
Kennedy Krieger Institute, Orthopedic Surgery,
Johns Hopkins Hospital, Baltimore, MD, USA
e-mail: psponse@jhmi.edu

# Springer Science+Business Media New York 2015 1047

J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_48
1048 A. Bazzi et al.

et al. 2012; Randsborg et al. 2013; Ward and

Abstract
Rihn 2006). The majority of distal radius fractures
Distal radius fractures are the most common frac-
in children occur as a result of falls, either during
tures in the pediatric population, with an incidence
sports activities or play, with boys sustaining frac-
of 2131 % of fractures. They commonly occur as
tures twice as often as girls (Ryan et al. 2010). The
a result of a traumatic fall, more commonly in
incidence peaks around the ages of 811 years in
males than females, and their prevalence is on the
girls and 1114 years in boys (Khosla et al. 2003),
rise. The pediatric wrist fracture has excellent
coinciding with a dissociation between skeletal
remodeling potential, as the distal radius physis
expansion and skeletal mineralization that results
contributes approximately 80 % of the longit-
in a period of relative bone weakness (Faulkner
udinal growth of the forearm. The remodeling
et al. 2006). Lower bone mineral density in chil-
potential is especially great in the younger patient
dren and later menarche in girls has been shown to
with more than 2 years of growth remaining.
correlate with an increased fracture risk
Clinical examination and radiographic evalua-
(Chevalley et al. 2011, 2012).
tion of the affected limb will reveal the fracture in
The incidence of distal radius fractures has
question. Distal radius fractures are commonly
increased over the past 40 years (Khosla
associated with ulnar fractures, either at the same
et al. 2003; de Putter et al. 2011). This rise may
level or at the ulnar styloid. One must assess the
be attributable to an increase in sports activities or
joints above and below to rule out a concomitant
better access to care and detection (de Putter
injury. The soft tissues may reveal signs of an
et al. 2011; Mathison and Agrawal 2010). The
open fracture, compartment syndrome, or vascu-
rising prevalence of childhood obesity may also
lar compromise. Growth arrest with displaced
contribute, as high adiposity is associated with
physeal injuries of the distal radius occurs in
increased fracture risk (Goulding et al. 2001;
45 % of cases, while an ulnar physeal injury
Ducher et al. 2009). Studies have found no differ-
can be present in up to 50 % of fractures involving
ence in fracture rates between urban and rural
the distal ulnar physis. It is imperative not to miss
areas or different ethnicities (Nellans et al. 2012;
associated dislocations, including Galeazzi or
Khosla et al. 2003).
Monteggia fracture dislocations.
Most pediatric distal radius fractures are
Treatment options include nonoperative
treated with closed reduction and immobilization
immobilization, closed reduction and percutane-
and have an excellent outcome. This chapter dis-
ous pinning, and open reduction internal xa-
cusses nonoperative and operative management
tion. Most non-displaced fractures, Salter-Harris
of various fracture patterns as well as potential
I and II, greenstick, buckle, complete or plasti-
complications.
cally deformed fractures, are amenable to rst-
line nonoperative treatment. Surgical treatment
is reserved for open fractures, irreducible frac-
Pathoanatomy and Applied Anatomy
tures, fractures with associated neurovascular
Relating to Fractures of the Distal
compromise, presence of excessive swelling,
Radius
displaced intra-articular fractures, concomitant
elbow fractures, polytrauma, fractures that had
Understanding of the functional anatomy and nor-
loss of their initial reduction, and displaced frac-
mal growth patterns of the forearm may assist in
tures in children nearing skeletal maturity.
the diagnosis and treatment of distal radius frac-
tures. While the ulna is a nearly straight bone, the
Introduction to Fractures of the Distal radial shaft has a lateral bow. During pronation
Radius and supination, this bow allows the radius to
rotate around the relatively stationary ulna. The
The distal radius is the most common site of radial (sigmoid) notch of the proximal ulna and
fracture in childhood, comprising approximately the ulnar (sigmoid) notch of the distal radius facil-
2131 % of all pediatric fractures (Nellans itate this rotation, stabilized proximally by the
47 Distal Radius Fractures
annular ligament and distally by the triangular
brocartilage complex (TFCC). The diaphyses
are additionally stabilized by the interosseous
membrane, of which the majority of bers are
oriented obliquely such that they travel distally
from the radius to the ulna and tighten during
pronation. This ligamentous complex normally
allows for up to 155165 of forearm rotation:
7580 of pronation achieved by the pronator
teres and pronator quadratus and 8085 of supi-
nation achieved by the biceps and supinator. The
biceps and supinator insert on the proximal radius;
the pronator teres inserts near the midshaft of the
radius; and the pronator quadratus inserts on the
distal third of the radius. Accordingly, in complete
distal radius fractures, the proximal fragment is
held in neutral position or supination, and the
distal fragment is typically pulled into pronation
by the unopposed action of the pronator quadratus
(Noonan and Price 1998).
In addition to the distal radioulnar joint
(DRUJ), the articular surface of the distal radius
is formed by two concavities, the scaphoid and
lunate fossae, separated by the scapholunate
ridge. The radiocarpal and ulnocarpal joints are
stabilized by the extrinsic ligaments of the wrist,
of which the volar ligaments are stronger than the
dorsal ligaments (Waters and Bae 2010). The pri-
mary volar stabilizers of the radiocarpal joint are
the radioscaphocapitate (radial collateral) and the
long and short radiolunate ligaments; the
radiolunotriquetral (dorsal radiocarpal) ligament
is the main dorsal stabilizer. The ulnocarpal joint
is stabilized volarly by the ulnocapitate,
ulnolunate, and ulnotriquetral ligaments, which
originate from the TFCC. Additionally, the exten-
sor carpi ulnaris (ECU) tendon sheath provides
ulnar collateral support. The ligaments of the wrist
normally permit 80 of exion, 75 of extension,
1525 of radial deviation, and 3045 of ulnar
deviation (Thompson 2010).
Ulnocarpal joint axial loads are transmitted
across the triangular brocartilage complex
(TFCC), which also stabilizes the DRUJ. The
TFCC originates at the ulnar notch of the radius
and inserts at the base of the ulnar styloid. It
includes the avascular central triangular
brocartilage disc and its bordering dorsal and
volar radioulnar ligaments, as well as the ECU
1049
tendon sheath and the meniscal homologue,
which originates at the dorsal radius, arcs to the
ulnar styloid, and inserts on the volar triquetrum.
The dorsal and volar radioulnar ligaments tighten
in pronation and supination, respectively. Some
also consider the ulnolunate and ulnotriquetral
ligaments to be part of the TFCC (Bae and Waters
2006).
Familiarity with epiphyseal ossication pat-
terns may enable detection of subtle physeal inju-
ries and recognition of normal development. The
distal radial epiphysis is normally sufciently
ossied to be seen on plain radiographs between
the ages of 521 months in girls and 627 months
in boys. Rarely, a separate radial styloid ossica-
tion center is present. The epiphysis progresses
from a transverse appearance to a triangular mor-
phology as the styloid lengthens. At skeletal
maturity, there is an average of 22 of radial incli-
nation, which is the angle on a posteroanterior
radiograph between the distal articular surface of
the radius and a line perpendicular to the radial
shaft. Also, throughout growth there is typically
11 of palmar tilt, the angle measured on a lateral
radiograph between the distal radial articular sur-
face and the line perpendicular to the radial shaft
(Fig. 1) (Waters and Bae 2010). The distal ulnar
epiphysis is apparent at approximately age 67
years; two distinct secondary ossication centers
are often observed. The ulnar styloid projects
from the posteromedial aspect of the epiphysis.
It is seen during the adolescent growth spurt and
elongates until physeal closure (Bae and Waters
2006).
The developmental variation in epiphyseal
morphology precludes accurate direct radio-
graphic measurement of the distal radioulnar
length relationship, termed the ulnar variance,
maintenance of which is important for force trans-
mission across the wrist. The radiocarpal joint and
ulnocarpal joint bear approximately 80 % and
20 %, respectively, of the axial load in a normal
wrist, and changes in ulnar variance alter this
load-bearing pattern (Waters and Bae 2010). It is
known that even small changes in ulnar variance
can cause alterations in TFCC axial loads of sig-
nicant magnitude (Bae and Waters 2006). In
skeletally mature patients, the articular surfaces
of the radius and ulna at the distal radioulnar joint
1050
Fig. 1 (a) PA radiograph
measuring radial inclination
and (b) Lateral radiograph
measuring palmar tilt
are compared on a posteroanterior (PA)
radiograph. By convention, if the ulna projects
distal to the radius, there is positive ulnar variance;
if the radius projects distally, there is negative ulnar
variance; and if the two extend equally, there is
neutral ulnar variance. In skeletally immature
patients, the radial and ulnar metaphyses are com-
pared rather than the articular surfaces (Hafner
et al. 1989). This indirect method reduces inaccu-
racies related to epiphyseal morphology.
Remodeling potential after fracture is directly
related to the remaining growth potential. Growth
at the distal radial and ulnar physes constitutes
approximately 7580 % of longitudinal growth
of the forearm. Ulnar physeal closure occurs on
average at age 16 years in girls and 17 years in
boys. Radial physeal closure typically follows
6 months later (Waters and Bae 2010). Thus,
childhood distal forearm fractures have excellent
remodeling potential. This potential is enhanced
by elevation of the periosteum, which is thicker
and more osteogenic in children than it is in adults
(Noonan and Price 1998). Moreover, deformities
in the plane of adjacent joint motion have better
remodeling compared to other deformities. With
continued growth, as much as 10 per year of
dorsal-volar angulation may remodel. Hence,
20 of dorsal-volar angulation in patients with at
least 2 years of remaining growth has been the
traditional standard of acceptable reduction (Bae
and Waters 2006).
A. Bazzi et al.
Assessment of Fractures of the Distal
Radius
Signs and Symptoms of Fractures
of the Distal Radius
Patients typically present after a fall onto an
outstretched hand with wrist pain, tenderness
over the fracture site, swelling, and limited motion
of the forearm and wrist. Deformity may be pre-
sent and indicates displacement, angulation, or
dislocation. In one series, a 20 % or more
decrease in grip strength compared to the
uninjured side was predictive of fracture
(Pershad et al. 2000). Examination should be
performed not only of the wrist but also of the
entire upper extremity to detect any associated
injuries, and the affected and contralateral
extremities should be compared. The skin and
soft tissues should be inspected and palpated to
assess for the possibility of an open fracture, com-
partment syndrome, or vascular compromise.
Careful neurologic examination should be
performed to identify median, ulnar, or posterior
interosseous neuropathies, which if present usu-
ally resolve within 23 weeks. In patients under
the age of 3 years, the possibility of
non-accidental injury must be considered
(Noonan and Price 1998; Waters and Bae 2010;
Bae and Waters 2006).
47 Distal Radius Fractures
Fractures of the Distal Radius Imaging
and Other Diagnostic Studies
Posteroanterior (PA) and lateral radiographs of the
forearm should be obtained in cases of a suspected
distal forearm or wrist fracture. The upper extrem-
ity should be positioned such that the radiograph will
be obtained perpendicular to the distal humerus.
Comparison lms of the contralateral forearm may
assist to distinguish subtle physeal injuries; these
should be acquired with the forearm in the same
rotational position. Dedicated views of the wrist and
elbow are helpful to assess for associated injuries such
as dislocations of the proximal or distal radioulnar
joints. The optimal lateral view of the distal radius is
achieved on wrist imaging by aiming the x-ray beam
15 proximally, following the palmar tilt of the distal
radius. To measure ulnar variance, PA views of the
wrist should be obtained with the shoulder abducted
90 , the elbow exed 90 , and the forearm pronated.
Knowledge of anatomic landmarks may aid in
the interpretation of forearm radiographs. The
radial head and the capitellum normally align on
all views. The radial tuberosity is normally oppo-
site the radial styloid; thus, it faces toward the ulna
in supination, faces away from the ulna in prona-
tion, and is obscured by the radial shaft in the
neutral position. The coronoid process and ulnar
styloid can be used to evaluate ulnar rotation.
Distal radius fractures are among the more
common fractures that pediatric emergency med-
icine physicians fail to detect while reviewing
plain radiographs (Mounts et al. 2011); however,
plain radiographs interpreted by radiologists have
been shown to be as sensitive for distal radius
fractures as computed tomography (Welling
et al. 2008). Although they are not routinely uti-
lized for this purpose, computed tomography and
magnetic resonance imaging (MRI) may assist in
the detection of injuries associated with distal
radius fractures (Zimmermann et al. 2007).
Injuries Associated with Fractures
of the Distal Radius
Although distal radius fractures typically present as
isolated injuries, the presence of associated injuries
1051
should be determined. Bilateral distal radius frac-
tures are rare, and while their occurrence in skele-
tally mature patients is usually due to a high-energy
mechanism of injury, in skeletally immature
patients the mechanism and fracture patterns are
typically the same as those for unilateral fractures
(Ehsan and Stevanovic 2010). A distal radius frac-
ture associated with a distal radial-ulnar joint
(DRUJ) dislocation is termed a Galeazzi fracture
dislocation. More commonly children have an
associated ulnar physeal fracture, known as a pedi-
atric Galeazzi equivalent. Fracture of the ulna asso-
ciated with dislocation of the radial head, termed a
Monteggia fracture dislocation, rarely presents
concomitantly with a distal radius fracture (Sen
et al. 2011). The combination of Monteggia and
Galeazzi fracture dislocations in the same childs
arm has also been described (Maeda et al. 2003).
Monteggia and Galeazzi injuries are discussed in
separate chapters in this book.
An ulnar styloid fracture commonly presents in
association with a distal radius fracture, although
the true incidence is difcult to determine due to
the variable ossication pattern of the ulnar sty-
loid. Traditionally, this injury has not been treated,
and nonunion, which occurs in approximately
80 % of untreated cases, is usually asymptomatic.
However, nonunion has been associated with
painful TFCC tears and DRUJ instability. Thus,
some advocate reduction of displaced ulnar sty-
loid fractures by casting the wrist in ulnar inclina-
tion (Abid et al. 2008).
A scaphoid fracture is occasionally associated
with a distal radius fracture. While the site of an
isolated scaphoid fracture is usually the distal third,
when concomitant with a distal radius fracture, the
scaphoid fracture is typically of the waist and
non-displaced. Although uncommon, the presence
of a scaphoid fracture should be identied because
there is a risk of displacing the fractured scaphoid
during manipulation of the radius. Other carpal
fractures and dislocations are similarly uncommon
and tend to occur after high-energy mechanisms
(Pretell-Mazzini and Carrigan 2011; Smida
et al. 2003). Scaphoid and other carpal fractures
can be identied on plain radiographs of the wrist;
however, MRI enables early denitive diagnosis
(Zimmermann et al. 2007).
1052
Plastic deformation, also known as traumatic
bowing, is a diaphyseal deformity due to multiple
microfractures. Distal radius fractures are some-
times associated with plastic deformation of the
ulna, but bowing of the radius has also been
described in association with distal radial
metaphyseal fractures (Vorlat and De Boeck
2001). Depending on the age of the child and the
degree of angulation, the presence of plastic
deformation may alter the course of treatment.
Reduction, if indicated, is accomplished by appli-
cation of constant pressure for several minutes
(Sanders and Heckman 1984).
Displaced fractures of both the distal forearm
(radius and/or ulna) and distal humerus is termed a
oating elbow and is caused by a high-energy
mechanism. Additive swelling and hemorrhage
makes these injuries prone to forearm compart-
ment syndrome, which occurs in approximately
1533 % of patients (Hwang et al. 2009;
Blakemore et al. 2000). Circumferential cast
immobilization can increase the risk of compart-
ment syndrome, which may be reduced by
treating both fractures with closed reduction and
percutaneous Kirschner (K-) wire xation
followed by immobilization in a bivalved cast
(Ring et al. 2001; Tabak et al. 2003). Prophylactic
fasciotomies may be appropriate for patients who
are unable to communicate symptoms of compart-
ment syndrome. There is disagreement regarding
whether to rst stabilize the humerus or the fore-
arm, and published series of each approach have
demonstrated similar results (Harrington et al.
2000; Dhoju et al. 2011).
Several forms of acute median nerve injury are
associated with distal radius fractures. While car-
pal tunnel syndrome is a common complication of
distal radius fractures in adults (Niver and Ilyas
2012), in children median neuropathy occurs less
frequently and is associated with closed Salter-
Harris type II fractures. Acute carpal and volar
compartment syndromes present similarly with
rapid progression of pain and paresthesias in the
median nerve distribution and are relieved by
decompression. In contrast, median neuropathy
due to tenting by fracture fragments should
resolve with prompt reduction. However,
A. Bazzi et al.
neuropathy due to stretching or contusion of the
nerve at the time of injury may require several
weeks to recover. Thus, if neuropathy is present
on the initial examination, immediate reduction
should be performed. If neuropathy persists and
there is a strong clinical suspicion for compart-
ment syndrome, then compartment pressures
should be measured and decompression
performed if warranted. Nonetheless, all neurop-
athy patients should be admitted and monitored
closely (Waters et al. 1994).
Fractures of the Distal Radius
Classification
Distal radius fractures are classied according to
location, pattern, displacement, angulation, rota-
tion, stability, and the presence of associated inju-
ries. The AO Pediatric Comprehensive
Classication of Long-Bone Fractures may also
be used (Slongo et al. 2006). Distal radius frac-
tures usually occur with wrist extension injuries,
resulting in dorsal displacement and apex-volar
angulation. Occasionally, palmar exion injuries
and resulting volar displacement and apex-dorsal
angulation are seen. An unstable fracture is one in
which closed reduction cannot be maintained
(Waters and Bae 2010). Associated ulnar fractures
are classied as styloid avulsions, physeal inju-
ries, and complete or incomplete metaphyseal
disruptions.
The location is typically the physis or
metaphysis. Physeal fractures are described
according to the Salter-Harris classication. Rare
triplane fractures have been reported and may be
at increased risk for growth arrest (Garcia-Mata
and Hidalgo-Ovejero 2006). Metaphyseal frac-
tures may be complete, greenstick, or torus frac-
tures. Disruption of both the volar and dorsal
cortices constitutes a complete fracture, which
may result from bending, rotational, or shear
forces (Fig. 2). Complete fractures are usually
unstable and dorsally displaced, and the fracture
fragments are often in bayonet apposition.
Greenstick, or incomplete, fractures entail disrup-
tion of one cortex and compression of the other.
47 Distal Radius Fractures
Fig. 2 (a) AP and Lat pre-reduction, and (b) Post-reduction and casting of displaced unstable diametaphyseal fractures of
the distal radius and ulna
The mechanism is a combination of compressive
and rotational forces, typically dorsiexion and
supination, leading to failure of the volar cortex
in tension and compression of the dorsal cortex.
Torus, or buckle, fractures occur with compres-
sion of a diametaphyseal cortex in axial loading.
By denition, the opposite cortex is intact, and
signicant angulation and distraction are not pre-
sent. Classically, there is not an associated ulnar
fracture. Torus fractures are inherently stable,
partly as a result of the intact surrounding
periosteum.
Fractures of the Distal Radius
Outcome Tools
Distal radius fracture outcomes may be assessed
clinically by measuring range of motion and grip
strength and by tracking the incidence of compli-
cations and the need for repeat manipulation.
Radiographic parameters followed include degree
of angulation and cast index. The cast index is the
ratio of the inner diameter of the cast in the sagittal
1053
plane to that in the coronal plane and is associated
with the need for repeat manipulation (Fig. 3)
(Chess et al. 1994). The Activities Scale for Kids
performance (ASKp) version contains 30 items
and is validated for self-reporting of physical
activity by children ages 515 years (Young
et al. 1995). The visual analog scale (VAS) is a
validated instrument for the assessment of pain
(Bijur et al. 2001). Both of these scales have
been utilized in studies of pediatric distal radius
fractures (Plint et al. 2006).
Fractures of the Distal Radius
Treatment Options
The treatment options for distal radius fractures
include splint immobilization, cast immobiliza-
tion, closed reduction and cast immobilization,
closed reduction and percutaneous pinning, and
open reduction. The indications and contraindica-
tions for, and the techniques, outcomes, and com-
plications of nonoperative and operative
management are described below.
1054 A. Bazzi et al.

Table 1 Nonoperative management

Indications Contraindications
Most non-displaced Open fractures
fractures
Most Salter-Harris Irreducible fractures
type I or II fractures
Most greenstick Neurovascular compromise
fractures
Most torus fractures Excessive swelling
Most complete Displaced Salter-Harris type
fractures III or IV fractures
Plastic deformation Triplane fractures or
injuries equivalents
Ipsilateral humerus fractures
Polytrauma
Loss of initial reduction
Refractures with displacement
Displaced fractures and less
than 2 years until skeletal
maturity

Fig. 3 The cast index is measurement of the inner diam-

eter of the cast in the sagittal plane (a) divided by that in the
coronal plane (b) ideally being less than 0.8
Nonoperative Management
of Fractures of the Distal Radius
Most distal radius fractures are managed
nonoperatively. Contraindications to nonoperative
management are the same as indications for oper-
ative management and are summarized in Table 1.
These include open fractures, irreducible fractures,
excessive swelling, and risk for or presence of
neurovascular compromise. Additionally,
displaced Salter-Harris type III or IV patterns and
triplane fractures or equivalents require surgical
management for anatomic reduction. Nonoperative
management is contraindicated in the presence of
polytrauma or ipsilateral humerus fracture due to
the risk of compartment syndrome. Furthermore,
fractures which lose their initial reduction and
refractures with displacement often have poor out-
comes when managed nonoperatively. Finally,
internal xation for all displaced fractures in
patients with less than 2 years remaining until
skeletal maturity can be considered due to their
reduced capacity for remodeling compared to
younger patients.
Nonoperative management for distal radius
fractures entails immobilization with or without
closed reduction. Traditionally, immobilization is
accomplished with berglass or plaster of Paris
casting or splinting; however, prefabricated
splints and bandage therapy are also used. Both
short-arm and long-arm casts are in widespread
use. The decision between long-arm and short-
arm cast immobilization depends on the displace-
ment of the fracture and age of the patient. This is
further discussed in the following sections. Closed
reduction is performed with adequate analgesia,
usually in the emergency room under conscious
sedation. Portable uoroscopy may be used for
guidance and assessment of the reduction. While
reduction is typically performed by an orthopedic
surgeon where available, many emergency medi-
cine physicians and family practitioners are also
trained to evaluate and provide nonoperative man-
agement of distal radius fractures.
Non-displaced fractures of the physis and
metaphysis with acceptable angulation and rota-
tion may be amenable to immobilization without
reduction. Metaphyseal fractures have excellent
remodeling potential and up to 10 per year of
dorsal-volar angulation may correct with contin-
ued growth. The range of angular deformity
47 Distal Radius Fractures 1055

Table 2 Acceptable angular deformity for metaphyseal mechanism and consequently displace dorsally
fractures (degrees) with apex-volar angulation (Fig. 4). Alignment
Dorsal-volar Radial-ulnar of the fragments is traditionally acceptable with
Age (year) Boys Girls Boys and girls less than 50 % displacement and no angular or
49 20 15 15 rotational deformity (Egol et al. 2010); however,
911 15 10 5 some advocate immobilization without reduction
1113 10 10 0 for Salter-Harris type II fractures with less than
>13 5 0 0 20 of angulation and less than 40 % displacement
in children under age 10 (Houshian et al. 2004).
Physical and occupational therapy are not usually
Table 3 Immobilization without reduction
required for pediatric distal radius fractures.
Indications Contraindications
Most torus fractures Excessive angular
deformity (see
Table 2)
Technique: Closed Reduction
Consider for S-H II, <40 % Most displaced of Physeal Fractures
displacement, <20 physeal fractures
angulation, child <10 years In Salter-Harris type I and II fractures, the dorsal
old periosteum is usually intact and can be used as a
Consider for complete Most complete
tension band to aid reduction. Although the thick-
fractures with bayonet fractures
apposition in very young child ness of the periosteum limits the utility of pulley-
Some greenstick fractures Rotational deformity weight traction, nger traps with less than 10 lb of
Plastic deformation counterweight or an assistant are helpful to sup-
port and stabilize the extremity for reduction and
casting. The fracture may reduce with traction
accepted in practice varies and is clinician depen- alone; otherwise, gentle thumb pressure applied
dent. As a general guide, the traditional tolerances at the fracture site in a distal and volar direction
are provided in Table 2, adapted from the facilitates atraumatic exion of the distal epiphy-
Rockwood and Wilkins text (Waters and Bae sis (Fig. 5). Alignment of the fragments is tradi-
2010). tionally acceptable with less than 50 %
In contrast, rotational deformity will not remodel displacement and no angular or rotational defor-
and is an indication for reduction. Malrotation is mity. Multiple reduction attempts may increase
often present when both the radius and ulna are the risk of growth arrest due to increased shear
fractured and the fracture sites are at two different forces across the physis. Immobilization in the
levels, proximal and distal to each other. Apex-volar neutral position or pronation is recommended.
angulation is often associated with supination of the Portable uoroscopy, if available, could be used
distal fragment; apex-dorsal angulation, with pro- to immediately assess the reduction before immo-
nation. Failure to recognize and reduce rotational bilization. Irreducibility is most often due to
deformity is a common pitfall in the treatment of entrapment of the periosteum or pronator
greenstick fractures. The indications and contrain- quadratus.
dications for immobilization without reduction for
distal radius fractures are summarized in Table 3.
Discussion: Nonoperative Management
of Torus Fractures
Discussion: Nonoperative Management
of Physeal Fractures The traditional standard of care for torus fractures
is immobilization in a short-arm cast for 34
Salter-Harris type I and II fractures of the distal weeks. The theoretical benets of casting are pro-
radius are typically the result of an extension tection against pain, displacement, and refracture.
1056
Fig. 4 (a) AP and Lat of a Salter-Harris II fracture of the
distal radius with dorsal displacement and mild angulation
(b) AP and Lat at 6 weeks and (c) at 4 months
A. Bazzi et al.
Fig. 5 Example of gentle thumb pressure in a distal and
volar direction to reduce dorsally-displaced non-shortened
physeal injury
Torus fractures usually do not require reduction,
are inherently stable, and have little risk of late
displacement; accordingly, alternatives to cast
immobilization have been studied (Bae and How-
ard 2012). Treatment with removable splints may
reduce the amount of clinic visits required,
enables easier bathing, and avoids cast-saw-
related anxiety. Several prospective, randomized
controlled trials have compared removable wrist
splints to casts. Davidson et al. reported success-
ful healing with no complications in all patients
treated with splinting or casting (Davidson
et al. 2001). In addition, Plint et al. found no
difference in pain on the VAS between the two
groups. The splint group had signicantly better
ASKp scores at one of four time points assessed,
suggesting that children have less difculty with
activities while in splints compared to while in
casts (Plint et al. 2006). In contrast, Oakley
et al. found that patients treated with a volar slab
splint had longer durations of pain and longer
times until resumption of normal activity (Oakley
et al. 2008). Splint management has been found to
reduce total cost per patient by approximately
$7382, with the majority of savings due to
attending one fewer clinic visit for cast or splint
removal (Davidson et al. 2001; von Keyserlingk
et al. 2011).
Soft casts are the preferred treatment in some
institutions. Trials by Khan et al. and Witney-
Lagen et al. reported full recovery, according to
parental evaluation, in both soft cast and rigid cast
47 Distal Radius Fractures
treatment groups (Khan et al. 2007; Witney-
Lagen et al. 2013). Parents may safely remove
precut plaster back slabs (Symons et al. 2001)
and soft casts (Khan et al. 2007) at home after
3 weeks if adequate explanation of removal is
provided during the initial treatment. Most fami-
lies prefer removal at home rather than in the
clinic and prefer soft casts to rigid casts. Thus,
although soft cast material is more expensive than
rigid cast material, removal at home contributes to
decreased total cost of care for both soft casts and
splints.
Studies have also demonstrated satisfactory
outcomes in the management of torus fractures
with bandage therapy. West et al. randomized
patients to either casting or treatment with a
layer of orthopedic wool covered by crepe ban-
dage and held in place with tape. They reported a
decreased incidence of pain and decreased dura-
tion of pain in the bandage group; however, a
validated pain scale was not used. Fracture
healing was universal, and the bandage group
had signicantly greater range of motion com-
pared to the cast group, as measured on the day
of cast removal at 4 weeks (West et al. 2005).
Kropman et al. performed a similar trial, and
there were no complications in either group. In
contrast to the ndings of West et al., they
reported that VAS pain scores were signicantly
increased in the bandage group during the rst
week of treatment. The cast group experienced
more discomfort (i.e., itching). Range of motion
was reduced in the casting group on the day of cast
removal; however, there was no signicant differ-
ence 2 weeks later. In one center, bandage therapy
was made the standard of care for torus fractures,
and no secondary angulation or refractures
occurred in 49 consecutively treated patients
(Vernooij et al. 2012).
In conclusion, there are several treatment
options for torus fractures, which are inherently
stable injuries. Although the traditional standard of
care is rigid immobilization in a short-arm cast for
34 weeks, the literature supports treatment in a
wrist splint or soft cast that can be removed at home
after 3 weeks with no radiographic follow-up.
1057
Additionally, a growing body of evidence sug-
gests that bandage therapy is safe and effective.
The use of splints, soft casts, and bandages
decreases the workload of the fracture clinic and
reduces the cost of care. While most parents of
children treated with splints, soft casts, and ban-
dages would prefer them to rigid casts, many
parents may be hesitant to forego traditional
rigid immobilization and radiographic follow-up.
Thus, treatment of torus fractures should be the
result of a shared decision-making process involv-
ing the patient, parents, and physician and should
consider any special needs of the child and the
impact on the family of attending a follow-up
visit. Finally, it warrants mentioning that errors
in torus fracture diagnosis are not uncommon and
are typically due to failure to recognize a
greenstick fracture (Fig. 6). Therefore, review of
the radiographs by an experienced physician is
mandatory.
Discussion: Nonoperative Management
of Greenstick Fractures
Greenstick fractures typically present as failure of
the volar cortex in tension and the dorsal cortex in
compression. Radial displacement and apex ulnar
angulation are often present. Traditional toler-
ances for angular deformity are shown in
Table 2. Fractures which are minimally angulated
on presentation may be immobilized without
reduction (Al-Ansari et al. 2007; Do et al. 2003).
However, excessive angulation presents a risk of
lost forearm rotation and should be corrected to
maintain the interosseous space.
Rotational deformity is also common, espe-
cially when there is an associated ulna fracture,
and correction of malrotation is essential to
achieving anatomic alignment. However, rota-
tional correction often fractures the intact cortex,
thus completing the fracture. Intentional comple-
tion of the fracture is controversial, and little data
is available to evaluate this practice. Some argue
that fracture completion decreases the risk of
redisplacement; however, some evidence suggests
1058
Fig. 6 (a) AP, (b) Lateral, and (c) Oblique of a greenstick fracture of the distal radius
that the opposite may be true (Waters and Bae
2010; Schmuck et al. 2010). Nevertheless,
fracture completion appears to be useful
when primary angulation exceeds remodeling
capacity.
Clinical practice varies regarding the position
and type of forearm immobilization. Some advo-
cate for immobilization in supination to minimize
the deforming effect of the brachioradialis; in the
neutral position, to maintain the interosseous
space and rotational range of motion; and in pro-
nation, to reduce the common supination defor-
mity (Waters and Bae 2010). Boyer et al. found no
difference in angulation in patients randomized to
supination, neutral position, or pronation (Boyer
et al. 2002). Local practice determines the choice
of long-arm cast, short-arm cast, or splint
(Bae and Howard 2012). Classically, a long-arm
cast is applied for 34 weeks, followed by a short-
arm cast for 13 weeks. Elbow immobilization is
thought to decrease the risk of displacement by
limiting the childs activities and reducing the
deforming effect of the brachioradialis (as in
supination). However, in trials by Bohm
et al. and Webb et al., there was no difference in
the rate of lost reduction between patients
A. Bazzi et al.
randomized to short-arm casts or long-arm casts
after closed reduction (Bohm et al. 2006; Webb
et al. 2006). Moreover, patients treated with a
long-arm cast were more likely to require assis-
tance with activities of daily living and missed, on
average, one more day of school than those treated
with a short-arm cast. Additionally, Boutis
et al. found no signicant difference between
patients randomized to short-arm casts or
prefabricated wrist splints in complication rate,
ASK scores, grip strength, range of motion, and
radiographic measurement of angulation (Boutis
et al. 2010).
In conclusion, most greenstick fractures are
treated with closed reduction and rigid immobili-
zation, but the acceptable angulation, position,
and type of immobilization vary in clinical
practice. Failure to recognize and reduce
rotational deformity is a common pitfall in the
treatment of greenstick fractures. While
long-arm casts are the traditional standard of
care, the literature supports the use of well-
molded short-arm casts. Redisplacement is com-
mon, and weekly radiographic follow-up is indi-
cated until there is evidence of sufcient callus
formation.
47 Distal Radius Fractures
Technique: Closed Reduction
of Greenstick Fractures
Correction of malrotation is essential to achieving
anatomic alignment in greenstick fractures. Finger
traps are not used. While pressure is applied to the
apex of deformity, the patients thumb should be
rotated toward the apex of angulation. In other
words, apex-volar fractures require pronation of
the distal fragment and apex-dorsal, supination.
Fracture of the intact cortex during this maneuver
may occur but is not necessary. Angular deformity
should be corrected to fewer than 10 . Mainte-
nance of alignment may be aided by immobilizing
the forearm in the rotational position used to
achieve the reduction. While portable uoros-
copy, if available, should be used to assess the
reduction, postreduction radiographs should
include the entire forearm to facilitate evaluation
of malrotation.
Discussion: Management of Complete
Fractures
Complete fractures of the distal radius often pre-
sent with associated ulna fractures, are usually
dorsally displaced, and are frequently in bayonet
apposition. In other words, the distal fragment
frequently lies in a side-to-side rather than end-
to-end relationship to the proximal fragment. The
traditional standard of care is closed reduction and
cast immobilization. Regardless of the presence of
an associated ulna fracture, reduction is difcult to
maintain. The rate of redisplacement following an
initial, acceptable closed reduction has been
reported between 21 % and 91 %, but in most
studies, it is approximately 25 %. The greatest
risk factors for redisplacement are initial angula-
tion greater than 30 , incomplete reduction, and
complete displacement, although displacement
greater than 50 % also increases this risk (Zamzam
and Khoshhal 2005; Alemdaroglu et al. 2008;
McQuinn and Jaarsma 2012).
Loss of reduction may lead to a malunion,
which has excellent potential for remodeling in
the dorsal-volar plane (see Pathoanatomy and
Applied Anatomy Relating to Fractures of the
1059
Distal Radius section). However, deformity in
the radial-ulnar plane has less potential for
remodeling (see Table 2). Repeat manipulation
may be indicated following loss of reduction to
avoid a malunion. One should use caution not
re-manipulate a physeal fracture after 710 days
of injury as this carries a higher risk of arrest. For
their study, Alemdaroglu et al. (2008) dened
redisplacement as dorsovolar angulation of 10
or greater, radioulnar angulation of 5 or greater,
translation of 3 mm or greater, or the combination
of dorsovolar angulation of 5 or greater and
translation of 2 mm or greater. The authors
remanipulated fractures that had dorsovolar angu-
lation of greater than 20 , radioulnar angulation of
greater than 10 , translation of greater than 4 mm,
or any combination of two of the following:
dorsovolar angulation of greater than 10 ,
radioulnar angulation of greater than 5 , and
translation of greater than 3 mm (see Table 4).
Some surgeons advocate conservative man-
agement to take advantage of the tremendous
remodeling potential of the distal radius. Do
et al. (2003) found that fractures with less than
15 of dorsovolar or radioulnar angulation and
less than 1 cm of shortening heal without defor-
mity or clinical sequelae. The average time to
bony healing and cast removal was 6 weeks, and
remodeling was complete after an average of
4 months (up to 13 months in older children).
Moreover, Crawford et al. (2012) reported excel-
lent results in 51 consecutive children with frac-
tures in bayonet apposition treated with no
attempt at anatomic reduction. Within 72 h of
injury, in an outpatient clinic with no analgesia
or sedation, short-arm casts were applied and
gently molded to correct angulation, leaving the
fractures overriding and shortened. The average
dorsovolar and radioulnar angulation after reduc-
tion were 4.0 and 3.2 , respectively, (range 013,
010) and at follow-up after 1 year were 2.2 and
0.75 (range 010, 05). All patients progressed
to union and full range of wrist motion, there were
no complications, and only a few patients had a
minimally noticeable clinical deformity. The cost
of care for this approach was approximately
one-fth that of closed reduction with conscious
sedation and approximately one-eighth that of
1060 A. Bazzi et al.

Table 4 Criteria for redisplacement and remanipulation

(Alemdaroglu et al. 2008)
Redisplacement Remanipulation
Dorsovolar 10 isolated, >20 isolated,
angulation 5 in >10 in
(degrees) combination combination
Radioulnar 5 isolated >10 isolated, >5
angulation in combination
(degrees)
Translation 3 isolated, 2 >4 isolated, >3
(mm) in combination in combination
Fig. 7 Depiction of intact dorsal periosteum in a pediatric
distal radius fracture

percutaneous pin xation with general anesthesia.

The authors advocate for this approach as rst-
line treatment, noting that it avoids the risks of
anesthesia, lessens the time required by the
treating physician, and reduces the cost of care.
In conclusion, the traditional standard of care
for complete fractures of the distal radius is closed
reduction and cast immobilization with close
radiographic follow-up until there is evidence of
healing (typically 6 weeks). Loss of reduction is
common, and repeat manipulation may be indi- Video 1
cated to avoid a malunion. The literature supports
primary percutaneous pin xation as an alterna-
tive for fractures at high risk of displacement or
when excessive swelling is present to reduce the
risk of neurovascular compromise. However,
these fractures have excellent remodeling poten-
tial, and good results have been obtained by
correcting angular deformity with gentle cast
molding and allowing healing to occur in an over-
riding, shortened position. Thus, treatment should
be guided by a shared decision-making process
involving the patient, parents, and physician with
consideration of the risk of loss of reduction based Fig. 8 Demonstration of placement of thumb pressure to
on the patients age and fracture characteristics. distract a shortened displaced fracture with intact dorsal
periosteum

designed to provide greater mechanical advantage

Technique: Closed Reduction
of Complete Fractures
Finger traps with weight of less than 10 lb may be
useful to stabilize the hand during casting, but the
intact periosteum will not usually stretch to permit
reduction through traction, and the tense perios-
teum may hinder reduction (Fig. 7). Eichinger
et al. (2011) described a traction technique
by securing the patients arm beneath the sur-
geons thigh. Regardless of the traction technique
employed, the key to anatomic reduction is initial
exaggeration of the deformity (usually dorsal dis-
placement and apex-volar angulation of the distal
fragment) [see Video 1]. The dorsum of the hand
is often brought to an acute angle with the dorsum
of the forearm. Thumb pressure is then applied to
distract the distal fragment (Fig. 8). Next, the distal
47 Distal Radius Fractures
fragment is exed volarly to obtain reduction, and at
the same time, malrotation is corrected if present.
Finally, residual translation is corrected through
toggling the distal fragment by slight dorsiexion
and volarly directed thumb pressure. Portable uo-
roscopy, if available, should be used to immediately
assess the reduction before immobilization.
Technique: Splint Immobilization
Prefabricated splints have been studied as deni-
tive therapy for torus and greenstick fractures.
Additionally, sugar-tong splints, often used as a
temporizing measure, are reportedly effective for
maintaining reduction of complete fractures
(Denes et al. 2007). Successfully applied splints
limit exion and extension of the wrist and pro-
nation and supination of the forearm. Following
the placement of well-tted stockinette, the elbow
should be exed to 90 and the forearm held in
neutral rotation. Cotton padding should be
rolled with 50 % overlap from the proximal
interphalangeal joints to three centimeters proxi-
mal to the antecubital fossa, with extra padding for
bony prominences. Measure a length of plaster to
extend just proximal to the dorsal metacarpo-
phalangeal (MCP) joints around the elbow in a
U-shape to the fracture site on the volar surface
(Egol et al. 2010). Ten layers of two- to four-
inch-wide plaster should be submerged in
room temperature water and then pressed together
to bond the layers and remove excess water. The
plaster should be held in place by an assistant or
by a cooperative patients contralateral hand,
while an elastic bandage is wrapped with
gentle tension and 50 % overlap from distal to
proximal along the length of the plaster. As the
splint starts to dry, carefully apply a three-
point mold or a banana-shaped mold, applying
pressure with the base of the palm. To correct
apex-volar angulation, the middle pressure
point should be on the volar aspect just proximal
to the fracture, and the proximal and distal
points should be on the dorsal aspect (Fig. 9).
The opposite placement will help correct apex-
dorsal angulation. After the splint dries, a
neurovascular exam of the affected extremity
1061
Fig. 9 Demonstration of the 3-point mold to hold the
reduction in cast
should be performed. It is important to keep the
MCP joints free for unhindered nger motion to
be permitted.
Technique: Cast Immobilization
Stockinette and cotton padding should be applied
and the extremity positioned as described above,
with the exception for smaller children of
extending the elbow to approximately 90 of ex-
ion. This allows for better forearm molding. The
plaster or berglass should extend from the prox-
imal palmar crease to either 3 cm distal to the
antecubital fossa (for a short-arm cast) or to the
mid-humerus (for a long-arm cast). The MCP
joints should move freely. The thumb should be
able to touch the small nger unless an elbow-
extension cast is applied, in which case the thumb
should be included in extension to prevent distal
migration of the cast. A three-point mold should
be applied around the fracture site as described
above (Fig. 9). Additionally, an oval-shaped mold
helps maintain the interosseous space, and a
straight ulnar mold and posterior humeral mold
help prevent migration. In anticipation of swell-
ing, the cast should be bivalved and over-wrapped
with an elastic bandage. A neurovascular exam
should be performed and nal radiographs should
be obtained.
Several cast parameters have been developed to
measure the quality of reduction and molding. The
cast index is the ratio of the inner cast diameters
(sagittal divided by coronal) at the fracture site, and
1062
higher values have been associated with loss of
reduction. Better outcomes are traditionally asso-
ciated with values of 0.7 or less, and evidence
suggests that values of 0.81 or greater are associ-
ated with an increased risk of loss of reduction
(Chess et al. 1994; Ortega Vadillo et al. 2010;
Kamat et al. 2012). Additionally, Edmonds
et al. (2009) identied an association between the
second metacarpal-radius angle and better out-
comes, noting that fractures were more likely to
have an ideal outcome if molded in ulnar deviation.
Finally, the three-point index is found by calculat-
ing the ratio of the sum of the critical gap dis-
tances and the length of contact area between the
two fracture fragments on PA and lateral radio-
graphs and then taking the sum of these two ratios.
The critical gaps are the distances between the
skin and cast at approximately the sites where
three-point molding should be applied (see
section Technique: Splint Immobilization).
In a prospective study by Alemdraglu et al. (2008),
a three-point index value of 0.8 or greater was 95 %
sensitive and 95 % specic for redisplacement.
Thus, the three-point index may be used to assess
the quality of cast molding and to predict
redisplacement.
Operative Treatment of Fractures
of the Distal Radius
Indications/Contraindications
The main indications for operative treatment of
distal radius fractures include those with associ-
ated neurovascular injuries, especially median
neuropathy, open fractures, a large amount of
volar swelling, irreducible fractures, and loss of
reduction after initial closed treatment. Median
neuropathy can be seen with injuries, which
cause a direct contusion to the nerve, stretch
neuropraxia, laceration from the fracture frag-
ment, and/or imminent compartment syndrome
with a large hematoma causing direct pressure.
Closed reduction with a circumferential cast,
especially with a large volar amount of swelling,
can potentially worsen symptoms. Fractures with
A. Bazzi et al.
Table 5 Preoperative planning
OR table: A standard OR table is utilized
Position: Supine, no bumps required
Fluoroscopy location: Placement of image intensier
ipped upside down, on affected side, parallel to bed and
can be used as the operative table itself. Alternatively, a
radiolucent arm table can be attached to the side of the
table and uoroscopy can enter from underneath
Equipment: Basic ortho-tray, power wire driver, smooth
Steinmann pins, cast cart
Tourniquet: A nonsterile tourniquet is placed on the
upper arm
an open injury to the soft tissue envelope warrant
surgical irrigation and debridement to prevent
infection, osteomyelitis, and delayed union. Irre-
ducible fractures are likely due to entrapment of
the periosteum and less often the pronator
quadratus.
Surgical Procedure
Closed Reduction Percutaneous Pin
Fixation
Preoperative planning (see Table 5).
Positioning
The patient is placed in the supine position on the
operative table and shifted to the edge of the bed
on the affected side, without any bumps neces-
sary. The table is rotated in the room as needed to
allow for the necessary room for the image
intensier.
Surgical Approaches/Technique
An anesthetic is delivered, and a nonsterile tour-
niquet is placed on the upper arm. The upper
extremity is prepped and draped in the usual ster-
ile manner. It remains a surgeons choice whether
to utilize a tourniquet during this minimally inva-
sive procedure. Closed reduction of the fracture is
performed rst with an adequate amount of trac-
tion. For metaphyseal and diametaphyseal frac-
tures, subsequent exaggeration of the fracture will
allow the surgeon to unhinge the fragments and
ease in the reduction. To minimize the risk of
growth arrest in physeal injuries, it is imperative
47 Distal Radius Fractures
Fig. 10 Skin landmarks of typical pattern of the super-
cial sensory branch of the radial nerve
to utilize an adequate amount of traction. This
alone can aid in reduction, with or without the
need for volarly or dorsally based digital pressure
over the epiphysis as indicated. Once the fracture
is conrmed to be adequately reduced on image
intensication, skeletal stabilization is ensued.
The radial styloid is palpated along with
Listers tubercle. For metaphyseal fractures
where the distal fragment cannot be captured
without crossing the physis of the distal radius or
physeal injuries, a small centimeter-length linear
incision is made distal to the radial styloid. This
incision is made sharply through skin and subcu-
taneous tissue, and then a hemostat is utilized to
bluntly dissect down to bone to prevent iatrogenic
injury to the supercial sensory branch of the
radial nerve and the extensor tendons (Fig. 10).
Then, a 1.6-mm smooth Steinmann pin is selected
and is driven through the epiphysis radial styloid
tip with the greatest attempt to remain perpendic-
ular to and central in the physis (1.1-mm
Kirschner wires are utilized in patients under the
age of 6). This is advanced into the proximal
ulnar-sided cortex of the radius (Fig. 11). It is
preferable to engage the cortex of the metaphyseal
fragment just proximal to the distal radial physis.
The stability is checked with uoroscopy, and if
needed a secondary pin is utilized in a crossing
fashion or parallel to the rst pin. Once stability is
conrmed, the pin is prebent and cut outside of the
skin. Passive motion of the wrist and digits is
checked to rule out any tethering of the tendons.
If a tourniquet was utilized, it is then deated and
1063
pulses are palpated. Sterile nonadherent dressings
are placed around the pin, and then the extremity
is immobilized in either a long-arm posterior
splint or a bivalved berglass cast.
The postoperative course includes a follow-up
appointment in 1 week to assess radiographic
alignment. Pins are maintained for a total of 34
weeks and then removed in the outpatient ofce.
The length of immobilization is a total of 6 weeks
on average. Once radiographic and clinical
healing is conrmed, the patient is eased back
into range of motion and subsequent strengthen-
ing, with or without the need for formal physical
therapy, which is determined on a case-by-case
basis.
Open Reduction of Irreducible
Fractures
Irreducible fractures are most likely due to
entrapped periosteum and/or the pronator
quadratus itself. Preoperative planning and patient
positioning is the same as above for the percuta-
neous technique. These fractures are accessed tra-
ditionally via an open volar approach to gain
access to the entrapped tissue/muscle. Once the
fracture is reduced, it can be stabilized with plate
osteosynthesis or K-wire xation. It is
recommended that 46 cortices are captured in
the metaphyseal (i.e., distal) fragment for stable
xation. The wound is then closed and a volar
plaster splint is applied or a bivalved cast
(Table 6).
Treatment-Specific Outcomes
of Percutaneous Pinning of Distal
Radius Fractures
Percutaneous pin xation has been shown to be
successful for the treatment of patients with
excessive swelling in order to reduce the risk of
neurovascular compromise and is often used
as alternative to repeat manipulation to correct
late redisplacement. Due to the risk of
redisplacement, some surgeons prefer closed
reduction and immediate percutaneous pinning.
1064
Fig. 11 (a) AP and Lat of completely displaced distal radius physeal fracture with associated moderate soft tissue
swelling and sensory changes warranting (b) K-wire xation to hold reduction (c) 1 month post-op (d) 6 weeks post-op
McLauchlan et al. (2002) and Miller et al. (2005)
randomized children to either closed reduction
and cast immobilization or the additional inser-
tion of a percutaneous K-wire (McLauchlan) or
Concept (C-) wire(s) (Miller), which was/were
removed three (McLauchlan) or four (Miller)
weeks later. Miller et al. studied fractures at
high risk for loss of reduction, including only
children older than age 10 years with either com-
plete displacement or angulation greater than
30 . To minimize the effects of poor reduction
and casting technique, these patients were treated
by an attending pediatric orthopedic surgeon. In
the control groups, 21 % (McLauchlan) and 39 %
(Miller) of patients underwent a second proce-
dure to correct unacceptable deformity. Miller
et al. used complete displacement or angulation
A. Bazzi et al.
greater than 25 as criteria for remanipulation. In
the pin groups, there was no loss of reduction;
however, the rates of pin-related complications
(pain, prominent scarring, and wire migration)
were 11 % (McLauchlan) and 38 % (Miller).
Pin-related complications included hyperesthe-
sia, prominent scarring, wire migration, pin-site
infections, and tendon irritation, all of which
resolved following pin removal. Both studies
reported no signicant differences between the
groups in long-term outcomes including wrist
range of motion and strength. Additionally,
Miller et al. reported that the cost of care was
not signicantly different between groups. Thus,
primary percutaneous pin xation is a safe and
effective alternative for fractures at high risk of
displacement.
47 Distal Radius Fractures 1065

Table 6 Surgical pitfalls and prevention open fractures, a large amount of volar swelling,
Potential pitfall Pearls for prevention irreducible fractures, and a loss of reduction after
Injury to the supercial Blunt dissection after initial initial closed treatment, the authors prefer skeletal
branch of the radial skin and subcutaneous stabilization via percutaneous pinning. Pins are
nerve incision utilizing hemostat generally removed in 34 weeks postoperatively.
nick and spread technique
The fracture is then protected for another 2 weeks
Utilization of a drill guide
during insertion of K-wire in a short-arm cast.
Tendon irritation/wire As above for nerve
migration protection
Prebending the wire outside Summary and Future Directions
the skin with adequate
padding
Distal radius fractures are the most common frac-
Infection Placement of wire under
sterile technique
tures in the pediatric population, with an incidence
Prompt removal once of 2131 % of all pediatric fractures. They com-
adequate callus formation monly occur as a result of a traumatic fall, are
Patient/parental education more common in males than females, and their
on cast care and prevalence is on the rise. The pediatric wrist frac-
maintenance of clean/dry
ture has excellent remodeling potential, as the
dressings
Pain associated with pin Wires can be prebent and
distal radius physis contributes to approximately
removal/scarring cut underneath skin for later 80 % of the longitudinal growth of the forearm.
removal in operative setting This is especially true in the younger patient with
more than 2 years of growth remaining.
Clinical examination and radiographic evalua-
Preferred Treatment tion of the affected limb will reveal the fracture in
question. This could include a diametaphyseal frac-
Distal radius fractures are for the most part man- ture of variable displacement and angular instabil-
aged by closed means. A closed displaced and/or ity, physeal injuries, most commonly of the Salter-
angulated fracture with parameters outside the Harris I and II patterns, torus (buckle) type, or
abovementioned tolerances is closed reduced greenstick fractures. These fractures are commonly
and immobilized in the emergency room setting associated with ulnar fractures, either at the same
under conscious sedation. The authors immobili- level or at the ulnar styloid. The rate of growth
zation of choice in the acute setting is a bivalved arrest with displaced physeal injuries of the distal
berglass long-arm cast for the highly unstable radius is on average 45 %, while an ulnar physeal
fractures, physeal injuries, and the younger injury can be present in up to 50 % of cases.
patient where a short-arm cast is at risk for falling It is imperative not to miss associated dislocations,
off. Diametaphyseal, torus, and greenstick frac- such as Galeazzi or Monteggia fracture disloca-
tures, which are otherwise more inherently stable, tions, or more proximal fractures at the elbow.
can be managed in a bivalved short-arm berglass Treatment options include nonoperative immo-
cast. Follow-up radiographs and clinical evalua- bilization, closed reduction and percutaneous pin-
tion are done at the 1-week mark to assess align- ning, and open reduction internal xation. Most
ment and stability. This may require multiple non-displaced fractures, Salter-Harris I and II,
1-week clinical visits to assess stability. The typ- greenstick, buckle, complete, or plastically
ical period of immobilization is 6 weeks total. deformed fractures, are amenable to rst-line
Stable torus fractures are seen in 34 weeks for nonoperative treatment. The orthopedic surgeon,
repeat imaging out of cast. the emergency physician, as well as the primary
As for the distal radius fractures associated care physician will continue to frequently address
with neurovascular injuries, median neuropathy, wrist injuries in children. The appropriate
1066
diagnosis of a fracture and the ability to follow a
prescribed treatment algorithm is imperative to
restore function, motion, and a symptom-free
wrist.
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 Forearm Fractures
48
Johnathan J. Whitaker, Brandon M. Tauberg,
Michael S. Kwon, and Martin J. Herman

Contents Emergency Department Management . . . . . . . . . . . . . . 1076

Analgesia/Sedation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1076
Part A: Nonoperative Treatment . . . . . . . . . . . . . . . . . 1070 Fracture Manipulation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1077
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1070 Greenstick Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1077
Epidemiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1070 Complete Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1078
Mechanism of Injury . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1070 Immobilization After Reduction . . . . . . . . . . . . . . . . . . . 1079
Applied Anatomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1071
Pathoanatomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1072 Acceptable Reduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1079
Children Younger than 10 Years of Age . . . . . . . . . . . 1080
Assessment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1072 Children 10 Years of Age and Older . . . . . . . . . . . . . . . 1080
Signs and Symptoms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1072
Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1073 Complications of Nonoperative Treatment . . . . . . 1080
Stiffness . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1080
Associated Injuries . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1073 Malunion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1080
Classication . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1073 Refracture . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1081
Fracture Patterns . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1073 Uncommon Complications . . . . . . . . . . . . . . . . . . . . . . . . . 1081
Greenstick Fracture . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1074 Part B: Operative Treatment . . . . . . . . . . . . . . . . . . . . . 1081
Complete Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1075 Indications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1081
Fracture Displacement: Angulation, Translation, Surgical Procedures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1081
and Malrotation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1075
Complications of Surgical Treatment . . . . . . . . . . . . 1087
Outcome Assessment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1076 IMN Versus ORIF? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1087
Nonoperative Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . 1076 Intramedullary Nailing . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1088
ORIF . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1090
Preferred Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1091
Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1092
J.J. Whitaker (*)
Department of Orthopaedic Surgery, Philadelphia References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1092
College of Osteopathic Medicine, Philadelphia, PA, USA
e-mail: johnathanwh@pcom.edu
B.M. Tauberg
Drexel University College of Medicine, Philadelphia,
PA, USA
e-mail: brandon.michael.tauberg@drexel.edu
M.S. Kwon M.J. Herman
Department of Orthopaedic Surgery, Drexel University
College of Medicine, St. Christophers Hospital for
Children, Philadelphia, PA, USA
e-mail: mikekwon.orthodoc@gmail.com;
martin1.herman@tenethealth.com

# Springer Science+Business Media New York 2015 1069

J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_49
1070 J.J. Whitaker et al.

treatment, especially exible intramedullary nail

Abstract
xation (Cheng et al. 1999; Flynn et al. 2010).
Diaphyseal fractures of the ulna and radius are
The indications for this procedure and the best
frequent injuries in the pediatric population.
methods of xation are evolving. Many children,
These fractures are typically the result of
particularly those older than 1012 years of age,
low-energy falls onto an outstretched hand.
with unstable fractures of the forearm may be best
Nevertheless, a careful assessment of the
treated with surgery. However, complications
patient is imperative, with evaluation of the
related to surgical treatment are not uncommon
skin and soft tissues for lacerations, compart-
and must be taken into consideration when decid-
ment swelling, as well as a complete
ing between operative and nonoperative manage-
neurovascular examination. The majority of
ment. Despite extensive experience with nail
forearm fractures are closed injuries without
xation, complications such as extensor tendon
associated nerve injuries or vascular compro-
injuries, nerve injuries, and prolonged fracture
mise. These fractures are most often best
healing remain problems to overcome.
treated with primary closed reduction and cast
immobilization.
Forearm fractures that necessitate surgical
Epidemiology
xation include irreducible or unstable frac-
tures that have failed an attempt at closed
Approximately 1/100 children per year will expe-
reduction, open fractures, and fractures associ-
rience a forearm fracture, with most occurring in
ated with severe soft tissue trauma, oating
the distal radius (Chung and Spilson 2001). The
elbow injuries, vascular injury, or compart-
age of fracture incidence peaks in boys and girls at
ment syndrome. Flexible intramedullary nail
age 9, but boys also have a second peak at the age
xation is the treatment of choice for most
of 14 (Landin 1983). Forearm fractures occur with
children and skeletally immature adolescents.
equal frequency in males and females until the age
Surgical treatment is generally associated with
of 11 or 12; after that, males sustain these fractures
an increased number of complications, espe-
almost twice as frequently as females. In addition
cially in children older than 10 years of age.
to being a common location for primary injury, the
Compartment syndrome, infections, nerve
pediatric forearm is the most common site of
injuries, and extensor tendon injuries are the
refracture after healing of a fracture in the same
most common complications associated with
location (Landin 1997).
surgical treatment. This chapter will focus on
closed reduction and casting, surgical treat-
ments, and avoidance of complications.
Mechanism of Injury

The majority of forearm fractures result from a fall

Part A: Nonoperative Treatment
Introduction
Forearm fractures in children and adolescents are
one of the most common causes for children to
receive orthopedic care (Chung and Spilson
2001). The majority of these fractures are best
treated with closed reduction and cast immobili-
zation (Jones and Weiner 1999). In the past 1015
years, however, an increasing number of children
with these injuries are undergoing surgical
onto an outstretched hand (Aktas et al. 1999).
These injuries typically occur from low- and
moderate-energy mechanisms such as falls from
a step or playground equipment or during sports
activities. Direct blows to the forearm represent
another important mechanism. Forearm fractures
are the type of fracture most commonly associated
with trampoline injuries and the second most
common fracture seen after falls from monkey
bars (Waltzman et al. 1999). In one report from
Finland, the incidence of forearm fractures has
been increasing, with the rising use of trampolines
48 Forearm Fractures
by children being cited as a potential reason for
the change (Sinikumpu et al. 2012). High-energy
mechanisms, such as being struck by a motor
vehicle or falling off a motorized vehicle, are
less common but are associated with an increased
risk of concomitant serious injuries, as well as the
potential for severe soft tissue damage and
neurovascular injury to extremities.
Applied Anatomy
The radius and ulna are the two bones that make
up the forearm, with the radius being the more
lateral bone. The shaft of the radius has three
sides, two of which are convex. One convexity
is along the midportion of the bone and is 10 with
the apex lateral-radial. The other convexity is 15
with its apex medial and more proximal (Firl and
Wunsch 2004). The radial bow refers to the
midportion deviation of the radius. Forearm rota-
tion is dependent on normal anatomic contour of
the radial bow (Sage 1959). Two important bony
landmarks of the radius are the radial styloid and
the bicipital tuberosity. The radial styloid is a
lateral, distal prominence, and the bicipital tuber-
osity is an anteromedial prominence. These two
structures are oriented slightly less than 180 from
each other (Milch 1944). Nine muscles attach to
the radius: the abductor pollicis longus, biceps,
brachioradialis, extensor pollicis brevis, exor
digitorum supercialis, exor pollicis longus,
pronator quadratus, pronator teres, and supinator
(Doyle 2003). The distal epiphysis of the radius
appears near the age of 1 year, while the proximal
epiphysis emerges around 4 to 6 years old.
(Silberstein et al. 1982, Ogden et al. 1981).
Approximately 70-80 % of longitudinal growth
of the radius occurs at the distal epiphysis (Ogden
et al. 1981). Physeal closure of the radius is vari-
able and gender dependent; the proximal physis
closes rst near 1415 years of age, followed by
the distal physis at around 1518 years old (Kraus
et al. 2011, Ogden 1982).
The ulna is prism shaped proximally and
becomes more cylindrical distally (Milch 1944).
The distal styloid process and proximal coronoid
process are important landmarks of the ulna.
1071
The syloid is dorsal and the coronoid is volar,
oriented almost 180 from each other (Milch
1944). Fourteen muscles attach to the ulna: abduc-
tor pollicis longus, anconeus, biceps, brachialis,
extensor carpi ulnaris, extensor indicis proprius,
extensor pollicis longus, exor carpi ulnaris,
exor digitorum profundus, exor digitorum
supercialis, pronator teres, pronator quadratus,
supinator, and triceps (Doyle 2003). The epiphy-
sis of the distal ulna appears in conjunction with
the radius at about 4 to 6 years old. The proximal
olecranon apophysis ossies near 9 to 10 years old
(Silberstein et al. 1982 - [the one about the ulna]).
Similar to the radius, 70-80 % of longitudinal
growth of the ulna occurs at the distal epiphysis
(Ogden et al. 1981). Physeal closure of the ulna
occurs in the same stages as the radius (Ogden
1982).
The forearm contains major vessels and
nerves in continuation from the proximal upper
extremity. The vascular composition of the
forearm is supplied by contributions from the
radial and ulnar arteries. The radial artery lies
supercial to the pronator teres on the exor
digitorum supercialis and exor pollicis longus,
lateral to the exor carpi radialis (Standring
et al. 2008). It gives off branches to the radial
recurrent artery and several muscular branches.
The ulnar artery is deep to the radial head of the
pronator teres, on the exor digitorum profundus,
and lateral to the ulnar nerve. It gives rise to the
anterior ulnar recurrent artery, posterior ulnar
recurrent artery, common interosseous artery,
and muscular branches. Important nerves include
the radial, ulnar, and median nerves, as well as the
anterior and posterior interosseous nerves, and the
lateral and medial cutaneous nerves of the forearm
(Standring et al. 2008).
The radius and ulna comprise a ring of bone in
the forearm that is reinforced proximally by the
articulations with the distal humerus and the liga-
mentous connections of the proximal radioulnar
joint and distally through the articulations with the
carpus and the distal radioulnar joint complex.
The interosseous membrane runs obliquely
between the radius and ulna providing stability
to the ring but permitting rotation of the radius
around the ulna. The normal forearm rotates
1072
through an arc of 160 , with approximately 80 of
pronation and 80 of supination. It is important to
understand that forearm rotation is best measured
at the level of the distal radius and ulna, not by
assessing the position of the hand. As much as 40
of rotation may occur through the radiocarpal
joint, making forearm rotational measurements
less precise. This carpal motion, however, may
improve the function of some patients by compen-
sating for loss of forearm rotation that resulted
from an injury or congenital difference.
Pathoanatomy
When falling onto an outstretched hand, the most
common mechanism of both-bone forearm frac-
tures, loading force is transmitted to the radius,
which typically fails rst, followed by failure of
the ulna if the force magnitude is great enough
(Treadwell et al. 1984). The rotational position of
the forearm upon impact (McGinley et al. 2003)
and the amount of energy applied dictate the frac-
ture locations. If the forearm is loaded while in
supination, the radial fracture occurs proximal to
the ulna fracture while the reverse is true if the
forearm is pronated on impact. The fractures
occur at the same level if the forearm is neutrally
rotated when the hand strikes the ground. Addi-
tionally, a large direct force, such as being struck
with a baseball bat, may cause fractures that occur
at the same level. Approximately 75 % of frac-
tures occur in the distal third of the forearm, 15 %
in the middle third, and 5 % in the proximal third
(Thomas et al. 1975).
Single-bone forearm fractures, i.e., isolated
fractures of either the radius or ulnar shaft, may
occur as well. These are most commonly the result
of a direct blow to either bone or lower-energy
mechanisms in younger children. An isolated
fracture of the ulnar shaft, however, must raise
suspicion for an ipsilateral subluxation or disloca-
tion of the radial head (Monteggia fracture).
Similarly, an isolated fracture of the radial shaft
demands careful assessment of the distal
radioulnar joint for dislocation of the distal ulna
(Galeazzi fracture) or a displaced fracture through
the ulnar physis (Galeazzi equivalent).
J.J. Whitaker et al.
Assessment
Signs and Symptoms
A child with a forearm fracture typically presents
after a traumatic injury complaining of pain,
swelling, and, if the fracture is displaced, a visible
deformity of the involved extremity. Occasion-
ally, pain with bearing weight on the involved
extremity and painful range of motion, particu-
larly pronation and supination (Soong and Rocke
1990), are the chief complaints, especially with
nondisplaced fractures or incomplete fractures
with minimal swelling. While most forearm frac-
tures present as isolated injuries, it is critical that a
careful history is taken and a primary physical
assessment including vital signs and the cardio-
vascular parameters is performed for patients who
sustain these injuries from higher-energy mecha-
nisms, such as a fall from a sizable height or a
motor vehicle accident. This will allow identica-
tion of other potentially more serious injuries of
the head, thorax, and abdomen.
Once a fracture is suspected, the extremity is
inspected for areas of swelling, open wounds with
exposed bone, and other soft tissue ndings such
as bleeding, abrasions, and tissue loss. The radius
and ulna are palpated along their lengths, and the
ipsilateral elbow and wrist joints are assessed for
swelling, tenderness, and painful or limited range
of motion. The soft tissue compartments are pal-
pated to identify extreme tautness, which may
indicate an impending compartment syndrome.
Painful passive stretch of the ngers is also sug-
gestive of an impending compartment syndrome
but may also be seen in those with severe pain
from the fractures, as is common in fractures with
displacement.
A complete neurovascular exam includes
motor and sensory testing of the ulnar, radial,
and median nerves. This may be done easily
with cooperative patients who are older than 56
years of age in a systematic way similar to adults.
However, it may be difcult to adequately assess
younger children, individuals with intellectual
impairments, and patients who are experiencing
severe anxiety or pain. Observing these types of
48 Forearm Fractures
patients or engaging them in simple tasks, such as
grabbing a pen, may provide clues to nerve func-
tion. Another method suggested by some is to
play the familiar childs game of rock-paper-
scissors with the patient, a technique that may
permit active assessment of the radial, ulnar, and
median nerve motor function (Davidson 2003).
Imaging
High-quality anteroposterior (AP) and lateral
radiographs of the entire forearm, including the
elbow and wrist, are necessary to evaluate poten-
tial forearm fractures effectively. If the child is
able to tolerate gentle positioning based on his or
her degree of discomfort, these radiographs are
ideally taken with the elbow and wrist extended
and the forearm neutrally rotated. Grossly unsta-
ble forearm fractures should be protected in a
long-arm splint prior to transport to the radiology
suite, for the childs comfort and to reduce the risk
of further soft tissue and neurovascular injury.
Advanced imaging of forearm fractures is indi-
cated in rare cases. Computed tomography
(CT) may be useful to assess intra-articular exten-
sion of fracture lines and for assessing the extent
of bone cysts or other bony defects when a path-
ologic fracture is suspected. Magnetic resonance
imaging (MRI), however, is the best advanced
imaging modality for assessing potential patho-
logic fractures that may have occurred secondary
to tumors or infectious processes.
Associated Injuries
Most forearm fractures are isolated injuries
resulting from low- and intermediate-energy trau-
matic events. In these patients, the most important
associated ndings not to miss include open frac-
tures, nerve or vascular injuries, and impending
compartment syndromes in the affected extremity.
Additionally, careful clinical evaluation of the
entire extremity and thorough scrutiny of the fore-
arm radiographs, including the elbow and wrist in
all cases, will prevent the surgeon from missing
Monteggia fracture dislocations, Galeazzi
1073
fractures, and their equivalents unique to pediatric
patients (Letts et al. 1985; Landfried et al. 1991).
A simultaneous ipsilateral fracture of the forearm
and a supracondylar fracture of the humerus has
been reported with a prevalence of 5.3 %.
Typically these are a result of high-energy mech-
anisms and are associated with nerve injuries
and/or open fractures (Roposch et al. 2001).
For those patients who present with forearm frac-
tures from high-energy mechanisms, such as falls
from a signicant height or motor vehicle trauma,
emergency department evaluation must consider the
possibility of more serious associated injuries.
Establishing the ABCs rst is paramount for these
patients. Head trauma, thoracoabdominal injuries,
spine fractures, and other life-threatening conditions
must rst be ruled out before the fractured extremity
is fully assessed. Provisional realignment and
splinting of an obviously injured forearm provides
pain relief and protects the extremity while resusci-
tation and further evaluation are completed. A sec-
ondary orthopedic survey may then be performed,
with particular attention paid to the entire injured
extremity to identify ipsilateral fractures of the shoul-
der, humerus, elbow, wrist, and hand.
Classification
No specic classication exists for pediatric fore-
arm fractures. Forearm fractures are typically
described based on which bones are fractured
(both bones or single bone), the level of the fracture
within the forearm (distal, middle, or proximal
third), and the fracture pattern of each bone (plastic
deformation, greenstick, complete). Fracture align-
ment is determined by measuring the degrees of
angulation in the anteroposterior (AP) and lateral
planes, the amount of translation of the fracture
fragments relative to each other, and the rotational
alignment of the fracture fragments.
Fracture Patterns
Plastic Deformation
Plastic deformation, when the bone is bent
but not broken, occurs when the load placed on
1074
Fig. 1 A nine-year-old female sustained a Monteggia
fracture dislocation (plastic deformation of the ulna and
radial head dislocation, AP-a and lateral-b). Reduction of
Fig. 2 A seven-year-old male fell from a standing height
and sustained a greenstick both-bone forearm fracture
(AP-a and lateral-b). The remaining cortex is in continuity
but is angulated. A torsional force is typically involved
the bone exceeds its elastic limits, but not its
ultimate strength. No obvious fracture line or
cortical discontinuity is seen, but multiple
microfractures along the length of the bow are
present (Sanders and Heckman 1984a). On
radiographs, an abnormal curve or narrowing of
the interosseous space may be seen. The ulna is
the bone that more commonly bows in the
forearm after trauma. Ulnar bowing may
occur in isolation, but often it is associated
with a radial head dislocation (chapter
Monteggia Fracture Dislocation Fig. 1) or The intact cortex helps to maintain length of the
radial shaft fracture.
J.J. Whitaker et al.
the radial head was achieved by correction of the ulnar
bowing using an osteotomy and plate xation (AP-c and
lateral-d)
with the mechanism of injury; therefore, greenstick frac-
tures usually have a rotational component associated with
the angulation. The patient underwent closed reduction and
application of a sugar-tong splint (AP-c and lateral-d)
Greenstick Fracture
In greenstick fractures, one to three cortices may
be disrupted on radiographs. The remaining cor-
tex is in continuity but is angulated (Fig. 2). A
torsional force is typically involved with the
mechanism of injury; therefore, displaced
greenstick fractures nearly always have a rota-
tional component. Apex dorsal fractures are
caused by hyperpronation, and apex volar frac-
tures are due to hypersupination during the injury.
fracture and facilitates reduction.
48 Forearm Fractures
Fig. 3 A twelve-year-old
male fell while
skateboarding and
sustained a distal third both-
bone forearm fracture
which was completely
translated and shortened in
bayonet apposition (AP-a
and lateral-b)
Complete Fractures
Complete fractures occur when all cortical contact
is lost between two fragments of bone. These
fractures are further described by the fracture pat-
tern. Transverse and short oblique
non-comminuted fractures of the radius and ulna
are most common in pediatric patients. Commi-
nuted and segmental fractures are relatively
uncommon, as they result from high-energy
mechanisms. The most difcult fractures to
reduce and cast in acceptable alignment, i.e., the
most unstable fracture patterns, are both-bone
complete forearm fractures. The degree of dis-
placement of complete fractures reects the sever-
ity of the injury and the amount of soft tissue
disruption that occurs. Complete fractures may
remain reasonably aligned if the periosteum and
muscle attachments are not completely disrupted
and stripped from the bones at the sites of fracture.
Fracture Displacement: Angulation,
Translation, and Malrotation
The displacement of forearm fractures is
described based on radiographic measurements
of the AP and lateral radiographic projections.
1075
Angulation is determined by measuring the
angle created by the fracture fragments at the
apex of the deformity and is assessed separately
for each bone and in orthogonal planes. Transla-
tion quanties the amount of cortical or bony
contact between the fragments. Fragments may
have a percentage of bone contact, or they may
be completely translated relative to one another
with no end-on cortex-to-cortex contact. Addi-
tionally, fragments with no contact that then rest
overlapped one on top of another are said to be in
bayonet apposition, a reference to the shape of
the swordlike weapon (Fig. 3). The degree of
shortening of each bone may then be determined
by measuring the length of cortical overlap.
Malrotation is more difcult to assess. Fracture
fragments that are not malrotated relative to each
other appear on radiographs to have similar diam-
eters in both the AP and lateral projections. A
more reliable way to assess rotation is to compare
the relative positions of the bicipital tuberosity of
the radius proximally and the radial styloid dis-
tally as viewed on an AP radiograph with the
forearm neutrally rotated. In the normal radius,
the bicipital tuberosity and the radial styloid
point 180 from each other. The degree of
malrotation can be estimated based on the radio-
graphic appearance of these landmarks.
1076
Outcome Assessment
Many studies dene outcomes based on radio-
graphic alignment of the fracture at healing and by
the clinical outcome, typically determined by mea-
suring forearm range of motion and the occurrence
of complications. In order to determine more con-
sistently the effectiveness of forearm fracture treat-
ment methods, Flynn et al. proposed the Childrens
Hospital of Philadelphia Forearm Fixation Outcome
Classication. In this classication, the results of
xation may be labeled as being good, fair, or
poor. A good outcome is classied as being one
where the child has full range of motion (<10 loss
of supination and/or pronation) and no postoperative
complications. A fair outcome is dened as the
child having minimal loss of range of motion (<30
supination and/or pronation) and/or minor, resolving
postoperative complications. A poor outcome
occurs with loss of range of motion (>30 supina-
tion and/or pronation) and/or major postoperative
complications, such as infection, compartment syn-
drome, or delayed union (Flynn et al. 2010).
Another similar outcome assessment was
described by Price et al., with categories being
labeled as excellent, good, fair, or poor.
To be classied as excellent, the patient must
have no complaints and a loss of range of motion
<10 . Good results include those having mild
complaints with vigorous activity and loss of motion
between 11 and 30 . A result is recorded as fair if
complaints occur with daily activities and loss of
motion is between 30 and 90 . If complaints are
more severe or there is greater loss of motion, the
outcome is considered poor (Price et al. 1990).
Nonoperative Treatment
Most pediatric forearm fractures are best treated
with closed reduction and long-arm cast immobi-
lization (Zionts et al. 2005; Jones and Weiner
1999). In one study of over 730 forearm fractures,
of which 300 were displaced signicantly, only
22 failed initial closed reduction and casting
(Jones and Weiner 1999). The trend toward oper-
ative xation over the last 15 years has helped to
dene operative indications. Surgical treatment is
J.J. Whitaker et al.
indicated as the primary treatment for those patients
with open fractures, vascular injuries, a oating
elbow, and severe soft tissue complications such
as a compartment syndrome or tissue loss. Only
after an unsuccessful attempt at closed fracture man-
agement is surgery indicated for the remainder of
patients, with few exceptions. Certain fractures
have a high risk of closed treatment failure including
displaced proximal third radius fractures, displaced
fractures in children over 10 years of age, and
mid-diaphyseal fractures with initial ulnar angulation
greater than 15 (Bowman et al. 2011). An attempt
should be made to treat children and adolescents with
forearm fractures nonsurgically at the outset, even if
they fall into these risk of failure categories.
Nonoperative Management of Forearm Fractures
Indications Contraindications
An attempt should be made to treat Vascular injury
children and adolescents with Open fracture
forearm fractures nonsurgically Compartment
syndrome
Severe soft tissue
injury
Floating elbow
Emergency Department Management
Closed reduction and immobilization of forearm
fractures is the preferred treatment option. After ade-
quate patient assessment and review of the radio-
graphs, the surgeon must dene the degree of
displacement. Children with nondisplaced or mini-
mally displaced fractures are placed into a long-arm
cast or a sugar-tong plaster splint with the elbow
exed 90 and neutral rotation of the forearm. Seda-
tion is rarely needed and patients are discharged from
the emergency department after fracture care instruc-
tions are given to the child and the family.
Analgesia/Sedation
Intravenous ketamine provides excellent sedation
and analgesia enabling a closed reduction. This
method induces a trancelike state that combines
sedation, analgesia, and amnesia with little cardio-
vascular depression (McCarty et al. 1999).
48 Forearm Fractures
Fig. 4 Plastic deformation of the forearm may be reduced
by applying gentle pressure proximal and distal to the apex
of the bow while the forearm rests on a bolster or bump that
Intravenous conscious sedation methods are effective
for closed fracture management but demand an expe-
rienced sedation team and safety protocols. Compli-
cations, such as an adverse reaction, can occur with
sedation. Therefore it is mandatory the emergency
department staff be trained to properly dose medica-
tions based on the childs weight, the use of pediatric
cardiovascular monitoring, and, most importantly,
resuscitation techniques (Cameron et al. 2000).
Other methods of analgesia for upper extremity frac-
ture management include alternative intravenous
sedation drug regimens, such as fentanyl-midazolam,
and regional anesthetic techniques, such as a Bier
block or axillary block. A hematoma block may be
plausible for distal forearm fractures; however, this
technique is less effective for proximal fractures.
Also, children may be anxious and/or uncooperative,
making it a challenge to inject the fracture site. One
report utilized inhaled nitrous oxide to obtain
sedation, and then a hematoma block was performed
allowing for fracture reduction (Hennrikus
et al. 1995).
Fracture Manipulation
Plastic Deformation
Fractures that are plastically deformed with an
unacceptable degree of angulation can typically
be managed in the emergency department. After
1077
acts as the fulcrum for deformity correction (From Sanders
and Heckman 1984b)
adequate sedation and analgesia has been
provided, the fractured forearm may be gently
manipulated straight by three-point bending
forces centered at the apex of the deformity. For
small children, the physician places his or her
thumb at the apex and applies steady pressure at
the ends of the bone. Alternatively, a rolled towel
or cushioned bump is placed on the stretcher, and
the apex of the deformity is placed on top of it,
allowing the physician to apply downward pres-
sure at the bone ends and gently rock the fracture
around the fulcrum over a period of 35 min to
achieve correction (Fig. 4). The goal is not to
complete the fracture but instead to restore align-
ment, ideally achieving anatomic alignment but
generally no more than 1520 of residual angu-
lation (Vorlat and De Boeck 2003). After reduc-
tion, a three-point molded long-arm cast is
applied. If the fracture does not reduce, operative
reduction under anesthesia, either with the above
technique or by percutaneous drill osteoclasis at
the apex of the deformity, may be indicated
(Blackburn et al. 1984).
Greenstick Fractures
Similar to plastic deformations, greenstick fractures
are usually treated with a closed reduction and
well-molded cast placed under conscious sedation.
1078
Fig. 5 Rotational
displacement of the radius is
caused by muscle forces
acting proximal and distal
to the fracture: (a) proximal
fractures and (b) distal
fractures (Adapted from
Cruess, R.L. Orthop Clin
North Am 1973; 4:969)
In order to reduce these fractures, the force oppo-
site to the mechanism of injury is applied. As
greenstick fractures result predominantly from
torsional forces that occur as the arm is axially
loaded, reduction is often easily achieved by
merely applying gentle traction and rotating the
distal forearm and thumb toward the apex of the
deformity (Noonan and Price 1998), often
referred to as the rule of thumbs. Therefore,
apex volar fractures are reduced by pronating the
forearm while apex dorsal fractures are reduced
by forearm supination. A topic of controversy
when treating these fractures is whether or not
the fracture should be completed. Advocates for
completing the fracture argue that this will help
prevent re-angulation or diminish the risk of
refracture (Rang 1983). In contrast, many believe
that the intact cortex helps maintain the alignment
of the fracture after reduction (Alpar et al. 1981).
After reduction, the arm is immobilized in a well-
molded long-arm cast in neutral rotation with the
elbow exed 90 .
J.J. Whitaker et al.
Complete Fractures
After adequate sedation and analgesia is achieved,
longitudinal traction is applied to the forearm.
This may be achieved by placing the ngers of
the affected extremity in nger traps and
suspending them from an intravenous pole. The
weight of the arm provides a traction force
allowing the fracture fragments to realign as mus-
cle forces are overcome. Additional traction can
be achieved by applying small weights to a cuff
wrapped around the upper arm. Without cortical
contact, the bone fragments are susceptible to
displacement by muscle forces acting on the
bone. Alternatively, an assistant may apply longi-
tudinal traction across the fracture by grasping the
upper arm proximally and the hand distally while
the physician manipulates the bone fragments.
Muscle forces inuence rotation of the fragments
and must be taken into account when reducing
complete fractures to avoid malrotation (Fig. 5).
Due to the forces of the biceps and supinator,
48 Forearm Fractures
Fig. 6 Finger traps may be
used to provide traction
while the long-arm cast is
applied. The ideal cast has
an interosseous mold
resulting in an oval shape in
the mid-forearm combined
with at ulnar and posterior
humeral borders
complete proximal radius fractures are best
immobilized in supination. In the middle third of
the forearm, the rotational forces are relatively
balanced so neutral rotation is appropriate. For
fractures of the distal third of the forearm, the
pronator quadratus is acting on the distal frag-
ment. Therefore, these fractures are best reduced
and held in some pronation. The forearm should
not be placed in extreme positions of supination or
pronation, as signicant stiffness can occur after
fracture healing. A xed supination deformity
from a contracture is particularly debilitating and
a challenge to manage.
Immobilization After Reduction
Careful application of a well-molded circumfer-
ential cast after reduction is critical for mainte-
nance of alignment and to prevent complications.
If there is a concern for severe swelling, the cast is
bivalved and overwrapped with an elastic ban-
dage prior to discharge or the child is admitted
and observed overnight. Alternatively, the child
1079
may initially be placed into a non-circumferential
splint, such as a sugar-tong splint (Younger
et al. 1997), and casted upon outpatient follow-
up. The ideal long-arm cast applied to treat fore-
arm fractures must have a three-point mold about
the fracture sites and be oval in shape at the middle
of the forearm with an indent between the radius
and ulna to create an interosseous mold. The
upper arm part of the cast should be tapered just
above the supracondylar area of the humerus, and
the ulnar and posterior humeral borders should be
essentially at to limit the distal migration or
shifting in the cast (Fig. 6).
Acceptable Reduction
After cast application, high-quality AP and lateral
radiographs are taken and analyzed. Acceptable
reduction parameters vary based on the chronolog-
ical age and, more importantly, the estimated years
of growth remaining, the location of the fracture,
and the postreduction alignment. The ideal reduc-
tion parameters that reliably yield satisfactory
1080
clinical results are controversial. Even small degrees
of residual angulation have been correlated with
some loss of forearm rotation (Alpar et al. 1981;
Noonan and Price 1998; Kasten et al. 2003). In
addition to bony malunion, soft tissue brosis, espe-
cially about the interosseous membrane, may lead to
limited forearm motion after fracture healing
(Nilsson and Obrant 1977). Angulation of the radius,
especially when the radial bow has been lost,
correlates with loss of forearm rotation while ulnar
angulation has a greater inuence on the cosmetic or
aesthetic appearance of the forearm (Dumont
et al. 2002). For children under the age of 8 years,
up to 20 of diaphyseal angulation may remodel
while angulation of as little as 10 may not in those
older than 10 years of age (Jones and Weiner 1999).
To confound the issue, residual radiographic angu-
lation does not always have a direct correlation with
functional outcomes and patient satisfaction (Price
et al. 1990). Shortening is well tolerated in patients
younger than 10 years of age, with up to one centi-
meter being acceptable after failed closed reduction
(Do et al. 2003). Even fractures in bayonet apposi-
tion treated with casting and no analgesia, sedation,
or a formal reduction can result in excellent clinical
outcomes (Crawford et al. 2012).
Children Younger than 10 Years of Age
The recommended acceptable forearm reduction
parameters for children younger than 10 years of
age include residual angulation of the radius or ulna
measuring 20 or less. There are two exceptions:
radius fractures in the proximal third and radius
fractures with apex ulnar angulation. Proximal
radius fractures with angulation of 10 or more and
radius fractures with any apex ulnar angulation are at
risk of rotation loss even in this young age group.
Complete translation, bayonet apposition with short-
ening of 1 cm or less, and malrotation less than 30
are other acceptable parameters of reduction.
Children 10 Years of Age and Older
Children who are 10 years of age and older have
less capacity for fracture remodeling. Greater than
J.J. Whitaker et al.
10 of angulation of either bone in any plane,
greater than 50 % translation, shortening or bay-
onet apposition, and greater than 30 of
malrotation are unacceptable reduction parame-
ters. Individuals with less than 2 years of growth
remaining have minimal remodeling capability;
therefore, near-anatomic alignment must be
obtained for acceptable reduction.
Complications of Nonoperative
Treatment
Stiffness
The most common complication to occur after a
forearm shaft fracture is signicant forearm stiff-
ness, with a decrease in pronation more common
than loss of supination (Hgstrm et al. 1976;
Holdsworth and Sloan 1982). This most commonly
occurs in patients with malunited fractures but may
occur even after anatomic healing. Avoiding extreme
positions of either pronation or supination in a cast
and permitting motion as soon as fracture healing
occurs are some ways to limit functional loss of
rotation after cast treatment of forearm fractures.
Malunion
Malalignment after healing occurs in 1025 % of
patients (Davis and Green 1976). This complica-
tion is cast related and mostly due to poor molding
after acceptable reduction. Occurrence of this
complication can often be remedied by repeating
the closed reduction and casting (Davis and Green
1976; Voto et al. 1990). A small percentage of
children will experience a malunion, but the
deformity is mostly cosmetic and may or may
not cause loss of functional forearm motion
(Daruwalla 1979). Close follow-up with serial
AP and lateral radiographs within the rst
2 weeks postreduction is critical to prevent this
complication. Cast wedging may be used to
improve unacceptable angulation if it is noticed
in a timely manner. Osteotomies of the radius and
ulna are salvage options for those patients with
healed malunions (Price and Knapp 2006).
48 Forearm Fractures
Refracture
Diaphyseal forearm fractures are the number one
location of refracture in children, with most occur-
ring at the original site (Landin 1997). Up to 8 %
of patients will experience a refracture. Children
are at highest risk for this complication up to
1 year following union of the initial fracture;
refractures are much less common during splint
wear (Chung and Spilson 2001). Most refractures
occur in the proximal and distal third of the fore-
arm (Baitner et al. 2007).
Uncommon Complications
Delayed union and nonunion are very rare com-
plications. Nonunion occurs most commonly in
the ulna in patients between 13 and 16 years old
(Adamczyk and Riley 2005). Synostosis,
although rare, may result in complete loss of fore-
arm rotation. Compartment syndrome is rare after
closed reduction and cast treatment of forearm
fractures but must be suspected if a child is not
comfortable 34 hrs postreduction (Crawford
1991). A non-circumferential splint may be
applied or the cast may be bivalved. The treatment
for compartment syndrome is emergent
fasciotomies. Finally, cast complications may
occur. Skin breakdown, skin burns from hot dip-
ping water, and cuts and/or burns from the cast
saw occur but are easily preventable if proper
techniques are utilized (Halanski and Noonan
2008). Refer to Table 1 for tips on safe berglass
cast application.
Table 1 Pearls for safe and effective fiberglass cast
application
Avoid excessive padding to enhance molding and reduce
the risk of loss of fracture reduction
Maintain joints in the same position while applying cast
to avoid pressure points at joint creases
Use cool dipping water to avoid excessive heat
generation and potential burns under the cast
Use stretch-relaxation of the berglass to avoid excessive
cast constriction after cast curing
Do not trim or bivalve berglass cast until it has cooled to
avoid cast saw burns of the skin
1081
Part B: Operative Treatment
Indications
Fractures of the forearm are typically treated suc-
cessfully with closed reduction and cast immobili-
zation. Surgical treatment is indicated for open
fractures (Greenbaum et al. 2001; Luhmann
et al. 2004), severe soft tissue injury or compartment
syndrome, vascular injuries, and oating elbow inju-
ries (ipsilateral fractures of the distal humerus and
forearm) (Ring et al. 2001). The inability to obtain
acceptable alignment via a closed reduction neces-
sitates surgical reduction and xation. Also, unstable
fractures that have lost alignment at follow-up may
require repeat closed reduction or operative xation.
Surgical Procedures
Options for surgical treatment include closed or
open reduction and intramedullary nailing, open
reduction and internal xation utilizing plates and
screws, and in rare circumstances external xa-
tion. Intramedullary nailing has become the stan-
dard operative treatment method for skeletally
immature patients and has demonstrated good
results (Lascombes et al. 1990; Till et al. 2000;
Flynn et al. 2010; Martus et al. 2013).
Intramedullary Nailing
Preoperative Planning
Intramedullary nailing can be done percutaneously
or with minimal surgical exposure using exible
nails (1.52.5 mm diameter) or smooth wires/pins
(0.062 or 5/64th inches diameter). The nail diame-
ter should ll approximately two thirds of the canal
isthmus. The implant is advanced across the frac-
ture site via a closed or limited open reduction.
Dual bone xation is most common, but occasion-
ally single-bone xation provides adequate stabili-
zation of the fracture reduction.
Positioning
The patient is positioned supine and general anes-
thesia is provided. A radiolucent hand table is
1082
Table 2 Preoperative planning for intramedullary nailing
of radius and ulna shaft fractures
OR table Standard OR table with radiolucent
hand table on operative side
Position Supine with the patient located close to
the edge of the OR table to provide
sufcient mobilization of the operative
extremity and unobstructed
uoroscopic visualization
Fluoroscopy Positioned at the distal end of the hand
table
Tourniquet Non-sterile; placed on upper arm close
to the axilla
Draping Ensure the elbow, forearm, and hand are
accessible
Equipment (a) Flexible nails (1.52.5 mm diameter)
or smooth wires (0.062 or 5/64th size)
(b) Drill or awl
(c) T-handled chucks
(d) Nail bender
(e) Small fragment set if open reduction
is needed
used to support the operative extremity. A
non-sterile pneumatic tourniquet is placed on the
upper arm, ensuring access to the elbow. The
extremity is then draped and prepped in sterile
fashion including the elbow, forearm, and hand.
Fluoroscopy is stationed at the distal end of the
hand table. Prior to draping, the adequacy of
imaging should be assessed (Table 2).
Approach
Antegrade and retrograde intramedullary nailing
techniques have been described for the ulna, while
retrograde nailing is standard for the radius. The
order of bone xation is variable between sur-
geons and may be determined based on which
bone is the most difcult to reduce and most
unstable. The ulna is the rst bone to be nailed,
as it is classically easier to reduce.
Technique
Antegrade nailing is utilized for the ulna, with
insertion across the olecranon apophysis. The
antegrade insertion site is directly posterior to
the olecranon, which provides a direct path to
the canal. Alternately, insertion can be done
through the metaphysis just distal to the apophysis
J.J. Whitaker et al.
on the lateral aspect of the ulna (anconeus starting
point). Signicant complications have not been
reported with either entry site.
The tourniquet can be kept deated during
xation of the ulna if an open reduction is not
required. A small incision is made over the tip of
the olecranon process. Fluoroscopic guidance is
used to drill a starting hole across the apophysis
into the intramedullary canal. A exible nail is
inserted using a T-handled chuck. Alternatively,
if a smooth wire/Steinmann pin is used, the sharp
end can be used to drill the starting point and then
advanced. The nail or wire is positioned just shy
of the fracture site; traction is applied and a closed
reduction obtained. The implant is moved past the
fracture site until the far tip terminates in the distal
metaphysis of the ulna. Sufcient length should
be allotted for the implant to be tamped into nal
position, ensuring it does not violate the distal
physis of the ulna. This is done by advancing the
nail to its nal position and then withdrawing it
12 cm. The nail is cut to the appropriate length
and gently readvanced to its nal position. The cut
proximal end should terminate beneath the skin.
Some surgeons may elect to leave the implant
percutaneous for early removal in the ofce set-
ting, but this can be challenging for younger
patients (Table 3).
Attention is then directed to the radius, which
is xed in a retrograde fashion. If the tourniquet
has yet to be inated, the limb is exsanguinated
and the tourniquet is inated. The dorsal physeal
sparing entry site is located at the proximal aspect
of Listers tubercle. This location is approached
utilizing a 12 cm longitudinal incision approxi-
mately 1 cm proximal to the distal radial physis in
the midline of the metaphysis between the third
and fourth dorsal extensor compartments. Fluo-
roscopy may be used to conrm proper placement
of the incision proximal to the physis. Dissection
is continued through the retinaculum, and the
interval between the third and fourth dorsal exten-
sor compartments is used to expose the distal
radius at Listers tubercle. Transposition of the
extensor pollicis longus tendon may be necessary
for a safe starting point. Alternatively, a lateral
entry point may be used via the oor of the rst
dorsal extensor compartment. An awl or drill is
48 Forearm Fractures
Table 3 Surgical steps for intramedullary nailing of the
ulna
Flex the elbow to 90
Fluoroscopy used to conrm location of apophyseal
starting point in line with intramedullary canal of the
ulna
1 cm longitudinal incision over olecranon followed by
blunt dissection down to bone
2.7 or 3.2 mm drill or awl used to penetrate the cortex
Insert nail using T-handled chuck and advance to fracture
site
Combine traction and rotation, also anterior/posterior
compression if needed, to obtain reduction
Advance nail past fracture to appropriate length in distal
ulna
Conrm ulnar styloid and coronoid process are
180 from each other on full-length lateral uoroscopic
image
Withdraw the nail 12 cm, cut the nail leaving 12 cm
proud, and then impact the nail to nal position
Close the skin with absorbable sutures and apply Steri-
Strips
used to create a unicortical entry hole after uo-
roscopic verication of the planned insertion posi-
tion. The hole is enlarged at a 30 angle by
directing the drill or awl proximal obliquely
across the radius. Once the starting point has
been created, the exible nail or smooth wire is
pre-contoured to a gentle C shape to accommo-
date the radial bow. Direct visualization of the
bone surface is needed to prevent tendon injury
during insertion and advancement. The implant is
positioned just shy of the fracture site. A closed
reduction is performed, and the nail is moved
across the fracture ensuring the contour of the
nail is aligned with the bow of the radius. The
nail should be advanced to its nal position short
of the radial neck physis. Subsequently, the nail is
withdrawn 12 cm, cut to its proper length and
tamped into nal position. It is important to verify
the cut end is not abrading the extensor tendons.
Once the tendons are visualized as being away
from the cut nail, the incision is closed with the
implant tip beneath the skin (Table 4). A nail or
wire placed using a lateral starting point can be
left percutaneous for ofce removal. Figure 7
exemplies a standard both-bone forearm fracture
treated with exible intramedullary nails, and
1083
Table 4 Surgical steps for intramedullary nailing of the
radius
Pre-contour the nail into a C shape
Inate tourniquet if needed
Palpate Listers tubercle on the distal radius
Fluoroscopy used to identify dorsal starting point 1 cm
proximal to physis at the base of Listers tubercle
12 cm longitudinal incision to expose radius
between the third and fourth dorsal extensor
compartments
Take care to protect the extensor tendons and be aware of
the supercial radial sensory nerve
2.7 or 3.2 mm Drill or Awl used to penetrate the dorsal
radius cortex perpendicular to the bone
Drop your hand (aiming drill/awl more proximal) to
enlarge hole obliquely at a 30 angle
Insert the nail using T-handled chuck; point the tip
directly into the hole while ensuring the extensor tendons
are not interposed
Within the canal, rotate the nail 180 to align the tip
parallel to the shaft of the radius
Advance the nail to the fracture site and then obtain
reduction
Advance the nail past the fracture, rotating the nail as
needed to optimize radial bow
Position the end of the nail just distal to the radial neck
physis
Conrm radial styloid and bicipital tuberosity are 180
from each other on full-length AP uoroscopic image
Withdraw the nail 12 cm, cut the nail leaving 12 cm
proud, and then impact the nail to nal position
Ensure the nail tip protrudes beyond extensor tendons
into the subcutaneous tissue
Close the skin with absorbable sutures and apply
Steri-Strips
Fig. 8 demonstrates a similar fracture treated
with K-wire xation using a radial styloid starting
point.
Single-Bone Fixation
Surgical treatment of both-bone forearm fractures
using singular bone xation with plate and screws
or an intramedullary device has been reported
with good outcomes (Flynn and Waters 1996;
Kirkos et al. 2000; Bhaskar and Roberts 2001;
Myers et al. 2004). Either the ulna or radius can
be stabilized. The relatively straight
intramedullary canal of the ulna allows for easier
xation. After single-bone xation of the ulna,
1084 J.J. Whitaker et al.

Fig. 7 A fteen-year-old male sustained a fracture of his room. The reduction was unsuccessful, and he subse-
forearm while playing football (AP-a and lateral-b). He quently underwent exible intramedullary nail xation
underwent closed reduction and casting in the emergency (AP-c and lateral -d)

Fig. 8 A ten-year-old female sustained a displaced subsequently underwent intramedullary nailing of both
midshaft both-bone fracture when she fell off her bike bones using smooth K-wires, which were inserted through
(AP-a and lateral-b). One week after closed reduction, the radial styloid and olecranon apophysis (AP-e and
the fracture had lost alignment (AP-c and lateral-d). She lateral-f). The radius required a limited open reduction

adequate reduction is conrmed for both bones, Pitfalls and Prevention

and if the radius is stable, then it may be left Conversion to an open reduction of either the ulna
without an implant. Figure 9 demonstrates a or radius should be done to prevent risk of com-
both-bone forearm fracture treated with singular partment syndrome if repeated closed manipula-
xation of the ulna. tions fail. Approximately 510 min should be
48 Forearm Fractures
Fig. 9 A thirteen-year-old
sustained an unstable
midshaft both-bone forearm
fracture (AP-a and lateral-b)
while skateboarding. After
a failed attempt at closed
reduction and casting, he
was treated in the operating
room with closed reduction
and single-bone xation of
the ulna using a smooth
K-wire. The radius reduced
anatomically and was stable
after ulnar xation,
therefore xation of the
radius was unnecessary
allotted for closed manipulation of each bone
before open reduction is recommended. One
third of closed forearm fractures treated with
intramedullary nailing require an incision at the
fracture site to achieve a reduction and facilitate
nail passage (Flynn et al. 2010; Martus
et al. 2013). Open reduction of the ulna is
performed utilizing a 12 cm incision made on
the subcutaneous border of the ulna at the fracture
site. The radius is opened in a similar manner
using a small volar incision for distal and
middle third fractures. Proximal third radius frac-
tures can be approached via a volar incision,
although some surgeons prefer a dorsal approach.
After exposing the bone fragments, each end is
grasped with bone-holding forceps and reduced
manually. An assistant may be needed to provide
simultaneous traction and/or rotation. Alterna-
tively, a freer elevator can be placed in the fracture
and utilized to lever the fragments to permit
reduction.
Malrotation of the fracture site must be avoided
intraoperatively. Anatomic landmarks can be used
to ensure proper rotation is achieved during frac-
ture reduction. The bicipital tuberosity of the
radius should be oriented 180 from the radial
styloid on a fully supinated AP radiograph of the
forearm. The bicipital tuberosity faces medial and
the radial styloid lateral. The ulna can be similarly
evaluated on a lateral radiograph of the forearm
using the coronoid process and ulnar styloid.
1085
The coronoid is positioned volarly 180 in
relation to the ulnar styloid, which is dorsal.
Incarceration of a nail within the
intramedullary canal may result in distraction of
the fracture site and cause malrotation. Removing
the nail and choosing a smaller diameter will
allow for easier passage within the canal and
improve alignment of the fracture. A downside
of reducing the diameter of a titanium nail is that
the rigidness is also decreased. Stainless steel
nails are stiffer and can be utilized when a smaller
diameter nail is required for stabilization.
Attritional rupture of extensor tendons is a risk
of intramedullary nailing. The extensor pollicis
longus should be transposed during the approach
for the dorsal insertion site on the radius to prevent
possible rupture (Table 5).
Postoperative Care
Immobilization options include either a sugar-
tong plaster splint or long-arm berglass cast
which should be bivalved to accommodate swell-
ing. At the 1-week post-op visit, a splint is
converted to a cast or the bivalved cast is
circumferentially overwrapped with berglass.
Duration of immobilization and timing of hard-
ware removal is variable between surgeons.
Multiple aspects play a role in the decision
process including the risks of stiffness, refracture,
percutaneous pin complications, or hardware
irritation. Six to eight weeks of cast
1086
Table 5 Potential pitfalls of surgery and pearls for
prevention: intramedullary nailing of radius and ulna
shaft fractures
Unable to achieve closed Conversion to a mini-open
reduction reduction should be done
without hesitation to
prevent risk of
compartment syndrome.
Typically, no longer than
ten minutes is spent
attempting a closed
reduction
Fracture is malrotated Carefully evaluate full-
and/or distracted length forearm AP and
lateral uoroscopy images
to assess the 180
relationship of the bicipital
tuberosity to the radial
styloid, as well as the
coronoid process to the
ulnar styloid
Consider backing out the
nail and adjusting the
rotation of the forearm
and/or nail. Then
readvance the nail
Unable to advance nail Be sure the nail is not
through the isthmus incarcerated in the canal
causing distraction and/or
malrotation. If so, then
remove the nail and choose
a smaller-diameter-sized
nail
If a 1 mm titanium nail is
too exible, consider using
a stainless steel nail
Extensor pollicis longus Transpose the EPL tendon
(EPL) is in the way of to minimize the risk of
your starting point attritional rupture
immobilization is recommended with removal of
buried implants no sooner than 3 months postop-
eratively, in the operating room. Implants may
require earlier removal due to local irritation par-
ticularly at the olecranon bursa. Any complaints
of difculty or pain with thumb extension should
prompt immediate removal of the radial nail with
exploration of the EPL tendon. Care should be
taken with early removal for concerns of
refracture. Nails or wires/pins left outside the
skin should not remain in place longer than
6 weeks and, for the cooperative child, can be
removed in the ofce (Table 6).
J.J. Whitaker et al.
Table 6 Postoperative protocol for intramedullary nailing
of radius and ulna shaft fractures
End of surgery Immobilize in sugar-tong plaster
splint or long-arm bivalved
berglass cast
1-week follow-up Check AP and lateral forearm
radiographs
Convert splint to long-arm
berglass cast or overwrap
bivalved cast
Duration of 68 weeks:
immobilization First 4 weeks in a long-arm cast
Next 24 weeks in a short arm cast
or sugar-tong splint
After removal of cast and
conrmation of complete
radiographic healing, the patient
may be weight-bearing as tolerated
and return to full activities
Radiographs 1 week, 4 weeks, 8 weeks,
12 weeks postoperatively; longer
if full union is not present
Hardware 36 months
removal
Plate and Screw Fixation
Patients who are skeletally mature or near skeletal
maturity are usually treated with plate xation.
Additionally, some younger children may require
initial plate xation if there is signicant fracture
comminution or an inability to maintain adequate
reduction with an intramedullary implant, typi-
cally occurring in cases of a high-energy injury.
Figure 10 demonstrates a fracture treated in an
adolescent female, following a high-energy fall,
with an unstable fracture pattern. Late indications
for plating are nonunions or an impending
malunion with abundant callus formation which
may block nail passage due to closure of the
medullary canal. The volar approach of Henry is
used for plating distal and midshaft fractures of
the radius. Occasionally, a dorsal Thompson
approach may be necessary for proximal radius
fractures. The ulna is exposed utilizing an incision
along the subcutaneous border between the exten-
sor carpi ulnaris and exor carpi ulnaris. Typi-
cally, 3.5 mm dynamic compression plates
(DCP) are used although smaller plates may be
utilized for young children, such as 2.7 mm com-
pression plates (Fig. 11). One third tubular plates
are also an option.
48 Forearm Fractures 1087

Fig. 10 A fourteen-year-
old female sustained a both-
bone forearm fracture from
a fall off the uneven bars
during gymnastics (AP-a
and lateral-b). She
underwent open reduction
and mini fragmentary plate
xation, the surgeons
preference (AP-c and
lateral-d)

External Fixation interosseous nerve before pin placement. Distally

External xation of forearm fractures in children
is rarely indicated and typically not used for den-
itive management. Severe soft tissue damage,
wound contamination, or segmental bone loss
may necessitate the use of external xation as a
temporary treatment. Half-pins 3.5 mm in size or
smaller are used to build the construct. Ulna pins
can be inserted along the subcutaneous border of
the entire bone. Fluoroscopy should be used to
conrm bicortical xation and ensure pin tips are
not too deep in the central region of the forearm
placing neurovascular structures at risk. Radial
pins should be placed with caution especially in
the proximal forearm. If stabilization is needed in
this region, a small incision should be used along
the lateral side with gentle dissection to the radius.
This will ensure safety of the posterior
pins can be placed in an oblique lateral position or
directly posterior, utilizing a small incision to
ensure injure to the extensor tendons or supercial
radial sensory nerve does not occur. As with the
ulna, uoroscopy must be used to visualize pin
depth in the radius.
Complications of Surgical Treatment
IMN Versus ORIF?
Several studies have compared intramedullary
nailing to open reduction and internal xation of
forearm fractures, without a signicant difference
in functional outcomes being noted. Complication
rates vary with no statistical benet of one
1088
Fig. 11 A ve-year-old male presented 6 months after
casting for a midshaft radius and ulna fracture. He had a
visible forearm deformity and severely limited forearm
rotation. Radiographs revealed a malunion of the radius
with 40 of dorsal angulation (AP-a and lateral-b). Due to
technique over the other (Van der Reis et al. 1998;
Fernandez et al. 2005; Smith et al. 2005; Ozkaya
et al. 2008; Reinhardt et al. 2008; Teoh
et al. 2009). The incidence of complications for
intramedullary nailing is 642 % compared to
1233 % for open reduction and internal xation.
Combined data indicates that nonunions are rare
events with only a 0.2 % (1/351) rate after
intramedullary nailing, and a 4 % (4/95) rate
after open reduction and internal xation (Van
der Reis et al. 1998; Fernandez et al. 2005;
Smith et al. 2005; Ozkaya et al. 2008; Reinhardt
et al. 2008; Teoh et al. 2009; Martus et al. 2013).
Delayed union for intramedullary nailing
occurred in 5 % (19/351) of patients from seven
studies, compared to 1 % (1/95) of patients in six
series having undergone open reduction and inter-
nal xation (Van der Reis et al. 1998; Fernandez
et al. 2005; Smith et al. 2005; Ozkaya et al. 2008;
Reinhardt et al. 2008; Teoh et al. 2009; Martus
et al. 2013). Intramedullary nailing has demon-
strated better cosmetic results (Fernandez
et al. 2005; Teoh et al. 2009).
J.J. Whitaker et al.
the concern that the deformity was too large for effective
remodeling to occur, he underwent a corrective osteotomy
using small fragmentary xation (AP-c and lateral-d). He
healed uneventfully (AP-e and lateral-f) and 6 months after
surgery had normal forearm rotation
Intramedullary Nailing
Complications following intramedullary nailing
include refracture, compartment syndrome,
delayed union, infection, neurovascular injury,
synostosis, and tendon injury. Refer to Table 7
for a summary of complications and management
recommendations.
Refracture after intramedullary nail removal or
even with implants in situ has been reported to
occur 4 % of the time, similar to the rate of those
treated nonoperatively. Refracture with nails
already in place have been successfully treated
with closed reduction (Muensterer and Regauer
2003; Martus et al. 2013). Prevention of refracture
is best accomplished by removing hardware only
after sufcient healing has occurred. This moti-
vates some surgeons to wait 612 months for nail
removal. No matter the chosen timeline for
removal, afterward, a removable splint should be
utilized for a short period to provide protection.
Compartment syndrome associated with
intramedullary xation has an incidence ranging
48 Forearm Fractures
Table 7 Complications of radius and ulna shaft fractures
treated with intramedullary nailing
Complications Management
Refracture Refracture after closed treatment
should be managed with surgical
intervention
Refracture with nails already in
place may be successfully managed
with closed reduction
Prevention of refracture can be
minimized by ensuring adequate
healing prior to hardware removal
A short period of immobilization
with a removable splint is
recommended after removal of
hardware
Compartment Fasciotomies should be performed
syndrome High-energy injuries, multiple
attempts at closed reduction, and
multiple attempts at nail passage are
all risk factors
Delayed union/ Rare in the pediatric population
nonunion especially those treated
nonoperatively
Higher rates are associated with
older patients, open treatment, and
open fractures; therefore, infection
should be ruled out rst
Delayed unions can be monitored
Intramedullary nailing should be
converted to compression plating
with bone grafting as needed for
nonunions
Synostosis After maturation at 6 months to
12 months, excision can be
performed
Consider interposition of fat or inert
material (bone wax) for prevention
Infection Treatment should be focused on the
severity, requiring either antibiotics,
removal of hardware, or irrigation
and debridement
Neuropraxia Complete recovery can typically be
expected
EMG may be considered if no signs
of recovery are seen by 3 months
Nerve exploration/decompression/
possible repair can done for
individuals who fail to recover
normal function within a satisfactory
time period
EPL rupture Can be avoided with transposition
EIP to EPL transfer can restore
function
1089
from 1.6 % to 10 %. Higher rates have been
associated with open fractures and increased oper-
ative times. The authors hypothesize that multiple
attempts at closed reduction and/or multiple passes
with the intramedullary nail may increase the risk
of compartment syndrome (Yuan et al. 2004;
Martus et al. 2013; Blackman et al. 2013). Parents
and patients should always be advised that there is
a potential for this complication intraoperatively or
postoperatively and that a fasciotomy will be
required if compartment syndrome is suspected.
Delayed union is dened as incomplete consol-
idation at about 12 weeks, and a nonunion is
incomplete healing by 6 months (Schmittenbecher
et al. 2008). A delayed union after intramedullary
nailing of pediatric forearm fractures is associated
with children older than 10 years, xation of the
ulna, open reduction required to pass the nail, and
open fractures (Schmittenbecher et al. 2008;
Fernandez et al. 2009; Flynn et al. 2010; Lobo-
Escolar et al. 2012), although recently a single
study demonstrated no correlation of delayed
union with open fractures, open reduction, or xa-
tion of the ulna (Martus et al. 2013). Radioulnar
synostosis following intramedullary nailing is rare,
occurring in only 3/225 cases between two studies
(Cullen et al. 1998; Martus et al. 2013).
Supercial infection has been reported to occur
in up to 5 % of cases following intramedullary
nailing. Deep infection is rare with a range of
0.21 % occurrence, with greater rates noted for
patients with open fractures (Lascombes
et al. 1990; Richter et al. 1998; Flynn et al. 2010;
Martus et al. 2013). Treatment should be focused
on the severity, understanding that patients may
require antibiotics, removal of hardware, irrigation
and debridement, or a combination of these.
Transient neuropraxia is the most common
form of nerve injury, usually involving the super-
cial radial sensory nerve. The incidence is 23 %
and can occur with either a dorsal or lateral
approach to the distal radius when inserting a
nail into the radius (Martus et al. 2013). Ulnar
nerve injury has also been reported, which
resolves spontaneously (Luhmann et al. 2004).
Extensor tendon injury is possible with
intramedullary nailing of the radius. Primarily, the
extensor pollicis longus (EPL) is damaged, with
1090
Fig. 12 A seventeen-year-old male had open reduction
internal xation for an isolated ulnar shaft fracture using a
1/3 tubular plate (AP-a and lateral-b). Four months later
the fracture had not united and the implant failed (AP-c and
lateral-d). Seven months after revision with a
rupture rates of 12 % (Flynn et al. 2010; Martus
et al. 2013). The dorsal entry site places the EPL at
greater risk compared to the lateral entry site. Inci-
sions should be made large enough to protect the
tendon(s) from harm during insertion of the nail and
at the time of nail removal. Ideally, the nail should
be cut ush with the bone, but subsequent removal
may be quite challenging. Therefore, nails left
proud should not encroach upon the extensor ten-
dons as they may lead to risk of an attritional
rupture. The EPL tendon can be transposed away
from Listers tubercle to minimize the risk of injury,
and/or the nail can be left supercial to the tendons.
ORIF
Similar complications are noted for plate xation
with additional concerns for implant-related
J.J. Whitaker et al.
reconstruction plate, the patient returned to the clinic with
persistent pain and incomplete union (AP-e and lateral-f).
He underwent a third surgery with hardware revision and
bone grafting. This time an LC-DCP plate was utilized and
the fracture went on to complete union (AP-g and lateral-h)
fractures and a higher risk of radioulnar synosto-
sis, especially if the procedure is performed
through a single incision. Pediatric bony anatomy
may not accommodate xation with 3.5 mm com-
pression plates; therefore, 2.7 mm plates or even
1/3 tubular plates can be considered. However,
1/3 tubular plates may not provide sufcient
strength and grossly fail, especially if the fracture
progresses to a delayed union and/or the patient is
nearing skeletal maturity (Fig. 12).
The need for removal of plates after bony
union continues to be a debated topic. Survivor-
ship of forearm plates at 10 years has been
reported to be 85 % (Clement et al. 2012).
Although retained plates have minimal reported
complications, there is a risk of fracture due to an
inherent stress riser present at the proximal and
distal aspect of the plates. This creates the poten-
tial for peri-implant fractures. On the other hand,
48 Forearm Fractures 1091

Treatment Algorithm for Pediatric Radius & Ulna Shaft Fractures

Radius/Ulna Shaft Fracture Radius/Ulna Shaft

(Closed Injury) Fracture with:
Open Injury
Severe Soft Tissue Injury
Closed Reduction Compartment Syndrome
& Immobilization Vascular Injury
Floating Elbow

Radiographic
Age < 10yrs Evaluation of < 2yrs growth remaining
Reduction
Near Anatomic Alignment
Angulation 20
Malrotation 30
Translation 100% Age > 10yrs
Bayonet Apposition OK
(shortening 1cm) Angulation 10
Malrotation 30
Translation 50%
NO Bayonet Apposition/Shortening

Acceptable Reduction?
Cast Immobilization:
6 12 wks Re-Reduce
Yes No
Weekly Imaging: (one attempt)
3 4 wks

Loss of Reduction? Surgical Intervention

Fig. 13 Closed reduction and immobilization is the preferred treatment for pediatric forearm shaft fractures. The
reduction parameters outlined in the algorithm are focused on age and growth remaining

there is also a chance of refracture after plate

removal. Interestingly, dynamic compression
plates have shown approximately a 7 % rate of
peri-implant fractures and a 7 % rate of refracture
after plate removal. In contrast there was only a 1 %
risk of peri-implant fracture with a 1/3 tubular plate
and no refractures reported after removal.
There were also no frank hardware failures
(Kim et al. 2005; Clement et al. 2012). Also, grow-
ing children may engulf the plate with bone. This
can impede later hardware removal or hinder treat-
ment of a secondary fracture. Other concerns with
plate retention include possible bacterial coloniza-
tion and theoretical risk of carcinogenicity from
metal corrosion or metallic allergy (Peterson
2005). The benets of plate removal should be
weighed against the complications of the surgical
procedure. In the adult population, a 40 % compli-
cation rate has been associated with forearm plate
removal (Langkamer and Ackroyd 1990). This rate
is 9 % in the pediatric population (Kim et al. 2005).
Preferred Treatment
Closed reduction and immobilization is the pre-
ferred treatment for pediatric forearm shaft frac-
tures. The reduction parameters outlined in the
algorithm (Fig. 13) are focused on age and growth
remaining. After inadequate reduction in the acute
setting, a single repeated attempt to achieve an
acceptable reduction is recommended. If this
fails, surgical intervention is warranted. A subset
of fractures are at greater risk for forearm rotation
loss in patients less than 10 years old. These
include proximal radius fractures with angulation
of 10 or more and radius fractures with any apex
ulnar angulation. Therefore, surgical xation is
considered for these fractures. Flexible
intramedullary nailing is the preferred technique
for surgical treatment of radius and ulna shaft
fractures. Skeletally mature patients or those
nearing skeletal maturity and children with
1092
high-energy injuries are treated with plate
xation. The decision to remove plates is based
on surgeon preference and discussion of benets/
risks with the patient and family. When plates are
removed, a brief period of immobilization and
restriction of activities is assigned to help decrease
the risk of refracture.
Summary
Forearm fractures are frequent injuries in children
and adolescents, which result most commonly
from falls onto an outstretched hand. Careful
assessment of these patients is imperative, with
evaluation of the skin and soft tissues for lacera-
tions, compartment swelling, and a complete
neurovascular examination. Most pediatric fore-
arm fractures, however, are closed injuries with-
out associated nerve injuries or vascular
compromise. The majority of these fractures are
treated with closed reduction and long-arm cast
immobilization under conscious sedation in the
emergency department. Important complications
of casting include stiffness, delayed union, and
refracture.
Indications for reduction and surgical xation
include irreducible or unstable fractures that
have failed an attempt at closed reduction, open
fractures, a oating elbow, vascular injuries,
and fractures associated with severe soft tissue
trauma or compartment syndrome. Flexible
intramedullary nail xation is the treatment of
choice for most children and skeletally immature
adolescents. For some fracture patterns in younger
patients and for older patients near skeletal matu-
rity, open reduction and plate xation is another
satisfactory option. Surgical treatment is generally
associated with an increased number of complica-
tions, especially in children older than 10 years of
age. Compartment syndrome, infections, nerve
injuries, and extensor tendon injuries are the
most common complications associated with
surgical treatment. Careful patient selection,
meticulous surgical technique, and careful post-
operative care are necessary to minimize
complications.
J.J. Whitaker et al.
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 Monteggia Fracture Dislocations
49
Lisa L. Lattanza and Sam Chen

Contents Abstract
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1096 The Monteggia injury was initially described
by Giovanni Monteggia as a proximal third
Pathoanatomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1096
ulnar fracture associated with a radial head
Classication . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1096 dislocation. Since that time, the Monteggia
Acute Injury Diagnosis/Assessment . . . . . . . . . . . . . . 1097 fracture eponym has evolved to encompass
any fracture of the ulna with a radial head
Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1098
dislocation. Furthermore, it is now commonly
Acute Injury Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . 1098 used to describe a radiocapitellar subluxation
Acute Monteggia Preferred Treatment . . . . . . . . . . 1099 or dislocation with an associated ulnar fracture
Chronic Injury Diagnosis/Assessment . . . . . . . . . . . 1100 or deformation. Monteggia fractures in the
pediatric population can result in excellent
Chronic Injury Treatment . . . . . . . . . . . . . . . . . . . . . . . . 1101
outcomes if recognized early and treated
Chronic Monteggia Preferred Treatment . . . . . . . 1104 promptly. Reduction of the fracture or defor-
Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1105 mity of the ulna usually results in reduction of
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1105
the radial head. However, the chronically
dislocated radial head from delayed recogni-
tion results in a more difcult injury and
treatment strategy. Multiple surgical options
for chronic Monteggia injuries have been
described. Unfortunately, the surgical
outcomes are less reliable for chronic injuries.
The treatment options for chronic Monteggia
fractures are still evolving and future large
clinical trials may yield more clarity. It is
important to promptly recognize the
L.L. Lattanza (*) Monteggia injury to avoid an unacceptable
Department of Orthopaedic Surgery, University of outcome.
California, San Francisco, CA, USA
e-mail: lattanza@orthosurg.ucsf.edu
S. Chen
University of California, San Francisco, San Francisco,
CA, USA
e-mail: chens@orthosurg.ucsf.edu

# Springer Science+Business Media New York 2015 1095

J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_50
1096
Introduction
The Monteggia injury was initially described in a
case report by Giovanni Monteggia as a proximal
third ulnar fracture associated with a radial head
dislocation. Subsequent studies in children have
demonstrated that this injury differs signicantly
in the skeletally immature (Bado 1967; Boyd and
Boals 1969; Speed and Boyd 1940). In children,
the peak incidence is between 4 and 10 years of
age and encompasses about 1 % of all forearm
fractures. It is typically sustained after a fall onto
the outstretched hand resulting in a hyperexten-
sion or hyperpronation load onto the elbow (Kay
and Skaggs 1998). The character and treatment of
this injury is dependent on skeletal maturity and
will be described specically for the pediatric
population.
The eponym Monteggia fracture became com-
monly used after it was described by Bado with
reference to the original case report by Monteggia
(Bado 1967). It was recognized that the ulnar
fracture did not always occur at the proximal
third of the ulna as originally described. The
Monteggia fracture eponym evolved to encom-
pass any fracture of the ulna with a radial head
dislocation. The Monteggia fracture is now com-
monly used to describe a radiocapitellar subluxa-
tion or dislocation with an associated ulnar
fracture or deformation. An ulnar fracture with
an associated radial neck fracture is commonly
referred to as a Monteggia equivalent.
The subsequent classication system described
by Bado emphasized the direction of radial head
dislocation. This classication helped to discern
the deforming forces of injury and guide the
reduction maneuver for treatment. Although use-
ful in understanding the injury mechanics, empha-
sis on the character of the ulnar fracture is used for
assessment and treatment of this injury (Ring
et al. 1998). Monteggia fractures in the pediatric
population can result in excellent outcomes if
recognized early and treated promptly. Reduction
of the fracture or deformity of the ulna usually
results in reduction of the radial head. However,
the chronically dislocated radial head from
delayed recognition results in a more difcult
L.L. Lattanza and S. Chen
injury and treatment strategy. It is important to
promptly recognize the Monteggia injury to
avoid an unacceptable outcome.
Pathoanatomy
A Monteggia fracture-dislocation consists of an
ulnar fracture or deformation with radial-sided
ligament failure and subsequent radial head sub-
luxation or dislocation. The forearm consists of
the radius and ulna with three strong stabilizing
ligamentous connections: the distal radioulnar
joint, the central interosseous membrane, and the
proximal radioulnar joint. The proximal joint is
stabilized by the quadrate ligament, radial collat-
eral ligament, and elbow capsule. The radial head
maintains congruency within the proximal
radioulnar joint with contribution from the annu-
lar ligament. Both the proximal and distal
radioulnar joints provide rotational movement
between these two bones. Therefore, any injury
to the bony or ligamentous structures will hinder
pronation and supination of the forearm and may
result in a posttraumatic arthrosis at either articu-
lar surface.
The radius and ulna form a concentric ring in the
forearm stabilized by these three ligamentous com-
ponents. Any injury to one portion of this ring may
damage an adjacent ring component. In a Monteggia
fracture, the bony injury to the ulna is accompanied
by rupture of the annular and quadrate ligaments
with subsequent dislocation of the radial head.
The injury can be associated with a compara-
tively high rate of complications. Radial head sub-
luxation or displacement in this injury can place the
surrounding neurovascular structures at risk. The
radial nerve along with the branching posterior
interosseous nerve lies directly adjacent to the
radiocapitellar joint. A nerve palsy is seen in up
to 10 % of acute Monteggia fractures (Chen 1992).
Classification
Numerous classication systems have been
described; however, Bados classication
(Fig. 1) is the most commonly used. The four
49 Monteggia Fracture Dislocations 1097

different types are based on the direction of radial

head displacement: anteriorly (type 1), posteriorly
(type 2), laterally (type 3), or with an associated
radius fracture (type 4). This classication was
designed to help understand the mechanism of
the initial injury and guide the reduction maneu-
ver and cast position (Bado 1967). In children, a
type I anterior dislocation is most common
followed by type II lateral dislocations (Ring and
Waters 1996).
A modication of Bados classication was
described specically for children by Letts and
associates (Letts et al. 1985). Five types were
described with type A, B, and C subtypes of
Bados type I anterior radial head dislocation. In
Letts type A, the dislocation is accompanied by
plastic deformation of the ulna with an apex ante-
rior deformity (Fig. 2). Type B is a greenstick
fracture of the ulna, and type C is a complete
fracture of the ulna (Fig. 3). A Letts type D
corresponds with a Bado type 2; a Letts type E
corresponds with a Bado type 3.

Acute Injury Diagnosis/Assessment

Monteggia fractures are uncommon in childhood

and can be easily misdiagnosed. Correct diagnosis
and early treatment is essential to avoid elbow
dysfunction and chronic dislocation of the radial
head. An injury should always be suspected when
a child presents with a painful elbow and limited
motion after a fall. A child with a Monteggia
injury will demonstrate pain, swelling, and possi-
ble deformity of the forearm and elbow. On exam,
Fig. 1 Bado classication of Monteggia fractures
both exion-extension and pronation-supination
(Courtesy of Dan A. Zlotolow, MD)
will be painful and limited. Obvious dislocation

Fig. 2 Acute Monteggia

fracture with ulnar plastic
deformation
1098
Fig. 3 Acute Monteggia fracture with anterior radial head
dislocation
may be noted with careful palpation of the radial
head. Palpation along the length of the ulna will
also be painful. If there is plastic deformation
along the ulna, a step-off may not be present. A
careful neurovascular exam should be undertaken
with special attention to the radial and posterior
interosseous nerve. Although less common,
median and ulnar nerve injuries have also been
described (Li et al. 2013).
Imaging
It is essential to obtain radiographic studies of the
elbow with true anteroposterior and lateral views.
The most common reasons for missed Monteggia
fractures are inadequate radiographs and inaccu-
rate interpretation of the radiographs (Gleeson and
Beattie 1994). With any suspicion of injury to the
elbow, high-quality dedicated radiographs must
be taken. To avoid missing a Monteggia fracture,
several assessments should be made on radiogra-
phy. The radial head must be carefully examined
for congruency and alignment with the
capitellum. A line drawn through the longitudinal
axis of the radius should align with the capitellum
on all radiographic views regardless of elbow
position. Additionally, plastic deformation of the
ulna is unique in children and should lead to
careful scrutiny for radiocapitellar dislocation.
The ulnar bow sign can assist in making the cor-
rect diagnosis (Lincoln and Mubarak 1994;
L.L. Lattanza and S. Chen
Degreef and De Smet 2004). On a true lateral
plain radiograph of the elbow, the ulnar bow line
is drawn along the dorsal border of the ulna from
the olecranon to the distal metaphysis. The ulna
normally has a linear border and any deviation
from this line may signify injury. When plastic
deformation occurs, the location of maximum
deformity is usually found to be near the
mid-ulna (Lincoln and Mubarak 1994).
Acute Injury Treatment
The goal of treatment in acute Monteggia frac-
tures is restoration of the length and alignment of
the ulna and reduction of the radiocapitellar joint.
Usually, anatomic reduction of the ulna will
reduce the radiocapitellar joint. The rich vascular
supply and periosteum surrounding the pediatric
elbow contributes to a rapid healing response. It is
critical to recognize and treat an acute Monteggia
fracture before chronic misalignment occurs. If
delayed beyond 1 week, a closed reduction of
the radiocapitellar joint may no longer be possible
(Ring and Waters 1996).
In acute Monteggia fractures, management
depends upon the type of ulnar injury (Ring
et al. 1998). With plastic deformation or incom-
plete fracture of the ulna, a closed reduction with
cast immobilization is performed. Following
closed reduction, the radiocapitellar joint can be
reduced with a combination of elbow exion,
supination, and direct manipulation over the radial
head. The elbow should be immobilized in 90 of
exion and full supination (Bado 1967; Kay and
Skaggs 1998). With complete fractures of the
ulna, there is a risk of displacement and loss of
joint reduction after closed reduction and cast
immobilization (Olney and Menelaus 1989, Ring
and Waters 1996, Nakamura et al. 2009). Opera-
tive treatment is necessary if stable reduction is
not achieved (Table 1). With a transverse or short
oblique fracture, closed reduction with
intramedullary wire/nail xation is often neces-
sary (Fig. 4). If xation with an intramedullary
wire/nail is not stable, a construct with plate and
screws may be needed (Table 2). Complete frac-
tures with a long oblique pattern are more unstable
49 Monteggia Fracture Dislocations
Table 1 Preoperative planning for acute Monteggia
fracture
Operative xation for acute Monteggia fracture
Preoperative planning
OR table: standard with hand table
Position/positioning aids: supine, body position at edge
of table allowing for uoroscopy
Fluoroscopy location: approach from end of hand table
Equipment: Kirschner wires, exible titanium
intramedullary nails, small fragment compression plating
system
Tourniquet: sterile, proximal
Fig. 4 Acute Monteggia fracture with closed reduction
and intramedullary nail xation
Table 2 Surgical steps for acute Monteggia fracture
Operative xation for acute Monteggia fracture
Surgical steps
Initial closed treatment of ulnar fracture
Reduction of radiocapitellar joint
If unstable with transverse or short oblique ulnar fracture,
intramedullary nail xation with insertion through
proximal olecranon
If unstable with long oblique or comminuted ulnar
fracture, plate xation using longitudinal incision over
the fracture site
If radiocapitellar joint irreducible, extended Kocher
approach with joint exploration
Assess elbow stability after reduction and fracture
xation
1099
Table 3 Postoperative protocol for acute Monteggia
fracture
Operative xation for acute Monteggia fracture
Postoperative protocol
Type of immobilization: long arm cast
Length of immobilization: 46 weeks or until evidence of
fracture healing
Rehab protocol: early active range of motion after
fracture healing; gradual return to full weight-bearing
Return to sport protocol: gradual increase in weight-
bearing; return to contact sports at 3 months
and prone to collapse or shortening. Fixation with
plate and screws is usually necessary to achieve
stable xation in this fracture pattern.
Anatomic reduction of the ulna usually allows
stable reduction of the radiocapitellar joint. How-
ever, radiocapitellar joint reduction may be
blocked by soft tissue interposition including the
capsule, annular ligament, and/or posterior
interosseous nerve. An irreducible joint warrants
exploration, especially in the setting of a posterior
interosseous nerve palsy, which may signify nerve
entrapment (Rahbek et al. 2011; Eygendaal and
Hillen 2007; Li et al. 2013). After fracture xa-
tion, the ligamentous stability of the elbow should
be assessed. Occasionally, the lateral ulnar collat-
eral ligament is disrupted and requires open repair.
Loss of reduction is important to recognize
after acute treatment. Weekly reevaluation follow-
ing initial treatment allows assessment for recur-
rent radial head dislocation or subluxation
(Table 3). This is more common with isolated
closed reduction of the ulna. When promptly rec-
ognized, a repeat closed reduction with xation is
performed.
Acute Monteggia Preferred Treatment
In the acutely diagnosed Monteggia fracture,
closed reduction with cast immobilization is the
initial and preferred treatment for children. This
can be accomplished with anatomic reduction of
the ulna which generally provides a stable and
anatomically reduced radiocapitellar joint. After
a careful preoperative evaluation, longitudinal
traction and reduction of the ulnar fracture is
1100
performed. The elbow is then exed and direct
pressure applied over the radial head guiding
reduction. With the radial head reduced, the
elbow is examined in pronation and supination
to assess the most stable position. The elbow is
then immobilized in approximately 90 of exion
in the most stable position of pronation or
supination.
After closed reduction and long arm cast appli-
cation, accurate postreduction radiographs are
obtained. All views should demonstrate a reduced
radiocapitellar joint. The patient then returns for
weekly radiographic reevaluation, including ded-
icated forearm and elbow series, for the rst
3 weeks following the manipulation. If the frac-
ture alignment and radiocapitellar joint reduction
are maintained, the patient returns at 6 weeks
postreduction for cast removal and a new series
of radiographs. It is important to obtain radio-
graphs of the forearm as well as true radiographs
of the elbow.
If anatomic reduction is not possible with
closed treatment, operative treatment is indicated.
With a transverse or short oblique ulnar fracture,
xation is attempted with an intramedullary nail.
If the ulnar fracture is long oblique, comminuted,
or unstable to pin xation, plate xation is
performed.
Occasionally the radiocapitellar joint is irre-
ducible after anatomic reduction of the ulna. Soft
tissue interposition may be blocking the reduction
which may involve a torn annular ligament, an
osteochondral fragment, or the posterior
interosseous nerve. Open reduction and evalua-
tion of the elbow joint is performed. An open
lateral approach using the extended Kocher
interval is performed. The radiocapitellar joint is
visualized and examined for any interposed soft
tissue. If the preoperative evaluation demon-
strated a posterior interosseous nerve palsy, then
the fracture is carefully examined for incarcera-
tion of the nerve. After removal of any soft tissue,
the joint is reduced and checked for stability.
The annular ligament is not routinely
reconstructed (although it may be repaired) and a
transcapitellar pin is not used since the
radiocapitellar joint is stable if the ulnar fracture
is anatomically reduced.
L.L. Lattanza and S. Chen
After open reduction and ulnar xation, the
arm is immobilized for 46 weeks in a long arm
cast. The arm is casted in 90 of exion and the
most stable position of pronation or supination.
The patient is brought in for weekly follow-ups
including dedicated elbow radiographs, for the
rst 3 weeks after surgery, to ensure the joint
remains reduced.
Chronic Injury Diagnosis/Assessment
If the acute Monteggia fracture is not recognized,
a persistent radiocapitellar dislocation will result.
The pediatric elbow is well vascularized and frac-
ture healing and remodeling occurs quickly. The
natural history of the child with a chronic
Monteggia fracture includes pain, loss of motion,
progressive cubitus valgus deformity, and insta-
bility. Subsequent tardy ulnar nerve palsy may
also develop.
The missed Monteggia fracture results in a
chronic dislocation of the radial head that
becomes more disabling with time (Nakamura
et al. 2009; Rahbek et al. 2011). The initial injury
may be misdiagnosed between 16 % and 33 % of
the time, especially with plastic deformation of
the ulna (Dormans and Rang 1990). Up to 60 % of
children may develop signicant pain and up to
50 % may develop limited range of motion with
conservative treatment alone (Nakamura
et al. 2009). Left untreated, the radial head
undergoes hypertrophic change with loss of the
articular surface concavity (Fig. 5). This is accom-
panied by attening of the capitellum. With time a
valgus deformity of the radial neck may also
develop. These morphological changes result in
the radial head failing as a component in elbow
stability. With time a progressive cubitus valgus
deformity develops (Fig. 6). This may place the
ulnar nerve on increasing stretch and friction
resulting in potential for a late ulnar nerve palsy.
Both posterior interosseous and median neuropa-
thies have also been described in missed
Monteggia injuries (Chen 1992; Nakamura
et al. 2009).
Differentiation between a congenital unilateral
radial head dislocation and chronic Monteggia
49 Monteggia Fracture Dislocations
injury may be difcult when the initial injury
radiographs are not available. Both conditions
may demonstrate radiographic changes including:
a dome-shaped convex radial head, a concave
appearance of the posterior ulnar border, and a
hypoplastic or attened capitellum. Arthrography
has been utilized to aid in determining the diag-
nosis. Traditionally, an intracapsular radial head
on arthrography is diagnostic of a congenital dis-
location, whereas an extracapsular location sig-
nies a prior traumatic dislocation. However, it
Fig. 5 Nonhealed chronic Monteggia fracture with radial
head changes
Fig. 6 Nonhealed chronic
Monteggia fracture.
Clinical image comparing
the progressive valgus
deformity (left) as
compared to the
contralateral normal elbow
(right)
1101
is now known that both radiographic changes and
radial head capsular location do not accurately
conrm the diagnosis (Kosay et al. 2002). The
differentiation is best accomplished by a combi-
nation of history, contralateral elbow exam, and
bone morphology. Without clear antecedent
trauma or injury radiographs, it may be difcult
to differentiate these two conditions.
Chronic Injury Treatment
The current trend for most chronic Monteggia
fractures is surgical management, because long-
term outcomes without treatment are poor. How-
ever, contraindications for surgical treatment are
still unclear (Stoll et al. 1992; Nakamura
et al. 2009; Rahbek et al. 2011). The increased
remodeling potential in younger patients is an
important factor for surgical outcome. There is
no consensus for a denitive age limit for surgery
(Horii et al. 2002; Wang and Chang 2006).
Although not an absolute contraindication for sur-
gery, one factor contributing to poor outcome is a
prolonged interval between the original
Monteggia injury and surgical treatment.
1102 L.L. Lattanza and S. Chen

Recommendations have varied with some authors
recommending a maximum time limit of 4 years
from the initial injury, to minimize poor outcomes
and complications (Wang and Chang 2006;
Rahbek et al. 2011; Stoll et al. 1992). However,
successful treatment can be accomplished with
much longer intervals and each case should be
considered individually based on symptoms and
degree of ulnar and radial head deformity.
In most cases, the chronic Monteggia fracture
should be corrected with the exception of severe
deformity or degeneration of the radiocapitellar
joint that cannot be corrected with reconstructive
techniques. Even in patients with radial head mal-
formation from prolonged chronic dislocation,
reconstruction is considered with progressive val-
gus deformity. Radial head replacement can be
used in this situation to achieve stability (Fig. 7).
However, this surgical option is not commonly
performed and an age criteria has yet to be
dened. It should not be performed in the skele-
tally immature patient with open physes.
The surgical treatment of chronic Monteggia
fractures aims to achieve radial head reduction
with an ulnar corrective osteotomy. Occasionally,
a radial osteotomy is also necessary depending on
the deformity. The goal of the ulnar osteotomy is
to restore ulnar length and correct any angular
malunion in order to reduce the radiocapitellar
joint (Table 4). Rigid plate xation of the ulnar
Table 4 Preoperative planning for chronic Monteggia
fracture
Operative xation for chronic Monteggia fracture
Preoperative planning
OR table: standard with hand table
Position/positioning aids: supine, body position at edge
of table allowing for uoroscopy, iliac crest also prepped
if corticocancellous autograft needed
Fluoroscopy location: approach from end of hand table
Equipment: Kirschner wires, small fragment
compression plating system, osteotomes, mini
reciprocating saw, small lamina spreader, small distractor,
tricortical allograft bula
Tourniquet: sterile, proximal

Fig. 7 Preoperative and

postoperative images of
chronic Monteggia fracture
treated with plate xation
and radial head
replacement. A radial head
replacement was required to
maintain correction,
provide stability, and
prevent recurrence of severe
progressive cubitus valgus
deformity
49 Monteggia Fracture Dislocations
Fig. 8 Corrective ulnar
osteotomies. Sliding,
opening wedge, and
distraction
Table 5 Surgical steps for chronic Monteggia fracture
Operative xation for chronic Monteggia fracture
Surgical steps
Careful preoperative planning for appropriate corrective
ulnar osteotomy
Posterior longitudinal skin incision over proximal ulna.
Proximal incision should curve laterally to allow Kocher
exposure if needed
Corrective ulnar osteotomy with assessment in all planes.
Lamina spreader or distractor to assist with lengthening
Assess radiocapitellar joint for reduction in all positions
of forearm rotation. Perform joint arthrotomy with
exploration and debridement if radial head irreducible
Contour and apply plate xation at the osteotomy site.
Recheck radial head reduction in all positions of forearm
rotation
Inpatient admission for observation and pain control
osteotomy is required (Horii et al. 2002). Various
types of ulnar osteotomies have been described
including sliding, opening wedge, and distraction
osteotomies (Fig. 8). Regardless of the osteotomy
type, the goal is correction of both length and
angular deformity to reduce the radial head
(Table 5). This frequently requires an exaggerated
correction of the ulnar posterior bow. The ulnar
corrective osteotomy has three components:
lengthening, posterior angulation correction, and
medial or lateral angulation correction. Careful
1103
preoperative planning of the ulnar osteotomy
should ensure appropriate correction in all planes.
Current recommendations suggest a more proxi-
mal ulnar osteotomy site (Fig. 9; Rahbek
et al. 2011, Nakamura et al. 2009, Degreef and
De Smet 2004). With a more proximal ulnar
osteotomy, there is preservation of the
interosseous membrane. This allows increased
tension along the interosseous membrane which
helps maintain the radial head reduction.
The ulnar osteotomy may also be combined
with reconstruction of the annular ligament (Bell
Tawse 1965; Nakamura et al. 2009). Controversy
exists whether ligamentous reconstruction
improves surgical outcome in maintaining a
reduced and congruent joint (Rahbek
et al. 2011). Several procedures have been
described for reconstruction of the annular liga-
ment with varying rates of success (Bell Tawse
1965; Rodgers et al. 1996). These include the use
of a primary repair, pedicled fascia of the triceps,
pedicled fascia of the forearm, and free palmaris
longus tendon. Complications with annular liga-
ment reconstruction include loss of motion and
progressive radial notching due to constriction
around the radial neck (Rodgers et al. 1996;
Nakamura et al. 2009).
1104
Fig. 9 Two examples of correct placement of ulnar osteotomy in the proximal third of ulna
Recently, 3D medical imaging and printing has
become a valuable tool for the surgical planning
and correction of chronic Monteggia injuries.
ACT scan is obtained of the normal and injured
extremity. The surgeon and an engineer have a
virtual computer planning session to plan the
operative correction necessary using appropriate
osteotomies. Interestingly, during the planning
phase a deformity of both the radius and ulna
have been detected in some Monteggia lesions.
Previously, the deformity was thought to only
involve the ulna. However, we have learned
from the modeling procedure that some chronic
deformities of the radius develop over time as a
compensatory deformity. This information allows
for a more precise correction. In one case correc-
tion of both the radius and ulna resulted in less
lengthening and angulation of the ulna than would
normally be required. It is likely that chronic
Monteggia radial head dislocations that are excep-
tionally difcult to reduce may be due to this
previously unrecognized radial deformity.
Correcting only the ulna in this situation may not
be adequate to obtain a good result.
Surgical complications for chronic Monteggia
injury include stiffness, transient nerve palsy, non-
union, infection, and compartment syndrome.
Weekly reevaluation, with dedicated elbow radio-
graphs, following treatment allows for assessment
L.L. Lattanza and S. Chen
Table 6 Postoperative protocol for chronic Monteggia
fracture
Operative xation for chronic Monteggia fracture
Postoperative protocol
Type of immobilization: long arm cast
Length of immobilization: 6 weeks or until evidence of
healing at osteotomy site
Rehab protocol: begin range of motion at 6 weeks;
gradual return to full weight-bearing
Return to sport protocol: return to contact sports no earlier
than 3 months
of a recurrent radial head dislocation (Table 6).
Stiffness after Monteggia fracture can result from
prolonged immobilization, heterotopic ossica-
tion, or myositis ossicans surrounding the joint.
Generally, stiffness from prolonged casting
should improve in several months. Both hetero-
topic ossication and myositis ossicans gener-
ally improve with time and conservative
management.
Chronic Monteggia Preferred
Treatment
A long oblique sliding osteotomy is preferred in
most cases. This allows for direct cortical contact
at the osteotomy site and negates the need for
49 Monteggia Fracture Dislocations
structural allograft bone. In some cases with
complex multiplanar deformity, it is easier to
perform a straight osteotomy and ll the defect
with tricortical allograft bone, but healing at the
osteotomy site takes much longer, and this is why
the sliding osteotomy is preferred.
A posterior skin incision is made over the ulna
curving laterally at the proximal aspect of the
incision which allows access to both the ulna
and the radiocapitellar joint if necessary, through
the same incision. Mini-uoroscan is used to help
identify the correct placement of the osteotomy.
Ideally this is located in the proximal 1/3 of the
ulna approximately 12 cm distal to the coronoid.
Placement should allow for at least three screws in
the proximal fragment without violating the
apophysis of the ulna. The osteotomy is made in
a long oblique fashion with careful attention to the
direction of correction that is needed (posterior
angulation with or without medial/lateral correc-
tion). The osteotomy is then completed in the
correct plane to allow for the appropriate correc-
tion. A laminar spreader or small distractor is used
to assist in regaining length at the osteotomy site.
With appropriate angular correction and lengthen-
ing, the radial head should be reduced. If irreduc-
ible, an arthrotomy is performed using an
extended Kocher approach. Any scar tissue
blocking reduction is removed. In some cases
the radial head may have button-holed through
the anterior capsule and will need to be carefully
dissected free by nding and protecting the radial
nerve.
Once the radial head is reduced (in all positions
of forearm rotation), the osteotomy site is provi-
sionally xed with Kirschner wires. The appropri-
ate size plate (depending on the size and age of the
patient) is selected and contoured to maintain the
osteotomy correction. The plate is secured to the
bone with the appropriate length screws. Final
images are taken to conrm reduction of the radial
head in all planes of forearm rotation. The annular
ligament is not reconstructed, but any capsule/
ligament available is repaired if the joint was
opened to reduce the radial head. The incisions
are irrigated and closed, and the patient is placed
in a long arm cast that is bivalved in the operating
room including splitting the cast padding.
1105
The patient is admitted for observation and pain
control.
The patient is seen in 1 week with radiographs
of the forearm and elbow to conrm reduction. A
well-padded long arm cast is reapplied. The patient
returns at 6 weeks postoperatively for cast removal
and radiographs. With healing at the osteotomy
site, the patient then begins range of motion with-
out further immobilization. Contact sports are not
allowed for an additional 6 weeks.
Summary
The Monteggia fracture is a radiocapitellar disloca-
tion with an associated ulnar fracture that requires
accurate diagnosis. The treatment outcomes are
excellent if recognized early and treated promptly.
Restoration of the ulnar length and alignment
allows reduction of the radial head. Unfortunately,
chronically dislocated Monteggia fractures have a
more difcult treatment strategy and less reliable
surgical outcomes. The treatment options for
chronic Monteggia fractures are still evolving and
future large clinical trials may yield more clarity. It
is important to promptly recognize the Monteggia
injury to avoid an unacceptable outcome.
References
Bado JL. The Monteggia lesion. Clin Orthop.
1967;50:7186.
Bell Tawse A. The treatment of malunited anterior
Monteggia fractures in children. J Bone Joint Surg Br
Vol. 1965;47(4):71823.
Boyd HB, Boals JC. The Monteggia lesion: a review of
159 cases. Clin Orthop. 1969;66:94100.
Chen W. Late neuropathy in chronic dislocation of the
radial head: report of two cases. Acta Orthop. 1992;63
(3):3434.
Degreef I, De Smet L. Missed radial head dislocations in
children associated with ulnar deformation: treatment
by open reduction and ulnar osteotomy. J Orthop
Trauma. 2004;18(6):3758.
Dormans J, Rang M. The problem of Monteggia fracture-
dislocations in children. Orthop Clin North
Am. 1990;21(2):2516.
Eygendaal D, Hillen R. Open reduction and corrective
ulnar osteotomy for missed radial head dislocations in
children. Strategies Trauma Limb Reconstr. 2007;2
(1):314.
1106
Gleeson A, Beattie T. Monteggia fracture-dislocation in
children. J Accid Emerg Med. 1994;11(3):1924.
Horii E, Nakamura R, Koh S, Inagaki H, Yajima H, Nakao
E. Surgical treatment for chronic radial head disloca-
tion. J Bone Joint Surg. 2002;84(7):11838.
Kay RM, Skaggs DL. The pediatric Monteggia
fracture. Am J Orthop (Belle Mead NJ). 1998;27
(9):6069.
Kosay C, Akcali O, Manisali M, Ozaksoy D, Ozcan
C. Congenital anterior dislocation of the radial head: a
case with radiographic ndings identical to traumatic
dislocation. Eur J Radiol. 2002;43(1):5760.
Letts M, Locht R, Wiens J. Monteggia fracture-
dislocations in children. J Bone Joint Surg Br Vol.
1985;67(5):7247.
Li H, Cai Q, Shen P, Chen T, Zhang Z, Zhao L. Posterior
interosseous nerve entrapment after Monteggia
fracture-dislocation in children. Chin J Traumatol.
2013;16(3):1315.
Lincoln TL, Mubarak SJ. Isolated traumatic radial-head
dislocation. J Pediatr Orthop. 1994;14(4):4547.
Nakamura K, Hirachi K, Uchiyama S, Takahara M,
Minami A, Imaeda T, et al. Long-term clinical and
radiographic outcomes after open reduction for missed
Monteggia fracture-dislocations in children. J Bone
Joint Surg. 2009;91(6):1394404.
Olney BW, Menelaus MB. Monteggia and equivalent lesions
in childhood. J Pediatr Orthop. 1989;9(2):21923.
Rahbek O, Deutch SR, Kold S, Sjbjerg JO, Mller-
Madsen B. Long-term outcome after ulnar osteotomy
for missed Monteggia fracture dislocation in children. J
Child Orthop. 2011;5(6):44957.
L.L. Lattanza and S. Chen
Ring D, Waters PM. Operative xation of Monteggia
fractures in children. J Bone Joint Surg Br Vol.
1996;78:7349.
Ring D, Jupiter JB, Waters PM. Monteggia fractures in
children and adults. J Am Acad Orthop Surg. 1998;6
(4):21524.
Rodgers W, Waters PM, Hall JE. Chronic Monteggia
lesions in children. Complications and results of recon-
struction*. J Bone Joint Surg. 1996;78(9):13229.
Speed J, Boyd HB. Treatment of fractures of ulna with
dislocation of head of radius. J Am Med Assoc.
1940;115(20):1699705.
Stoll TM, Willis RB, Paterson D. Treatment of the missed
Monteggia fracture in the child. J Bone Joint Surg Br
Vol. 1992;74(3):43640.
Wang MN, Chang W. Chronic posttraumatic anterior dis-
location of the radial head in children: thirteen cases
treated by open reduction, ulnar osteotomy, and annular
ligament reconstruction through a Boyd incision. J
Orthop Trauma. 2006;20(1):15.
Further Reading
Hui JH, Sulaiman AR, Lee H, Lam K, Lee E. Open reduc-
tion and annular ligament reconstruction with fascia of
the forearm in chronic Monteggia lesions in children. J
Pediatr Orthop. 2005;25(4):5016.
Song KS, Ramnani K, Bae KC, Cho CH, Lee KJ, Son
ES. Indirect reduction of the radial head in children
with chronic Monteggia lesions. J Orthop Trauma.
2012;26(10):597601.
 Galeazzi and Essex Lopresti Injuries
50
Kevin J. Little, Philip To, and Reid Draeger

Contents Management of Complications . . . . . . . . . . . . . . . . . . . 1118

Introduction to Galeazzi Fractures . . . . . . . . . . . . . . 1108 Summary and Future Directions . . . . . . . . . . . . . . . . . 1119
Anatomy and Pathoanatomy Relating to References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1120
Galeazzi Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1108
Distal Radioulnar Joint Anatomy . . . . . . . . . . . . . . . . . . 1108
Forearm and Interosseous Membrane Anatomy . . . 1109
Pathoanatomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1109
Assessment of Galeazzi Fractures . . . . . . . . . . . . . . . . 1109
Signs and Symptoms of Galeazzi Fractures . . . . . . . . 1109
Galeazzi Fractures Imaging and Other Diagnostic
Studies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1110
Injuries Associated with Galeazzi Fractures . . . . . . . 1110
Classication of Galeazzi Fractures . . . . . . . . . . . . . . . . 1110
Galeazzi Fracture Outcome Tools . . . . . . . . . . . . . . . . . . 1110
Galeazzi Fracture Treatment Options . . . . . . . . . . . 1111
Nonoperative . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1111
Operative Treatment for Galeazzi Fractures . . . . . . . 1113
Reduction and Fixation of the Radius . . . . . . . . . . . . . . 1114
DRUJ Operative Treatment . . . . . . . . . . . . . . . . . . . . . . . . 1116
Postoperative Immobilization . . . . . . . . . . . . . . . . . . . . . . 1117
Surgical Treatment Outcomes . . . . . . . . . . . . . . . . . . . . . . 1117
Preferred Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1118
Surgical Pitfalls and Prevention . . . . . . . . . . . . . . . . . . 1118

K.J. Little (*)

University of Cincinnati Department of Orthopaedic
Surgery, Hand and Upper Extremity Surgery, Cincinnati
Childrens Hospital Medical Center, Cincinnati, OH, USA
e-mail: kevin.little@cchmc.org
P. To R. Draeger
Hand Surgery Specialists, Inc, Cincinnati, OH, USA
e-mail: philip.to3182@gmail.com;
reid.draeger@gmail.com

# Springer Science+Business Media New York 2015 1107

J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_51
1108
Abstract
Injuries to the forearm can lead to fractures of
either or both the radius and ulna as well as
dissociation between the bones in the forearm.
Galeazzi fractures occur when the current of
injury in the forearm fractures the radius shaft
and then proceeds through the distal radioulnar
joint (DRUJ) to cause dislocation or subluxa-
tion of the joint. In children, this can be a true
DRUJ dislocation or can also be a fracture-
separation of the distal ulnar physis. Anatomic
reduction of the fracture typically leads to
DRUJ stability unless soft tissues are inter-
posed in the joint or physis. The key to treat-
ment of these complex injuries is an anatomic
reduction and the creation of a stable joint
whether by closed or open methods. Once frac-
ture healing is identied, therapeutic exercises
can be initiated and a gradual return to full
activities can be expected. Essex-Lopresti inju-
ries are seen in patients that have interruption
of the interosseous membrane and dissociation
of the radius and ulna. They have not been
described in skeletally immature patients, but
clinicians should be aware of the risks of prox-
imal migration of the radius that can occur after
radial head resection in these patients.
Introduction to Galeazzi Fractures
Galeazzi described a series of fractures of the
radius shaft and dislocation of the distal-radial
ulnar joint, which have since come to bear his
name, in 1934 (Galeazzi 1934). However, he is
not the rst person to describe the injury pattern,
as it was rst described by Sir Astley Cooper in
1822 (Reckling and Peltier 1964; Reckling and
Cordell 1968). Walsh was the rst to describe a
series consisting solely of pediatric Galeazzi frac-
tures (Walsh et al. 1987), and Landfried et al.
described the pediatric variant of Galeazzi frac-
tures which consists of a fracture of the shaft of the
radius and a physeal fracture of the distal ulna
(Landfried et al. 1991). Galeazzi fractures in chil-
dren are uncommon with a noted incidence of 2.8
K.J. Little et al.
% among 1,453 children with radial shaft frac-
tures (Walsh et al. 1987). The authors also noted
that 41 % of the cases were not recognized at rst
and the peak age was between 9 and 13 years
(Walsh et al. 1987). Galeazzi fractures in children
often occur at the junction of middle and distal
thirds of the radius, and the direction of disloca-
tion of the ulna can be either volar or dorsal
(Walsh et al. 1987; Eberl et al. 2008).
Essex-Lopresti injuries are described as a frac-
ture of the radial head with disruption of the distal
radioulnar joint (DRUJ) which occurs through a
concomitant injury to the interosseous membrane.
Peter Essex-Lopresti was a British orthopedic sur-
geon who reported a case series in 1951 describ-
ing the injury (Essex-Lopresti 1951). There are no
reported Essex-Lopresti injuries in skeletally
immature children. However, there are multiple
case series of pediatric Galeazzi fractures and with
ipsilateral radial head dislocations, known as a
Monteggia fracture variant (Reckling 1982;
Kontakis et al. 2008; Akalin et al. 2010).
Anatomy and Pathoanatomy Relating
to Galeazzi Fractures
Distal Radioulnar Joint Anatomy
The distal radioulnar joint (DRUJ) is the articula-
tion of the convex ulnar head and the concave
distal-radial sigmoid notch. The ulnar head, with
its smaller radius of curvature, sits shallowly in
the sigmoid notch, whose radius of curvature is
greater, which allows for some translation of the
ulnar head in the sigmoid notch as the distal radius
moves around the ulnar head with forearm prona-
tion and supination. Only 1060 % of the DRUJ
articular surface represents actual contact surface
of the sigmoid notch and the ulnar head through
various degrees of pronosupination (Leversedge
et al. 2010).
This incongruous articulation is stabilized by
multiple ligamentous and soft tissue structures. The
triangular brocartilage complex (TFCC) is the most
important of these structures. The TFCC inserts at
the ulnar fovea at the base of the ulnar styloid.
50 Galeazzi and Essex Lopresti Injuries
It is composed of the triangular brocartilage articu-
lar disk centrally, the ulnocarpal meniscus homo-
logue, the dorsal and volar radioulnar ligaments,
the oor of the extensor carpi ulnaris tendon sheath,
and the volar ulnocarpal ligaments (V ligament) run-
ning from the ulna to the lunate and the triquetrum.
The dorsal and volar radioulnar ligaments are the
primary stabilizers of the DRUJ. The conuence of
the dorsal and volar radioulnar ligaments insert prox-
imally into the fovea as the ligamentum
subcruentum as well as distally, directly into the
base of the ulnar styloid independent of the insertion
of the ligamentum subcruentum.
Attachments of the TFCC provide tensile sta-
bility to the DRUJ throughout forearm rotation to
counterbalance the bony compression at the artic-
ulation. At extremes of pronation and supination,
the tension through the TFCC is maximized
thereby stabilizing the joint the greatest in these
positions, where, otherwise, the joint would be at
most risk of dislocation. In pronation, the bony
articulation dorsally is under compression,
whereas the volar TFCC is under tension. The
opposite pattern of compressive and tensile forces
is seen in supination. Thus, any disruption of the
volar TFCC would allow for dorsal displacement
of the ulnar head in the sigmoid notch in prona-
tion, while disruption of the dorsal TFCC would
allow for volar translation of the ulnar head in the
sigmoid notch in supination (Waters and Bae
2010).
Forearm and Interosseous Membrane
Anatomy
In addition to the DRUJ, the radius and ulna are
stabilized by their proximal articulation and the
interosseous ligament complex (IOLC). The
IOLC stabilizes both longitudinal and transverse
forces on the radius and ulna and allows for load
transfer between the two. The IOLC is composed
of three groups of bers all originating proximally
on the radius and inserting distally on the ulna.
The proximal band, or oblique ligament, is the
most proximal of these bers. The central liga-
ment is the largest and strongest of these bers and
1109
is primarily responsible for providing longitudinal
and transverse stability between the radius and
ulna. The accessory bands can number between
1 and 5 and are distal to the central band with
bers running in the same direction (Leversedge
et al. 2010). Additionally, the distal oblique band
of the distal interosseous membrane, which can be
seen in 40 % of specimens, assists with stability of
the DRUJ via its insertion on the volar lip of the
sigmoid notch (Moritomo 2012).
Pathoanatomy
Galeazzi fractures in adults involve a fracture of
the radius coupled with dislocation of the DRUJ,
most commonly dorsally. This is due to disruption
of the DRUJ capsule and often the strong liga-
mentous stabilizers of the DRUJ. In children,
energy transmitted through the DRUJ more com-
monly leads to a distal ulnar physeal fracture
rather than injury to the ligamentous attachments
(Imatani 1996; Waters and Bae 2010). This is due
to the fact that the physeal plate of children is
biomechanically weaker than the ligamentous sta-
bilizers of the DRUJ (Imatani 1996). Addition-
ally, periosteum or other soft tissues such as the
extensor digiti minimi and extensor carpi ulnaris
can become entrapped in the fracture site
preventing reduction of the distal ulnar physis
(Landfried et al. 1991).
Assessment of Galeazzi Fractures
Signs and Symptoms of Galeazzi
Fractures
Most children do not recall their exact mechanism
of injury. It is postulated that the mechanism is
from an axial load with some rotational compo-
nent. Assessing instability of the DRUJ clinically
is difcult, as a child frequently will not allow the
examiner to move the wrist. Subtle injury to
DRUJ can be easily missed. The neurovascular
exam is typically normal. However, anterior
interosseous nerve palsy has been seen with
1110 K.J. Little et al.

pediatric Galeazzi fractures (Stahl et al. 2000),

and the ulnar nerve can be injured with volar
dislocation of the DRUJ.

Galeazzi Fractures Imaging and Other

Diagnostic Studies

It is important to image the joint above and below

the level of injury. If the elbow joint is not imaged,
then a concomitant Monteggia injury can be
missed. In pediatric Galeazzi fractures, lateral
radiographs typically show a dorsal or volar dis-
location of the ulna. Typically, the apex of the
radius fracture will be the same direction as the
distal ulna dislocation (Fig. 1). A true lateral is
paramount in order to assess for a DRUJ disloca-
tion. This true lateral view has the pisiform at the
distal aspect of the scaphoid with the pisiform in
line with the central axis of scaphoid. Additional
advanced imaging, such as three-dimensional or
cross-sectional imaging, is not typically necessary
in skeletally immature children.

Injuries Associated with Galeazzi
Fractures
Galeazzi fractures in children are associated with
distal ulnar physeal separations. These Salter-
Harris I, II, or III injuries lead to a remarkably
high rate of distal ulnar physeal arrest, as high as
50 %. Additionally, Galeazzi fractures have been
associated with ipsilateral radial head dislocations
or proximal radioulnar joint dislocations
(Reckling 1982; Kontakis et al. 2008; Akalin
et al. 2010). These injuries are described as
Monteggia variants of Galeazzi fractures.
Classification of Galeazzi Fractures
Letts and Rowhani developed the classication
for pediatric Galeazzi fractures (Letts and
Rowhani 1993) which is described in Table 1. In
Letts case series, the most common type of
Galeazzi fracture was Type BII. This type of
Fig. 1 Lateral radiograph of a Galeazzi fracture with apex
volar angulation and volar dislocation of the DRUJ. The
distal ulnar physis is intact (Photo courtesy of Kevin
J. Little, MD)
fracture is a distal third radius fracture with an
epiphyseal fracture of distal ulna and dorsal dis-
placement of ulnar metaphysis (Fig. 2).
Galeazzi Fracture Outcome Tools
In assessing the outcomes, no validated instru-
ment has specically been designed for these
types of injury. Previous studies have classied
Galeazzi fracture outcomes as excellent, fair, and
poor. Table 2 shows the classication and
the denition as described by Mikic in 1975
(Miki 1975; Letts and Rowhani 1993).
The authors do not use this outcome measure
and prefer to base outcomes based on range of
motion, pain, and functional use of the
upper extremity.
50 Galeazzi and Essex Lopresti Injuries 1111

Table 1 Galeazzi equivalent fractures in children

Letts and Rowhani classication of pediatric Galeazzi
equivalent fractures
Type A: Fracture of the I: Dorsal dislocation of
radius at the junction of the the distal ulnar end
middle third with the distal II: Distal epiphysiolysis
third of the ulna with dorsal
displacement of the
metaphysis
Type B: Fracture of the I: Dorsal dislocation of
radius at the distal third the distal ulnar end
level II: Distal epiphysiolysis
of the ulna with dorsal
displacement of the
metaphysis
Type C: Greenstick fracture I: Dorsal dislocation of
of the radius with dorsal the distal ulnar end
bowing II: Distal epiphysiolysis
of the ulna with dorsal
displacement of the
metaphysis
Type D: Fracture of the I: Volar dislocation of the
radius with volar angulation distal ulnar end
II: Distal epiphysiolysis
of the ulna with volar
displacement of the
metaphysis
Adapted from Letts and Rowhani (1993)
Fig. 2 Lateral radiograph of a Letts and Rowhani Type B
II pediatric Galeazzi equivalent fracture with partial epiph-
yseal fracture (yellow arrow) (Photo courtesy of Kevin
J. Little, MD)
Galeazzi Fracture Treatment Options

Nonoperative case series of pediatric Galeazzi fractures (Walsh

Indications/Contraindications
Unlike adults, where Galeazzi fractures have been
termed the fracture of necessity (necessitating
operative xation), the majority of Galeazzi frac-
tures in children can be successfully treated
nonoperatively. However, the moniker of frac-
ture of necessity still applies, in that, in order to
treat this fracture nonoperatively, an anatomic
reduction is necessary, as is weekly radiographic
follow-up to ensure appropriate alignment is
maintained. The indications and contraindications
to nonoperative management are illustrated in
Table 3. Though successful treatment with immo-
bilization in both long- and short-arm casts has
been reported, inferior results have been reported
with short-arm compared to long-arm casting in a
et al. 1987). As children approach skeletal maturity,
the chance for failure of nonoperative treatment
approaches that of adults (up to 92 %) and opera-
tive treatment should be strongly considered in any
patient with closed physes (Eberl et al. 2008).
Technique
The reduction maneuver for pediatric Galeazzi
fractures is dependent on the characteristics of
the injury, including the apex of the radius fracture
and associated ulnar head dislocation. In the case
of an apex volar radius fracture and an associated
volar dislocation of the ulnar head, forearm pro-
nation and volar forearm pressure often provide
the reduction force necessary to allow for reduc-
tion of the radius angulation and a dorsal reloca-
tion of the ulnar head. Apex dorsal radius fracture
1112
Table 2 Mikic classification of outcomes following
Galeazzi fractures
Outcome Criteria
Excellent Radiographic union
Anatomical alignment
Congruent DRUJ
Full wrist and elbow motion
Full forearm pronosupination
Fair Patient satised with outcome but one or
more of the following
Delayed union
Minimum malalignment or shortening of
the radius
Subluxation of the ulnar head
Excessive scar formation
Limitation of pronosupination of less
than 45
Poor Patient dissatised with outcome plus one
or more of the following
Pain
Deformity of the forearm
Nonunion
Remarkable shortening or angulation of
the radius
Limitation of pronosupination of more
than 45
Excessive restriction of elbow or wrist
motion
angulation with an associated dorsal ulnar dislo-
cation is reduced with supination of the forearm
and dorsal forearm pressure. The arm is placed in
a sugar-tong splint following reduction and
overwrapped into a cast after the initial period of
swelling subsides. Healing typically takes 46
weeks, and a protective splint is recommended
for an additional 612 weeks due to increased
refracture rates in distal shaft fractures (Fig. 3).
In the event that an anatomic closed reduction
cannot be obtained or maintained, open reduction
is required. Reduction of the DRUJ can be
blocked by the extensor tendons, buttonholing of
the ulnar head through the wrist capsule and
extensor retinaculum, and interposed periosteum
(Karlsson and Appelqvist 1987; Hanel and Scheid
1988; Castellanos et al. 1999; Landfried et al.
1991). Figure 4 illustrates the pediatric Galeazzi
fracture variant involving a radius fracture
coupled with a dorsal DRUJ dislocation, with a
K.J. Little et al.
Table 3 Indications for nonsurgical management
Pediatric Galeazzi fractures
Nonoperative management
Indications Contraindications
Closed fractures in Open fractures
skeletally immature
patients
Skeletally mature patients
Inability to reduce DRUJ
(malreduced radius fracture,
interposed extensor tendon,
ulnar head buttonholed
through capsule, infolded
periosteum)
Loss of closed reduction
Malreduced unrecognized
injury
block to reduction by interposed extensor retinac-
ulum (Landfried et al. 1991). Additionally,
malreduction of the radius fracture can lead to an
irreducible DRUJ.
Close radiographic follow-up is essential in
these cases as loss of reduction can occur in up
to 15 % of cases. The chance of a Galeazzi frac-
ture requiring surgical treatment in a child
increases as the patient nears skeletal maturity.
Additionally, late presentation of an unrecognized
Galeazzi fracture can prove challenging to treat
nonoperatively, due to difculty in obtaining an
anatomic reduction of the radius and DRUJ, and
operative stabilization may be necessary for these
patients.
Outcomes
Outcome data for nonoperative treatment of
Galeazzi fractures are limited, as there are few
large series of these injuries. However, the overall
outcomes for closed reduction and casting for
these injuries are generally excellent or good
with minimal sequelae (Eberl et al. 2008; Imatani
et al. 1996). Even patients with Galeazzi fractures
that are misdiagnosed initially and treated as sim-
ple radial shaft or distal radius fractures tend to
regain full range of motion and suffer no long-
term disability (Eberl et al. 2008). A minority of
patients treated without surgery may lose some
terminal motion (usually around 10 ) at the
50 Galeazzi and Essex Lopresti Injuries 1113

Fig. 3 (a) Lateral

radiograph of a greenstick
fracture of the radius with a
dorsal DRUJ dislocation
treated with closed
reduction and casting with
(b) radiographic union
noted at 3 months and a
clinically stable DRUJ with
full ROM (Photo courtesy
of Kevin J. Little, MD)

Operative Treatment for Galeazzi

Fractures

Indications/Contraindications
Surgical treatment is indicated for a subset of
pediatric Galeazzi fractures who fail initial closed
reduction attempts. Open fracture/dislocations,
injuries treated closed that experience loss of
reduction or are unable to be anatomically
reduced primarily, or those fractures in skeletally
mature individuals are indications for operative
intervention.

Fig. 4 Clinical photograph of a patient with an irreducible
dorsal DRUJ dislocation. Note the ulnar head (black
arrow) subcutaneously with the extensor retinaculum
(white arrow) blocking reduction (Photo courtesy of
Kevin J. Little, MD)
wrist, most often terminal supination or wrist
extension (Imatani et al. 1996; Letts and Rowhani
1993). Additionally, one series reported a 10 % rate
of subjective occasional mild weakness and pain in
nonoperatively treated patients (Eberl et al. 2008).
Surgical Procedure
The patient is placed under general anesthesia and
placed on a regular operating table with a hand
table attached (Table 4). Care is taken to position
the patient as close to the hand table as possible to
maximize the amount of the patients upper
extremity located on the hand table. A non-sterile
tourniquet is used. Prior to skin incision, the sur-
geon should attempt to reduce the fracture in the
manner mentioned in the previous section. If ana-
tomic reduction cannot be obtained, open reduction
is necessary. The arm is exsanguinated with an
1114
Table 4 Operative procedure
Operative xation of Galeazzi fractures
Preoperative planning
OR table Regular OR bed
Position Supine with arm on hand table
Fluoroscopy Mini-C arm coming in from the end of
location the hand table
Equipment Small fragment xation set. Kirschner
wires. Flexible nails
Tourniquet Unsterile tourniquet based on the
patients arm circumference
Table 5 Surgical checklist for operative treatment of
Galeazzi fractures
Open reduction and internal xation for Galeazzi
fractures
OR table: standard with arm table
Position/positioning aids: supine with arm table without
legs
Fluoroscopy location: lateral from end of arm table
Equipment: small or mini fragment plates, exible
intramedullary nails of appropriate size as was measured
preoperatively
Tourniquet: non-sterile to brachium
Esmarch bandage, and the pneumatic tourniquet
is then inated. The recommended tourniquet
pressure in children is 50100 mmHg above sys-
tolic pressure (Table 5).
Reduction and Fixation of the Radius
Plate Fixation
Anatomic open reduction of the radius is
performed rst. A standard volar approach of
Henry is used for the radius fracture. The majority
of these fractures are located at the junction of the
middle and distal thirds of the radius, so the inter-
val commonly exploited to access the fracture is
the internervous plane between the brachioradialis
(BR) and the exor carpi radialis (FCR). Fluoros-
copy is used to localize the fracture, and an
810 cm incision is made centered about the frac-
ture site on the volar-radial side of the forearm
along a line drawn from just lateral to the biceps
tendon at the elbow proximally to the radial sty-
loid distally. The interval between the BR and
K.J. Little et al.
Table 6 Surgical steps for plate fixation
Open reduction and internal xation for Galeazzi
fractures with plate xation
Volar approach along FCR tendon
Retract FCR ulnarly and incise subsheath of FCR
Dissect FPL ulnarly and expose interval between radial
artery and FCR
Dissect FPL and PQ off radius shaft subperiosteally to
expose fracture
Precontour plate to t anatomic bow of radius
Lag screw across fracture if appropriate
Apply plate in compression mode if no lag screw possible
Irrigate wound
Layered closure
Apply above-elbow cast or splint based on edema and
risks of swelling
FCR is identied, and the fascia between the two
is incised longitudinally allowing for radial retrac-
tion of the BR and the supercial branch of the
radial nerve on its undersurface. The FCR can be
retracted ulnarly along with the radial artery. In
the distal forearm, the pronator quadratus
(PQ) and the exor pollicis longus (FPL) are
encountered deep in this interval. With the fore-
arm supinated, the lateral aspect of the PQ may be
released from the radius to allow for ulnar retrac-
tion of the PQ and FPL and exposure of the
fracture site. It should be noted that in many
cases the fracture will have traumatically dis-
sected the tissue, and in this case, the fracture
site may be accessed through the interval created
by the traumatic dissection (Table 6).
Upon exposure of the fracture site, the fracture
ends are irrigated and debrided of any hematoma.
A pointed reduction clamp or manual force may
then be used to anatomically reduce the fracture
under direct visualization. Anatomic reduction,
relying on cortical keys when present, is important
in neutralizing potential deforming forces on the
DRUJ, which could negatively impact its stable
reduction.
Plate xation is then undertaken using a
3.5 mm small frag dynamic compression plate
which has been precontoured to t the patients
anatomy. In younger patients with smaller diame-
ter bone, exible nail insertion is ideal but may not
be possible to restore anatomical alignment.
50 Galeazzi and Essex Lopresti Injuries
Fig. 5 (a) AP and (b)
lateral radiographs
following ORIF of the same
Galeazzi fracture depicted
in Fig. 2 (Photo courtesy of
Kevin J. Little, MD)
In these cases a 2.7 mm Mini-Frag plate or
2.4 mm Modular Hand plate can be used with
similar results. Oblique fractures may be amenable
to initial lag screw xation followed by neutraliza-
tion plating, while transverse fractures are appro-
priately treated with compression plating (Fig. 5).
At least six cortices of screw purchase proximal
and distal to the fracture site are customary. The
wound is thoroughly irrigated. The deep dermal
tissue is reapproximated with absorbable braided
suture, and the skin is closed with an absorbable
monolament running subcuticular suture.
Flexible Intramedullary Nail Fixation
In transverse fractures or those too unstable after
closed reduction for cast immobilization alone, a
exible intramedullary nail may be elected for
fracture xation in skeletally immature patients.
To insert the nail, a 0.51 cm incision is made
either over the volar-radial lip of the radius or just
proximal to Listers tubercle. There is a higher rate
of complications associated with the dorsal entry
point, which is typically related to extensor
pollicis longus (EPL) tendon irritation or rupture
and can easily be treated with tendon transfer.
1115
However, radial entry portal complications are
typically injuries to a branch of the radial sensory
nerve, which is difcult to treat. Fluoroscopy is
used to localize the incision just proximal to the
distal-radial physis. When utilizing a radial entry
portal, branches of the supercial radial nerve
must be protected with careful blunt dissection.
The abductor pollicis longus (APL) and extensor
pollicis brevis (EPB) tendons are identied and
retracted dorsally, and the radius cortex is identi-
ed. When utilizing the dorsal entry portal, dis-
section is carried down to Listers tubercle and the
extensor retinaculum is incised longitudinally.
The interval between the extensor carpi radialis
longus (ECRL) and EPL is used to nd the bare
area just proximal to Listers tubercle. Under uo-
roscopic visualization, a curved awl is used to
enter the intramedullary canal of the radius prox-
imal to its distal physis with care taken not to
violate the far cortex. A exible nail with a diam-
eter 60 % less than the diameter of the isthmus of
the radius (determined from preoperative radio-
graphs) is then gradually bent over the course of
the nail to approximate the radial bow. The nail is
then introduced at the entry site and advanced in a
1116
distal-to-proximal direction to the level of the frac-
ture under uoroscopic imaging. The radius frac-
ture is manipulated with manual force and traction
until the desired reduction can be obtained and the
nail passed into the proximal medullary canal. If
closed reduction is not possible, a limited exposure
through a volar approach of Henry is employed and
the reduction is held with a pointed reduction
clamp or towel clamp while the exible nail can
be advanced across the fracture site.
Once across the fracture site, the nail is
advanced to a point just distal to the proximal
radial physis. The nail is cut subcutaneously and
supercial to the fascia and tendons to facilitate
later removal. The wound is irrigated and the skin
is closed with a running, absorbable, subcuticular
monolament suture (Table 7). Nail removal is
advocated at 6 months postoperatively when
bridging callus is noted on 4/4 cortices.
DRUJ Operative Treatment
Following xation of the radius fracture, the
DRUJ should be assessed clinically and with
multiplanar uoroscopic imaging. If the DRUJ
appears well reduced and is stable to stress testing,
the patient can be immobilized in the position
of maximal stability of the dislocation pattern,
which may be neutral, pronation, or supination
depending on the injury.
If the patients DRUJ remains dislocated or
there remains a displaced distal ulnar physeal
fracture, there is likely soft tissue interposition
that is blocking its reduction. This requires open
reduction of the DRUJ or the distal ulna physeal
fracture and possible xation if, after reduction,
the joint remains unstable and easily subluxatable
or dislocatable.
The DRUJ can be approached through a dorsal
incision overlying the fth extensor compartment.
Care is taken to avoid the dorsal sensory branch of
the ulnar nerve, and the fth extensor compart-
ment is opened longitudinally. The extensor digiti
minimi tendon is retracted ulnarly, and the oor
of the compartment is incised longitudinally.
Capsular aps are then raised exposing the
DRUJ. The DRUJ can then be reduced under
K.J. Little et al.
Table 7 Surgical steps for flexible nail insertion
Operative xation for Galeazzi fractures with exible
nail insertion
Using uoroscopy, identify the physis and mark just
proximal to physis
Make a 0.51 cm incision at either (A) Listers tubercle or
(B) over the volar lip of the radius between the radial
artery and rst compartment
Carefully dissect bluntly to the fascia, and either
(A) identify the interval between the EPL and ECRB
tendons at a bare area proximal to Listers tubercle or
(B) protect supercial radial nerves and identify the bare
area between the radial artery and rst compartment on
the volar lip of the radius
Using uoroscopy, use a curved awl to penetrate the
cortex and angle proximally, taking care not to disturb the
physis
Choose a nail with a diameter 60 % less than the inner
diameter at the radius isthmus
Precontour the nail with a large C-shape bend in the
direction of the pointed tip
Using gentle twisting motion or a mallet, advance the nail
to the fracture
Manually reduce the fracture under uoroscopic
visualization
If unable to pass the nail after 3 tries, proceed to open
reduction as in Table 5
Pass the nail proximally until just distal to radial neck
physis, rotating the nail to match the normal radial bow
Verify anatomic reduction and DRUJ stability clinically
and radiographically
Irrigate wound and close the wound with absorbable,
buried sutures
direct visualization. Often the traumatic injury
creates a dissection plane along this interval, and
care must be taken not to injure structures that are
not in an anatomic location due to the DRUJ
dislocation. In the event that extensor tendons,
capsule, or extensor retinaculum are blocking
the reduction of the joint (Fig. 4), these can be
extracted through this approach to allow for
reduction. If a distal ulnar physeal fracture is
present, infolded periosteum can block the reduc-
tion of the physeal injury (Landfried et al. 1991).
This can be manually extracted with a Freer ele-
vator or forceps. The distal ulnar epiphysis can
then be anatomically reduced.
After reduction of the DRUJ or the distal
ulnar epiphysis, one or two appropriately sized
Kirschner wires may be placed through the skin
50 Galeazzi and Essex Lopresti Injuries
Table 8 Surgical steps for open DRUJ reduction
Operative xation for irreducible distal radioulnar joint
dislocation
Using uoroscopy, examine and stress the DRUJ to
identify an irreducible dislocation or physeal fracture
Make a 35 cm incision longitudinally over the 5th dorsal
compartment
Bluntly dissect down to the extensor digiti minimi
(EDM) tendon and release it from the extensor
retinaculum
Retract the EDM ulnarly and incise the oor of the
compartment longitudinally until the TFCC is identied
and the distal margin
Transversely incise the capsule just proximal to the
TFCC to create a triangular ap to expose the DRUJ
Identify and release the soft tissue structure blocking
reduction
Reduce the DRUJ or physeal fracture
Unstable SH 3 fractures of the ulnar styloid may need
xation with a tension band or headless compression
screw if persistent DRUJ instability is identied
Irrigate the joint and suture the capsule closed
Ensure DRUJ stability throughout pronosupination
Leave the EDM tendon outside the extensor retinaculum
during layered closure
Close the retinaculum and skin
Immobilize with above-elbow cast or splint
outside of the dorsal incision to secure the joint or
fracture. This is done under multiplanar uoro-
scopic guidance. Fixation is not always required
once the block to reduction is removed and the
DRUJ is able to be reduced (Table 8).
Postoperative Immobilization
An above-elbow plaster splint is applied follow-
ing wound closure with absorbable monola-
ment suture. This can be a sugar-tong-type
splint to immobilize the forearm in the most
stable rotational position. Stability of the DRUJ
in neutral, pronation, or supination depends on
the initial injury and on intraoperative uoro-
scopic assessment of stability following fracture
xation. This splint is overwrapped with ber-
glass at the rst postoperative visit, approxi-
mately 1 week following the operation. Films
are obtained at this visit to verify maintenance
of reduction. The overwrapped splint is left in
1117
Table 9 Postoperative protocol for Galeazzi fractures
Operative treatment for Galeazzi injuries
Postoperative protocol
Type of immobilization: above-elbow cast or sugar-tong
splint
Duration of immobilization: 46 weeks based on fracture
healing
Rehab protocol: 46 weeks AROM, 68 weeks add
AAROM, 812 weeks add PROM. At 1012 weeks, one
can add strengthening based on return of motion
Return to sport: may return with forearm brace at
12 weeks for upper-extremity contact. Full return at
6 months
place for four additional weeks at which time it is
removed and additional lms are obtained.
With closed treatment of Galeazzi fractures, an
above-elbow cast is maintained for 4 weeks to
immobilize forearm rotation followed by a
below-elbow cast when possible for an additional
2 weeks. Following operative xation of the
radius, a cast or splint can be discontinued once
sufcient bony healing is obtained to allow for
gentle passive ROM exercise, typically in 46
weeks. Once osseous union is conrmed with
radiographs, gentle active ROM exercises can be
initiated, but full-time brace wear, either with a
forearm splint or Sarmiento brace, is recommended
for an additional 6 weeks to limit the risks of
refracture. Active assist and passive ROM exer-
cises are generally initiated at 8 weeks after surgery
or injury, followed by strengthening at 1012
weeks for patients who have obtained full active
and passive ROM to the arm and wrist. After
12 weeks bracing is discontinued unless the patient
wishes to participate in high-contact, upper-
extremity activities such as football, basketball, or
gymnastics, where the brace should be worn for an
additional 3 months or more (Table 9).
Surgical Treatment Outcomes
There are limited outcome data on surgical treat-
ment of pediatric Galeazzi fractures. Most studies
report normal postoperative forearm, wrist, and
hand functions in patients treated operatively
(Imatani et al. 1996). In a small percentage of
patients, slightly limited wrist extension is reported
1118
(Letts and Rowhani 1993). Overall, our experi-
ence with surgical xation is that results are gen-
erally good with minimal long-term sequelae.
Preferred Treatment
Nearly all pediatric Galeazzi fractures deserve an
attempt at nonoperative management. Even frac-
tures that will be treated operatively should
undergo an initial closed reduction attempt and
splinting. However, reduction attempts should be
limited to avoid damage to the distal ulnar physis
in cases in which a distal ulnar physeal fracture is
present. Initial immobilization should be in a
sugar-tong (above-elbow) splint to accommodate
initial soft tissue swelling and to immobilize fore-
arm rotation. Weekly radiographs for 3 weeks
following the injury allow for the detection of
any loss of reduction that might then necessitate
intervention. This should be followed by long-
arm casting for a total of 6 weeks post-injury
until both the radius fracture and the DRUJ injury
have healed. In patients with a distal ulnar physeal
injury, radiographic growth checks should be
obtained at 3-month intervals during the rst
year to ensure adequate resumption of growth
via a symmetric Park-Harris line on the distal
ulna or via a consistent ulnar variance in the
presence of radial growth.
If anatomic reduction cannot be obtained with
nonoperative treatment, surgical treatment is nec-
essary. Transverse radial shaft fractures can often
be managed best with a exible intramedullary
nail, while oblique fractures, which are length
unstable, are best treated with open reduction
and plate xation. Following reduction of the
radius, it is uncommon for the DRUJ to remain
unreduced. If despite anatomic reduction of the
radius, the DRUJ does remain unreduced, a dorsal
exposure of the DRUJ can aid in visualization and
extraction of any interposed soft tissue that may be
obstructing reduction. Fixation of the DRUJ is often
not necessary following reduction, but the joint
should be interrogated with multiplanar uoroscopic
images to ensure that the DRUJ remains stably
reduced. Postoperative immobilization is accom-
plished with an above-elbow sugar-tong splint.
K.J. Little et al.
Above-elbow immobilization is continued for 46
weeks postoperatively.
Surgical Pitfalls and Prevention
Patients with apex volar angulation and volar
dislocation should be immobilized in pronation,
whereas patients with apex dorsal angulation and
dorsal DRUJ dislocation should be immobilized
in supination. Fractures involving the distal
metadiaphyseal radius are prone to angulation
with intramedullary rods due to the proximity of
the fracture to the entry portal. As such, in patients
with these distal injuries, the surgeon should have
a low threshold to proceed to open reduction and
internal xation of the fracture and appropriately
counsel the patient and family preoperatively. In
patients with irreducible DRUJ dislocations, the
distal ulna typically lies subcutaneously and can
easily trap neurovascular structures in the super-
cial subcutaneous layers. Careful blunt dissection
should be performed to ensure that these struc-
tures are not sharply divided during the skin
incision.
Essex-Lopresti injuries are rare in childhood
and usually present only after radial head resec-
tion with radial shortening. The clinician should
attempt to salvage the radial head in all pediatric
patients with radial head or neck fractures to
ensure the radial buttress can prevent proximal
migration and signicant pain. Additionally, in
pediatric patients, stump overgrowth of the prox-
imal radius can lead to signicant radiocapitellar
arthropathy following radial head resection
(Table 10).
Management of Complications
Fortunately, complications are somewhat rare in
the treatment of pediatric patients. The most sig-
nicant complication noted is a loss of reduction
seen in 15 % of patients following closed treat-
ment of injuries. This can be managed by repeat
closed reduction or surgical xation as indicated
by the amount of displacement and the patients
age. In patients with a distal ulna physeal fracture,
50 Galeazzi and Essex Lopresti Injuries 1119

ulnar growth arrest can occur in up to 50 % of epiphysiodesis is performed to prevent overgrowth

patients (Fig. 6). This complication is best man-
aged by anticipating physeal arrest and monitoring
wrist radiographs every 3 months. If an asymptom-
atic growth arrest is identied, physeal imaging
with an MRI is preferred, and distal radius
Table 10 Pitfalls and prevention strategies for Galeazzi
fractures
Galeazzi fractures
Pitfalls and prevention strategies
Tented neurovascular Carefully make a skin
structures can be present incision down to the
over the prominent, subcutaneous layer and
dislocated ulnar head then bluntly dissect to
isolate ulnar nerve and
artery volarly or dorsal
sensory nerves dorsally
Distal-radial shaft Proceed to plate xation if
fractures may be unstable this occurs
after intramedullary Ensure all appropriate
xation implants are available
prior to case
Intramedullary nail will Start with a nail with a
not t down the proximal diameter no greater than
radius shaft 60 % of the internal
diameter of the isthmus of
the radius
Straighten the pre-bent tip
of the nail so that it may
pass easily
Ulnar growth arrest is Follow longitudinal
common following distal growth until evidence of
ulnar physeal injury ulnar growth or growth
arrest occurs
and ulnar tethering. In symptomatic patients, wrist
arthroscopy and possible step cut ulnar lengthening
can be performed to restore ulnar length in addition
to distal radius epiphysiodesis.
Pin tract infections can occur in patients where
the DRUJ has been stabilized with percutaneous
pins. These typically resolve with oral antibiotics
and, if necessary, pin removal. Deep infections are
rare and should be treated with operative debride-
ment and hardware removal if the fracture
has healed. In closed Galeazzi fractures with
neurovascular injury, the nerve injury is typically
a neurapraxia and will resolve with observation.
Summary and Future Directions
Proper education of orthopedic and emergency
room physicians can lead to early diagnosis and
treatment of Galeazzi and Essex-Lopresti injuries.
Once anatomic alignment has been restored,
healing of these injuries is typically routine with
minimal long-term complications. Future direc-
tions in treatment may include strategies to pre-
vent reoperation for hardware removal or to limit
complications inherent to operative treatment.
Additionally, validated outcome measurements
should be created to appropriately measure return
of forearm, wrist, and hand functions, especially
in terms of forearm rotation, as well as incorporate
patient goals and satisfaction.

Fig. 6 (a) Lateral

radiograph of a patient with
a volar Galeazzi equivalent
fracture and distal ulnar
physeal fracture with
complete displacement. (b)
1 year following closed
treatment, the fracture
healed but resulted in a
distal ulnar physeal arrest.
Note the Park-Harris line
(white arrow) on the distal
radius implying growth of
the radius without matching
ulnar growth (Photo courtesy
of Kevin J. Little, MD)
1120
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 Supracondylar Humerus Fracture
51
Afamefuna Nduaguba and John Flynn

Contents Open Reduction and Percutaneous Pinning

of Supracondylar Humerus Fracture . . . . . . . . . . . . . . . 1131
Introduction to Supracondylar Humerus Management of Complications . . . . . . . . . . . . . . . . . . . . . 1133
Fracture . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1122 Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1135
Pathoanatomy and Applied Anatomy References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1135
Relating to Supracondylar Humerus
Fracture . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1122
Assessment of Supracondylar Humerus
Fracture . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1123
Signs and Symptoms of Supracondylar Humerus
Fracture . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1123
Supracondylar Humerus Fracture Imaging
and Other Diagnostic Studies . . . . . . . . . . . . . . . . . . . . . . 1124
Supracondylar Humerus Fracture Classication . . . 1125
Supracondylar Humerus Fracture
Outcome Tools . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1126
Supracondylar Humerus Fracture Treatment
Options . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1126
Nonoperative Management of Supracondylar
Humerus Fracture . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1127
Operative Treatment of Supracondylar
Humerus Fracture . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1128
Closed Reduction and Percutaneous Pinning
of Supracondylar Humerus Fracture . . . . . . . . . . . . . . . 1128
For Type IV Fractures or Flexion-Type
Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1131

A. Nduaguba (*)
Childrens Hospital of Philadelphia, Philadelphia, PA, USA
e-mail: afam@hms.harvard.edu
J. Flynn
Department of Orthopaedic Surgery, Childrens Hospital of
Philadelphia, Philadelphia, PA, USA
e-mail: ynnj@email.chop.edu

# Springer Science+Business Media New York 2015 1121

J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_52
1122
Abstract
Supracondylar humerus fractures are the most
common elbow fractures in children. While
nondisplaced type I supracondylar fractures
can be managed nonoperatively with reduction
and casting, operative xation with closed
reduction and percutaneous pinning (CRPP)
is indicated for most displaced injuries (Types
II, III, and IV). Two or three lateral pins are
usually sufcient to stabilize most fractures;
however, in very rare cases of persistent insta-
bility after third lateral pin, a medial pin may be
required. Consideration of management of
supracondylar humerus injuries should include
a thorough evaluation of limb perfusion as this
has consequences for treatment. Limbs that
regain perfusion but remain pulseless after oper-
ative xation can be observed for an additional
48 h. But limbs that remain poorly perfused
require urgent open exploration. Surgicalmana-
gement of supracondylar humerus fractures has
good outcomes and very low complication rates.
Introduction to Supracondylar
Humerus Fracture
Supracondylar humerus fractures are the most
common elbow fractures in the pediatric popula-
tion (Cheng et al. 1999; Otsuka 1997). They
account for two-thirds of all elbow injuries in
children (Kasser and Beaty 2006) and represent
16 % of all pediatric fractures (Cheng and Shen
1993). These fractures may occur in association
with ipsilateral diaphyseal forearm fractures,
neurovascular complications, and/or compartment
syndrome (Dhoju et al. 2011). Supracondylar frac-
tures of the humerus occur equally in girls and
boys, more commonly between 5 and 7 years of
age (Cheng et al. 2001; Farnsworth et al. 1998;
Omid 2008).
Current evidence supports surgical xation for
management of Gartland type II and III supra-
condylar fractures; (Abzug and Herman 2012;
Howard et al. 2012; Skaggs et al. 2004) however,
A. Nduaguba and J. Flynn
controversies remain regarding the timing of treat-
ment and management of complications.
Pathoanatomy and Applied Anatomy
Relating to Supracondylar Humerus
Fracture
The increased incidence of supracondylar frac-
tures in skeletally immature children compared
to adults is likely due to maximum ligamentous
laxity at this age and the thinner cortex surround-
ing the supracondylar area (Brubacher and Dodds
2008; Nork et al. 1999; Fig. 1) which makes the
distal humerus more fragile and predisposes it to
fracture. The distal humerus is made up of the
medial and lateral columns, which are connected
by a thin segment of bone. This segment of bone is
even thinner at the olecranon fossa (posterior
humerus) and the radial and coronoid fossa (ante-
rior humerus), and while allowing for full range of
motion at the elbow without neurovascular
impingement, it is especially weak and thus at
high risk for fracture.
Depending on the direction of displacement of
the distal humerus fragment, supracondylar
fractures can be classied into extension and
exion types. Extension-type fractures account
for 9799 % of all supracondylar humerus frac-
tures (Mahan et al. 2007) and are usually due to a
fall on an outstretched hand with the elbow in full
extension. A typical injury mechanism is a fall
from playground equipment onto the
non-dominant hand (usually the left) with the
elbow in hyperextension to brace the fall. In this
type of injury, the distal fragment is displaced
posteriorly, thus leading to an extension-type frac-
ture. In contrast, exion-type supracondylar frac-
tures are most commonly caused by a direct blow
to the elbow with the arm in exion. In this sce-
nario, the distal fragment is displaced anteriorly.
Flexion-type supracondylar fractures are rare and
therefore have not been as extensively character-
ized as extension-type fractures.
In extension-type supracondylar fractures, the
elbow is locked in hyperextension, which engages
51 Supracondylar Humerus Fracture
Fig. 1 Lateral view of the distal humerus. Note the thin
bone separating the coronoid fossa (anterior) from the
olecranon fossa (posterior) (Courtesy of Shriners Hospital
for Children, Philadelphia)
the olecranon in the olecranon fossa of the poste-
rior humerus. Linear forces transmitted to the
anterior capsule provide bending forces against
the olecranon, which now serves as a fulcrum. In
this type of supracondylar fracture, the proximal
segment is often displaced anteriorly, and the
anterior periosteum is almost always torn (Skaggs
and Flynn 2010). The distal fragment can be either
displaced posteromedially (in 75 % of cases) or
posterolaterally. This distinction of direction has
implications for whether the arm should be
pronated during reduction. In posteromedially
displaced fractures, the medial periosteum remains
intact. Applying tension on medial periosteum with
the forearm pronated closes this hinge corrects
valgus malalignment and stabilizes the fracture.
In contrast, posterolaterally displaced fractures
have a torn medial periosteum. Pronation will fur-
ther destabilize these fractures; thus, supination is
better at aiding in reduction. The key take home
1123
point is that not all extension-type supracondylar
fractures should be reduced with the forearm
pronated.
In Gartland type IV fractures, both the anterior
and posterior periosteum are disrupted. These
fractures are unstable in both extension and ex-
ion and require a different management.
Assessment of Supracondylar
Humerus Fracture
Signs and Symptoms of Supracondylar
Humerus Fracture
The child with a supracondylar fracture typically
complains of elbow pain, swelling, and restricted
range of motion at the elbow following a trau-
matic event, most often a fall onto the outstretched
arm (Baratz et al. 2006). When evaluating the
patient, the entire upper extremity should be
examined thoroughly and assessed for concomi-
tant fractures, such as forearm fractures, as they
can occur in association with supracondylar frac-
tures and greatly increase the risk of compartment
syndrome (Blakemore et al. 2000). Soft-tissue
swelling, ecchymosis and skin puckering (Fig. 2)
suggest a more severe fracture pattern with the
associated possibility of a neurovascular injury
(Omid 2008).
A thorough neurologic exam must be
performed because of the high prevalence of neu-
rologic injury in association with supracondylar
fractures. Sensation should be tested in children
old enough to comply with the exam, typically
8 years of age or older. The sensory distribution of
the radial nerve (rst dorsal digital space), median
nerve (palm of the rst three digits) and ulnar
nerve (ulnar side of little nger) should be
assessed. The motor exam should assess function
of the radial nerve (nger metacarpophalangeal
(MP) extension and wrist extension), anterior
interosseous nerve (distal interphalangeal (DIP)
joint of the index/long nger and thumb inter-
phalangeal joint exion), median nerve (proxi-
mal interphalangeal (PIP) joint nger exion),
1124 A. Nduaguba and J. Flynn

Fig. 3 A posterior fat pad sign may be the only evidence

of a nondisplaced supracondylar fracture

Fig. 2 An anterior pucker sign may be present if proximal

fracture segment penetrates the brachialis muscle and other
soft tissues

and ulnar nerve (abduction and adduction of

the digits).
It is essential to assess the vascular status as the
prevalence of displaced supracondylar fractures
presenting with vascular compromise has been
reported to be as high as 20 % (Pirone
et al. 1988; Campbell et al. 1995). The vascular
status is categorized as present pulses with a warm
hand, pulseless with a warm hand, and pulseless
with a cold hand (Skaggs and Flynn 2010).
Fig. 4 The anterior humeral line (AHL) should intersect
the capitellum in a normal elbow
Supracondylar Humerus Fracture
Imaging and Other Diagnostic Studies but can be obtained for comparison to the
uninvolved elbow in order to detect minimally
Standard radiographs for supracondylar humerus displaced fractures. Computed tomography (CT)
fracture evaluation should include anteroposterior scan and magnetic resonance imaging (MRI) are
(AP) of the distal humerus (not an AP of the not routinely used.
elbow) and a true lateral of the elbow in anatomic In radiographic evaluation of supracondylar
position. Oblique views are not routinely required humeral fractures, three key parameters should be
51 Supracondylar Humerus Fracture
Fig. 5 In a displaced supracondylar humerus fracture, the
AHL is anterior to the capitellum
Fig. 6 The Baumanns angle is usually >10
assessed: (a) presence of a posterior fat pad sign
(which may be the only sign of a supracondylar
humerus fracture and thus should not be missed)
(Skaggs and Mirzayan 1999); (b) location of the
1125
anterior humeral line (AHL) in relation to the
capitellum; and (c) measurement of Baumanns
angle. The AHL is drawn down along the anterior
humeral cortex (Herman et al. 2009) and should
intersect through the middle third of the capitellum
except in children younger than 4 years of age,
where it may intersect the anterior third (Herman
et al. 2009; see Figs. 3, 4, 5, and 6).
In displaced extension-type fractures, the
AHL is usually anterior to the capitellum. The
Baumanns angle, which is the angle between the
long axis of the humeral shaft and the physeal line
of the lateral condyle, should be 10 . Baumanns
angle <9 signies a fracture in varus angulation.
Supracondylar Humerus Fracture
Classification
Several classication systems exist for supra-
condylar fractures; however, the modied Gartland
system is the most widely used (Omid 2008;
Abzug and Herman 2012; Howard et al. 2012;
Gartland 1959). The Gartland system categorizes
extension-type supracondylar fractures into three
types: Type I is a nondisplaced or minimally
displaced (<2 mm) supracondylar fracture with
an intact AHL. In Gartland type I supracondylar
fractures, often a posterior fat pad sign is the only
evidence of fracture (Fig. 7).
Gartland type II supracondylar fractures are
displaced (>2 mm) with an intact, hinged, poste-
rior periosteum (Fig. 8). In this fracture type, the
AHL is often anterior to the capitellum, but in
some mildly displaced cases, it just abuts
it. Modications by Wilkins (1984) further subdi-
vide type II fractures into subtypes A and B. Type
IIA fractures are angulated posteriorly but lack
rotational deformity. These fractures are often sta-
ble after exion reduction and in some rare cases
can be managed with nonoperatively with casting,
as long as the fracture is completely stable and
remains reduced while casted at 8090 . Type IIB
fractures still retain an intact posterior hinge but
have some degree of rotational displacement.
These fractures are generally unstable after reduc-
tion and require xation with Kirschner wires
1126
Fig. 7 Gartland type I fracture with posterior fat pad sign
and subtle cortical irregularity
Fig. 8 Gartland type II supracondylar fracture. Note that
the capitellum is posterior to the AHL, indicating that
closed reduction and pinning is indicated
(K-wires). In, Gartland type III, there is complete
displacement without any hinge and there is usually
a rotational deformity in the frontal and transverse
planes (Fig. 9). A fourth type has been recently
A. Nduaguba and J. Flynn
Fig. 9 In Gartland type III, there is complete displacement
without any meaningful cortical contact
classied by Leitch et al. for fractures that are
unstable in both exion and extension (Table 1).
Supracondylar Humerus Fracture
Outcome Tools
No outcome tools exist specically for supraco-
ndylar humerus fractures.
Supracondylar Humerus Fracture
Treatment Options
Initial management of displaced supracondylar
fractures involves elbow splinting at approxi-
mately 2040 of exion with avoidance of
tight bandaging. This position prevents vascular
compromise that may occur with excessive
exion (Mapes and Hennrikus 1998; Battaglia
et al. 2002). Upon arrival in the emergency depart-
ment, a careful and thorough physical exam
should be undertaken, followed by denitive
fracture treatment, most commonly with closed
reduction and percutaneous pin xation. Other
treatment options include simple immobilization
for nondisplaced/minimally displaced Gartland
type I fractures and open reduction and pin
xation in cases of failed closed reduction and
open fractures.
51 Supracondylar Humerus Fracture 1127

Table 1

Gartland AHL relationship to Cortex intact?

classication capitellum Stability? Recommended treatment
Type I AHL intersects Cortex intact Long arm cast immobilization 3 weeks
center of capitellum circumferentially
Type IIa AHL anterior to Posterior cortex Generally closed reduction and pinning, but some
capitellum intact, stable can be treated with cast immobilization for 3 weeks
Type IIb AHL anterior to Posterior cortex Closed reduction with pin xation
capitellum intact, rotational
instability
Type III AHL anterior to No intact cortex, Closed reduction with pin xation
capitellum unstable in extension
Type IV AHL anterior or No intact cortex Closed reduction with pin xation
posterior to Unstable in extension
capitellum and exion

Nonoperative Management Supracondylar humerus fracture

of Supracondylar Humerus Fracture Nonoperative management
Indications Contraindications
The nonoperative treatment of type I fractures Gartland type I fracture Displaced fractures in
involves short-term simple immobilization AHL transects the which the AHL is
followed by range of motion exercises. The indi- capitellum on lateral anterior to capitellum
radiograph
cation for nonoperative treatment of
The Baumann angle is Open fractures
supracondylar fractures is a Gartland type I >10 on affected side
(nondisplaced or minimally displaced <2 mm)
supracondylar fracture without evidence of nerve
injury or instability. The decision regarding
Techniques
nonoperative treatment is greatly enhanced
The patient is initially treated with a long arm
by assessment of the AHL. If, on the initial
cast with the elbow exed 8090 for
presentation lm, the AHL intersects the
3 weeks, followed by range of motion exercises
capitellum ossication center, then the fracture
(Williamson and Cole 1993; Charnley 1961;
is dened as minimally displaced, and cast
Cuomo et al. 2012). At the completion of immobi-
immobilization alone is the recommended
lization, patients can begin active range of
treatment (Skaggs and Flynn 2010). The orthope-
motion exercises and will often not require
dic surgeon should also assure that on the AP
physical therapy. A return visit for a range of
x-ray, there is not excessive medial impaction
motion check at 46 weeks post immobilization is
that could cause cubitus varus. Baumanns
typically offered.
angle should be assessed and be in the
acceptable range.
Supracondylar humerus fracture
Nonoperative management
Indications/Contraindications Elbow is casted for approximately 3 weeks at 8090 of
The indication for nonoperative treatment of exion followed by an active range of motion program.
supracondylar humerus fractures is a Gartland Return to all activities is allowed when the patient is
asymptomatic
type I supracondylar fracture.
1128
Outcomes
Nondisplaced/minimally displaced supracondylar
fractures treated with simple immobilization have
universally excellent outcomes. In a recent study,
Cuomo and associates reviewed 53 patients with
Gartland type I fractures and found intact AHL
and Baumanns angle >9 at 3 weeks follow-up
after treatment with only splint immobilization
(Cuomo et al. 2012). Ballal et al. (2008) also
reported excellent outcomes in all subjects in
their series of 40 patients.
Operative Treatment of Supracondylar
Humerus Fracture
Operative treatment consists of either closed
reduction with pin xation or, in rare circum-
stances, open reduction with pin xation.
Closed Reduction and Percutaneous
Pinning of Supracondylar Humerus
Fracture
Indications/Contraindications
Closed reduction with pin xation (CRPF) is the
preferred treatment for Gartland type II, III, and IV
supracondylar fractures. Indications for CRPF are
(1) a displaced fragment in which the capitellum is
posterior to the anterior humeral line and (2) mini-
mally displaced fragments with medial column
comminution or any malalignment in the coronal
plane, making Baumanns angle in the unaccept-
able range. Contraindications for closed reduction
with xation are (1) open fractures, (2) failed
closed reduction, and (3) a limb that remains
dysvascular after CRPF. These patients should
undergo an immediate open reduction (Table 1).
Supracondylar humerus fracture
Closed reduction and pin xation
Indications Contraindications
Displaced (Gartland type Nondisplaced or minimally
IIIV) fractures displaced fractures
Fractures with medial Open fractures requiring
column comminution debridement
Fractures with coronal
plane malalignment
A. Nduaguba and J. Flynn
Surgical Procedure: Closed Reduction
Preoperative Planning
Reduction and xation of a supracondylar fracture
is generally an urgent issue, with treatment in the
rst 24 h after injury. In circumstances when there
is either poor perfusion to the hand or in severe
fractures with median nerve injury, these fractures
are reduced and xed emergently (Abzug and
Herman 2012).
Closed reduction and percutaneous for supracondylar
humerus fracture
Preoperative planning
OR Table: standard
Position/positioning aids: Supine/radiolucent arm board
Fluoroscopy location: On same side as the surgeon;
place monitor on opposite side for easy viewing
Equipment: Basic orthopedic set, K-wires, K-wire
driver
Positioning
Supine with the C arm parallel to the table and on
the same side as the injured arm. For ease of
viewing by the surgeon, the uoroscopy unit mon-
itor should be placed on the other side of the bed,
opposite the surgeon (Fig. 10).
In children less than 3 years of age, a radiolu-
cent hand table is valuable because the upper arm
is not long enough to bring the elbow to the center
of the image intensier. Additionally, in very
unstable fractures, such as Gartland type IV frac-
tures, the radiolucent hand table is also valuable
so that the image intensier can be rotated to get a
lateral x-ray.
Surgical Approach
After prophylactic antibiotics and endotracheal
intubation, the injured hand, forearm, elbow, and
arm are prepped and draped up to the shoulder.
In type II fractures or moderate type III fractures,
reduction is performed by gentle longitudinal
realignment and then exion. In severe type III
fractures, gentle massaging of the brachialis and
biceps is performed rst, in order to free the
metaphyseal fragment. This brachialis milking
technique should be done with patience, and if
done skillfully will avoid many unnecessary
open reductions. Next, reduction in the sagittal
51 Supracondylar Humerus Fracture
plane should be attempted by slowly exing the
elbow with the non-dominant hand and at the
same time pushing forward on the olecranon
with the thumb of the dominant hand. Satisfac-
tory reduction can be assessed by exing the
childs elbow to see if the ngers can touch
Fig. 10 Patient is supine on the operating table and uo-
roscopy unit monitor is on the opposite side of the surgeon
Fig. 11 (a) Jones view of the elbow after closed reduc-
tion; (b) to obtain this view, the elbow is maximally exed
with the forearm pronated. The image is obtained by
1129
the shoulder. If the reduction is unsuccessful,
the patient will be unable to do so.
Fluoroscopy images in AP, lateral, and oblique
planes should be obtained, and successful reduc-
tion is conrmed by checking for intersection of
the capitellum by the AHL on the lateral view,
Baumanns angle 10 on the AP view and intact
medial and lateral columns on oblique views.
Highly unstable fractures may preclude moving
the arm to obtain images; instead, the uoroscopy
should be rotated to obtain images in the lateral
and oblique views.
The elbow should be held in a exed position
to obtain a Jones view as the initial image, (Fig. 11)
and then the entire arm (not the forearm) should be
rotated to obtain the lateral image of the elbow.
Once the reduction is deemed satisfactory, pin x-
ation is performed while using the Jones view.
Technique: Percutaneous Pinning
Pin xation allows for maintenance of a stable
construct without the need to secure the elbow in
excessive exion. Successful xation is achieved
by maintaining sufcient separation of the pins at
fracture site (>2 mm) while ensuring bicortical
engagement of both the lateral and medial col-
umns. Typically two smooth Kirschner wires
(K-wires) (Zimmer, Warsaw, IN) are sufcient
for Gartland type II fractures, while Gartland
type III fractures usually require three or even
four K-wires. The use of 0.062 in. K-wires is
directing the radiographic tube perpendicular to the distal
elbow in an anterior to posterior direction
1130
Fig. 12 (a) (AP view) A supracondylar fractures imaged
3 weeks after xation, showing good conguration for two
pins. Note the wide pin spread and divergence. (b)
typically adequate but smaller or larger sizes may
be used depending on the size of the child.
Prior to placement of the rst pin, the starting
point and trajectory should be assessed. This can
be done easily by holding the free K-wire against
the lateral condyle and conrming the path with
uoroscopy.
Once the starting point is conrmed, the wires
are advanced with the drill. The rst wire should
be low and somewhat transverse, often coursing
through the olecranon fossa, and should engage
the medial cortex. This is referred to as four
cortex xation. The second wire is placed in a
divergent trajectory from the rst, also engaging
the medial cortex. A third pin is added to type III
fractures or type II fractures that remain unstable
even after placement of two lateral pins.
In oblique fracture patterns that prevent a third
pin placement or fractures that remain unstable
after reduction, a medial pin is placed. To mini-
mize potential ulnar nerve damage, the elbow is
extended after placement of lateral pins. Then, the
medial pin is inserted at the medial epicondyle in a
slight posterior to anterior direction (Fig. 12).
After the wires are placed, a lateral image in full
exion is obtained. Subsequent images in 90 , 60 ,
and 30 of exion are obtained, carefully studying
the capitellum on the lateral view to assure that it
does not displace with progressive extension.
A. Nduaguba and J. Flynn
(AP view) Intraoperative uoroscopy after placing three
divergent lateral entry pins. (c) Optimal conguration for
two pins (lateral view)
Alternatively, the elbow can be brought through a
range of motion while live uoroscopy is performed
to ensure no displacement occurs. If the xation is
stable, then the AP view is checked to ensure align-
ment and stability. Static images or live uoroscopy
should be performed while a varus and valgus stress
is applied to assess stability in the coronal plane.
Following successful pin placement, the
k-wires are bent and cut to a length 12 cm
above the skin to prevent migration under skin.
The pins are then wrapped in Xeroform, and anti-
microbial Vaseline gauze, which also aids in the
prevention of pin migration. The limb is then
casted in about 7080 of exion
Closed reduction with percutaneous pinning for
supracondylar humerus fracture
Surgical steps
Perform gentle longitudinal realignment of fracture
Reduce fracture in sagittal plane
Conrm successful reduction on Jones and lateral views
Place rst k-wire lower and more transverse in distal
humerus, often engaging four cortices, including the
olecranon fossa
Place second wire divergent from the rst, engaging the
medial cortex more proximally
Assess stability in both the AP and lateral planes
Bend and cut k-wires to 12 cm above the skin. Wrap
pins in Xeroform or antimicrobial Vaseline gauze
Cast arm in 7080 of exion
51 Supracondylar Humerus Fracture
Postoperative Care
The upper limb should be elevated such that the
hand is above the heart for the rst day after
surgery. As long as the xation was stable, the
rst follow-up can be at three weeks following
the surgery. At this time, AP and lateral radio-
graphs are taken with the cast off but the pins still
in position. If the fracture is healed, and it is
universally, except in much older children, the
pins are removed and active range of motion
exercises are begun. Typically, the child returns
6 weeks postoperatively for a range of motion
check and can resume normal physical activity
afterward. Formal physical therapy is not rou-
tinely recommended for supracondylar fractures,
unless the child lacks signicant range of motion
at 68 weeks after injury.
Closed reduction with percutaneous pinning for
supracondylar humerus fracture
Postoperative protocol
Elevate upper limb above heart for about 24 h after
surgery
Follow up in 3 weeks after surgery remove cast, obtain
AP and lateral radiographs to assess reduction; remove
pins if healed
Second postoperative visit at 6 weeks postoperatively for
range of motion check. Full activities can be resumed if
asymptomatic
For Type IV Fractures or Flexion-Type
Fractures
Type IV and exion-type supracondylar fractures
are treated with the protocol described by Leitch
et al. (2006). The fracture is manually manipu-
lated into an anatomic reduction on a radiolucent
hand table. Often, the reduction is assisted by a
small towel rolled and placed behind the distal
humerus, just above the fracture line. The rst
pin is then placed using tactile sense alone.
Then, while holding the elbow steady, AP and
lateral images are obtained. If the reduction is
satisfactory, two more pins are placed in a diver-
gent pattern.
Outcomes of Closed Reduction and Percutane-
ous Pinning of Supracondylar Humerus Fractures.
1131
Many authors have noted good outcomes after
surgical xation of type II and III supracondylar
fractures (Skaggs et al. 2004; Sankar et al. 2007;
Skaggs et al. 2008). Albrektson and associates
(Skaggs et al. 2008) reviewed 189 patients with
Gartland type II fractures treated with closed
reduction and lateral pinning and reported excel-
lent outcomes, according to Flynn criteria (Flynn
et al. 1974) in 95 % of subjects. Furthermore, none
of the 189 patients had any intraoperative compli-
cations, loss of reduction, or neurological injury
following fracture reduction. Similarly, Skaggs
et al. (2004) reported excellent outcomes and no
loss of reduction at 9 weeks follow-up in 124 chil-
dren xed with only lateral entry pins. Loss of
reduction with pinning is quite uncommon.
Sankar et al. (2007) reviewed 8 of 279 patients
with loss of reduction after lateral pin xation and
found all cases to be due to technical errors and
the use of only two pins in cases where three was
likely more appropriate. While both lateral entry
only pins and cross pinning have been shown to
achieve great fracture xation, (Mahan et al. 2007;
Gaston et al. 2010) cross pinning is associated with
increased iatrogenic injury to the ulnar nerve
(Skaggs et al. 2001; Babal et al. 2010; Slobogean
et al. 2010). In a recent meta-analysis, Slobogean
et al. reviewed 39 studies including 2,639 patients
and found iatrogenic ulnar nerve injury was present
in 3.4 % of patients treated with cross pins and
in just 0.7 % of patients treated with lateral pins
alone (Slobogean et al. 2010).
Open Reduction and Percutaneous
Pinning of Supracondylar Humerus
Fracture
Indications/Contraindications
Indications for open reduction are open fractures
requiring debridement and fractures that failed
satisfactory closed reduction due to interposed
tissue. Additionally, fractures associated with
poor hand perfusion in which there is concern
about brachial artery injury are treated with open
reduction so that the artery can be explored and
managed as necessary.
1132
Supracondylar humerus fracture
Open reduction and pin xation
Indications Contraindications
Open fractures requiring
debridement
Fractures that failed initial closed
reduction
Fractures in which there is concern
about brachial artery injury
Surgical Procedure
Preoperative Planning and Positioning
Same as in closed reduction and percutaneous pin
placement.
Surgical Approach(es)
A transverse anterior approach is utilized for
open reduction as it allows for visualization of
the median nerve and the brachial artery. Typi-
cally a 45 cm transverse incision made at the
antecubital fossa is both cosmetically ideal and
also allows adequate exposure for fracture
manipulation. Following the incision, dissection
should proceed through the fascia down to the
bicipital aponeurosis. The brachial artery and
median nerve should be identied where they
lie immediately deep to bicipital aponeurosis
and medial to the biceps tendon. Next, incise
the bicipital aponeurosis taking care to avoid
damage to the brachial artery and median nerve.
The artery and nerve are removed from the frac-
ture site. Now, the fracture can be reduced by
applying posterior force on proximal fragment
with concomitant traction to forearm with the
elbow exed at 90 . Pin xation can proceed
similarly as described for closed reduction.
Open reduction with percutaneous pinning for
supracondylar humerus fracture
Surgical steps
45 cm transverse incision at the antecubital fossa
Dissect through fascia to bicipital aponeurosis. Identify
and protect median nerve and brachial artery
(continued)
A. Nduaguba and J. Flynn
Open reduction with percutaneous pinning for
supracondylar humerus fracture
Surgical steps
Attempt reduction of fracture segments
Secure reduced fracture with three divergent lateral entry
pins
Check pin placement and reduction with uoroscopy
Close incision with bioabsorbable suture
Postoperative Care
Postoperative care for supracondylar humerus
fractures managed with open reduction and per-
cutaneous pin xation proceeds similar to frac-
tures treated with closed reduction.
Outcomes of Open Reduction and Percutane-
ous Pinning of Supracondylar Humerus Fractures.
Open reduction is similarly associated with
low rates of complications. In 52 displaced frac-
tures treated with an open approach, Weiland
et al. (1978) reported excellent outcomes in all
patients. Reitman and associates (Reitman
et al. 2001) reported 75 % of patients with excel-
lent results according to Flynn criteria (Flynn
et al. 1974) and loss of motion in only 4 cases.
Flexion-Type Supracondylar Fracture
Management
Similar to extension-type injuries, exion-type
supracondylar fractures can be classied using a
modied Gartland system (Wilkins 1990) as
types I, II, and III. Like in extension-type injuries,
type I exion-type supracondylar is nondisplaced
and can be managed similarly by placing in a long
arm cast with the elbow exed to 90 for comfort.
Type II injuries are moderately displaced with an
intact anterior hinge, whereas type III injuries are
characterized by severe displacement of both the
anterior and posterior cortices. Stable type II frac-
tures with minimal angulation can be reduced and
held in place with a long arm cast with the elbow
in extension; however, unstable type II and type
III fractures usually require closed reduction and
pinning.
In exion-type supracondylar fractures, the pos-
terior periosteum is torn precluding exion of the
elbow for reduction. Rather, the elbow is extended
51 Supracondylar Humerus Fracture
so as to place tension on the anterior periosteum.
The elbow is exed to about 30 for reduction. Pin
placement is greatly aided by placing a rolled towel
just above the fracture behind the distal humerus.
Once anatomic reduction is assured, the image
intensier is rotated around the arm board to obtain
AP and lateral radiographs, in order to conrm the
reduction. The rst pin is placed using primarily
tactile sensation, penetrating the cortices as is
typical for all supracondylar humerus pinning.
If reduction and xation are maintained, two addi-
tional pins are placed as described above.
Open reduction may be indicated for exion-
type injuries that fail closed reduction.
Preferred Treatment
Type I Long arm casting with the elbow in
90 of exion for approximately 3 weeks.
Type II Closed reduction and pinning for most
type II fractures. Placement of two divergent
pins is usually adequate, but we do not hesitate
to place a third pin if there are concerns of
instability.
Type III Closed reduction and placement of
three lateral entry pins. Given that these frac-
tures are highly unstable, three lateral pins are
placed rather than two.
Type IV Treatment is utilization of the protocol
recommended by Leitch et al.
Surgical Pitfalls and Prevention
Supracondylar humerus fracture
Potential pitfalls and preventions
Potential pitfall Pearls for prevention
Iatrogenic ulnar Use lateral entry pins only
nerve injury
If a medial pin is required,
insert lateral pins rst, then
extend elbow and protect ulnar
nerve during insertion of medial pin
Pin migration Dont insert and back out pins
repeatedly during pinning.
Bend and cut k-wires leaving at
least 12 cm protruding above
the skin; cover wires with
Xeroform or antimicrobial
Vaseline gauze
(continued)
1133
Supracondylar humerus fracture
Potential pitfalls and preventions
Potential pitfall Pearls for prevention
Loss of reduction Ensure at least 2 mm pin separation
at fracture site
Make sure both pins engage the
proximal and distal segments
Use two lateral pins for type II
fractures and three for type III
fractures; if unsure do not hesitate
to insert another pin
Stress fracture after pinning to
ensure adequate stability
Compartment Avoid tight bandaging and
syndrome excessive arm exion; avoid
casting in 90 of exion
In children, an increasing
analgesia requirement is the
most sensitive indicator for
an evolving compartment
syndrome
Management of Complications
Vascular Injury
Supracondylar fractures presenting with vascular
injuries are fairly common (312 %); however,
vascular reconstruction is rarely required.
While a pulseless and well-perfused hand can
be managed urgently, a dysvascular limb
requires an emergency procedure. In most
cases, perfusion is restored after anatomic reduc-
tion and xation. If the hand remains poorly
perfused, immediate exploration and repair is
undertaken or consultation with a vascular
surgeon should occur.
For a previously well-perfused limb with
present radial pulses that subsequently looses
perfusion after reduction, urgent pin removal
and arterial exploration should be performed
to release a possible entrapped artery from
the fracture site. Treatment for well-perfused
hands that remain pulseless after reduction is
still controversial. These patients are admitted
for 48 h and monitored closely for any signs
or symptoms of an evolving compartment
syndrome.
1134 A. Nduaguba and J. Flynn

Compartment Syndrome enough at the time of detection of the infection, to

Compartment syndrome of the forearm in associa- permit pin removal without loss of reduction.
tion with supracondylar humerus fractures is esti-
mated at 0.10.5 % (Battaglia et al. 2002; Bashyal
Pin Migration
et al. 2009); however, incident rates increase to 7 %
Pin migration is estimated at 1.8 % and can be
with concomitant forearm injuries (Blakemore
prevented by leaving at least 1 cm of the K-wire
et al. 2000). In children, the classic ve Ps (pain,
above skin and protecting the skin by bending the
pallor, pulseless, paresthesias, and paralysis) are
wire at a 90 angle or by covering the wire with a
poor indicators for evolving compartment syn-
felt cover (Bashyal et al. 2009).
drome; rather increased analgesic requirement is a
more sensitive indicator (Bae et al. 2001).
In patients with suspicion of an evolving com- Loss of Reduction
partment syndrome, initial management should Loss of xation following pinning of supra-
include removal of dressings, reduction of exion condylar fractures is rare and is usually associated
to several degrees lower than 90 , and immediate with technical errors in xation. This complica-
fracture stabilization with K-wires. tion can be prevented by engaging both the
proximal and distal fragments with at least two
Neurologic Injuries pins and ensuring 2 mm of pin separation at the
The anterior interosseous nerve (4 %) is the most fracture site (Sankar et al. 2007).
commonly injured nerve in supracondylar frac-
tures, followed by the radial nerve (3 %) (Babal
Cubitus Varus
et al. 2010). Ulnar nerve injuries are rare and are
Cubitus varus occurs with malunions, typically in
usually associated with exion-type supra-
supracondylar fractures treated with casting only
condylar fractures or an iatrogenic complication
or fractures pinned in malalignment, or without
of medial pin placement. In most cases of nerve
stable pinning. Pirone and associates reported
injury, recovery is spontaneous and management
cubitus varus in 8 % of patients with fractures
generally involves observation for 22.5 months
held in place with just cast immobilization com-
(Brown and Zinar 1995). However, iatrogenic
pared to 2 % in patients who underwent pin
ulnar nerve injuries may require immediate
stabilization (Pirone et al. 1988). Traditionally
removal of the pin to allow for faster recovery of
thought to be just a cosmetic deformity, cubitus
neural function. The use of lateral entry pins as
varus can be a cause of chronic elbow pain,
opposed to crossed pins is recommended to avoid
tardy rotational instability, and additionally can
potential damage to the ulnar nerve. In the rare
increase the rate of lateral condyle fractures of
cases where medial pins become necessary, lateral
the elbow (ODriscoll et al. 2001; Abe et al.
pins should be placed rst, followed by extension
1997, 1995).
of elbow and/or a small incision performed prior
Ensuring an intact Baumanns angle after
to placement of a medial pin.
reduction and during fracture healing can prevent
this complication. In children who develop sub-
Pin Tract Infection stantial cubitus varus after supracondylar elbow
Pin tract infections after operative management of fractures, we recommend correctional elbow
supracondylar fractures are estimated at less than osteotomy. We prefer lateral closing wedge osteo-
12.5 % (Skaggs et al. 2004, 2008; Bashyal tomy of the distal humerus and pin xation through
et al. 2009; Cheng et al. 1995). Standard manage- a lateral or posterior approach using a modication
ment is removal of the pin(s) and oral antibiotics of the osteotomy technique described by Wiltse
(Skaggs et al. 2008). Typically, the fracture is stable (Skaggs et al. 2011).
51 Supracondylar Humerus Fracture
Supracondylar humerus fracture
Complication Management
Vascular injury Urgent closed reduction and
percutaneous pinning for poorly
perfused limbs
Exploration and repair or vascular
surgery consult for hands that
remain poorly perfused
Compartment Removal of dressings, reduction of
syndrome exion, and immediate fracture
stabilization with K-wires
Iatrogenic ulnar Immediate removal of pins
nerve injury
Summary
Supracondylar fractures are common fractures in
children. Gartland type I fractures are managed
nonoperatively, but displaced fractures (Gartland
type II, III and IV) are treated with closed
reduction and pinning. Surgical management
has good outcomes and very low complication
rates.
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humerus fracture malunions in children. J Child
Orthop. 2011;5:30512.
Slobogean BL, Jackman H, Tennant S, Slobogean GP,
Mulpuri K. Iatrogenic ulnar nerve injury after the sur-
gical treatment of displaced supracondylar fractures of
the humerus: number needed to harm, a systematic
review. J Pediatr Orthop. 2010;30:4306.
Weiland AJ, Meyer S, Tolo VT, Berg HL, Mueller
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tures of the humerus in children. Analysis of fty-two
cases followed for ve to fteen years. J Bone Joint
Surg Am. 1978;60:65761.
Wilkins K. Fractures and dislocations of the elbow region.
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3. Lippincott-Raven; 1984. p. 363575.
Wilkins KE. The operative management of supracondylar
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ulation and strapping in exion. Injury. 1993;24:
24952.
 Distal Articular Humerus Fractures
52
Anish G. R. Potty, Sasha Job Tharakan, and B. David Horn

Contents Fractures of the Medial Condyle

of the Humerus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1149
Intra-articular Fractures of the Distal Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1149
Humerus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1138 Pathoanatomy and Applied Anatomy . . . . . . . . . . . . . . 1150
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1138 Assessment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1150
Lateral Condyle Fracture . . . . . . . . . . . . . . . . . . . . . . . . . . . 1138 Classication . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1151
Pathoanatomy and Applied Anatomy . . . . . . . . . . . . . . 1138 Treatment Options . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1151
Assessment of Fractures Around the Elbow . . . . . . . 1139 Operative Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1152
Imaging and Other Diagnostic Tests . . . . . . . . . . . . . . . 1140 Surgical Pitfalls and Prevention . . . . . . . . . . . . . . . . . . . . 1153
Classication . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1140
Treatment Options . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1142 Fractures of the Capitellum . . . . . . . . . . . . . . . . . . . . . . 1153
Operative Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1144 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1153
Preoperative Planning . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1145 Pathoanatomy and Applied Anatomy . . . . . . . . . . . . . . 1153
Positioning . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1145 Assessment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1153
Closed Reduction and Percutaneous Pinning . . . . . . 1145 Treatment Options . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1154
Arthroscopic-Assisted Reduction and Surgical Procedure . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1155
Percutaneous Pinning . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1146
T-Condylar Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1157
Open Reduction and Internal Fixation . . . . . . . . . . . . . 1146
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1157
Reduction and Fixation of Fracture . . . . . . . . . . . . . . . . 1147
Pathoanatomy and Applied Anatomy . . . . . . . . . . . . . . 1157
Postoperative Management . . . . . . . . . . . . . . . . . . . . . . . . . 1148
Assessment of T-Condylar Fractures . . . . . . . . . . . . . . . 1158
Outcomes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1148
Classication . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1158
Pitfalls and Prevention . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1148
Treatment Options . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1158
Complications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1149
Outcomes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1159
Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1149
Operative Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1159
Surgical Technique for Open Reduction Internal
Fixation of T-Condylar Fracture . . . . . . . . . . . . . . . . . . . . 1160
Treatment-Specic Outcomes . . . . . . . . . . . . . . . . . . . . . . 1162
A.G.R. Potty (*) Surgical Pitfalls and Prevention . . . . . . . . . . . . . . . . . . . . 1162
The Childrens Hospital of Philadelphia, Philadelphia, Management of Complications . . . . . . . . . . . . . . . . . . . . . 1163
PA, USA Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1163
e-mail: pottya@email.chop.edu; anishpotty@gmail.com
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1163
S.J. Tharakan
Department of Surgery, Childrens Hospital of
Philadelphia, Philadelphia, PA, USA
e-mail: tharakans@email.chop.edu; sasha@tharakan.ch
B.D. Horn
Perelman School of Medicine, The Childrens Hospital of
Philadelphia, University of Pennsylvania, Philadelphia,
PA, USA
e-mail: hornd@email.chop.edu

# Springer Science Business Media New York (outside the USA) 2015 1137
J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_53
1138
Abstract
Distal humeral articular fractures include very
common fractures such as lateral condyle and
intercondylar fractures as well as uncommon
fractures such as medial condyle and capitellum
shear fractures. While the principles of orthope-
dic management remain the same, recent
advances in understanding the blood supply
and techniques of xation have been rened to
obtain improved outcomes. The various surgical
techniques and thought processes in managing
these fractures will be highlighted, as well as the
potential pitfalls encountered.
Intra-articular Fractures of the Distal
Humerus
Introduction
Distal humeral articular fractures may include any
articulating part of the distal humerus and frac-
tures of the metaphysis, physis, or epiphysis.
Thus, fractures about the lateral condyle, medial
condyle, intercondylar region, trochlea, and
capitellum are part of this broad categorization.
Distal humeral physeal separation may also be
included if the articular surface is involved.
Medial epicondyle fracture is an apophyseal frac-
ture with the possibility of the fragment being
located in the elbow joint. However, this is not a
true articular surface fracture. All of these frac-
tures can occur in isolation or in combination with
other fractures.
Lateral Condyle Fracture
Lateral condyle fractures account for 1015 % of
all pediatric elbow fractures and are the second
most common fracture occurring about the pedi-
atric distal humerus, with supracondylar fractures
being the most common (Landin 1992; Milch
1964). Historically, lateral condyle fracture was
associated with signicant complications such as
nonunion and malunion, primarily due to the diag-
nosis being delayed or missed altogether.
A.G. Potty et al.
Lateral condyle fractures usually present in
children between 5 and 8 years of age with a
peak incidence in 6-year-old children. Interest-
ingly, obese children seem to have a higher risk
of sustaining lateral condyle fractures compared
with supracondylar fractures when they have frac-
tures around the elbow (Fornari et al. 2013). Typ-
ically, lateral condyle fractures are isolated
injuries, but associated fractures of the elbow
(with or without a dislocation) or forearm may
also occur.
Pathoanatomy and Applied Anatomy
The understanding of the appearance of the ossi-
cation centers is important in order to properly
diagnose and manage fractures around the elbow.
Most complications are from missing a diagnosis
and lack of clear understanding of the ossication
patterns about the elbow, which can in turn lead to
missing the diagnosis or an improper diagnosis.
The ossication of the humerus appears in intra-
uterine life, and by full term ossication extends
into the condyles. The ossic nucleus of the
capitellum appears by 1 year of age but can be
delayed until 2 years of age (Cheng et al. 1998).
The radial head ossic nucleus is present around
3 years of age and the medial epicondyle at age
5. The trochlea appears by age 7 and the lateral
epicondyle is visible at 12 years of age. The sec-
ondary ossication centers around the elbow can
be remembered by the mnemonic CRITOE
(capitellum, radial head, internal (medial)
epicondyle, trochlea, olecranon, and external
(lateral) epicondyle). These ossication centers
eventually fuse together by 1416 years of age.
The physis of the capitellum is wider posteriorly
and can be easily mistaken for a fracture in lateral
elbow radiographs (Brodeur et al. 1983).
Two mechanisms of injury have been proposed
for lateral condyle fractures: the rst is an avulsion
fracture of the lateral condyle that occurs second-
ary to the pull of the extensor carpi radialis longus,
the extensor carpi radialis brevis, and the
brachioradialis muscles. The second proposed
mechanism is that fractures occur due to the
impaction of the radial head on the capitellum
52 Distal Articular Humerus Fractures
Fig. 1 Green wavy line
represents growth plate.
Red line outlines the joint
capsule
such as would occur from a fall on an outstretched
hand. Lateral condyle fractures usually begin at
the posterior lateral corner of the metaphysis and
propagate through the physis. These fractures may
be incomplete or may continue through the troch-
lea or the capitellum into the elbow joint (Fig. 1).
Haraldsson described in children two crucial
blood vessels that enter the lateral condyle
through the posterior portion close to the lateral
capsular origin and the origin of the anconeus
(Haraldsson 1959). These vessels are end arteries
with an anastomosis in the ossic nucleus and do
not communicate with the metaphyseal or the
epiphyseal vessels. Hence, during surgery, pre-
serving the posterolateral soft tissue attachment,
which provides the blood supply to the fragment
through the attachment of the lateral collateral
ligament and the extensor carpi radialis longus
and brevis muscles, is critical.
Assessment of Fractures Around
the Elbow
Thorough history and physical examination is key
in order to identify lateral condyle fractures. Chil-
dren will typically present with a history of a fall
on an outstretched hand. This is the most common
mechanism of injury for lateral condyle as well as
supracondylar fractures of the humerus. Axial and
valgus loading forces from the fall force the frac-
ture to propagate into the lateral condyle. Avul-
sion of the lateral condyle may also occur if an
1139
Table 1 Differential diagnosis of lateral condyle fracture
Bony injury Soft tissue injury
Supracondylar fracture Sprain/lateral
collateral ligament
Radial neck fracture Contusion or
bruising
Medial epicondyle fracture Nonaccidental
trauma
Olecranon fracture/Monteggia
fracture dislocation
Trochlea and capitellum fracture
Physeal separation
Elbow dislocation
adducted arm is forcefully supinated. Pain and
swelling of the elbow is the most common pre-
sentation. The common differential diagnosis of
injures around the elbow is listed in the Table 1.
While examining children with an elbow
injury, it can be challenging to obtain a complete
examination in one encounter, and therefore, care-
ful attention should be given to any pertinent
positive ndings. The examiner should look for
deformity, ecchymosis, and swelling around the
elbow. Any associated bleeding seen around the
elbow should be carefully assessed for the pres-
ence of an open fracture. Increased swelling might
suggest increased soft tissue damage and later
displacement of the fracture. The distal humerus
and its bony prominences should be carefully
palpated while asking and looking for a pain
response. It is often better to start examining the
noninjured side or the shoulder of the injured side
1140
as this may help ease the fears of an injured child
and help build trust with the patient and their
parents. Subtle nondisplaced fractures can be eas-
ily missed on radiographs, and clinical examina-
tion is critical in recognizing them. Often, crepitus
can be felt while palpating the humerus, which
can be indicative of an underlying fracture. Some-
times, a valgus or varus force has to be applied to
appreciate the crepitus. It is recommended to per-
form this maneuver under general anesthesia, as
this may be very painful.
A complete neurovascular examination of the
limb should be performed with particular focus on
examining the sensory and motor components of
the median nerve, anterior interosseous nerve,
radial nerve, and ulnar nerve. All equivocal or
positive ndings should be documented. It is
important to realize that the sensory examination
is generally not reliable in children less than
8 years of age. It is often difcult to obtain all of
the necessary examination components in the rst
encounter, and therefore, multiple examinations
performed at various time points by the same
person are necessary to complete a complete ini-
tial examination and detect any deterioration of
nerve function over time. The next step of the
physical examination is to move the elbow joint
actively and passively to check the range of move-
ment, as pain permits. Often, this is not possible in
the emergency department and might have to be
done in a setting when the child is sedated or
anesthetized. Finally, it is imperative to examine
the joint above and below to avoid missing any
concomitant injuries.
Imaging and Other Diagnostic Tests
Radiographic interpretation of elbow fractures in
children can be challenging because of the chang-
ing ossication pattern as they grow. It is impor-
tant to obtain standard anteroposterior (AP) and
lateral radiographs centered about the elbow, as
well as additional AP and lateral radiographs that
include the joint above and below. Often it is not
possible to fully extend the elbow for the standard
AP view as the fracture may prevent full extension
of motion and may be limited by pain. In these
A.G. Potty et al.
scenarios, a Jones view, a shoot-through AP with
the elbow exed, may be helpful as it gives a good
view of the distal humerus despite the overlapping
radius and ulna. An internal oblique view should
also be obtained as this best demonstrates the
amount of displacement present in lateral condyle
fractures (Song et al. 2007). A 20 tilt view, with
the elbow elevated so that the arm is exed 20 ,
has also been reported as benecial in assessing
fracture displacement in lateral humeral condyle
fractures (Imada et al. 2010). Comparison radio-
graphs should only be obtained in extreme set-
tings, as Kissoon et al. demonstrated that routine
comparison radiographs did not increase the accu-
racy of diagnosis (Kissoon et al. 1995).
Advanced imaging such as magnetic reso-
nance imaging (MRI) and computed tomography
(CT) is not routinely required for lateral condyle
fractures, and its use can add to unnecessary
expenditure and delay in operative intervention.
Computed tomography scans may be used for
extensive fractures such as intercondylar fractures
or in high-energy trauma where interpretation
may be difcult with routine radiographs. Mag-
netic resonance imaging (MRI) can also be
obtained to look for nondisplaced or minimally
displaced fractures and to determine articular
involvement. The stability of lateral humeral con-
dyle fractures is closely related to the cartilage
hinge integrity, and MRI can be used to assess
this better than plain radiographs (Horn
et al. 2002). However, intraoperative arthrography
is cheap and can easily be performed to study the
articular surface congruence. This modality
should be used whenever there is doubt about
the morphology of the distal humerus or the ade-
quacy of the reduction of the fracture (Marzo
et al. 1990). Other imaging modalities, such
as ultrasound, may be of value in younger
children with large amounts of unossied carti-
lage (Davidson et al. 1994).
Classification
Milch classied these fractures based on the exit
of the fracture line at the articular surface of the
distal humerus (Milch 1964) (Fig. 2):
52 Distal Articular Humerus Fractures
Fig. 2 Milch classication
Type I: This is the less common pattern and is akin
to a Salter-Harris (SH) type IV injury. The
fracture involves the metaphysis, the physis,
and the epiphysis of the lateral condyle ossi-
cation nucleus.
Type II: This fracture extends from the epiphysis
and traverses through the physis and extends to
the trochlea. It is usually believed to be analo-
gous to an SH type II injury. However, if the
articular surface is involved, it may also be
considered an SH type III or IV.
1141
A classication based on the amount of dis-
placement present was described by Jakob
et al. (1954) (Fig. 3).
Unlike the Milch classication, which only
describes the appearance of the fracture and does
not help guide treatment, the Jakob classication
is valuable in determining the treatment for these
injuries:
Stage I: The metaphysis and the physis are
involved, but the articular cartilage is spared.
1142
Fig. 3 Jakob classication
The fracture is incomplete and therefore the
fracture is stable. These fractures should be
managed nonoperatively.
Stage II: The fracture violates the articular cartilage,
but there are minimal displacement and no
noticeable step-off of the articular surface. This
type of fracture can displace over time and may
require operative intervention.
Stage III: This fracture pattern involves the
metaphysis, the physis, and the articular
surface with displacement and rotation of the
fracture fragment. Jakob stage III injuries are
best managed with operative intervention.
Weiss et al. described another classication
based on the amount of displacement. This system
is particularly useful in predicting complications
such as late displacement or delayed healing
(Weiss et al. 2009):
Type I: Fractures have less than 2 mm of
displacement.
Type II: Fractures have 2 mm or more of displace-
ment with an intact articular hinge, which is
determined with the aid of an arthrogram.
Type III: Fractures have 2 mm or more of dis-
placement and articular cartilage disruption.
The authors found signicantly greater com-
plication rate as the fracture pattern progressed
from type I to type III.
A.G. Potty et al.
Treatment Options
Nonoperative Management of Lateral
Condyle Fracture
The nonoperative management of lateral con-
dyle fractures is controversial with practices
varying from surgeon to surgeon. While some
recommend surgical pinning of all lateral con-
dyle fractures, others recommend cast treatment
and close observation of nondisplaced fractures.
The uncertainty in management is due to
the difculty of assessing fracture stability.
Our preference is to use nonoperative manage-
ment only for nondisplaced fractures. This is
performed by placement of a long arm cast
in 90 of elbow exion and the forearm in
neutral rotation. The cast should be appropri-
ately molded and snug enough such that the
fracture will not displace as the swelling goes
down.
Lateral condyle fractures treated nonoperatively
should be closely watched for displacement.
Radiographs of the elbow, including an AP,
lateral, and internal oblique view, should be
performed weekly for the rst 3 weeks to
assess for any displacement and avoid
potential complications (Marcheix et al. 2011)
(Fig. 4a, b, c, d).
The cast is maintained for 6 weeks or until
radiographic evidence of healing is seen. Once
52 Distal Articular Humerus Fractures 1143

Fig. 4 Lateral condyle

fracture. (a, b) AP and
lateral elbow radiograph of
a 5-year-old male with a
nondisplaced lateral
condyle fracture. (c, d)
1 week post casting
showing interval
displacement of fracture
(solid arrows)

the cast is removed, active range of motion exer-
cises are initiated. Most children can resume nor-
mal non-weight-bearing activity in 6 weeks and
gradually increase to full function over the subse-
quent 6 weeks. The child and parents are
counseled to refrain from contact sports for at
least 6 months following the injury. Though
there is no evidence regarding return to sport
timing in this fracture, we have noticed that
6 months will give a reasonable chance for this
fracture to remodel and decrease the chances for a
secondary facture.
1144 A.G. Potty et al.

Fig. 5 (a, b) AP and lateral

radiographs of a 9-year-old
girl who fell from the
monkey bars. (c, d)
Intraoperative arthrogram
showing intact articular
surface. She was treated
with closed reduction and
percutaneous pinning

Lateral condylar fracture more than 2 mm of displacement, or if there is

Nonoperative management obvious rotation or malalignment of the fracture.
Indications Contraindications Operative techniques include closed reduction
Jakob types I and II Displacement and loss of with percutaneous pins or open reduction and inter-
reduction in cast nal xation with pins or screws. Both pins and screw
Weiss types I and II 2 mm displacement of xation have it own merits, but no one treatment has
<2 mm displacement fracture been shown to be superior to the other (Li and Xu
Intact periosteal hinge in
2012, Andrey et al. 2013) (Fig. 5a, b, c, d).
arthrogram
Displacement greater than 5 mm in any one
radiographic view usually requires an open reduc-
Lateral condylar fracture tion with internal xation. An attempt can be
Physical/occupational therapy recommendations made to treat fractures with 25 mm of displace-
Restriction in cast for 46 weeks depending on ment with closed reduction and percutaneous pin-
radiographic union. Encourage shoulder, wrist, and ning, but this technique is technically demanding
nger motion. Then start active range of motion about the
elbow as tolerated. Return to school when comfortable and should be used judiciously (Song et al. 2008).
Any intraoperative struggle to obtain or maintain
an anatomic, congruent reduction should warrant
Operative Management open reduction. It is critical to assess the articular
surface through either direct visualization or an
In general, surgical intervention is recommended intraoperative arthrogram in order to conrm the
if nonoperative treatment fails, if there initially is reduction of the elbow joint.
52 Distal Articular Humerus Fractures
Preoperative Planning
Planning should include careful documentation
of the distal neurovascular status. Make efforts to
discuss all potential complications with parents
including elbow stiffness, growth disturbance,
delayed union, and lateral condylar overgrowth
(pseudovarus). Additionally, a discussion regard-
ing return to school and activities, especially
in an athlete involved in pitching and throwing,
should occur.
Plain radiographs should include standard AP,
lateral views, and internal oblique views.
Positioning
Position the child in a supine position with
the injured arm extended. In smaller children,
a uoroscopy tube can be inverted and used
as the hand table. Place the childs torso close
to the hand table (or uoroscopy tube) and
place an arm board at the side of the head to
prevent the head from slipping off of the operat-
ing table. This also allows easy uoroscopy
access and helps in rotating the shoulder joint.
Pad the shoulder well and make sure that it
does not extend beyond 90 of abduction as this
can cause stretching of the brachial plexus.
Secure the torso to the table with a safety
belt and position the head on a foam doughnut
with the anesthetic tubes coming off the opposite
side. Turn the operating table 90 and position
the ventilator on the opposite side of the hand
table.
Apply a proper-sized tourniquet and set it to
100 mmHg above the mean systolic blood pres-
sure. A sterile tourniquet may be necessary if
access to the operating site is compromised due
to the small nature of the extremity.
The uoroscopy unit should be brought in
from the foot end of the table to allow the surgeon
and assistant adequate space. Furthermore, the
uoroscopy screen should be placed at the oppo-
site end of the hand table to permit direct visual-
ization. The instrument table should be placed on
the right side of the surgeon, thus allowing the
scrub nurse to pass instruments easily.
1145
Closed Reduction and Percutaneous
Pinning
The closed reduction and percutaneous pinning
technique is only attempted on minimally
displaced fractures, those with <5 mm of dis-
placement (Mintzer et al. 1994). An intraoperative
arthrogram is frequently helpful in assessing the
reduction (Fig. 5c). The authors preference is to
perform the arthrogram from a posterior
approach, with the needle entering the elbow pos-
teriorly just proximal to the tip of the olecranon.
Implants needed are usually 2  1.6 mm
smooth Kirschner wires. In larger children (gener-
ally > 5 years of age), 2 mm smooth pins are used.
A divergent pin conguration is desirable as it
provides better stability than parallel pins (Bloom
et al. 2011). Application of slight valgus stress at
the elbow, along with a posteriorly directed force,
will aid in obtaining the reduction. Once the
desired reduction has been obtained, the surgeon
positions the rst pin, and the orientation and the
direction of the pin are assessed with uoroscopy.
The rst wire is placed perpendicular to the
fracture and is aimed proximally and medially to
obtain bicortical xation within the medial
humeral metaphysis. This method gives better
xation and prevents spinning of the fragment
while placing the second wire. The second wire
is then oriented transversely in a divergent manner
to the fracture, which provides an increase in
stability. The wires may be placed through the
ossication center of the capitellum, without the
need for signicant concern for growth arrest.
At times, a third wire is needed if the initial two
wires are inadequate to provide the necessary
stability. The wires are bent and cut, and nal
images, including AP, lateral, and internal oblique
views, are obtained. The wires may be either
buried or left out of the skin. Sterile dressings
and a long arm cast are then placed.
Lateral condylar fracture
Preoperative planning for closed reduction percutaneous
pinning
OR table: standard operating table rotated 90
Position/position aids: patient supine with injured limb
on hand table
(continued)
1146
Lateral condylar fracture
Preoperative planning for closed reduction percutaneous
pinning
Fluoroscopy location: on the foot end of the patient with
the screen on the opposite side for direct visualization for
the surgeon.
Equipment: Kirschner wires set, wire driver, contrast for
arthrogram
Tourniquet: tourniquet may be used
Lateral condylar fracture
Surgical steps for closed reduction percutaneous pinning
Reduce the fracture under uoroscopy
Perform an arthrogram to assess stability and integrity of
the joint
Place 23 wires in divergent fashion
Assess stability
Apply a long arm cast or splint depending on swelling
Arthroscopic-Assisted Reduction
and Percutaneous Pinning
Arthroscopic-assisted reduction and pinning were
developed in order to avoid the excessive strip-
ping of the soft tissues that is necessary with open
reduction. However, elbow arthroscopy can be
challenging in the setting of a fracture. Hausman
et al. reported their results in arthroscopic-assisted
percutaneous pinning. In this six-patient series, all
patients healed completely within 4 weeks and
had full range of motion. The patients were pain-
free at nal follow-up of 8 months (Hausman
et al. 2007).
Open Reduction and Internal Fixation
Indications for open reduction and internal xa-
tion consist of initial fracture displacement of 5
mm, an incongruent joint surface, and failure of
closed reduction. Stress views showing 5 mm or
more of displacement with an incongruent articu-
lar surface are also an indication for open reduc-
tion internal xation, even if the non-stress views
show less than 5 mm of displacement.
The positioning and setup for this procedure
are similar to that for a closed reduction with a
A.G. Potty et al.
hand table and sitting stool for the surgeon. A
head lamp and surgical loupes are often very
helpful for this procedure.
Lateral condylar fracture
Preoperative planning for open reduction internal xation
OR table: standard operating table rotated 90
Position/position aids: patient supine with injured limb
on hand table
Fluoroscopy location: on the foot end of the patient with
the screen on the opposite side for direct visualization for
the surgeon
Equipment: Kirschner wires set, wire drivers, Hohmann
retractors
Tourniquet: sterile tourniquet
Additional items: head lamp, loupe magnication
Surface Marking and Exposure
Mark the lateral epicondyle, the olecranon, and
the radial head. A curvilinear incision is made
along the lateral edge of the distal humerus placed
such that the proximal two third of the incision is
on the humerus and the remaining third crosses
the joint line. A lateral Kocher approach to the
elbow is then performed (Fig. 6a, b). Alterna-
tively, a more anterior Kaplan approach can be
utilized. The fracture usually guides the dissection
through the rent it created in the soft tissues. A
large fracture hematoma is typically present
within the elbow joint.
The next step is to carefully strip the anterior
humeral periosteal layer and follow it distally to
the articular surface of the elbow. This permits
visualization of the fracture line and its extent
into the articular surface. The dissection should
not be extended posteriorly as the blood supply to
the lateral condylar epiphysis enters the bone
through the posterior soft tissue attachments. Ade-
quate visualization is achieved when the trochlea
and the fracture line can be seen anteriorly.
Lateral condylar fracture
Surgical steps for open reduction internal xation
Approach is the standard Kocher approach through a
lateral incision (alternatively, a more anterior Kaplan
approach can be utilized)
An interval between triceps and brachioradialis is
developed guided through the fracture rent
(continued)
52 Distal Articular Humerus Fractures 1147

Fig. 6 Preoperative (a) and

intraoperative (b)
photographs of an open
reduction of a lateral
humeral condyle. The
fracture is well visualized in
picture b (arrow)

Lateral condylar fracture Retractors should be placed anteriorly within the

Surgical steps for open reduction internal xation
The posterior attachment of the fragment is preserved
with minimal dissection
Reduce the fragment with reduction clamps or
provisional Kirschner wires
Fix the fragment with Kirschner wires in a divergent
fashion
Apply a long arm cast or posterior splint
Reduction and Fixation of Fracture
The key to obtaining a successful reduction of
lateral condyle fractures is good visualization.
elbow capsule to see the trochlea and the fracture
line. The fracture edges should be irrigated with
copious saline, and the fracture hematoma should
be removed. A congruent reduction should be
obtained and maintained with a reduction forceps
or with provisional Kirschner wires. The initial
wire placed can also be used as a joystick to help
rotate and reduce the fragment. The fracture site
should then be assessed with direct visualization
and uoroscopically. If the reduction is satisfac-
tory, the xation wires can be placed as described
above. A third wire can be used if needed.
In children above 12 years of age, a large frac-
ture fragment can be xed with a cannulated screw.
1148 A.G. Potty et al.

This xation might give better compression at the open reduction after 3 weeks predisposes the
fracture site although the screw heads are often patient to loss of range of motion and other com-
prominent and necessitate later removal. plications listed below (Wattenbarger et al. 2002).
Once the reduction and xation are adequate,
the periosteum overlying the fracture laterally
should be closed with absorbable suture in order Pitfalls and Prevention
to help decrease the chances of lateral spur forma-
tion. The extremity is then placed in a long arm Many pitfalls can be identied when managing
cast at 90 of elbow exion with the forearm in these fractures. If nonoperative treatment is opted
neutral to mild pronation. for, then weekly follow-up radiographs are neces-
sary for 3 weeks to detect late displacement of the
fracture. Internal oblique views may help demon-
Postoperative Management strate displacement better. Additional techniques,
such as examination under anesthesia, stress
The child is admitted overnight to administer views, and/or an arthrogram, may be needed to
intravenous antibiotics, neurovascular monitor- assess the congruency of the fracture.
ing, and pain control medications. Radiographs In an operative setting, it is imperative not to
in the cast are obtained 12 weeks following extend the dissection into the posterior soft tissue
surgery. The pins are maintained for 46 weeks, attachments of the fragment, as this can lead to
although 3 weeks of pin stabilization may be avascular necrosis (AVN) of the capitellum and
sufcient (Thomas et al. 2001). Gentle active nonunion of the fracture. The fracture and the
motion is begun as soon as the cast is removed. trochlea should be well visualized before any
A splint or sling may be used for additional attempt at reduction is performed. A head light
protection if desired. and loupe magnication will aid in obtaining an
Isometric exercises are initiated after 6 weeks. anatomic reduction.
Return to sports should occur after 36 months, as The pins should be divergent and bicortical in
long as motion and strength are satisfactory. Phys- order to obtain optimal stability.
ical or occupation therapy referral is seldom
needed, and most children return to full function Lateral condylar fracture
without it. However, therapy may be benecial in Potential pitfalls and prevention
children who fail to show steady progress. Potential pitfall Pearls for prevention
Pitfall 1 Prevention 1
Lateral condylar fracture Late displacement in 1a: Follow up weekly for
Postoperative protocol nonoperative 3 weeks
management 1b: Obtain advanced
Type of immobilization: long arm cast
imaging to assess
Length of immobilization: 46 weeks
displacement
Rehab protocol: active range of motion exercises after
Pitfall 2 Prevention 2
6 weeks
Unable to determine the 2a: Obtain arthrogram
Return to sport: after 36 months
congruency of the 2b: Visualize the trochlea
articular surface and fracture before
reduction
Outcomes Pitfall 3 Prevention 3
AVN of capitellum and 3a: Careful soft tissue
nonunion handling
No long-term clinical outcomes exist regarding
3b: Avoid dissection into
pediatric lateral condyle fractures. Most children posterior soft tissue
who get immediate treatment tend to do well. attachments of the fragment
However, 10 % of children will have a 1015 Pitfall 4 Prevention 4
extension loss at 12 years post injury. Delayed (continued)
52 Distal Articular Humerus Fractures
Lateral condylar fracture
Potential pitfalls and prevention
Potential pitfall Pearls for prevention
Loss of xation of pins 4a: Divergent pin until skeletal maturity (Cates and Mehlman 2012).
conguration
4b: Bicortical pin
placement. Conrm on Common
uoroscopy after cutting complications
the pins
Pitfall 5 Prevention 5
Skin necrosis 5a: Place a well-padded cast
5b: Point the sharp edges of
the pins away from the skin
Complications
Complications from lateral condyle fractures are
usually due to late/delayed presentation or from
an inadequately treated fracture. Delayed presen-
tation and/or a missed diagnosis may lead to non-
union of the fracture. Nonunions can result in a
cubitus valgus deformity due to growth arrest of
the lateral condyle with normal growth of the
medial condyle. Left untreated, a nonunion can
ultimately result in elbow instability, cubitus val-
gus, and tardy ulnar nerve palsy. Treatment of
nonunion of the lateral humeral condyle generally
consists of in situ surgical repair of the nonunion
with internal xation, along with a humeral
osteotomy and ulnar nerve transposition, if
needed (Tien et al. 2005).
Posterolateral spur formation (lateral over-
growth) is a common problem after surgical treat-
ment of lateral condyle fractures and may give the
elbow the appearance of being in varus (Pribaz
et al. 2012). This typically improves over time,
and no specic treatment is needed.
Avascular necrosis of the capitellum may occur
after open reduction of lateral condyle fractures.
Excessive exposure of the posterior structures of
the fragment is thought to predispose to this com-
plication as it disrupts the blood supply to the
epiphysis. The vascular supply to the trochlea is
also at jeopardy during surgical exposure. This
risk seems to be minimized by avoiding posterior
dissection (Wattenbarger et al. 2002).
Growth arrest in children after lateral condyle
fractures has been described and usually is
1149
manifest on radiographs as a shtail deformity.
This typically does not cause any symptoms and
children with this nding should be monitored
Lateral condylar fracture
Management
Pin site Antibiotics and removal of the pins
infection when healing is achieved
Posterolateral Reassurance. Will usually resolve
spur
Delayed union Prolonged immobilization for up to
12 weeks
Nonunion Compression xation and bone
grafting with screws. Simultaneous
corrective osteotomy if deformity is
present
Cubitus varus Valgus supracondylar osteotomy of
distal humerus
Cubitus valgus Varus supracondylar osteotomy or
gradual distraction with Ilizarov
external xator
Fishtail Monitor until skeletal maturity, as it
deformity can cause functional decits
Tardy ulnar Decompression of cubital tunnel and
nerve palsy ulnar nerve transposition
Summary
Lateral humeral condyle fractures are very com-
mon in children. Nonoperative treatment is suf-
cient for many of these injuries, but the
practitioner must be aware of the propensity for
these fractures to displace while in a cast. Opera-
tive treatment is frequently successful and closed
or open reduction may be required. Closed reduc-
tion is technically demanding and has limited
indications, so the practitioner should not hesitate
to open the fracture if required.
Fractures of the Medial Condyle
of the Humerus
Introduction
Fractures of the medial condyle of the elbow are
uncommon, and therefore, a high index of suspi-
cion is needed to make the correct diagnosis of
1150
this injury. These fractures may be considered the
medial version of fractures of the lateral humeral
condyle as fractures of the medial humeral con-
dyle typically begin in the metaphyseal region and
propagate distally and laterally. Medial condyle
fractures also extend into the joint itself and share
similar classication systems to lateral condyle
fractures, and the principles of treatment are the
same for both fractures.
Medial condyle fractures are rare in children
and are believed to occur in an older age group
compared with fractures of the lateral humeral
condyle. Medial condyle fractures usually occur
after ossication of the medial condyle begins
(Papavasiliou et al. 1987; Ghawabi 1975).
Pathoanatomy and Applied Anatomy
The medial and lateral secondary ossication cen-
ters of the distal humerus form the distal humeral
articulation of the elbow. The medial condylar
physis contributes to the medial portion of the
distal humerus and is separated from the lateral
(capitellar) ossication center by the trochlear
notch. The lateral crista of the trochlea derives
from the lateral condylar physis, while the medial
portion originates from the medial capitellar sec-
ondary ossication center. Fractures of the medial
condyle of the humerus typically have a fracture
line that originates in the medial metaphysis and
propagates distally and laterally. The fracture line
usually extends to the cartilage of the distal
humerus and may extend into the elbow joint.
The intra-articular extension of the fracture line
is variable in location but frequently occurs
through the trochlear notch lateral to the medial
ossication center of the trochlea.
The common exors are attached to the
metaphyseal portion of the distal humerus through
their origin about the medial epicondyle, which is
commonly not injured and remains in place when
a fracture of the medial condyle is present. There-
fore, the condylar fracture fragment is typically
displaced in a medial and forward direction due to
the pull of the exor muscles. The blood supply to
the metaphyseal fragment as well as to the medial
condyle is from the exor attachments to the
A.G. Potty et al.
medial epicondyle. In contrast, the lateral-most
aspect of the medial trochlear ossication center
obtains its blood supply from medial to lateral
intraosseous vessels; thus, its blood supply may
be at risk in displaced intra-articular fractures.
Two mechanisms of injury have been
described for medial humeral condyle fractures.
The rst is a fall onto a exed elbow (Bensahel
et al. 1986). With this mechanism, the semilunar
notch of the olecranon acts as a wedge splitting
the trochlea, resulting in a fracture of the medial
condyle. The second mechanism is a fall onto an
outstretched hand (Fowles and Kassab 1980).
This is thought to create a valgus stress across
the elbow joint, causing an avulsion fracture of
the medial condyle.
Assessment
Signs and Symptoms
Medial humeral condyle fractures typically present
with a swollen and tender elbow, particularly on the
medial aspect. Attention should also be paid to the
entire elbow as injuries to the olecranon and lateral
structures, including the radial neck and head, have
also been reported in association with medial con-
dyle fractures (Bensahel et al. 1986). The shoulder,
humerus, forearm, and wrist should also be exam-
ined for a concomitant injury. Additionally, a thor-
ough neurovascular exam should be performed to
search for an associated neurological injury. The
ulnar nerve is particularly at risk for injury from
this fracture.
Imaging and Other Diagnostic Studies
High-quality orthogonal elbow radiographs are
the minimum imaging required for evaluating
elbow injuries and are typically sufcient for mak-
ing the diagnosis of a fracture involving the
medial condyle of the elbow. Radiographs should
include the wrist and elbow and should be scruti-
nized for a fracture through the medial
metaphysis. The secondary ossication center of
the medial condyle typically appears between
8 and 10 years of age. Before this ossication
center is well dened, medial condyle fractures
may be mistakenly diagnosed as a fracture of the
52 Distal Articular Humerus Fractures 1151

medial epicondyle (Fowles and Kassab 1980). To

help differentiate these two injuries, it should be
noted that elbows with fractures of the medial
epicondyle typically exhibit instability to valgus
stress, while elbows with fractures of the medial
condyle will be unstable to varus stress. In addi-
tion, while elbow dislocations may occur with
either aforementioned fracture, they are rarely
seen in children younger than 8 years of age.
The presence of a posterior fat pad sign should
also alert the physician to an intra-articular injury
(Skaggs and Mirzayan 1999). Other imaging
modalities such as an MRI, elbow ultrasound,
and elbow arthrogram (typically performed at
the time of surgery) may also be helpful to further
dene the injury and determine proper treatment
(Davidson et al. 1994).

Classification

Fractures of the medial condyle of the humerus

can be classied either by the fracture location or
by the amount of fracture displacement. Milch
classied fractures of the medial condyle into
two types: type I and type II (Milch 1964). In
type I fractures, the fracture line ends in the troch-
lear notch. In type II fractures, the fracture line
ends in the capitulotrochlear groove and involves
the capitellum. This classication is based only on
a radiographic nding and therefore is not signif-
icantly useful in guiding treatment (Fig. 2).
Kilfoyle described a classication based on
fracture displacement (Kilfoyle 1965). In type I
fractures, the fracture line extends down to the
physis but does not extend through the medial
condylar physis. Type II fractures go through the
medial condylar physis but are nondisplaced.
Type III fractures are complete fractures with
rotation and displacement of the condylar
fragment.
Treatment Options
Nonoperative Management
Nondisplaced fractures of the medial humeral
condyle in younger children can be treated with
Fig. 7 Medial condyle fracture. AP and lateral image of a
12-year-old boy with a medial condyle elbow fracture.
Note extension of the fracture line into the capitellum
(Milch type II)
a long arm cast for 46 weeks (Bensahel
et al. 1986; Ghawabi 1975; Kilfoyle 1965;
Papavasiliou et al. 1987). The cast can be
removed and range of motion exercises initiated
when bony callus is visible after 46 weeks. Full
range of motion is typically obtained about
6 weeks after the cast is removed. Nondisplaced
injuries that extend to the joint surface should be
followed weekly with radiographs for 3 weeks to
observe for possible fracture displacement.
Medial condyle fractures may heal slowly, par-
ticularly in older children (Fowles and Kassab
1980) (Fig. 7).
1152
Fractures of the medial humeral condyle
Nonoperative management
Indications Contraindications
Nondisplaced Displaced fractures
fractures
Fracture with associated elbow
dislocation
Operative Treatment
Indications: Displaced fractures of the medial
condyle of the humerus should undergo operative
xation (Bensahel et al. 1986; Ghawabi 1975;
Fowles and Kassab 1980; Kilfoyle 1965;
Papavasiliou et al. 1987).
Preoperative Planning: Adequate preopera-
tive radiographs should be available and plan for
an elbow arthrogram.
Positioning: The patient is positioned supine
with the arm on a hand table. The patient is placed
supine with the shoulder abducted 90 . A
nonsterile tourniquet is typically applied, although
a sterile tourniquet may be needed in smaller
patients. The arm is then placed on a radiolucent
hand table. A tourniquet is used to aid in visualiza-
tion. Younger children and those with small arms
may need the utilization of a sterile tourniquet.
Approach: A medial approach is made to the
elbow, taking care to identify and protect the ulnar
nerve. Care should also be taken to avoid posterior
dissection on the condylar fragment in order to
avoid unnecessary disruption of the blood supply
to the fracture fragment. Fracture xation typi-
cally is achieved by either smooth Kirschner
wires or cannulated screws (in older patients
close to skeletal maturity).
Fractures of the medial condyle
Preoperative planning
OR table: standard table with an arm board
Position/positioning aids: supine
Fluoroscopy location: from the head of the table
Equipment: orthopedic set, curettes, Kirschner wires
(usually 1.6 or 2.0 mm diameter), cannulated screws (4.0
or 4.5 mm diameter)
(continued)
A.G. Potty et al.
Fractures of the medial condyle
Preoperative planning
Tourniquet: sterile or nonsterile, depending upon size of
patient
Additional items: plan for an elbow arthrogram, if
needed (contrast, syringe, hypodermic needle)
Surgical procedure: Open reduction internal
xation of medial condyle fractures
A medial approach is used starting at the elbow
joint and extending proximally to the medial
epicondyle. The ulnar nerve should be identied
and protected for the duration of the procedure.
Once the fracture is visualized, the dissection
should be extended anteriorly into the elbow
joint. Posterior dissection should be avoided, if
possible. The fracture is then debrided of hema-
toma and early callus and the fracture is reduced.
After the fracture is reduced, provisional
(or denitive) xation can be obtained either with
cannulated screw guide wires or 2 or 3 smooth
Kirschner wires, typically 1.6 mm in diameter.
The wires should be placed in a divergent pattern.
Once xation has occurred, the reduction and posi-
tion of the pins should be assessed with direct
visualization and uoroscopically with particular
attention paid to achieving a congruent joint sur-
face. If cannulated screws are used, they would
now be inserted. If Kirschner wires are being
used as the denitive xation, they should be left
out through the skin in order to facilitate later
removal. A splint is then applied. If stable xation
is obtained (e.g., with screws), active elbow motion
can be started 710 days after surgery. Otherwise, a
cast is applied and the pins are removed and motion
begun 46 weeks following the surgery.
Fractures of the medial condyle
Postoperative period
Type of immobilization: splint or long arm cast
Length of immobilization: 710 days (stable (e.g.,
screw) xation), 46 weeks for wire xation
Rehab protocol: begin active and active-assisted range
of motion exercises several times daily
Return to sport protocol: full activities are allowed
once the fracture is fully healed and there is return of
signicant range of motion and strength
52 Distal Articular Humerus Fractures
Surgical Pitfalls and Prevention
Complications have been reported in up to 33 %
of medial condyle fractures (Leet et al. 2002).
These include nonunion, delayed union, loss of
reduction, and osteonecrosis of the trochlea. Rec-
ognition of these uncommon injuries, particularly
in younger children, is difcult. Missed and
untreated fractures have signicant risk of
malunion or nonunion (Fowles and Kassab
1980). Nonunions of medial condyle fractures
typically result in cubitus varus (as opposed to
nonunions of lateral condyle fractures, which
result in cubitus valgus). Nonunions of the medial
humeral condyle are probably best treated with
surgical stabilization, similar to the treatment of
nonunion of the lateral humeral condyle (Kilfoyle
1965; Ghawabi 1975). Delayed union and loss of
reduction are typically related to inadequate sta-
bilization of the fracture. Proper technique should
help minimize these complications.
Osteonecrosis of the trochlea may occur either
from the fracture itself or from surgical repair
(Ghawabi 1975; Fowles and Kassab 1980;
Papavasiliou et al. 1987). Care should be taken
to avoid posterior dissection on the condylar frag-
ment in order to avoid disrupting its blood supply.
In addition, varus and valgus deformities of the
elbow have been reported in medial condyle frac-
tures that have healed uneventfully. These are
believed to be secondary to either growth stimu-
lation or growth retardation of the medial condyle
or trochlea, respectively.
Fractures of the Capitellum
Introduction
Fractures of the capitellum are unusual but seri-
ous injuries in children. There are little data on
the true incidence of this injury in children, but
they seem to be more common in children older
than 12 years of age (Grantham et al. 1981;
Johansson and Rosman 1980). In younger chil-
dren, this fracture may be especially difcult
1153
to diagnosis due to the lack of ossication of
the capitellum.
Pathoanatomy and Applied Anatomy
The capitellum is the distal anterior extension of
the lateral column of the humerus and is directed
distally and anteriorly at a 30 angle to the long
axis of the humerus when viewed from a lateral
projection. It is the rst secondary ossication
center of the elbow to appear, typically appearing
around 23 years of age. The anterior portion of
the capitellum is covered with articular cartilage
and articulates with the radial head. In exion, the
radial head engages the capitellum on its anterior
surface. In extension, the radial head articulates
with the inferior surface of the capitellum. The
blood supply to the capitellum courses in a poste-
rior to anterior direction and is vulnerable to
injury with capitellar fractures.
Capitellar fractures are believed to occur from
either a fall on an outstretched hand or directly
onto the elbow (Ruchelsman et al. 2008b;
ODriscoll et al. 1992). The radial head is believed
to impact the capitellum, resulting in shear forces
that cause the fracture. This is thought to be more
likely by elbow hyperextension or cubitus valgus.
Typically, the capitellar fragment(s) will displace
proximally into the radial fossa.
Assessment
Signs and Symptoms: Patients typically complain
of elbow pain, although swelling may be minimal.
There is restricted exion of the elbow joint, as the
fracture fragment usually impinges with the elbow
joint in exion. A thorough upper extremity
examination should be performed, since fractures
of the capitellum are frequently accompanied by
other fractures, including those of the radial neck
and radial head (Dubberley et al. 2006; Johansson
and Rosman 1980).
Imaging of capitellar fractures may be chal-
lenging. Radiographs of the ipsilateral forearm
1154
Fig. 8 AP (a) and lateral (b) radiographs of a 12-year-old
female with a type I capitellar fracture. Note double
bubble sign on lateral radiograph (arrow)
and wrist should be obtained in addition to the
standard elbow radiographs (AP and lateral)
(Fig. 8). Large fracture fragments can be readily
seen on lateral radiographs, but smaller or carti-
laginous fragments may be difcult to appreciate
on lateral images. When present, the double bub-
ble appearance of the fracture has been described
as pathognomonic (Ruchelsman et al. 2008a;
McKee et al. 1996). The distal humerus may
A.G. Potty et al.
obscure visualization of fracture fragments on
anteroposterior elbow radiographs. Oblique
radiographs and computerized tomography scan
may be very helpful to denitively assess the
fracture morphology and also often help guide
treatment (Ruchelsman et al. 2008b; Letts et al.
1997).
Classication: The McKee modication of
the Bryan and Morrey classication is com-
monly used to classify these injuries (McKee
et al. 1996, Bryan and Morrey 1985). Type I
fractures describe complete fractures of the
capitellum, with no to little extension into
the trochlea. Type II fractures are supercial
articular chondral fractures of the capitellar
joint surface. Frequently, these fracture frag-
ments have little or no subchondral bone
attached to them and may represent sequelae
from an underlying osteochondral lesion. Type III
fractures are comminuted capitellar fractures,
while type IV fractures are coronal shear fractures
of the capitellum involving the medial distal
humerus that includes a portion of the trochlea
(Fig. 9).
Treatment Options
Nonoperative Management
The indications for nonoperative management of
capitellar fractures are limited. All displaced
capitellar chondral or osteochondral fractures are
best treated surgically, and surgical treatment of
nondisplaced fractures will allow for early elbow
motion.
Indication: The indication for surgical treat-
ment is any displaced capitellar fracture. The
goals of treatment are to restore anatomic align-
ment of the elbow and to allow early range of
motion to prevent stiffness (Fig. 10).
Fractures of the capitellum
Nonoperative management
Indication Contraindication
Completely nondisplaced Any displacement of the
fracture fracture
52 Distal Articular Humerus Fractures
Fig. 9 Capitellum fracture. Bryan and Morrey classica-
tion of capitellar fracture. (a) Type I (Hahn-Steinthal),
complete capitellar fracture with little or no extension
into the lateral trochlea. (b) Type II (Kocher-Lorenz), ante-
rior osteochondral fracture with minimal subchondral
Surgical Procedure
Preoperative Planning
Open reduction internal xation of capitellar frac-
tures can be accomplished through either a lateral
or an anterolateral approach. The former has the
advantage of being familiar to most orthopedic
surgeons, while the latter offers a more direct view
of the capitellum and facilitates placement of inter-
nal xation. Either approach requires supine
1155
bone. (c) Type III (Broberg-Morrey variant), commi-
nuted/compression fracture of the capitellum. (d) Type IV
extends medially to include most of the trochlea. McKee
modication of the Bryan and Morrey classication
positioning of the patient with the shoulder
abducted 90 and the elbow extended. Tourniquet
control is imperative for surgical treatment of this
injury.
Fixation: There are many choices for xation.
These include headless compression screws
placed in the anterior to posterior direction, tra-
ditional cancellous or cannulated screws
inserted in a posterior to anterior direction,
bioabsorbable implants, and plates and screws
1156
Fig. 10 AP and lateral postoperative radiographs after
fracture healing
(for large type IV fractures). Headless compres-
sion screws placed in an anterior to posterior
direction are preferred, as these can be placed
under direct visualization, compress the fracture
site, and do not need later removal (Elkowitz
et al. 2002).
Positioning: The patient is placed supine with
the shoulder abducted 90 and the elbow extended
on a radiolucent hand table.
A.G. Potty et al.
Fractures of the capitellum
Preoperative planning
OR table: standard with a hand table
Position/positioning aids: supine with a hand table
Fluoroscopy location: either from the end of the hand
table or from the cephalad edge of the hand table
Equipment: orthopedic hand set, drill, Kirschner wires,
headless compressions screws
Tourniquet: nonsterile
Surgical Approaches
The anterolateral approach to the elbow will be
described: an S-type incision is made about the
antecubital fossa with the transverse limb extended
along the elbow exion crease. The interval
between the brachioradialis and biceps muscles is
then developed, and the lateral antebrachial cutane-
ous and radial nerves are identied and protected.
The brachioradialis muscle and radial nerve are
then retracted laterally, exposing the brachialis mus-
cle. The brachialis is then divided longitudinally,
which exposes the anterior elbow capsule lying
beneath the muscle. The elbow capsule is divided
longitudinally and the fracture site is exposed.
Technique
After the fracture is exposed, the fracture site is
debrided of early callus and hematoma. Particular
care should be taken to avoid reduction of the
capitellum in exion. Using smooth Kirschner
wires or the guidewires designed for headless
compression screws, the fracture is provisionally
stabilized. The position of the fracture and hard-
ware should be carefully assessed with uoros-
copy and if satisfactory denitive xation can be
performed. Postoperatively, the patient is placed
in a posterior splint.
Fractures of the capitellum
Surgical steps
Lazy S incision centered on the lateral elbow joint
Develop interval between the brachioradialis and biceps
muscles
Identify lateral antebrachial cutaneous and radial nerves
Retract brachioradialis and radial nerve laterally to
expose brachialis muscle
Split the brachialis muscle
Perform the capsulotomy
Reduce the fracture and stabilize the fracture
52 Distal Articular Humerus Fractures
Postoperative Protocol: Postoperatively, the
patient is placed in splint for 710 days in elbow
exion and neutral rotation. If stable xation has
been achieved, gentle active and active-assisted
range of motion can begin. The patient may also
be transitioned to a hinged elbow brace to facili-
tate range of motion exercises. After about
6 weeks, the brace can be removed and return to
activities can be gradually instituted.
Fractures of the capitellum
Postoperative protocol
Type of immobilization: splint for about 710 days
Length of immobilization: 710 days
Rehab protocol: gentle active and active-assisted range
of motion can begin following splint removal
Return to sport protocol: fracture union and
satisfactory motion and strength are needed prior to
return to sport
Outcomes
There is little information about the outcome of
surgically treated capitellar fractures in children
and adolescents; most published reports have
described the results of treatment in adults
(Ruchelsman et al. 2008a; McKee et al. 1996;
Imatani et al. 2001). In general, most patients in
these series obtained good to excellent results,
although lack of full extension was a common
nding. Interestingly, almost 20 % of patients in
these series developed osteoarthritis despite not
having evidence of capitellar avascular necrosis.
Fractures of the capitellum
Potential pitfalls and preventions
Potential pitfall Pearls for prevention
Pitfall 1: Prevention: ensure satisfactory
inadequate reduction using interoperative
reduction uoroscopy
Pitfall 2: Prevention: visualize and mobilize
neurological nerves for protection
injury
Complications: The most common complica-
tion after surgical treatment of a capitellar fracture
is elbow stiffness, usually loss of exion and
extension. Treating the fracture with anatomic
reduction, stable xation, and early motion can
minimize loss of motion. Postoperative stiffness
1157
may require prolonged therapy, splints, and pos-
sibly even capsular release for treatment. The
other signicant complication from fractures of
the capitellum is posttraumatic arthrosis. This
may be caused by the initial injury or from
osteonecrosis of the fracture fragment. Treatment
for this complication needs to be individualized
for each patient.
T-Condylar Fractures
Introduction
T- or Y-condylar fractures are very rare in skele-
tally immature children since they are usually seen
in adults involved in high-energy trauma. There-
fore, the pediatric literature has very little infor-
mation about the management of this injury.
Current literature is limited to few case reports
(Mok and Lui 2013; Re and Waters 1999).
Re et al. reported a retrospective study on
17 patients, and Papavasilious reported on six
cases of T-condylar fractures (Re and Waters
1999; Papavasiliou and Beslikas 1986).
Pathoanatomy and Applied Anatomy
This injury usually results from a higher energy
mechanism than required to sustain supracondylar
fractures. The fracture typically arises from the
trochlear groove and splits the medial and lateral
columns of the humerus. It is postulated that the
fracture occurs when a large axial force is trans-
mitted through the ulna onto the olecranon fossa
of the distal humerus in a hyperextended elbow.
The edge of the coronoid process acts like an ax,
splitting the trochlea and separating the humeral
condyles at the olecranon fossa. There is also a
rare exion variety where the wedge effect is
produced by the trochlea and the fragments lie
anterior to the humerus (Rockwood et al. 1991).
The treatment principles of T-condylar fractures in
children are similar to those in the adult although,
in young children, a T-condylar fracture of the
distal humerus could also be treated by closed
reduction and casting or closed reduction and
1158
percutaneous pinning. The use of less invasive
techniques in younger children is possible
because of their thick periosteum and abundant
cartilage, which limits displacement and makes
the fracture more amenable to closed treatment.
In addition, children heal more quickly and are
less prone to joint stiffness than adults. Thus,
these techniques should be reserved for children
and not employed in the adolescent and adult
populations.
Assessment of T-Condylar Fractures
The clinical ndings are similar to other fractures
around the elbow and can be easily confused with
a presentation of a supracondylar humerus frac-
ture. The swelling may be marked and associated
nerve injury is common due to the high-energy
trauma and soft tissue disruption. In particular, the
ulnar nerve is at risk secondary to its position
behind the medial epicondyle. Standard orthogo-
nal AP and lateral radiographs remain the gold
standard for diagnosis. Computerized tomogra-
phy is very useful to aid in assessing the fracture
pattern and operative plan. Two- and three-
dimensional reconstructions further delineate the
fracture lines and fragments. MRI may also be
useful to assess the extent of the vertical split in
young patients as well as to assess the presence of
soft tissue damage in cases with extreme swelling
and neurological symptoms. Arthrography may
also be helpful to further evaluate the elbow in
younger patients.
Classification
Multiple classication systems for these injuries
have been proposed, as it is very difcult to
include all these fractures in a single complete
system with accuracy. T-condylar fractures in
older children and adolescents can be classied
according to the system of Mullerin where a C1
fracture is a bicondylar fracture without commi-
nution, a C2 fracture is a bicondylar fracture with
supracondylar comminution, and a C3 fracture
is one with articular comminution (Marsh
A.G. Potty et al.
et al. 2007). It is difcult to use this classication
for children since the unossied articular cartilage
is not visible on plain radiographs. Toniolo and
Wilkins proposed a classication for children
based on the comminution of the fragments and
their displacement (Toniolo and Wilkins 1996). In
type I fractures, the displacement is minimal. In
type II fractures, there is displacement but no
comminution of the metaphyseal fragments.
Lastly, in type III fractures, there is comminution
of the metaphyseal fragments and displacement.
Treatment Options
Nonoperative Management
There is currently only a limited role regarding
nonoperative management of T-condylar frac-
tures. Current literature suggests operative treat-
ment for fractures that involve the articular
surface and those with displacement of the con-
dyles. The goals of the procedure are anatomic
reduction of the articular surface and internal x-
ation to facilitate early motion and restoration of
function.
It would be reasonable to treat nondisplaced or
minimally displaced fractures in younger children
with a long arm cast in 90 of exion and neutral
pronation until healing. Active and active-assisted
range of motion exercises can then be instituted
with return to full activities in 36 months. The
treatment of these fractures, however, is complex
and should be individualized based on the patient-
related factors and the expertise of the treating
surgeon. It may be prudent to refer these patients
to an upper limb surgeon in a center with high
volumes of similar cases.
Intercondylar fracture
Nonoperative management
Indications Contraindications
Young children Older and adolescent
No displacement or Fracture displacement or
comminution comminution
Severe comminution and Some Toniolo type I and all
multiple fragments type II and type III fractures
where reconstruction is
next to impossible
52 Distal Articular Humerus Fractures 1159

Fig. 11 Nine-year-old
boy with a T-condylar
fracture treated with
closed reduction and
percutaneous stabilization.
(a, b) Preoperative AP
and lateral radiographs.
(c, d) Postoperative AP
and lateral radiographs
(Images courtesy of Keith
Baldwin, MD)

Intercondylar fracture (Kanellopoulos and Yiannakopoulos 2004).

Physical/occupational therapy recommendations
In nonoperative management, immobilize in long arm
cast for 46 weeks. Then start exion and extension as
tolerated, aiming for full range of movement by the 12th
week
Outcomes
No outcome studies are available for nonoperative
management. There are a few case reports, which
show no displacement of fracture fragments and
full range of motion at nal follow-up (Re and
Waters 1999).
Operative Treatment
The series reported in the literature shows great
advantage to operative intervention, including
either closed reduction with percutaneous pin-
ning or open reduction with internal xation
The basic principles of operative xation are
similar to fracture management of any intra-
articular fracture. In order to achieve good
elbow mobility, reconstruction of articular con-
gruity is prerequisite. Removal of the bony
debris from the olecranon fossa and restitution
of the geometric shape of the fossa are also
important for impingement-free mobility of
the elbow. Stable xation constructs should be
used to facilitate early motion of the elbow
(Fig. 11a, b, c, d).
The general principle is to restore the articular
joint surface rst and then stabilize the medial and
lateral supracondylar columns. The articular sur-
face can be addressed with an intercondylar com-
pression screw. Next, the column stability is
restored utilizing parallel plates or a 90-90 plate
construct. Very distal T-condylar fractures should
be addressed with compression screw xation and
parallel plating. Early range of motion is
recommended in order to obtain good outcomes
and prevent stiffness.
1160
Intercondylar fracture
Preoperative planning
OR table: standard operating table
Position/position aids: lateral decubitus position with
bean bag and axillary roll
Fluoroscopy location: placed over the head end of the
patient and screen at the foot end of the patient
Equipment: Kirschner wires, contoured 3.5 distal
humerus locking plate, 3.5 LC-DCP, 3.5 pelvic
reconstruction plates, large and medium pointed
reduction clamps; saw and osteotomes if olecranon
osteotomy is planned
Tourniquet: sterile tourniquet usually avoided
Additional items: sterile and radiolucent arm holder
The preferred surgical treatment of the Toniolo
and Wilkins type I fracture is an attempt to perform
a closed reduction and percutaneous xation. The
technique and setup are similar to the percutaneous
xation described for lateral condylar fractures;
however, a two-to-four cross pin conguration,
typically including a transverse intercondylar pin
depending on the stability of the fracture, is uti-
lized. A medial-based pin starting at the medial
epicondyle will be needed to stabilize the medial
column. Type II T-condylar fractures can be man-
aged with either open reduction and percutaneous
pinning or open reduction and internal xation
(authors preference). In extremely comminuted
type III fractures, an attempt can be made to treat
the fracture with minimal internal xation through
a limited soft tissue exposure and traction.
Intercondylar xation is rst achieved with pins,
compression screws, or plating. Olecranon trac-
tion is applied following adequate articular recon-
struction and maintained until adequate callus is
noted over the columns to provide metaphyseal
stability. Elbow motion is started in traction to
prevent stiffness. The outcome of this surgery is
poor, with residual pain and deformity common
after treatment.
Surgical Technique for Open Reduction
Internal Fixation of T-Condylar
Fracture
The patient is placed in a lateral decubitus position
on a beanbag with an axillary roll (Fig. 12). The
A.G. Potty et al.
Fig. 12 T-condylar fractures. Lateral decubitus position
with a radiolucent arm holder utilized for open reduction
and internal xation of a T-condylar fracture
injured arm is then placed on a radiolucent, pad-
ded arm holder. This gives excellent access to the
posterior distal humerus and allows for position-
ing of the uoroscope for intraoperative AP and
lateral images. Several different approaches to the
distal humerus may be used. These include a
triceps-splitting approach, the Bryan-Morrey tri-
ceps-sparing approach, and an extensile posterior
approach utilizing an olecranon osteotomy (Kasser
et al. 1990; Bryan and Morrey 1982). The triceps-
splitting approach is limited in its proximal expo-
sure by the radial nerve and may offer only limited
intra-articular exposure, while the olecranon
osteotomy, although affording excellent exposure
of the distal humeral articulation, adds time to the
procedure and is prone to delayed or nonunion of
the olecranon, and the hardware required to repair
the ulna may become symptomatic. The triceps-
sparing approach may offer only limited intra-
articular exposure so it may not be the best option
in severely comminuted fractures.
Intercondylar fracture
Surgical steps for open reduction internal xation
Bryan-Morrey triceps-sparing approach
Dissection is performed under loupe magnication and
the ulnar nerve is isolated and protected
Anatomic reduction of the intercondylar component with
subsequent xation utilizing an interfragmentary
compression screw
Reduction of the columns with provisional Kirschner
wires
(continued)
52 Distal Articular Humerus Fractures
Fig. 13 Fifteen-year-old
boy with a grade I open
T-condylar fracture treated
with open reduction and
internal xation utilizing
an olecranon osteotomy.
(a, b) Preoperative AP
and lateral radiographs.
(c, d) Postoperative AP
and lateral radiographs
(Images courtesy of Keith
Baldwin, MD)
Intercondylar fracture
Surgical steps for open reduction internal xation
Stable xation of the columns to the intercondylar
component with parallel plates or 90-90 plate
conguration
Ulnar nerve is transposed anteriorly if needed
In general, the triceps-sparing approach is uti-
lized. Most T-condylar fractures in children
and adolescents have minimal comminution of
the articular surface, and the elbow can be
easily and safely approached with this exposure.
A 1015 cm curvilinear extensile posterior inci-
sion is made over the distal humerus bringing the
incision medially about the tip of the olecranon.
The ulnar nerve is identied, mobilized, and
protected. Subsequently, the medial aspect of the
triceps is elevated from the humerus along the
intramuscular septum to the posterior capsule and
reected laterally (Bryan and Morrey 1982). Ana-
tomic reduction of the articular surface is obtained,
and stabilization occurs via a transtrochlear
1161
cannulated lag screw. Alternatively, provisional
xation can be achieved with 1.6 or 2.0 mm
Kirschner wires. This essentially converts the
fracture to a supracondylar fracture by reducing
and stabilizing the articular fracture. The lateral
and the medial columns can then be reduced to the
xed articular component, using provisional
Kirschner wires or reduction clamps. Once
reduced, the fracture is stabilized using two
3.5 mm precontoured distal humerus locking
plates. An alternative to precontoured plates is to
use a 3.5 mm low-contact dynamic compression
plate for the medial column and a 3.5 mm pelvic
reconstruction plate for the lateral column
(Fig. 13, b, c, d). While placing screws, careful
attention has to be taken to avoid penetration of
the olecranon fossa and the articular surface. At
the end of the procedure, the ulnar nerve is
assessed and transposed anteriorly if necessary.
The triceps is repaired loosely to the septum, and
the subcutaneous tissue and skin is closed. A
small drain can be used if needed, and a posterior
1162 A.G. Potty et al.

splint is applied. After 710 days, active range of Nonoperative management is limited to young
motion exercises are begun. Transitioning the children with minimally or nondisplaced frac-
patient to a hinged elbow brace may facilitate the tures; most adolescents require open reduction
early initiation of active range of motion. and internal xation. Meticulous preoperative
planning can help avoid some complications
Intercondylar fracture such as nonanatomic reduction and inadequate
Postoperative protocol xation. The elbow joint needs to be adequately
Type of immobilization: limited mobilization with
visualized and stable xation achieved. Early
posterior splint until swelling resolves
mobilization is important to avoid postoperative
Length of immobilization: 57 days
Rehab protocol: start early mobilization after splint
stiffness.
removal with active/active-assisted exercises at home
Return to sport: 612 months depending on healing
Intercondylar fracture
Potential pitfalls and prevention
Treatment-Specific Outcomes Potential pitfall Pearls for prevention
Pitfall 1 Prevention 1
Jarvis and DAstous looked at their case series, Failure to diagnose 1a: Meticulous
which included 14 patients with follow-up from examination and advanced
4 months to 5 years (Jarvis and DAstous 1984). imaging such as a CT scan
should be used
The authors assessed the loss of exion and exten-
1b: Operative intervention
sion and graded accordingly. Eight good, four is needed in most cases
satisfactory, and two poor results were noted. Of Pitfall 2 Prevention 2
the two poor results, one patient had hypoxic Inadequate preoperative 2a: Careful positioning
ischemic brain injury, and the other patient was planning and failure to and placement of
treated without internal xation but rather with achieve optimal xation uoroscope
traction and casting. Kasser et al. evaluated eight 2b: Using locking plate to
enhance xation
patients with T-condylar fractures treated with
Pitfall 3 Prevention 3
internal xation and a triceps-splitting approach
Failure of implant and 3a: Use cross pin
(Kasser et al. 1990). The authors reported good construct conguration for closed
outcomes and recommended the triceps division reduction and
rather than an olecranon osteotomy. Papavasiliou percutaneous method
et al. reported a satisfactory outcome in six 3b: Parallel plates or
9090 plate conguration
patients treated with open reduction and internal
in adolescent patients with
xation (Papavasiliou and Beslikas 1986). They displacement
noted a better outcome in those patients treated Pitfall 4 Prevention 4
with a posterior triceps-splitting approach Technical inadequacy 4a: Anatomic reduction of
opposed to those with a lateral approach or articular surface with
nonoperative management. Remia compared the placement of an
interfragmentary
functional outcome of elbows after a Bryan- compression screw
Morrey approach versus the triceps-splitting 4b: Reduce columns with
approach (Remia 2004). They found no statisti- Kirschner wires and
cally signicant difference between the two with stabilize with plate
regard to either range of motion or function. conguration
Pitfall 5 Prevention 5
Failure to start early 5a: Start early motion
Surgical Pitfalls and Prevention motion might develop 5b: Discuss possibility of
stiffness second surgery (e.g.,
capsular release) with
Managing an uncommon and severe injury such family at the time injury
as a T-condylar fracture in children is challenging.
52 Distal Articular Humerus Fractures
Management of Complications
It is important to counsel the parents that a
T-condylar fracture is a serious injury and 100 %
recovery is unlikely. There is almost always residual
elbow stiffness regardless of the treatment modality
used. Inform the family that if adequate motion is
not achieved by about 6 months from surgery, a
second surgery may be required to regain motion.
Malunion or nonunion of the fracture has been
reported and is usually related to inadequate
reduction, insufcient xation, or both.
Neurovascular compromise has not been reported
in the literature but can be a potential complica-
tion as in supracondylar fractures. Ulnar nerve
neuropraxia has been reported postoperatively
which usually resolves over a few weeks.
Intercondylar fracture
Common
complications Management
Stiffness of the Capsular release
elbow
Ulnar nerve Observation. Usually resolves over
neuropraxia 612 weeks
Nonunion and Corrective osteotomy for malunion
malunion and compression plating and bone
grafting for nonunion
Osteonecrosis of No successful management.
the trochlea Symptomatic relief achieved with
removal of loose bodies and
arthroscopic debridement
Summary
In summary, distal articular humerus fractures are
commonly encountered in a pediatric trauma prac-
tice. Both operative and nonoperative treatments
should be considered based on the expertise of the
surgeon and the available resources. A clear
understanding of the anatomy, pathoanatomy,
and surgical principles is necessary for successful
management of these often challenging fractures.
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 Medial Epicondyle Fractures, Elbow
Dislocations, and Transphyseal 53
Separations

Rachel Y. Goldstein, James Lee Pace, and David L. Skaggs

Contents Abstract
Medial Epicondyle Fractures . . . . . . . . . . . . . . . . . . . . . 1167 Medial epicondyle fractures, transphyseal frac-
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1167 tures, and elbow dislocations represent a spec-
Pathoanatomy and Applied Anatomy . . . . . . . . . . . . . . 1168 trum of pediatric elbow injuries. These injuries
Mechanism of Injury . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1168 can coexist, such as in the case of elbow dislo-
Assessment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1169
Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1171 cations and medial epicondyle fractures.
Preferred Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1177 Transphyseal fractures are thought to represent
Complications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1177 the corollary to the elbow dislocation in the
Transphyseal Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . 1178 very young patient. A thorough understanding
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1178 of both pediatric elbow anatomy and develop-
Pathoanatomy and Applied Anatomy . . . . . . . . . . . . . . 1179 ment is required to effectively identify and
Mechanism of Injury . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1179
Assessment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1180
treat these injuries. A high index of suspicion
Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1182 should exist in any pediatric elbow swollen
Preferred Technique . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1185 secondary to trauma, as these injuries may be
Complications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1186 difcult to identify.
Elbow Dislocations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1187
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1187
Pathoanatomy and Applied Anatomy . . . . . . . . . . . . . . 1187 Medial Epicondyle Fractures
Mechanism of Injury . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1188
Assessment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1188
Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1190 Introduction
Preferred Technique . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1195
Complications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1195 Medial epicondyle fractures are common frac-
Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1198 tures in the pediatric population. They are the
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1198 third most common pediatric elbow fracture,
following supracondylar and lateral condyle
fractures, and account for anywhere from 11 %
to 20 % of all pediatric elbow fractures (Smith
1950; Chessare et al. 1977; Landin 1983; Cheng
R.Y. Goldstein (*) J.L. Pace D.L. Skaggs et al. 1999). These fractures most commonly
Keck School of Medicine, Childrens Orthopaedic Center, occur in boys between 9 and 14 years of age,
Childrens Hospital Los Angeles and University of
with the peak incidence at 1112 years of age
Southern California, Los Angeles, CA, USA
e-mail: rgoldstein@chla.usc.edu; jpace@chla.usc.edu; (Smith 1950). Up to 60 % of medial epicondyle
dskaggs@chla.usc.edu fractures are associated with an elbow dislocation
# Springer Science+Business Media New York 2015 1167
J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_54
1168
(Patrick 1946; Smith 1950). Despite the frequent
occurrence of these fractures, and the plethora of
retrospective literature evaluating medial epico-
ndyle fractures, there continues to be controversy
about the evaluation and indications for treatment
(Mehlman and Howard 2012).
Pathoanatomy and Applied Anatomy
The distal humerus has four primary ossication
centers. The capitellum is the rst to appear at age
612 months. The medial epicondylar ossication
center appears around 57 years of age. The troch-
lea begins to ossify at 710 years, and the lateral
epicondyle appears at 1214 years. The medial
epicondyle is the last ossication center to
fuse to the distal humerus and does not fuse until
1520 years of age (Haraldsson 1959). Like most
biologic systems, there are variations in this pattern.
Eccentric ossication and multicentric ossication
of the medial epicondyle have both been previously
described (Silberstein et al. 1981). Sex-specic
variations in the appearance and closure of the
medial epicondyle ossication center have also
been cited, with females tending to have their
ossication center appear and fuse at an earlier
age than males (Patel et al. 2009).
The medial epicondyle is the anatomic origin
for the exor-pronator mass, which includes the
exor carpi radialis, exor digitorum supercialis,
palmaris longus, and a portion of the pronator teres.
In addition, the ulnar collateral ligament originates
from the most inferior portion of the medial
epicondyle (Fuss 1991; Munshi et al. 2004). The
anterior band of the ulnar collateral ligament acts as
the major stabilizing ligamentous structure of the
medial elbow (Schwab et al. 1980). Disruption of
the anterior band of the anterior bundle can produce
marked valgus instability (Schwab et al. 1980).
The ulnar nerve is derived from the medial
cord of the brachial plexus and contains contri-
butions from both the C8 and T1 nerve roots. It
enters the cubital tunnel as it passes under the
medial epicondyle and rests against the posterior
portion of the ulnar collateral ligament. It is in
this anatomic position that it is most at risk from
injury with an associated medial epicondyle
R.Y. Goldstein et al.
fracture (Roberts 1934; Patrick 1946; Josefsson
and Danielsson 1986; Farsetti et al. 2001; Lima
et al. 2013).
The vascular anatomy about the elbow consists
of three major arcades: the medial, lateral, and
posterior. The medial arcade is formed by the
superior and inferior ulnar collateral arteries, as
they anastomose with the posterior ulnar recurrent
branch of the ulnar artery. This arcade loops
around the medial epicondyle. The medial
epicondyle derives its blood supply anteriorly
from branches of the inferior ulnar collateral
artery and posteriorly from smaller branches of
the medial arcade (Yamaguchi et al. 1997).
Mechanism of Injury
Three mechanisms of injury have been proposed to
cause medial epicondyle fractures: direct trauma,
avulsion, and combined in association with an
elbow dislocation (Gottschalk et al. 2012). The
direct trauma mechanism hypothesizes that a direct
blow to the medial epicondyle can result in this
fracture. With this mechanism of injury, the medial
epicondyle is typically fragmented and there is
signicant swelling and ecchymosis of the sur-
rounding soft tissue (Gottschalk et al. 2012).
More commonly, however, a medial epicondyle
fracture occurs secondary to an indirect pull of the
exor-pronator wad and the ulnar collateral liga-
ment (Smith 1950; Kilfoyle 1965; Lee et al. 2005).
A valgus stress is placed on the elbow joint while
falling on to an outstretched hand with the elbow in
extension. The sudden increased tension in the
exor-pronator mass and ulnar collateral ligament
places tension on the medial epicondyle, and an
avulsion fracture can result. Authors have proposed
that further stress to the medial epicondyle can be
imparted with supination of the forearm and exten-
sion of the wrist and hand (Smith 1950). To support
this theorized mechanism, isolated avulsions of the
medial epicondyle have been documented in ado-
lescents during arm wrestling (Lokiec et al. 1991;
Nyska et al. 1992).
In up to 60 % of cases, medial epicondyle
fractures are associated with an elbow dislocation
(Aitken and Childress 1938; Fowles et al. 1990).
53 Medial Epicondyle Fractures, Elbow Dislocations, and Transphyseal Separations
Fig. 1 (a) AP radiograph depicting incarcerated medial epicondyle fracture in ulnohumeral joint. (b) Lateral radiograph
also showing incarcerated fragment
This pattern results when the ulnar collateral lig-
ament combined with the exor-pronator wad
provides the avulsion force. The elbow disloca-
tions are most commonly posterolateral, though
they may be posteromedial or directly lateral. In
1520 % of cases, the medial epicondyle becomes
incarcerated in the joint (Smith 1950; Lee
et al. 2005; Fig. 1ab).
Assessment
Clinical Presentation
Patients presenting with medial epicondyle frac-
tures tend to be older children and younger ado-
lescents and are generally male (Smith 1950;
Chessare et al. 1977; Cheng et al. 1999). The
patient may give a history of a fall onto an out-
stretched, extended arm and may describe an
associated dislocation.
The physical examination is often variable
depending on the amount of fracture displacement
(Gottschalk et al. 2012). Common ndings include
soft tissue swelling, ecchymosis, local tenderness,
and crepitus on palpation. A defect can sometimes
be palpable in highly displaced fractures, although
this is not always assessed for due to patient
1169
discomfort. Range of motion will often be limited
secondary to pain. However, with an incarcerated
fragment, there may be a block to elbow extension
with a hard stop on attempted range of motion
testing. Patients with an associated elbow disloca-
tion may also have an obvious deformity.
In all patients with a medial epicondyle frac-
ture, it is essential to test for medial elbow stability
(Schwab et al. 1980; Gottschalk et al. 2012).
Stress testing may be difcult secondary to pain
and is generally not recommended in the alert
child, but can be performed under sedation if
necessary for a complete evaluation. A gravity-
assisted valgus stress test may be performed
(Schwab et al. 1980). This is performed by having
the patient lie supine with his/her arm abducted
90 . The shoulder and arm are externally rotated
90 with the elbow held in 15 degrees of exion
(which eliminates the olecranon as a stabilizing
force). Simple gravitational stress alone should
demonstrate medial elbow instability. This infor-
mation can be useful in guiding treatment.
Radiographic Evaluation
Radiographic evaluation of the pediatric elbow
begins with the standard anteroposterior (AP) and
lateral X-rays. However, radiographic interpretation
1170 R.Y. Goldstein et al.

of the pediatric elbow can be difcult secondary to

normal variations, including eccentric ossication
and multicentric ossication (Silberstein et al.
1981), as well as sex-specic variations (Patel
et al. 2009). Nondisplaced medial epicondyle
fractures may show a loss of soft tissue planes;
however, no displacement of the elbow fat pads is
usually seen, as this is an extra-articular fracture
pattern. Minimally displaced fractures will dem-
onstrate a loss of parallelism along the smooth
margins of the physis.
In signicantly displaced fractures, the fracture
becomes quite obvious and the fragment typically
remains proximal to the ulnohumeral joint line
(Fig. 2). If the fragment appears at the level of
the joint, it must be considered in the joint until
proven otherwise (Gottschalk et al. 2012). Radio-
graphs need to be carefully assessed for joint
congruity, as medial epicondyle fractures are
often associated with an elbow dislocation. Even
radiographs that appear to have a congruent joint
must be carefully evaluated for an incarcerated
joint fragment. Previous authors have described
elbows in which the joint appeared radiographi-
cally reduced despite a fragment entrapped in the Fig. 2 AP radiograph of a highly displaced medial
epicondyle fracture. Even with the high degree of displace-
joint (Haah et al. 2010; Petratos et al. 2012; Lima ment, it remains proximal to the elbow joint
et al. 2013).
While recognizing a medial epicondyle frac-
ture is typically straightforward for a trained reliability on internal oblique views (Gottschalk
orthopedist, previous studies have demonstrated et al. 2013). The authors noted that reviewers were
poor reliability in the measurement of displace- 60 % accurate in predicting true displacement on a
ment of medial epicondyle fractures on standard 45 internal oblique radiograph and recommended
radiographs (Edmonds 2010; Pappas et al. 2010; multiplying the measured displacement by 1.4 to
Gottschalk et al. 2013). A 2010 study described better estimate the true displacement of the fracture
signicant variability in intra-observer agree- fragment. Other studies have also recommended
ment in measuring the displacement of medial the use of the internal oblique view to better esti-
epicondyle fractures (Pappas et al. 2010). mate the true displacement of the medial
Reviewers disagreed by greater than two milli- epicondyle fracture fragment (Edmonds 2010).
meters 26 % of the time on AP radiographs.
Disagreement occurred 64 % and 87 % of the Classification
time on lateral and internal oblique radiographs, To date, no single validated classication scheme
respectively. In a study using three-dimensional exists for pediatric medial epicondyle fractures
computed tomography (CT) reconstructions as the (Gottschalk et al. 2012; Mehlman and Howard
gold standard, Edmonds and colleagues found that 2012). Most classication systems describe the
up to 1 cm of displacement was missed on both AP amount of fracture displacement and the associa-
and lateral radiographic views, mostly in the ante- tion with an elbow dislocation. Smith provided the
rior direction (Edmonds 2010). A cadaveric study rst classication schema in 1950 (Smith 1950).
in 2013, however, found excellent intra-observer Later classication systems by Papavasiliou and
53 Medial Epicondyle Fractures, Elbow Dislocations, and Transphyseal Separations
Table 1 Classification of medial epicondyle fractures
Papavasiliou (1982)
Type 1 Small avulsion of the epicondylar fragment
Type 2 Avulsed epicondylar fragment on the same
level as the joint, but not incarcerated
Type 3 Avulsed fragment trapped in the joint
Type 4 Avulsion of the fragment associated with an
elbow dislocation and the fragment in the joint
Mercer-Rang (Mehlman and Howard 2012)
Type 1 Minimally displaced fragment
Type 2 Displaced, rotated fragment
Type 3 Avulsed fragment trapped in the joint
Type 4 Avulsion of the fragment associated with an
elbow dislocation and the fragment in the joint
Gottschalk et al. (2012)
Type 1 Acute
A Nondisplaced: physeal line remains intact
B Minimally displaced (<5 mm)
C Signicantly displaced (>5 mm)
Type 2 Chronic
A Tension stress injuries
B Little league elbow
Mercer-Rang followed and have been used to vary-
ing degrees in clinical practice and for research
purposes (Table 1; Papavasiliou 1982; Dias
et al. 1987; Lee et al. 2005; Haxhija et al. 2006).
However, these classications do not address val-
gus instability (Mehlman and Howard 2012).
Another proposed classication schema that has
been applied to medial epicondyle fractures by
Gottschalk et al. is based on the chronicity of the
fracture and associated displacement (Table 1;
Gottschalk et al. 2012). None of these classication
systems are uniformly used in guiding treatment
decision-making.
Management
Treatment Controversies
There has been signicant debate regarding the
treatment of medial epicondyle fractures. Previous
studies have attempted to summarize the existing
literature, but have been unable to provide conclu-
sive evidence to guide treatment of these fractures
(Kamath et al. 2009a; Mehlman and Howard
2012). No consensus exists in the literature as to
the amount of fracture displacement that warrants
1171
surgical intervention. Studies have argued for oper-
ative intervention in fractures with as little as 2 mm
of displacement (Hines et al. 1987; Fowles
et al. 1990; Lee et al. 2005), while other authors
argue that up to 20 mm of displacement on plain
radiographs is acceptable (Smith 1950; Josefsson
and Danielsson 1986; Farsetti et al. 2001).
Advocates of nonsurgical management have
demonstrated that patients who are treated
nonoperatively have outcomes similar to or better
than those who undergo surgical treatment (Smith
1950; Chessare et al. 1977; Josefsson and
Danielsson 1986; Wilson et al. 1988). These
authors argue patients have excellent outcomes
even if the fracture heals with a brous union
(Josefsson and Danielsson 1986).
Those who argue for surgical treatment of
medial epicondyle fractures tout the importance
of the medial soft tissue attachments in maintaining
joint stability and allowing early elbow motion, to
minimize the risk of arthrobrosis. These authors
argue that, given the mechanism of injury in medial
epicondyle fractures (valgus force in extension),
the capsuloligamentous and anteromedial muscular
structures can be damaged and inuence joint sta-
bility and eventual outcome far more than actual
fragment displacement (Schwab et al. 1980;
Louahem et al. 2010; Smith 1950; Kilfoyle
1965). A 2010 study of pediatric medial epicondyle
fractures demonstrated that 21 % of patients treated
nonoperatively developed a symptomatic non-
union requiring operative treatment (Smith
et al. 2010).
The amount of fracture displacement and its
exact contribution to medial elbow instability has
never been clearly dened. The classic study by
Josefsson suggests that the approximate rate of
late valgus instability among children with medial
epicondyle fractures displaced between 4 and
8 mm will be approximately 2 % (Josefsson and
Danielsson 1986).
Nonoperative Treatment
Indications/Contraindications
Indications for nonoperative treatment include
those patients who have a minimum amount of
displacement, though current literature does not
1172 R.Y. Goldstein et al.

Table 2 Indications/contraindications for non-operative

management of medial epicondyle fracture
Indications Contraindications
Minimal Open fracture
displacement Fracture fragment incarcerated in
joint
Inability to achieve concentric joint
reduction

give a clear indication for the amount of accept-

able displacement. Patients should also not have
evidence of signicant ulnar nerve dysfunction,
though again this is not clearly dened. Nono-
perative treatment is contraindicated in patient
with open fractures, those with fracture fragments
incarcerated in the joint, concomitant elbow dis-
location, those in whom a concentric reduction of
the elbow joint cannot be achieved, and possibly
valgus stress athletes with displaced fractures
(Table 2).

Technique
In those patients to be managed without surgery,
immobilization in a long arm cast with the elbow
exed 90 and neutral forearm rotation for
4 weeks is generally sufcient, though radio-
graphic union is not always the expected outcome,
especially for displaced fractures (Fig. 3). Once
the period of immobilization is complete, range of
motion exercises can be initiated with a gradual
return to full activity once range of motion is
maximized. For those patients with the fracture
fragment incarcerated in the joint, a nonoperative
reduction technique has been described.
The Roberts maneuver involves a valgus force to
open the joint, followed by forearm supination
and nger extension (Roberts 1934). However,
most now consider an incarcerated fracture frag-
ment to be an indication for operative treatment,
so the usefulness of the Roberts maneuver may be
questioned.
Outcomes
Traditionally, nonsurgical management of humeral
medial epicondyle fractures largely yields good to
excellent results (Josefsson and Danielsson 1986;
Farsetti et al. 2001). A 1975 study demonstrated
that almost 80 % of nonoperatively treated
Fig. 3 AP radiograph showing a healed medial
epicondyle fracture with at least partial bony healing
fractures had a functional, pain-free, stable, non-
deformed elbow with less than a 15 loss of range
of motion (Bede et al. 1975). Similarly, a 2007 study
demonstrated that no patient treated nonoperatively
had loss of strength or range of motion and had
similar outcomes scores to those treated surgically
(Ip and Tsang 2007). A systematic review found a
trend towards less pain in those patients treated
nonoperatively (Kamath et al. 2009a).
Operative Treatment
Indications and Contraindications
There are very few absolute indications for oper-
ative treatment of medial epicondyle fractures
(Table 3). The only absolute indications are open
fractures and fractures incarcerated in the joint
(Aitken and Childress 1938; Fowles et al. 1984b,
1990; Dias et al. 1987; Wilson et al. 1988; Farsetti
et al. 2001; Gottschalk et al. 2012). Relative indi-
cations for operative intervention are much
53 Medial Epicondyle Fractures, Elbow Dislocations, and Transphyseal Separations
Table 3 Indications/contraindications for operative man-
agement of medial epicondyle fracture
Absolute Relative
indications indications Contraindications
Open fracture Ulnar nerve Minimal
dysfunction displacement
Fragment Valgus
incarcerated in joint instability
Inability to achieve High-
concentric joint demand
reduction patient
broader. Measured displacement on radiographs is
inconsistent (Edmonds 2010; Pappas et al. 2010;
Gottschalk et al. 2013), and no cutoffs for accept-
able displacement have been established (Smith
1950; Josefsson and Danielsson 1986; Hines
et al. 1987; Fowles et al. 1990; Lee et al. 2005;
Smith et al. 2010).
Ulnar nerve dysfunction is often considered an
indication for operative xation (Roberts 1934;
Patrick 1946; Josefsson and Danielsson 1986;
Farsetti et al. 2001). Signicant ulnar nerve symp-
toms in the acute setting raise concerns that the
ulnar nerve is impinging in the fracture site or
within the elbow joint. Moreover, late ulnar nerve
symptoms secondary to compromise of the ulnar
nerves normal path around the medial epicondyle
occur in approximately 5 % of nonoperatively
treated fractures (Josefsson and Danielsson 1986;
Farsetti et al. 2001) and can occur up to 39 months
after the injury (Royle and Burke 1990). However,
a systematic review found that there were no dif-
ferences in outcomes for patients with preoperative
ulnar nerve symptoms that were treated surgically
versus nonsurgically (Kamath et al. 2009a).
Valgus instability has frequently been cited as
an indication for operative treatment of medial
epicondyle fractures given the likely associated
soft tissue injuries and potential implications on
long-term outcomes (Smith 1950; Kilfoyle 1965;
Louahem et al. 2010). Some authors argue that
all patients with a medial epicondyle fracture
should undergo valgus stress testing to look
for medial instability (Schwab et al. 1980;
Gottschalk et al. 2012).
The third relative indication is a medial
epicondyle fracture in high-demand valgus stress
1173
athletes such as pitchers and gymnasts (Kamath
et al. 2009a). However, it has been pointed out
that at the age of 11 or 12 it is often difcult to
determine which patients will be high-level ath-
letes (Mehlman and Howard 2012). A 2013 case
series of 20 athletes compared patients who
underwent operative and nonoperative treatment
of their medial epicondyle fractures (Lawrence
et al. 2013). Patients were treated nonoperatively
if their injury resulted from a low-energy injury
and the patient had no evidence of elbow instabil-
ity on valgus stress testing. The authors did not
employ a strict cutoff for the amount of acceptable
displacement. Of the 14 patients treated nono-
peratively, all were able to return to their sports
at the next appropriate level and none of the
patients felt that their performance was limited
after treatment.
Techniques
Numerous techniques for the operative treatment
of medial epicondyle fractures have been
described including suture repair (Wilson et al.
1988; Fowles et al. 1990; Lee et al. 2005),
Kirschner (K-) wire xation (Papavasiliou 1982;
Hines et al. 1987; Wilson et al. 1988; Fowles
et al. 1990; Farsetti et al. 2001; Lee et al. 2005;
Ip and Tsang 2007; Louahem et al. 2010; Park and
Kwak 2012), screw xation (Wilson et al. 1988;
Fowles et al. 1990; Case and Hennrikus 1997;
Lee et al. 2005; Haxhija et al. 2006; Kamath
et al. 2009a, b; Park and Kwak 2012), and exci-
sion of the epicondylar fragment and suturing the
soft tissue to the periosteum of the medial elbow
(Farsetti et al. 2001; Gilchrist and McKee 2002).
Screw xation is the most often utilized technique
today. This treatment allows for an anatomic
reduction, rigid xation, and early mobilization
(Gottschalk et al. 2012). However, K-wires have
also been utilized with similarly good results
(Park and Kwak 2012). Wire migration, although
a rare phenomenon, must be monitored (Cheng
et al. 1997; Sykora et al. 2013). Excision of the
epicondylar fragment has been known to lead to
persistent elbow pain despite suturing the soft
tissue back to the periosteum of the medial
elbow (Farsetti et al. 2001) and has largely fallen
out of favor.
1174 R.Y. Goldstein et al.

Surgical Procedure Table 4 Preoperative planning for screw fixation of

medial epicondyle fracture
Preoperative Planning OR table Hand table attachment
See Table 4. Position Supine
Lateral
Positioning Prone (Glotzbecker et al. 2012)
Patients can be positioned in several different Fluoroscopy From patients head
ways to maximize access to the medial aspect of Equipment 40 cannulated screw set +/ washers
Tourniquet Sterile tourniquet
the elbow. A supine position can be used with the
Misc Esmarch to aid in reduction (Kamath
patients entire operative extremity on a hand table
et al. 2009b)
(Kamath et al. 2009b). Alternatively, the patient
may be positioned laterally with the operative arm
draped across the patient and the surgical team
standing towards the patients foot. Finally, a
prone position may be employed. A hand table is
used and the arm is kept internally rotated, exed,
and pronated (Glotzbecker et al. 2012).
Fluoroscopy setup is based on surgeon prefer-
ence and patient positioning. While bringing uo-
roscopy in from the patients head is common,
other methods can be employed, with the focus
on obtaining the maximum amount of space avail-
able to work. Given the supercial location of the
epicondyle, a tourniquet can be considered
optional, but the authors nd it useful. If a tourni-
quet is used, it may be either sterile or non-sterile.
In smaller children, a sterile tourniquet may be
preferable, as it tends to increase the available
surgical eld relative to a non-sterile tourniquet
after prepping and draping.

Surgical Approach
Medial epicondyle fractures are accessed via a
posteromedial approach that is centered just ante-
rior to the anatomic position of the medial
epicondyle (Kamath et al. 2009b), though an inci-
sion just posterior to the medial epicondyle allows
better exposure of the nerve and may be more
cosmetic. The incision should allow for exposure
of the fracture site, the fracture fragment, and the
ulnar nerve (Fig. 4). The medial epicondyle frag-
ment is usually displaced anteriorly and distally.
Careful debridement of the fracture bed should be
undertaken and obstructions to fracture reduction
should be removed to allow for an anatomic
reduction. The ulnar nerve should be identied
and kept out of harms way with a vessel loop
(Fig. 4). If there are preoperative ulnar nerve
Fig. 4 Intraoperative photo of operative xation of a
medial epicondyle fracture. The exposure is wide in this
case to fully expose and protect the ulnar nerve. In this
case, the patient had a dense preoperative ulnar nerve palsy.
The fracture fragment is grasped with a pointed reduction
clamp and the fracture bed is fully exposed for later
reduction
symptoms, identication and dissection of the
ulnar nerve is highly recommended.
Surgical Technique
To reduce the fracture fragment back to the fracture
bed, wrist exion relaxes the exor wad attached to
the fracture fragment (Table 5). Additionally, an
53 Medial Epicondyle Fractures, Elbow Dislocations, and Transphyseal Separations
Esmarch bandage can be applied from the distal to
proximal direction to allow for gentle milking of
the soft tissues and the fragment towards the
humerus (Kamath et al. 2009b). While making
certain the ulnar nerve is out of harms way, provi-
sional reduction is often achieved with some vari-
ety of a pointed instrument such as a dental pick,
18-gauge needle (Kamath et al. 2009b), pointed
reduction forceps, or towel clamp (Fig. 4). The
guidewire for the cannulated screw can be used as
well to joystick the fracture into position.
A narrow gauge K-wire can be used to transx the
fragment to the humerus in order to prevent rotation
as the guidewire for the cannulated screw is
advanced (Kamath et al. 2009b; Fig. 5). Great care
Table 5 Surgical steps
Posteromedial incision
Identify and protect ulnar nerve
Identify displaced fracture fragment
Reduce fragment
Transx fragment to distal humerus
Pass guidewire in superolateral direction
Place 4.0 mm cannulated partially threaded screw
(+/ washer)
Reattach soft tissues to medial epicondyle
Irrigate and close
Fig. 5 Intraoperative
photo depicting provisional
reduction and xation with
a K-wire while the
cannulated guidewire is also
in place. The ulnar nerve is
also exposed at the posterior
aspect of the incision, but it
is not widely dissected due
to an intact preoperative
neurological exam
1175
must be taken with this technique, particularly with
small fragments of bone that may be easily split.
The guidewire for the cannulated screw should
be advanced in a superolateral direction between
the medial cortex and the olecranon fossa (Fig. 6a
and b). Since the medial epicondyle is both pos-
terior and medial, the guidewire should be
directed anterior enough to stay within the distal
shaft of the humerus. The screw should be
directed up the lateral column. Avoid the pitfall
of a screw in the olecranon fossa, which acts as a
mechanical block to extension (Fig. 7ab).
In addition, the guidewire should not penetrate
the far cortex, as the radial nerve is at risk of
iatrogenic injury (Marcu et al. 2011). Once the
guidewire position is conrmed on AP and lateral
uoroscopic images, the near cortex is breached
with a 2.7 mm drill, and a 4.0 mm partially or fully
threaded cancellous screw is placed. Care should
be taken not to apply too much compression, or
risk splitting the fracture fragment. In very
hard bone one may want to consider drilling the
proximal screw path as well. A washer can be used
to increase the surface area for compression and
prevent screw head penetration or fragmentation
of the piece. However, inclusion of a washer
increases screw head prominence and may result
in an increased need for hardware removal.
1176 R.Y. Goldstein et al.

Fig. 6 Appropriate screw placement on (a) AP and (b) lateral radiographs

Fig. 7 Inappropriate screw placement on (a) AP and (b) lateral radiographs. The screw is too horizontal and crosses into
the olecranon fossa, which may cause a mechanical block to extension

Postoperative Protocol pain after surgery. After that time, immobilization

Postoperatively, patients are immobilized in a is removed and patients are encouraged to begin
long arm cast versus a posterior splint for 12 range of motion exercises. The use of a hinged
weeks to allow the soft tissues to heal (Table 6). elbow brace can be utilized to protect any healing
A simple sling is also an option, but may increase medial soft tissue structures. Strengthening
53 Medial Epicondyle Fractures, Elbow Dislocations, and Transphyseal Separations 1177

Table 6 Postoperative protocol several factors need to be taken into account. First
Type of Long arm cast is the presence of a concomitant elbow dislocation.
immobilization Patients with a medial epicondyle fracture and an
Length of 2 weeks elbow dislocation have an inherently more unsta-
immobilization ble fracture pattern with more soft tissue damage
Rehab protocol Gentle active range of motion, and are thus more likely to benet from operative
advance to active assist as pain
allows intervention. The second factor is the amount of
Return to Once full motion returns displacement seen. Minimally displaced fractures
sports (less than 510 mm) can be treated conservatively
in the absence of an associated elbow dislocation.
Generally, patients with greater than 10 mm of
exercises can typically begin 46 weeks after sur-
displacement, an associated elbow dislocation,
gery, and most patients can return to sports,
and ulnar nerve symptoms or high-demand valgus
including throwing, 3 months after surgery.
stress athletes are considered appropriate surgical
candidates.
Outcomes
Surgical intervention is generally carried out as
In general, quality outcomes data are sparse. Early
described above with the patient in the supine
outcome studies show good functional results in
position and the operative extremity on a hand
62.5 % of surgically treated fractures (Bede
table. The uoroscopy machine is brought in
et al. 1975). When patients were compared using
from the head to allow the surgical team to sit in
the Mayo Clinic Performance Index, an instru-
the region of the patients axilla. Dissection,
ment which emphasizes pain and activities of
reduction, and xation are carried out as described
daily living, both groups achieved high average
above. Use of a washer is based on surgeon pref-
scores, and there was no signicant difference
erence. Use of a washer increases screw head
between the two groups (Ip and Tsang 2007).
prominence and decreases patient comfort; how-
Most patients are able to return to their previous
ever, its use may minimize the chance of fracture
level of activity after treatment for a medial
fragmentation. Patients are immobilized for a
epicondyle fracture. In a case series of 20 athletes
period of generally 1 week to allow for soft tissue
treated surgically for medial epicondyle fractures
healing before beginning range of motion exer-
and followed for greater than 2 years, all of the
cises. Strengthening is initiated at 34 weeks post-
patients achieved excellent outcomes and all of
operatively. Patients are allowed to return to
the overhead athletes were able to return to their
sports typically 3 months postoperatively. Screw
sport at the next appropriate level (Lawrence
removal is based on surgeon preference. Some
et al. 2013). None of the patients felt that their
prefer to leave hardware in unless symptomatic,
performance was limited after treatment. How-
while others prefer to routinely remove hardware
ever, in a large meta-analysis, operatively treated
at 36 months postoperatively if the patient has
patients also trended towards more pain at nal
signicant growth remaining.
follow-up when compared with those patients
treated nonoperatively (Kamath et al. 2009a).
Given the nature of the study, the authors were
Complications
unable to comment on the reason(s) for the
increased pain (prominent hardware, higher-
Complications in the treatment of medial
energy injury with more soft tissue damage, etc.).
epicondyle fractures include loss of motion,
cubitus valgus, bony nonunion, and surgical com-
Preferred Treatment plications such as infection and nerve and blood
vessel damage. Loss of motion is commonly seen
In determining whether a medial epicondyle fracture in both operatively and nonoperatively treated
should be treated operatively or nonoperatively, fractures. In nonsurgically treated patients loss of
1178
extension is reported in 2030 % of patients with
an average loss of extension of 10 or more. Less
than 5 % will have an extension loss greater than
30 (Smith 1950; Josefsson and Danielsson
1986; Wilson et al. 1988). In those patients
treated operatively, loss of extension was found
to be higher than those treated without surgery
(Fowles et al. 1990; Louahem et al. 2010). In
1990, Fowles noted an average loss of 37 degrees
of extension loss in patients who underwent
operative treatment for a medial epicondyle frac-
ture with an associated elbow dislocation com-
pared with only 15 in the nonsurgical group
(Fowles et al. 1990). Similarly, Hines et al.
(1987) noted a loss of range of motion in patients
requiring arthrotomy to remove an interposed
fracture fragment, which was unrelated to the
degree of displacement, the time from injury to
surgery, or the duration of immobilization. How-
ever, this difference may be secondary to the fact
that those patients who underwent surgery
sustained a more severe injury with increased
risk of elbow stiffness from the injury itself.
Additionally, most of the cited studies are some-
what dated and operative techniques and rehabil-
itation protocols have changed considerably. The
authors have not found signicant loss of motion
if therapy is started early.
Recent small case series have reported on
patients with symptomatic valgus instability after
nonoperative treatment (Gilchrist and McKee
2002; Smith et al. 2010). Gilchrist and McKee
(2002) published a report on three pediatric
patients with symptomatic valgus instability sec-
ondary to long-standing medial epicondyle non-
unions. A second case series reported on eight
patients with medial epicondyle nonunions, all
of whom experienced ongoing pain (Smith
et al. 2010). Half of these patients had concomi-
tant valgus instability of the elbow, and all of these
patients were successfully treated with open
reduction and screw xation with selective use
of bone grafting. The postoperative rate of valgus
instability is less than 1 % (Lee et al. 2005; Ip and
Tsang 2007; Louahem et al. 2010; Mehlman and
Howard 2012).
Nonunion in patients treated nonoperatively is
of unclear signicance. In a recent meta-analysis,
R.Y. Goldstein et al.
patients who were treated nonoperatively had
only a 49.2 % rate of bony union (Kamath
et al. 2009a). Other studies have identied a non-
union rate of more than 60 % when medial
epicondyle fractures are treated nonoperatively,
all with excellent clinical results and no ongoing
issues of instability (Josefsson and Danielsson
1986; Wilson et al. 1988; Farsetti et al. 2001). In
comparison, a systematic review found a 92.5 %
rate of bony union in patients treated surgically
(Kamath et al. 2009a), and a pooled analysis of
recent studies found only one out of 188 patients
treated surgically for a medial epicondyle fracture
failed to achieve a bony union (Lee et al. 2005; Ip
and Tsang 2007; Louahem et al. 2010; Mehlman
and Howard 2012).
Cubitus valgus has been reported in up to
35.5 % of patients (Bede et al. 1975) and is a
phenomenon of nonoperatively treated fractures.
Most cases are mild and not clinically signicant,
with less than 10 % of patients demonstrating
substantial valgus deformity (Pimpalnerkar
et al. 1998; Farsetti et al. 2001; Lee et al. 2005).
Due to the relatively sparse literature, it is
unknown if this is a clinically signicant problem.
Surgical complications are rare in operatively
treated medial epicondyle fractures (Bede
et al. 1975; Hines et al. 1987; Wilson et al. 1988;
Ip and Tsang 2007; Marcu et al. 2011; Mehlman
and Howard 2012). The infection rate seen with
these fractures is approximately 0.5 % (Lee
et al. 2005; Ip and Tsang 2007; Louahem
et al. 2010; Mehlman and Howard 2012). There
have been no reports to date of late ulnar nerve
symptoms seen postoperatively (Mehlman and
Howard 2012); however, a case series exists
describing two iatrogenic radial nerve injuries
secondary to far cortex penetration by a guidewire
or screw (Marcu et al. 2011).
Transphyseal Fractures
Introduction
Transphyseal distal humerus fractures are a rare
fracture pattern. The rst complete description of
this injury that differentiated it from supracondylar
53 Medial Epicondyle Fractures, Elbow Dislocations, and Transphyseal Separations
humerus fractures and elbow dislocations appeared
in 1850 (Smith 1850). However, prior to the
1970s, these fractures were largely described in
individual case reports (Marmor and Betchol
1960; Siffert 1963; Mauer et al. 1967; Omer and
Simmons 1968; Kaplan and Reckling 1971).
Later series eventually reported on a total of
45 transphyseal distal humerus fractures (Mizuno
et al. 1979; DeLee et al. 1980; Holda et al. 1980;
Peiro et al. 1981; McIntyre et al. 1984; Akbarnia
et al. 1986; de Jager and Hoffman 1991). Even
with that, data on this fracture is sparse.
Transphyseal fractures tend to occur in neo-
nates and young children, with almost all reports
in patients younger than 4 years of age (DeLee
et al. 1980; Moucha and Mason 2003). The peak
incidence is seen from newborn to two and a half
years old, and there are no reports in patients older
than 7 years (DeLee et al. 1980).
The true incidence of these fractures is likely
underreported, as they tend to occur in very young
patients and often go unrecognized. One series
found that 50 % of fractures that were eventually
diagnosed as a transphyseal fracture were not
correctly diagnosed until 16 days after the injury
(DeLee et al. 1980). Prompt diagnosis and treat-
ment of these fractures requires a high index of
suspicion.
Pathoanatomy and Applied Anatomy
At birth, the entire distal humerus is cartilaginous.
Secondary ossication centers in the distal
humerus do not appear until approximately 111
months of age in girls and 126 months in boys
(Haraldsson 1959). The distal humeral physes
fuse to one another at 1012 years of age and
fuse to the distal humerus by 1216 years of age
(Haraldsson 1959).
The volume of the distal humerus occupied by
the distal epiphysis is larger in younger children.
As the humerus matures, the physeal line pro-
gresses more distally with a central V forming
between the medial and lateral condylar physes.
This V-shaped conguration of the physeal line
helps to protect the more mature distal humerus
from physeal fractures (Ashurst 1910).
1179
The blood supply to the medial crista of
the trochlea courses directly through the physis
(Haraldsson 1959). As such, the blood supply to
this area is vulnerable to injury with transphyseal
fractures. Injury to these vessels places the patient
at risk for osteonecrosis in the medial crista of the
trochlea.
Mechanism of Injury
The exact mechanism of injury for these fractures
is unknown and likely varies by age. In neonates,
a transphyseal fracture can present as a birth injury
(Siffert 1963; Berman and Weiner 1980; Downs
and Wirth 1982; Barrett et al. 1984; Akbarnia
et al. 1986). Some authors postulate that because
the physeal line is more proximal in young infants
and nearer to the center of the olecranon fossa, a
hyperextension injury in this age group is more
likely to result in a physeal separation as opposed
to a bony supracondylar fracture (Dameron 1981).
In older children, with a relatively more distal
physis, a hyperextension injury to the elbow con-
centrates the forces generated by the olecranon in
the supracondylar area of the humerus, resulting
in a supracondylar humerus fracture (DeLee
et al. 1980).
Others argue that infants and younger children
tend to have some residual exion contracture of
the elbow from intrauterine positioning, and these
contractures may prevent the hyperextension-type
mechanisms (DeLee et al. 1980). Previous studies
have shown that the physis is more likely to fail
with rotatory shear forces than with pure bending
or tension forces (Bright et al. 1974). In younger
children, forces applied to the elbow are more
likely to be rotatory shear, such as those secondary
to birth trauma or child abuse (DeLee et al. 1980).
Most importantly, many reports have identied
a very high incidence of conrmed or suspected
child abuse in infants and young children with
these injuries (DeLee et al. 1980; Akbarnia
et al. 1986; Willems et al. 1987; de Jager and
Hoffman 1991). One series found that out of
16 transphyseal fractures, six were secondary to
conrmed or suspected child abuse (DeLee
et al. 1980).
1180
Assessment
Clinical Presentation
A careful history is required in all patients
suspected of having this fracture. Birth trauma is
a common cause of transphyseal fractures. How-
ever, in all patients in whom this is not a viable
mechanism, child abuse must be ruled out (DeLee
et al. 1980; Akbarnia et al. 1986; Willems
et al. 1987; de Jager and Hoffman 1991). Previous
reports have conrmed or suspected child abuse
as the mechanism for transphyseal fractures in up
to a third of patients being evaluated (DeLee
et al. 1980; Akbarnia et al. 1986; Willems
et al. 1987; de Jager and Hoffman 1991).
The clinical presentation of transphyseal frac-
tures can be variable by age. Birth injuries at the
time of delivery resulting in these fractures tend
not to have especially impressive exams, with
only moderate swelling and little crepitus being
reported (Siffert 1963). Often, the only sign pre-
sent in newborns with this injury may be
pseudoparalysis of the involved limb. Careful
clinical examination and radiographic evaluation
are required to differentiate this injury from a
brachial plexopathy and/or other birth fractures.
As young children may have only minimal swell-
ing and this fracture is easily missed, transphyseal
fractures should be suspected in any infant under
18 months of age whose elbow is swollen second-
ary to trauma (Siffert 1963).
In younger children, there may be marked
swelling apparent, with an elbow that appears
dislocated on radiographs (DeLee et al. 1980).
If palpable, the relationship, however, between
the epicondyles and the olecranon is maintained
(Fig. 8a). Rotatory deformity, as opposed to angu-
lar deformity, is typically seen with these fractures
as the fracture fragments have a wide surface area,
which minimizes tilting of the distal fragment
(Fig. 8b). Poland described a soft crepitus pre-
sent with transphyseal fractures (DeLee
et al. 1980). Gentle manipulation of the forearm
against the humerus produces abnormal motions
about the elbow joint and a mufed crepitus. This
occurs as motion between the two cartilaginous
fracture fragments produces a crepitus distinct
R.Y. Goldstein et al.
from that produced with two bony fragments
(DeLee et al. 1980). This mufed crepitus is con-
sidered diagnostic of a physeal separation.
Radiographic Evaluation
Radiographic evaluation of these fractures can be
difcult, especially if the ossication center of the
capitellum is not visible yet, as in an infant.
Although the distal humerus cannot be seen on
routine radiographs in extremely young patients,
its location can be inferred from the position of the
ossied portions of the proximal radius and ulna.
However, in patients who do not yet have a
capitellar ossication center, other imaging may
be needed to conrm the diagnosis of this fracture,
including magnetic resonance imaging (MRI),
ultrasound (US), or an elbow arthrogram (Mizuno
et al. 1979; Hansen et al. 1982; Barrett et al. 1984;
Akbarnia et al. 1986).
These fractures can be difcult to distinguish
from other pediatric elbow injuries (DeLee
et al. 1980), namely, elbow dislocations. In addi-
tion, transphyseal fractures can also be confused
with other injuries including supracondylar frac-
tures, lateral condyle fractures, or Monteggia frac-
tures. All reports of transphyseal fractures, with
rare exceptions (Berman and Weiner 1980), dem-
onstrate posteromedial displacement of the distal
fragment (Marmor and Betchol 1960; Siffert
1963; Mauer et al. 1967; Omer and Simmons
1968; Kaplan and Reckling 1971; Sutherland
and Wrobel 1974; DeLee et al. 1980; Oh
et al. 2000). Elbow dislocations tend to be lateral,
with a medial dislocation being extremely rare
(Linscheid and Wheeler 1965; Roberts 1969;
Neviaser and Wickstrom 1977; Royle 1991).
Additionally, as most transphyseal fractures in
older children have a small ake of metaphyseal
bone, or corner sign, with the distal fragment
(Fig. 9), the absence of a bony fragment in older
children can assist in distinguishing an elbow
dislocation from a transphyseal fracture (DeLee
et al. 1980). On the radiograph of a normal elbow,
a line drawn down the long axis of the radius
passes through the capitellum (Smith 1947,
1967; Storen 1959; Rogers 1978; Ruo 1987).
This relationship should be maintained in all
53 Medial Epicondyle Fractures, Elbow Dislocations, and Transphyseal Separations
Fig. 8 (a) AP of a transphyseal fracture. No ossication of
the humeral epiphysis is present and this could easily be
mistaken for an elbow dislocation. (b) A lateral radiograph
shows a lateral image of the proximal forearm but a near
elbow fractures and is disrupted only in disloca-
tions that include disruption of the radiohumeral
joint. Conrmation of the injury can be performed
with live uoroscopy under sedation. As the fore-
arm is manipulated anteriorly and posteriorly, the
medial and lateral condyles of the humerus
(if ossied) and olecranon maintain a constant
relationship to each other (DeLee et al. 1980).
This clearly differentiates a physeal separation
from an elbow dislocation. Finally, transphyseal
fractures tend to occur in neonates and young
children (DeLee et al. 1980; Holda et al. 1980;
Akbarnia et al. 1986; de Jager and Hoffman
1991), while elbow dislocations are much more
common in children nearing skeletal maturity
(Henrikson 1966; Josefsson and Nilsson 1986).
The key distinguishing features of a
supracondylar fracture are that the entire fracture
line is visible in the supracondylar area above the
epiphyseal plate and the relationship between the
1181
AP of the distal humerus. This nicely depicts the rotatory
displacement that is often characteristic of transphyseal
fractures and is one of the distinguishing characteristics
from an elbow dislocation
proximal forearm and humeral epiphysis is
maintained. Some transphyseal fractures are
actually SH II injuries with a small ake of
metaphyseal bone. This can be confused with a
lateral condyle fracture. However, in lateral con-
dyle fractures, the radius will maintain its rela-
tionship with the intact portion of the capitellum,
while the fractured portion displaces laterally. In
transphyseal fractures, the ake of bone will stay
with the proximal radius (Fig. 9a and b). With
Monteggia fractures, the relationship between
the radius and ulna is altered, while the relation-
ship between the ulna and humeral epiphysis,
and therefore with the distal humeral metaphysis,
is maintained.
Classification
No single, validated, widely used classication
system exists for transphyseal fractures of the
distal humerus (Table 7). DeLee and colleagues
1182 R.Y. Goldstein et al.

Fig. 9 (a) AP radiograph of a transphyseal fracture in the radius and the capitellum are in line with each other. (b)
setting of child abuse. The capitellar ossication center is The lateral radiograph helps conrm the diagnosis with the
just now forming. The forearm has an abnormal relation- small metaphyseal ake of bone characteristic of a
ship with the distal humeral metaphysis but the proximal transphyseal fracture

Table 7 Classification of transphyseal fractures (DeLee These patients may have a small ake of
et al. 1980) metaphyseal bone on the distal fragment (Fig. 9a
Lateral and b). Group C is older children 37 years of age.
condylar These patients have a large Thurston-Holland-type
epiphysis Metaphyseal
metaphyseal fragment. These fragments are most
Age ossication fragment
Group A <12 months No No
commonly lateral, though they may be medial or
Group B 12 months Yes Minimal
posterior.
to 3 years
Group C 3 years to Yes Large
7 years Management

proposed a classication system based on the
degree of ossication of the lateral condyle epiph-
ysis (DeLee et al. 1980). Group A is infants up to
12 months of age, prior to the appearance of the
lateral condyle epiphysis. No metaphyseal frag-
ment is seen on the distal fragment. These frac-
tures are usually Salter-Harris type I fractures
and are often missed secondary to the lack of
ossication in the lateral epiphysis. Group B is
children 12 months to 3 years of age with den-
itive ossication of the lateral condyle epiphysis.
Nonoperative Management
Indications/Contraindications
There are very few indications for nonoperative
management of transphyseal distal humerus frac-
tures (Table 8). The rst is delayed presentation or
recognition. If the patient does not present until
56 days after the initial injury, the epiphysis is
usually no longer mobile, and callus is already
present (DeLee et al. 1980). Attempted manipula-
tion at this point puts the physis at increased risk
for further injury and will likely not result in an
53 Medial Epicondyle Fractures, Elbow Dislocations, and Transphyseal Separations
Table 8 Indications/contraindications for operative man-
agement of transphyseal distal humerus fractures
Absolute indications Contraindications
Open fracture Delayed
presentation
Signicant fracture Neonates
displacement
improved outcome. In addition, neonates or very
small infants in whom general anesthesia or pin
xation may be difcult may be suitable candi-
dates for nonoperative management.
Techniques
For patients who present in a delayed fashion, no
attempts should be made at reduction after 57
days, depending on the age of the patient. These
patients should be splinted or casted for comfort
for a total of 3 weeks from the time of injury. It is
better to allow these patients to heal and treat any
resulting deformity with a supracondylar osteo-
tomy than to risk physeal injury or osteonecrosis
of the physis (DeLee et al. 1980; Holda et al.
1980; Morrissy and Wilkins 1984; Moucha and
Mason 2003). In neonates who are felt to be too
young to undergo closed reduction and pinning,
the affected extremity may be immobilized in
90 degrees of exion with the forearm pronated
for posterior medial displacement while being
very cognizant of swelling and the risk of com-
partment syndrome. The extremity may be stabi-
lized with a gure-of-eight splint or a cast if size
permits. Total duration of immobilization should
be approximately 3 weeks.
Outcomes
In patients younger than 2 years old, restoration of
appearance and function can be complete even
without treatment (DeLee et al. 1980). Many
essentially untreated transphyseal separations
remodel completely without any residual defor-
mity if the distal fragment is only medially trans-
lated and not tilted. Most of these fractures have
very little tilt as the fracture fragments have wide
surfaces on which to rest, minimizing the varus or
valgus angulation. Moreover, because the intra-
1183
articular surface is not involved, complete func-
tional recovery can usually be expected.
Operative Management
Indications/Contraindications
In most older infants and younger children, casting
or splinting is not enough to maintain a reduction
(Table 8). Additionally, as with supracondylar frac-
tures, it is important to avoid casting elbows in
greater than 90 degrees of exion to decrease the
risk of compartment syndrome. Almost all acute
transphyseal distal humerus fractures are indicated
for operative intervention. Previous authors have
recommended treatment of this fracture when it is
recognized in the acute setting to prevent late defor-
mity (de Jager and Hoffman 1991).
Techniques
Transphyseal distal humerus fractures are treated
with closed reduction and percutaneous pinning.
Surgical Procedure
Preoperative Planning
See Table 9.
Positioning
Patients can be positioned in several different ways
to maximize radiographic evaluation of the elbow.
A supine position can be used with the patients
entire operative extremity on a hand table. If the
patient is small, he or she may be brought to the
very edge of the bed with his/her head positioned
on the hand table. Care should be taken to position
the childs head in such a way that it is not at risk
during fracture reduction. Alternatively, if the
patient is very small, he or she can be rotated 90
such that the legs rest on the hand table and the
operative extremity is extended on the edge of the
bed. This decreases the risk of inadvertently pulling
the patient off of the bed during fracture reduction.
If the patient is very small, one can consider using a
attop Jackson table. In all patient positions, the
uoroscopy machine can come in from the
patients axilla, allowing the surgical team the max-
imum amount of room to work.
1184
Table 9 Preoperative planning for closed reduction and
percutaneous pinning of transphyseal distal humerus
fracture
OR table Hand table attachment
Position Supine
Fluoroscopy From patients axilla
Equipment 0.062 in. K-wires
Tourniquet Not needed
Misc Radio-opaque dye if considering
arthrogram
Surgical Approach
Reduction of transphyseal fractures is performed
using a closed technique. Open reduction of this
fracture carries a high risk of damage to the pos-
teriorly based blood supply and increases the risk
of osteonecrosis of the trochlea (Rogers and
Rockwood 1973; Holda et al. 1980; Akbarnia
et al. 1986; Willems et al. 1987). However,
Mizuno et al. (1979) recommended a primary
open approach posteriorly by removing the triceps
insertion from the olecranon with a small piece of
cartilage secondary to his poor results their closed
reduction. This technique is rarely necessary and
closed reduction techniques generally provide
excellent results for transphyseal fractures.
Surgical Technique
Initially, the elbow is manipulated into extension
(Table 10). Correction of the medial displacement
is then performed by translating the distal piece
laterally. Once the piece has been manipulated
into position, the forearm is pronated and the
elbow is exed to secure the fragment reduction.
Reduction should then be evaluated on AP and
lateral uoroscopic images.
Fracture reduction may be difcult to evaluate
in patients with a transphyseal fracture secondary
to limited ossication of the distal humerus. In
these patients, an arthrogram may aid in the eval-
uation of the reduction (Josefsson et al. 1984a;
Akbarnia et al. 1986). The arthrogram can be
performed through a lateral approach within the
triangle formed by the radial head, the lateral
condyle, and the olecranon. Alternatively, the
arthrogram may be performed by passing the nee-
dle proximal to the olecranon into the joint.
A mixture of one half radio-opaque dye and one
R.Y. Goldstein et al.
Table 10 Surgical steps Supine position
Manipulation of arm
into extension
Correction of medial
displacement
Forearm pronation and
elbow exion
Consider arthrogram
to aid in evaluation of
reduction
Place 2  0.062
K-wires from lateral
entry
Evaluate fracture
reduction and pin
position
Cast in neutral forearm
rotation and elbow
exion
half normal saline is injected into the joint,
allowing the surgeon to visualize the cartilaginous
distal humerus (Fig. 10a and b).
Once fracture reduction is conrmed, an
appropriately sized Kirschner wire, typically
0.062 in., is placed from the lateral aspect of the
elbow, crossing the fracture site and engaging the
medial cortex. Once the pin position is conrmed,
a second pin is passed in a similar fashion, ensur-
ing it is divergent to the rst pin. Fluoroscopic
images should be used to evaluate the pin position
and nal fracture reduction. The pins are cut and
bent, and a long arm cast or splint is applied.
Postoperative Protocol
Patients are casted with the forearm in a neutral
position and slightly less than 90 degrees of elbow
exion (Table 11). Radiographs are taken 1 week
postoperatively to conrm maintenance of the frac-
ture reduction and pin position. The cast is removed
at 3 weeks and the pins are removed in the ofce at
that time once repeat radiographs have conrmed
union. Ranges of motion exercises are initiated,
with the family instructed in how to perform them,
and full activity is allowed 2 weeks afterwards.
Outcomes
As no large series of these injuries exist, outcomes
research on transphyseal fractures is scarce. The
data is typically reported in terms of range of
53 Medial Epicondyle Fractures, Elbow Dislocations, and Transphyseal Separations 1185

Fig. 10 (a) AP and (b) lateral intraoperative photos of a reduction and pin placement in this child who has no
successful closed reduction and pinning of a transphyseal ossication of the humeral epiphysis
fracture. The arthrogram is critical in conrming adequate

Table 11 Postoperative protocol presenting with traumatic elbow injuries. Careful

Type of Long arm cast
radiographic evaluation is required, and advanced
immobilization imagining is utilized when the diagnosis is
Length of 3 weeks unclear. All patients who are diagnosed with a
immobilization transphyseal distal humerus fracture must be
Rehab protocol Gentle active range of motion, screened for child abuse.
advance to active assist as pain Surgical intervention is carried out as described
allows
above with the patient in the supine position
Return to Once full motion returns
sports and the operative extremity on a hand table. The
uoroscopy machine is brought in from the axilla
to allow the surgical team to work in the region of
the patients head. Closed manipulation of the frac-
motion, growth arrest, and residual deformity, ture is carried out as described above. Two lateral
though complications after treatment of this frac- pins are placed. Use of a medial pin is rarely, if
ture are rare. To date, there are no studies that ever, needed as the fracture site is much broader
report on patient or parent satisfaction, or on and thus more stable than a supracondylar humerus
return to activity following transphyseal fractures. fracture.
Postoperatively, patients are immobilized in a
Preferred Technique long arm cast with foam padding to allow for
swelling. Casts are placed with the forearm in
It is essential to have a high index of suspicion for neutral rotation and the elbow in slightly less
this fracture in all neonates and young children than 70 degrees of exion. The remainder of the
1186
protocol is as described above. Physical therapy is
rarely needed for these patients.
Complications
Complications in the treatment of transphyseal
distal humerus fractures include cubitus varus,
trochlear avascular necrosis (AVN), loss of range
of motion, and delayed diagnosis (DeLee
et al. 1980). Other complications that are common
to elbow injuries including growth arrest and
neurovascular injury have not been seen with
this fracture pattern. DeLee postulated that growth
arrest does not occur with transphyseal fractures
because compressive damage to physeal cells is
not common with this injury pattern (DeLee
et al. 1980). Shear forces, not compressive forces,
are responsible for this injury. Neurovascular inju-
ries associated with this fracture have not been
reported in the literature (DeLee et al. 1980; Oh
et al. 2000). This is most likely secondary to the
fact that the fracture fragments are covered in
cartilage and that the fragments are not usually
markedly displaced.
Cubitus varus is a well-described complication
of transphyseal fractures (DeLee et al. 1980;
Holda et al. 1980; Micheli et al. 1980; McIntyre
et al. 1984; Morrissy and Wilkins 1984; de Jager
and Hoffman 1991; Oh et al. 2000). The risk of
this deformity has been reported at variable rates.
DeLee described 9 out of 12 of the patients in his
series as having a measurable difference in carry-
ing angle at nal follow-up but noted that only
3 of these 9 showed a varus deformity of 5 10
(DeLee et al. 1980). A second series found varus
deformity in 3 out of 10 patients (de Jager and
Hoffman 1991), while a third series found 7 out of
12 patients with cubitus varus ranging from 5 to
15 (Oh, Park et al. 2000). In all three series, all of
the patients with varus deformity were less than
2 years old. Despite the emphasis on this defor-
mity, cubitus varus remains less commonly seen
in transphyseal fractures than after supracondylar
humerus fractures. This may be due to the large
cross-sectional area of the fracture surface at the
epiphyseal plate. This makes the fracture contact
much greater, making rotation, tilt, and cubitus
varus less likely (DeLee et al. 1980).
R.Y. Goldstein et al.
Some authors postulate that the cubitus varus
that is seen with these injuries is secondary to
trochlear AVN (Yoo et al. 1992; Oh et al. 2000).
Yoo et al. (1992) reported on a series of eight
patients with trochlear AVN after transphyseal frac-
tures. The authors found that six of the eight were
initially misdiagnosed as medial condyle fractures,
lateral condyle fractures, or elbow dislocations.
And all eight patients had rapid dissolution of the
trochlea 36 weeks after the injury followed by a
medial or central condylar shtail defect. In a series
of seven patients with cubitus varus at 2 years after
transphyseal fractures, six patients had an ossica-
tion defect of the medial humeral condyle, consis-
tent with AVN of the trochlea.
Toniolo and Wilkins (1996) suggested a classi-
cation system for trochlear AVN. Type A is a
shtail deformity. Only the lateral portion of the
medial cristae or apex of the trochlea is involved.
This results in the typical shtail deformity. This
type of AVN is seen in very distal supracondylar
fractures or in those involving the lateral condylar
physis. Type B is a malignant varus deformity. This
involves AVN of the entire trochlea and sometimes
part of the metaphysis. It occurs as sequelae of
fractures involving the entire distal humeral physis
or fractures of the medial condylar physis (Varma
and Srivastava 1972). This can lead to progressive
angulation as the child matures. Treatment of this
complication is aimed at the sequelae of the AVN.
For patients who have a loss of range of motion,
there are no good treatment options. For those who
develop a clinically signicant cubitus varus defor-
mity, a supracondylar osteotomy with ulnar nerve
transposition can be performed (Davids et al. 2011).
Because this fracture pattern does not have an
intra-articular component, signicant loss of
range of motion after treatment is rare. One
study found that less than half of patients had a
measurable loss of elbow exion of 5 15 and
just over half had a measurable loss of elbow
extension of 5 15 (DeLee et al. 1980). None
of these limitations in range of motion were of any
functional signicance. Patients with the largest
measurable loss were those who were immo-
bilized for greater than 3 weeks.
Delayed diagnosis of transphyseal distal
humerus fractures is a signicant concern. Of the
53 Medial Epicondyle Fractures, Elbow Dislocations, and Transphyseal Separations 1187

12 patients seen with a transphyseal fracture, de Table 12 Elbow stabilizers

Jager and colleagues (1991) found that four had Static stabilizers
initially been diagnosed with a different type of Dynamic Ligamentous
elbow fracture: three lateral condyle fractures and stabilizers Osseous Radial Ulnar
one elbow dislocation. Another study found that Anconeus Coronoid Annular Ulnar
3 out of 16 patients were not seen for treatment ligament collateral
ligament
until after callus formation had already begun
Triceps Olecranon Radial
(DeLee et al. 1980). A high index of suspicion is
collateral
necessary to appropriately diagnose and treat these ligament
fractures. Patients who present after callus forma- Brachialis Radial head Lateral
tion generally are splinted for comfort until healing. ulnar
Most patients have no clinical deformity or residual collateral
ligament
disability on 2-year follow-up (DeLee et al. 1980).
Capitellum
Nonunion is an extremely rare complication
Ulnohumeral
with this fracture. Only one nonunion of a articulation
transphyseal distal humerus fracture has been Radiocapitellar
reported in the literature (Mizuno et al. 1979). joint
Likely, the rich vascularity and propensity for
rapid bone formation around the young childs
elbow is responsible for the rarity of nonunions. joint, namely, the triceps, brachialis, and anconeus
muscles. They play a modest role in elbow stabil-
ity by providing joint compressive forces. The
Elbow Dislocations static elbow stabilizers provide most of the stabil-
ity of the elbow. These are divided into osseous
Introduction and ligamentous structures. The osseous struc-
tures include the coronoid and olecranon of the
Pure elbow dislocations in children are not com- ulna. These structures create a semicircle with
mon. Henrikson studied 1,579 elbow injuries in which the trochlea of the distal humerus articu-
skeletally immature patients in Sweden and found lates and thus forms a stable ulnohumeral articu-
only 45 elbow dislocations, equivalent to an inci- lation. The ulnohumeral joint provides stability
dence of 3 % (Henrikson 1966). The peak inci- against medial-lateral or longitudinal translation.
dence of pediatric elbow dislocations occurs at Additionally, the concave surface of the radial
approximately 1214 years of age, when the head matches the convex capitellum and provides
physes begin to close (Henrikson 1966; Josefsson stability to the lateral aspect of the elbow joint.
and Nilsson 1986; Carlioz and Abols 1984). The radiocapitellar joint provides resistance to
These injuries are seen more commonly in boys axial compression and is a secondary restraint to
than girls (Carlioz and Abols 1984). Most pediat- valgus stress. Flexion and supination are positions
ric elbow dislocations occur in conjunction with of stability, while extension and pronation are
falls or sports activities (Carlioz and Abols 1984; positions of relative instability.
Josefsson et al. 1984a). Discussion of the stabilizing ligamentous
structures begins with the annular ligament that
encircles the radial head and neck. It both origi-
Pathoanatomy and Applied Anatomy nates and inserts on the lateral aspect of the prox-
imal ulna and prevents distal translation of the
Elbow stability is provided by two sets of struc- radius. The radial collateral ligament originates
tures: dynamic elbow stabilizers and static elbow from the lateral epicondyle of the humerus and
stabilizers (Table 12). The dynamic elbow stabi- fans out to merge with the annular ligament. It
lizers consist of the muscles that cross the elbow functions as a restraint to varus stress and
1188
stabilizes the annular ligament (Martin 1958;
Mehta and Bain 2004). The lateral ulnar collateral
ligament (LUCL) is a thickening of the capsule
that attaches proximally to the lateral humeral
epicondyle and distally to the tubercle of the supi-
nator crest of the ulna. This ligament acts to both
stabilize the lateral aspect of the joint and as a
posterior buttress to prevent radial head subluxa-
tion (ODriscoll et al. 1991, 1992; McKee
et al. 2003; Mehta and Bain 2004). Medially, the
ulnar collateral ligament is divided into three com-
ponents: the anterior band, the posterior band, and
the transverse ligament of Cooper. The anterior
band originates from the medial epicondyle of the
humerus and inserts on the sublime tubercle of the
ulna and is the primary stabilizer to valgus stress
(Morrey and An 1985). The posterior band spans
from the medial epicondyle to the olecranon pro-
cess and is a restraint against ulnar internal rota-
tion on the humerus (Mehta and Bain 2004). The
transverse ligament of Cooper runs from the olec-
ranon to the coronoid but has little functional
importance.
Mechanism of Injury
Posterior and posterolateral elbow dislocations
occur with an abduction/valgus and extension
force. The injury begins with disruption of the
ulnar collateral ligaments and/or failure of the
medial epicondyle apophysis with a large valgus
load (Schwab et al. 1980; Sojbjerg et al. 1987;
ODriscoll et al. 1992). This results in valgus
instability. With continued stress, the proximal
radius and ulna displace laterally with the intact
biceps acting as the center of rotation for the
displaced forearm. The radial head displacement
strips away the capsule and periosteum from the
posterolateral aspect of the lateral condyle.
Anterior elbow dislocations typically occur
secondary to a direct blow to the posterior aspect
of a exed elbow (Inoue and Horii 1992). How-
ever, hyperextension of the elbow has also been
implicated. Twisting of the forearm on the elbow
also typically occurs, resulting in medial or lateral
displacement. Convergent and divergent disloca-
tions are usually caused by high-energy trauma. In
R.Y. Goldstein et al.
addition to the hyperextension of the elbow that
causes the dislocation, a strong proximally
directed force is applied parallel to the long axis
of the forearm, disrupting the annular ligament
and interosseous membrane and allowing the
divergence of the proximal radius and ulna
(Altuntas et al. 2005).
Assessment
Clinical Presentation
Elbow dislocations are typically seen in older
children and adolescents (Henrikson 1966;
Josefsson and Nilsson 1986; Carlioz and Abols
1984). The elbow will typically have considerable
swelling but without evidence of crepitus. The
arm position will differ based on the direction of
the dislocation. Patients with a posterior elbow
dislocation typically hold the affected elbow
semi-exed, while those with an anterior disloca-
tion will hold their elbow in extension.
Patients with a posterior elbow dislocation may
appear to have shortening of their forearm. Prom-
inence is produced by the distal humeral articular
surface, which becomes more distal and may be
palpated. The tip of the olecranon is typically
dislocated posteriorly and proximally so that its
triangular relationship with the medial and lateral
epicondyles is lost. In posterolateral dislocations,
the radial head may be prominent and easily pal-
pable. However, swelling with an elbow disloca-
tion may be considerable and palpation of bony
landmarks is often difcult.
Patients who have sustained an anterior dislo-
cation typically present with fullness in the
antecubital fossa. These patients have marked
swelling secondary to the signicant soft tissue
disruption associated with this injury (Rasool
2004). Lateral dislocations may have good exion
and extension of the elbow. However, these
patients will demonstrate a widened appearance
of the elbow secondary to the olecranon, which is
displaced lateral to the capitellum.
Patients with elbow dislocations have a high
rate of associated fractures, and therefore, a high
index of suspicion should be present for concom-
itant injuries when evaluating these patients
53 Medial Epicondyle Fractures, Elbow Dislocations, and Transphyseal Separations
(Winslow 1913; Henderson and Robertson 1952;
Linscheid and Wheeler 1965; Neviaser and
Wickstrom 1977; Carey 1984; Carlioz and Abols
1984; Royle 1991; Inoue and Horii 1992; Vicente
and Orduna 1993; Wilkerson 1993). More than
half of pediatric patients seen for a posterior
elbow dislocation have an associated fracture
(Henderson and Robertson 1952; Linscheid and
Wheeler 1965; Neviaser and Wickstrom 1977;
Carlioz and Abols 1984; Royle 1991). Medial
epicondyle fractures are the most commonly seen
fractures associated with elbow dislocations
(Carlioz and Abols 1984). The origins of the
ulnar collateral ligament and the medial forearm
exor muscles remain as a unit with the medial
epicondyle. With signicant valgus force, this unit
is stripped from its humeral origin as the underly-
ing apophysis of the medial epicondyle is generally
weaker than the musculoligamentous attachments
(Cromack 1960). Less commonly, fractures of the
radial head or neck, coronoid process, lateral
epicondyle, lateral condyle, olecranon, capitellum,
or trochlea can be seen (Carlioz and Abols 1984).
Anterior dislocations may be associated with a
triceps avulsion fracture (Winslow 1913) or an
olecranon fracture (Winslow 1913). Inoue and
Horii (1992) reported an anterior dislocation that
was associated with displaced fractures of the
trochlea, capitellum, and lateral epicondyle.
Divergent or convergent dislocations may be
associated with fractures of the radial neck, prox-
imal ulna, or the coronoid process (Carey 1984;
Vicente and Orduna 1993).
Soft tissue injury associated with elbow dislo-
cations appears to be the rule rather than the
exception. In a series of 62 adolescents and adults
with elbow dislocations requiring surgical treat-
ment, McKee and colleagues found that all 62 had
disruption of the lateral collateral ligament com-
plex (McKee et al. 2003). If the medial epicondyle
remains attached to the humerus, generally the
ulnar collateral ligaments and the muscular ori-
gins of the common exor muscles tear (McKee
et al. 2003). The structure most commonly torn
on the lateral aspect of the elbow is the annular
ligament (Sojbjerg et al. 1987). Occasionally,
the lateral collateral ligament either avulses a
small osteochondral fragment from the lateral
1189
epicondyle or tears completely within its sub-
stance (Carlioz and Abols 1984).
While a rare occurrence, open elbow disloca-
tions do occur (Henderson and Robertson 1952;
Linscheid and Wheeler 1965; Louis et al. 1974;
Rubens and Aulicino 1986; Stans 2010). These
dislocations are usually secondary to a high-
energy mechanism and are associated with signif-
icant soft tissue injury, including a high incidence
of associated arterial injury (Linscheid and
Wheeler 1965; Louis et al. 1974). Operative treat-
ment is always necessary in these cases to debride
the open wound and evaluate the brachial artery.
Radiographic Evaluation
AP and lateral radiographs are usually sufcient
to diagnose an elbow dislocation (Stans 2010).
Radiographic appearance is variable based on
the direction of the dislocation. Often times there
is increased superimposition of the distal humerus
on the proximal radius and ulna in the AP view
(Fig. 11a). Posterior dislocations will demonstrate
a radial head that is displaced proximally and
laterally or directly behind the mid-distal
humerus. Valgus angulation between the forearm
and the arm will be greater than normal. Most
telling is the lateral view, which will show the
coronoid process lying posterior to the condyles
(Fig. 11b). In anterior dislocations, the proximal
radius and ulna dislocate anteromedial.
Radiographs need to be carefully evaluated for
concomitant fractures, as these are seen in more
than 50 % of pediatric elbow dislocations (Hen-
derson and Robertson 1952; Linscheid and
Wheeler 1965; Neviaser and Wickstrom 1977;
Carlioz and Abols 1984; Royle 1991). CT scan
or MRI may be required to further delineate asso-
ciated injuries (Fig. 12ad).
Classification
Classication of elbow dislocations is based on
the position of the proximal radioulnar joint rela-
tive to the distal humerus (Table 13; Stans 2010).
Posterior elbow dislocations are the most com-
monly seen elbow dislocations in both children
and adults (Linscheid and Wheeler 1965; Roberts
1969; Neviaser and Wickstrom 1977; Carlioz and
Abols 1984; Josefsson and Nilsson 1986;
1190
Fig. 11 (a) AP radiograph of an elbow dislocation showing the more subtle superimposition of the distal humerus and
olecranon. (b) The lateral radiograph clearly shows the dislocation
Royle 1991). As the forearm often rotates around
an intact biceps tendon resulting in medial or
lateral displacement, posterior elbow dislocations
can be further subdivided into posteromedial and
posterolateral dislocations. Anterior elbow dislo-
cations are rare and constitute less than 2 % of all
pediatric elbow dislocations (Linscheid and
Wheeler 1965; Roberts 1969; Neviaser and
Wickstrom 1977; Royle 1991). These dislocations
are associated with an increased incidence of com-
plications compared to the more common poste-
rior dislocations (Jackson 1940; Wilkerson 1993).
Direct medial and lateral dislocations are
extremely rare injuries, though lateral dislocations
are more common than medial. There are no
recent reports of medial dislocations in children.
Divergent dislocations are posterior elbow dis-
locations that are associated with a disruption of
the interosseous membrane between the proximal
radius and ulna with the radial head displaced
laterally and the proximal ulna displaced medially
(Carey 1984; Andersen et al. 1985; Vicente and
Orduna 1993). Similarly, there have been reports
of convergent dislocations (Antonis et al. 2011;
Agashe et al. 2012). These are posterior disloca-
tions associated with a proximal radioulnar trans-
location (Fig. 13a and b). Both of these
R.Y. Goldstein et al.
dislocation patterns are extremely rare and are
reported only as individual case reports; no large
series of these injuries exist.
Management
All elbow dislocations require prompt reduction.
If they are left unreduced, there is a predictable
and dramatic loss of elbow function that is char-
acterized initially by limited range of motion and
eventually pain (Naidoo 1982). Unreduced elbow
dislocations are extremely rare in the United
States and most are reported from other countries
(Fowles et al. 1984), and late treatment generally
produces poor outcomes (Naidoo 1982; Fowles
et al. 1984; Mehta et al. 2007).
Nonoperative Management
Indications/Contraindications
Closed reduction of elbow dislocations is success-
ful in most cases. In a combined series of pediatric
elbow dislocations, only 2 out of 317 elbow dis-
locations could not be reduced by closed means
(Linscheid and Wheeler 1965; Roberts 1969;
Neviaser and Wickstrom 1977; Royle 1991).
53 Medial Epicondyle Fractures, Elbow Dislocations, and Transphyseal Separations
Fig. 12 (ab) Pre- and post-reduction photos of an elbow
dislocation and an associated coronoid fracture. (c) CT
scan showing a small, incarcerated fragment in the joint
Another series found that while two dislocations
reduced spontaneously before attempted closed
reduction, 10 % of cases could not be reduced
by closed means (Carlioz and Abols 1984).
A third series reported that 100 % of dislocations
without associated fractures were successfully
reduced closed (Josefsson et al. 1984b). Those
elbow dislocations that were reduced soon after
injury had better outcomes than those in which the
reduction was delayed (Royle 1991). All elbow
1191
that was not seen on plain radiograph. (d) MRI showing a
medial condyle fracture that occurred in conjunction with
an elbow dislocation
dislocations should have an attempt at prompt
closed reduction.
Techniques
Many techniques exist for the reduction of an
elbow dislocation. These techniques are based
on the direction of the dislocation and age of the
patient. All techniques, regardless of the direction
of the dislocation, rely on a reducing force along
the long axis of the humerus to overcome the
1192
contractions of the biceps, brachialis, and triceps
muscles (Stans 2010). A secondary force is then
applied along the long axis of the forearm to pass
the proximal ulna and radius into position.
Regardless of the technique applied, all reductions
require adequate anesthesia or sedation to permit
sufcient muscle relaxation.
In older children and adolescents with a poste-
rior elbow dislocation, a pulling technique may
be utilized (Crosby 1936; Parvin 1957). The fore-
arm is hypersupinated to dislodge the coronoid
process and the radial head from their position
behind the distal humerus (Osborne and Cotterill
1966). The elbow is then exed 90 . A force is
Table 13 Anatomic classification of elbow dislocations
Posterior Medial
Lateral
Anterior Medial
Lateral
Direct lateral
Associated PRUJ dislocation Divergent
Convergent
Fig. 13 (a) AP radiograph of a convergent elbow dislocation. The radius is medial to the ulna. (b) The lateral radiograph
is not so obvious
R.Y. Goldstein et al.
applied to the anterior portion of the forearm with
one hand, while the other hand pulls traction along
the long axis of the forearm. A counterforce is
applied with either direct stabilization or a sheet
around the chest.
Alternatively, a single provider without assis-
tance may employ a similar reduction maneuver.
The patient is placed supine with the shoulder
abducted 90 . The clinician then holds the
patients hand or wrist, hypersupinating the fore-
arm, and places his/her own arm on the patients
arm. The clinician then provides gentle traction
and exes the patients elbow while simulta-
neously applying a downward force on the
patients arm with his or her own arm. The clini-
cians free hand can then be used to translate the
olecranon medially or laterally as needed.
In younger children, the patient may be seated
in the parents lap. The arm is hung over the back
of a well-padded chair. Again the forearm is
maintained in hypersupination. The olecranon is
pushed past the humerus by the physicians
thumb, while the physicians other arm pulls dis-
tally along the axis of the forearm (Lavine 1953).
53 Medial Epicondyle Fractures, Elbow Dislocations, and Transphyseal Separations
This technique may also be utilized in older chil-
dren with the patient lying prone on the table
(Meyn and Quigley 1974).
Anterior elbow dislocations require a different
technique for reduction. Similar to other patterns
of elbow dislocation, force must rst be applied
longitudinally along the axis of the humerus with
the elbow semi-exed in order to overcome the
pull of the biceps and triceps (Stans 2010). Lon-
gitudinal force along the axis of the forearm is
then directed towards the elbow. In patients with
an anterior dislocation, the patient is positioned
supine and the elbow is exed while the forearm is
simultaneously pushed proximally and down-
wards (Winslow 1913). This may be combined
with an anteriorly directed force on the posterior
aspect of the distal humerus.
Lateral elbow dislocations are reduced with a
longitudinal force directed along the long axis of
the humerus to distract the elbow and a direct
medial or lateral pressure applied over the proxi-
mal forearm (Stans 2010). Divergent dislocations
can be reduced by applying a longitudinal traction
with the elbow semi-extended while simulta-
neously compressing the proximal radius and
ulna together (Stans 2010).
Regardless of the technique employed, all
patients require a post-reduction assessment of
stability. Stable range of motion and the results
of varus and valgus stress testing under sedation
should be documented in order to guide further
treatment decisions. A concentric reduction must
be documented with radiographic evaluation.
Care should be taken in patients old enough to
have an ossied medial epicondyle to evaluate the
post-reduction position of the medial epicondyle.
Up to 60 % of pediatric medial epicondyle frac-
tures are associated with an elbow dislocation
(Patrick 1946; Smith 1950), and up to 50 % of
elbow dislocations have associated medial sided
fractures or avulsions (Carlioz and Abols 1984).
Post reduction, patients should be immobilized
either in a long arm cast or in a posterior splint for
a period of 13 weeks (Parvin 1957; Linscheid
and Wheeler 1965; Shankarappa 1998). The stan-
dard position for immobilization, regardless of
dislocation pattern, is 90 degrees of elbow exion
1193
with neutral forearm rotation. Elbows that are not
stable in this position should be immobilized in
the position of stability.
Early range of motion is recommended in sim-
ple elbow dislocations. In adults, a recent study
compared 2 weeks of cast immobilization with the
use of a sling and early motion and found that
early range of motion improved early and nal
outcomes (Maripuri et al. 2007). ODriscoll and
colleagues argue that if the elbow is stable in
response to valgus stress with the forearm
pronated, then the anterior portion of the medial
collateral ligament must be intact and the patient
can begin early range of motion (ODriscoll
et al. 1992). While loss of range of motion is
less common and less substantial in children
than in adults (Josefsson et al. 1984b), immobi-
lization should not extend past 3 weeks (Carlioz
and Abols 1984).
Outcomes
Closed reduction is successful in the majority of
pediatric elbow dislocations (Linscheid and
Wheeler 1965; Roberts 1969; Neviaser and
Wickstrom 1977; Carlioz and Abols 1984; Royle
1991; Stans 2010; Josefsson et al. 1984b). The
usual cause of a failure of closed reduction is
entrapment of a medial epicondyle fracture frag-
ment (Carlioz and Abols 1984). Most patients
treated nonoperatively for elbow dislocations
have successful outcomes. Excellent results have
been reported by Josefsson in 28 children and
adolescents with simple posterior dislocations
treated nonoperatively (Josefsson et al. 1984b).
In the series by Carlioz, only one poor outcome
was observed in patients managed nonoperatively
(Carlioz and Abols 1984). This patient developed
a radioulnar synostosis after concomitant closed
reduction of an elbow dislocation and radial neck
fracture with cast immobilization for 6 weeks.
Operative Management
Indications
While closed reduction is the treatment of choice
for the majority of pediatric elbow dislocations
(Linscheid and Wheeler 1965; Roberts 1969;
1194
Neviaser and Wickstrom 1977; Carlioz and Abols
1984; Royle 1991; Stans 2010; Josefsson et al.
1984b), up to one third of children may require
surgical intervention (Carlioz and Abols 1984;
Table 14). In one series of pediatric elbow dislo-
cations requiring surgical intervention, 30 % were
operated on due to an inability to obtain a concen-
tric reduction, and more than 50 % required sur-
gery to treat a displaced medial epicondyle
fracture (Carlioz and Abols 1984). The remainder
of the patients were treated operatively for other
associated fractures.
Inability to obtain and maintain a concentric
reduction is an absolute indication for operative
intervention. This is a rare occurrence in pediatric
elbow dislocations, with reported rates ranging
from zero to 10 % of patients (Linscheid and
Wheeler 1965; Roberts 1969; Neviaser and
Wickstrom 1977; Carlioz and Abols 1984; Royle
1991; Stans 2010; Josefsson et al. 1984b). Other
absolute indications for operative intervention
include open elbow dislocations (Henderson
and Robertson 1952; Rubens and Aulicino
1986), vascular disruption (Henderson and Rob-
ertson 1952; Rubens and Aulicino 1986), a
displaced osteochondral fracture (Carlioz and
Abols 1984), and a concomitant displaced fracture
(Wheeler and Linscheid 1967; Carlioz and Abols
1984; Fowles et al. 1984, 1990). Factors that were
found to predict the need for operative intervention
include older patient age, instability of the elbow
during examination under sedation at the time of
reduction, presence of a displaced intra-articular
fracture, injury to multiple elbow stabilizers, injury
to the dominant arm, and anticipated signicant
sports or activity demands (Stans 2010).
Most instability related to elbow dislocations
in the pediatric population involves a medial
epicondyle fracture (Henderson and Robertson
1952; Linscheid and Wheeler 1965; Neviaser
and Wickstrom 1977; Carlioz and Abols 1984;
Royle 1991). Other fractures (large coronoid, lat-
eral epicondyle, radial head, etc.) can be seen as
well but are more rare (Winslow 1913; Henderson
and Robertson 1952; Linscheid and Wheeler
1965; Neviaser and Wickstrom 1977; Carey
1984; Carlioz and Abols 1984; Royle 1991;
R.Y. Goldstein et al.
Table 14 Indications/contraindications for operative
management of elbow dislocations
Absolute indications Contraindications
Inability to obtain a Concentric reduction
concentric closed obtained and maintained via
reduction closed means
Open dislocation
Displaced
osteochondral fracture
Concomitant displaced
fracture
Vascular disruption
Inoue and Horii 1992; Vicente and Orduna 1993;
Wilkerson 1993). There has been considerable
debate about the need for operative intervention
in patients with medial epicondyle fractures, even
those associated with elbow dislocations (Smith
1950; Josefsson and Danielsson 1986; Hines
et al. 1987; Fowles et al. 1990; Lee et al. 2005).
Repair of an associated medial epicondyle frac-
ture in the setting of an elbow dislocation may
improve stability (Woods and Tullos 1977;
Schwab et al. 1980).
In older adolescents who are skeletally mature
about the elbow, residual instability after reduc-
tion is typically due to ligamentous injury that
typically requires direct repair or reconstruction
(Durig et al. 1979; Schwab et al. 1980; Josefsson
et al. 1987; McKee et al. 2003).
Techniques
Techniques for the operative treatment of pediat-
ric elbow dislocations are variable and are based
largely on the indication for operative intervention
(Table 15). Inability to achieve and maintain a
concentric closed reduction usually necessitates
an arthrotomy in order to identify the block to
reduction. Most commonly this is an entrapped
medial epicondyle fracture fragment. Alterna-
tively, a cartilaginous ap lifted from the articular
surface of the ulna may prevent reduction (Carlioz
and Abols 1984). Open dislocations require a
thorough irrigation and debridement, including
irrigation and debridement of the joint
(Henderson and Robertson 1952; Rubens and
Aulicino 1986). Patients with a displaced
53 Medial Epicondyle Fractures, Elbow Dislocations, and Transphyseal Separations
Table 15 Surgical techniques in the operative treatment
of elbow dislocations
Indication for surgery Recommended intervention
Inability to obtain a Arthrotomy and open
concentric closed reduction of the joint
reduction
Open dislocation Arthrotomy with thorough
irrigation and debridement
Displaced osteochondral Arthrotomy versus
fracture arthroscopy and treatment
of osteochondral fracture
Concomitant displaced Reduction and xation of
fracture fracture
Vascular disruption Vascular repair or
reconstruction
osteochondral fracture may require arthroscopy
versus arthrotomy for removal of the loose body
and treatment of the donor site. In elbow disloca-
tions with concomitant vascular disruption, most
advocate for vascular repair or reconstruction even
in the presence of adequate capillary rell, to lessen
the risk of cold intolerance, dysesthesias, and
dysvascularity (Louis et al. 1974; Hofammann
et al. 1984; Wilmshurst et al. 1989; Yamaguchi
et al. 1997; Rasool 2004). Concomitant displaced
fractures, such as a medial epicondyle fracture,
require operative treatment of the fracture.
Outcomes
Outcomes for surgical intervention for pediatric
elbow dislocations tend to be good to excellent
(Carlioz and Abols 1984). Only one operatively
treated elbow dislocation out of 19 had a poor
outcome in a single series (Carlioz and Abols
1984). This elbow dislocation was associated
with a radial neck fracture, which was treated
with open reduction and pin xation, and the
patient went on to develop limited forearm rota-
tion secondary to a radioulnar synostosis. Surgical
repair of an associated displaced fracture produces
better results than those treated nonoperatively, as
repair of these associated fractures can improve
stability of the elbow joint, allowing for a more
rapid initiation of range motion (Wheeler and
Linscheid 1967; Woods and Tullos 1977; Schwab
et al. 1980; Carlioz and Abols 1984).
1195
Preferred Technique
Most pediatric elbow dislocations can be treated
without surgery. Closed reduction should be
performed under conscious sedation in the emer-
gency department as soon as possible after the
diagnosis is made. Adequate sedation is required
for any closed reduction to succeed. Any of the
above-described techniques may be used to obtain
reduction. The sensation of a reduction is gener-
ally a palpable clunk; however uoroscopic
imaging will aid in conrming the reduction.
Once reduction is conrmed, the patient is taken
through an arc of exion and extension. Care is
taken to note when a concentric reduction is lost.
Varus and valgus stress maneuvers are also
performed to test for medial and lateral stability.
The patient is then immobilized in a long arm cast
in 90 degrees of elbow exion and neutral forearm
rotation. Post-casting radiographs are taken and
the medial epicondyle is identied in order to
ensure that is not incarcerated in the joint. Patients
are immobilized for a maximum of 2 weeks. Once
the cast is removed, gentle range of motion and
strengthening exercises are initiated. Return to
sports is allowed at 23 months based on the
progress with therapy.
Inability to obtain a concentric reduction, con-
comitant displaced fractures or osteochondral
fragments, open dislocations, and vascular inju-
ries are all absolute surgical indications. As
described above, all displaced medial epicondyle
fractures associated with an elbow dislocation are
treated with open reduction and internal screw
xation.
Complications
Complications associated with elbow dislocations
are rare but well described. These include vascular
injuries, nerve injuries, stiffness, myositis
ossicans, recurrent dislocations or instability,
and unreduced elbow dislocations. Vascular inju-
ries are uncommon in posterior elbow disloca-
tions in children. A combined series of elbow
dislocations in children and adolescents found
1196
vascular injuries in only 3 % of the patients
(Linscheid and Wheeler 1965; Roberts 1969;
Neviaser and Wickstrom 1977; Royle 1991;
Stans 2010). There is, however, an increased inci-
dence of brachial artery rupture or thrombosis
associated with anterior elbow dislocations
(Jackson 1940; Spear and Janes 1951).
Arterial injuries have been associated with
open dislocations in which the collateral circula-
tion is disrupted (Henderson and Robertson 1952;
Louis et al. 1974; Rubens and Aulicino 1986). In
these types of injuries, the brachial artery can be
ruptured (Henderson and Robertson 1952; Louis
et al. 1974), thrombosed (Wilmshurst et al. 1989),
or entrapped in the joint (Hennig and Franke
1980; Wilmshurst et al. 1989; Pearce 1993). The
medial aspect of the distal humerus lies subcuta-
neously between the pronator teres posteriorly
and the brachialis anteriorly. The brachial artery
lies directly over the distal humerus in the subcu-
taneous tissues, leaving it vulnerable to injury.
Louis found a consistent pattern of disruption of
the anastomosis between the inferior ulnar collat-
eral artery and the anterior ulnar recurrent artery
(Louis et al. 1974). If the main brachial artery is
also compromised, loss of the collateral system
can lead to loss of circulation in the forearm and
hand (Louis et al. 1974). Prompt reduction of the
elbow dislocation will return the displaced bra-
chial vessels to their normal position, allowing
normal circulation to resume (Hennig and Franke
1980; Wilmshurst et al. 1989). Vessels that are
incarcerated in the joint or those that are ruptured
or severely damaged require vascular repair or
reconstruction (Louis et al. 1974; Hofammann
et al. 1984; Rubens and Aulicino 1986).
Nerve injuries can also occur with elbow dis-
locations. In the combined series of 317 pediatric
posterior elbow dislocations, 10 % of the patients
had nerve symptoms post reduction (Linscheid
and Wheeler 1965; Roberts 1969; Neviaser and
Wickstrom 1977; Royle 1991; Stans 2010).
Almost two thirds had isolated ulnar nerve symp-
toms, 22 % had isolated median nerve symptoms,
and 12 % had combined ulnar and median nerve
symptoms. There have also been isolated reports
of radial nerve injuries associated with elbow
dislocations (Watson-Jones 1930; Rasool 2004).
R.Y. Goldstein et al.
Table 16 Types of median nerve entrapment (Fourrier
et al. 1977)
Type Features
1 Entrapment within the elbow joint with the
nerve coursing posterior to distal humerus
2 Entrapment of the nerve between the fracture
surfaces of the medial epicondyle and medial
condyle
3 Kinking of the nerve into the anterior portion of
the joint
The ulnar nerve is most at risk with posterior
elbow dislocations secondary to its position pos-
terior to the medial epicondyle. It is the most
commonly injured nerve in elbow dislocations,
and injuries to this nerve almost always resolve
spontaneously (Linscheid and Wheeler 1965;
Roberts 1969; Neviaser and Wickstrom 1977;
Royle and Burke 1990).
The median nerve can be damaged by disloca-
tion directly or can become entrapped in the joint
during closed reduction (Rana et al. 1974;
Fourrier et al. 1977; Ayala et al. 1983; Green
1983; Pritchett 1984; Boe and Holst-Nielsen
1987; Rao and Crawford 1995; Petratos et al.
2012). Entrapment of the median nerve is
suspected in cases of persistent median nerve
motor and/or sensory loss, severe pain, and resis-
tance with elbow exion-extension during the arc
of motion (Stans 2010). MRI can be helpful in
delineating the course of the median nerve in
patients with suspected entrapment (Akansel
et al. 2003). Three types of median nerve entrap-
ment have been described (Table 16; Fourrier
et al. 1977). Type 1 is dened as entrapment
within the elbow joint with the median nerve
coursing posterior to the distal humerus (Steiger
et al. 1969; Fourrier et al. 1977; Ayala et al. 1983;
Green 1983; Pritchett 1984; Boe and Holst-
Nielsen 1987; Rao and Crawford 1995). This
occurs secondary to avulsion of the medial
epicondyle or the medial muscles at their origin.
This allows the median nerve to displace posteri-
orly where it is prone to being entrapped between
the trochlea and olecranon during reduction if the
lateral displacement is not corrected before reduc-
tion. Type 1 entrapment may be indicated by a
Matev sign, a depression seen on the posterior
53 Medial Epicondyle Fractures, Elbow Dislocations, and Transphyseal Separations
surface of the medial epicondylar ridge where the
nerve has been pressed against the bone (Matev
1976). Treatment of this may require neuroma
resection and transposition of the nerve with
direct repair or nerve grafting (Green 1983; Boe
and Holst-Nielsen 1987). Type 2 is entrapment of
the median nerve between the fracture surfaces of
the medial epicondyle and the medial condyle
(Fourrier et al. 1977). With this type of entrap-
ment, the fracture heals and nerve becomes
surrounded by bone (Roaf 1957; Steiger
et al. 1969; Pritchett 1984). In order to treat this,
the medial epicondyle must be osteotomized to
free the nerve. Type 3 is a simple kinking of the
nerve into the anterior portion of the joint
(Pritchard et al. 1973; Rana et al. 1974; Ayala
et al. 1983). This may be treated with decompres-
sion, neuroma resection, and repair.
Almost all patients with elbow dislocations
lose some range of motion of their elbow (Carlioz
and Abols 1984; Josefsson et al. 1984, 1987a, b;
Fowles 1990). Loss of motion may be a signicant
problem in adult elbow dislocations; however,
this loss is signicantly less in children than in
adults (Josefsson et al. 1984b). The motion lost is
usually less than ten degrees of extension and is
rarely of functional or cosmetic signicance
(Josefsson et al. 1984b). This potential for loss
of motion should be discussed with the patients
family prior to reduction and should be reinforced
throughout the treatment period. Range of motion
loss should be minimal, and therefore, severe lim-
itation in range of motion may indicate a displaced
medial epicondyle nonunion, an incongruent
elbow joint, or nerve entrapment (Stans 2010).
Myositis ossicans involves ossication
within the muscle sheath that can lead to a signif-
icant loss of motion about the elbow (Stans 2010).
Disruption of the brachialis is believed to contrib-
ute to the formation of myositis ossicans
(Loomis 1944). This nding is rare in children
and must be distinguished from the more common
but more benign heterotopic ossication
(Josefsson et al. 1984b). In one series of pediatric
elbow dislocations, 10 out of 115 patients had
radiographic evidence of myositis ossicans but
all were asymptomatic (Neviaser and Wickstrom
1977). Another series found that out of 60 patients
1197
with calcication about their elbow after a dislo-
cation, only three had true myositis ossicans
(Roberts 1969). In contrast, heterotopic ossica-
tion is common in the ligaments and capsule of the
elbow after a dislocation, but rarely restricts range
of motion (Linscheid and Wheeler 1965; Roberts
1969; Josefsson et al. 1984b). It occurs in just over
a quarter to one half of patients and is most com-
monly seen around the condyles (Linscheid and
Wheeler 1965; Josefsson et al. 1984b).
Recurrent dislocations are rare. In a combined
series of pediatric elbow dislocations, only 0.6 %
of patients experienced recurrent dislocations
(Linscheid and Wheeler 1965; Roberts 1969;
Neviaser and Wickstrom 1977; Royle 1991;
Stans 2010). The mechanism for recurrent dislo-
cation is thought to be related to collateral liga-
ment laxity, capsular laxity, and bone or articular
cartilage defects (Stans 2010). Lack of a strong
reattachment of the posterior capsule with healing
is thought to contribute to recurrent instability
(Osborne and Cotterill 1966). A large amount of
cartilage on the posterolateral aspect of the lateral
condyle leaves little surface area for soft tissue
reattachment, and the presence of synovial uid
further inhibits bony healing.
Recurrent dislocation is predominantly treated
surgically. Historic reports depict bony proce-
dures that aim to correct the semilunar notch of
the olecranon through the use of a bone block
(Milch 1936; Gosman 1943; Wainwright 1947;
McKellar Hall 1953) and soft tissue procedures
that transfer the biceps tendon distal to the
coronoid process to reinforce it (Reichenheim
1947; King 1953). The lateral capsule may also
be reattached to the posterolateral aspect of the
capitellum with sutures passing through drill
holes (Osborne and Cotterill 1966). Most of these
techniques are only of historical signicance; how-
ever, little has been written about direct repair or
reconstructions in pediatric patients.
Unreduced elbow dislocations are extremely
rare in the United States and most are reported
from other countries (Fowles et al. 1984). Typi-
cally, these patients present with limited range of
motion and increasing pain. Unreduced elbow
dislocations go through a predictable pattern
of pathology (Allende and Freytes 1944;
1198
Fowles et al. 1984). Subperiosteal new bone for-
mation produces a radiohumeral horn and myosi-
tis ossicans develops in the brachialis muscle.
The capsule and the medial and lateral ligaments
contract, while the triceps shortens, and compres-
sion of the ulnar nerve develops. Treatment
includes closed reduction for those recognized
within 3 weeks (Fowles et al. 1984). For those
older than 3 weeks, open reduction can be
performed through a posterior approach (Speed
1925). With this procedure, the triceps needs to
be lengthened and the ulnar nerve released or
transposed. If a stable, concentric reduction can
be achieved within 3 months of the initial dislo-
cation, satisfactory results can occur (Allende and
Freytes 1944). After 3 months, the results gener-
ally decline, but surgery can produce improve-
ment in both pain and function (Naidoo 1982;
Fowles et al. 1984; Mehta et al. 2007).
Summary
Medial epicondyle fractures, transphyseal distal
humerus fractures, and elbow dislocations repre-
sent a spectrum of pediatric elbow injury. A high
index of suspicion along with a thorough under-
standing of pediatric elbow development and
anatomy is required in order to diagnose these
fractures. All three of these injuries are at risk of
being missed or confused for other injury patterns.
Prompt recognition and treatment ensure a good
outcome for patients who sustain these injuries.
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 Radial Head, Radial Neck, and
Olecranon Fractures 54
Brandon S. Schwartz, Joshua M. Abzug, Charles Chan, and
Joshua E. Hyman

Contents Operative Treatment of Radial Head and Neck

Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1208
Introduction to Radial Head Surgical Procedure . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1208
and Neck Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1204 Surgical Approaches . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1209
Pathoanatomy and Applied Anatomy Preferred Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1214
Related to Radial Head and Neck Fractures . . . . 1204 Surgical Pitfalls and Prevention . . . . . . . . . . . . . . . . . . . . 1214
Assessment of Radial Head and Management of Complications . . . . . . . . . . . . . . . . . . . . . 1215
Neck Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1204 Introduction to Olecranon Fractures . . . . . . . . . . . . 1216
Signs and Symptoms of Radial Head and Neck
Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1204 Pathoanatomy and Applied Anatomy Related
Radial Head and Neck Imaging and Other to Olecranon Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . 1216
Diagnostic Studies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1205
Assessment of Olecranon Fractures . . . . . . . . . . . . . 1216
Injuries Associated with Radial Head and Neck
Signs and Symptoms of Olecranon Fractures . . . . . . 1216
Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1206
Olecranon Fracture Imaging and Other
Radial Head and Neck Fracture Classication . . . . . 1206
Diagnostic Studies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1217
Radial Head and Neck Fracture Outcome Tools . . . 1207
Injuries Associated with Olecranon Fractures . . . . . 1217
Radial Head and Neck Fracture Treatment Olecranon Fracture Classication . . . . . . . . . . . . . . . . . . 1217
Options . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1207 Olecranon Fracture Outcome Tools . . . . . . . . . . . . . . . . 1218
Nonoperative Treatment of Radial Head and Neck
Olecranon Fracture Treatment Options . . . . . . . . . 1218
Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1207
Nonoperative Treatment of Olecranon
Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1218
Operative Treatment of Olecranon Fractures . . . . . . 1219
Surgical Procedure . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1219
Surgical Approaches . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1219
B.S. Schwartz (*) J.M. Abzug
Preferred Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1223
University of Maryland School of Medicine, Baltimore,
Surgical Pitfalls and Prevention . . . . . . . . . . . . . . . . . . . . 1223
MD, USA
Management of Complications . . . . . . . . . . . . . . . . . . . . . 1224
e-mail: brandon.schwartz@som.umaryland.edu;
jabzug@umoa.umm.edu Summary and Future Research . . . . . . . . . . . . . . . . . . 1224
C. Chan References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1224
Department of Orthopaedic Surgery, Columbia University,
New York, NY, USA
e-mail: charles530@gmail.com
J.E. Hyman
Department of Orthopaedic Surgery, Columbia University,
College of Physicians and Surgeons, New York, NY, USA
e-mail: jh736@columbia.edu; jh736@mail.cumc.
columbia.edu

# Springer Science+Business Media New York 2015 1203

J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_55
1204 B.S. Schwartz et al.

landing onto an outstretched hand such as what

Abstract
occurs from a fall off the monkey bars (Rodriguez
Radial head and neck fractures, as well as olec-
Merchan 1994; Tan and Mahadev 2011). This
ranon fractures, are often seen in the pediatric
mechanism results in a fracture via transference
and adolescent populations. This chapter exam-
of valgus stress through the cartilaginous radial
ines the epidemiology, pathoanatomy, assess-
head to the radial neck. A second mechanism
ment, diagnosis, and treatment options of radial
involves impaction of the capitellum onto the
head and neck fractures as well as olecranon
radial head during relocation of a dislocated
fractures. Neither nonoperative nor surgical out-
elbow. Fractures of the radial neck may also
comes of these injuries in children have been
occur in conjunction with a posterior elbow dis-
extensively studied. Data from the limited num-
location. In these cases, the proximal radius
ber of pediatric studies, as well as data extrapo-
epiphysis is pushed posteriorly and sustains a
lated from the adult literature, will be discussed.
fracture due to impact from the capitellum as the
The nonoperative and operative management
elbow is reduced. Alternatively, injuries to the
options are presented from technique to out-
proximal radius can also present as stress injuries
come, along with their associated complications.
due to longitudinal and rotational forces exerted.
These injuries typically occur as a result of high-
performance athletic activity such as what is seen
Introduction to Radial Head and Neck
in the throwing/pitching motion of baseball
Fractures
players. Repetitive stress can disrupt growth and
eventually lead to deformity. Lytic lesions of the
Radial neck fractures account for approximately
radial head, such as those seen with
1 % of all fractures in children and between 5 %
osteochondritis dissecans, can also arise from
and 10 % of pediatric elbow fractures (Chambers
repetitive stress.
2001). The majority of proximal radius fractures in
children are radial neck fractures in contrast to radial
head fractures due to the physis being the weakest
Assessment of Radial Head and Neck
biomechanical facet of this region and the sizable
Fractures
cartilaginous nature of the radial head. However, in
older children and adolescents with closing physes
Signs and Symptoms of Radial Head
and signicant ossication, fractures of the radial
and Neck Fractures
head can occur (Landin and Danielsson 1986).
The majority of radial neck fractures occur in
The clinical presentation of a radial neck fracture
children 912 years of age (Chambers 2001; Green
in a child may vary depending on the severity of
and Zeeland 2008); however fractures can occur in
the precipitating trauma. Children who fall onto
the very young as well as in adolescents (Steinberg
an outstretched hand will experience pain local-
et al. 1988; Tan and Mahadev 2011). There is no
ized to the elbow region without any visually
signicant difference in incidence between boys and
apparent deformity or signicant swelling of the
girls, although fractures in girls tend to occur
joint. Patients who experience a posterior elbow
approximately 2 years earlier (Steinberg et al. 1988).
dislocation may exhibit a deformed elbow. How-
ever, in some cases, no deformity will be seen
Pathoanatomy and Applied Anatomy because the elbow dislocation has spontaneously
Related to Radial Head and Neck reduced.
Fractures A careful physical examination noting tender-
ness to palpation about the radial head and neck
The typical mechanism responsible for injury is a region will provide the physician with the correct
valgus force onto an extended outstretched arm. diagnosis. If the elbow is brought through a pas-
The most common mechanism of injury is a child sive range of motion, the child will experience
54 Radial Head, Radial Neck, and Olecranon Fractures
Fig. 1 Anteroposterior (a) and lateral (b) radiographs of the elbow demonstrating a displaced radial neck fracture
(Courtesy of Joshua M. Abzug, MD)
pain with passive exion and extension. Typi-
cally, however, patients experience the most sig-
nicant discomfort with forearm rotation
(Chambers 2001; Hart et al. 2011). It is important
to remember that it is not uncommon for radial
neck fractures to occur in conjunction with other
fractures about the elbow region due to similar
mechanisms of injury. For example, children may
sustain a concomitant medial epicondyle or olec-
ranon fracture (Monteggia variant). Therefore, it
is imperative to be systematic with the physical
examination, palpating the distal humerus as well
as the olecranon and the entire forearm.
While neurovascular injuries are uncommonly
associated with radial neck fractures, a thorough
sensorimotor and neurovascular exam is neces-
sary to identify decits, especially if the history
suggests that an elbow dislocation has occurred.
In acute injuries, there is often loss of rotation and
pain with pronation and supination. The exam can
be limited due to pain or swelling. Assessment of
the posterior interosseous nerve (PIN) is vital as it
can be injured due to its proximity to the radial
neck. The soft tissues of the elbow and forearm
must also be examined for swelling, lacerations,
and abrasions.
1205
Radial Head and Neck Imaging
and Other Diagnostic Studies
A suspected radial neck fracture is best evaluated
with AP and lateral views of the elbow and fore-
arm (Fig. 1). Most fractures involve the physis or
occur in the metaphysis just distal to the physis.
The normal apex medial angulation (or lateral tilt)
of the proximal radius on an AP radiograph mea-
sures 1015 , and there is 5 of angulation (apex
inferior) seen on the lateral radiograph. Fracture
displacement is calculated by determining the
degree of angulation, measured on the AP view,
and the amount of proximal fragment translation
relative to the distal metaphysis and shaft of the
radius.
Minimally displaced radial neck fractures may
be difcult to identify on plain radiographs
because the fracture line may be superimposed
over the ulna. Additionally, in those patients in
whom ossication of the epiphysis has not yet
occurred, it may be difcult to determine the
amount of displacement of the radial head. The
only clue to a signicantly displaced fracture in a
young child may be a small eck of bone about
the proximal radius or deformation of the
1206
proximal metaphysis because the unossied radial
head is not visualized.
Identication of a posterior fat pad sign on the
lateral radiograph is another important but subtle
indicator that a fracture is present. While the pres-
ence of this sign is most commonly associated
with a non-displaced supracondylar fracture, it
may also indicate the presence of a radial neck
fracture in a child, as the physis is intra-articular
and therefore fractures in this region will result in
the presence of an elbow effusion (Skaggs and
Mirzayan 1999). Additionally, the presence of
the supinator fat pad, a thin layer of fat that lies
on top of the supinator muscle in the proximal
forearm that can often be seen on plain radiogra-
phy, should be assessed. It has been hypothesized
that displacement of this fat pad can indicate that
there is injury to the proximal radius (Chambers
2001). However, this is not always a reliable sign
of fracture, and absence of fat pad displacement
may be more reliable as an indication of the
absence of a radial neck fracture (Irshad
et al. 1997).
Magnetic resonance imaging is the best
advanced imaging modality for evaluating proxi-
mal radius injuries in children with incomplete
radial head ossication. However, because this
test generally requires sedation or general anes-
thesia to complete, its utility is limited. If the
examination and radiographs are suspicious for a
displaced fracture, it may be preferable instead to
examine the elbow while the child is under anes-
thesia. An elbow arthrogram coupled with live
uoroscopy allows a complete assessment of the
fracture type and degree of displacement. Reduc-
tion can then be performed, taking advantage of
the arthrogram to assess the success of realign-
ment maneuvers.
Injuries Associated with Radial Head
and Neck Fractures
Fractures of the radial neck may occur with a
concomitant posterior elbow dislocation. Patients
may also sustain an associated medial epicondyle
or olecranon fracture (Monteggia variant).
Neurovascular injury is uncommon; however,
B.S. Schwartz et al.
the posterior interosseous nerve is within close
proximity to the fracture site and may become
damaged.
Radial Head and Neck Fracture
Classification
The most commonly utilized classication
scheme for pediatric radial neck fractures is the
Salter-Harris (SH) system. SH I (SH) fractures
traverse the physis. SH II fractures traverse part
of the physis and exit out the metaphysis and are
the most common fracture pattern seen in
displaced pediatric radial neck fractures. SH III
fractures involve the physis and epiphysis, and SH
IV fractures involve the epiphysis, physis, and
metaphysis. Type III and IV fractures are rela-
tively uncommon and are mostly seen in older
children and adolescents (Salter and Harris 1963).
Wilkins classied pediatric radial neck frac-
tures into two types, each with additional subtypes.
Type I injuries are valgus fractures; subtype A
injuries are SH I and SH II fractures, subtype B
are SH IV fractures, and subtype C are isolated
fractures of the radial metaphysis. Type II injuries
are radial neck fractures associated with posterior
elbow dislocations; subtype D describes proximal
radius fractures that occur from reduction of the
elbow dislocation and subtype E fractures are
those proximal radius fractures that occur concom-
itantly with elbow dislocations (Wilkins 1991).
A classication scheme has also been proposed
by Judet and colleagues. Type I fractures have
minimal or no displacement, Type II fractures
have lateral displacement of less than the shaft
diameter and angulation of less than 30 , Type III
fractures have contact between the fracture frag-
ments and angulation between 30 and 60 , and
Type IV fractures have complete loss of contact
between the bony fragments and angulation of
more than 60 (Judet et al. 1962).
The OBrien classication is based on the
degree of angulation and recommends treatment
based on these parameters. Type 1 fractures are
angulated less than 30 and require immobiliza-
tion. Type 2 fractures are angulated 3060 and
can be treated with closed reduction. Type
54 Radial Head, Radial Neck, and Olecranon Fractures
3 fractures are angulated greater than 60 and
require open reduction (OBrien 1965).
Radial neck fractures with associated proximal
ulna or olecranon fractures are considered
Monteggia equivalents and classied according
to the Bado classication.
Radial Head and Neck Fracture
Outcome Tools
No specic outcome measures exist to assess
pediatric radial head and neck fractures. The
most commonly utilized outcome tool for all pedi-
atric upper extremity fractures is the DASH score.
Radial Head and Neck Fracture
Treatment Options
Nonoperative Treatment of Radial
Head and Neck Fractures
Indications/Contraindications
The majority of pediatric radial neck fractures can
be managed without operative intervention
(Table 1). Minimally to moderately displaced
proximal radius fractures can often be treated
conservatively due to the potential for
remodeling. In children younger than 68 years
of age, up to 30 of angulation and less than 50 %
translation is acceptable because predictable
remodeling occurs in this age group (Chambers
2001). Younger children with more severe angu-
lation and older children and adolescents with
greater than 1015 of angulation, however, are
best treated with fracture reduction.
Techniques
Minimally displaced Salter-Harris I and II frac-
tures and metaphyseal fractures, regardless of the
age of the child, can be treated in a long arm cast
with the elbow exed to 90 for 3-week duration.
After cast removal, active elbow range of motion
is initiated. Most children return to full activities
within 36 weeks after cast removal.
Closed reduction can be achieved using one of
several described techniques. One method
1207
Table 1 Radial head and neck fractures: Nonoperative
management
Indications Contraindications
Non-displaced or minimally Radial neck fracture
displaced fractures angulation >30
Radial neck fracture Signicantly
angulation <30 with displaced fractures
translation <50 % in any plane (>50 % translation)
involves exing the elbow to 90 and fully supi-
nating the forearm. Next, pressure is applied to the
shaft of the proximal radius in order to reduce the
head. Applying the pressure to the shaft as
opposed to the head utilizes a long lever arm,
achieving a greater mechanical advantage
(Monson et al. 2009).
Other commonly used techniques are
two-person maneuvers. One such technique
involves rotating the forearm under uoroscopy
to identify the position in which the maximum
degree of angulation is visible on the AP view of
the elbow (Fig. 2). Next, longitudinal traction and
varus stress is applied to the elbow by an assistant
while the surgeon pushes downward and laterally
on the radial shaft in the interosseous space
(Neher and Torch 2003).
Another technique fully supinates the forearm
rst. Longitudinal traction and a varus force is
then applied across the elbow, while a medially
directed force is applied via thumb pressure to the
skin overlying the radial head (Patterson 1934).
One nal closed reduction technique involves
wrapping an Esmarch bandage from distal to
proximal in an attempt to realign the proximal
radial fragment over the radial shaft.
Following the chosen maneuver, repeat radio-
graphs or uoroscopic images are obtained to
assess the reduction. Fractures that are not ade-
quately reduced are then taken to the operating
room for further manipulation. Once an accept-
able reduction is obtained, the patient is placed in
a long arm cast at 90 of elbow exion for
3 weeks.
Outcomes
Both Vocke and Berntstein demonstrated good
results with conservative management of radial
1208
Fig. 2 Fluoroscopic view of the elbow demonstrating the
maximal amount of angulation of the radial neck fracture
(Courtesy of Joshua M. Abzug, MD)
neck fractures angulated up to 5060 in children
aged under 6 and under 10, respectively (Bern-
stein et al. 1993; Vocke and Von Laer 1998).
However, there is a lack of research to support
an exact age cutoff as it relates to an acceptable
degree of angulation and predicted outcomes.
Operative Treatment of Radial Head
and Neck Fractures
Indications/Contraindications
Operative intervention should be performed for all
pediatric radial neck fractures that are signi-
cantly angulated (greater than 30 ) despite failed
closed reduction maneuvers and all fractures that
have signicant displacement (greater than 50 %
translation). Additionally, fractures with small
ecks of bone in young children should be
brought to the operating room to have an
arthrogram performed to ensure adequate align-
ment is present. Should closed reduction attempts
B.S. Schwartz et al.
fail to achieve an acceptable reduction, percuta-
neous or open techniques should be employed.
Advancing directly to an open reduction is some-
times necessary when signicant displacement of
the fracture is present, as there can be soft-tissue
interposition or the radial head fragment can be
extracapsular. However, open techniques are
associated with worse outcomes, and therefore, a
closed or percutaneous technique should be
attempted prior to proceeding to open reduction
when possible.
Surgical Procedure
Preoperative Planning
A systematic approach in the operating room is
utilized to reduce the fracture in the most mini-
mally invasive manner.
The algorithm begins by performing an elbow
arthrogram in children with minimal or no
ossication of the proximal radial epiphysis,
typically children 5 years of age and younger.
Subsequently, closed reduction maneuvers are
attempted with the patient under general anesthe-
sia. If a closed reduction maneuver reduces the
fragment, stability is checked with live uoros-
copy, and if the fragment is stable, a long arm
cast is applied. Due to the thick periosteum
and typical SH II fracture pattern, there is
almost always continuity of the periosteum,
which aids in maintaining the reduction once it
is achieved.
If closed reduction maneuvers fail, the arm is
prepped and draped in the usual fashion and
reduction of the fracture utilizing a percutaneous
pin reduction is attempted. Successful reduction
of the fragment allows for treatment with casting
alone.
If percutaneous pin reduction maneuvers are
unsuccessful, the Metaizeau technique is
performed, as long as the fracture is angulated
60 or less (Judet Type III). Fractures that have
no bony contact between the fragments and/or are
angulated more than 60 are difcult to treat with
the Metaizeau technique and require open reduc-
tion (Endele et al. 2010). Furthermore, manipula-
tive reduction attempts for fractures angulated
54 Radial Head, Radial Neck, and Olecranon Fractures
Table 2 Reduction of proximal radius fractures:
Preoperative planning
OR table: standard OR table
Position/positioning aids: supine with hand table
extension or the elbow can be placed directly on the
inverted uoroscopy machine
Fluoroscopy location: ipsilateral side
Equipment: K-wires, exible nails
Tourniquet (sterile/nonsterile): nonsterile pneumatic
tourniquet to upper arm
Draping: extremity or split drapes
more than 60 have the potential to damage the
cartilaginous epiphysis (OBrien 1965).
The nal step in the algorithm is to perform an
open reduction and utilize K-wire xation. As
outcomes are less optimal and there is a higher
complication rate following open reduction, this
technique is reserved until after all other means of
obtaining a successful reduction have been
attempted (Dsouza et al. 1993; Schmittenbecher
et al. 2005).
Positioning
Regardless of the procedure that is being exe-
cuted, positioning is the same (Table 2). The
patient is placed supine on the operating room
table with a hand table that permits uoroscopic
evaluation of the elbow and forearm. Alterna-
tively, the elbow can be placed directly onto the
inverted uoroscopy unit. A nonsterile pneumatic
tourniquet is applied to the upper arm, and the
remainder of the upper extremity is prepped and
draped in the usual fashion. These steps permit the
progression through the aforementioned algo-
rithm if necessary without having to spend time
re-prepping or draping.
Surgical Approaches
It is important for the surgeon to have knowledge
regarding the course of the posterior interosseous
nerve (PIN) when operating on the proximal
radius. The PIN is the deep motor branch of the
radial nerve that innervates the vast majority of
musculature of the dorsal side of the forearm.
1209
After branching from the radial nerve proper in
the region of the lateral epicondyle, it enters the
forearm by splitting the supinator muscle. The
PIN then passes near the dorsal aspect of the
radius opposite the bicipital tuberosity. It has
been shown to make contact with the proximal
radius in up to 25 % of patients (Calfee
et al. 2011).
The surgical approach for open reduction is the
classic Kocher approach, a Kaplan approach
which permits more anterior exposure, or a Boyd
approach which may decrease the risk of PIN
injury but is not as direct. The Kocher approach
utilizes the interval between the anconeus muscle,
innervated by the radial nerve proper, and the
extensor carpi ulnaris muscle, innervated by the
posterior interosseous nerve. In the Kaplan
approach, the extensor digitorum communis mus-
cle is split, providing a more anterior exposure,
which, for this fracture, may be helpful depending
on where the displacement is. It is imperative to
pronate the forearm throughout either approach as
this has been shown to move the PIN away from
the bone.
The Boyd approach utilizes an incision that
begins approximately 2.5 cm proximal to the
elbow joint, lateral to the triceps tendon, and con-
tinues distally over the lateral side of the tip of the
olecranon, ending at the junction of the proximal
and middle thirds of the ulna. A plane is then
created between the ulna medially and the
anconeus and extensor carpi ulnaris laterally.
The surgeon then strips the anconeus from the
bone subperiosteally in the proximal part of the
incision and reects it in a radial direction, thus
exposing the radial head.
Technique
An elbow arthrogram is typically performed prior
to reduction (Fig. 3). A small-gauge needle
attached to extension tubing is inserted into the
elbow joint, either through the soft spot on the
posterolateral aspect of the elbow, dened by the
lateral distal humerus and the radial head, or pos-
teriorly through the triceps into the olecranon fossa.
After uoroscopic conrmation, a 10 cc syringe
lled with a solution of equal parts saline and
contrast dye (Dormans 1994) is attached to the
1210
Fig. 3 Elbow arthrogram performed on a 3-year-old
female following a fall down the stairs. (a) Anteroposterior
view before reduction. (b) Anteroposterior view following
tubing. Under live uoroscopy, the dilute contrast
material is then injected. For a small child, only
35 cc of contrast is needed. The elbow is then
taken through a range of motion to distribute the
contrast material throughout the joint, and images
are taken. Visualization of the entire radial head is
now possible, and one can determine exactly how
angulated and displaced the fracture fragment is.
When closed reduction attempts fail to achieve
an acceptable reduction, percutaneous or open
techniques should be employed. Percutaneous
pin reduction can be performed following the
elbow arthrogram, once the radial head is easily
visualized (Table 3). A 0.062 in. or 5/64 in.
K-wire is percutaneously inserted from the ulnar
side of the radius to minimize potential injury to
the PIN (Canale 2007). Once the sharp side of the
B.S. Schwartz et al.
reduction. (c) Lateral view following reduction (Courtesy
of Joshua M. Abzug, MD)
wire punctures the skin, the wire is removed and
turned around such that the blunt end of the wire is
now inserted through the puncture hole. With the
aid of uoroscopy, the blunt end is now advanced
to make contact with fracture fragment. Subse-
quently, the K-wire is used to push the radial
head into anatomic alignment (Fig. 4). As some
healing may have already occurred, it may be
helpful to gently tap the K-wire with a mallet to
facilitate movement of the fragment. This should
be performed while utilizing uoroscopy liber-
ally, as the K-wire may not be advancing the
fragment but may rather be advancing into the
cartilaginous fragment. It is important to try and
avoid entering the fracture site with the K-wire to
avoid potential injury to the physis. Occasionally,
two wires may be utilized to reduce the fragment:
54 Radial Head, Radial Neck, and Olecranon Fractures 1211

Table 3 Percutaneous pin reduction of proximal radius

fractures: Surgical steps
Following elbow arthrogram, insert a 0.062 in. or 5/64
in. K-wire percutaneously from the ulnar side of the
radius
Remove wire following skin puncture and insert blunt
end of wire into puncture site
Under uoroscopic guidance, advance blunt end of wire
to make contact with fracture fragment, while avoiding
entering the fracture site
Push radial head into anatomic alignment. Light taps with
a mallet may be necessary

one to push the fragment up, thus improving the

angulation, and one to push the fragment over,
thus improving the translation.
The Wallace Joker technique is an alternate
method of reduction. First, the level of the bicip-
ital tuberosity is identied under uoroscopy. A
dorsal skin incision is then made at the same level
lateral to the border of the ulna. Next, a joker is
introduced between the radius and ulna, with care
taken to avoid periosteal disruption. Using the
joker, lateral pressure is then applied along the
medial surface of the radius as counterpressure is
applied to the proximal radial head fragment in
order to achieve reduction.
If the fragment is angulated approximately 60
or less, but alignment remains unacceptable after
percutaneous wire manipulation, the Metaizeau
technique should be performed (Table 4). The
Metaizeau technique utilizes an intramedullary
K-wire or exible titanium nail as a tool to facil-
itate reduction of the radial neck fracture fragment
(Metaizeau et al. 1980) (Fig. 5). In either case, the
last 1.5 cm should be bent approximately 2030
prior to insertion. The intramedullary device is
inserted in a retrograde fashion utilizing either a
dorsal or radial starting point. The limb should be
exsanguinated and the tourniquet inated. A dor-
sal incision approximately 23 cm in length is
made in the region of Listers tubercle, ensuring
that this location is proximal to the physis by 12
cm. Soft-tissue dissection is carried out to identify
the extensor pollicis longus (EPL) tendon in the
third dorsal extensor compartment. Once it is
identied, the compartment is released, and the
tendon is transposed and protected to reduce the
Fig. 4 Percutaneous reduction of a radial neck fracture
utilizing the blunt end of a K-wire to engage and realign the
displaced fragment (Courtesy of Joshua M. Abzug, MD)
Table 4 Metaizeau technique: Surgical steps
Exsanguinate limb and inate tourniquet
Make dorsal incision in region of Listers tubercle,
approximately 23 cm in length, after ensuring that the
physis is distal to this location
Soft-tissue dissection to identify EPL tendon in third
dorsal extensor compartment
Release compartment. Transpose and protect EPL tendon
Make oblong hole in dorsal cortex of radius
Bend tip of intramedullary device 2030
Place intramedullary device through the radius in a
retrograde fashion
Advance tip past fracture site into the proximal radial
head fragment
Rotate intramedullary device 180 , reducing the fracture
fragment
Obtain multiple uoroscopic images to ensure
appropriate fragment movement and subsequent
reduction
Cut intramedullary device just above dorsal radial cortex
Deate tourniquet, obtain hemostasis, and close incision
Place long arm cast and obtain nal images
risk of injury during nail insertion and while the
nail remains in place during fracture healing.
Under direct visualization, with the EPL
protected, an oblong hole is then made in the
dorsal cortex of the radius. The intramedullary
1212
Fig. 5 Reduction of a radial neck fracture utilizing an
intramedullary device (Metaizeau technique). (a)
Anteroposterior radiograph demonstrating a displaced
radial neck fracture. (b) Insertion of the intramedullary
exible nail utilizing a radial-sided entry point. (c)
Advancement of the exible nail to engage the fracture
B.S. Schwartz et al.
fragment. Note the utilization of an arthrogram to aid in the
visualization of the radial head. (d) Rotation of the
intramedullary nail 180 to reduce the fragment. (e)
Anteroposterior view following reduction. (f) Lateral
view following reduction (Courtesy of Joshua M. Abzug,
MD)
54 Radial Head, Radial Neck, and Olecranon Fractures
Table 5 Open reduction of distal radius fractures:
Surgical steps
With the forearm pronated, perform standard Kocher
approach by making posterolateral incision from the
lateral condyle extending obliquely to end approximately
5 cm distal to the tip of the olecranon
Identify and explore plane between ECU and anconeus
Retract anconeus ulnarly and ECU radially
Identify supinator muscle and visualize joint capsule
Incise joint capsule and obtain visualization of fracture
Reduce fracture under direct visualization
Stabilize reduction with a minimum of two K-wires
placed obliquely through the fracture site and protruding
through the skin
Utilize uoroscopy to ensure adequate stability
Deate tourniquet, obtain hemostasis, and close incision
Place long arm cast with elbow exed to 90 and forearm
in neutral rotation
device is inserted and passed retrograde in the
radius up to the fracture site. Under uoroscopic
guidance, the bent tip is now advanced across the
fracture site to engage the fracture fragment.
Repeating the arthrogram at this point may facil-
itate visualization of the fracture fragment and the
reduction. Once the tip of the intramedullary
device has engaged the fracture fragment, the
intramedullary device is rotated 180 , thus reduc-
ing the fracture fragment. Multiple uoroscopic
images are obtained during this process to ensure
that the fragment is moving appropriately. After
the fragment is reduced into acceptable alignment,
the intramedullary device is cut just above the
dorsal radial cortex and the tourniquet is deated.
Hemostasis is obtained and the dorsal incision is
closed. The child is then placed in a long arm cast,
and nal images are obtained to ensure mainte-
nance of reduction.
As noted earlier, open reduction should only be
used when all other attempts to reduce the fracture
have failed (Table 5). A standard Kocher approach
is utilized by making a posterolateral incision
from the lateral condyle and extending obliquely
in a distal medial direction to end approximately
5 cm distal to the tip of the olecranon. The plane
between the extensor carpi ulnaris and the
anconeus muscles is then identied and explored.
With the forearm held in a pronated position, the
1213
anconeus is retracted ulnarly and the extensor
carpi ulnaris is retracted radially. The bers of
the supinator muscle can be identied crossing at
a right angle to the wound. Its proximal bers
should be gently retracted distally to avoid poten-
tial injury to the posterior interosseous nerve. The
joint capsule is now visualized in the depth of the
proximal aspect of the wound. The capsule is then
incised to obtain adequate visualization of the
fracture, and the fracture is now able to be reduced
under direct visualization. Stabilization is typi-
cally performed with a minimum of two K-wires
placed obliquely through the fracture site and left
protruding through the skin for easy removal.
Transcapitellar pins should be avoided as they
make break during the healing period, even if
the child is in a long arm cast.
Once the fracture is stabilized, live uoroscopy
is utilized to ensure adequate stability is present,
and if so, the tourniquet is deated, hemostasis
obtained, and the incision closed.
Following each of these techniques, the child is
placed in a long arm cast with the elbow exed to
90 and the forearm in neutral rotation. Additional
AP and lateral radiographs are obtained at 1 week
postoperatively to ensure maintenance of reduc-
tion. At 3 weeks postoperatively, the cast is
removed, and additional radiographs are obtained.
If there is evidence of fracture healing, the
K-wires are removed, and the child can progress
to active range of motion of the elbow. The patient
is seen 23 weeks later for a range of motion
check. If there has not been signicant improve-
ment, then formal occupational therapy is insti-
tuted. At approximately 3 months postoperatively,
the intramedullary device is removed in the oper-
ating room if one was placed.
Treatment-Specific Outcomes
The prognosis for the injury and treatment out-
come is based on the severity of injury, the age of
the patient, the type of treatment received, and the
presence of any associated injuries. Younger chil-
dren have a greater remodeling potential and
therefore greater degrees of fracture angulation,
and displacement can be accepted with limited
residual decits on functional range of motion.
1214
The need for an open reduction is associated with
poor outcome. In a large series of displaced radial
neck fractures requiring open reduction, the inci-
dence of poor outcomes was as high as 50 % in
severely displaced fractures (Zimmerman
et al. 2013). This is reective of the generally
accepted notion that the magnitude of the injury
force and thus severity of injury are the most
important factors in predicting long-term
outcome.
Good to excellent results can be expected from
closed treatment and treatment methods that do
not require open reduction and internal xation
(Brandao et al. 2010; DSouza et al. 1993; Endele
et al. 2010). Metaizeau reduction has been
reported to be successful at obtaining reduction
in all Judet Type II and III fractures as well as
some Judet Type IV fractures with up to 98 % of
patients having good or excellent results (Brandao
et al. 2010; Endele et al. 2010).
DSouza and colleagues reviewed 100 patients
treated with either immobilization alone, closed
reduction maneuvers, or open reduction. All
patients in the immobilization group and all but
one patient in the closed reduction group had a
good or excellent result compared to only approx-
imately half of the patients in the open reduction
group. Furthermore, two thirds of the patients in
the open reduction group had pain at nal follow-
up compared to approximately 15 % in the immo-
bilization and closed reduction groups. Forearm
rotation was signicantly limited in more than
30 % of patients in the open reduction group
whereas this occurred in only about 10 % of
patients in the other two groups (DSouza
et al. 1993).
Preferred Treatment
Multiple radiographic images should be obtained
to assess the maximal severity of fracture angula-
tion and displacement prior to determining the
method of optimal treatment. When possible, it
is preferable to obtain reductions by closed
methods under sedation in the operating room.
The arm is fully prepped and draped so that the
B.S. Schwartz et al.
surgeon may progress to percutaneous techniques
and nally an open reduction if needed. If the
reduction is achieved percutaneously, stability is
assessed as often no xation is typically required.
The arm is placed into a long arm cast in 90 of
elbow exion and neutral rotation. Radiographic
studies are obtained to ensure maintenance of
reduction at 1 week and 3 weeks from the start
of immobilization. Open reduction is performed
as a last resort utilizing a standard Kocher
approach. Following reduction, two K-wires are
utilized to maintain the reduction. The wires are
left outside the skin and removed at 34 weeks
postoperatively.
Surgical Pitfalls and Prevention
As the highest complication rates are noted with
open reduction techniques, all measures possible
are attempted to avoid performing an open reduc-
tion. Despite these attempts, it is sometimes nec-
essary to proceed to an open reduction in order to
obtain acceptable alignment.
When performing the percutaneous pin reduc-
tion technique, it is important to utilize the blunt
end of the K-wire against the cartilaginous epiph-
ysis to avoid driving the wire into the bone and to
minimize the chance of damaging surrounding
structures such as the physis, articular surface of
the capitellum, and the posterior interosseous
nerve. In addition, utilizing uoroscopy will aid
in identifying the entire radial head to avoid dam-
age to the articular surface and help in determin-
ing adequate reduction. Furthermore, the surgeon
should avoid placing the pin directly into the
physis to minimize the chance of physeal arrest.
Violation of the radial and ulnar periosteum
while performing the Wallace Joker technique
can lead to an iatrogenic synostosis. Care must
be taken to avoid disruption of the periosteum.
Specic complications related to the
Metaizeau technique include irritation of the dor-
sal radial sensory nerve (DRSN) if the device is
inserted from the radial side of the radius, extensor
pollicis longus (EPL) tendon rupture if inserted
from the dorsal aspect of the radius, pin tract
54 Radial Head, Radial Neck, and Olecranon Fractures 1215

infections if the pin is left outside the skin, and Table 6 Radial head and neck fractures: Potential pitfalls
pseudobursa development if the pin is buried and preventions
beneath the skin (Endele et al. 2010). For most Potential pitfall Pearls for prevention
patients, DRSN irritation resolves within 612 Pin tract infections Ensure adequate burial of
weeks following rod removal. EPL tendon rupture Pseudobursa device
is best treated with transfer of the extensor indicis development
Iatrogenic synostosis Avoid disruption of
proprius to the EPL distal stump. Direct repair of
periosteum when utilizing
the tendon is not typically possible since this Joker technique
rupture is attritional and not the result of a lacer- Irritation of DRSN Avoid radial placement of
ation. When performing the Metaizeau technique, intramedullary rod
the dorsal approach should be utilized with trans- EPL tendon rupture Transpose EPL tendon
position of the EPL tendon to minimize the risk of following intramedullary rod
insertion
EPL injury. Placement of the device from the
Injury to posterior Ensure forearm pronation
radial side risks injury to the DRSN, which unfor- interosseous nerve during procedure
tunately does not have a good outcome. Addition- (PIN)
ally, the devices should be buried to minimize the Pin breakage Avoid transcapitellar pins
risk of infection.
When performing an open reduction, the
Kocher approach is utilized while the forearm is Table 7 Radial head and neck fractures: Management of
pronated to minimize the chance of injury to the complications
posterior interosseous nerve. Transcapitellar pins Loss of forearm rotation Occupational
should not be used, as if the pin breaks, the broken therapy
ends can signicantly damage the articular sur- Radial head overgrowth No management
faces and removal is technically difcult. Patients Cubitus valgus deformity No management
who require ORIF typically receive formal occu- Avascular necrosis of radial Radial head
pational therapy to aid in regaining their motion epiphysis resection
once the pins are removed (Table 6).

Radial head overgrowth occurs in approxi-

Management of Complications
Several complications may occur as result of
radial neck fractures and their treatment
(Table 7). Loss of forearm rotation and growth
abnormalities of the proximal radius are the most
common complications. Additional complica-
tions include PIN palsy, radioulnar synostosis,
and elbow crepitus following fracture healing.
Decreased elbow motion, most notably fore-
arm rotation, occurs in a greater number of
patients who undergo open reduction compared
to those treated with immobilization alone or by
other percutaneous reduction techniques. Severe
residual angulation may be treated with a radial
osteotomy, but this procedure is technically
demanding and does not guarantee restoration of
motion.
mately 2040 % of pediatric radial neck fractures
(Chambers 2001) and occurs at signicantly
higher rates in patients who have undergone
open reduction. While the overgrowth may limit
elbow rotation, for many children, it has no sig-
nicant sequelae (DSouza et al. 1993;
Schmittenbecher et al. 2005). Cubitus valgus
deformity may also occur due to premature clo-
sure of the proximal radial physis. This rare com-
plication may cause a cosmetic deformity, but this
is rarely of clinical consequence.
The most devastating and dreaded complication
of proximal radius fractures is avascular necrosis of
the radial epiphysis. While very uncommon, it may
cause stiffness and painful motion about the elbow.
Unfortunately, this complication has no treatment
alternative other than radial head resection for
severely symptomatic cases.
1216
Introduction to Olecranon Fractures
Olecranon fractures in the pediatric population are
relatively uncommon, accounting for approxi-
mately 5 % of all elbow fractures in children
(Evans and Graham 1999). These injuries can
occur as either metaphyseal or apophyseal inju-
ries. Typically, olecranon fractures occur in ado-
lescents and become only minimally displaced
due to the spongy bone and thick adjacent carti-
lage. Adolescents with more mature bone require
a greater force for injury and are therefore more
likely to have displaced fractures. Olecranon frac-
tures seen in younger children are generally either
due to osteogenesis imperfecta, a high-energy
mechanism, or direct trauma to the olecranon.
Children with osteogenesis imperfecta can present
with an olecranon sleeve fracture, where the frac-
ture fragment is primarily cartilaginous in nature.
Pathoanatomy and Applied Anatomy
Related to Olecranon Fractures
Metaphyseal olecranon fractures are more com-
mon than apophyseal injuries and occur as a result
of a fall directly onto the olecranon while the elbow
is exed, a fall onto an extended elbow along with
a varus or valgus force, or a shear injury produced
from a direct posterior force. Conversely, apophy-
seal stress fractures, which may involve a
metaphyseal component as well, most often occur
as an avulsion injury due to recurrent tension forces
across the olecranon. Violent triceps contraction,
typically coupled with a varus or valgus force
across the elbow, can result in avulsion of the
olecranon. If the associated force is valgus, there
may also be a concomitant radial neck fracture.
However, if the accompanying force is varus in
nature, there may be an associated dislocation of
the radial head, a Monteggia variant. These avul-
sion injuries are more commonly seen in high-
performing athletes, such as baseball players, gym-
nasts, and tennis players.
The elbow joint is a complex hinge joint
involving the olecranon of the ulna and trochlea
of the distal humerus. The proximal aspect of the
B.S. Schwartz et al.
ulna is the site of attachment for several important
ligamentous stabilizers, including the ulnar collat-
eral ligament which provides valgus stability as
well as the ulnohumeral ligament, part of the
lateral collateral ligament complex, which pro-
vides varus stability. The olecranon itself prevents
anterior translation of the ulna with respect to the
distal humerus (An et al. 1986). The articular
cartilage of the olecranon may contain a bare
area midway between the olecranon and coronoid
process (Shiba et al. 1988). It is critical to recog-
nize this, as over-compression may result in an
incongruous reduction with subsequent
narrowing of the olecranon fossa.
Knowledge of the path of the ulnar nerve is
essential during the treatment of olecranon frac-
tures. When the nerve is within the cubital tunnel,
it passes just medial to the olecranon. As the nerve
exits the cubital tunnel and enters the exor carpi
ulnaris, it joins the ulnar artery in the volar com-
partment of the forearm. Therefore, the ulnar nerve
and artery can be at risk when K-wires, guide wires,
or screws penetrate the anterior cortex of the ulna.
Assessment of Olecranon Fractures
Signs and Symptoms of Olecranon
Fractures
The diagnosis of an olecranon fracture in a child is
made by having a high suspicion for the injury
based on a focused examination of the childs
elbow. Patients often present with swelling and
tenderness localized over the olecranon. There
may be an overlying abrasion with an associated
palpable defect if the fracture is displaced. As with
any pediatric elbow fracture, it may be difcult for
the patient to localize the precise area where the
pain is occurring. Performing a methodical exam-
ination will allow the clinician to elicit the point of
maximal tenderness at the olecranon. It is critical
to assess the remainder of the elbow as well, as
associated fractures may be present, such as frac-
tures of the radial neck or Monteggia fracture-
dislocation variants. Examination may reveal
loss of active extension of the elbow secondary
to extensor mechanism disruption or pain.
54 Radial Head, Radial Neck, and Olecranon Fractures
Fig. 6 Anteroposterior (a) and lateral (b) radiographs demonstrating an olecranon fracture (Courtesy of Joshua
M. Abzug, MD)
Olecranon Fracture Imaging and Other
Diagnostic Studies
Initial imaging of any pediatric elbow fracture
should consist of an AP and lateral view of the
elbow and forearm (Fig. 6). Oblique views may aid
in providing enhanced visualization of the olecranon
without the overlap of the proximal radius. The
radiocapitellar alignment should always be assessed
when an olecranon fracture has been diagnosed, as
there may be an associated radial neck fracture or
radial head dislocation (Monteggia fracture-
dislocation variant). Computed tomography
(CT) is sometimes indicated to evaluate complex
fracture patterns but is not routinely necessary.
Because the ossication center of the olecranon
apophysis appears around age 10, magnetic reso-
nance imaging (MRI) can also be useful if the diag-
nosis is not clear in the younger pediatric population.
Injuries Associated with Olecranon
Fractures
Patients with an olecranon fracture may have a
concomitant radial neck fracture or a Monteggia
fracture-dislocation variant, in which the radial
head has been dislocated.
1217
Olecranon Fracture Classification
Fractures of the olecranon are divided into sub-
types based on whether the fracture is apophyseal
or metaphyseal. Apophyseal olecranon injuries
are classied by the stage of the disease as it
relates to the natural history of apophyseal stress
fractures. Type 1 apophysitis presents with pain
and tenderness with minimal loss of strength to
elbow extension. Type 2 injuries are incomplete
stress fractures that present with pain and loss of
strength to elbow extension. Type 3 injuries are
complete fractures, and patients will demonstrate
loss of elbow extension. Further classication is
utilized for Type III injuries depending on if they
involve the metaphysis as well. Type 3a variants
are pure apophyseal avulsion injuries, whereas
Type 3b variants will demonstrate a displaced
metaphyseal component on radiographs.
Metaphyseal olecranon injuries can alterna-
tively be classied based on the mechanism of
injury. Group A metaphyseal injuries are exion
injuries occurring due to a fall onto a exed elbow
resulting in a transverse fracture line with exten-
sion into the semilunar notch. Displacement is
dependent on the severity of the force applied and
resultant attempts to mobilize the elbow prior to
treatment. Group B metaphyseal injuries are
1218
extension injuries occurring when the elbow is
hyperextended, locking the olecranon into its
fossa, with a varus or valgus force applied. This
type of fracture presents as a greenstick-type frac-
ture with multiple intra- and extra-articular fracture
lines and can lead to residual varus or valgus insta-
bility, depending on the direction of injury force.
Group C metaphyseal injuries are shear injuries
occurring due to a direct blow to the olecranon
resulting in an anteriorly displaced distal fragment.
Matthews proposed a pediatric olecranon frac-
ture classication system based on four types.
Type I fractures are non-displaced without a con-
comitant injury. Type II fractures are also
non-displaced but are associated with a proximal
radial or supracondylar fracture. Type III fractures
are non-displaced with associated soft tissue or
neurovascular injury. Type IV fractures are
displaced fractures with or without a concomitant
injury (Matthews 1980).
Most often, descriptive terminology is utilized to
dene olecranon fractures. Fractures are typically
described as non-displaced, minimally displaced,
or displaced. The fracture patterns are most com-
monly transverse or short oblique, and most frac-
tures occur in the proximal ulnar metaphysis and are
intra-articular. These fractures are described as
Monteggia variant fracture-dislocations when they
are associated with a proximal radius fracture or
dislocation of the radial head.
Olecranon Fracture Outcome Tools
No specic outcome measures exist to assess
pediatric olecranon fractures. The most com-
monly utilized outcome tool for all pediatric
upper extremity fractures is the DASH score.
Olecranon Fracture Treatment Options
Nonoperative Treatment of Olecranon
Fractures
Indications/Contraindications
The majority of pediatric olecranon fractures,
either apophyseal or metaphyseal, can be treated
conservatively by immobilization in a long arm
B.S. Schwartz et al.
Table 8 Olecranon fractures: Nonoperative management
Indications Contraindications
Non-displaced fractures Displaced fractures (>2
mm)
Minimally displaced Comminuted fractures
fractures (<2 mm) without with shortening or
intra-articular gap or step- angulation
off
cast for 34 weeks, with only 1020 % requiring
surgery (Wilkins 1991). Non-displaced and min-
imally displaced fractures (less than 2 mm) with-
out intra-articular gap or step-off may be treated
nonoperatively. Although there are no clear
guidelines regarding how much displacement is
acceptable, it has been suggested that operative
xation should be performed in fractures with
greater than 2 mm of displacement and for com-
minuted fractures with shortening or angulation
(Table 8).
Techniques
Apophyseal injuries early in the continuum can be
treated with rest, anti-inammatories, and cessa-
tion of the offending activity. Physical therapy and
athletic training can often help maintain strength
in the upper body while resting the injured
extremity as well as improve upon poor
mechanics.
Both non-displaced and minimally displaced
apophyseal and metaphyseal olecranon fractures
can be immobilized in a long arm cast at 7580
of elbow exion in neutral rotation and slight
overcorrection of the initial varus/valgus defor-
mity. Following 3 weeks of immobilization, the
cast is removed and active range of motion is
begun. Casting the elbow in extension may
improve the alignment of some avulsion
fractures, but closed reduction of displaced frac-
tures is generally not successful. The force of the
triceps may cause further displacement of
minimally displaced fractures or displacement
of adequately reduced fractures, even in a cast.
Therefore, close follow-up with weekly
radiographs may be warranted for worrisome
fractures.
54 Radial Head, Radial Neck, and Olecranon Fractures
Outcomes
There is limited data available regarding the treat-
ment of pediatric olecranon fractures. However,
the vast majority of fractures treated conserva-
tively have excellent results with no long-term
sequelae.
Operative Treatment of Olecranon
Fractures
Indications/Contraindications
Operative xation should be undertaken in frac-
tures with greater than 2 mm of displacement and
for comminuted fractures with associated angula-
tion or shortening, especially when associated
with radial head subluxation or dislocation. If
apophyseal nonunions persist despite a period of
conservative management, a single cannulated
screw can be placed across the apophysis to pro-
mote healing. Surgery should also be considered
for metaphyseal exion or extension-type injuries
in which an acceptable fracture alignment cannot
be maintained or if there is an intra-articular
gap/step-off.
Surgical Procedure
Preoperative Planning
It is important to assess the fracture pattern and
have various implant options available. Frac-
tures that appear to be simple transverse patterns
may have comminution that is present and that
was difcult to visualize on the plain radio-
graphs. Therefore, having the ability to convert
to an open procedure and place a plate/screw
construct is essential when treating pediatric
and adolescent olecranon fractures. In shear
injuries, minimally displaced fractures should
rst be treated with closed reduction and immo-
bilization with the elbow extended. If an accept-
able reduction cannot be achieved or if early
motion is desired, percutaneous pinning or x-
ation with a screw placed perpendicular to the
fracture line can be considered. Signicantly
displaced fractures are treated with a tension
band technique.
1219
Table 9 Reduction of olecranon fractures: Preoperative
planning
OR table: standard OR table
Position/positioning aids: supine with elbow exed over
the chest. Alternatively, one can utilize lateral decubitus
position with an arm holder
Fluoroscopy location: ipsilateral side
Equipment: 4.5 mm cannulated screws, plate screw
constructs capable of being appropriately contoured,
K-wires, and wire to make a tension band
Tourniquet (sterile/nonsterile): nonsterile pneumatic
tourniquet
Draping: extremity drape or split drapes
Positioning
Patients are positioned supine on the operating
room table, and the entire arm is prepped and
draped in the usual fashion after placement of a
nonsterile pneumatic tourniquet (Table 9). The
procedure is performed with the arm draped across
the patients chest and the uoroscopy unit next to
the operating room table such that AP views can be
obtained by bringing the arm into abduction and
the elbow into extension and lateral views obtained
by abducting and externally rotating the shoulder.
Alternatively, the patient can be placed in the lat-
eral decubitus position and an arm holder utilized.
Typically, with this positioning, mini-uoroscopy
is utilized such that the machine can be rotated
around the arm while it is in the arm holder.
Surgical Approaches
A direct posterior approach to the olecranon is
utilized. Sharp dissection is carried down to create
full thickness skin aps. The periosteum is incised
to expose the fracture.
Technique
Several techniques are available when surgical
intervention is necessary to provide adequate
reduction of olecranon fractures. Simple trans-
verse and non-comminuted oblique fractures can
be treated with either tension band techniques or
percutaneous screw xation, while comminuted
fractures are best treated with ORIF utilizing a
plate and screw construct.
1220
Fig. 7 Intraoperative uoroscopic views of an olecranon
fracture reduction utilizing a bone reduction clamp for
compression. In this case a longitudinal fracture line was
Percutaneous screw placement is performed
utilizing a stab incision made along the posterior
aspect of the elbow through the skin and triceps
tendon. Blunt dissection is then carried out down
to bone, and the guide wire for the 4.5 mm can-
nulated screw system is introduced from the pos-
terior aspect of the olecranon in an antegrade
fashion. Reduction can then be achieved by uti-
lizing the guide wire as a joystick, by direct com-
pression with the surgeons nger, by extension of
the elbow, or any combination of these tech-
niques. A second stab incision can be made, if
necessary, and a bone reduction clamp can be
placed on the distal fragment to aid in achieving
compression (Fig. 7).
Once the guide wire crosses the fracture site, it
is driven out the distal fragment, exiting through
the anterolateral cortex of the ulna, thus staying
away from the course of the ulnar nerve and
artery. The depth gauge is utilized and the appro-
priate length 4.5 mm partially threaded cannulated
screw is placed in a bicortical fashion (Fig. 8).
Compression is obtained by the bicortical nature
of the screw as well as the partially threaded
component. Subsequently, the incision is closed
and the patient is placed into a long arm cast with
the elbow exed to 80 .
Adding tension band wiring to axial screw x-
ation has been proven to signicantly improve the
B.S. Schwartz et al.
present. (a) Anteroposterior view. (b) Lateral view (Cour-
tesy of Joshua M. Abzug, MD)
stability of the construct. However, the wire is often
prominent and requires a subsequent surgery for
removal in up to 80 % of patients (Richards et al.
2012). The tension band technique is performed
under tourniquet control. A posterior approach to
the olecranon is performed by utilizing an incision
that begins approximately 5 cm proximal to the tip
of the olecranon, curves laterally around the olec-
ranon, and ends approximately 5 cm distal to the
olecranon. Sharp dissection is carried out directly
onto the posterior aspect of the ulna until the frac-
ture site is visualized and exposed. Following
removal of the fracture hematoma and incising of
the adjacent periosteum, the fracture is reduced
with utilization of a reduction clamp. A small
unicortical drill hole may be required to anchor
the clamp in the distal fragment.
The tension band construct is created with
either heavy non-absorbable suture, which
decreases the chance of hardware irritation, or a
K-wire construct with steel wire. The goal of this
method is conversion of the extension forces from
the triceps to compressive forces across the artic-
ular surface. A drill hole is made transversely in
the distal fragment, approximately 23 cm distal
to the fracture line. Utilizing either a suture passer
or an angiocatheter, an 18-gauge steel wire is
placed through the transverse hole. Next, two
0.062 in. K-wires are inserted from the posterior
54 Radial Head, Radial Neck, and Olecranon Fractures
Fig. 8 Intraoperative uoroscopic views demonstrating a
percutaneous screw reduction of a displaced olecranon
fracture. (a) Lateral uoroscopic image before reduction.
aspect of the olecranon across the fracture site.
These wires can either remain intramedullary or
breach the anterior cortex. The proximal aspects
of the K-wires are bent 180 to create a loop
through which the 18-gauge steel wire can be
passed, and the K-wires are tapped into the prox-
imal ulna. The 18-gauge steel wire is then placed
into a gure of eight fashion and passed beneath
the bent K-wires. Subsequently, the 18-gauge
wire is twisted, tightened, and cut to provide
compression across the fracture site (Fig. 9).
The cut end of the wire is bent and placed against
the cortex of the ulna to minimize hardware
irritation.
1221
(b) Lateral view following screw placement. (c)
Anteroposterior view following screw placement (Cour-
tesy of Joshua M. Abzug, MD)
When performing the tension band technique,
some technical tips to follow include rmly seating
the K-wires against the olecranon by creating slits
in the triceps tendon to decrease the chance of
hardware prominence. These slits can subse-
quently be closed, also reducing the chance of the
wires backing out. Additionally, after the wire
knots are placed and cut, the wire should be bent
away from the subcutaneous border of the elbow.
An alternative to the steel gauge wire is utilization
of non-absorbable suture or braided wire, which
may lead to a lower hardware irritation rate. Lastly,
it is imperative that the tension band technique be
used only for simple transverse fractures. If it is
1222
Fig. 9 Lateral elbow radiograph demonstrating the ten-
sion band technique for olecranon fracture reduction
(Courtesy of Joshua M. Abzug, MD)
utilized for comminuted or oblique fractures,
malunions or nonunions will occur.
Fluoroscopic images are obtained to ensure an
adequate articular reduction was obtained and that
there is no obvious penetration of the proximal
radioulnar joint. Subsequently, the elbow is
brought through a passive range of motion, includ-
ing exion-extension as well as forearm rotation, to
assess for any obvious crepitus or blocks. The
incision is irrigated, the tourniquet deated, hemo-
stasis obtained, and the wound closed. After sterile
dressing of the wound, a long arm cast is applied
with the elbow in approximately 80 of exion.
To perform open reduction and internal xa-
tion (ORIF) with a plate and screw construct
(Fig. 10), the same approach described above for
a tension band is utilized to expose the fracture
site. Subsequently, the fracture hematoma is
removed and the fragments are reduced. This
may require application of the plate rst on the
distal fragment to aid in the initial reduction of
fractures that have extensive comminution pre-
sent. There are multiple commercially available
precontoured plates to t the proximal part of the
ulna of adolescents and adults. Alternatively, a
3.5 mm dynamic compression plate (DCP), recon-
struction plate, or a one-third tubular plate can be
bent to t the contour of the proximal ulna. This is
B.S. Schwartz et al.
especially necessary in younger patients where
the precontoured plates are too large. Because
many of these high-energy, comminuted fractures
occur in adolescents, crossing the apophysis with
the plate is not problematic, as little growth
remains. Once the plate and screws are applied,
it is imperative that the elbow is brought through a
full range of motion in both exion/extension as
well as pronation/supination to ensure there is no
crepitus or block from screws that may be too long
or that have violated the proximal radioulnar joint.
The wound is subsequently irrigated, the tourni-
quet deated, hemostasis obtained, and the inci-
sion closed.
When surgical intervention is required in either
apophyseal or metaphyseal olecranon fractures, a
long arm cast or splint in 7080 of exion should
be applied for 46 weeks. Radiographs are
obtained at 1 week postoperatively to ensure no
loss of reduction has occurred. Physical therapy
and active range of motion is instituted once
radiographic union is conrmed. The patient is
seen 23 weeks following cast removal for a
range of motion check. Hardware can remain in
place unless it is symptomatic. Hardware removal,
if necessary, is performed in the operating room
after full elbow motion has been restored.
Treatment-Specific Outcomes
There is little data available regarding outcomes
of pediatric olecranon fractures. No study has
directly compared the results of the aforemen-
tioned treatment options with regard to outcomes
or complications. In practice, most children are
able to be treated nonoperatively or with the utili-
zation of the percutaneous screw technique and
have good or excellent outcomes with restoration
of elbow motion and a very low complication rate.
Patients that require ORIF and those who have a
tension band placed have higher rates of stiffness
and have a higher complication rate, mainly
related to hardware irritation. Associated injuries
such as radial head, neck, and shaft fractures,
Monteggia variants, and lateral condyle fractures
can complicate the outcome. This is likely due to
the need for additional surgery, increased duration
of immobilization, and severity of initial injury
force to produce these concomitant fractures.
54 Radial Head, Radial Neck, and Olecranon Fractures
Fig. 10 Open reduction and internal xation of an olec-
ranon fracture utilizing a plate and screw construct. (a)
Lateral uoroscopic view prior to reduction. (b) Lateral
uoroscopic view following open reduction and internal
Preferred Treatment
True lateral elbow radiographs are obtained in
order to conrm displacement prior to determin-
ing a treatment choice. Non-displaced or mini-
mally displaced olecranon fractures are treated
with a long arm cast in 80 of exion for 34
weeks. When an acceptable reduction cannot be
achieved or maintained, the percutaneous screw
technique is utilized if the fracture pattern permits.
Postoperative immobilization for 4 weeks is uti-
lized followed by early initiation of active range
1223
xation. (c) Anteroposterior uoroscopic view following
open reduction and internal xation (Courtesy of Joshua
M. Abzug, MD)
of motion. Open reduction with internal xation
utilizing a plate screw construct is performed for
comminuted and oblique fractures. Tension band
techniques are not routinely performed given the
high rate of hardware irritation.
Surgical Pitfalls and Prevention
Excessive stripping of the periosteum about the
olecranon places the apophysis at risk for
devascularization and growth arrest. Transverse
fractures treated with inadequate xation are
1224 B.S. Schwartz et al.

Table 10 Olecranon fractures: Potential pitfalls and Table 11 Olecranon fractures: Management of
preventions complications
Potential pitfall Pearls for prevention Hardware irritation Removal of hardware
Apophyseal Avoid excessive stripping Pin migration
devascularization and of the periosteum of the Wire breakage
growth arrest olecranon Bursal formation
Delayed union/nonunion Ensure adequate xation Loss of terminal
elbow extension
Spur formation Resection of offending bone
Elongation of
more likely to develop healing complications such olecranon tip
as delayed union or nonunion. Ensuring that the Delayed union/ Revision of hardware to
construct provides adequate reduction and com- nonunion compress fracture site
pression of the fracture site is essential (Table 10). Bone grafting

Management of Complications
The most common complication following oper-
ative treatment of olecranon fractures is hardware
irritation. Symptomatic hardware prominence has
been reported to occur in up to 80 % of adult cases
following operative treatment (Murphy
et al. 1987). Pin migration, wire breakage, and
bursal formation may be seen following olecranon
xation. In children, this complication is low
since plate and screw constructs are rarely used.
Elbow stiffness after olecranon fractures is rel-
atively uncommon in the pediatric population.
However, loss of terminal extension may occur
in children who undergo open reduction and inter-
nal xation. This may be improved with removal
of the hardware once the fracture has completely
healed. Early physical therapy for range of motion
typically reduces the occurrence elbow stiffness
or loss of terminal extension.
Since olecranon fractures in children are often
oblique and minimally displaced, delayed unions
or nonunions are rare. Transverse fractures treated
nonoperatively or with inadequate xation are
most likely to develop complications regarding
healing. Revision of hardware to a construct that
compresses the fracture site, along with bone
grafting in some cases, is the best method for
managing nonunions. Additionally, spur forma-
tion and elongation of the olecranon tip can
occur, resulting in olecranon impingement in full
extension. Both are treated with resection of the
offending bone (Table 11).
Summary and Future Research
Pediatric radial head/neck and olecranon fractures
account for 1015 % of all pediatric elbow frac-
tures. The vast majority of these fractures can be
treated nonoperatively. When operative xation is
necessary, numerous techniques exist. Prospec-
tive studies comparing the outcomes and compli-
cations of the various techniques are needed. This
will likely require multicenter collaboration in
order to power the studies with enough numbers
for each treatment option.
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Calfee RP, Wilson JM, Wong AHW. Variations in the
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 Proximal Humerus Fractures
55
Casey M. de Deugd and Steven L. Frick

Contents Management of Complications . . . . . . . . . . . . . . . . . . . 1241

Introduction to Proximal Humerus Summary and Future Research to Improve
Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1228 Diagnosis, Treatment, and
Outcome Assessment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1241
Pathoanatomy and Applied Anatomy Relating
to Proximal Humerus Fractures . . . . . . . . . . . . . . . . . 1228 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1242
Assessment of Proximal Humerus Fractures . . . 1229
Signs and Symptoms of Proximal
Humerus Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1229
Imaging for Proximal Humerus Fractures . . . . . . . . . . 1230
Injuries Associated with Proximal
Humerus Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1231
Proximal Humerus Fractures Classication . . . . . . . . 1231
Proximal Humerus Fractures
Outcome Assessment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1233
Proximal Humerus Fractures
Treatment Options . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1233
Nonoperative Management of Proximal
Humerus Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1233
Operative Treatment of Proximal
Humerus Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1235
Surgical Procedure: Closed Reduction
and Percutaneous Pinning . . . . . . . . . . . . . . . . . . . . . . . . . . 1236
Preferred Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1239
Surgical Pitfalls and Prevention . . . . . . . . . . . . . . . . . . 1241

C.M. de Deugd (*)

University of Central Florida College of Medicine,
Orlando, FL, USA
e-mail: casey.dedeugd@gmail.com
S.L. Frick
Department of Orthopaedic Surgery, University of Central
Florida College of Medicine, Nemours Childrens
Hospital, Orlando, FL, USA
e-mail: steven.frick@nemours.org

# Springer Science Business Media New York (outside the USA) 2015 1227
J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_56
1228
Abstract
Proximal humerus fractures in skeletally imma-
ture patients are usually easily managed with
brief immobilization, even for very displaced
fractures. This anatomic area has great
remodeling potential in patients with greater
than 2 years of growth remaining, and the shoul-
der joint has a wide range of motion that can
easily accommodate a mild malunion without
functional loss. Clinical decision making for
patients who sustain very displaced fractures
near skeletal maturity can be difcult, as the
guidelines for acceptable position and align-
ment in the literature are not clear. This chapter
covers evaluation, classication, and treatment
guidelines for fractures of the proximal humerus
for all age groups and specically provides
guidelines for patients who are approaching
skeletal maturity.
Introduction to Proximal Humerus
Fractures
Proximal humerus fractures involving the growth
plate account for approximately 0.45 % of pediatric
fractures and 3 % of epiphyseal fractures (Mooney
and Webb 2009). Although they are infrequent,
they are often very displaced. Proximal humerus
fractures with minimal displacement are managed
easily with brief immobilization, regardless of the
age of the patient. The controversy in proximal
humerus fractures involves treatment choices for
displaced fractures, especially in patients older
than 11 years. Due to the immense remodeling
capabilities of bone in the proximal humerus,
even displaced fractures have reliably good out-
comes in skeletally immature patients. Classically,
displaced fractures have been treated by closed
reduction, and immobilization accepting residual
angulation and displacement (Neer and Horwitz
1965; Aufranc et al. 1969; Dameron and Reibel
1969; Hohl 1976; Cumming 1979). More recently,
however, there has been literature supporting sur-
gical intervention in older children within 2 years
C.M. de Deugd and S.L. Frick
of skeletal maturity and with severely displaced
fractures (Schwendenwein et al. 2004; Rajan
et al. 2008; Bahrs et al. 2009; Pahlavan
et al. 2011; Pandya et al. 2012).
Pathoanatomy and Applied Anatomy
Relating to Proximal Humerus
Fractures
The pathoanatomy of proximal humerus and
humeral fractures in children is inuenced by the
mechanism of injury, the attached muscles which
produce deforming forces, and the age of the
patient. The mechanism of injury for proximal
humeral fractures can be through direct (hyperex-
tension and excessive external rotation during
childbirth) (Cumming 1979) or indirect forces
(fall onto an outstretched hand or overuse) (Hohl
1976). Muscle attachments affecting alignment of
unstable fractures include the rotator cuff muscles
inserting on the proximal fragment, causing
abduction and external rotation of the proximal
portion, while the distal fragment is adducted and
shortened by the forces of the pectoralis major and
deltoid. Fracture displacement is inuenced by
the periosteum attaching the metaphysis to the
physis and epiphysis. The anterior periosteum is
thinner and thus angulation is usually directed
anteriorly, as most commonly the metaphysis dis-
places anteriorly. Physeal maturity is an important
consideration and can result in a predictable frac-
ture pattern depending on the patients age. Salter-
Harris type I fractures occur more frequently in
children younger than 5 years, when the physis
has a more transverse orientation (Ogden 2000),
and Salter-Harris type II fractures occur more
frequently in older children and adolescents,
when the physis has a pyramidal or tent shape
(Ogden 2000) (Fig. 1). Seventy-ve percent of
proximal humerus fractures in children greater
than 11 years of age are Salter II physeal injuries.
Other types of physeal fractures are rare in the
proximal humerus (Neer and Horwitz 1965;
Salter 1970). Between the ages of 5 and
11 years, there is an increased incidence of
55 Proximal Humerus Fractures
Fig. 1 In this rare open proximal humerus fracture in an adolescent, the tent-shaped morphology of the proximal humeral
physis is clearly displayed
proximal humeral metaphyseal fractures, likely
related to the rapid elongation of the metaphysis
during growth of the arm in these years
(Ogden 2000).
The most important pathoanatomical consid-
erations in treating proximal humerus fractures
are related to the growth potential of the
proximal humerus and the wide range of motion
possible through the shoulder. The proximal
humeral physis contributes 80 % of the length
of the humerus, and the metaphyseal region of
the humerus is capable of remodeling substan-
tial amounts of displacement and angulation
(Mooney and Webb 2009). The combination of
physeal reorientation with growth and perios-
teal remodeling of bone accounts for the ability
of growing bones to straighten themselves in
very skeletally immature patients (Abraham
1989), and the proximal humerus may have the
most remodeling potential of any area of the
body. In addition, the growth potential
and active periosteum of this area lead to
rapid fracture union. Coupled with this, the
wide range of motion of the shoulder can
accommodate substantial residual deformity
and angulation without functional loss.
This explains the reason that the vast majority
1229
of proximal humeral fractures in children can be
treated nonoperatively.
Assessment of Proximal Humerus
Fractures
Signs and Symptoms of Proximal
Humerus Fractures
Most patients with proximal humerus fractures
present following a traumatic event and direct or
indirect force application to the affected arm. The
signs and symptoms of proximal humerus frac-
tures are pain, decreased movement, swelling, and
ecchymosis in older children, which may be
delayed up to 24 hours after the injury. In infants
the only sign may be pseudoparalysis of the
affected arm. Associated neurological or vascular
injury is rare (Hwang et al. 2008). If there is
axillary nerve involvement, it can be tested for
by assessing sensation on the lateral shoulder in
cooperative patients. Severe swelling and a pulsa-
tile mass should prompt vascular examination, but
the authors have never encountered a vascular
injury in a skeletally immature patient with a
proximal humerus fracture.
1230 C.M. de Deugd and S.L. Frick

Fig. 2 Anteroposterior and lateral views of a proximal humerus fracture

Imaging for Proximal Humerus

Fractures

Good quality anteroposterior and lateral radio-

graphs of the shoulder are needed to properly
assess the fracture (Fig. 2). It is important to get
a lateral view without rotating the arm, with a
transthoracic or scapular Y-view preferred
(Fig. 3). Rotating the arm will be painful in unsta-
ble fractures and will only provide a lateral view
of the distal fragment. Contralateral shoulder
radiographs are rarely of benet but can be used
for comparison in infants or younger patients.
Advanced imaging is rarely needed in proximal
humerus fractures. In infants ultrasound can be
used to diagnose proximal humeral physeal inju-
ries. Computed tomography or magnetic reso-
nance imaging can be utilized to evaluate for a
possible physeal arrest if deformity develops fol-
lowing fracture. Fig. 3 In this scapular-Y lateral view, there is 100 %
Radiographs should be carefully scrutinized to displacement of the metaphysis (distal fragment) anterior
assess whether or not the bone is normal or to the proximal humeral epiphysis/humeral head
55 Proximal Humerus Fractures
Fig. 4 (a) The most common pathologic lesion of the
proximal humerus is a unicameral bone cyst as shown
here. These frequently present with pathologic fracture
following a minimal traumatic mechanism of injury. (b)
When fractures are sustained after minimal trauma or
abnormal, especially when the mechanism of
injury involved minimal or minor trauma. The
proximal humerus is a common site of pathologic
fractures in skeletally immature patients, espe-
cially secondary to unicameral or simple bone
cysts (Fig. 4). General management principles
are to allow healing of the fracture rst and then
to address the benign bone lesion.
Injuries Associated with Proximal
Humerus Fractures
Neurovascular injuries are a rare complication of
proximal humerus fractures, with a reported inci-
dence of 0.4 %. There is increased likelihood with
valgus displacement, as the metaphyseal fragment
displaces medially and can impinge on the neuro-
logical structures exiting the axilla (Hwang
et al. 2008). If nerve injury does occur, it is most
1231
secondary to muscle contractions in sport, the radiograph
should be carefully scrutinized for a bone lesion that would
weaken the bone. This fracture through a nonossifying
broma was sustained when throwing a ball
likely to involve the axillary nerve, with lesser
incidence of involvement of other nerves exiting
the axilla. Vascular injury to the axillary artery is
extremely rare in children.
The rotator cuff is a related anatomic structure
that may be injured in proximal humerus fractures
in adults, but is not typically involved in younger
patients. Since the supraspinatus, infraspinatus,
and teres minor insert onto the greater tuberosity
and the subscapularis on the lesser tuberosity,
contractile forces on the proximal fragment will
usually result in abduction and some external
rotation.
Proximal Humerus Fractures
Classification
The Neer-Horowitz classication is used most
commonly for proximal humerus fractures it is
1232
Fig. 5 Neer grade III or IV displacement. (a, b) shows a
Neer grade III fracture in a 14-year-old linebacker
sustained during a football tackle. (c) shows a Neer grade
confusing as the grade I uses millimeters of dis-
placement, and grades II through IV use displace-
ment as a percentage of the width of the shaft of
the humerus: grade I, no or minimal displacement
(<5 mm); grade II, less than 1/3 shaft width
displacement; grade III, less than 2/3 shaft width
displacement; and grade IV, more than 2/3 shaft
width displacement (Neer and Horwitz 1965). This
classication system is used to determine the
method of treatment (operative vs. nonoperative)
and the adequacy of reduction (Fig. 5). Classifying
C.M. de Deugd and S.L. Frick
IV fracture sustained by a 15-year-old multitrauma patient
who also had bilateral femur fractures
fractures using the Salter-Harris system can be
used as well, with younger patients tending to
have Salter-Harris type I fractures and older
children and adolescents most commonly
having Salter-Harris type II fracture patterns.
As intra-articular (trans-epiphyseal) fractures
(Salter-Harris types III and IV) and growth arrest
following proximal humeral fractures are both
exceedingly rare in the proximal humerus, the use
of the Salter-Harris classication is not clinically
very useful.
55 Proximal Humerus Fractures
Proximal Humerus Fractures
Outcome Assessment
The reported outcomes of proximal humerus frac-
tures are almost always satisfactory, regardless of
the method of treatment. This does not mean that an
individual patient is guaranteed to have a satisfac-
tory outcome, but it does make it quite difcult to
perform rigorous outcome studies with the statisti-
cal power capable of determining the suitability of
one treatment option over another for a particular
patient. Currently such a study does not exist, and
the majority of the literature available consists of
small retrospective case series and the expert opin-
ions of the authors. Assessment tools available to
evaluate shoulder function outcomes after proximal
humerus fracture include many standardized scores
(Constant score, Hospital for Special Surgery
(HSS) score system, and the American Shoulder
and Elbow Surgeons Standardized Shoulder
Assessment Form), but their value for injuries in
this area is questionable. A recent retrospective
case-control study comparing reduction and pin-
ning to reduction and intramedullary rod xation
for proximal humerus fractures points out the dif-
culty in designing studies and using outcome
assessments to discern clinically important differ-
ences in outcome based on treatment choices. In
this large series of 73 skeletally immature patients,
all achieved clinical and radiographic healing
without functionally limiting loss of global shoul-
der motion compared to the contralateral shoulder,
pain or weakness (Hutchinson et al. 2011).
Proximal Humerus Fractures
Treatment Options
Nonoperative Management
of Proximal Humerus Fractures
Indications/Contraindications
Most proximal humerus fractures can be treated
nonoperatively and result in good radiographic
healing and functional results (Table 1).
Nondisplaced or minimally displaced fractures
(Neer grade I or II) are treated with immobilization
with a sling and swathe or sling for 23 weeks.
1233
Table 1 Indications and relative contraindications for the
nonoperative management of proximal humerus fractures
Proximal humerus fractures
Nonoperative management
Contraindications
Indications (relative)
All patients < Neer grade Severe displacement
II displacement (Neer grade IIIIV)
Any amount of Patient approaching
displacement in <12 years skeletal maturity
Neurovascular injury
Open fracture
In displaced fractures (Neer grade III or IV),
closed reduction should be attempted in most
cases (Fig. 6). In some very young children,
even severely displaced fractures may be treated
with only immobilization. In these cases, an
above-the-elbow cast with a neck collar and cuff
around the wrist to suspend the cast and allow it
to apply traction to the fracture while the patient
is upright may be used. This has been called
hanging arm cast treatment and can be effective
for younger patients (less than 11 years) with
displaced fractures (Fig. 7). Patients are instructed
to sleep in a semi-erect position for 12 weeks
after the fracture.
Dening the amount of residual deformity after
closed reduction that is acceptable is dependent
on the age of the child; expert opinion by Beaty
has provided some general guidelines any
amount of displacement and up to 70 angulation
in children less than 5 years old, displacement up
to 50 % width of shaft and angulation up to
4045 in ages 512 years, and displacement of
<30 % width of the shaft and up to 1520 angu-
lation in children greater than 12 years old (Beaty
1992). There are few specic contraindications to
closed reduction open fractures or fractures with
vascular injury should be treated with open
reduction.
Techniques
Closed reduction with immobilization: Closed
reduction is achieved using traction, 90 of ex-
ion, then 90 of abduction and external rotation.
Occasionally manual pressure applied to the prox-
imal aspect of the distal fragment (proximal
1234
Fig. 6 This case nicely illustrates the value of attempting
closed reduction, even in markedly displaced fractures.
This 11-year-old had an apparent fracture dislocation
(note inferior position of humeral head relative to the
glenoid (a) and a completely displaced (Neer grade IV)
metaphysis) from anterior to posterior is helpful to
push the fragment back inside the soft tissue enve-
lope as traction is applied. Once the closed reduc-
tion maneuver is performed, uoroscopy can be
used to assess the reduction with the arm held in
abduction. Once reduced, the traction is then
released, and some axial load applied from distal
to proximal to attempt to engage the fragments
and hold the reduction as the arm is brought back
to the patients side. As in the management of all
displaced fractures, the goal is to obtain an accept-
able reduction and then maintain the reduction
through fracture union. Often the difculty in
proximal humerus fractures is maintenance of
the reduction as the arm is brought back to the
side. Historically this resulted in treatment of
these fractures with overhead traction or salute
casts with the arm held in abduction, exion, and
external rotation (Mooney and Webb 2009).
These techniques do not have a role in modern
fracture management. After closed reduction,
patients are placed in a sling and swathe, and
post-reduction radiographs should be analyzed
carefully. The position should be judged as
acceptable or unacceptable. If mild residual
varus is present, the use of a small abduction
pillow between the elbow and the trunk can help
maintain improved alignment. If acceptable,
immobilization for approximately 3 weeks is all
C.M. de Deugd and S.L. Frick
fracture). The inferior subluxation may have been so-called
pseudosubluxation. Traction, abduction, and external rota-
tion resulted in an anatomic, stable reduction under con-
scious sedation in the emergency department (b)
that is necessary. The patient can then begin pro-
gressive range-of-motion exercises. Formal phys-
ical therapy is rarely necessary in pediatric
orthopedic practice after fracture. If the position
is unacceptable, then closed reduction and xa-
tion in the operating room is scheduled electively.
Outcomes
Fractures at this site tend to have very good out-
comes as a result of increased growth (proximal
humeral physis contributes 80 % of humeral
growth) and remodeling that occurs here (Blount
1954; Salter 1970; Hohl 1976; Kohler and Trillaud
1983; Mooney and Webb 2009). Nonoperative
management is the treatment of choice for the
majority of proximal humeral fractures, even in
severely displaced fractures (Bahrs et al. 2009).
Large-scale studies of nonoperative management
of proximal humerus fractures in the pediatric pop-
ulation, with 5 and 8 years of follow-up, have
shown excellent functional and radiographic out-
comes (Kohler and Trillaud 1983; Baxter and
Wiley 1986). A recent review and meta-analysis,
which studied 12 publications with follow-up rang-
ing from 0.6 to 9 years, revealed no nonunions
reported and few malunions (Pahlavan et al. 2011).
Some of the long-term complications reported
were stiffness, loss of motion, weakness, and
functional limitations.
55 Proximal Humerus Fractures 1235

Fig. 7 Two large pins are used to stabilize a proximal removed in the clinic. Note the large bends in the pins
humerus fracture after acceptable closed reduction (a, b). outside the skin (c)
At 3 weeks early periosteal healing is noted and the pins are

Operative Treatment of Proximal Table 2 Indications and relative contraindications

Humerus Fractures for the operative management of proximal humerus
fractures
Indications/Contraindications Proximal humerus fractures
Surgical intervention is indicated in the rare open Operative management
fracture in this location; however, most opera- Indications Contraindications
tively treated fractures will be severely displaced Open fracture Young children
fractures in adolescents near skeletal maturity Near skeletal Acceptable alignment
(Neer grades IIIIV) (Table 2). In nearly all of maturity + severe achievable and maintained
fracture with closed reduction
these patients, the surgeon will be able to achieve maneuvers
Inability to reduce/
closed reduction under uoroscopy once adequate unstable reduction
anesthesia is obtained. Intra-articular fractures in Intra-articular
skeletally immature patients are very rare, but if displacement
encountered, the principles are the same as for
1236
displaced joint fractures in other locations. Ana-
tomic restoration of the articular surface is desired
provided that can be maintained until fracture
union with the use of xation. In young patients,
stiffness is not as much of a concern as in adults,
and so often, less rigid xation that is easily
removed is coupled with immobilization until
fracture union.
The main indication for operative treatment is
age greater than 11 years and a fracture that cannot
be reduced and maintained in a position with less
than 2/3 shaft width displacement (better than
Neer grade III position). Beaty recommended a
position of less than 40 angulation and less than
50 % displacement for patients age 12 years and
older (Beaty 1992), while Dobbs et al. (2003)
described their indications for attempted closed
reduction in the OR under general anesthesia sur-
gical reduction and pinning as greater than 2/3
width displacement (Neer grade III or grade IV)
and angulation greater than 45 in older adoles-
cents (>12 years). If a successful reduction could
be obtained (to grade II or less displacement and
less than 45 angulation), then stability was tested
(i.e., bring the arm to the side and check fracture
position). If unstable, the fracture was treated with
percutaneous pin xation and immobilization.
If an acceptable reduction could not be achieved
closed, an open reduction was performed using a
deltopectoral approach. Hutchinson et al. (2011)
dened their indications for surgical treatment as
age greater than 12 years and Neer grade IV
position (>40 angulation, greater than 2/3 shaft
width displacement).
Surgical Procedure: Closed Reduction
and Percutaneous Pinning
Preoperative Planning
The patient should be taken to the operating room
for general anesthesia and is positioned supine at
the edge of a completely radiolucent table to
facilitate uoroscopy (Table 3). A bump is placed
beneath the ipsilateral scapula. The arm and
shoulder are sterilely prepared and draped from
the ngertips to the chest in case an open
deltopectoral approach is needed. Repeat closed
C.M. de Deugd and S.L. Frick
Table 3 Preoperative planning for closed reduction and
percutaneous pinning of proximal humerus fractures
Percutaneous pinning for proximal humerus fractures
Preoperative planning
OR table: radiolucent table
Position/positioning aids: supine with large bump under
ipsilateral scapula, prep injured arm into eld from
ngertips to chest
Fluoroscopy location: C-arm can come in from foot or
head. Place monitor across body on opposite side
Equipment: split drapes, power wire driver, Kirschner
wires (0.062 in and larger dependent on age) or Steinman
pins, or 2.5 mm half pins with terminally threaded tips
Tourniquet (sterile/nonsterile): N/A
Table 4 Surgical procedure for closed reduction and
percutaneous pinning of proximal humerus fractures
Percutaneous pinning for proximal humerus fractures
Surgical steps
Closed reduction under uoroscopy
Stabilize fracture with pins
Start pin in safe zone laterally to avoid injury to axillary
and radial nerves
reduction is attempted. If an adequate reduction is
obtained, it is stabilized with two large pins
placed percutaneously (Table 4). The image inten-
sier can be brought in from cephalad over the
patients injured shoulder. Because of the risk of
migration with use of smooth pins, some prefer
pins with threaded tips, and it is recommended to
place a large bend in the end of the pins left
outside the skin to limit migration. The axillary
nerve is at risk and runs from posterior to anterior
around the proximal humerus in a zone 57 cm
distal to the tip of the acromion. Pins should thus
start distal to this and are placed from the lateral
proximal humeral metadiaphyseal area and
directed proximally into the humeral epiphysis
(Fig. 7). A small incision can be made and a
hemostat used to bluntly spread down to bone,
with a soft tissue guide then used around the pin to
prevent wrapping up nearby soft tissues. One
anatomical study describes the safe zone for
starting the pins, being between a spot two
humeral head heights on the anteroposterior
(AP) view below the articular surface (will be
below anterior axillary nerve branches) and
above the deltoid tuberosity (will be above radial
55 Proximal Humerus Fractures
Fig. 8 A combination of a cannulated screw and a prox-
imal distal pin was chosen to stabilize this severely
displaced proximal humerus fracture in an adolescent
nerve) (Rowles and McGrory 2001). The rst pin
starts anterolaterally, and then a second pin is
placed anterior or posterior to the rst, with an
attempt to get some divergence of the pins in the
epiphysis to enhance stability. The humerus is
circular in this area and the cortical bone is often
hard, making accurate pin placement technically
challenging. Drilling a small pilot starting hole
perpendicular to the bone and then reinserting
the tip of the wire and directing it proximally
into the epiphysis using uoroscopic guidance
are recommended. Larger pins are preferred
( 0.062 in.). There often is a posteromedial
Thurston-Holland fragment, and if large enough,
one pin can stay below the physis and engage this
fragment. If adequate stability cannot be obtained
with pins placed distal and lateral, a proximal to
distal pin can be placed lateral to the acromion,
through the greater tuberosity, across the fracture,
and engage the medial metaphysis of the distal
fragment. Some prefer to use cannulated screws
for improved bone purchase and less likelihood of
implant migration (Fig. 8). Catastrophic compli-
cations of smooth pin migration following pin
xation around the shoulder girdle has been
described in the literature (Lyons and Rockwood
1990). If screws are used and they cross the
physis, they should likely be removed in patients
with more than 2 years of growth remaining to
prevent the development of angular deformity
(Fig. 9).
1237
male. (a) shows the valgus displacement that should raise
concern for a possible brachial plexus injury. (b) shows the
cannulated screw and pin construct
If an acceptable reduction cannot be obtained,
an open reduction is performed through a
deltopectoral interval approach. An incision
made in the axillary crease anteriorly improves
cosmesis. The cephalic vein will mark the interval
between the deltoid and the pectoralis major mus-
cles, and the vein can be retracted to the side with
the most branches to limit bleeding. Developing
the plane beneath the deltoid down to the fracture
will identify any interposed soft tissues that need
to be removed from the fracture site. The long
head of the biceps tendon or periosteum may be
interposed and obstructing an accurate reduction
(Dobbs et al. 2003; Pandya et al. 2012), so dissec-
tion to identify the long head of the biceps is
recommended. After reduction, xation is
achieved percutaneously as noted above.
After xation following closed or open reduc-
tion, the arm is immobilized with a sling and
swathe for 34 weeks, at which time radiographs
are taken to assess healing (Table 5). At this time
the pins are usually discontinued in the ofce but
may be removed as an outpatient procedure in the
operating room based on individual patient factors.
The other commonly used technique for
maintaining the reduction until healing in skeletally
immature patients is intramedullary nail xation
(Dietz et al. 2006; Rajan et al. 2008; Hutchinson
et al. 2011; Pandya et al. 2012). Patient position for
these cases is supine on a radiolucent table, with the
patient moved to the edge of the table on the
1238
Fig. 9 In a severely displaced open fracture, two screws were chosen for added stability and to avoid a possible portal for
infection with percutaneously placed wires (a and b)
Table 5 Postoperative care following closed reduction
and percutaneous pinning of proximal humerus fractures
Percutaneous pinning for proximal humerus fractures
Postoperative protocol
Type of immobilization: sling and swathe
Length of immobilization: 34 weeks
Rehab protocol: progressive ROM exercises
Return to sport protocol: return to noncontact sports
4 weeks after pin removal and contact sports 8 weeks
after pin removal
Pins are usually removed in the ofce without anesthesia
uninjured side. The injured arm then completely
lies on the radiolucent table, and the C-arm unit
can be positioned opposite the injured arm. The
surgeon can come in for anteroposterior imaging
during nail insertion, with the forearm and arm
supported by the operating table (Dietz
et al. 2006). A small bump placed under the distal
humerus will facilitate starting the intramedullary
nails. Orbiting the uoroscopy unit back 60 ,
coupled with forward elevation of the arm, will
typically give an adequate lateral view. The nails
C.M. de Deugd and S.L. Frick
are inserted retrograde, with the distal insertion site
typically being lateral along the lateral column of
the distal humerus proximal to the olecranon fossa
(Dietz et al. 2006; Hutchinson et al. 2011). Typi-
cally two rods are used, but occasionally one rod
may be used based on the size of the humerus.
Good results have been reported with the use of
only one rod in older patients (Chee et al. 2006).
The rod diameter recommended is one-third the
diameter of the medullary canal (Dietz
et al. 2006). One rod is contoured into a C-shape,
and the second rod contoured into an S-shape. If the
surgeon elects to use two C-shaped rods with one
lateral and one medial entry hole, the ulnar nerve
should be protected on the medial side. The entry
hole is made with a drill bit larger than the size of
the nail, after blunt dissection down to the insertion
site exposes the bone. An oblique hole is drilled
from lateral to medial, and the nails are introduced
with the curved tip of the nail away from the medial
cortex. The nails are then advanced up the
intramedullary canal. Fracture reduction is con-
rmed by uoroscopy or direct inspection (for
55 Proximal Humerus Fractures
open cases), and the nail is advanced into the epiph-
ysis. If the reduction is not optimal after the rst nail
is inserted into the epiphysis, rotation of the nail can
be attempted to improve the reduction of the frac-
ture. A second nail is then inserted distally and also
guided up into the epiphysis using the tip of the nail
to direct it as it is driven into place. An attempt is
made to get maximal spread between the tips of the
nails in the epiphysis to enhance rotational stability.
Once both nails are appropriately positioned prox-
imally, the nail is cut near its entry site with a rod
cutter, leaving enough nail outside the bone to
facilitate later implant removal (11.5 cm). Nails
that protrude too much can cause soft tissue
impingement and skin problems. A sling is used
for comfort after surgery but can be removed once
muscular control of the arm returns. Motion of the
elbow and shoulder as tolerated is then encouraged.
The necessity of implant removal in skeletally
immature patients is debatable. Routine removal
of intramedullary nails is often recommended as a
secondary outpatient procedure (Dietz et al. 2006;
Hutchinson et al. 2011).
Treatment-Specific Outcomes
The outcomes for patients younger than 12 years
reported in the literature are uniformly excellent
following nonoperative treatment (Neer and
Horwitz 1965; Aufranc et al. 1969; Dameron
and Reibel 1969; Baxter and Wiley 1986; Beaty
1992; Beringer et al. 1998; Ogden 2000; Bahrs
et al. 2009; Mooney and Webb 2009; Pahlavan
et al. 2011). The controversy over treatment
choices in adolescents with severely displaced
fractures is best outlined by three studies.
Beringer et al. (1998) studied 48 patients with an
average age of 14 years and an average fracture
displacement of 78 %. All had an attempt at closed
reduction, and in 55 % the attempt did not
improve the position substantially (30 % or
more). Nine were treated operatively. All patients
reported excellent outcomes with no patient in
either the nonoperatively treated or operatively
treated group reporting limitations of activity or
employment. Three operatively treated patients
had complications (fracture through pin site,
impingement by plate, osteomyelitis) leading the
authors to favor nonoperative treatment in older
1239
patients regardless of the magnitude of fracture
displacement. Following attempted closed reduc-
tion, the authors stated that persistent malposi-
tion does not appear to justify operative
intervention with its attendant risk of complica-
tion (Beringer et al. 1998). Dobbs et al. (2003)
published a series of adolescents with severely
displaced proximal humerus fractures, with
23 undergoing operative treatment with no
reported complications. This study showed that
modern operative treatment could be performed
safely in patients with persistent malposition and
limited remodeling potential to obtain and main-
tain good fracture position. A case-control retro-
spective study comparing percutaneous pinning
and intramedullary nail utilization for proximal
humerus fractures showed good and functional
results with no loss of motion with either method
(Hutchinson et al. 2011). A recent retrospective
study of open reduction of proximal humerus
fractures in adolescents reported a mean radio-
graphic union time of 4 weeks with excellent
functional results (Pandya et al. 2012).
Preferred Treatment
Minimally and moderately displaced fractures in
patients of all ages are treated with immobilization
using a sling for 3 weeks. Displaced (Neer grade
III and IV) fractures in children less than 12 years
old are treated with an attempt at closed reduction
if the patient presents acutely (same day of injury)
in an environment where conscious sedation can
be performed safely (emergency department)
(Fig. 6). Obtaining an improved position in
patients this age will lessen reliance on
remodeling and will shorten the time to return of
full unrestricted motion, thus justifying an attempt
at closed reduction of markedly displaced frac-
tures even in young patients. Because of the
immense remodeling potential and accommoda-
tive ability of the shoulder joint, residual displace-
ment and angulation are accepted if near anatomic
reduction is not achieved or maintained. A hang-
ing arm cast is applied and worn for 3 weeks.
Patients are instructed to sleep in a semi-reclining
position for 1 week. Radiographs are repeated at
1240
Fig. 10 This 10-year-old sustained a completely displaced and shortened metaphyseal proximal humerus fracture (a) that
was treated with a hanging arm cast. It healed in 3 weeks (b) and remodeled nearly completely at 12 months (c)
1 week to document acceptable alignment.
Healing in a completely displaced position with
angulation less than 70 will remodel (Fig. 10).
Indications for reduction and operative stabiliza-
tion in children less than 10 years are limited to
rare open fractures or multitrauma patients.
In patients older than 12 years of age with
fracture displacement greater than two-thirds of
the shaft or angulation greater than 40 , closed
reduction under conscious sedation is attempted if
the patient presents to the emergency department
on the day of injury. If an acceptable closed reduc-
tion is obtained, a sling and swathe is applied and
early radiographic follow-up is scheduled (prefer-
ably within 5 days) to document maintenance of
acceptable reduction. For patients who present
later after injury, who cannot be reduced to an
C.M. de Deugd and S.L. Frick
acceptable position, or who lose reduction at
early follow-up evaluation, a closed reduction
under general anesthesia with the possibility of
an open reduction is scheduled. Improvement to
a Neer grade II (1/3 shaft diameter or less) position
is accepted after closed reduction. After open or
closed reduction, the fracture is stabilized with
two or three large smooth wires. The wires are
bent at 90 or greater angle outside the skin and
covered with sterile felt and a bandage, and the
patient is immobilized in a sling and swathe for
3 weeks. Radiographs are taken at 1 week and
3 weeks after reduction and pinning to document
maintenance of reduction and early healing. The
pins are removed in the clinic at 3 weeks after
evaluation of the radiographs, and the patient is
instructed in shoulder range-of-motion exercises.
55 Proximal Humerus Fractures 1241

Table 6 Potential pitfalls and suggested strategies for Table 7 Complications and suggested management for
prevention of when caring for children with proximal patients with operative and nonoperatively managed prox-
humerus fractures based on historical data and reviewed imal humerus fractures
literature
Proximal humerus fractures
Proximal humerus fractures Common
Potential pitfalls and preventions complication Management
Potential pitfall Pearls for prevention Proximal humerus Corrective osteotomy (Ugwonali
Overtreatment Nonoperative management and varus et al. 2007) rarely needed
remodeling successful for vast Measure neck-shaft angle
majority of fractures Observation self-resolving
Pin migration Cut pins long, large bend, use Limb length Limb lengthening usually
terminally threaded pins inequality functionally inconsequential
Pin site infection Dressings to minimize pin-skin (solved by a good tailor)
motion, immobilization Loss of motion Instruct in home range-of-motion
Misplaced pins Careful uoroscopic check prior exercises, especially in older
to leaving OR children
Iatrogenic axillary Start pin two humeral head heights Inferior More common in Salter-Harris
nerve injury below articular surface glenohumeral type II fractures
Iatrogenic radial Start pin above deltoid tuberosity subluxation Period of immobilization
nerve injury followed by rotator cuff
strengthening
Osteonecrosis Associated disruption of branch
of anterior circumex artery
The evaluation of return of motion and radio- Rarely occurs no specic
graphic surveillance are completed 1 and 2 months prevention or treatment effective
later. Noncontact sports activities are resumed Nerve injury Usually axillary nerve
1 month after pin removal. If satisfactory healing Document thorough
is noted and motion is full, the patient is cleared neurovascular exam
for return to contact sports 2 months after pin Study/explore if no signs of
recovery after 4 months
removal.
Growth arrest Limb lengthening rarely needed
Recognize physeal injuries
monitor for varus
Surgical Pitfalls and Prevention Follow-up until normal growth
and function noted
See (Table 6).

Management of Complications activities. Improvements in surgical techniques

See (Table 7).
Summary and Future Research
to Improve Diagnosis, Treatment, and
Outcome Assessment
The management of proximal humerus fracture is
relatively straightforward, with the majority
being amenable to closed treatment with expec-
tations for rapid healing, excellent remodeling
potential, and return to unrestricted, full
for management of fractures has claried the role
of reduction and stabilization with pins or
intramedullary nails for nearly all skeletally
mature patients with severely displaced fractures,
with excellent outcomes reported and very low
complication rates. The striking displacements
sometimes seen with these fractures in younger
patients will often tempt surgeons to operate;
however, it is highly likely that an attempt at
closed reduction with acceptance of whatever
position can be achieved will result in an
excellent outcome with low risk to the patient.
Blount stated in 1954 that the greatest fallacy is
1242
to think that accurate reduction of an epiphyseal
fracture at the proximal end of the humerus is
important enough to require an open reduction
(Blount 1954). In the modern era of orthopedic
surgery and operative fracture management, the
greatest risk for pediatric patients with proximal
humerus fractures is that future generations of
orthopedic surgeons will have a poor understand-
ing of closed treatment methods and the
remodeling potential of this region and therefore
will be overly aggressive in managing these inju-
ries operatively. Except for patients older than
12 years of age with Neer grade III or IV
displaced fractures, almost all pediatric
proximal humerus fractures should be managed
nonoperatively.
References
Abraham E. Remodeling potential of long bones following
angular osteotomies. J Pediatr Orthop. 1989;9(1):3743.
Aufranc OE, Jones WN, Bierbaum BE. Epiphysial fracture
of the proximal humerus. JAMA. 1969;207(4):7279.
Bahrs C, Zipplies S, Ochs BG, Rether J, Oehm J,
Eingartner C, et al. Proximal humeral fractures in chil-
dren and adolescents. J Pediatr Orthop. 2009;29(3):
23842.
Baxter MP, Wiley JJ. Fractures of the proximal humeral
epiphysis. Their inuence on humeral growth. J Bone
Joint Surg. 1986;68(4):5703.
Beaty JH. Fractures of the proximal humerus and shaft in
children. Instr Course Lect. 1992;41:36972.
Beringer DC, Weiner DS, Noble JS, Bell RH. Severely
displaced proximal humeral epiphyseal fractures: a
follow-up study. J Pediatr Orthop. 1998;18(1):317.
Blount W. Fractures in children. Baltimore: Williams &
Wilkins; 1954.
Chee Y, Agorastides I, Garg N, Bass A, Bruce C. Treatment
of severely displaced proximal humeral fractures in
children with elastic stable intramedullary nailing. J
Pediatr Orthop B. 2006;15(1):4550.
Cumming WA. Neonatal skeletal fractures. Birth trauma or
child abuse? J Can Assoc Radiol. 1979;30(1):303.
Dameron Jr TB, Reibel DB. Fractures involving the prox-
imal humeral epiphyseal plate. J Bone Joint Surg Am.
1969;51(2):28997.
Dietz H, Schmittenbecher P, Slongo T, Wilkins K.
Humerus. In: Shlongo T (ed) Elastic stable intra-
medullary nailing in children. Davos: Thieme; 2006.
p. 2030.
C.M. de Deugd and S.L. Frick
Dobbs MB, Luhmann SL, Gordon JE, Strecker WB,
Schoenecker PL Severely displaced proximal humeral
epiphyseal fractures. J Pediatr Orthop. 2003;23(2):
20815.
Hohl JC. Fractures of the humerus in children. Orthop Clin
North Am. 1976;7(3):55771.
Hutchinson PH, Bae DS, Waters PM. Intramedullary
nailing versus percutaneous pin xation of pediatric
proximal humerus fractures: a comparison of compli-
cations and early radiographic results. J Pediatr Orthop.
2011;31(6):61722.
Hwang RW, Bae DS, Waters PM. Brachial plexus palsy
following proximal humerus fracture in patients who
are skeletally immature. J Orthop Trauma. 2008;22(4):
28690.
Kohler R, Trillaud JM. Fracture and fracture separation of
the proximal humerus in children: report of 136 cases.
J Pediatr Orthop. 1983;3(3):32632.
Lyons FA, Rockwood Jr CA. Migration of pins used in
operations on the shoulder. J Bone Joint Surg
Am. 1990;72(8):12627.
Mooney JF, Webb LX. Fractures and dislocations about the
shoulder. In: Green NE, Swiontkowski MF, editors.
Skeletal trauma in children, vol. 3. 4th
ed. Philadelphia: Saunders/Elsevier; 2009. p. 283311.
Neer 2 CS, Horwitz BS. Fractures of the proximal humeral
epiphysial plate. Clin Orthop Relat Res. 1965;41:
2431.
Ogden JA. Humerus. In: Skelty etal injury in the child. 3rd
ed. In: Ogden JA (ed) New York: Springer; 2000.
p. 46675.
Pahlavan S, Baldwin KD, Pandya NK, Namdari S,
Hosalkar H. Proximal humerus fractures in the pediat-
ric population: a systematic review. J Child Orthop.
2011;5(3):18794.
Pandya NK, Behrends D, Hosalkar HS. Open reduction of
proximal humerus fractures in the adolescent popula-
tion. J Child Orthop. 2012;6(2):1118.
Rajan RA, Hawkins KJ, Metcalfe J, Konstantoulakis C,
Jones S, Fernandes J. Elastic stable intramedullary
nailing for displaced proximal humeral fractures in
older children. J Child Orthop. 2008;2(1):159.
Rowles DJ, McGrory JE. Percutaneous pinning of the
proximal part of the humerus. An anatomic study.
J Bone Joint Surg Am. 2001;83-A(11):16959.
Salter R. chapter 16: Fractures, dislocations and soft tissue
injuries. In: Textbook of disorders & injuries of
the musculoskeletal system. Baltimore: Williams &
Wilkins; 1970. p. 5312.
Schwendenwein E, Hajdu S, Gaebler C, Stengg K, Vecsei
V. Displaced fractures of the proximal humerus in
children require open/closed reduction and internal
xation. Eur J Pediatr Surg. 2004;14(1):515.
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52932.
 Humeral Shaft Fractures
56
Brandon S. Schwartz and Joshua M. Abzug

Contents Abstract
Introduction to Humeral Shaft Fractures . . . . . . . 1243 Humeral shaft fractures in skeletally immature
patients are usually managed with immobiliza-
Pathoanatomy and Applied Anatomy Relating
to Humeral Shaft Fractures . . . . . . . . . . . . . . . . . . . . . . 1244
tion, even for displaced fractures, as this area
has great remodeling potential in patients with
Assessment of Humeral Shaft Fractures . . . . . . . . 1245 greater than 2 years of growth remaining. The
Signs and Symptoms of Humeral Shaft
Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1245 wide range of motion from the shoulder joint
Humeral Shaft Fracture Imaging and can easily accommodate a mild malunion with-
Other Diagnostic Studies . . . . . . . . . . . . . . . . . . . . . . . . . . . 1246 out functional loss. Akin to the controversy
Injuries Associated with Humeral Shaft between nonoperative and operative manage-
Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1246
Humeral Shaft Fracture Classication . . . . . . . . . . . . . 1247 ment in the adult literature, some favor opera-
Humeral Shaft Fracture Outcome Tools . . . . . . . . . . . 1248 tive intervention in those patients approaching
Humeral Shaft Fracture Treatment Options . . . . 1248
skeletal maturity, but the vast majority of
Nonoperative Treatment of Humeral Shaft patients can be treated nonoperatively.
Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1248
Operative Treatment of Humeral Shaft
Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1251
Introduction to Humeral Shaft
Preferred Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1254 Fractures
Surgical Pitfalls and Prevention . . . . . . . . . . . . . . . . . . . . 1256
Management of Complications . . . . . . . . . . . . . . . . . . . . . 1256
Fractures of the diaphysis, or shaft, of the humerus
Summary and Future Research . . . . . . . . . . . . . . . . . . 1256 are uncommon in the pediatric population and
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1257 account for only 25 % of all childhood fractures
and less than 10 % of all humerus fractures in
children (Cheng and Shen 1993). The estimated
incidence of pediatric humeral shaft fractures has
been reported to be between 1 and 3:10,000 chil-
dren per year (Landin 1983; Worlock and Stower
1986). These injuries occur most frequently in
neonates, with a prevalence reported between
B.S. Schwartz (*) J.M. Abzug 0.035 % and 0.35 %, and are second to clavicle
University of Maryland School of Medicine, Baltimore,
fractures as the most common birth fracture
MD, USA
e-mail: brandon.schwartz@som.umaryland.edu; (Madsen 1955; Shrader 2007). There is a greater
jabzug@umoa.umm.edu risk of neonatal humeral shaft fractures in cases of
# Springer Science+Business Media New York 2015 1243
J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_87
1244
Fig. 1 (a) Anteroposterior and (b) lateral radiographs demonstrating a humeral shaft fracture in a 6-day-old infant, which
occurred as a result of a complicated delivery (Courtesy of Joshua M. Abzug, MD)
macrosomia and breech presentation. Although
birthing injury is a common mechanism of pedi-
atric humeral diaphyseal fractures, a bimodal dis-
tribution has been reported, with the majority of
fractures occurring in children less than 3 years of
age and children aged 12 years and older (Beaty
1992). Fractures in older patients are more likely
to be due to direct trauma, falls, penetrating inju-
ries, or motor vehicle collisions.
Special consideration should be given to chil-
dren presenting with a humeral shaft fracture, as
non-accidental trauma is a possible mechanism
for these injuries. In cases of child abuse, humeral
fractures are thought to account for over 60 % of
newly diagnosed injuries and 12 % of all fractures
(Loder and Bookout 1991; Shaw et al. 1997).
Pathoanatomy and Applied Anatomy
Relating to Humeral Shaft Fractures
The shaft of the humerus extends from the prox-
imal margin of the pectoralis major insertion to the
supracondylar ridge distally. On the posterior
aspect of the diaphysis, the spiral groove of the
humerus contains the radial nerve (Carroll
et al. 2012). In a cadaveric study by Gerwin et al.,
B.S. Schwartz and J.M. Abzug
the radial nerve was shown to traverse the poste-
rior humerus approximately 14 cm proximal to the
lateral epicondyle and remains in close proximity
to the posterior humerus for approximately 6.5 cm
(Gerwin et al. 1996). Due to the location of the
radial nerve adjacent to the humerus, the nerve is
at increased risk of concomitant injury with
a humeral shaft fracture (Carroll et al. 2012).
A Holstein-Lewis fracture can occur when a frac-
ture of the distal third of the humerus results in
entrapment of the radial nerve.
The humerus is enclosed in a thick periosteal
sleeve along the length of the diaphysis, which
aids in resisting displacement and promoting
healing, following a fracture (Bachman and
Santora 2006). Therefore, fractures resulting in
signicant angulation or translation frequently
occur via high-energy mechanisms such as
motor vehicle collisions.
The mechanism of neonatal fractures of the
humeral diaphysis typically involves rotation or
hyperextension of the upper extremity during pas-
sage through the birth canal (Fig. 1) (Shrader
2007). The most common fracture pattern
observed in neonates is a complete, transverse
fracture of the proximal third of the humerus
(Caviglia et al. 2005).
56 Humeral Shaft Fractures
Fig. 2 (a) Anteroposterior
and (b) lateral radiographs
of the humerus
demonstrating a benign
unicameral bone cyst within
the proximal humerus.
Pathologic fractures are
common when large cysts
are present in this area
(Courtesy of Joshua
M. Abzug, MD)
Humeral shaft fractures in children younger
than 3 years of age may be associated with
child abuse/non-accidental trauma. Fracture
patterns correlated with abuse include transverse
fractures, typically caused by direct trauma;
oblique fractures, produced by traction with
rotation of the humerus; and metaphyseal
bucket-handle fractures (Fisher 1958). The pres-
ence of concomitant fractures of the ribs, sternum,
or scapula is highly suspicious for child abuse
and should be investigated further. Fractures of
the humeral diaphysis may also occur following a
fall on an outstretched hand (FOOSH) or from
direct trauma to the upper arm (Shrader 2007;
Carson et al. 2006).
The mechanisms of injury in children older
than 10 years of age are most frequently
associated with motor vehicle collisions and
sporting activities. However, pathologic fractures
may be seen in this age group due to various
tumors and diseases affecting bone integrity, as
the humerus is a common site of bone cysts and
other benign lesions (Shaw et al. 1997; Caviglia
et al. 2005; Carson et al. 2006; Shrader 2007)
(Fig. 2). Stress fractures may also be observed,
although there will be no displacement or
visible deformity.
1245
Assessment of Humeral Shaft
Fractures
Signs and Symptoms of Humeral Shaft
Fractures
Children and adolescents with humeral shaft frac-
tures typically present with mid-arm pain, swell-
ing, and ecchymosis. Patients often hold the
affected arm in adduction and internal rotation.
A concomitant radial nerve injury may be present
when a humeral shaft fracture occurs or as a com-
plication of manipulation (Caviglia et al. 2005;
Shrader 2007). When a radial nerve injury is pre-
sent, patients of sufcient age (typically 8 years or
greater) will complain of numbness to the dorsum
of the hand between the rst and second metacarpal
and motor decits, including decreased thumb and
wrist extension, and difculty with forearm supi-
nation. Therefore, radial nerve function should
be evaluated by testing the rst dorsal web space
sensation as well as wrist, thumb, and digital exten-
sion. In addition to a decrease in function of
forearm muscles, patients who sustain a Holstein-
Lewis fracture will frequently display a signicant
decrease in strength of the triceps as well.
1246
Physical ndings in the neonate are similar,
with mid-arm swelling, ecchymosis, and possible
deformity. However, as infants cannot adequately
express pain and discomfort, these patients may
exhibit irritability when held. In addition, neo-
nates sustaining a humeral shaft fracture may
refuse to move the affected extremity secondary
to pain, termed a pseudoparalysis, or may dem-
onstrate an asymmetric Moro reex (Caviglia
et al. 2005; Benjamin and Hang 2007). Differen-
tial diagnoses for a diaphyseal humerus fracture in
a newborn include a clavicle fracture, brachial
plexus injury, and separation of the proximal
humeral epiphysis (ONeill et al. 1973; Szaley
and Rockwood 1982; Leonidas 1983; Caviglia
et al. 2005). However, it is important to remember
that a humerus fracture can coexist with a brachial
plexus birth palsy.
Humeral Shaft Fracture Imaging
and Other Diagnostic Studies
Plain radiographs of the humerus are sufcient for
diagnosis of a humeral shaft fracture.
Anteroposterior (AP) and lateral views of the
entire humerus should be obtained (Fig. 3).
Fig. 3 (a) Anteroposterior
and (b) lateral views of the
humerus demonstrating a
diaphyseal humerus
fracture in a 12-year-old
boy (Courtesy of Joshua
M. Abzug, MD)
B.S. Schwartz and J.M. Abzug
Additionally, the shoulder and elbow joints
should be visualized, with dedicated radiographic
series, in order to ensure there are no concomitant
injuries. Magnetic resonance imaging and bone
scan with technetium may be utilized to conrm
the diagnosis in cases of a suspected stress frac-
ture (Caviglia et al. 2005). If non-accidental
trauma is a possible mechanism of injury, a full
skeletal survey should be obtained.
Injuries Associated with Humeral Shaft
Fractures
The radial nerve is vulnerable to injury when a
diaphyseal humerus fracture is sustained. The
incidence of radial nerve palsies related to an
injury to the humerus ranges from 2.4 % to 20 %
(Caviglia et al. 2005). These palsies are described
as either primary radial nerve palsies, occurring at
the time of the fracture, or secondary radial nerve
palsies, which occur during manipulation. Com-
plete laceration of the radial nerve is rare; instead
the radial nerve is commonly entrapped at the site
of the fracture. The prognosis is generally excel-
lent in the majority of cases with observation
alone. Therefore, observation for a minimum of
56 Humeral Shaft Fractures
Fig. 4 (a) Anteroposterior
and (b) lateral radiographs
demonstrating a gunshot
wound to the upper
extremity resulting in a
comminuted humeral shaft
fracture in a 15-year-old
boy (Courtesy of Joshua
M. Abzug, MD)
8 weeks is the mainstay of treatment for radial
nerve palsies, with complete resolution of symp-
toms occurring in 78100 % of patients (Amillo
et al. 1993).
Humeral Shaft Fracture Classification
Pediatric humeral shaft fractures are classied
based on the location, fracture pattern, displace-
ment, and angulation present. The Association
for the Study of Internal Fixation (AO-ASIF)
has proposed a classication scheme for diaphy-
seal fractures of the humerus, although its utili-
zation has been limited for fractures in children
and adolescents (Muller et al. 1991). The Muller
AO classication involves three subgroups
(A-C) based on the type of fracture (simple,
wedge, or complex). The A subgroup consists
of simple fractures. A1 fractures are spiral frac-
tures, A2 fractures are oblique (>30 ), and A3
1247
fractures are transverse (<30 ). The B subgroup
consists of wedge fractures. B1 fractures exhibit
a spiral wedge fragment, B2 fractures involve a
bending wedge fragment and are considered
unstable due to their short fracture zone, and B3
fractures involve a fragmented wedge pattern and
are quite rare. B1 fractures are all at risk of
having an associated radial nerve injury. The C
subgroup consists of complex fractures and typ-
ically involves high-energy mechanisms of
injury. C1 fractures contain fragments created
from a spiral fracture and can extend into both
the proximal or distal metaphysis. C2 fractures
are rare and consist of segmental fragments. C3
fractures are complex fractures with irregular
fragments and are typically the result of
extremely high-energy mechanisms such as a
high velocity missile injury (Fig. 4). These
fractures are commonly open and oftentimes
exhibit a concomitant radial nerve injury
(Muller et al. 1991).
1248
Humeral Shaft Fracture Outcome Tools
No specic outcome tools exist to evaluate
humeral shaft fractures.
Humeral Shaft Fracture Treatment
Options
Nonoperative Treatment of Humeral
Shaft Fractures
Indications/Contraindications
The majority of pediatric diaphyseal humerus
fractures can be treated nonoperatively with
immobilization alone. However, open fractures,
fractures with obvious clinical deformity or sig-
nicant angulation, fractures resulting in signi-
cant limb shortening, and fractures with a
developing compartment syndrome should be
treated operatively. An age-based algorithm is
frequently utilized to determine acceptable angu-
lation and displacement: indications for
nonoperative management include children
under 5 years of age with less than 70 of angula-
tion and total displacement, children 512 years
of age with 4070 of angulation, and children
greater than 12 years of age with less than 40 of
angulation and 50 % apposition. Additionally,
fractures resulting in limb shortening of less than
23 cm may be treated with immobilization alone
(Table 1) (Beaty 1992; Shrader 2007; Carroll
et al. 2012).
Techniques
The rst step in management of diaphyseal
humerus fractures is administration of appropriate
analgesia, as pain should be controlled prior to
radiographic evaluation. Intravenous morphine
should be considered for initial pain control in
patients with moderate to severe pain while oral
ibuprofen is typically sufcient for patients with
mild pain. Infants with a humeral shaft fracture
should be treated conservatively with immobili-
zation of the arm utilizing a swathe technique,
regardless of fracture displacement. Due to rapid
healing in infants, immobilization is only neces-
sary for 34 weeks (Fig. 5) (Shrader 2007).
B.S. Schwartz and J.M. Abzug
Table 1 Humeral shaft fractures: Nonoperative
management
Indications Contraindications
Nondisplaced fractures Open fractures
Minimally displaced/ Fractures associated with a
angulated fractures vascular injury
Fractures with clinical
deformity or signicant
angulation based on age
Under 5: 4070
512: 4070
Over 12: >40 and 50 %
apposition
Fractures resulting in greater
than 23 cm of limb
shortening
Fractures with evidence of
compartment syndrome
The neurovascular status should be evaluated
prior to and following swathe application. It is
important to assess for a concomitant brachial
plexus birth palsy following swathe removal, as
once callus is present, children will move the
extremity if no brachial plexus palsy is present.
As the humeral shaft is enclosed in a thick
periosteal sleeve that limits fracture displacement,
children and adolescents who have sustained an
incomplete fracture may also be managed with
immobilization via a sling and swathe, collar and
cuff sling, shoulder immobilizer, hanging arm
cast, or fracture brace (Bachman and Santora
2006). Patients with moderately displaced or
completely displaced fractures should be placed
into an upper arm sugar tong splint with a
supporting forearm sling or a hanging long
arm cast may be utilized (Carson et al. 2006;
Shrader 2007).
Closed reduction may be performed in some
cases of diaphyseal humerus fractures when a
clinical deformity is apparent. Following closed
reduction, the correction of the clinical deformity
is often believed to be of more value than the
radiographic alignment (Shrader 2007). The
patient is then placed in a coaptation splint for
approximately 2 weeks. If after this time the
fracture remains in acceptable alignment, a
Sarmiento-type clamshell brace is applied for
56 Humeral Shaft Fractures 1249

Fig. 5 (a) Anteroposterior and (b) lateral radiographs demonstrating a healing humeral shaft fracture with callous
formation in a 5-week-old infant (Courtesy of Joshua M. Abzug, MD)

Fig. 6 A hanging arm cast

is used to immobilize a
humeral shaft fracture in a
young boy (Courtesy of
Joshua M. Abzug, MD)

approximately 4 weeks in order for callus forma-
tion to occur (Sarmiento et al. 1990; Shrader
2007). Patients sustaining humeral shaft frac-
tures that exhibit shortening or some anterior or
posterior angulation may be treated with a hang-
ing arm cast until acceptable healing has
occurred. The hanging arm cast produces a lon-
gitudinal traction utilizing the weight of the arm
and can aid in correcting anterior or posterior
angulation as well as varus or valgus alignment
(Fig. 6) (Caviglia et al. 2005). Radiographs
should be obtained on a weekly basis for the
initial 3 weeks, and shoulder range of motion
exercises are generally initiated 4 weeks follow-
ing the initial injury (Caviglia et al. 2005;
Shrader 2007).
1250
Fig. 7 (a) Anteroposterior
and (b) lateral radiographs
demonstrating a healing
humeral shaft fracture with
callous formation in a
14-year-old boy (Courtesy
of Joshua M. Abzug, MD)
Humeral shaft fractures that lack a plausible
or documented mechanism must be investigated
further by a team of child protection specialists.
In the United States, it is mandatory for the
treating professional to report any suspicious
ndings to Child Protective Services. Addition-
ally, the patient should undergo a full musculo-
skeletal examination as well as a full
physical, including a funduscopic, examination
to assess for retinal hemorrhages, to assess
for any other potential signs of abuse such as
bruising. A skeletal survey should also be
obtained.
Outcomes
The prognosis for skeletally immature patients
who have sustained a diaphyseal humerus fracture
managed nonoperatively is typically excellent,
especially in younger patients, due to an increased
potential for healing and remodeling (Fig. 7)
(Caviglia et al. 2005).
B.S. Schwartz and J.M. Abzug
Although outcomes for nonoperative manage-
ment in children have not been extensively
reported on, nonunion of humeral shaft fractures
across all ages is rarely observed, with union
rates greater than 90 % achieved with
nonoperative treatment (Koch et al. 2002;
Ekholm et al. 2006). Sarmiento et al. reported
less than 2 % of patients with closed fractures
and 6 % of patients with open fractures went on to
nonunion following functional bracing. The
mean time to union was 914 weeks in closed
and open fractures, respectively. Despite a major-
ity of patients experiencing good to excellent
outcomes following nonoperative treatment of
humeral shaft fractures, some studies report
lower overall functional outcomes in patients
with a fracture as compared to an uninjured pop-
ulation (Koch et al. 2002; Ekholm et al. 2006).
Additional research is needed to fully understand
the outcomes of nonoperative treatment in the
pediatric population.
56 Humeral Shaft Fractures
Operative Treatment of Humeral Shaft
Fractures
Indications/Contraindications
Intramedullary nailing of humeral diaphyseal
fractures is indicated for moderately to
completely displaced fractures as well as frac-
tures with signicant angulation in adolescents
approaching skeletal maturity. Open reduction
and internal xation (ORIF) of humeral shaft
fractures with compression plating should
be performed for open fractures, fractures with
concomitant vascular injury, delayed union or
nonunion, and in cases when radial nerve explo-
ration is justied.
Surgical Procedure
Preoperative Planning
See Tables 2 and 3
Positioning
Patients are positioned on a standard operating
room table in either the lateral decubitus position
Table 2 Intramedullary nailing of humeral shaft frac-
tures: Preoperative planning
OR table: Radiolucent OR table
Position/positioning aids: Supine or beach chair position
with a small bump placed under the distal humerus
Fluoroscopy location: Ipsilateral side
Equipment: Flexible titanium or stainless steel
intramedullary nails
Tourniquet (sterile/nonsterile): Sterile
Table 3 Compression plating of humeral shaft fractures:
Preoperative planning
OR table: Standard OR table
Position/positioning aids: Lateral decubitus utilizing an
arm holder or supine with the arm placed over the
patients chest
Fluoroscopy location: Ipsilateral side
Equipment: Large fragment bone set, 3.5/4.5 mm
compression plates
Tourniquet (sterile/nonsterile): Sterile may be utilized
depending on fracture location
1251
with an arm holder utilized to support the humerus
or the supine position with the arm bent across the
patients chest. When performing intramedullary
nailing, the patient may alternatively be placed in
the beach chair position.
Surgical Approaches
The choice of surgical approach for ORIF of the
humeral shaft is typically determined by location
of the fracture as well as any concomitant soft
tissue or neurovascular injuries. An anterolateral
approach is the most commonly utilized approach
for proximal and middle third humeral shaft frac-
tures. Posterior approaches are also frequently
performed, and a modied posterolateral
approach can be utilized if a radial nerve palsy is
suspected, as the radial nerve can be completely
visualized on both sides of the intermuscular sep-
tum with this approach (Mills et al. 1996; Gerwin
et al. 1996; Zlotolow et al. 2006; Carlan
et al. 2007). However, in cases of vascular com-
promise, an extensile medial approach is utilized
in order to expose the brachial artery and vein as
well as permit access to the humerus.
The anterolateral approach is a distal extension
of the deltopectoral approach. Due to the fact that
both the radial and musculocutaneous nerves
innervate the brachialis, it is difcult to determine
a true internervous plane distally in this approach.
Therefore, the brachialis muscle is divided along
the middle and lateral thirds of the muscle belly.
The radial nerve is protected from damage by the
lateral segment of the brachialis when retraction
is performed within the split muscle belly.
The lateral antebrachial cutaneous nerve must be
carefully protected, as it exits between the biceps
and brachialis muscles. The radial nerve is also at
risk of injury with distal extension as it lies
between the brachialis and brachioradialis
(Zlotolow et al. 2006).
In the posterior approach to the humeral shaft,
the triceps must be either mobilized from lateral to
medial (paratricipital approach) or split along its
bers. The paratricipital approach is often favored
as it can be performed without the use of a
tourniquet due to lack of blood loss when dividing
the planes. Additionally, this approach is a
triceps-sparing procedure, which may limit scar
1252
formation and adhesions and may theoretically
lead to improved triceps function postoperatively.
Moreover, the risk of triceps denervation is
decreased with this approach. In the lateral
paratricipital approach, the tissue plane between
the lateral head of the triceps and the lateral
intermuscular septum is utilized. The radial
nerve is identied approximately 14 cm proximal
to the lateral epicondyle as it exits the spiral
groove. The nerve then penetrates the
intermuscular septum approximately 10 cm from
the articular surface. The posterior antebrachial
cutaneous nerve arises from the radial nerve as it
exits the spiral groove and courses along the pos-
terior edge of the lateral intermuscular septum. As
the radial nerve is identied and mobilized from
the spiral groove, care must be taken to protect the
posterior antebrachial cutaneous nerve. Following
identication and isolation of the radial nerve, the
triceps is elevated subperiosteally and reected
medially. In order to visualize a larger portion of
the humeral shaft, the incision may be extended
proximally between the posterior deltoid and the
lateral head of the triceps. However, extension is
limited by the position of the axillary nerve
(Zlotolow et al. 2006). Approximately 94 % of
the humeral shaft can be exposed utilizing this
approach (Gerwin et al. 1996).
The posterior triceps-splitting approach
involves the supercial division of the long head
of the triceps from the lateral head in order to
visualize the deeper medial head, where it origi-
nates from the spiral groove. The radial nerve is
identied and may be mobilized so that a plate can
be inserted beneath it. Mobilization of the radial
nerve allows for exposure of the distal 76 % of the
humeral diaphysis, as compared to 55 % exposure
of the humeral shaft without mobilization (Gerwin
et al. 1996). In this approach, plating may be
limited distally by violation of the olecranon
fossa by the plate. Unlike the paratricipital
approach, a tourniquet should be utilized for the
initial exposure, as signicant bleeding may occur
(Zlotolow et al. 2006).
Technique
Intramedullary xation utilizing exible titanium
or stainless steel nails can be performed to
B.S. Schwartz and J.M. Abzug
stabilize diaphyseal fractures of the humerus
(Fig. 8) (Ligier et al. 1984; Hall and Pankovich
1987) (Table 4). The patient is positioned in the
supine or beach chair position on a radiolucent
operating room table, and closed fracture reduc-
tion is performed prior to exposure. Once an
acceptable alignment has been achieved, a longi-
tudinal incision is made along the lateral column
of the distal humerus at the superior aspect of the
olecranon fossa. The underlying soft tissue is
bluntly dissected down to the level of the
humerus. While protecting the soft tissue, a corti-
cal window is created in the lateral column with a
3.2 or 4.5 mm drill bit, depending on the size of
the nail being inserted. The starting hole should be
formed obliquely from distal-lateral to proximal-
medial so that the nails can be passed effectively.
Flexible titanium nails of the appropriate size
(2.54 mm in diameter) are bent prior to passage.
One nail should be bent in the shape of a gentle
C, while the other is bent into a lazy S in order
to create divergence of the ends of the nail in the
proximal fragment. The nails are passed retro-
grade through the intramedullary canal of the
humerus, traversing the fracture site, and into
the proximal fracture fragment. In order to engage
the nails into the proximal humerus, they must be
impacted carefully to avoid distraction of the pre-
viously reduced fracture. Once the proximal frag-
ment is engaged, the nails can be manipulated to
correct any translation or angulation about the
fracture site. Fracture alignment, nail placement,
and stability are evaluated with intraoperative
uoroscopy. Following assessment of nail place-
ment and fracture reduction, the nails are cut
beneath the skin and the distal ends are left prom-
inent to permit future removal. The wound is
meticulously closed, and the upper extremity is
immobilized with a sling and swathe.
Compression plating is utilized when open
reduction and internal xation of a diaphyseal
humerus fracture is performed (Fig. 9) (Table 5).
Several approaches can be utilized, and the choice
of approach is based on fracture location, concom-
itant radial nerve injury, and surgeon preference.
One common technique is to use a lateral incision
longitudinally in order to create wide skin aps.
The triceps fascia is divided posterior to the lateral
56 Humeral Shaft Fractures
Fig. 8 Radiographs demonstrating xation utilizing
intramedullary elastic nailing and subsequent healing cal-
lous formation in a 16-year-old girl. (a) Preoperative
anteroposterior radiograph (b) Preoperative lateral (c)
intermuscular septum. The triceps is subsequently
swept posteriorly and medially away from the
septum, from distal to proximal. The radial nerve
is then identied, freed, and protected. To locate
the radial nerve, it may be necessary to identify
the posterior antebrachial cutaneous nerve at the
junction of the distal third and proximal
two-thirds of the humerus and trace it proximally.
Alternatively, the radial nerve may be located as it
1253
Immediate postoperative anteroposterior view (d) Imme-
diate postoperative lateral. Six weeks postoperative (e) AP
and (f) lateral (Courtesy of Joshua M. Abzug, MD)
crosses the lateral intermuscular septum from the
posterior to anterior compartment. In general, the
radial nerve is located directly posterior to the
humeral diaphysis at the level of the deltoid inser-
tion (Carlan et al. 2007). Following identication
and protection of the radial nerve, the periosteum
of the humerus is divided in order to visualize the
fracture site. The fracture is reduced and plate
xation is performed utilizing standard
1254
Table 4 Intramedullary nailing of humeral shaft fractures:
Surgical steps
Closed reduction is performed
Longitudinal incision along the lateral column of the
distal humerus
Blunt dissection of soft tissues to the level of the humerus
Drill a cortical window in the lateral column with a 3.2 or
4.5 mm drill bit depending on the size of the nails being
inserted
Pre-bend appropriately sized exible titanium nails into
C and S shapes
Pass nails into intramedullary canal and across the
fracture site into the proximal fracture fragment
Fluoroscopy performed to conrm appropriate nail
placement
Nails are cut beneath the skin with prominent distal ends
Wound closure and dressing
Immobilize upper extremity with a sling and swathe
compression plating techniques. A 3.5 or 4.5 mm
plate can be utilized depending on the size and age
of the child. The periosteum and wound are closed
in layers, and the extremity is placed in posterior
long arm splint.
Treatment-Specific Outcomes
Outcomes of humeral shaft fractures treated with
intramedullary nailing are generally excellent.
Union rates achieved with intramedullary nailing
have been reported to range from 87 % to 97 % in
all ages (Changulani et al. 2007; Rommens
et al. 2008). Nailing in an antegrade fashion has
been associated with decreased shoulder function
secondary to nail impingement, while retrograde
nailing signicantly decreases the risk of poor
shoulder functional outcome (Changulani
et al. 2007). Fernandez and colleagues reported on
31 children at an average age of 11.4 years who
underwent elastic stable intramedullary nailing
(ESIN) of humeral diaphyseal fractures. Following
an average follow-up period of 32 months, all
patients scored 100 points on the Constant-Murley
scale and were able to return to sports activities.
Five complications were observed, although all
were related to technical error or poor surgical
indications. These included postoperative damage
to the radial nerve in a patient with a secondary
fracture-dislocation, one case of skin irritation, one
B.S. Schwartz and J.M. Abzug
case of fracture-dislocation with axial deviation,
one case of a secondary dislocation of the nails
following a subsequent injury, and one secondary
axial deviation (Fernandez et al. 2010).
The outcomes of intramedullary nailing com-
pared with compression plating are controversial
in the literature. In a study of 30 patients with
closed humeral shaft fractures, Zatti et al. treated
14 with retrograde intramedullary elastic nailing
and 16 with open reduction and plating. The
authors reported similar outcomes regarding frac-
ture healing time (mean of 11 weeks in both
groups) and functional recovery between the two
treatment groups. Complications in the plate xa-
tion group included ve cases of radial nerve
injury, one case of pseudoarthrosis, one case of
supercial infection, and a painful, hypertrophic
scar in one patient (Zatti et al. 1998). In a study by
Changulani et al., it was reported that the rate of
nerve injury across all ages was nearly identical in
patients treated with intramedullary nailing or
compression plating. One patient out of 23 in the
nailing group sustained an axillary nerve injury,
while a single patient out of 24 in the plating
group experienced a radial nerve injury. However,
it was noted that the rate of infection was report-
edly four times higher with plating than with
nailing (Changulani et al. 2007). Conversely, in
a recent meta-analysis, Heineman et al. reported a
lower risk of complication with ORIF than with
intramedullary nailing across all ages (Heineman
et al. 2012).
Preferred Treatment
Immobilization is the preferred treatment choice
for fractures of the humeral diaphysis in children
and adolescents. The majority of these fractures
can be treated nonoperatively with excellent out-
comes. Neonates with humeral shaft fractures are
treated with immobilization utilizing a swathe
technique for 34 weeks, regardless of the radio-
graphic appearance or amount of angulation.
Older children with nondisplaced or minimally
displaced fractures may also be managed with
immobilization via a sling and swathe or shoulder
immobilizer. Moderately displaced or completely
56 Humeral Shaft Fractures
Fig. 9 Radiographs demonstrating a humeral diaphyseal
fracture managed with open reduction and internal xation
utilizing compression plating in a 17-year-old boy. (a)
Preoperative anteroposterior radiograph (b) Preoperative
displaced fractures should be managed with a
hanging long arm cast.
In cases of clinical deformity, a hanging long
arm cast is applied. Weekly radiographs should be
obtained for the initial 3 weeks, followed by ini-
tiation of shoulder range of motion exercises.
Alternatively, a closed reduction may be
performed, followed by immobilization in a coap-
tation splint for 2 weeks.
1255
lateral (c) Immediate postoperative anteroposterior view
(d) Immediate postoperative lateral. Eight weeks postop-
erative (e) AP and (f) lateral (Courtesy of Joshua
M. Abzug, MD)
Open fractures, fractures with an unacceptable
amount of angulation, and fractures with possible
radial nerve injuries should be managed opera-
tively. Intramedullary xation utilizing exible
titanium or stainless steel nails is the preferred
surgical treatment, as this procedure reportedly
has similar outcomes, has a lower complication
rate, and is less technically demanding than open
reduction with plate xation. However, when a
1256
Table 5 Compression plating of humeral shaft fractures:
Surgical steps
Choose surgical approach based on location of fracture,
concomitant radial nerve injury, and surgeon preference
Lateral paratricipital approach involves a longitudinal
lateral incision, creating wide skin aps
The triceps fascia is divided posterior to the lateral
intermuscular septum
The radial nerve is identied and freed while care is taken
to protect the nerve
The triceps is elevated subperiosteally and reected
medially
The periosteum is divided over the humeral diaphysis and
the fracture site is visualized and exposed
Fracture reduction and plate xation are performed
Close periosteum
Layered wound closure
Apply posterior long arm splint
Table 6 Humeral shaft fractures: Potential pitfalls and
preventions
Potential pitfall Pearls for prevention
Radial nerve Identify and protect the radial
injury nerve
fracture necessitates vascular repair or exploration
of the radial nerve, plate xation is performed.
Additionally, in cases of an open fracture, plate
xation is also performed.
Surgical Pitfalls and Prevention
Table 6 shows the potential pitfalls and preven-
tions inolved in humeral shaft fractures.
Management of Complications
Table 7 shows the common complications
involved in humeral shaft fractures and ways to
manage them.
Summary and Future Research
Fractures of the humeral shaft in children
and adolescents can occur by various mecha-
nisms depending on the age of the patient.
B.S. Schwartz and J.M. Abzug
Table 7 Humeral shaft fractures: Management of
complications
Radial nerve palsy Observation
Hardware prominence Removal of hardware
Delayed union/malunion Observation
Limb length discrepancy Observation
Compartment syndrome Decompressive fasciotomy
Neonates most commonly sustain humeral
diaphyseal fractures during delivery and are
always managed conservatively with a swathe.
Humeral shaft fractures observed in children
under 3 years of age should prompt the
healthcare professional to investigate the possi-
bility of non-accidental trauma/child abuse,
especially in the case of additional fractures or
soft tissue injuries. Other common mechanisms
of injury include a fall on an outstretched hand
and direct trauma, frequently from motor vehicle
collisions.
The vast majority of humeral shaft fractures in
children and adolescents can be managed
nonoperatively utilizing various immobilization
techniques. Younger patients exhibit remarkable
remodeling potential and frequently heal sponta-
neously with conservative treatment. Closed
reduction maneuvers followed by immobiliza-
tion can be utilized in older children who exhibit
a clinical deformity. In cases of severe displace-
ment and angulation, surgical management with
open reduction and internal xation may be jus-
tied. The specic procedure and approach are
typically chosen based on the location of the
fracture and any concomitant neurovascular
injury. Surgical options include plate xation or
intramedullary elastic nailing. Reported out-
comes are similar for both procedures; however,
complication rates are lower with intramedullary
nailing techniques.
Future research is needed to investigate the
outcomes of both nonoperative and surgical man-
agement of humeral shaft fractures in children and
adolescents. While the literature on adults can be
extrapolated to children, these younger patients
exhibit different physiology and may ultimately
show alternate outcomes.
56 Humeral Shaft Fractures
References
Amillo S, Barrios RH, Martinez-Peric R, et al. Surgical treat-
ment of the radial nerve lesions associated with fractures
of the humerus. J Orthop Trauma. 1993;7:2115.
Bachman D, Santora S. Orthopedic trauma. In: Fleisher
GR, Ludwig S, editors. Textbook of pediatric emer-
gency medicine. 6th ed. Philadelphia: Lippincott Wil-
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Beaty JH. Fractures of the proximal humerus and shaft in
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1257
Koch PP, Gross DF, Gerber C. The results of functional
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1109.
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Ligier JN, Metaizeau JP, Prevot J. Closed exible medul-
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the humeral shaft for acute fractures with and
without radial nerve injuries. J Orthop Trauma.
1992;6:103.
 Clavicle Fractures
57
Brandon S. Schwartz, Raymond Pensy, W. Andrew Eglseder,
and Joshua M. Abzug

Contents Introduction to Distal Clavicle Fractures . . . . . . . 1268

Introduction to Midshaft Clavicle Fractures . . . . 1260 Pathoanatomy and Applied Anatomy
Relating to Distal Clavicle Fractures . . . . . . . . . . . . 1269
Pathoanatomy and Applied Anatomy Relating
to Midshaft Clavicle Fractures . . . . . . . . . . . . . . . . . . . 1260 Assessment of Distal Clavicle Fractures . . . . . . . . . 1269
Signs and Symptoms of Distal Clavicle
Assessment of Midshaft Clavicle Fractures . . . . . 1260 Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1269
Signs and Symptoms of Midshaft Distal Clavicle Fracture Imaging and
Clavicle Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1260 Other Diagnostic Studies . . . . . . . . . . . . . . . . . . . . . . . . . . . 1269
Midshaft Clavicle Fracture Imaging and Other Injuries Associated with Distal Clavicle
Diagnostic Studies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1261 Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1269
Injuries Associated with Midshaft Distal Clavicle Fracture Classication . . . . . . . . . . . . . 1269
Clavicle Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1262 Distal Clavicle Fracture Outcome Tools . . . . . . . . . . . 1270
Midshaft Clavicle Fracture Classication . . . . . . . . . . 1262
Midshaft Clavicle Fracture Outcome Tools . . . . . . . . 1262 Distal Clavicle Fracture Treatment Options . . . . 1270
Nonoperative Treatment of Distal
Midshaft Clavicle Fracture Treatment Clavicle Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1270
Options . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1262 Operative Treatment of Distal Clavicle
Nonoperative Treatment of Midshaft Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1271
Clavicle Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1262 Surgical Procedure . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1271
Operative Treatment of Midshaft
Clavicle Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1264 Preferred Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1273
Surgical Procedure . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1264 Surgical Pitfalls and Prevention . . . . . . . . . . . . . . . . . . . . 1274
Management of Complications . . . . . . . . . . . . . . . . . . . . . 1274
Preferred Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1267
Surgical Pitfalls and Prevention . . . . . . . . . . . . . . . . . . . . 1267 Summary and Future Research . . . . . . . . . . . . . . . . . . 1274
Management of Complications . . . . . . . . . . . . . . . . . . . . . 1268
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1275
Summary and Future Research . . . . . . . . . . . . . . . . . . . . . 1268

B.S. Schwartz (*) R. Pensy W.A. Eglseder

J.M. Abzug
University of Maryland School of Medicine, Baltimore,
MD, USA
e-mail: brandon.schwartz@som.umaryland.edu;
rpensy@umoa.umm.edu; aeglseder@umoa.umm.edu;
jabzug@umoa.umm.edu

# Springer Science+Business Media New York 2015 1259

J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_57
1260
Abstract
Clavicle fractures are common in the pediatric
and adolescent populations. This chapter exam-
ines the epidemiology, pathoanatomy, assess-
ment, diagnosis, and treatment options of
midshaft and distal clavicle fractures. Despite
the high incidence of these injuries, outcomes in
children have not been extensively studied.
Data from the limited number of pediatric stud-
ies as well as data extrapolated from the adult
literature will be discussed. The nonoperative
and operative management options are
presented from technique to outcome, along
with their associated complications.
Introduction to Midshaft Clavicle
Fractures
Clavicle fractures are common pediatric injuries,
accounting for 515 % of all fractures in children.
The most common site of fracture is at the
midshaft of the bone, accounting for up to 80 %
of cases. Despite this high incidence, little litera-
ture exists pertaining to the management and out-
comes of pediatric clavicle fractures. Therefore,
much of the information from this chapter has
been extrapolated from studies regarding either
adult or adolescent clavicle fractures. With the
rising trend of operative xation in adults, further
research regarding the management of pediatric
clavicle fractures is being performed.
Pathoanatomy and Applied Anatomy
Relating to Midshaft Clavicle Fractures
The clavicle, or collarbone, is an S-shaped bone
positioned along the subcutaneous border of the
anterior aspect of the shoulder girdle. Medially,
there is an anterior convexity that allows the pas-
sage of the brachial plexus and axillary vessels
from the neck region into the upper arm, while
laterally, there is an anterior concavity.
Development of the clavicle begins via
intramembranous ossication at ve and a half
weeks gestation, with the S-shaped conguration
present by 8 weeks (Gardner 1968). Postnatally,
B.S. Schwartz et al.
the clavicle maintains growth at a rate of approx-
imately 8.4 mm per year until age 12. In the
subsequent years, the clavicle grows approxi-
mately 2.6 mm per year in females and 5.4 mm
per year in males. Therefore, 80 % of the nal
clavicular length is achieved by age 9 in females
and age 12 in males (McGraw et al. 2009).
The clavicle articulates with the sternum medi-
ally, forming the sternoclavicular joint, while lat-
erally, the bone articulates with the acromion,
forming the acromioclavicular joint. Several sites
of ligamentous attachment occur on the inferior
aspect of the clavicle. Medially lies the site of
attachment of the costoclavicular ligament, while
laterally, the conoid tubercle and trapezoid line are
present, which are the sites of attachment for the
conoid and trapezoid ligaments, respectively.
Because these ligaments angle posteriorly as
they approach the clavicle, the ligaments are put
on stretch when the clavicle elevates and when it
rotates posteriorly. Furthermore, since these liga-
ments offer substantial stability at both ends of the
clavicle, fractures of the middle third of the clav-
icle are more likely to occur.
The pectoralis major originates from the medial
aspect of the clavicle as well as from the sternum
and inserts onto the intertubercular groove of the
humerus, while the deltoid originates from the
lateral aspect of the clavicle, acromion, and
scapular spine and inserts onto the deltoid tuber-
osity of the humerus. The sternocleidomastoid and
sternohyoid muscles also originate from the
clavicle, while the trapezius and subclavius
muscles insert onto the clavicle.
Assessment of Midshaft Clavicle
Fractures
Signs and Symptoms of Midshaft
Clavicle Fractures
Neonatal clavicle fractures commonly present
after difcult deliveries as decreased active move-
ment about the shoulder, crying upon passive
movement of the shoulder and/or entire upper
extremity, an asymmetrical bony contour,
edema, and/or crepitation (Fig. 1). The Moro
57 Clavicle Fractures
Fig. 1 Neonatal clavicle
fracture on the right side
sustained during a difcult
vaginal delivery (Courtesy
of Joshua M. Abzug, MD)
(startle) reex may be decreased as well. Presence
of limited digital motion or Horners syndrome
(ptosis, miosis, and anhydrosis) signies the exis-
tence of a more serious concomitant brachial
plexus birth palsy with an injury affecting the
lower portion of the brachial plexus.
Children who have clavicle fractures from
suspected abuse should undergo a comprehensive
head-to-toe survey as if he or she is a trauma
patient, so that any concomitant injuries and/or
signs of abuse may be detected. Included in this
survey are a thorough neurological evaluation,
ophthalmologic examination, and a skeletal sur-
vey to identify corner fractures or any additional
fractures in various phases of healing.
Physical examination of a child with a clavicle
fracture involves observing for swelling, defor-
mity, and ecchymosis around the affected clavicle.
Open wounds should be noted, as these signs may
dictate management. Furthermore, the lateral
aspect of the shoulder should be examined for
abrasions or erythema, as this is the most common
site of impact. Drooping of the involved side may
also be seen, as the scapula appears internally
rotated and the shoulder appears shortened com-
pared to the contralateral side. However, if signif-
icant swelling occurs, this may be difcult to
appreciate.
Clavicle fractures typically cause pain about
the entire shoulder girdle; however, signicant
tenderness to palpation is most prominent at the
fracture itself. Crepitus may also be present with
1261
any attempt of either active or passive range of
motion. An associated injury to the brachial
plexus may occur, with the lower trunk being
especially susceptible due to its location adjacent
to the middle third of the clavicle. Therefore, a
thorough neurological examination, including
assessment of motor and sensory function
throughout the entire upper extremity, is manda-
tory for all patients who sustain clavicular frac-
tures. It may be challenging for a child in pain to
perform certain tasks required to complete the
neurological evaluation; however, it is crucial to
remain patient and repeat the examination as often
as necessary to acquire the necessary information.
A comprehensive vascular examination is also
necessary due to the location of the subclavian
vessels, particularly in patients who have under-
gone high-energy mechanisms of injury, as the
vessels may spasm or undergo thrombosis from
blunt trauma. Palpation of both radial arteries
should reveal symmetric pulses, and if there is
concern for vascular injury, further evaluation
with advanced imaging should be performed.
Midshaft Clavicle Fracture Imaging
and Other Diagnostic Studies
Initially, imaging of a suspected clavicle fracture
should include plain radiographs of the
clavicle in two projections, typically a standard
anteroposterior (AP) radiograph and an apical
1262
lordotic view or 45
cephalic tilt view. The apical
lordotic view is an AP view with the tube directed
45
cephalad and can be utilized to evaluate the
amount of displacement. With these views, one
can also visualize the shoulder girdle region as
well as the upper lung elds, both of which should
be assessed for additional injuries. If clinical sus-
picion for additional injuries is present, dedicated
series of the suspected region(s) should be
acquired. Advanced imaging is rarely needed to
evaluate clavicle fractures, as the fracture pattern,
displacement, and presence of comminution can
be easily assessed on plain lms. In the setting of
high-energy trauma, a chest CT is typically
obtained and can be utilized to further evaluate
the clavicle fracture.
Injuries Associated with Midshaft
Clavicle Fractures
Injuries associated with clavicle fractures vary
based on the age of the child. Brachial plexus
palsies can be associated with midshaft clavicle
fractures in neonates, most commonly affecting
the C5 and C6 nerve roots. This can result in
limited shoulder movement, elbow exion, fore-
arm supination, and wrist extension (Gilbert and
Whitaker 1991). Differentiation between a
pseudopalsy, the child not moving his or her arm
secondary to the clavicle fracture itself, and an
associated neonatal brachial plexus palsy can be
made by 34 weeks of age, when the pain from the
fracture will be markedly decreased due to early
callus formation. Children who sustain clavicle
fractures resulting from nonaccidental trauma are
likely to sustain additional injuries such as frac-
tures of the rib, tibia/bula, humerus, or femur,
intracranial bleeding, eye contusions, retinal hem-
orrhage, and/or burns (Chang et al. 2004; Pandya
et al. 2009). Adolescents with clavicle fractures
due to high-energy mechanisms of injury may
have associated polytrauma, including injury to
vital organs or adjacent structures. Concomitant
rib fractures, scapula fractures, pneumothorax, bra-
chial plexus injury, or subclavian vessel injury can
also occur (Jeray 2007). Abdominal, head, spine,
and/or lower extremity trauma may also be present.
B.S. Schwartz et al.
Midshaft Clavicle Fracture
Classification
Clavicle fractures are typically classied based on
fracture location, pattern, and the presence or
absence of displacement. Thus, clavicle fractures
are described as medial, midshaft, or lateral;
non-displaced or displaced; open or closed; and
comminuted or simple. Additional classication
schemes to assess adult clavicle fractures have
been proposed, but none are extensively utilized,
as their nomenclature is either purely descriptive
of fracture location or cumbersome with several
types and subtypes (Allman 1967; Orthopedic
Trauma Association 1996; Robinson 1998).
Midshaft Clavicle Fracture Outcome
Tools
There are currently no outcome scores specically
utilized to evaluate results following pediatric
clavicle fractures. Outcome measures such as
patient satisfaction, range of motion, pain, frac-
ture union, and complications are commonly
employed. Additional measures including the
Disability of the Arm, Shoulder, and Hand
(DASH) score, QuickDASH, Simple Shoulder
Test, and Constant Shoulder Score have been
used to assess outcomes. Radiographic criteria
evaluating shortening and/or vertical displace-
ment of the clavicle have also been utilized to
evaluate outcomes.
Midshaft Clavicle Fracture Treatment
Options
Nonoperative Treatment of Midshaft
Clavicle Fractures
Indications/Contraindications
The established treatment of pediatric and adoles-
cent clavicle fractures is nonoperative, allowing
the fracture to form callous and heal in situ,
regardless of whether there is signicant displace-
ment (Fig. 2) (Bae et al. 2013). Well-established
indications for nonoperative management include
57 Clavicle Fractures
Fig. 2 Healed neonatal clavicle fracture demonstrating
the abundance of callus formation that occurs (Courtesy
of Joshua M. Abzug, MD)
Table 1 Midshaft clavicle fractures: Nonoperative
management
Indications Contraindications
Non-displaced fractures Open fractures
Minimally displaced Fractures associated with
fractures (<1.52 cm) neurovascular injury
non-displaced or minimally displaced fractures,
dened as displacement less than 1.52
cm. Conversely, open fractures and fractures
with associated neurovascular injury or limb at
risk should all proceed directly to operative inter-
vention (Table 1).
Techniques
Nonoperative treatment of clavicle fractures is
achieved by immobilizing the childs shoulder
girdle, classically with a sling. A gure of eight
dressing or shoulder immobilizer can alternatively
be used. Immobilization in neonates who have
sustained a clavicle fracture during the birthing
process can be achieved by performing a swathe
technique, such as placing cast padding followed
by an ACE bandage around the torso and arm.
Follow-up radiographs are obtained at 4-week
intervals until fracture union is achieved. The
child is allowed to resume normal activities as
tolerated once union is accomplished. Calder
et al. have proposed that follow-up radiographs
are unnecessary in the pediatric population due to
the near universal fracture-healing rate of children
1263
(Calder et al. 2002). However, in children wishing
to return to sports, radiographs should be obtained
until union is established in order to decrease the
refracture risk.
Outcomes
Even though pediatric clavicle fractures have a
relatively high incidence and the majority of pedi-
atric clavicle fractures are treated nonoperatively,
there is little data regarding the outcomes of these
injuries. Union rates from 95 % to 100 % have
been reported with nonoperative treatment (Grassi
et al. 2001; Khan et al. 2009; Vander Have
et al. 2010). Most non-displaced fractures unite
by 48 weeks post-injury, while displaced frac-
tures achieve union in approximately 10 weeks
(Vander Have et al. 2010).
The majority of patients obtain excellent out-
comes and are capable of returning to their activ-
ities without restrictions. A small percentage of
patients with signicant fracture displacement
who are treated nonoperatively may have subjec-
tive complaints of pain with prolonged activity,
easy fatigability, axillary pain, or drooping shoul-
ders with bony prominence (Vander Have
et al. 2010). Bae et al. evaluated 16 patients with
displaced (>2 cm) mid-diaphyseal clavicle frac-
tures treated nonoperatively. All fractures
achieved union with no meaningful loss of shoul-
der motion or abduction-adduction strength by
isokinetic testing. The majority of patients had
low DASH and pain Visual Analog Scores
(VAS) that were very low, with means of 4.9 and
1.6, respectively. Only one patient required a cor-
rective osteotomy (Bae et al. 2013). The authors
concluded that routine surgical xation for
displaced, nonsegmental clavicle fractures may
not be justied based on concerns regarding
shoulder motion and strength alone. Further
investigations are needed to determine the risk
factors and causes of pain and functional compro-
mise in the minority of pediatric patients with
symptomatic malunions. In contrast, adult studies
have shown that patients with signicantly
displaced midshaft fractures that are treated
nonoperatively have signicantly worse Constant
Shoulder Scores and DASH scores, higher rates of
nonunion, longer times to union, and more
1264
Fig. 3 Displaced right
clavicle fracture with a
central zed-type
fragment. (a). Preoperative.
(b). Postoperative
(Courtesy of Joshua
M. Abzug, MD)
symptomatic malunions than those who have
undergone plate xation (Canadian Orthopedic
Trauma Society 2007).
Operative Treatment of Midshaft
Clavicle Fractures
Indications/Contraindications
Absolute indications for operative management of
pediatric and adolescent clavicle fractures include
open fractures, comminuted fractures in which the
central fragment is markedly displaced (Fig. 3),
and fractures associated with neurovascular
injury. Floating shoulder injuries and fractures
associated with polytrauma are considered rela-
tive indications for surgical treatment by some
authors. A oating shoulder injury created by a
midshaft clavicle fracture and glenoid neck frac-
ture may be managed by open reduction and inter-
nal xation (ORIF) of the clavicle exclusively, as
ligamentotaxis via the coracoclavicular ligament
will reduce the accompanying glenoid fracture
(Bahk et al. 2009).
Signicantly displaced fractures in adults that
are managed nonoperatively have demonstrated
healing with a malunion that can trigger changes
in shoulder mechanics, including pain with over-
head activities, decreased strength, and decreased
endurance (Hill et al. 1997; McKee et al. 2003).
The benet of operative xation versus
B.S. Schwartz et al.
nonoperative treatment of displaced midshaft
clavicle fractures has been the subject of several
studies. One recent meta-analysis of randomized
clinical trials comparing operative and
nonoperative treatment in adults revealed a signif-
icantly higher nonunion and symptomatic
malunion rate in the nonoperative group. Further-
more, patients managed with operative interven-
tions demonstrated earlier functional return, but it
is unclear whether this data is applicable to ado-
lescents (McKee et al. 2012). It is clear, however,
that young children, especially younger than age
8, have the potential to remodel foreshortened,
displaced fractures.
Surgical Procedure
Preoperative Planning
Similar to any procedure that utilizes medical
implants, it is essential to have the necessary
hardware available prior to proceeding to the
operating room (Table 2). Several options exist
for treating pediatric and adolescent clavicle frac-
tures, including anatomically designed clavicle
plates, standard non-locking and locking plates,
and intramedullary devices such as pins, wires,
screws, and elastic nails.
Plate xation requires one to determine if the
location of the plate will be anteroinferior or supe-
rior. The benet of using anteroinferior plates is
57 Clavicle Fractures
Table 2 ORIF of midshaft clavicle fractures: Preoperative
planning
OR table: standard OR table capable of beach chair
positioning or utilization of a Mayeld head positioner
Position/positioning aids: supine beach chair or Mayeld
headrest position with the head/neck tilted away
Bump placed behind the scapula
Fluoroscopy location: contralateral to fracture
Equipment: surgeons choice of implant
Tourniquet (sterile/nonsterile): none
Draping: shoulder girdle, entire clavicle, and ipsilateral
limb and prepped and draped into the eld to allow for
visualization, traction, and manipulation
Medially, the contralateral sternoclavicular joint should
be included in the operative eld
Fig. 4 Midshaft clavicle fracture treated with an
intramedullary device (Courtesy of Dan A. Zlotolow, MD)
the ability to drill in a posterosuperior direction so
that the drill is not directed toward the surround-
ing neurovascular structures. Moreover, the plate
is less prominent in this location. Superior place-
ment of the plate is technically easier and provides
strong resistance of the biomechanical forces act-
ing to displace the fracture.
Compared to plate xation, intramedullary x-
ation provides the potential benets of requiring
less soft tissue stripping at the fracture site,
smaller skin incisions leading to better cosmesis,
decreased risk of hardware irritation, easier hard-
ware removal, and less bony weakness after plate
removal (Fig. 4). However, intramedullary xa-
tion provides inferior resistance to torsional forces
when compared to plating, which can result in
fracture of the intramedullary implant.
1265
Additionally, the intramedullary device has the
potential to migrate, which raises major concerns
and has thus limited usage of these devices.
Positioning
Positioning options during ORIF or
intramedullary xation of clavicle fractures
include the beach chair position with or without
a Mayeld head positioner or having the patient
supine. With either position, a bump is placed
behind the scapula to assist in reducing the
fracture.
Surgical Approaches
ORIF is performed via a direct approach to the
clavicle by creating a skin incision that follows
Langers lines (Table 3). Skin incisions placed in
locations not directly overlying the planned plate
location on the clavicle can be done in order to
avoid wound complications and to improve
cosmesis (Coupe et al. 2005). Following the inci-
sion, electrocautery is utilized to divide the
platysma, fascia, and periosteum in line with the
initial skin incision. Throughout this process, it is
imperative to identify and protect the cutaneous
supraclavicular nerves which may number as
many as 34. Subperiosteal dissection is then
performed to expose the fracture site while ensur-
ing that soft tissue attachments to any malrotated
or segmental fracture fragments are preserved.
Intramedullary xation is accomplished via an
approach that utilizes a small incision over the
fracture site to expose only the ends of fracture
fragments (Table 4). In order to place the
intramedullary device in an antegrade manner,
an additional percutaneous incision is positioned
over the superolateral region of the clavicle.
Technique
Once the fracture site and fragments are exposed,
the fracture is reduced using bone-holding for-
ceps. A separate interfragmentary screw or mini-
fragment plate xation can be used in segmental
fractures to reduce the fracture from three parts to
two. The fracture is then anatomically reduced
and clamped, ensuring that areas of comminution
are accounted for. Either an anatomic clavicular
1266
Table 3 ORIF of midshaft clavicle fractures: Surgical
steps
Skin incision approximately 1 cm inferior to the clavicle
in line with Langers lines
Electrocautery through the platysma, fascia, and
periosteum directly onto the clavicle while avoiding
injury to the supraclavicular cutaneous nerves
Expose fracture site in a subperiosteal manner while
preserving soft tissue attachments to malrotated and
comminuted fragments
Reduce fracture fragments utilizing bone-holding forceps
Apply plate in desired location
Assess reduction and screw lengths with direct
visualization and uoroscopic imaging in multiple planes
Close periosteum
Meticulous skin closure with absorbable suture
Apply sling or shoulder immobilizer
Table 4 Intramedullary xation of midshaft clavicle frac-
tures: Surgical steps
Skin incision overlying the fracture site in line with
Langers lines
Electrocautery through the fascia and periosteum directly
onto the clavicle while avoiding injury to the
supraclavicular cutaneous nerves
Expose fracture site in a subperiosteal manner while
preserving soft tissue attachments to malrotated and
comminuted fragments
Drill the medial segment of the fracture in preparation for
device placement while ensuring no violation of the
anterior medial cortex occurs
Drill distal fragment medullary canal and exit out
posterior lateral cortex so that the drill can be visualized
just beneath the skin
Make a percutaneous skin incision where the drill is
tenting the skin
Place intramedullary device in a retrograde manner
through fracture site to exit through posterior lateral skin
incision
Reduce fracture fragments
Advance device antegrade across the fracture
Utilize device-specic mechanisms, if available, to
prevent migration or permit compression
Close periosteum
Meticulous skin closure with absorbable suture
Apply sling or shoulder immobilizer
plate or a small pelvic reconstruction plate is then
contoured to permit rigid internal xation. The
plate is subsequently applied in the preferred posi-
tion, and the reduction, screw placement, and
B.S. Schwartz et al.
length are assessed with a combination of direct
visualization and uoroscopic imaging in multiple
planes. Subsequently, the supraclavicular nerves
are protected and the periosteum is closed. Layered
closure with absorbable suture, including meticu-
lous skin closure, is then performed to decrease the
risk of wound complications and allow for the best
possible cosmesis. Lastly, the patient is placed in a
sling or shoulder immobilizer.
Intramedullary xation is performed by expos-
ing the fracture ends and then drilling the distal
fragment in a retrograde direction through the
intramedullary canal to exit the posterior-lateral
cortex. The medial segment is then drilled while
ensuring that there is no violation of the anterior
medial cortex. The device is then placed retro-
grade through the canal to exit the posterior-lateral
hole followed by the skin. Subsequently, the frac-
ture is reduced and the intramedullary device is
advanced antegrade across the fracture site. Sev-
eral devices have mechanisms that may now be
employed in order to prevent migration of the
device and to permit fracture compression.
Treatment-Specific Outcomes
The majority of studies regarding the manage-
ment of pediatric and adolescent midshaft
clavicle fractures are retrospective and involve
preadolescents and adolescents. Mehlman
et al. retrospectively reviewed 24 children, with
a mean age of 12 years, who underwent operative
treatment of completely displaced clavicle shaft
fractures. This series reported zero nonunions or
infections, and 21 of the 24 patients were able to
return to unrestricted sports activity. Three com-
plications were reported, including two patients
with scar sensitivity and one patient who devel-
oped a transient ulnar nerve neurapraxia. Hard-
ware removal was performed on an elective basis
for all patients (Mehlman et al. 2009).
Namdari et al. also performed a retrospective
review of 14 skeletally immature patients who
underwent ORIF for displaced midshaft clavicle
fractures. There were no nonunions reported, but
eight patients had numbness about the surgical
site. Four patients required hardware removal
(Namdari et al. 2011).
57 Clavicle Fractures
The only comparative study to date evaluating
nonoperative versus operative management of
midshaft clavicle fractures in adolescents was
performed by Vander Have et al. In this retrospec-
tive review of 43 fractures, 25 were treated
nonoperatively and 17 were treated operatively.
Neither group reported a nonunion although
5 symptomatic malunions occurred in the
nonoperative group, 4 of which were managed
with corrective osteotomy. All complications
within the operative group were associated with
hardware prominence. Return to full activities
occurred faster in the operative group, by approx-
imately 4 weeks, when compared to the
nonoperative group (Vander Have et al. 2010).
Although the Vander Have study reported a
high rate (20 %) of symptomatic malunion in the
nonoperative group, with many patients requiring
corrective osteotomy, Bae et al. have recently
reported that the majority of signicantly displaced
(>2 cm) diaphyseal clavicle fractures treated
nonoperatively result in an asymptomatic
malunion that does not require corrective
osteotomy. Of the 16 fractures included, all
progressed to malunion with only 1 patient requir-
ing a corrective osteotomy. The mean DASH score
was low at 4.9 and the mean pain VAS was 1.6.
There was no signicant loss of strength or motion
reported (Bae et al. 2013; Vander Have et al. 2010).
Preferred Treatment
The vast majority of pediatric and adolescent
clavicle fractures are managed nonoperatively
with 34 weeks of immobilization. Schulz
et al. determined that nonoperative treatment of
adolescents with displaced and shortened
midshaft clavicle fractures is effective, with
good functional outcomes (Schulz et al. 2013).
Additionally, a long-term study by Randsborg
and colleagues demonstrated good to excellent
patient-reported outcomes in the majority of ado-
lescents following nonoperative treatment of clav-
icle fractures (Randsborg et al. 2013). The authors
of these studies concluded that nonoperative man-
agement of clavicle fractures in adolescents
1267
should remain the mainstay of treatment. Home
or formal rehabilitation is then performed to
restore range of motion and strength prior to
resuming full activities. Operative treatment is
performed for open fractures, fractures associated
with neurological or vascular injury, and signi-
cantly displaced fractures in athletes.
The beach chair position is utilized along with
a Mayeld head positioner with a skin incision
placed approximately 1 cm inferior to the clavicle.
Once the fracture fragments are exposed, the frac-
ture is reduced utilizing bone-holding forceps.
In comminuted fractures, mini-fragment plates
are placed to convert multiple small pieces into
large fragments. During the reduction process, it is
critical to restore the length and contour of
the clavicle, which is achieved by utilizing
smooth wires, suture, mini-fragment plates, or
interfragmentary screws. When the reduction is
anatomic, the plate is applied to the superior
aspect of the clavicle. A malleable retractor is
placed inferior to the clavicle in order to protect
the surrounding neurovascular structures. Follow-
ing plate placement, uoroscopic imaging and
direct visualization are utilized to assess the frac-
ture reduction and screw lengths. Thorough irri-
gation of the wound is then performed and the
periosteum is closed. The wound is then closed
with a meticulous subcuticular closure in order to
obtain the best cosmesis and decrease the chance
of wound complications. Sterile dressings includ-
ing a Tegaderm are applied, and the patient is
placed into either a sling or shoulder immobilizer.
Surgical Pitfalls and Prevention
The most dreaded complication during operative
treatment of clavicle fractures is damage to a
neurovascular structure and/or creation of a pneu-
mothorax. Utilizing meticulous technique during
fracture fragment exposure and while drilling/
placing screws during plate application can pre-
vent these extremely rare iatrogenic complica-
tions. It is critical to stay subperiosteal while
exposing fracture fragments in order to create a
layer between the bone and surrounding
1268
Table 5 Midshaft clavicle fractures: Potential pitfalls and
preventions
Potential pitfall Pearls for prevention
Neurovascular Utilize subperiosteal dissection
injury/
pneumothorax
Place retractors inferiorly when
drilling from superior to inferior
direction
Delayed union/ Maintain soft tissue attachments
nonunion to comminuted or malrotated
fragments
Malunion Maintain soft tissue attachments
to comminuted or malrotated
fragments
Anatomically reduce and
stabilize segmental fractures
Wound Use inferior skin incision
complications
Meticulous closure
structures. Retractors can then be positioned in
this layer, and direct visualization can be
employed during drilling and screw placement to
limit any chance of damage to the nearby
neurovascular structures.
The surgeon should take care to maintain soft
tissue attachments to comminuted or malrotated
fragments, as doing so will aid in the reduction
process. If the fragments are completely devoid of
soft tissue attachments, bony union may be
delayed or absent due to devitalization.
Utilizing the inferior skin incision rather than a
direct approach to the clavicle can prevent wound
complications. Furthermore, a meticulous closure
at the conclusion of the procedure will allow for
the best cosmesis while minimizing the risk of
wound complications (Table 5).
Management of Complications
Patients with hardware prominence can be suc-
cessfully treated with hardware removal
(Mehlman et al. 2009; Namdari et al. 2011; Vander
Have et al. 2010). In patients initially treated
nonoperatively who develop a symptomatic
malunion, corrective osteotomy has been shown
to successfully ameliorate symptoms. In the
B.S. Schwartz et al.
Table 6 Midshaft clavicle fractures: Management of
Complications
Hardware prominence Removal of hardware
Malunion Corrective osteotomy
Nonunion ORIF with stable construct
Vander Have study, all patients with a malunion
who underwent corrective osteotomy progressed
to union and reported resolution of their symptoms
(Vander Have et al. 2010). To date, only a single
nonunion has been reported in the pediatric and
adolescent literature (Nogi et al. 1975). Successful
treatment of nonunion can be performed by sub-
sequent ORIF with a stable construct. Rarely, a
vascularized bone graft or corticoperiosteal
ap may be needed (Table 6).
Summary and Future Research
Nonoperative management is the preferred treat-
ment for the vast majority of pediatric and adoles-
cent midshaft clavicle fractures. Complicated
fractures such as open fractures, fractures with
skin compromise, and fractures with associated
neurovascular injury should be treated by ORIF.
Furthermore, utilizing ORIF of signicantly
displaced (>2 cm) or segmental fractures has
been shown to provide a quicker return to activi-
ties in older patients. Prospective studies designed
to determine the potential benets and complica-
tions of operative xation versus nonoperative
management in adolescents should be performed.
Introduction to Distal Clavicle
Fractures
The distal clavicle is the second most common site
for a clavicle fracture, accounting for 1030 % of
all clavicle fractures. Currently, there are few
studies examining the management and outcomes
of these fractures in pediatric and adolescent
populations. Therefore, the information presented
here will largely be extrapolated from adult stud-
ies and from experience.
57 Clavicle Fractures
Pathoanatomy and Applied Anatomy
Relating to Distal Clavicle Fractures
Distally, the clavicle articulates with the scapula
via the acromioclavicular joint. The ligamentous
connections that span between the distal clavicle
and scapula include the acromioclavicular liga-
ments and the coracoclavicular ligaments, includ-
ing the trapezoid ligament and conoid ligament.
The trapezoid ligament is located more laterally
and attaches to the distal clavicle approximately
2 cm from the acromioclavicular joint, while the
conoid ligament is more medial with an attach-
ment to the distal clavicle approximately 4 cm
from the acromioclavicular joint (Renfree
et al. 2003). These ligamentous attachments in
association with the acromioclavicular joint cap-
sule allow uid scapulothoracic motion (Banerjee
et al. 2011). The acromioclavicular ligaments pro-
vide stability of the clavicle in the horizontal/
anteroposterior plane, while the coracoclavicular
ligaments provide stability in the vertical/
superoinferior plane (Fukada et al. 1986). This
stability permits the formation of the
coracoclavicular space, the space between the
coracoid process and the undersurface of the clav-
icle, which should be between 1.1 and 1.3 cm
(Bearden et al. 1973).
Assessment of Distal Clavicle Fractures
Signs and Symptoms of Distal Clavicle
Fractures
Patients with distal clavicle fractures typically
present with pain about the involved shoulder
with any attempt at arm movement. Symptoms
may include paresthesias if a concomitant brachial
plexus injury is present or if there is edema lead-
ing to injury of the supraclavicular nerves.
Observation for swelling and ecchymosis
should be the rst step in the physical examina-
tion. The entire upper extremity, hemithorax, and
cervical spine should be palpated to detect the site
of maximal tenderness. Additionally, a brief sec-
ondary survey of the remainder of the body should
1269
be performed to ensure there is no concurrent
injury. A comprehensive neurovascular examina-
tion should be performed to assess for any bra-
chial plexus injury, although this is rare. A
complete head-to-toe survey should be performed
by a member of the trauma team or the emergency
department physician in patients injured in high-
energy mechanisms.
Distal Clavicle Fracture Imaging and
Other Diagnostic Studies
Initial imaging utilizing plain radiographs of the
shoulder, including a true AP view and an axillary
lateral view, should be obtained. A Zanca view,
performed by aiming the x-ray beam in 1015
of
cephalic tilt, can also be obtained to better evalu-
ate the acromioclavicular joint for intra-articular
involvement (Zanca 1971). Alternatively, a CT
scan may more easily identify intra-articular frac-
tures, which may require operative intervention.
Injuries Associated with Distal Clavicle
Fractures
Floating shoulder-type injuries involving addi-
tional fractures about the shoulder girdle, includ-
ing proximal humerus and scapular fractures, are
commonly associated with distal clavicle frac-
tures. Associated rib fractures, lung injuries, and
brachial plexus injuries may also be present.
High-energy mechanisms of injury raise concerns
for cervical spine injuries, which must be
ruled out.
Distal Clavicle Fracture Classification
Distal clavicle fractures are most commonly clas-
sied using the scheme proposed by Neer and
modied by Craig (Neer 1984). In this scheme,
fractures are divided into ve types based on the
relationship of the fracture line to the
coracoclavicular ligaments, the acromioclavicular
ligaments, and the physis. The majority of distal
clavicle fractures in skeletally immature patients
1270
involve disruption of the periosteum, where the
bone is displaced from the periosteal sleeve while
the ligaments remain attached to the intact inferior
portion of the periosteum.
Type I fractures occur distal to the
coracoclavicular ligaments but do not involve
the acromioclavicular joint. In Type I fractures,
minimal displacement occurs due to stabilization
of the proximal fragment by the intact
coracoclavicular ligaments and stabilization of
the distal fragment by the acromioclavicular
joint capsule, the acromioclavicular ligaments,
and the deltotrapezial fascia.
Type II fractures are subdivided into Type IIA,
fractures medial to the coracoclavicular liga-
ments, and Type IIB, fractures between the
coracoclavicular ligaments with concomitant
injury to the conoid ligament. In Type IIA frac-
tures, the stability of the proximal fragment
provided by the coracoclavicular ligaments is lost,
and the fragment displaces superiorly from the
periosteal sleeve. The distal fragment, however,
remains stable due to the attachments of the
acromioclavicular joint capsule, acromioclavicular
ligaments, and coracoclavicular ligament(s). This
is true for Type IIB fractures as well, because the
trapezoid ligament remains attached even though
the conoid ligament is disrupted.
Type III fractures are located distal to the
coracoclavicular ligament and extend into the
acromioclavicular joint. Minimal displacement is
common since these fractures do not disturb the
ligamentous structures.
Type IV fractures are seen in skeletally imma-
ture patients and occur medial to the physis.
Although the physis and epiphysis remain
uninjured and attached to the acromioclavicular
joint, signicant displacement between the physis
and metaphyseal fragment can occur, as the
coracoclavicular ligaments are attached to the
physis. This is especially true if the periosteal
sleeve is disrupted. Essentially, this is analogous
to a Type IIA fracture.
Type V fractures contain one fracture line that
creates a free-oating inferior cortical fragment
attached to the coracoclavicular ligaments along
with a separate fracture line that separates the
distal clavicle from the remainder of the clavicle.
B.S. Schwartz et al.
Thus, neither the proximal nor distal fragment is
attached to the coracoclavicular ligaments, lead-
ing to instability of the distal end of the proximal
fragment with the potential for signicant dis-
placement (Neer 1984).
Distal Clavicle Fracture Outcome Tools
There are currently no specic outcome tools
to assess distal clavicle fractures. Outcomes are
classically based on union rates and subjective
patient outcome measures. Various studies
have utilized adult-oriented outcome measures
such as the Constant Score, the American
Shoulder and Elbow Surgeons (ASES) score,
and the Medical Outcomes Study 36-Item
Short Form, but none of these have been adopted
universally, and furthermore, they have not been
validated in the pediatric or adolescent
populations.
Distal Clavicle Fracture Treatment
Options
Nonoperative Treatment of Distal
Clavicle Fractures
Indications/Contraindications
Nonoperative management of distal clavicle
fractures with immobilization alone is the
mainstay of treatment provided that no signicant
displacement is present (Table 7). This is typically
the case in Type I and Type III fractures. Type II,
IV, and V fractures, however, may exhibit
signicant displacement and associated instability
about the shoulder girdle and may be managed
with operative intervention. Contraindications to
nonoperative treatment include open fractures,
fractures associated with skin compromise,
and fractures with concomitant neurovascular
injury requiring surgical intervention. Displaced
fractures (Types II, IV, and V) in children
and adolescents should be managed on an indi-
vidual basis depending on the patients age, the
amount of displacement, and the patients
activities.
57 Clavicle Fractures
Table 7 Distal clavicle fractures: Nonoperative
management
Indications Contraindications
Non-displaced fractures Open fractures
and minimally displaced
fractures (Type I and Type
III fractures)
Fractures associated with
skin compromise
Fractures with
concomitant
neurovascular injury
requiring surgical
intervention
Techniques
A sling or shoulder immobilizer is worn for
approximately 34 weeks, followed by active
range of motion exercises. At the 34-week
follow-up visit, radiographs are obtained to con-
rm adequate healing is occurring and that there
has been no further displacement.
Outcomes
Patients with non- or minimally displaced distal
clavicle fractures who are treated nonoperatively
typically have outstanding outcomes with fracture
union achieved and a return to full activities. Con-
versely, Type I and III fractures in adults have been
reported to progress to delayed-onset symptomatic
acromioclavicular joint arthrosis (Neer 1968).
Due to a relatively high nonunion rate shown in
adult studies, the treatment of signicantly
displaced distal clavicle fractures is controversial.
A retrospective review conducted by Neer
reported all patients with Type II distal clavicle
fractures who were treated nonoperatively had
either a delayed union (67 %) or a nonunion
(33 %) (Neer 1963). Edwards et al. managed
20 patients with Type II distal clavicle fractures
nonoperatively and observed a 45 % delayed
union rate and a 30 % nonunion rate (Edwards
et al. 1992). Additional studies have reported sim-
ilar nonunion rates for Type II fractures treated
nonoperatively, ranging from 25 % to 44 %
(Norqvist et al. 1993; Robinson and Cairns 2004;
Robinson et al. 2004; Rokito et al. 20022003).
Conversely, all Type II fractures treated
1271
operatively with ORIF achieved union (Edwards
et al. 1992; Neer 1963; Rokito et al. 20022003).
Operative Treatment of Distal Clavicle
Fractures
Indications/Contraindications
Absolute indications for surgical management of
distal clavicle fractures include open fractures,
fractures with signicant skin compromise,
displaced intra-articular extension, and fractures
with associated neurovascular injuries that require
operative intervention. Relative indications
include signicantly displaced fractures in com-
petitive athletes and adolescents, entrapment in
the trapezius muscle, oating shoulder type inju-
ries, and polytrauma patients.
Surgical Procedure
Preoperative Planning
Numerous techniques exist to stabilize the
distal clavicle, so it is necessary to establish the
plan for xation preoperatively (Table 8). Multi-
ple options are available to the surgeon, including
various nonabsorbable sutures, Dacron tape, and
locking/non-locking plates such as anatomic clav-
icle plates and hook plates. Positioning of the
patient should be discussed with the anesthesiol-
ogist and operating room staff, as beach chair
positioning is often desired.
Positioning
The patient should be positioned in either the
beach chair position/Mayeld head positioner
with the head and neck tilted away or supine on
a radiolucent Table. A bump should be placed
behind the scapula in either position. The entire
shoulder girdle, beginning at the medial edge of
the clavicle, and entire limb should be prepped
and draped in the operative eld to permit move-
ment of the limb, enabling fracture reduction and
xation. The area above the shoulder and adjacent
to the head should be sterile so that the surgeon
may work both inferior and superior to the clavi-
cle and shoulder.
1272 B.S. Schwartz et al.

Table 8 ORIF of distal clavicle fractures: Preoperative Surgical Approach(es)

planning
OR table: standard operating room table capable of being
put into beach chair position or using Mayeld head
positioner
Position/positioning aids: beach chair position or
Mayeld positioner with the head and neck tilted away or
supine
Bump placed behind the scapula.
Fluoroscopy location: contralateral side of fracture
Equipment: nonabsorbable suture, Dacron tape, anatomic
clavicle plates, hook plates, mini-fragment, or modular
hand locking plates
Tourniquet (sterile/nonsterile): none
Draping: entire shoulder girdle region and ipsilateral limb
are prepped and draped into the eld to allow for traction
and manipulation
Medially, the contralateral sternoclavicular joint should
be included in the operative eld
The initial incision should be slightly curved and
follow Langers skin lines over the distal third of
the clavicle and acromioclavicular joint. Follow-
ing skin division, a thick ap is made by dividing
the subcutaneous tissue, fascia, and periosteum.
Care should be taken to avoid damaging the cuta-
neous nerves. Subsequently, subperiosteal dissec-
tion beginning from the intact clavicle and
extending toward the fracture site is performed
to expose the fracture fragments.
Technique
In skeletally mature adolescents, low prole ana-
tomic distal locking plates or hook plates may be
utilized for xation. In younger patients, however,
modular hand instrumentation or mini-fragment
locking plates may be required (Synthes, Inc.,
West Chester, PA) (Fig. 5). Distal radius plate

Fig. 5 (a). Fluoroscopic

image of a distal clavicle
fracture in an 11-year-old
male athlete. (b). Immediate
postoperative upright chest
x-ray to ensure no
pneumothorax is present.
(c). Follow-up radiograph
showing a healed fracture in
excellent alignment that
was xed with modular
hand instrumentation
(Courtesy of Joshua
M. Abzug, MD)
57 Clavicle Fractures
xation by placement of the 2.4 mm locking
screws in the distal clavicle fragment has also
been suggested (Kalamaras et al. 2008).
The age and size of the patient, as well as the
size and location of the fracture fragment(s), will
determine the choice of implant. Low prole
locking plates may be used in larger patients and
fragments, while smaller fragments may require
xation with suture, Kirschner wire, or even a
hook plate. A dorsal tension band using either
suture or wire should supplement Kirschner wire
xation if utilized. Threaded wires should be
used to decrease the risk of migration (Badhe
et al. 2007; Kao et al. 2001; Levy 2003;
Mall et al. 2002).
In adults, a decreased risk of nonunion has
been associated with supplementary xation of
the coracoclavicular ligaments, accomplished
with suture or Dacron tape with or without addi-
tional xation (Goldberg et al. 1997; Webber and
Haines 2000). Arthroscopic techniques utilizing
suture, the Tightrope system (Arthrex, Naples,
FL), or a double-button device to stabilize the
coracoclavicular ligaments have also been
reported in adults (Baumgarten 2008; Checcia
et al. 2008; Nourissat et al. 2007; Pujol
et al. 2008). Additionally, placement of a screw
between the coracoid and clavicle has been pro-
posed, but this requires screw removal after frac-
ture union (Ballmer and Gerber 1991; Edwards
et al. 1992; Fazal et al. 2007; Jin et al. 2006;
Macheras et al. 2005; Yamaguchi et al. 1998).
These techniques are seldom used in pediatric or
adolescent patients due to the periosteal insertion
of the ligaments. Once the periosteum is repaired,
the ligaments are usually stable (Table 9).
Treatment-Specific Outcomes
In the pediatric and adolescent populations, oper-
ative management of distal clavicle fractures pro-
duces excellent results with high union rates.
Hardware complications are the main concern
since utilization of smooth wires in this region
has led to wire migration into areas such as the
lung, abdomen, trachea, spine, and vascular struc-
tures. Smooth wires should be avoided when pos-
sible; if used, leave them out of the skin and
remove them early. Employing Ethibond suture
1273
Table 9 ORIF of distal clavicle fractures: Surgical steps
Skin incision over distal 1/3 of the clavicle and acromion
in line with the Langers lines
Electrocautery through the subcutaneous tissue, fascia,
and periosteum directly onto the clavicle
Expose fracture site in a subperiosteal manner while
preserving the acromioclavicular and coracoclavicular
ligaments
Reduce fracture fragments with reduction clamps and
temporary Kirschner wire xation if necessary
Apply plate on the superior aspect of clavicle
Assess reduction and screw lengths with direct
visualization and/or uoroscopic imaging in multiple
planes
Repair periosteum to tighten coracoclavicular and
acromioclavicular ligaments. May need to apply
supplemental xation utilizing suture around the
coracoid and clavicle if xation is marginal
Irrigate wound and close periosteum
Meticulous skin closure with absorbable suture
Apply sling or shoulder immobilizer
as a tension band lessens the risk of hardware
irritation, but suture granulomas can develop
and sometimes require subsequent removal
(Lee et al. 2009).
Fracture union rates with plate xation have
been reported to be as high as 100 % (Kalamaras
et al. 2008). Utilization of hook plates in adults
has resulted in a lower complication rate and
produced better results with regard to return to
work and sports participation (Flinkkila
et al. 2002; Lee et al. 2009). To avoid secondary
complications, however, the hook plate should be
removed once the fracture is healed.
Preferred Treatment
The vast majority of children and adolescents with
distal clavicle fractures are treated nonoperatively.
A sling or shoulder immobilizer is worn for 34
weeks, and then the patient is advanced to active
range of motion, presuming union has been
achieved. Operative treatment is performed for
open fractures, fractures with skin compromise,
fractures with associated neurovascular injury
requiring operative intervention, displaced
1274 B.S. Schwartz et al.

intra-articular fractures, and signicantly Table 10 Distal clavicle fractures: Potential pitfalls and
displaced fractures, particularly those displaced preventions
posteriorly with trapezius muscle entrapment. Potential pitfall Pearls for prevention
A direct approach to the fracture is performed Nonunion/hardware Ensure adequate xation in
via a skin incision that follows Langers skin lines. failure distal fragment
Electrocautery is then utilized to divide the sub- Supplement xation with
suture and prolong
cutaneous tissue, fascia, and periosteum. Care is immobilization until union
taken to avoid any cutaneous nerves. The perios- is conrmed
teum is then elevated using a freer elevator or Screw penetration into the Directly visualize the joint
similar blunt instrument while preserving the acromioclavicular joint and utilize uoroscopy in
multiple planes following
acromioclavicular and coracoclavicular ligament
plate xation
attachments. The fracture fragments are then
exposed and irrigated to remove hematoma and
debris prior to reduction.
Ideal xation of a distal clavicle fracture is Table 11 Distal clavicle fractures: Management of
performed using a plate and screw construct, Complications
with the assumption that there is adequate bone
Hardware prominence Removal of hardware
laterally. Anatomically contoured distal clavicular Hardware migration Removal of hardware
locking plates are utilized in older adolescents Nonunion ORIF
when feasible. If the fragment is too small for Symptomatic malunion ORIF
these implants, xation is performed using mini-
fragment or modular hand locking plates
(Synthes, Inc., West Chester, PA). Supplementa-
tion of the plate xation with suture around the Management of Complications
coracoid and clavicle is not routinely performed
unless the xation was marginal, as the periosteal Symptomatic hardware is the most common com-
repair is typically adequate for ligamentous and plication related to operative treatment of distal
soft tissue stability. clavicle fractures and can be easily resolved by
If plate xation is not appropriate, interosseous removal of hardware. If a nonunion or symptom-
suture xation of the fracture fragments is uti- atic malunion occurs, ORIF of the fracture can be
lized. In the pediatric and adolescent populations, performed. If necessary, the distal fragment can be
it is unnecessary to place suture around the cora- excised, and the acromioclavicular joint can be
coid and clavicle. Hook plates are only employed reconstructed via a modied Weaver-Dunn proce-
as a last resort since they require a subsequent dure, where the coracoacromial ligament is trans-
procedure for removal. However, they should be ferred to the distal aspect of the remaining clavicle
available during all procedures in case adequate (Anderson 2003) (Table 11).
xation cannot be obtained.

Summary and Future Research

Surgical Pitfalls and Prevention
Inadequate xation of the distal clavicular frag-
ment or excessive early activity can lead to non-
union or hardware failure. Furthermore, it is
essential to avoid screw penetration into the
acromioclavicular joint. This area should be eval-
uated with direct visualization and/or utilizing
uoroscopy in multiple planes (Table 10).
The majority of studies regarding distal clavicle
fractures survey the adult population. If a child or
adolescent sustains a distal clavicle fracture,
immobilization alone is usually sufcient to
achieve a successful outcome. However, opera-
tive intervention may be justied in older adoles-
cents and highly competitive athletes. Utilization
of a plate and screw construct generates excellent
57 Clavicle Fractures
results with a rapid return to function, high union
rate, and low complication rate. Additional stud-
ies are needed to evaluate the treatment and out-
comes of these fractures in adolescents.
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 AC Dislocations, SC Dislocations,
and Scapula Fractures 58
Brandon S. Schwartz, Raymond Pensy, W. Andrew Eglseder,
and Joshua M. Abzug

Contents Acromioclavicular Dislocations Imaging and

Other Diagnostic Studies . . . . . . . . . . . . . . . . . . . . . . . . . . . 1285
Introduction to Scapula Fractures . . . . . . . . . . . . . . . 1278 Injuries Associated with Acromioclavicular
Pathoanatomy and Applied Anatomy Relating Dislocations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1285
to Scapula Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1278 Acromioclavicular Dislocations Classication . . . . 1285
Acromioclavicular Dislocation Outcome Tools . . . . 1286
Assessment of Scapula Fractures . . . . . . . . . . . . . . . . 1279
Signs and Symptoms of Scapula Fractures . . . . . . . . 1279 Acromioclavicular Dislocation
Scapula Fracture Imaging and Other Diagnostic Treatment Options . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1286
Studies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1279 Nonoperative Treatment of Acromioclavicular
Injuries Associated with Scapula Fractures . . . . . . . . 1279 Dislocations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1286
Scapula Fracture Classication . . . . . . . . . . . . . . . . . . . . . 1279 Operative Treatment of Acromioclavicular
Scapula Fracture Outcome Tools . . . . . . . . . . . . . . . . . . . 1280 Dislocations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1287
Treatment Specic Outcomes . . . . . . . . . . . . . . . . . . . . . . 1288
Scapula Fracture Treatment Options . . . . . . . . . . . . 1280
Nonoperative Treatment of Scapula Fractures . . . . . 1280 Preferred Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1288
Operative Treatment of Scapula Fractures . . . . . . . . . 1281 Surgical Pitfalls and Prevention . . . . . . . . . . . . . . . . . . . . 1289
Management of Complications . . . . . . . . . . . . . . . . . . . . . 1289
Preferred Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1283
Surgical Pitfalls and Prevention . . . . . . . . . . . . . . . . . . . . 1283 Summary and Future Research . . . . . . . . . . . . . . . . . . 1289
Management of Complications . . . . . . . . . . . . . . . . . . . . . 1283 Introduction to Sternoclavicular
Summary and Future Research . . . . . . . . . . . . . . . . . . 1284 Fracture-Dislocations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1290

Introduction to Acromioclavicular Pathoanatomy and Applied Anatomy Relating

Dislocations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1284 to Sternoclavicular Fracture-Dislocations . . . . . . . 1290

Pathoanatomy and Applied Anatomy Relating Assessment of Sternoclavicular

to Acromioclavicular Dislocations . . . . . . . . . . . . . . . 1284 Fracture-Dislocations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1290
Signs and Symptoms of Sternoclavicular
Assessment of Acromioclavicular Fracture-Dislocations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1290
Dislocations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1284 Sternoclavicular Fracture-Dislocations
Signs and Symptoms of Acromioclavicular Imaging and Other Diagnostic Studies . . . . . . . . . . . . . 1291
Dislocations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1284 Injuries Associated with Sternoclavicular
Fracture-Dislocations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1291
Sternoclavicular Fracture-Dislocations
Classication . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1291
B.S. Schwartz (*) R. Pensy W.A. Eglseder
Sternoclavicular Fracture-Dislocations
J.M. Abzug
Outcome Tools . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1292
University of Maryland School of Medicine, Baltimore,
MD, USA Sternoclavicular Fracture-Dislocations
e-mail: brandon.schwartz@som.umaryland.edu; Treatment Options . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1292
rpensy@umoa.umm.edu; aeglseder@umoa.umm.edu;
jabzug@umoa.umm.edu

# Springer Science+Business Media New York 2015 1277

J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_66
1278 B.S. Schwartz et al.

Nonoperative Treatment of Sternoclavicular Abstract

Dislocations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1292 Fractures and dislocations about the shoulder
Operative Treatment of Sternoclavicular
Fracture-Dislocations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1293
girdle are commonly seen in the pediatric pop-
ulation. This chapter discusses scapula frac-
Preferred Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1295
tures, acromioclavicular dislocations, and
Surgical Pitfalls and Prevention . . . . . . . . . . . . . . . . . . . . 1295
Management of Complications . . . . . . . . . . . . . . . . . . . . . 1295 sternoclavicular dislocations in children and
adolescents. The epidemiology, pathoanatomy,
Summary and Future Research . . . . . . . . . . . . . . . . . . 1296
assessment, diagnosis, and treatment options
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1296 of these injuries are discussed. The chapter
will include data from the limited number of
pediatric studies on these topics as well as data
extrapolated from the adult literature.

Introduction to Scapula Fractures

Scapula fractures are uncommon, accounting for

1 % of all fractures in adults with an even lower
incidence in children (Goss 1995; Thompson
et al. 1985). The most common scapula fracture
involves the scapular body and accounts for
approximately 45 % of fractures. The remaining
scapula fractures involve the glenoid neck (25 %),
glenoid cavity (10 %), acromion process (8 %),
coracoid process (7 %), and scapular spine (5 %)
(Thompson et al. 1985; McGahan et al. 1980).
Scapulothoracic dissociation can occur very rarely
and has been reported in two separate case reports
of children, ages 8 and 11 (An et al. 1988; Nettrour
et al. 1972). Owing to the low incidence of scapular
fractures, the majority of literature regarding treat-
ment and outcomes in the pediatric and adolescent
populations exists as case reports and small retro-
spective case series.

Pathoanatomy and Applied Anatomy

Relating to Scapula Fractures

The scapula is a at bone situated on the posterior

chest wall that is covered almost entirely by muscle
due to the 17 muscular attachments on it. Only the
dorsal aspect of the scapular spine and acromion
are subcutaneous, with the remainder of the scap-
ula lying deep and well protected from low-energy
mechanisms of injury. The scapula is involved in
three articulations: the acromion articulates with
the clavicle at the acromioclavicular joint, the
58 AC Dislocations, SC Dislocations, and Scapula Fractures
proximal humerus articulates with the glenoid at
the glenohumeral joint, and the posterior chest wall
articulates with the anterior scapula to comprise the
scapulothoracic articulation.
Assessment of Scapula Fractures
Signs and Symptoms of Scapula
Fractures
A large amount of force is required to sustain a
scapula fracture; therefore, a complete head-to-toe
survey is indicated and should be performed by
either the trauma team or emergency room physi-
cian. Difculty breathing may be observed due to
associated rib fractures or lung injury, whereas
ruptured viscera will lead to an acute abdomen.
Suspected non-accidental trauma requires a com-
plete evaluation of the patient including a head CT
scan, skeletal survey, ophthalmologic exam, and
social work consultation.
Patients who have sustained scapula fractures
often complain of signicant pain around their
chest, back, and shoulder region. Numbness may
occur due to concomitant brachial plexus injury or
signicant edema. Physical examination should
begin with observation for signicant swelling
and ecchymosis. A comprehensive neurovascular
examination of the involved extremity is then
necessary. Palpation of the affected areas should
be performed to determine the site of maximal
tenderness as well as any additional areas of ten-
derness, as associated shoulder girdle fractures
may be present. The orthopedic surgeon should
then perform a secondary survey to ensure there
are no additional musculoskeletal injuries.
Scapula Fracture Imaging and Other
Diagnostic Studies
Scapula fractures may initially be discovered on a
chest radiograph obtained as part of the trauma
workup; however, further imaging is required to
fully assess the fracture. Plain radiographs with
true AP and lateral scapula views as well as a
glenohumeral axillary view should be obtained
1279
when a scapula fracture is suspected. Further-
more, due to the signicant amount of overlying
bony and soft tissue structures, a CT scan will aid
the surgeon in evaluating the fracture pattern. If
operative treatment is being considered, the addi-
tion of three-dimensional reconstructions will
assist in preoperative planning.
Injuries Associated with Scapula
Fractures
Regardless of the mechanism of injury causing a
scapula fracture, associated injuries are common
and may be life-threatening. These include closed
head injuries, pneumo- or hemothorax, rib frac-
tures, ruptured viscera, and concomitant long-
bone fractures (Goss 1995; Thompson
et al. 1985; Imatani 1975). Almost 50 % of all
children admitted to the hospital for
non-accidental trauma have at least one fracture,
and approximately one-third have the diagnosis of
a contusion (Bullock et al. 2009). Neurovascular
injury involving the brachial plexus, subclavian
artery/vein, or axillary vessels may concomitantly
occur. Additional fractures or dislocations can
also occur about the shoulder girdle, leading to a
oating shoulder.
Scapula Fracture Classification
Scapula fractures are classied based on the frac-
ture location within the scapula: body, glenoid
cavity, glenoid neck, acromion, or coracoid. Addi-
tionally, the term scapulothoracic dissociation is
used to describe complete separation of the scap-
ula from the posterior chest wall.
Glenoid neck fractures are further subdivided
based on their angulation and displacement. Type I
fractures are angulated less than 40
and displaced
less than 1 cm, while a Type II fracture is angulated
greater than 40
and has more than 1 cm of dis-
placement (Goss 1994). Type I fractures account
for 90 % of glenoid neck fractures (Zdravkovic
and Damholt 1974; Ada and Miller 1991).
Glenoid cavity fractures are divided into six
types based on the location of the fracture and
1280 B.S. Schwartz et al.

the severity (Ideberg et al. 1995; Goss 1992). However, open fractures, fractures with associ-
Type I fractures involve either the anterior ated neurovascular injuries requiring operative
(Type Ia) or posterior (Type Ib) aspect of the glenoid intervention, scapulothoracic dissociation, large
rim. Type II fractures are transverse fractures that glenoid rim fractures with associated proximal
divide the superior and inferior aspects of the humerus subluxation/dislocation, Type II glenoid
glenoid and exit inferiorly through the lateral scap- neck fractures, and glenoid cavity fractures
ular border. Type III and IV fractures also involve a with greater than 5 mm of displacement are all
fracture line bisecting the superior and inferior exceptions and should be treated operatively
aspects of the glenoid, but Type III fractures exit (An et al. 1988; Ada and Miller 1991; Kavanagh
superiorly near or through the scapular notch, while et al. 1993; Nettrour et al. 1972). All of these are
Type IV fractures exit medially through the medial extremely rare in children, but must not be
border of the scapula. Type V fractures involve overlooked.
several fracture lines, representing a combination
of Types IIIV, and are further subdivided into a, Scapula fractures
b, and c. Type Va fractures are a combination of Nonoperative management
Types II and IV; Type Vb fractures are a combina- Indications Contraindications
tion of Types III and IV; and Type Vc fractures are a Non-/minimally displaced Open fractures
combination of Types II, III, and IV. Lastly, Type VI scapula body fractures
fractures are severely comminuted fractures Acromion fractures Fractures with associated
neurovascular injuries
(Ideberg et al. 1995; Goss 1992).
requiring surgical
intervention
Coracoid fractures with Glenoid cavity fractures
Scapula Fracture Outcome Tools <2 cm displacement with displacement
>5 mm
Currently, no specic outcome tools exist for Glenoid neck fractures with Large glenoid rim
<1 cm angulation and fractures with associated
evaluating scapula fractures. Previous outcome 40 degrees of angulation proximal humerus
measures used in the adult literature include sub- subluxation/dislocation
jective complaints of pain, fracture displacement, Glenoid cavity fractures Type II glenoid neck
residual deformity, nonunion, and development of with <5 mm of intra- fractures
posttraumatic arthritis (Mayo et al. 1998; articular displacement
Severely comminuted
Kavanagh et al. 1993; Nordqvist and Petersson
glenoid fractures unable to
1992; Edwards et al. 2000; Labler et al. 2004). support stable xation
Specic pediatric outcomes have not been devel-
oped, but the goals of outcome are identical: res-
toration of motion, function, and strength without Techniques
long-term limitations and/or pain. The patient is tted with a sling or shoulder immo-
bilizer for 36 weeks depending on the patient,
injury severity, and healing. Once healing is suf-
Scapula Fracture Treatment Options cient and there is a reduction in pain, rehabilita-
tion begins with pendulum exercises and
Nonoperative Treatment of Scapula progresses to full range of motion and strengthen-
Fractures ing. Return to sports generally occurs 812 weeks
after injury.
Indications/Contraindications
The majority of scapula fractures can be treated Outcomes
nonoperatively by immobilization alone, regard- There are currently no large studies evaluating the
less of the anatomic location of the fracture. outcomes of children treated for scapula fractures.
58 AC Dislocations, SC Dislocations, and Scapula Fractures
A large proportion of adult patients progress to
fracture union and have minimal to no pain with
excellent functional outcomes (Goss 1995;
Nordqvist and Petersson 1992).
Operative Treatment of Scapula
Fractures
Indications/Contraindications
Surgical indications for scapula fractures in the
pediatric and adolescent populations include open
fractures, fractures with associated neurovascular
injuries requiring operative intervention, large
glenoid rim fractures with associated proximal
humerus subluxation/dislocation, Type II glenoid
neck fractures, coracoid process fractures with
greater than 2 cm of displacement, and glenoid
cavity fractures with displacement greater than
5 mm (An et al. 1988; Ada and Miller 1991;
Kavanagh et al. 1993; Nettrour et al. 1972). Float-
ing shoulder injuries involving the glenoid neck
and midshaft of the clavicle can be managed by
ORIF of the clavicle, as the glenoid neck will
reduce via ligamentotaxis provided by the intact
coracoclavicular ligament (Bahk et al. 2009). Like-
wise, oating shoulder injuries involving fractures
of the glenoid neck, midshaft of the clavicle, and
scapular spine can be treated by ORIF of the clav-
icle and scapular spine due to ligamentotaxis pro-
vided by the intact coracoclavicular and/or
coracoacromial ligaments (Bahk et al. 2009). The
remainder of injuries should be managed
nonoperatively with immobilization.
Surgical Procedure
Preoperative Planning
Positioning of the patient and essential implants
vary based on the location of the scapula fracture.
Plates with the capability of being bent and
twisted are typically required to match the shape
of the scapula. Planning for ORIF of scapula
fractures can be aided by the use of advanced
imaging. Utilization of three-dimensional recon-
structions can be especially helpful for assessment
of glenoid rim fractures.
1281
ORIF of scapula fractures
Preoperative planning
OR table: attop Jackson table or standard OR table with
the ability to go into beach chair position depending on
the approach being utilized
Position/positioning aids: lateral decubitus in bean bag or
beach chair
Fluoroscopy location: contralateral side of fracture
Equipment: 2.7 mm or 3.5 mm plate/screw constructs,
heavy nonabsorbable suture
Tourniquet (sterile/nonsterile): none
Positioning
Patient positioning is dependent on the anatomic
location of the scapular fracture as well as the
approach being utilized. Anterior exposure is
performed with the patient in the beach chair
position, using a standard deltopectoral approach.
If posterior exposure is necessary, the patient is
placed in the lateral decubitus position in a bean-
bag, allowing the shoulder and trunk to droop
forward slightly.
Surgical Approach(es)
A standard deltopectoral approach is utilized to
gain anterior access to the glenoid and coracoid.
An incision is made along the deltopectoral
groove beginning at the coracoid proximally
and extending 1015 cm distally. Sharp dissec-
tion is performed through the skin, and the
cephalic vein is identied in the deltopectoral
groove. The deltoid is then retracted laterally
and the pectoralis major medially; the cephalic
vein can be taken in either direction. The short
head of the biceps and the coracobrachialis are
then identied and retracted in a medial direc-
tion. Access to the anterior aspect of the shoulder
joint is now easily gained. In order to obtain
adequate exposure of the glenoid, the
subscapularis must be taken down and a retractor
placed in the glenohumeral joint to retract the
humeral head.
The majority of coracoid fractures are xed via
an incision over the coracoid in Langers lines.
The deltopectoral approach may be extended
proximally to achieve coracoid exposure if there
is an associated superior glenoid fracture that also
requires exposure. Dissection should continue
1282
down the slope of the coracoid to the base so that
the fracture can be best visualized. If a coracoid
base fracture involves part of the superior glenoid
fossa, a posterior approach with indirect reduction
of the superior glenoid may be used (Anavian
et al. 2009). Fractures of the lesser tubercle of
the humerus may be exposed via an anterolateral
approach or standard deltopectoral approach.
A posterior approach to the glenoid requires a
vertical incision overlying the posterior glenoid
and full thickness aps to be raised. The deltoid is
split longitudinally in line with its bers in order
to visualize the infraspinatus and teres minor.
These muscles can be partially or completely
detached, or the interval between them can be
utilized, depending on the amount of exposure
necessary. The capsule is now visualized and can
be incised to access the glenoid. Alternatively, a
transverse incision can be made along the length
of the scapular spine and extended to the posterior
corner of the acromion. Next, the deltoid is
detached from its origin on the scapular spine,
and the plane between the deltoid and
infraspinatus is identied and established. The
teres minor is then identied, and the plane
between the teres minor and infraspinatus is
established. Retracting the infraspinatus superi-
orly and the teres minor inferiorly will expose
the posterior aspect of the glenoid and scapular
neck. A longitudinal incision of the glenohumeral
joint capsule along the edge of the scapula will
then provide access to the joint.
Technique
The posterior approach is performed to visualize
displaced glenoid neck fractures, and a plate is then
placed along the posterior aspect of the glenoid and
extending down along the lateral angle of the scap-
ula. Surgical treatment of Type Ib, Type II, and
Type IV glenoid cavity fractures are also performed
via a posterior approach. The infraspinatus attach-
ment can remain intact during xation of Type Ib
fractures; however, detachment is required for
Type II and IV fractures. Fixation of Type Ib frag-
ments typically requires two interfragmentary
screws, whereas Type II and IV fractures usually
require plate and screw xation.
B.S. Schwartz et al.
ORIF of Type Ia and III glenoid cavity fractures
or coracoid fractures displaced greater than 2 cm is
performed via the anterior deltopectoral approach.
Fixation of Type Ia and large coracoid process
fractures is achieved utilizing interfragmentary
screws if the fragment is large enough, whereas
Type III fractures typically require plate and screw
xation. Suture anchors can alternatively be used to
stabilize Type Ia fragments, and small coracoid
process fractures can be reattached with the conjoint
tendon by utilizing heavy nonabsorbable suture
placed in a Bunnell fashion through the tendon
and passed through a drill hole in the intact coracoid
process. Additionally, arthroscopic xation of Type
Ia fractures can be performed via suture anchor
xation to the intact labral attachment of the frag-
ment (Sugaya et al. 2005).
ORIF of posterior glenoid cavity fractures and glenoid
neck fractures
Surgical steps
Lateral decubitus position utilizing bean bag
Prep and drape entire upper extremity and hemithorax
Posterior approach with the arm abducted
Retract posterior part of deltoid superolaterally without
detaching its origin or insertion the muscle can be
detached if needed for improved visualization
Identify and explore interval between the teres minor and
infraspinatus by retracting the teres minor inferiorly and
infraspinatus superiorly
Detach infraspinatus insertion if necessary and incise
capsule if xing a glenoid cavity fracture
Reduce fragment utilizing K-wires as joysticks and
provisional xation
Fix fragment with either interfragmentary screws or
plate/screw construct
ORIF of anterior glenoid cavity fractures
Surgical steps
Beach chair position with Mayeld headrest
Prep and drape entire upper extremity and hemithorax
Standard deltopectoral approach
Place stay sutures in subscapularis and detach muscle
from humerus
Longitudinal incision to enter glenohumeral joint
Place Fukuda retractor on humeral head to expose
glenoid
Reduce fragment utilizing intact labrum or K-wires as
joysticks and provisional xation
(continued)
58 AC Dislocations, SC Dislocations, and Scapula Fractures
ORIF of anterior glenoid cavity fractures
Surgical steps
Fix fragment with either interfragmentary screws or
plate/screw construct
ORIF of displaced coracoid process fractures
Surgical steps
Beach chair position with Mayeld headrest
Prep and drape entire upper extremity and hemithorax
Standard deltopectoral approach
Identify and protect musculocutaneous nerve
Fix large coracoid process fractures with
interfragmentary screw xation
Fix small coracoid process fractures using heavy
nonabsorbable suture through the conjoint tendon and
passed through a drill hole in the intact coracoid process
Treatment Specific Outcomes
There is currently no data evaluating outcomes of
pediatric and adolescent patients treated with
ORIF of scapula fractures. Adult studies have
reported that the results of operative xation of
glenoid cavity fractures depend on near-anatomic
restoration of joint alignment, with good to excel-
lent results expected for 8090 % of patients who
have residual incongruity less than 2 mm (Mayo
et al. 1998; Kavanagh et al. 1993).
Preferred Treatment
The majority of children and adolescents who have
a scapula fracture are managed nonoperatively with
immobilization for 34 weeks followed by pendu-
lum exercises progressing to active range of motion
as tolerated. This regimen is indicated for scapula
body fractures, acromion fractures, coracoid pro-
cess fractures, and glenoid neck and cavity frac-
tures without signicant displacement. Surgical
intervention is reserved for open fractures, glenoid
cavity fractures with signicant size and/or dis-
placement leading to glenohumeral subluxation/
dislocation, and coracoid process fractures
displaced greater than 2 cm.
Arthroscopic reduction of Type Ia glenoid
cavity fractures is performed, whereas ORIF is
1283
performed for the remainder of glenoid cavity
and glenoid neck fractures requiring operative
xation. Three-dimensional CT scans are rou-
tinely obtained to assist in preoperative planning
and determination of the best surgical approach to
use based on the fracture pattern.
Surgical Pitfalls and Prevention
Damage to neurovascular structures can occur if
forceful retraction is used, thus care must be taken
during ORIF when retracting structures about the
shoulder. For example, the musculocutaneous
nerve and lateral cord are at risk during excessive
medial retraction about the glenohumeral joint
and coracoid.
Near-anatomic reduction of the articular sur-
face is critical during ORIF of glenoid cavity
fractures as residual displacement greater than
2 mm leads to poorer outcomes (Mayo
et al. 1998; Kavanagh et al. 1993). Additionally,
persistent glenohumeral subluxation/dislocation
can develop if large glenoid cavity fragments are
not properly reduced.
Scapula fractures
Potential pitfalls and preventions
Potential pitfall Pearls for prevention
Neurovascular injury Avoid over-vigorous
retraction
Persistent glenohumeral Obtain near-anatomic
subluxation/dislocation (<2 mm) alignment of
glenoid cavity fragments
Management of Complications
Nonoperative management of scapular body frac-
tures can be complicated by nonunion and symp-
tomatic malunion (Ferraz et al. 2002; Martin and
Weiland 1994; Michael et al. 2001). Nonunions can
be treated with good to excellent results by
performing ORIF. Moreover, signicant displace-
ment associated with glenoid neck fractures has
been shown to be a poor prognostic indicator.
Therefore, xation of fractures with angulation
1284
greater than 40
or more than 1 cm of displacement
will generate improved outcomes (Nordqvist and
Petersson 1992; Edwards et al. 2000; Labler
et al. 2004). Finally, large glenoid rim fractures
should be treated surgically to prevent subluxa-
tion/dislocation of the glenohumeral joint.
Scapula fractures
Common complication Management
Nonunion ORIF
Symptomatic malunion ORIF
Glenohumeral subluxation/dislocation ORIF
Summary and Future Research
Scapula fractures are rare injuries caused by high-
energy mechanisms or non-accidental trauma.
Nonoperative treatment with immobilization gen-
erates excellent outcomes in the vast majority of
cases. It is essential, however, to identify fractures
that can potentially lead to adverse outcomes and
complications. Advanced imaging with CT scans,
including three-dimensional reconstructions, can
assist the surgeon in evaluating the fracture pat-
tern. Glenoid fractures with signicant displace-
ment, as well as fractures leading to glenohumeral
subluxation/dislocation, should be xed opera-
tively. Due to the paucity of these fractures, future
multicenter studies will likely be necessary to
evaluate treatments and their outcomes for pedi-
atric and adolescent scapula fractures.
Introduction to Acromioclavicular
Dislocations
Acromioclavicular (AC) dislocations are common
in adults but rather rare in children. They are likely
overdiagnosed in children, as injuries appearing to
disrupt the acromioclavicular joint may actually
be an epiphyseal separation of the distal clavicle,
termed a pseudodislocation, rather than a true
acromioclavicular joint disruption (Rockwood
et al. 1998). Adolescents, however, particularly
those participating in competitive sports, can sus-
tain true acromioclavicular dislocations (Dameron
and Rockwood 1984; Kaplan et al. 2005).
B.S. Schwartz et al.
Treatment of these injuries, particularly complete
dislocations, remains controversial and is based
on individual patient needs.
Pathoanatomy and Applied Anatomy
Relating to Acromioclavicular
Dislocations
The acromioclavicular joint is comprised of the
distal edge of the clavicle and medial aspect of
the acromion with a brocartilaginous disk situated
between them. This joint is a signicant contributor
to the superior suspensory complex of the shoulder,
a bone-soft tissue ring composed of the glenoid,
coracoid, coracoclavicular ligaments, distal clavi-
cle, acromioclavicular joint, and acromion. The
complex maintains a natural relationship between
the scapula, upper extremity, and axial skeleton to
permit uid scapulothoracic motion. While the
majority of motion occurs synchronously, the clav-
icle does rotate relative to the acromion via the
acromioclavicular joint (Flatow 1993).
The majority of stability about the acromio-
clavicular joint is provided by the ligamentous
structures, with the remainder provided by the
muscular attachments of the anterior deltoid onto
the clavicle and the trapezius onto the acromion.
Horizontal stability is due to the posterior and
superior acromioclavicular ligaments, which rein-
force the joint capsule. The coracoclavicular liga-
ments, including the conoid ligament medially and
trapezoid ligament laterally, provide vertical stabil-
ity (Fukuda et al. 1986). The normal distance of the
coracoclavicular space, the area between the cora-
coid and the clavicle, should be 1.11.3 cm
(Bearden et al. 1973).
Assessment of Acromioclavicular
Dislocations
Signs and Symptoms
of Acromioclavicular Dislocations
Pain in the shoulder region localized to the
acromioclavicular joint area is the most common
complaint of patients who have sustained
58 AC Dislocations, SC Dislocations, and Scapula Fractures
acromioclavicular dislocations. Patients may also
complain of numbness and tingling due to edema
or concomitant cervical spine and/or brachial
plexus injury. Sometimes, however, patients only
complain of a bump in the region.
Physical examination should begin with obser-
vation of the shoulder with the patient in an upright
position, allowing the weight of the arm to make
any deformity more evident. While observing, note
any swelling, ecchymosis, and/or abrasions. Palpa-
tion of the acromioclavicular joint will cause sig-
nicant discomfort and thus should be performed
at the conclusion of the examination. Surrounding
areas including the proximal humerus, midshaft
and medial clavicle, sternoclavicular joint, and cer-
vical spine should be palpated rst. Potential con-
comitant brachial plexus or cervical spine injury
should be evaluated for with a thorough neurologic
examination. Most displaced distal clavicles are
malpositioned superiorly and demonstrate both
visual and palpable deformity. However, the clav-
icle may displace posteriorly, become entrapped in
the trapezius muscle, and exhibit a palpable prom-
inence with tenderness being present medial and
posterior to the acromion. These Type IV injuries
may be difcult to diagnose unless explicitly
evaluated for.
When an acromioclavicular injury is suspected,
the joint should be evaluated for stability
once the acute pain has subsided, approximately
57 days post-injury. Horizontal and vertical
stability should be assessed before considering
a potential closed reduction. Closed reduction
is performed by using one hand to stabilize
the clavicle while generating upward force
under the ipsilateral elbow with the other hand.
Once reduction in the coronal plane has been
achieved, the midshaft of the clavicle can be
gripped and translated in an anterior and posterior
direction to assess horizontal stability (Simovitch
et al. 2009).
Acromioclavicular Dislocations
Imaging and Other Diagnostic Studies
Plain radiographs are the preferred initial imaging
modality and should include a true AP view,
1285
axillary lateral view, and a Zanca view of the
shoulder to visualize the acromioclavicular joint.
The Zanca view requires the patient to be posi-
tioned upright so that the injured arm can hang by
the weight of gravity, while the x-ray beam is
aimed 10
15
cephalad (Zanca 1971). Stress
views can also be obtained to differentiate
between Types II and III injuries by asking the
patient to hold a weight in their hand. A CT scan
may be necessary to diagnose posterior fracture-
dislocations (Type IV) as they are often difcult
to identify on plain radiographs.
Injuries Associated
with Acromioclavicular Dislocations
Akin to any injury about the shoulder region, the
entire shoulder girdle must be examined for an
associated injury. If enough force was present at
the time of impact, anterior sternoclavicular dis-
locations as well as additional scapula, humerus,
or clavicle fractures may occur concurrently. Fur-
thermore, brachial plexus or cervical spine inju-
ries may also be present, particularly if the injury
occurred during a collision sport such as football.
Acromioclavicular Dislocations
Classification
Acromioclavicular injuries in adults are classied
using a scheme developed by Tossy et al. and
Allman, which was subsequently modied by
Rockwood (Tossy et al. 1963; Allman 1967; Wil-
liams et al. 1989). Type I injuries demonstrate
normal radiographs and exhibit the sole nding
of tenderness to palpation over the acromio-
clavicular joint caused by a sprain of the
acromioclavicular ligaments. In Type II injuries,
the acromioclavicular ligaments are disrupted
along with a sprain of the coracoclavicular liga-
ments. Radiographs demonstrate a widened AC
joint with slight vertical displacement exhibited
by a mild increase in the coracoclavicular space.
Type III injuries involve disruption of the
acromioclavicular and coracoclavicular liga-
ments, and radiographs show displacement of
1286
the clavicle superiorly relative to the acromion by
25100 % the width of the clavicle. Type IV
injuries have disruption of the acromioclavicular
and coracoclavicular ligaments as well as the
deltopectoral fascia, permitting posterior dis-
placement of the clavicle into or through the tra-
pezius muscle. In Type V injuries, there is
disruption of the acromioclavicular and
coracoclavicular ligaments and deltopectoral fas-
cia, with concomitant injury to the deltoid and
trapezius muscle attachments to the clavicle.
Thus, the clavicle is typically displaced greater
than 100 % and lies in the subcutaneous tissue.
Type VI injuries involve disruption of the
acromioclavicular ligaments and deltopectoral
fascia; however, the coracoclavicular ligaments
remain intact. This injury occurs via a high-energy
mechanism that causes the shoulder to be
hyperabducted and externally rotated, resulting
in a subacromial or subcoracoid position of the
clavicle and a decrease in the coracoclavicular
distance seen on radiographs (Tossy et al. 1963;
Allman 1967; Williams et al. 1989).
Due to the low incidence of true acromio-
clavicular injuries in skeletally immature patients
compared to fractures of the distal clavicle, this
classication has been modied for the pediatric
and adolescent populations (Dameron and
Rockwood 1984). Generally, the clavicle itself
displaces out of the periosteal sleeve, leaving the
coracoclavicular and acromioclavicular ligaments
attached to the periosteum. The clavicle injuries
that result are then analogous to the six types
described for adults.
Acromioclavicular Dislocation
Outcome Tools
Currently, no outcome scores exist to explicitly
evaluate the results of acromioclavicular injuries,
or any injury about the shoulder, in children and
adolescents. Several adult shoulder and upper
extremity outcome measures are available, how-
ever, to assess these injuries in older adolescents.
Reported outcomes from acromioclavicular inju-
ries have typically been based on subjective
B.S. Schwartz et al.
measures, the development of acromioclavicular
osteoarthritis, and range of motion.
Acromioclavicular Dislocation
Treatment Options
Nonoperative Treatment
of Acromioclavicular Dislocations
Indications/Contraindications
Type I and II acromioclavicular injuries are uni-
versally managed nonoperatively; however, Type
III injury treatment remains controversial. Most
Type IV, V, and VI injuries should be treated
surgically in order to reduce the acromioclavicular
joint and restore stability to the superior shoulder
suspensory complex. Open injuries and injuries
with associated neurovascular injury requiring
operative intervention are absolute contraindica-
tions to nonoperative management.
Acromioclavicular dislocations
Nonoperative management
Indications Contraindications
Type I Open injuries
injuries
Type II Dislocations with associated
injuries neurovascular injuries requiring
operative treatment
Techniques
Nonoperative management involves immobiliza-
tion in a sling or shoulder immobilizer for 24
weeks. Patients are then gradually advanced from
pendulum exercises to active range of motion.
Once range of motion is equal to the noninjured
side, strengthening is begun. Contact sports
should be avoided for 3 months post-injury to
permit complete ligamentous healing and to pre-
vent conversion of an incomplete injury (Type II)
to a complete injury (Type III) (Dameron and
Rockwood 1984).
Outcomes
Few studies regarding nonoperative management
of Type I and II injuries in pediatric and
58 AC Dislocations, SC Dislocations, and Scapula Fractures
adolescent patients have been published. In the
adult literature, 930 % of patients expressed pain
and limitation of activities with closed treatment
of Type I injuries and 2342 % of patients
expressed similar complaints with closed treat-
ment of Type II injuries, some of which required
surgical intervention (Bergeld et al. 1978;
Mounshine et al. 2003). Children and adolescents
appear to exhibit better results regarding pain and
restoration of function, but this has not been eval-
uated extensively.
Due to the variety of outcomes described in
adult studies, treatment of Type III injuries has
remained controversial. Bannister et al. found that
injuries with 2 cm or more of displacement treated
nonoperatively demonstrated 20 % good or excel-
lent results versus 70 % in the surgically treated
group (Bannister et al. 1989). Conversely, a sep-
arate study of athletes and laborers with
nonoperatively managed Type III injuries demon-
strated that these patients were able to recover
sufcient strength and endurance to return to
their pre-injury activities (Wojtys and Nelson
1991). Phillips and colleagues supported
nonoperative treatment of Type III injuries in a
meta-analysis showing that patients treated surgi-
cally demonstrated a higher complication rate,
while patients treated nonoperatively were able
to return to work and pre-injury activities sooner
(Phillips et al. 1998).
Operative Treatment
of Acromioclavicular Dislocations
Indications/Contraindications
The indications for surgical intervention of
acromioclavicular injuries include complete dis-
ruption of the joint progressing to true disloca-
tions in adolescents or fracture-dislocations in
children, mainly Types IV, V, and VI injuries. In
young patients, the most common operative indi-
cation is a Type IV injury with displacement and
entrapment in the trapezius muscle posteriorly.
Open injuries and injuries with concomitant
neurovascular injury requiring operative interven-
tion should also be managed surgically. As noted
1287
above, the treatment of Type III injuries remains
controversial.
Surgical Procedure
Preoperative Planning
Planning for the operative treatment of
acromioclavicular injuries is crucial so that the
appropriate equipment is available. Potential
implants utilized include a hook plate, cannulated
screws, Kirschner wires, or heavy nonabsorbable
suture. Ligament reconstruction, however,
requires planning to either obtain hamstring auto-
graft or have allograft available.
ORIF of acromioclavicular dislocations
Preoperative planning
OR table: standard table capable of beach chair
positioning
Position/positioning aids: beach chair position with
adequate sterile space above the shoulder adjacent to the
head and neck
Fluoroscopy location: contralateral side
Equipment: implants may include a hook plate,
cannulated screws, Kirschner wires, heavy
nonabsorbable suture, hamstring autograft, and allograft
Tourniquet (sterile/nonsterile): none
Positioning
The beach chair position with or without a May-
eld head positioner is utilized whether open
reduction or ligament reconstruction is being
performed. A bump is placed behind the scapula
to shift the acromion into a more anterior position.
Surgical Approach(es)
Performing a direct approach to the acromio-
clavicular joint entails making an incision along
the lateral clavicle and anterior aspect of the joint
in line with Langers skin lines. Sharp dissection is
performed through the skin only. The remainder of
dissection down to bone is carried out by electro-
cautery to permit hemostasis at the time of dissec-
tion. Subsequently, it is easiest to incise the
periosteum of the distal clavicle and acromion
before entering the joint. In Type VI injuries,
avoiding disruption of the coracoclavicular liga-
ments is critical as they remain intact following the
1288
injury. The acromioclavicular and coracoclavicular
ligaments as well as the deltopectoral fascia are
disrupted in adults with Types II, IV, and V injuries,
but are attached to the periosteum in skeletally
immature patients and therefore must be preserved
in this younger population.
Technique
Open reduction of the acromioclavicular joint is
performed following exposure of joint. Type IV
injuries require careful extraction of the distal clav-
icle from the trapezius muscle, Type V injuries
involve reduction of the distal clavicle from the
subcutaneous tissue, and Type VI injuries necessi-
tate removal of the distal clavicle from beneath the
coracoid process. Following reduction of the distal
clavicle to the level of the acromion, it may be
necessary to maintain the reduction with temporary
pin xation. In the pediatric population, because
the periosteum is torn but still afxed to the
acromion, simple repair of the periosteum and lig-
amentous structures may be all that is required,
once reduction of the clavicle is achieved.
If utilizing a hook plate in an older patient, the
lateral end of the plate is positioned deep to
the acromion, and the medial side is placed on
the superior surface of the clavicle, thus facilitat-
ing joint reduction and maintenance. The plate is
then secured utilizing bicortical screws.
Various methods of ligament reconstruction
and/or augmentation have been performed as the
primary management of the injury in adults. Fortu-
nately, these operations are rarely required for ado-
lescents in the acute setting, as they are typically
indicated for chronic, painful acromioclavicular
separations in adults. Both semitendinosus auto-
graft and allograft can be utilized. The graft can
be looped around the coracoid and clavicle (Jones
et al. 2001) or guided through bone tunnels in the
coracoid and clavicle and subsequently secured
with interference screws, which are placed
(Mazzocca et al. 2006) at the sites of the
coracoclavicular ligaments in order to reestablish
normal anatomy.
Coracoclavicular screw placement or loops of
heavy nonabsorbable suture/Dacron tape around
the coracoid and clavicle have also been suggested
to treat acromioclavicular injuries, either solely or
B.S. Schwartz et al.
in conjunction with ligament reconstruction
(Bosworth 1941). However, screw placement
requires removal, and the loop technique can lead
to suture cutout or aseptic foreign body reactions
(Boldin et al. 2004; Stewart and Ahmad 2004). The
modied Weaver-Dunn procedure can be
performed in arthritic scenarios by resecting the
distal aspect of the clavicle, detaching the
coracoacromial ligament from the deep surface of
the acromion, and transferring it to the distal end of
the clavicle. Again, this operation is rarely
performed in children and adolescents.
ORIF of acromioclavicular dislocations
Surgical steps
Skin incision in Langers lines directly anterior to
acromioclavicular joint
Electrocautery down to distal clavicle and acromion
Reduce acromioclavicular joint
Repair periosteum and ligamentous structures and assess
stability
Utilize hook plate if joint remains unstable, for segmental
fractures, or intra-articular fractures
Place lateral end of hook plate under acromion and
facilitate AC joint reduction by placing medial part of
plate on superior clavicle
Place bicortical screws in medial part of hook plate
Irrigate wound and close
Plate removal 23 months postoperatively
Treatment Specific Outcomes
Evaluation of acromioclavicular injury treatment
in the pediatric and adolescent populations has not
been specically studied. Operative management
of Type IV, V, and VI injuries yields excellent
outcomes in the majority of patients. Restoration
of joint congruity and stability facilitates rapid
return to function. However, no long-term data
has been gathered to calculate the number of
patients who develop degenerative arthritis.
Preferred Treatment
All Type I and II acromioclavicular injuries, along
with the vast majority of Type III injuries, are
managed nonoperatively with immobilization in
58 AC Dislocations, SC Dislocations, and Scapula Fractures
a sling or shoulder immobilizer for 24 weeks,
followed by early range of motion. Contact sports
are avoided for at least 3 months. Operative treat-
ment is performed for the majority of Type IV, V,
and VI injuries. Once the distal clavicle is
exposed, a determination is made of whether
repair of the periosteum and ligaments surround-
ing the clavicle is adequate or if a plate is neces-
sary. Operative repair is most commonly reserved
for Type IV fracture-dislocations with entrapment
within the trapezius. In older patients with either
segmental or intra-articular fractures, hook plates
are commonly utilized. The periosteum and liga-
mentous structures are then repaired subsequent
to plate placement.
Surgical Pitfalls and Prevention
Failure to identify a Type IV injury is one of the
greatest pitfalls that can occur when treating
acromioclavicular injuries. Type V and VI injuries
are generally obvious on AP plain radiographs,
while Type IV injuries may not be easily detected
due to their posterior displacement. Type IV inju-
ries may also be increasingly difcult to recognize
due to inadequate or difcult to obtain lateral
views. Therefore, a high index of clinical suspi-
cion, a thorough examination, and frequently a
CT scan are necessary for accurate diagnosis and
proper surgical treatment.
Acromioclavicular dislocations
Potential pitfalls and preventions
Potential pitfall Pearls for prevention
Missing a Type IV Careful assessment of radiographs
injury
Adequate lateral radiograph
Utilize physical examination to aid
in diagnosis
CT scan
Management of Complications
A common complication encountered with surgi-
cal management of acromioclavicular injuries is
development of degenerative arthritis, which can
1289
be treated by distal clavicle resection. However, if
the coracoclavicular ligaments are disrupted, the
results of this are unfavorable due to the ensuing
instability (Corteen and Teitge 2005). If instability
persists following closed treatment of an
acromioclavicular injury, ligament reconstruction
or augmentation can be performed.
Complications of open reduction include
migration of pins, symptomatic hardware, and
persistent pain. Utilization of synthetic material
carries the risk of suture cutout or aseptic foreign
body reaction. Coracoid fracture or injury to the
musculocutaneous nerve can occur as a conse-
quence of any technique that passes material
around the coracoid.
Acromioclavicular dislocations
Common
complication Management
Posttraumatic arthritis Distal clavicle resection
Persistent instability Ligament reconstruction/
augmentation
Symptomatic Hardware removal
hardware
Pin migration Hardware removal
Persistent pain Determine cause
arthritis resection,
instability ligament
reconstruction
Suture cutout
Aseptic foreign body
reaction
Summary and Future Research
Injuries to the acromioclavicular joint are rare in
children and adolescents. Classication of the
injury patterns is similar to adults, although
the periosteum in young patients is torn, allowing
the clavicle to displace, while the periosteal
attachments to the acromion and coracoid remain
intact. Immobilization alone can be utilized for
injuries that are not considerably displaced,
though surgical intervention is necessary for sig-
nicantly displaced injuries. Reduction of the
acromioclavicular joint, suture repair of the peri-
osteum, and ligamentous repair to restore normal
anatomy can provide excellent results in the
1290
pediatric population while avoiding the need for
metal implants. Future studies evaluating
acromioclavicular injury outcomes in children
and adolescents are needed.
Introduction to Sternoclavicular
Fracture-Dislocations
Injuries to the sternoclavicular joint are rare and
account for less than 5 % of shoulder girdle
injuries (Cave 1958; Jaggard et al. 2009). High-
energy forces are required to produce sterno-
clavicular injuries, and therefore these injuries
can be associated with life-threatening complica-
tions. Observation was at one time considered to
be the treatment of choice in children and adoles-
cents. However, more recent trends involve oper-
ative reduction and stabilization of acute posterior
fracture-dislocations to restore anatomy and
improve functional outcomes.
Pathoanatomy and Applied Anatomy
Relating to Sternoclavicular Fracture-
Dislocations
The sternoclavicular joint, composed of the
medial clavicle and the clavicular notch of the
sternum, is a true diarthrodial joint and is the
only link between the upper extremity and axial
skeleton. Less than 50 % of the clavicular head
articulates with the clavicular notch of the ster-
num, which results in little bony congruity and
stability. Therefore, the multiple ligamentous and
muscular attachments, such as the sternoclei-
domastoid, pectoralis major, and sternohyoid,
are the main contributors to stability. The liga-
mentous structures include the anterior and poste-
rior sternoclavicular ligaments that reinforce the
joint capsule, the interclavicular ligaments that
connect the medial ends of the clavicles, and the
costoclavicular ligaments that span between the
inferior aspect of the clavicle and the superior costal
cartilage of the adjacent rib. The sternoclavicular
joint also contains an intra-articular disk that
attaches to the superior-posterior aspect of the
B.S. Schwartz et al.
clavicular articular surface and to the costocarti-
laginous junction of the rst rib. Together, the
posterior capsule and sternoclavicular ligaments
provide the greatest resistance to anterior transla-
tion. The greatest stability with regard to posterior
translation is provided by the posterior capsule
(Spencer and Kuhn 2004; Spencer et al. 2002).
Ossication of the medial epiphysis of the
clavicle does not occur until approximately
1820 years of age. Furthermore, the physis
closes between 22 and 25 years of age, making
radiographic sternoclavicular injury diagnoses in
children and adolescents difcult to discern
between fractures and dislocations. Previously, it
was thought that the majority of posterior
sternoclavicular injuries were physeal fractures;
however, surgical treatment of these injuries has
suggested that there is actually a near equivalent
incidence between fractures and dislocations.
Although approximately 80 % of longitudinal
growth of the clavicle takes place at the medial
physis, the amount of remodeling possible with a
physeal fracture is undetermined. Remodeling
clearly cannot occur following a dislocation.
Assessment of Sternoclavicular
Fracture-Dislocations
Signs and Symptoms
of Sternoclavicular Fracture-
Dislocations
Patients presenting with sternoclavicular joint
injuries typically complain of pain localized to
the sternoclavicular region. Additional complaints
may include shortness of breath, dyspnea, dyspha-
gia, odynophagia, or hoarseness (Waters et al.
2003). Patients may also experience paresthesias
and/or weakness in the ipsilateral arm if there is a
concomitant brachial plexus injury.
Physical examination will often reveal a sig-
nicant amount of edema and ecchymosis, mak-
ing the direction of dislocation difcult to assess
(Groh and Wirth 2011). Prominence of the
medial clavicle, best observed with the patient
supine, may indicate an anterior dislocation.
58 AC Dislocations, SC Dislocations, and Scapula Fractures
In posterior dislocations, however, the corner
of the sternum may be palpable due to the poste-
rior displacement of the medial clavicle (Nettles
and Linscheid 1968). Swelling in posterior
fracture-dislocations can be more subtle than
expected, and the edema can mimic normal
sternoclavicular alignment.
Movement of the ipsilateral shoulder
passively will elicit pain, and the patient may
experience a sensation of instability. A formal
evaluation by the trauma team or emergency
room physician is critical to rule out associated
life-threatening injuries. Compression of vessels
can lead to signs of venous congestion and arte-
rial insufciency in the affected extremity or
neck region.
Sternoclavicular Fracture-Dislocations
Imaging and Other Diagnostic Studies
Plain radiographs are the initial imaging modality
of choice for sternoclavicular dislocations. Asym-
metry of the sternoclavicular articulations or clav-
icle lengths may be identied on a routine AP
chest radiograph; however, this can be difcult
to assess due to overlap of the medial clavicle,
lungs, ribs, sternum, and spine.
The difculty of evaluating sternoclavicular
injuries has led to the development of specic
radiographic views to better identify these inju-
ries. A tangential view of the sternoclavicular
joint, proposed by Heining, is performed by posi-
tioning the patient supine and placing the cassette
behind the opposite shoulder. The beam is then
angled coronally, parallel to the longitudinal axis
of the opposite clavicle (Heining 1967). Hobbs
suggested a 90
cephalocaudal lateral view,
obtained by having the patient seated and exed
over a table with the beam directed through the
cervical spine (Hobbs 1968). Finally, the seren-
dipity view described by Rockwood is performed
by positioning the cassette behind the chest and
angling the x-ray beam 40
cephalad while it is
centered on the sternum, thus providing a view of
both sternoclavicular joints (Wirth and Rockwood
1996). The affected side will appear superiorly
1291
displaced in anterior dislocations and appear infe-
riorly displaced in posterior dislocations.
However, the easiest way to evaluate these inju-
ries is with computed tomography, which provides
a three-dimensional view of the joint. With a CT
scan, the adjacent soft tissue structures such as the
esophagus, trachea, lungs, and brachiocephalic
vessels can also be evaluated. Distinguishing
between a physeal fracture and a true dislocation
may also be possible if the secondary center of
ossication has ossied.
Magnetic resonance imaging can also be
performed to evaluate the sternoclavicular joint
as well as the surrounding soft tissues. The integ-
rity of the costoclavicular ligaments and intra-
articular disk may also be possible to assess
(Groh and Wirth 2011). Due to its speed and
availability, CT is preferred over MRI to evaluate
acute injuries.
Injuries Associated
with Sternoclavicular Fracture-
Dislocations
Due to the high-energy mechanisms that cause
posterior sternoclavicular injuries, associated
chest wall injuries such as rib fractures can
occur. Additionally, the trachea, esophagus,
lungs, or great vessels can be compressed, and
patients may experience a brachial plexopathy.
Very rarely, a oating shoulder injury can occur
where the entire clavicle may dislocate from both
the sternoclavicular and acromioclavicular joints.
Evaluation of the entire shoulder girdle for con-
comitant fractures or dislocations is imperative.
Sternoclavicular Fracture-Dislocations
Classification
Classication of sternoclavicular dislocations is
based on the direction of displacement (anterior
or posterior) as well as the chronicity of the injury
(acute or chronic). The injury must be dened
as either a dislocation (displacement between
the epiphysis and sternum) or a fracture
1292
(displacement through the physis with the epiph-
ysis still articulating with the sternum). Finally, a
sprain, rather than a true dislocation, may occur
leading to subluxation.
Sternoclavicular Fracture-Dislocations
Outcome Tools
Currently, no specic outcome scores to assess
sternoclavicular joint injuries are available. Previ-
ously reported outcomes have utilized subjective
complaints of pain, recurrence of instability,
return to function, and utilization of adult shoulder
outcome measures, such as the American Shoul-
der and Elbow Surgeons score (ASES), the simple
shoulder test, and Rockwood scores.
Sternoclavicular Fracture-Dislocations
Treatment Options
Nonoperative Treatment
of Sternoclavicular Dislocations
Indications/Contraindications
Atraumatic anterior dislocations should be treated
nonoperatively. Closed reduction maneuvers for
acute posterior fracture-dislocations have been
suggested by some, due to the potential stability
following reduction and/or limited remodeling
potential of the medial clavicle (Wirth and
Rockwood 1996; Leighton et al. 1986). Open
reduction should clearly be performed for acute
posterior dislocations with associated
neurovascular injury, dyspnea, dysphagia,
odynophagia, or hoarseness. However, it is rea-
sonable to treat all posterior fracture-dislocations
operatively.
Sternoclavicular dislocations
Nonoperative management
Indications Contraindications
Atraumatic Acute posterior dislocations with
anterior associated neurovascular injury,
dislocations dyspnea, dysphagia,
odynophagia, or hoarseness
B.S. Schwartz et al.
Techniques
Nonoperative management involves sling immo-
bilization for approximately 3 weeks followed by
gradual return to activities. Closed reduction of
anterior dislocations can be attempted by
retracting the scapula with a bump placed between
the shoulders and directing a posteriorly directed
force over the medial clavicle. The patient is sub-
sequently placed in a gure-of-eight strap or
Velpeau-type sling for 6 weeks (Groh and Wirth
2011). Successful reduction is often achieved;
however, recurrent instability is common.
Closed reduction of a posterior dislocation is
performed by positioning the patient supine on
an operating room table with a thick bump placed
between the scapulae to extend the shoulders and
the affected arm off the edge of the table. With
traction applied, the ipsilateral arm is then
abducted in line with the clavicle while an assis-
tant applies countertraction and stabilizes the
patient. Traction is sustained and increased
while the arm is brought into extension as the
joint reduces (Groh and Wirth 2011). Alterna-
tively, the arm can be placed in adduction while
posterior pressure is applied to the shoulder,
levering the clavicle over the rst rib to permit
reduction of the joint (Buckereld and Castle
1984). If these maneuvers fail, a sterile towel
clip can be used to percutaneously grasp the
medial clavicle and pull it anteriorly while trac-
tion is applied to the ipsilateral limb. An audible
snap is typically heard as the joint reduces (Groh
and Wirth 2011).
Closed reductions of posterior sternoclavicular
injuries are at risk of mediastinal hemorrhage and
hemodynamic compromise. Therefore, closed
reductions should be performed in the controlled
setting of the operating room with vascular sur-
gery backup readily available. Concern remains
for recurrent instability following closed reduc-
tion (Waters et al. 2003; Groh et al. 2011;
Goldfarb et al. 2001).
Outcomes
The majority of patients who sustain anterior dis-
locations and are treated with immobilization
58 AC Dislocations, SC Dislocations, and Scapula Fractures
alone experience good outcomes despite the high
rates of recurrent instability (de Jong and Sukul
1990). Patients developing symptoms following
closed management of anterior dislocations may
benet from physical therapy to promote scapular
retraction and education on how to avoid provoc-
ative positions. Ligament reconstruction can be
performed if therapy is unsuccessful, with reason-
able outcomes expected (Spencer et al. 2002;
Burrows 1951; Bae et al. 2006).
Posterior fracture-dislocations that are reduced
via closed methods have been reported by some to
be stable following reduction (Groh et al. 2011),
while others have described recurrent instability
(Waters et al. 2003; Groh et al. 2011; Goldfarb
et al. 2001). Return to full activities can be
expected in the majority of patients if the reduc-
tion is maintained over time.
Operative Treatment
of Sternoclavicular Fracture-
Dislocations
Indications/Contraindications
Although many surgeons have attempted closed
treatment of posterior fracture-dislocations with
either immobilization alone or closed reduction
followed by immobilization, recurrent instability
can occur leading to symptomatic patients requir-
ing operative intervention (Baumann et al. 2010).
Thus, most patients with acute traumatic posterior
sternoclavicular fracture-dislocations are cur-
rently managed surgically in order to provide
symptomatic relief, restore anatomy, and decrease
the chance of late complications such as recurrent
instability and degenerative arthritis (Waters
et al. 2003).
Other indications for operative management
include patients with symptomatic acute or
chronic anterior dislocations who have failed con-
servative measures and symptomatic patients with
chronic posterior dislocations. Contraindications
to surgical intervention include patients with
asymptomatic anterior dislocations or patients
with atraumatic recurrent anterior instability.
1293
Surgical Procedure
Preoperative Planning
Familiarity with the anatomy surrounding the
sternoclavicular joint as well as the bony articula-
tion of the medial clavicle and clavicular notch of
the sternum is critical. During the reduction
maneuver or open reduction, it is essential to
have a general surgeon or thoracic surgeon avail-
able to assist the orthopedic surgeon in case of
hemodynamic compromise.
ORIF of sternoclavicular fracture-dislocations
Preoperative planning
OR table: standard table capable of beach chair
positioning
Position/positioning aids: beach chair position with
Mayeld head positioner and bump behind scapula
Fluoroscopy location: contralateral side if at all
Equipment: heavy nonabsorbable suture, drill
General or thoracic surgery backup
Tourniquet (sterile/nonsterile): none
Positioning
Patients undergoing any procedure involving the
sternoclavicular joint are positioned in the modi-
ed beach chair position with the head placed in a
Mayeld head positioner and a large bump or
rolled towel placed between the scapulae to pro-
vide retraction. The entire limb and hemithorax
including the contralateral sternoclavicular joint,
medial clavicle, and chest is prepped and draped
into the operative eld. In case an emergency
median sternotomy is needed, the sternum to
upper abdomen is also prepped and draped.
Surgical Approach(es)
A transverse skin incision is made from the medial
surface of the clavicle to the ipsilateral sterno-
clavicular joint in Langers lines. Electrocautery
is then utilized to divide the subcutaneous tissue
and platysma, while taking care to protect the
supraclavicular nerves. The periosteum of the
medial clavicle is elevated, and a bone clamp is
applied to the clavicle for control. The anterior
periosteum is delicately divided over the posteri-
orly displaced clavicle until either the epiphysis or
1294
Fig. 1 Intraoperative
photograph of an open
reduction of a
sternoclavicular dislocation
showing the drill holes
placed in the epiphysis and
sternum (Courtesy of
Shriners Hospital for
Children, Philadelphia, PA)
sternum is reached, depending on whether there is
a dislocation or a physeal fracture. Commonly, the
posterior periosteum of the clavicle and the pos-
terior joint capsule are intact, providing a protec-
tive layer between the bony injury and mediastinal
structures.
Technique
Once the physeal fracture or sternoclavicular dis-
location has been exposed, gentle reduction is
performed with the aid of a fracture reduction
clamp. At this point, it is important to converse
with the anesthesiologist to ensure that the patient
has remained hemodynamically stable. Direct
visualization is then performed to ensure that the
clavicular head is anatomically seated in the cla-
vicular notch of the sternum.
Following anatomic reduction of either the
fracture or dislocation, drill holes are made in
the anterior metaphysis and epiphysis of the clav-
icle in cases of a fracture or the anterior epiphysis
and sternum in cases of a dislocation (Fig. 1). In
order to prevent the drill from entering the medi-
astinum, placement of malleable retractors
between the bone and posterior periosteum is
helpful. Heavy nonabsorbable suture is subse-
quently passed in a gure-of-eight fashion to pro-
vide the necessary stability. In older patients or
chronic situations, ligament reconstruction with
tendon autograft or allograft may be utilized
(Fig. 2). Next, the periosteum is reapproximated
with heavy suture to provide additional stability,
B.S. Schwartz et al.
particularly with a true dislocation as this provides
indirect repair of the costoclavicular and
sternoclavicular ligaments. Stability is then eval-
uated by ranging the ipsilateral shoulder and limb.
Finally, the wound is irrigated and closed in
sequential layers once stability is satisfactory.
ORIF of sternoclavicular fracture-dislocations
Surgical steps
Prep and drape entire limb and hemithorax including
contralateral sternoclavicular joint, chest, and upper
abdomen
Transverse skin incision in Langers lines from the
diaphysis of the clavicle to the sternoclavicular joint
Divide subcutaneous tissue and platysma in line with skin
incision. Protect supraclavicular nerves
Expose clavicle and sternum and incise periosteum
working from lateral to medial on clavicle and from
midline to lateral on sternum
Evaluate the sternoclavicular joint to determine whether a
true dislocation or physeal fracture occurred
Reduce dislocation/fracture with aid of a fracture
reduction clamp
Converse with anesthesia to ensure hemodynamic
stability of patient
Place drill holes in anterior epiphysis and metaphysis for
physeal fractures or anterior epiphysis and sternum for
dislocations
Pass heavy nonabsorbable suture/tendon autograft or
allograft in a gure-of-eight fashion and tie
Reapproximate periosteum with heavy suture
Irrigate and close wound in sequential layers
Immobilize patient in sling and swathe or shoulder
immobilizer
58 AC Dislocations, SC Dislocations, and Scapula Fractures 1295

Fig. 2 Intraoperative
photograph of a tendon
allograft utilized to
reconstruct the
sternoclavicular ligaments.
Note the gure-of-eight
conguration (Courtesy of
Shriners Hospital for
Children, Philadelphia, PA)

Treatment Specific Outcomes allograft. In painful chronic dislocations that

In the vast majority of reported cases, outcomes have deformity of the bone and early arthritis
following open reduction and internal xation of of the joint, medial clavicle resection may be
posterior sternoclavicular dislocations or medial required.
clavicle physeal fractures in children and adoles-
cents have been very favorable (Waters et al. 2003;
Goldfarb et al. 2001; Baumann et al. 2010; Koch Surgical Pitfalls and Prevention
and Wells 2012; Tompkins et al. 2010). Waters
et al. conducted a retrospective review in which Over-reduction of the clavicle into the clavicular
all patients treated with open reduction and suture notch of the sternum can occur, thus it is impera-
xation of a posterior sternoclavicular joint tive to be familiar with the sternoclavicular bony
fracture-dislocation had restoration of joint stability alignment. Additionally, utilization of Dacron
and shoulder motion with full return to activities tape may cause osteolysis. Pins should be avoided
(Waters et al. 2003). Goldfarb and colleagues due to the potential of migration (Reilly
reported similar results, with all patients returning et al. 1999; Lyons and Rockwood 1990; Venissac
to their pre-injury function including sports partic- et al. 2000).
ipation (Goldfarb et al. 2001).
Sternoclavicular dislocations
Potential pitfalls and preventions
Preferred Treatment Potential pitfall Pearls for prevention
Over-reduction of clavicle Be knowledgeable about
Acute atraumatic anterior dislocations are man- into clavicular notch of the bony anatomy of the
sternum sternoclavicular joint
aged with immobilization alone for 14 weeks
Osteolysis from Dacron Utilize heavy
followed by gradual return to function. Therapy nonabsorbable suture
is started in patients who experience recurrent Kirschner wire migration Avoid Kirschner wires
instability. Surgical intervention typically
involves reconstruction of the ligaments and is
reserved for patients with persistent symptoms.
Acute posterior dislocations are managed oper- Management of Complications
atively with open reduction and internal xation.
Chronic symptomatic posterior dislocations are Recurrent instability following acute repair is rel-
treated with ligament reconstruction utilizing atively rare but can occur, particularly if the
1296
sternoclavicular joint is over-reduced. These
patients will present with persistent pain and a
sense of instability. Treatment with ligament
reconstruction can be performed using
semitendinosus autograft or allograft passed in
a gure-of-eight fashion, similar to the suture
utilized during the acute repair. In order to min-
imize the risk of recurrent instability, the tendon
is ideally passed on the instability side, anterior
for anterior dislocations and posterior for poste-
rior dislocations. Alternatively, medial clavicle
resection arthroplasty can be performed with
supplemental ligament reconstruction or soft
tissue interposition as a salvage procedure.
Approximately one centimeter of medial clavicle
is excised in an oblique fashion to preserve the
inferior ligamentous attachments. The intra-
articular disk can be passed into the medullary
canal of the clavicle by detaching the superior
end while preserving the inferior attachments.
Sutures are then passed through drill holes in
the superior clavicle and tied over a bony bridge.
Passing sutures between the costoclavicular lig-
ament and the clavicle can provide additional
stability.
Bae and colleagues reported the results of treat-
ment for recurrent anterior instability. Sixty per-
cent of patients had stable, pain-free joints
following the procedure, and no patients devel-
oped instability following treatment. However,
many patients still had minor limitations of func-
tion or persistent pain (Bae et al. 2006).
Sternoclavicular dislocations
Common complication Management
Persistent pain
Recurrent instability Ligament reconstruction
Summary and Future Research
Although sternoclavicular joint injuries are rare,
they do occur even in the pediatric and adolescent
populations. Due to the high-energy mechanisms
necessary to cause sternoclavicular injuries, con-
comitant damage to vital mediastinal structures
can occur. Therefore, a thorough examination is
B.S. Schwartz et al.
required to avoid overlooking an associated
life-threatening injury. Most anterior dislocations
can be managed nonoperatively with immobiliza-
tion alone, while acute posterior injuries are gen-
erally treated with surgical intervention with good
results anticipated. Future studies investigating
the natural history of nonoperatively managed
posterior dislocations are necessary to determine
who should undergo immediate operative inter-
vention versus those that should undergo closed
reduction or observation alone.
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 Part XII
Infection
 Pediatric Hand Infections
59
Leo Kroonen

Contents Other Hand Infections . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1315

Herpetic Whitlow . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1315
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1302
Atypical Infections . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1316
General Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1303
Assessment of Hand Infections . . . . . . . . . . . . . . . . . . . . 1303 Bite Wounds . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1317
Classication of Hand Infections . . . . . . . . . . . . . . . . . . . 1304 Human Bite Wounds . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1317
Outcome Tools . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1304 Animal Bite Wounds . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1318
Occupational Therapy Recommendations . . . . . . . . . 1304
Septic Arthritis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1318
Surgical Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1306
Osteomyelitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1318
Microbiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1307
Foreign Body Osteitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1319
Finger Infections . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1307 Surgical Pitfalls and Prevention . . . . . . . . . . . . . . . . . . . . 1320
Acute Paronychia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1307 Management of Complications . . . . . . . . . . . . . . . . . . . . . 1320
Operative Technique . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1308 Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1320
Chronic Paronychia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1308 Future Research . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1321
Felon . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1309 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1321
Operative Technique . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1309
Pyogenic Flexor Tenosynovitis . . . . . . . . . . . . . . . . . . . 1310
Operative Technique . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1311
Deep Space Infections . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1312
The Thenar Space . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1312
Midpalmar Space (Also Known as the Deep
Palmar Space) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1313
Hypothenar Space . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1314
Paronas Space . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1314
Interdigital Subfascial Space . . . . . . . . . . . . . . . . . . . . . . . 1314
Dorsal Subaponeurotic Space . . . . . . . . . . . . . . . . . . . . . . 1314

L. Kroonen
Department of Orthopaedic Surgery, Naval Medical Center
San Diego, San Diego, CA, USA
e-mail: ltkroonen@mac.com; leo.kroonen@med.navy.mil

# Springer Science Business Media New York (outside the USA) 2015 1301
J.M. Abzug et al. (eds.), The Pediatric Upper Extremity,
DOI 10.1007/978-1-4614-8515-5_58
1302 L. Kroonen

then, that the hand is the most frequently injured

Abstract
part of the body, particularly in a young popula-
Hand infections are an important cause of mor-
tion (Salazard et al. 2006). Infection in the pedi-
bidity in the pediatric population. Such infec-
atric hand has been reported after between 440 %
tions should be promptly evaluated including a
of injuries. (Fetter-Zarzeka and Joseph 2002;
history taking into consideration any traumatic
Frazier et al. 1978). Finger or thumb-sucking
exposure, the patients immunization status,
and biting behavior in children and a childs nat-
the presence of comorbidities, and environ-
ural curiosity with domestic or wild animals all
mental exposures. Infections can be catego-
provide chances for exposure of the small hands
rized into supercial spreading infections,
of children to bacteria.
closed space infections, and other infections.
The rst descriptions of hand infections come
Infections involving the nger include
from the days of Hippocrates, in which dressings
paronychia, felons, and exor tenosynovitis.
soaked with wine were the primary treatment.
Deep space infections can involve the thenar,
Many of the earliest principles from Hippocrates
hypothenar, midpalmar, and/or Paronas space,
are still in effect today. They have been coupled
as well as the interdigital subfascial space.
with the pioneering work of Allen B. Kanavel,
Atypical infections of the hand often behave
whose name is inextricably tied to hand infec-
in a more indolent manner and are typically
tions. Dr. Kanavel was the rst to explore the
caused by nonbacterial organisms including
anatomic basis of hand infections through his
viruses or mycobacterial species. A high
studies of the potential spaces of the hand, and
index of suspicion is necessary to diagnose
his name has become synonymous with the diag-
atypical hand infections, as the organisms
nosis of exor tenosynovitis (Green 2005).
often require special culture media in order to
Hand infections in adults often are associated
obtain an accurate diagnosis. Bite wounds
with comorbidities including diabetes, intrave-
from both animals and humans are common
nous (IV) drug abuse, and work exposure to
in the pediatric hand and require antibacterial
name a few. While immunosuppression is some-
coverage accounting for unique organisms
times seen as a contributing factor to the develop-
such as Pasteurella and Eikenella. Septic
ment of infections in a childs hand, the rate of
arthritis and osteomyelitis are particularly wor-
infection in the pediatric hand is less than that of
risome hand infections that require aggressive
the adult population since the same list of
treatment to eradicate.
comorbidities often does not exist (Sydnor and
A careful history can often provide clues as
Perl 2011). The etiology of hand infections in a
to the underlying microbiology in pediatric
child is often different both in terms of the causal
hand infections. These infections must be
organisms and the mechanisms by which inocula-
closely monitored early on, and if any closed
tion occurs. More importantly, the presenting
collection of purulence is found, surgical drain-
symptoms of the infection can be quite varied,
age is indicated. Postoperative care includes
and often time the history is difcult or impossible
short periods of immobilization and elevation,
to obtain depending on the age of the patient and
followed by early mobilization, once resolu-
whether or not any injury was witnessed.
tion of the acute infection is achieved.
Very few studies have specically examined
hand infections in the pediatric population, so
extrapolations from the adult population must be
Introduction used when discussing specic types of infection
and their treatment, but caution should be taken
Hand infections are an important cause of mor- when applying all of these extrapolations to a
bidity in the pediatric population. Children begin young patient population in whom the physiology
exploring their environments at an early age is different. Because children with hand infections
through the use of their hands. It is no wonder, present in a varied manner, the diagnosis is often
59 Pediatric Hand Infections
delayed. Such delay can result in the transforma-
tion of a simple supercial infection to a deep
infection, such as osteomyelitis. The effects of
this spread of disease cannot be understated. A
missed supercial infection that spreads deeply
and progresses to osteomyelitis, septic arthritis,
or other more involved infections can ultimately
result in signicant morbidity or even amputation.
It is imperative that a high index of suspicion for
infection is always present when evaluating the
painful, swollen, or erythematous hand of a child.
General Principles
Assessment of Hand Infections
A child presenting for evaluation of a painful,
swollen hand should be promptly evaluated. Care-
ful history should be obtained from the child, if
possible, or from the parent. Specic questioning
should be directed as to whether or not any trauma
was sustained or if there was environmental expo-
sure, animal or human bite exposure, or any recent
illnesses. Often times, a careful history can be very
useful in determining the underlying organism
responsible for causing the infection. Attention
should also be given to the immunization history
of the child. If any puncture wound was sustained,
appropriate tetanus immunization or administration
of a booster should be given when appropriate.
Examination of the painful extremity should be
done carefully, by rst starting away from the site
of the most signicant swelling. The limb is
examined for any painful joint motion which
may suggest a septic arthritis and any proximal
lymphadenitis. Attention is then given to the most
painful or swollen part of the extremity. It is
important to realize that the dorsum of the hand
is often edematous regardless of the location of
the actual infection, since the lymphatic drainage
is most prominent here. The skin on the dorsum of
the hand is also bound less tightly compared to the
palmar side, so it is easier for uid to collect in this
region. If dorsal swelling is present, it is critical to
evaluate the palmar side carefully as well, since
often times the dorsal swelling is actually a red
herring (Carter 1983). If erythema is present,
1303
Fig. 1 Six hours after a self-inicted stab wound, this
child presented with increasing pain and a popping sen-
sation with digital motion. Subcutaneous air is seen at the
volar wrist (Courtesy of Kevin Little, MD)
a skin marker is used to delineate the area of
erythema and labeled with the date and time.
This is useful to track the progress of the erythema
and the effectiveness of the treatment with time.
Plain lm imaging of the affected portion of the
extremity is recommended to look for signs of air
in the soft tissues that might suggest gas gangrene
(Fig. 1) or changes within the bone to indicate the
presence of osteomyelitis. Advanced imaging should
be performed for any case in which there is suspicion
of a collection of purulence (Fig. 2). Ultrasound,
computed tomography (CT), or magnetic resonance
imaging (MRI) can all be useful in order to localize
an underlying abscess. An MRI or bone scan can be
invaluable for the diagnosis of early osteomyelitis.
Laboratory workup should also be performed
for all but the simplest of hand infections. Routine
inammatory laboratory tests including a com-
plete blood count (CBC) with differential, eryth-
rocyte sedimentation rate (ESR), and C-reactive
protein (CRP) should be obtained to establish a
baseline upon which further treatment can be
based. Blood cultures and wound cultures, when
possible, also are recommended prior to the initi-
ation of antibiotics.
1304
Fig. 2 MRI demonstrates signicant uid collection
within the palm, surrounding the exor tendons. Ulti-
mately, this infection was diagnosed as an atypical myco-
bacterial infection
Once the workup is complete, and a working
diagnosis is established, treatment is initiated.
Empiric antibiotics can be initiated once cultures
are obtained. Table 1 presents options for empiric
antibiotic treatment depending on the etiology of
the infection.
Cases that present early, and without any sys-
temic toxicity, can be treated nonoperatively
(Table 2). Appropriate treatment in this early
stage includes soft tissue rest via splinting, usually
in a forearm-based plaster resting splint. Elevation
with the use of a sling or foam pillow is also
important, along with empiric antibiotic adminis-
tration. However, any systemic toxicity or the pres-
ence of a developing infection for over 48 h should
prompt a decision for surgical intervention.
Classification of Hand Infections
Hand infections can be classied into three broad
categories: supercial spreading infections,
L. Kroonen
closed space infections, and other infections.
Supercial spreading infections include condi-
tions such as cellulitis and necrotizing fasciitis. It
is important to acknowledge that cellulitis can
coexist with a deeper infection, and this possibil-
ity should always be at the forefront of the treating
surgeons mind. If a cellulitis fails to resolve or
worsens within 24 h of initiation of appropriate
antibiotic treatment, other deeper sources of infec-
tion should be sought. Careful attention should
also always be paid to the spread of supercial
infections and the rate. Rapid spread of skin infec-
tions should prompt immediate further evaluation
and a trip to the operating room, if necessary, to
assess for a surgical emergency such as necrotiz-
ing fasciitis.
Closed space infections are characterized by a
contained collection of purulence. The treatment
for any of these is surgical drainage; antibiotics
alone are insufcient treatment, since the antibi-
otics will not penetrate or cause resolution of a
uid collection.
Other infections will be addressed individually
below and include atypical infections (viral, fun-
gal, and mycobacterial), bite wounds, and
osteomyelitis.
Outcome Tools
No specic outcome tools are available for the
assessment of hand infections. Appropriate eval-
uation of the outcomes of treatment for hand
infections would include the Disabilities of the
Arm, Shoulder and Hand (DASH) or QuickDASH
or other anatomically specic outcome tools.
Occupational Therapy
Recommendations
Early management of hand infections consists of
immobilization in a position of comfort, which a
hand therapist can assist with as necessary, though
plaster or berglass splints are more commonly
used. Once there is evidence of improvement,
gentle range of motion and edema control should
be initiated with the assistance of a hand therapist
59 Pediatric Hand Infections 1305

Table 1 Empiric Treatment for Common Pediatric Hand Infections

Type of Most common Less common
infection organism organisms Empiric antibiotic coverage
Acute Staphylococcus Dicloxacillin
paronychia aureus
Beta-hemolytic Cephalexin
streptococci Amoxicillin-clavulanate
Clindamycin
Chronic Fungi Topical corticosteroids and antifungals (usually no role
paronychia Mycobacterial for systemic antimicrobials)
Bacterial (rare)
Felon Staphylococcus Dicloxacillin
aureus
Oral anaerobes Cephalexin
Amoxicillin-clavulanate
Clindamycin (PCN allergic)
Herpetic Herpes simplex Acyclovir (preferred)
whitlow virus 1 or 2 Valacyclovir or famciclovir (alternatives)
Flexor Staphylococcus Pasteurella Community acquired
tenosynovitis aureus multocida
Gram-negative Kingella kingae Vancomycin plus cefotaxime
rods Enterobacter spp. Vancomycin plus ciprooxacin (older adolescents)
Sporothrix Postoperative
schenckii
Alternaria spp. Vancomycin plus cefepime and metronidazole
Mycobacteria Vancomycin plus piperacillin-tazobactam
Deep space Staphylococcus Community acquired
infections aureus
Anaerobes Vancomycin plus ampicillin-sulbactam
Gram-negative Vancomycin plus moxioxacin (older adolescents)
rods Vancomycin plus cefotaxime and metronidazole
Postoperative
Vancomycin plus cefepime
Vancomycin plus piperacillin-tazobactam
Human bites Staphylococcus Actinomyces Amoxicillin-clavulanate (supercial)
aureus
Eikenella Treponema Ampicillin-sulbactam (deep)
corrodens pallidum (syphilis)
Streptococcus Mycobacterium Clindamycin plus trimethoprim-sulfamethoxazole
spp. tuberculosis (PCN allergic)
Anaerobes Hepatitis B virus
Bacteroides
melaninogenicus
Animal bites Gram-positive Bacteroides spp. Amoxicillin-clavulanate (supercial)
cocci
Anaerobes Viridans Ampicillin-sulbactam (deep)
Pasteurella streptococci Clindamycin plus trimethoprim-sulfamethoxazole
multocida (PCN allergic)
(continued)
1306 L. Kroonen

Table 1 (continued)
Type of Most common Less common
infection organism organisms Empiric antibiotic coverage
Septic Staphylococcus Community acquired
arthritis aureus
Streptococcus Vancomycin plus cefotaxime or ceftriaxone (especially
spp. if gonococcal suspected)
Neisseria Vancomycin plus ciprooxacin (older adolescents,
gonorrhoeae non-gonococcal)
Postoperative
Vancomycin plus cefepime
Vancomycin plus piperacillin-tazobactam
Osteomyelitis Staphylococcus Streptococcus Community acquired
aureus pneumoniae
Streptococcus Bartonella Vancomycin plus cefotaxime or ceftriaxone
spp. henselae
Gram-negative Prevotella spp. Vancomycin plus ciprooxacin (older adolescents)
rods Porphyromonas Postoperative
spp. Vancomycin plus cefepime and metronidazole
Vancomycin plus piperacillin-tazobactam

Table 2 Indications and Contraindications for preferred for the patients comfort. A standard
Nonoperative Treatment of Pediatric Hand Infections instrument set for hand surgery is typically ade-
Nonoperative management quate to perform an irrigation and debridement
Indications Contraindications procedure (Table 3). In general, these cases are
Presentation within 24 h of Obvious underlying performed under tourniquet control, but the limb
onset of symptoms uctuance
should NOT be exsanguinated with an elastic
Mild appearance without Systemic toxicity
bandage (Esmarch), as this might actually
systemic signs
No focal uid collection is Immunocompromised
push the bacteria up the arm and cause more
present host systemic spread of the bacteria. Gravity exsangui-
nation is preferred.
Incisions should be planned carefully and will
if the patient is at an age where they can follow be more fully described below in conjunction with
instructions. If the edema associated with the the different types of infection. However, special
infection is allowed to persist, and the extremity care should be taken to avoid incision lines that
is rested for too long, stiffness becomes a much cross exion creases at a right angle, since such
more signicant problem. incisions are prone to creating scar contractures.
Bruner incisions should also be avoided in the
digits, if possible, as there is a higher risk for tip
Surgical Treatment necrosis of aps with narrow bases in the setting
of a hand compromised by infection.
Surgical management of hand infections is simple Once a pocket of purulence is opened, cultures
in principle. If there is underlying purulence, it should be obtained. Since the etiologic organisms
must be evacuated expeditiously and thoroughly. can be varied, a good rule of thumb is to send at
The method of anesthesia is dependent upon the least aerobic and anaerobic cultures, fungal cul-
type of infection being treated. However, when tures, and acid-fast cultures. It is important to
deep infections are present, general anesthesia is recognize that most laboratories will require tissue
59 Pediatric Hand Infections
Table 3 Preoperative planning
OR table: supine on a standard OR bed with attached
hand table
Position/positioning aids: For digital infections that will
be approached from volarly, a lead or aluminum hand or
other hand positioning device is useful
Fluoroscopy: Fluoroscopy is not usually required for an
irrigation and debridement procedure, unless bony
debridement is anticipated, such as in cases of
osteomyelitis
Equipment
Low-pressure irrigation tubing (e.g., cystoscopy
tubing) versus bulb irrigation
Standard hand surgery tray, equipped with skin hooks,
traumatic and atraumatic forceps, dissecting scissors,
hemostats
Pediatric feeding tubes if closed catheter irrigation is
anticipated
Penrose or other appropriate small drains
Tourniquet: An upper arm tourniquet is almost invariably
used, though the arm should be exsanguinated with
gravity, and not with an elastic (Esmarch) bandage
for acid-fast cultures. The uncovered pocket of
purulence should then be copiously irrigated. It
is critical to thoroughly and systematically
explore all edges of the infected area and to
probe fairly aggressively in order to convince
oneself that no further pockets of purulence
remain. Once a thorough irrigation and debride-
ment of any necrotic tissue has been performed, a
loose closure over a drain or open packing should
be performed. The limb is then placed in a splint
for soft tissue rest for 2448 h.
Microbiology
Most epidemiologic studies agree that Gram-
positive organisms including Staphylococcus
aureus and beta-hemolytic streptococci are the
most common organisms responsible for infec-
tions in the adult hand, accounting for 4080 %
of cases (Fowler and Ilyas 2013; Tosti and Ilyas
2010; Houshian et al. 2006; McDonald
et al. 2011). However, what is less clear is the
extent to which this data translates to children.
Harness and Blazar (2005) reviewed a series of
31 pediatric patients with hand infections
and found that Staphylococcus aureus was the
1307
primary organism in 37 % and that the rate of
mixed and anaerobic infections was proportion-
ally higher in pediatric patients than in adults.
Based on their data, the authors recommended
the use of more broad-spectrum coverage as
empiric treatment for pediatric hand infections.
Also of particular concern is the rising preva-
lence of community-acquired methicillin-resistant
Staphylococcus aureus (MRSA). This trend is
well documented in the adult population, in
which community-acquired MRSA has been
reported to be present in between 30 % and
80 % of cases (Tosti and Ilyas 2010; Fowler and
Ilyas 2013; OMalley et al. 2009). More recently,
Chung et al. (2012) reported on a cohort of
415 patients younger than 15 years old with hand
infections, with the rate of MRSA-positive cultures
being 30 %. Because of this growing body of data,
careful attention should be paid to the local preva-
lence of MRSA. If the local rate is >10 %, then
empiric treatment for all hand infections should
avoid 1st-generation cephalosporins. For milder
cases, treatment with trimethoprim-sulfamethoxazole
orally is recommended. For more involved cases,
admission to the hospital and intravenous vancomy-
cin are indicated (Chung et al. 2012).
Though the most common organisms are
Staphylococcus spp. and Streptococcus spp., the
list of other organisms that can be implicated in
hand infections is long and diverse. Thus, care and
attention should be given to other risk factors that
might play a role (Table 1). Particularly in milder
or more indolent cases, consideration should be
given to the possibility of an atypical infection
from a fungal or mycobacterial organism. While
such atypical infections are rare, the treatment
required for eradication of the infection is markedly
different and must be tailored accordingly. Specic
atypical organisms will be discussed below.
Finger Infections
Acute Paronychia
An infection of the skin folds around the nail plate,
otherwise known as a paronychia, is one of the
most common infections in the pediatric hand.
1308
Both acute and chronic forms of paronychia exist.
While the acute infection can often be associated
with a hangnail, nail biting, splinters, or other
minor trauma, the chronic infections are often the
result of a persistently moist environment. In chil-
dren, this is usually the result of persistent thumb
or digital sucking (Harness and Blazar 2005;
Stone and Mullins 1968; Rayan and Turner
1989). In addition, acute infections may present
superimposed on a chronic infection.
It is important to determine historically
whether or not a chronic infection is present, as
the microbiology associated with a chronic
paronychia is signicantly different from that of
an acute paronychia. As such, the treatment is also
different. In the acute form, Staphylococcus
aureus is the usual causative organism. However,
because the etiology of paronychia in children can
be digital sucking, a mixed organism infection or
atypical organisms may be present. For chronic
infections, atypical organisms such as fungi are
usually implicated.
The typical presentation of a paronychia is local-
ized pain, erythema, and swelling along the
paronychial fold. While these infections are most
commonly found on only one side of the nail plate,
they can sometimes progress around the proximal
(eponychial) fold to include the other side in a
runaround abscess (Green et al. 2011).
Operative Technique
Treatment of acute paronychia in children is sim-
ilar to the treatment in adults (Table 4). If it is
caught in the earliest stages and only localized
erythema is present without obvious uctuance
or purulence, then treatment with oral antibiotics,
elevation, and warm compresses is appropriate
with close observation. However, if this treatment
has not been effective after 24 h or if there is any
evidence of purulence, then a more aggressive
approach is warranted. If the patient is able to
tolerate a local digital block, then this method is
preferred. However, in young children the use of
conscious sedation can relieve the anxiety of both
the child and the parents.
L. Kroonen
Table 4 Incision and drainage of acute paronychia
Surgical steps
A blunt instrument such as a Freer elevator can be used to
lift the affected paronychial fold
Alternatively, a longitudinal incision is made along the
fold
If obvious purulent material is present under the nail
plate, then a Freer is used to gently slide under the nail
plate, and either a portion or the entire nail plate is
removed
If the infection extends proximal to the eponychial
fold, then one or two longitudinal incisions are extended
in line with the paronychia, and the eponychial fold is
elevated to expose the underlying germinal matrix
Once the infected area is exposed, it is thoroughly
irrigated, with gentle debridement using the blunt Freer
A small strip of gauze or the native nail plate can then be
gently placed back under the eponychial fold to preserve
that space
Once anesthesia is achieved, surgical drainage
is performed. The method of drainage depends on
the location of the purulence, with the bottom line
of achieving complete surgical drainage of any
underlying purulence. In simple paronychia local-
ized to the paronychial fold, a Freer elevator can
be used to gently develop the plane between the
nail plate and the paronychia, or alternatively, a
longitudinal incision in line with the paronychial
fold is adequate to relieve tension. Once the infec-
tion progresses, purulence can track underneath
the nail plate. If this is seen, partial or complete
nail plate removal is necessary. Depending on
how proximally the infection tracks, the
eponychial fold can also be lifted through the
use of longitudinal incisions in line with the
paronychia (Fig. 3).
Chronic Paronychia
In the case of a chronic paronychia, the offending
organism must be identied. The clinical course of
a chronic paronychia is often indolent, with peri-
odic ares of symptoms that do not rapidly pro-
gress as in the acute infection. Treatment is often
difcult because of the mixed microbiology
59 Pediatric Hand Infections
Fig. 3 A longitudinal incision, in line with the nail plate,
can be used to decompress the paronychia as seen on the
index nger. If the infection tracks proximally to involve
the eponychial fold, then incisions can be extended parallel
to the paronychial folds to permit lifting the eponychial
fold away from the nail plate as seen on the long nger
responsible for these infections, which can include
bacterial, mycobacterial, and fungal components.
The mainstay of treatment for chronic
paronychial infections is topical creams that con-
tain a combination of antifungals and steroids
(3 % clioquinol in triamcinolone-nystatin mix-
ture). However, in refractory cases, marsupia-
lization is necessary. Marsupialization involves
removing a full-thickness, crescent-shaped seg-
ment of the skin proximal to the eponychial fold
(a pocket) that is then allowed to freely drain
and heal by secondary intention. This procedure is
done with or without the removal of the nail plate
depending on the involvement of the nail bed and
the nail plate itself (Canales et al. 1989;
McDonald et al. 2011; Green 2005).
Felon
A felon is a closed space infection involving the
pulp of the ngertip. The anatomy of the ngertip is
characterized by the presence of multiple septa,
each of which can create walled-off collections of
purulence if inoculated (McDonald et al. 2011).
With the ngers of the hand as the primary means
of connecting with the environment, it is no surprise
that these are some of the more common infections
seen in the pediatric hand. In adults, these infections
1309
are most commonly caused by Staphylococcus
aureus. However, the bacterial etiology of felons
in the pediatric population is not known.
The source of inoculation can be anything from
a plant thorn to a pet bite (in particular cat bites),
to penetration of the ngertip with a household
object such as a sewing needle. The presentation
involves throbbing pain of the affected nger,
accompanied by erythema and swelling localized
to the ngertip pulp. While sometimes the inocu-
lation wound can be seen, often there is no obvi-
ous wound present.
If the inoculated nger is caught early, the
infection may be treated effectively with eleva-
tion, oral antibiotics, and warm compresses. How-
ever, more commonly these infections have
already progressed to include an abscess (Canales
et al. 1989; McDonald et al. 2011). In these cases,
early surgical drainage is essential, since untreated
abscesses within the ngertip can quickly lead to
more extensive infections. The building pressure
of purulence within the pulp of the digit can cause
local spread to the underlying bone, leading to
osteomyelitis, or can travel more proximally to
seed the exor tendon sheath or the distal
interphalangeal joint resulting in a pyogenic
exor tenosynovitis or a septic arthritis, respec-
tively. These infections should be treated early
and aggressively to avoid such complications.
Operative Technique
Surgical treatment is performed under local anes-
thesia with or without conscious sedation or under
general anesthesia (Table 5). There is no agree-
ment on the optimal incision for surgical drainage,
though the authors preference is for a high lateral
incision placed just below the level of the nail
plate on the affected side (Fig. 4). This incision
prevents disturbance of the volar fat pad and
neurovascular bundle. Though in many cases,
the placement of the incision is based on the
location of a traumatic wound, when possible,
careful consideration of the placement of the inci-
sion can avoid signicant postoperative morbid-
ity. Thumb and small nger incisions are best
1310
Table 5 Incision and drainage of Felon
Surgical steps
If no obvious traumatic wound is present, then a high
lateral incision just beneath the nail is preferred
Once the skin incision is made, a blunt dissecting
instrument such as a hemostat is used to aggressively
break up loculations
Any purulence is drained. A small curette can be used to
gently debride the surrounding soft tissues
Copious bulb/angiocath irrigation is used to thoroughly
irrigate the wound
A small amount of plain or iodoform packing strip can be
gently packed into the wound in order to allow for
ongoing drainage
Bulky uffed gauze is then used to dress the wound, and
the hand and arm are immobilized in a volar resting splint
extending out beyond the tips of the ngers
Fig. 4 A high lateral incision located away from the
border of the hand or away from the surface involved
with pinch is preferred for drainage of a felon
made radially, while other digits should be
approached from the ulnar side to avoid the pri-
mary areas involved in pinch. Once the incision is
made, aggressive spreading with a small hemostat
or dissecting scissors is necessary to break up any
septa and ensure a complete surgical decompres-
sion. A small drain should be left in place for a day
or two to prevent additional accumulation.
Splinting, strict elevation, and appropriate antibi-
otics are then initiated. When there is clinical
evidence of improvement, usually at about 48 h
after drainage has been performed, the drain is
removed and the wound is allowed to heal by
secondary intention. A standard postoperative
protocol can be found in Table 7.
L. Kroonen
Table 6 Closed catheter irrigation of flexor tenosynovitis
Surgical steps
An oblique or transverse incision is made at the level of
the A1 pulley of the affected digit. The oblique incision
has the advantage of being extensile
Blunt dissection is used to get down to the proximal edge
of the A1 pulley
Any localized purulence is evacuated
A second incision is made in a high lateral position (as in
drainage of a felon) of the distal phalanx
A 5F pediatric feeding tube is then fenestrated with a 22G
needle along the entire course that will be in the nger
The pediatric feeding tube is then gently fed into the
exor tendon sheath (see Fig. 6b)
Irrigation is then performed by hooking the feeding tube
up to a 1012 cc syringe. Irrigation uid should pass
freely through the tube and exit through the distal wound.
At least 200 cc should be put through in the OR
The skin is closed at the proximal incision around the
tube. The distal wound is left open to drain
The hand is placed in a bulky soft dressing, and a volar
resting splint is applied to all digits for soft tissue rest
Further irrigation is performed on the ward with 1020 cc
of uid pushed through the tube three times daily for the
rst 4872 h
Pyogenic Flexor Tenosynovitis
Pyogenic exor tenosynovitis is a bacterial closed
space infection involving the exor tendon sheath
of the digit. Inoculation of the tendon sheath is
most commonly the result of penetrating trauma,
and this can occur at any point along the sheath.
Regardless of where the sheath is penetrated, the
closed nature of this sheath results in a rapid
spread of infection throughout the sheath.
The exor tendon sheath begins proximally at
the level of the A1 pulley and extends distally to
the distal interphalangeal joint. On the radial side
of the hand, the thumb exor tendon sheath runs
contiguous with the radial bursa, while the ulnar
bursa is contiguous with the small nger exor
tendon sheath. The radial and ulnar bursae then
communicate proximally via Paronas space,
which is the area supercial to the pronator
quadratus but deep to the exor tendons. Thus, a
exor tendon sheath infection can also rapidly
spread from the small nger through Paronas
space to the radial bursa and the thumb or in the
59 Pediatric Hand Infections
Table 7 Surgical drainage of hand infections
Postoperative protocol
Any skin closure, if performed at all, should be loose and
over some form of a drain
Dressings should be loosely applied to allow for easy
drainage of any additional accumulation of purulence
A splint for soft tissue rest should always be applied
Close collaboration with infectious disease specialists is
highly recommended if there is any question about the
organism or appropriate antibiotic therapy
Immobilization should be limited to 48 h, once control of
the infection has been gained
Occupational therapy is initiated for gentle range of
motion and edema control in patients who can comply
In more severe cases, inammatory markers such as a
CBC and C-reactive protein can be followed every other
day until a downward trend is noted
Close follow-up is warranted after discharge from the
hospital
opposite direction and create a horseshoe
abscess (McDonald et al. 2011).
Diagnosis of a exor tenosynovitis is based on
the classic cardinal signs of Kanavel. Kanavel
(Kanavel 1925) reported that pyogenic exor
tenosynovitis is characterized by four signs:
exquisite tenderness over the course of the exor
tendon sheath, the digit held in a semi-exed
posture, pain with passive extension of the digit,
and fusiform swelling of the entire digit. While all
symptoms might not be present, the constellation
of these symptoms is diagnostic and indicates the
need for immediate treatment (Fig. 5).
For cases that are caught within the rst
1224 h in otherwise healthy children, an attempt
may be made at nonoperative management with
splinting, elevation, and appropriate antibiotic
therapy. However, if there is any evidence of
systemic illness or overt signs of uctuance, sur-
gical drainage should be undertaken without
delay.
Operative Technique
Operative intervention depends, to some degree,
on the extent of the infection and the characteris-
tics of the traumatic wound. In milder cases,
closed catheter irrigation through a limited
1311
Fig. 5 Characteristic appearance of pyogenic exor teno-
synovitis is seen in this child with diffuse swelling of the
thumb who presented with exquisite pain with passive
stretch of the thumb (Courtesy of Theresa Wyrick, MD)
exposure can be performed with incisions proxi-
mally at the level of the A1 pulley and distally at
the level of the DIP joint (Table 6). A 5F pediatric
feeding tube can then be fenestrated with a hypo-
dermic needle, fed into the exor tendon sheath,
and sutured in place (Figs. 6 and 7). Copious
irrigation is then run through the tube to irrigate
the entire exor tendon sheath. After loose closure
of the wounds, the hand is then placed into a well-
padded splint, and the tube is left in place. Closed
irrigation is then performed with 1020 cc of
saline with or without lidocaine every 68 h over
the course of 23 days (Harris and Nanchahal
1999). For more severe cases, a full open exposure
to the exor tendon sheath is recommended.
Though a Bruner incision has been described, a
midlateral approach is preferable to avoid prob-
lems with necrosis of the tips of the Bruner aps.
At the base of the digit, a midlateral incision can
be carried obliquely across the A1 pulley in a
Bruner fashion.
Staphylococcus aureus is the most commonly
isolated organism, though particularly in children,
the offending organisms can be more diverse
depending upon the source that inoculated the
sheath. Animal and human bites might result in a
polymicrobial infection or more classic bacterial
etiologies associated with bite wounds such as
Pasteurella multocida. Kingella kingae has also
been isolated (Ceroni et al. 2013). Additionally,
1312 L. Kroonen

plant thorns have been noted to be associated with

atypical organisms such as Enterobacter spp. and
fungi such as Sporothrix schenckii and Alternaria
(Brady and Sommerkamp 2001). Cases with such
atypical organisms often are less aggressive than
those with Staphylococcus aureus, but can
become more chronic problems due to diagnostic
delays.

Deep Space Infections

Just as with a felon in the ngertip, the palm of the

hand has unique anatomy that can lead to the
development of closed space abscesses known as
deep space infections. These infections happen
in predictable spaces based on the anatomy of
the palm.
A prerequisite to understanding and identify-
ing these infections is a good knowledge of the
anatomic boundaries of the various closed spaces
of the hand, including the thenar space, the
midpalmar space, the hypothenar space, Paronas
quadrilateral space, the interdigital subfascial
spaces, and the dorsal subaponeurotic space
(Fig. 8).

The Thenar Space

The thenar space lies deep to the exor tendon of

Fig. 6 (a) In this early case of pyogenic exor tenosyno-
vitis, the puncture wound can be seen. (b) A limited
approach was made to expose the A1 pulley and the distal
extent of the exor tendon sheath, and a 5F pediatric
feeding tube was placed for closed catheter irrigation
the index nger and is contained dorsally by the
adductor pollicis. It is a closed spaced, with an
ulnar septum that separates this space from the
midpalmar bursa. On the radial side, the space is
limited by the thenar muscle fascia and the inser-
tion of the adductor pollicis. An infection in this

Hypothenar Space
Fig. 7 This cross section
through the palm Midpalmar Space
demonstrates the locations
of the thenar, midpalmar, Thenar Space
and hypothenar spaces
(From Greens Operative
Hand Surgery Figs 38a,
page 71, 5th ed)
59 Pediatric Hand Infections 1313

of these incisions, or will extend an incision from

dorsal through the commissure and continue into a
palmar incision. Any incision running parallel to
the commissure is discouraged as such an incision
can lead to contracture of the rst web space.
Once the skin is incised, careful dissection is
made down to the level at which purulence is
encountered. Complete evacuation of the space
is performed, again using a Freer or a small curette
to gently debride to the outer limits of the infected
space. The wound is then closed over a small
Penrose drain and splinted in a position of com-
fort. A standard postoperative protocol can be
found in Table 7.