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CURRENT CONCEPTS

Management of the Spastic Wrist and Hand in


Cerebral Palsy
Nels D. Leafblad, BS, Ann E. Van Heest, MD

CME INFORMATION AND DISCLOSURES


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Statement of Need: This CME activity was developed by the JHS review section editors Editors
and review article authors as a convenient education tool to help increase or affirm reader’s Ghazi M. Rayan, MD, has no relevant conflicts of interest to disclose.
knowledge. The overall goal of the activity is for participants to evaluate the appropri-
Authors
ateness of clinical data and apply it to their practice and the provision of patient care.
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ASSH Disclaimer: The material presented in this CME activity is made available by the Learning Objectives
ASSH for educational purposes only. This material is not intended to represent the only
methods or the best procedures appropriate for the medical situation(s) discussed, but
 Clarify the concept of neuroplasticity.
rather it is intended to present an approach, view, statement, or opinion of the authors
 Elucidate the clinical presentation of the wrist and hand among cerebral palsy patients.
 List classifications of upper extremity cerebral palsy.
that may be helpful, or of interest, to other practitioners. Examinees agree to participate
in this medical education activity, sponsored by the ASSH, with full knowledge and
 Examine the nonsurgical treatment including therapy for wrist and hand cerebral palsy.
awareness that they waive any claim they may have against the ASSH for reliance on any
 Discuss the surgical treatment and outcomes for the wrist and hand among cerebral
information presented. The approval of the US Food and Drug Administration is required palsy patients.
for procedures and drugs that are considered experimental. Instrumentation systems Deadline: Each examination purchased in 2015 must be completed by January 31, 2016, to
discussed or reviewed during this educational activity may not yet have received FDA be eligible for CME. A certificate will be issued upon completion of the activity. Estimated
approval. time to complete each JHS CME activity is up to 1 hour.
Provider Information can be found at http://www.assh.org/Pages/ContactUs.aspx. Copyright ª 2015 by the American Society for Surgery of the Hand. All rights reserved.

Research from the last 5 years on the pathophysiology and treatment of upper extremity
sequelae of cerebral palsy (CP) is presented. The development of new treatments of CP-
affected limbs, utilizing the brain’s inherent neuroplasticity, remains an area of promising
and active research. Functional magnetic resonance imaging scans have evaluated the role of
neuroplasticity in adapting to the initial central nervous system insult. Children with CP
appear to have greater recruitment of the ipsilateral brain for motor and sensory functions of
the affected upper limb. Studies have also shown that constraint-induced movement therapy
Current Concepts

From the Department of Orthopaedic Surgery and the Medical School, University of Minnesota, Corresponding author: Ann E. Van Heest, MD, Department of Orthopaedic Surgery,
Minneapolis, MN. University of Minnesota, 2450 Riverside Ave., Ste. R200, Minneapolis, MN 55454; e-mail:
Received for publication November 10, 2014; accepted in revised form November 20, 2014. vanhe003@umn.edu.

No benefits in any form have been received or will be received related directly or indirectly 0363-5023/15/4005-0032$36.00/0
http://dx.doi.org/10.1016/j.jhsa.2014.11.025
to the subject of this article.

 2015 ASSH r Published by Elsevier, Inc. All rights reserved. r 1035


1036 STEREOGNOSIS CHANGE IN CEREBRAL PALSY

results in localized increase in gray matter volume of the sensorimotor cortex contralateral to
the affected arm targeted during rehabilitation. Recent therapy interventions have emphasized
the role of home therapy programs, the transient effects of splinting, and the promise of
constraint-induced movement therapy and bimanual hand training. The use of motion labo-
ratory analysis to characterize the movement pattern disturbances in children with CP con-
tinues to expand. Classification systems for CP upper limb continue to expand and improve
their reliability, including use of the House Classification, the Manual Ability Classification
System, and the Shriner’s Hospital Upper Extremity Evaluation. Surgical outcomes have
greater patients’ satisfaction when they address functional limitations, also in addition to
aesthetics, which may improve patients’ self-esteem. Surgical techniques for elbow, wrist,
fingers, and thumb continue to be refined. Research into each of these areas continues to
expand our understanding of the nervous system insults that cause CP, how they may be
modified, and how hand surgeons can continue to serve patients by improving their upper
limb function and aesthetics. (J Hand Surg Am. 2015;40(5):1035e1040. Copyright  2015
by the American Society for Surgery of the Hand. All rights reserved.)
Key words Cerebral palsy, stereognosis.

T
HE PURPOSE OF THIS ARTICLE IS TO review litera- timing of the insult during development. Structural
ture from the last 5 years to update hand neuroplasticity has already been demonstrated in
surgeons regarding the pathophysiology and adult stroke patients undergoing neurorehabilitation.
treatment of upper extremity sequelae secondary to Several interventions are now being employed to
cerebral palsy (CP). By definition, CP is “a group of increase upper limb function in children with uni-
disorders of development of movement and posture lateral CP. Inguaggiato et al3 found that successful
causing activity limitations that are attributed to non- noninvasive rehabilitative strategies, including
progressive disturbances that occurred in the devel- constraint-induced movement therapy (CIMT) and
oping fetal or infant brain.”1 virtual reality therapy, result in enlargement of the
Etiologies can include fetal stroke, brain anoxia, primary hand motor area contralateral to the paretic
central nervous system (CNS) infections, maternal in- hand. Increased activation was also found in the
fections, or CNS congenital malformations. Population- contralateral sensory cortex, supplementary motor
based studies report prevalence estimates of CP ranging area, premotor cortex, and cerebellum. These plastic
from 1.5 to 4 per 1,000 live births, which makes it the changes correlate with the enhancement of motor
most common motor disability in childhood.2 Tradi- skills of the paretic upper limb.
tionally, the hand surgeon has treated the secondary Eyre et al4 examined brain plasticity utilizing
peripheral manifestations of the primary CNS insult, transcranial magnetic stimulation to characterize
because few CNS treatments exist. However, future corticospinal tract development in healthy children,
treatments of upper extremity pathology due to CP children with perinatal stroke with hemiplegia, and
may evolve by gaining a greater understanding of the bilateral lesions. Magnetic resonance imaging (MRI)
primary CNS insults and CNS plasticity from such in- and anatomical studies showed that ipsilateral corti-
sults. Considerable work has been done in the last cospinal axons from the noninfarcted hemisphere to
5 years in attempt to better understand these processes the paretic side undergo compensatory hypertrophy.
regarding upper extremity use and function. Such hypertrophy predicts severe impairment in the
Current Concepts

upper limb after 2 years. Similar to amblyopia, it


appears that the increased projections from the ipsi-
NEUROPLASTICITY lateral cortex actually competitively displace the
The functional and structural changes that take place remaining projections from the contralateral (injured)
after insult to the CNS are part of a process referred cortex. Krageloh-Mann and Cans5 state that the
to as adaptive plasticity. These changes occur in an healthy hemisphere plays an important role after
attempt to offset or improve functions compromised unilateral insults. They confirm that, in the motor
by the pathological insult, whether they arise from system, there is compensatory recruitment of ipsilat-
malformations, ischemia, or parenchymal lesions. eral tracts with limited functionality in the affected
Mechanisms of plasticity differ depending on the limb.

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STEREOGNOSIS CHANGE IN CEREBRAL PALSY 1037

Holmström et al6 have reported that, of the various constraining the less-affected upper limb with CIMT
lesions responsible for CP, the lesion associated with may lessen primary motor cortex activity controlling
the most favorable hand function is white matter both upper limbs—a suboptimal therapy. Specula-
damage of immaturity, in which there is mild white tively, bimanual training may be a more appropriate
matter loss and the presence of contralateral motor therapy for these children, but its efficacy may depend
projections to the paretic hand. The most impaired on interhemispherical connections.
hand function was observed in children with ipsilat-
eral motor projections, regardless of the type of
damage. In addition, van de Winckel et al7 examined PATIENT EVALUATION
brain activation patterns in children with unilateral Several studies in the last 5 years describe advances in
CP compared to normally developing children dur- motion laboratory assessment for evaluation of chil-
ing active and passive movement as well as tactile dren with upper extremity CP. By definition, CP is a
stimulation. They concluded that, in children with movement disorder, but the type of movement im-
CP, there is an increased reliance on the ipsilateral pairment may clinically manifest as spastic, flaccid,
brain for motor and sensory function to the paretic dystonic, or a combination thereof. In addition, the
limb. manifestations of CP may vary in the different seg-
Using MRI and transcranial magnetic stimulation, ments of the limb: shoulder, elbow, forearm, wrist,
Mackey et al8 found decreased intracortical and in- fingers, and thumb.
terhemispherical inhibitory mechanisms in children Fitoussi et al11 report using a specific kinematic
with CP. More intact inhibition correlates with protocol to measure the ability of hemiplegic patients
higher limb function. Hopefully, interventions can be with CP to carry out simple daily tasks before and
developed that would enhance intracortical and after therapy with either botulinum toxin injection or
interhemispherical inhibition in order to restore the surgery. The study found that, as problems in the
excitatory balance in the brain in patients with CP. forearm, wrist, and thumb were addressed and im-
Such interventions are already being explored in the proved with therapy, so did the proximal kinematic
adult stroke population. problems. This suggests that proximal deficits could
be related to compensatory movement strategies and/
Role of therapy based on neuroplasticity or cocontractions and should be addressed second-
CIMT, a rehabilitative modality rooted in neuro- arily after treatment of distal issues.
plasticity, has been used for years in the adult pop- Klotz et al12 report that motion analysis is a useful
ulation and has more recently been shown to improve clinical tool for evaluating both coordination and
upper extremity functionality in children with CP. range of motion limitations. They describe use of a
Sterling et al9 found that CIMT resulted in localized fingertip reach task, in which the child touches the
increase in contralateral gray matter volume of the top of a bottle with the index fingertip followed
sensorimotor cortex during rehabilitation. In addition, immediately by touching the nose tip. This analysis
increased gray matter volumes were observed in the shows movement deviations due to lack of coordi-
ipsilateral sensorimotor cortex and contralateral hip- nation very clearly for children with CP.
pocampus. There was a significant improvement in Jaspers et al’s study13 of spatiotemporal and ki-
spontaneous use of the upper extremities in daily nematic parameters in 3-dimensional motion anal-
activities following CIMT. Although not yet proven, ysis revealed that children with hemiplegic CP have
there seems to be a causal relationship between in- longer movement durations and less straight tra-
creases in gray matter volume and the magnitude of jectories in upper extremity movements. Compa-
motor improvement. red with normally developing children, children
Gordon et al10 have suggested that there may be with CP also have more wrist flexion and less
Current Concepts

limitations to the utility of CIMT, particularly when elbow extension and forearm supination during
there is early-onset damage. When ipsilateral pro- reach, reach to grasp, and gross motor tasks. In
jections from the unaffected hemisphere reorganize addition, children with CP have increased trunk
and are strengthened to the paretic hand, and con- movements and reduced shoulder elevation during
tralateral projections are weakened, the patients reach activities.
typically have more adversely affected hand function. Motion laboratory analysis coupled with dynamic
The earlier the onset of the damage, the greater the electromyography testing was used in tendon transfer
reorganization that occurs. In children with sub- surgery.14 Van Heest et al14 compared the preoper-
stantial ipsilateral corticospinal tract reorganization, ative and postoperative firing pattern of the flexor

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1038 STEREOGNOSIS CHANGE IN CEREBRAL PALSY

carpi ulnaris (FCU) in children treated with an FCUe


TABLE 1. Manual Ability Classification System
toeextensor carpi radialis brevis tendon transfer. (http://www.macs.nu/)
Before surgery, the most common pattern seen was
activation of the FCU during grasp and relaxation of Level Description
the FCU during release. This pattern did not change I Handles objects easily
in 6 out of 7 patients after surgery, suggesting that the II Handles objects with reduced quality and speed
FCU does not change phase after its transfer. III Handles objects with difficulty requiring modification
Classification systems IV Handles objects only in adapted situations
V Does not handle objects
Early detection and classification of hand abnormal-
ities in CP can guide operative and nonoperative
therapies. Various classification systems are available
for this process. The Manual Abilities Classification movements, and a substantial amount of time is spent
System (MACS) (Table 1) is a commonly used tool in training. When considering the various noninva-
and evaluates the child’s ability to handle objects in sive interventions for unilateral upper extremity CP,
daily activities. The House functional classification15 Taub and Uswatte21 suggest that CIMT and bimanual
is useful in describing grip function in each hand training have demonstrated the most efficacy and
separately. The Zancolli classification of active finger clinical practicality for improving functionality. Both
and wrist extension, along with the spastic thumb- papers advocate continuing at-home therapy to
in-palm deformity classification of House, estimates maximize clinical improvement.
dynamic spasticity of the hand. A recent article Jackman et al,22 in a systematic meta-analysis,
evaluated hand function in 367 children using these evaluated the effectiveness of hand orthoses in
common classification systems.16 Gajewska et al17 children with CP. Although they found that the use
used the MACS system and noted that the presence of hand orthoses in addition to therapy may offer a
of epilepsy in children with CP is associated with small benefit for manual skill development, this ef-
worse manual function, limitations in conscious fect diminishes 2 to 3 months after discontinuation
motor functions, and increased mental impairment. of orthosis use. Orthoses can create discomfort and
Compagnone et al18 compared the gross motor cosmetic problems. Consideration of these factors
function classification system, expanded and revised, should influence the clinical appropriateness of
the MACS, and the Communication Function Clas- orthosis use. Further methodical research is neces-
sification System as 3 systems that are used to clas- sary to determine the overall effectiveness of hand
sify functional levels of children with CP. These orthoses to improve outcomes.
3 systems correlate strongly with one another and A randomized, double-blind, placebo-controlled
complement each other in describing the complete study by Koman et al23 evaluated the short-term
functional profile of CP. In addition, an intelligence effects of botulinum toxin injections for treatment
quotient test seems to influence the global functional of upper extremity spasticity in children with CP.
profile of CP patients. Another classification schedule Study participants underwent an average of 3 in-
has been developed to allow clinicians to assess spon- jections based on their individual spasticity patterns.
taneous function, dynamic positioning, and child’s A higher percentage of children treated with botu-
ability to perform grasp-release maneuvers. This is linum toxin injections showed improvement in the
known as the Shriners Hospital for Children Upper Melbourne assessment after 6 months when compared
Extremity Evaluation (SHUEE), a validated video- with children receiving placebo. They concluded that,
based tool.19 when surgery is not indicated, repeated botulinum A
Current Concepts

toxin injections are safe and efficacious in providing


NONSURGICAL TREATMENT AND OUTCOMES short-term functional improvement of children’s upper
Nonsurgical treatment options for the upper extremity extremity spasticity.
in CP have primarily focused on physical therapy,
splinting, and botulinum toxin injections. Sakzewski
et al20 recently reported that the most effective SURGICAL TREATMENT AND OUTCOMES
rehabilitative programs have several elements in Two treatment outcomes that have not often been
common: therapy is goal directed, measureable goals evaluated in surgical treatment of the CP upper ex-
are identifiable by children and caregivers, motor tremity are self-esteem and aesthetics. Riad et al24 have
training is focused on activities rather than individual advocated that health care providers should take into

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STEREOGNOSIS CHANGE IN CEREBRAL PALSY 1039

account the mental health of patients with CP when elbow flexion contractures,29,30 swan neck de-
making treatment decisions. Although movement de- formities,31 and thumb-in-palm deformity.32 An al-
viations in upper and lower extremities often occur gorithm for treatment of elbow flexion contractures
simultaneously in patients with unilateral CP, the au- has been outlined by Carlson and Carlson.31 The
thors report that upper extremity deviations correlate amount of preoperative contracture must be accoun-
most with lower self-esteem. Even in high-functioning ted for when determining the extent of elbow muscle
patients with mild CP, self-esteem may be adversely lengthening. In this study, patients with fixed elbow
affected by such deviations. Elbow flexion deformity is deformities less than 45 underwent partial length-
the main contributor to decreased self-esteem, when ening, whereas those with fixed deformities greater than
compared with shoulder flexion, shoulder abduction, 45 underwent full release. A direct correlation exists
and wrist flexion deformity. between increasing age and the degree of preoperative
Similarly, Makki et al25 have reported that co- contracture; patients 12 years of age or older are 5.5
smetic appearance after surgical correction may have times as likely to have fixed contracture (passive
a greater influence on patient’s satisfaction than extension  45 ) than patients younger than 12 years
functional outcome. Older children (> 12 y) are more of age. The appropriate surgical treatment, based
self-conscious about the appearance of their hand. on the degree of preoperative contracture, can
These authors suggest that it is important not to un- greatly improve elbow flexion posture angle at
derestimate the psychosocial implications of hand ambulation, active and passive extension, and total
deformity. Libberecht et al26 reported that patient range of motion. Van Heest32 described use of thumb
satisfaction is significantly higher in patients with adductor release, extensor pollicis longus rerouting
considerable cosmetic improvement after surgery. It and metacarpophalangeal stabilization procedures
is not unreasonable to suspect that patients may be depending on the preoperative thumb-in-palm
seeking treatment primarily for cosmetic reasons. As deformity.
such, even if surgical intervention is not expected to Research from the last 5 years on the pathophysiology
deliver considerable functional improvement, cor- and treatment of CP upper extremity sequelae is pre-
recting the deformity for cosmetic reasons can be a sented in this update article. Functional MRI scans have
worthwhile endeavor for the patient. evaluated the role of neuroplasticity in adapting to the
Gong et al27 have reported on the use of the initial CNS insult. Children with CP appear to have
MACS to categorize outcomes after surgery. They greater recruitment of the ipsilateral brain for motor and
reported that, by dichotomizing the MACS into high sensory function of the affected upper limb. Studies have
and low levels for baseline hand function prior to also shown that CIMT results in localized increases in
surgery, one can effectively predict surgical outcome, gray matter volume of the sensorimotor cortex contra-
regardless of the specific type of surgery performed. lateral to the affected arm targeted during rehabilitation.
Although patients in both groups experience sub- The development of new treatments of CP-affected
stantial improvement, patients with high MACS limbs, utilizing the brain’s inherent neuroplasticity, re-
levels have greater improvements in function (utiliz- mains an area of promising and active research.
ing the House functional scale) and overall satisfac- Recent reviews of therapy interventions have
tion, but less improvement in hygiene status emphasized the role of therapy extending into home
compared with those with low MACS levels. programs, the transient effects of fabricating an
Using the SHUEE to assess therapeutic outcomes, orthosis, and the promise of CIMT and bimanual
Smitherman et al28 found that single-event multilevel hand training. The use of motion laboratory analysis
surgery for children with hemiplegic CP can signifi- to characterize the movement pattern disturbances
cantly improve thumb, finger, wrist, and forearm present in children with CP continues to expand.
segmental positioning as well as spontaneous func- Classification systems for upper limb involvement
Current Concepts

tion, but does not significantly change grasp-release due to CP continue to expand and improve their
ability. In addition, the single-event multilevel sur- reliability, including use of the House classification,15
gical approach provides the patients and families with the MACS, and the SHUEE. Outcomes after surgery
the benefits of avoiding staged surgical interventions. have been reported to have greater patient satisfaction
The SHUEE has been demonstrated to be reliable in when they address not only functional limitations but
clinical decision making and in assessment of func- also aesthetics, which may improve patients’ self-
tional outcomes after surgery. esteem. Lastly, surgical techniques continue to be
Other specific surgical technique articles and their refined for treatment of the elbow, wrist, fingers, and
results have been published regarding treatment of thumb. Continued research into each of these areas

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1040 STEREOGNOSIS CHANGE IN CEREBRAL PALSY

continues to expand our understanding of the CNS 16. Arner M, Eliasson AC, Nicklasson S, Sommerstein K, Hagglund G.
Hand function in cerebral palsy. Report of 367 children in a
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how we, as hand surgeons, can continue to serve 2008;33(8):1337e1347.
our patients to improve their upper limb function and 17. Gajewska E, Sobieska M, Samborski W. Associations between
esthetics. manual abilities, gross motor function, epilepsy, and mental capacity
in children with cerebral palsy. Iran J Child Neurol. 2014;8(2):
45e52.
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Current Concepts

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