FK UNISMA

EMERGENCY PEDIATRI
HERNIAS AND
ABDOMINAL WALL
DEFECT
Congenital/ Posterolateral Diaphragmatic Hernia
(CDH)
1. One of most severe conditions of neonate
2. Defect in diaphragm during early fetal development
3. Left side most commonly affected
4. Content of the hernia:
- small bowel
- colon
- spleen
- stomach
- liver, kidney, tail of pancreatic
Embryology

Week89
division of coelomic cavity into the pleural
and peritoneal cavity by the diaphragm; a
triangular area in the posterolateral site was left
open.

Week1012
herniation occur through this opening into the
pleural cavity at the return of midgut



1Hypoplasia of the lung

Pulmonary weight (ipsilateral+contralateral)
Alveoli number
Hypertrophy of the media of pulmonary arteriole
Resistance of the vessels

Pathophysiology
2.Pulmonary hypertension
Abdominal viscera into the thoracic cavity
compression of the lung
PaO2 , PaCO2 acidosis, hypoxemia
PH<7.30 pulmonary vessels spasm
vessel resistance, right to left shunting
through patent ductus arteries and foramen
ovale
Aggravate acidosis and hypoxemia in the body
circulation (fetal circulation syndrome)

Clinical manifestations

1. Severe respiratory distresscyanosis, vomit
2. Breath sounds: diminished on the side of hernia
3. Heart sounds: deviated to the contralateral
chest
4. Scaphoid abdomen
Diagnosis

Prenatal diagnosis

Ultrasound : abdominal organ the fetal visibel
in chest.
After birth

X-ray film:

Typical air-filled stomach and bowels in the chest,
which continues into the abdominal cavity.
Diaphram can not be seen at the affected side.
Absence or scarcity of intestine in the abdominal cavity

Treatment
Before delivery: cortisone could induce the
maturation of pulmonary tissue
Preoperative preparation:
1mechanical ventilation with pure oxygen
2nasogastric tube to decompress
stomach and intestine
3semi-supine and inclined to
the ipsilateral side keep warm
4 i.v. fuild, correction of acidosis
(5surgical repair


CONGENITAL DEFECTS OF
ABDOMINAL WALL
( Omphalocele & Gastroschizis )
HISTORY
14
A.C. Celsus ( First Century AD ) : First Report of
newborns with Abdominal Wall Defects.
Ambroise Pare ( 16
th
Century ) : First description
of Omphalocele.
Lycosthenes ( 16
th
Century ) : First description of
Gastrochizis.
Taruffi ( 1894 ) : Introducing the term
Gastrochizis.
Massabau & Guibal, Moore & Stokes, Bernstein
: Described specific clinical entity to
Abdominal Wall defects.
HISTORY
15
Hey ( 1802 ) : First successful repair of Omphalocele.
Visick ( 1873 ) : First successful repair of Gastrochizis.
Ahfeld ( 1899 ) : Described painting an Omphalocele
sac with Alcohol .
Max Grob ( ? ) : The use of Mercurochrome.
Ein & Shandling ( ? ) : The use of Semi permeable
Artificial Membrane.
Olhausen & Gross ( 1887 ) : The use of Skin flap after
membrane removal.
Schuster ( 1967 ) : Introducing reduction of large
Omphaloceles with prosthetic material.
ABDOMINAL WALL DEFECTS SPECTRUM
16
Omphalocele ( Lateral Fold )
Cephalic Fold Omphalocele
( Pentalogy of Cantrell )
Caudal Fold Omphalocele ( Cloacal
Extrophy, Vesicointestinal Fissure )
Gastroschizis
Ectopia Cordis Thoracis
Umbilical Cord Hernia
NORMAL EMBRYOLOGY
17
3 Weeks gestation : the flat cellular disk of
embryo develops four folds enclose the
body cavities.
Two lateral folds form the pleuroperitoneal ( PP
) canal meet anteriorly in the midline.
The cephalic fold will takes its place within
anterior chest wall, and also carries the Septum
Transversum continues posteriorly divides
the PP canal into pleural and peritoneal
cavities.

NORMAL EMBRYOLOGY
18
The Caudal Fold brings with it the
developing Bladder or Allantois started
off distal to the anus.
During the fold process, the gut tube has
formed along the length of the embryo.
5 Weeks Gestation : the gut tube begin to
elongate and develop within the umbilical
coelom.
10 Weeks Gestation : the gut returns from the
umbilical coelom to peritoneal cavity
undergoes rotation and fixation.
NORMAL EMBRYOLOGY
CEPHALO CAUDAL FOLD
19
NORMAL EMBRYOLOGY
CEPHALO CAUDAL FOLD
20
NORMAL EMBRYOLOGY
LATERAL FOLD
21
OMPHALOCELE EMBRYOLOGY
22
Failure of the mostly lateral body folds
to complete the journey, and defect
are always at the umbilicus ( midline )
Failure of return of the gut from the
umbilical coelom to peritoneal cavity.
Stopped at Extra coelomic Evisceration
Stage
Occurs early in embryogenesis
affect other organ system
associated anomalies frequently.
GASTROCHIZIS EMBRYOLOGY
23
Abnormal dissolution of abdominal wall
vascularization Failure of the umbilical
coelom to develop The elongating intestine
has no space to expand, and ruptures out the
body wall.
Occurs just the right side of the umbilicus
Because the right side is relatively unsupported,
as a result of complete abnormal dissolution of
the right umbilical vein ( at 4 weeks gestation ).
GENETICS CONSIDERATION
24
Rare reports of Abdominal wall defects, mainly
omphalocele, occuring in families and even in
twins.
No specific genes have been identified.
More likely to be associated with
Chromosomal Anomaly ( Trisomy 13, 18, or 21 )
Usually do occur as part of syndromes (
Beckwith - Wiedemann, Gershoni - Baruch,
Donnai Barrow, or Fryns Syndrome )

CLINICAL FEATURES : OMPHALOCELE
25
Omphalocele = Exomphalos = Amniocele =
Coelosmia
The second most common of the abdominal
wall defects.
Incidence : 1 2.5 / 5000 live birth.
Male preponderance
Usually happened in full term baby.
CLINICAL FEATURES : OMPHALOCELE
26
Centrally Abdominal Wall defect, larger than 4
cm in diameter, and always covered by a
translucent membrane from which the
umbilical cord extends.
The outer membrane layer is formed by
amnion, the inner layer by peritoneum, with
mesenchymal tissue, called Wharton jelly
among them.
Variation in defects size. Smaller defect, better
in outcome.
27

28
CLINICAL FEATURES : OMPHALOCELE
29
Normal muscles of abdominal wall, and the
sac usually contains the liver, midgut, and
frequently other organ such as spleen, or
gonad.
Associated with anomalies condition, such as
Beckwith - Wiedemann, Prune Belly, Gershoni
- Baruch, Donnai Barrow, Down, or Fryns
Syndrome.

DIFFERENT DIAGNOSIS : OMPHALOCELE
30
Umbilical Hernia
( The defect is covered by normal skin not a
membrane. Rarely present at birth, but
usually becoming apparent in the first week
or months of life )
Persistent Vitelline / Omphalomesenteric Duct
( There are no umbilical cord in this defect )

CLINICAL FEATURES : GASTROCHIZIS
31
Gastroschizis ( Greek ) = Belly Cleft
The most common of the abdominal wall
defects.
Incidence : 2 5 / 10000 live birth.
Male preponderance
Usually happened in premature baby, Low Birth
Weight, or baby with respiratory problems. It is
associated with Intrauterine Distress, and
younger mother.

CLINICAL FEATURES : GASTROCHIZIS
32
Periumbilical ( right ) Abdominal Wall defect,
less than 4 cm in diameter, with a skin bridge
may be present between umbilical cord and
the defect.
Normal muscles of abdominal walls, with no
sac or remnant of a sac.
Herniation of midgut, stomach, and
occasionally gonad. Liver is very rare.
33
34

CLINICAL FEATURES : GASTROCHIZIS
35
At birth, the bowel in Gastroschizis appear
perfectly normal, but about 20 min. later it
begins thickened and covered with a fibrinous
exudate matted together so that individual
loops cant be distinguished.
Associated with anomalies related to the
midgut, Intestinal atresia, Malrotation, or
Undescended Testicles

DIFFERENT DIAGNOSIS :
GASTROSCHIZIS
36
Rupture of Omphalocele sac
( The umbilical cord is on the tip of the
defect, with is covered by ruptured
membrane )

DEFECTS PREVIEW
Components Omphalocele Gastrochizis
Location Umbilical cord Lateral ( Right ) to
cord / Paraumbilical
Defect Size Large ( 2 10 cm ) Small ( 2 4 cm )
Cord Inserts in sac Normal insertion ( to
left of defect )
Abdominal Cavity Small Normal
Bowel Normal Matted, inflamed
Malrotation Present Present
Gastrointestinal
Function
Normal Prolonged ileus
Associated Anomalies Common
( 30 70 % )
Unusual
Outcome Good Good
37
DIAGNOSTICS :
ANTENATAL INTERVENTION
38
Prenatal Ultrasound Detection
Irregular Bowel Contour, or Dilated bowel free
in amniotic fluid adjacent to the umbilical
insertion
Amniotic Fluid and Serum Test
Elevated AFP ( Both Maternal serum and
Amniotic Fluid ), and Elevated Amniotic Fluid
AcH Esterase have been correlated with this
defects ( when there is no Myelomeningocele
)

MANAGEMENT OF OMPHALOCELE
CONSERVATIVE / INITIAL CARE
39
Maintenance of body temperature.
Naso-gastric Tube insertion to keep the
intestines decompressed.
Ventilator support and supplemental
oxygen.
Intravenous fluid are provided at
maintenance rate
Prophylactic Antibiotics
Keep the Omphalocele sac intact, wet, and
sterile
Cardiologic evaluation and
Echocardiography are in order.

MANAGEMENT OF OMPHALOCELE
SURGICAL DEFECT CLOSURE
40
Primary Closure
Should be performed for small / moderate
sized defect.
Reduction of Abdominal contents, Incision of
Omphalocele sac, Abdominal Inspection,
Mal rotation correction, and Skin Closure.


MANAGEMENT OF OMPHALOCELE
SURGICAL DEFECT CLOSURE
41
Delayed Primary Closure
Especially for giant Omphalocele, with large
defect that need staged reduction of the
intestinal contents by performing Silastic silo
sheeting
( Extraabdominal Pouch ), with Antibiotics or
Silver Sulfadiazine is applied around the
edges as a dressing.
MANAGEMENT OF GASTROSCHIZIS
CONSERVATIVE / INITIAL CARE
42
Maintenance of body temperature.
Naso-gastric Tube insertion to keep the
intestines decompressed.
Ventilator support and supplemental
oxygen.
Intravenous fluid are provided at
maintenance rate, or rehydrating rate if its
needed.
Prophylactic Broad Spectrum Antibiotics
Intestinal contents closure with Saran Wrap,
Handi Wrap, or Bogotas Bag.

MANAGEMENT OF GASTROSCHIZIS
SURGICAL DEFECT CLOSURE
43
Primary Closure
Should be done after evacuating intestinal
contents.
Delayed Primary Closure
Should be performed if primary closure
could not be done or failed. Using Silastic
Silo Pouch.
44

COMPLICATION POSSIBILITIES
45
Omphalocele
Related with associated anomalies, Rupture
of Omphalocele sac.
Gastroschizis
Related with prematurity, or gastrointestinal
tract anomalies, Dehydration, Hypothermia,
and Sepsis
PROCESUS VAGINALIS
REMNANTS
Inguinal Hernias

Defined as a protrusion of a portion of an
organ or tissue through an abnormal
opening .
Inguinal hernias in children : indirect type.
An indirect inguinal hernia protrudes
through the internal inguinal ring, within the
cremaster fascia, extending down the
spermatic cord.
The direct hernia protrudes through the posterior
wall of the inguinal canal, i.e., medial to deep inferior
epigastric vessels, destroying or stretching the
transversalis fascia..

The Embryology

The indirect inguinal hernia is as follows
the duct descending to the testicle is a small
offshoot of the great peritoneal sac in the
lower abdomen.
During the third month of gestation, the
processus vaginalis extends down toward the
scrotum and follows the chorda gubernaculum
to the scrotum.
During the seventh month, testicle descend into
the scrotum, processus vaginalis forms a
covering for the testicle and the serous sac in
which it resides.
At about the time of birth, the portion of the
processus vaginalis between the testicle and the
abdominal cavity obliterates, leaving a peritoneal
cavity separate from the tunica vaginalis that
surrounds the testicle

Incidence
Approximately 1-3% of children.

Premature babies (9-11%) is higher than full-
term (3-5%), with a dramatic risk of
incarceration (30%).
Diagnosis
Typical patient intermittent lump or bulge in
the groin, scrotum, or labia noted at times of
increased intra-abdominal pressure.
If a loop of bowel entrapped (incarcerated),
develops pain followed by signs of intestinal
obstruction.
If not reduced, compromised blood supply
(strangulation) leads to perforation and
peritonitis.
Most incarcerated hernias in children can be
reduced
Treatment
Simple high ligation of the sac

Elective herniorrhaphy is treatment of choice.

Risk of incarceration, repair should be undertaken
shortly after diagnosis.

Pediatric patients are allowed to return to full activity
immediately after hernia repair.

Patients presenting with incarceration should have
an attempt at reduction (possible in greater than 98%
with experience), and then admission for repair
during that hospitalization.

Bilateral exploration is done routinely by most
experienced pediatric surgeons.

Recently the use of groin laparoscopy through the
hernial sac permits visualization of the contralateral
side.

Hydroceles

A hydrocele is a collection of fluid in the space
surrounding the testicle between the layers of the
tunica vaginalis.

Hydroceles can be scrotal, of the cord, abdominal, or
a combination of the above.
A hydrocele of the cord is the fluid-filled remnant of
the processus vaginalis separated from the tunica
vaginalis.

A communicating hydrocele is one that
communicates with the peritoneal cavity by way of a
narrow opening into a hernial sac.

Some are associated with an inguinal hernia. They
are often bilateral, and like hernias, are more
common on the right than the left.
Hydrocele noncommunican Hydrocele communican
Most hydroceles will resolved spontaneously by 1-2
years of age.

After this time, elective repair can be performed at
any time.

Operation is done through the groin and search
made for an associated hernia.

Aspiration of a hydrocele should never be
attempted.
INVAGINASI
Intusussepsi atau Invaginasi sering terjadi
pada bayi dan anak, dimana satu segmen usus
mengalami konstriksi oleh gelombang
peristaltik dan tiba tiba masuk ke dalam
segmen distalnya ( Dayal dan DeLellis ,1989).
Ujung usus yang masuk : intussuseptum
Bagian usus yang menerima : intussussepiens
Intussussepsi pertamakali dipublikasikan oleh
Paul Baebette pada pertengahan abad ke 17,
Salah satu sebab obstruksi usus

Tindakan pembedahan

Invaginasi usus ini akan diikuti oleh mesenterium yang
berisi pembuluh darah, kelenjar limfe, saraf serta
lemak dibelakangnya.
Terjadi strangulasi pembuluh darah dan limfe
Edema jaringan usus,
Jaringan mukosa membengkak berisi darah
dan mukus produksi sel goblet.







Red Current Yelly Stools .


Lebih lanjut darah dan mukus masuk ke
dalam lumen keluar bersama feses,
sehingga feses merah dan berlendir
Jika keadaan ini berlanjut dapat terjadi nekrosis
dan gangren usus (Ong dan Beasley, 1990 ;
Spitz,1990 ).
Pada Invaginasi terjadi :
cedera vasa mesenterika,
pertumbuhan bakteri berlebihan,
malabsorbsi,
translokasi kuman ( Spitz, 1990 ).
Semua keadaan ini perlu dipertimbangkan pada
penanganan kasus invaginasi.
Keterlambatan diagnosis penatalaksanaan
penderita berbeda satu dengan yang lain disertai
resiko kematian menjadi besar.


Abad ke 17, Paul Barbatte dari amsterdam menulis
tentang intussussepsi dan dapat direduksi secara
operatif.

Tahun 1876 Hirschprung dari copenhagen telah
mempublikasikan beberapa laporan mengenai
reduksi intussussepsi dengan menggunakan
tekanan hidrostatis. Hasilnya mempunyai
keunggulan dibanding terapi operatif selama 70
tahun berikutnya.




SEJARAH
Tahun 1913 Ladd mempublikasikan reproduksi
radiografi yang pertama, dengan menggunakan
kontras enema pada intussussepsi. Dia menerangkan
bahwa, diagnosis mungkin dapat ditegakkan pada
kasus ragu ragu, tetapi prosedur radiografi ini tidak
ditujukan untuk terapi.
Tahun 1927 di Amerika Serikat Rettan dan Steven
melaporkan keberhasilan dengan menggunakan
kontras enema. Paul Laquen dari francis, Olsson dan
Pallin dari Skandinavia juga telah melaporkan
pengalamanya dengan menggunakan metode ini.



Laki-laki dan perempuan 3 : 2.

Kasus intussussepsi terjadi dibawah umur 1 tahun
sebesar 65 %,

Jumlah yang terbanyak ditemukan pada usia 5-9 bl
( Mark, 1979 ).


INSIDENSI
Intussussepsi dapat ditentukan berdasarkan
:

a. Atas dasar perjalanan penyakitnya:
Gibson, Dockerty dan Dixon
membagi dalam 3 macam :
1. Akut : gejala-gejala terjadi kurang dari
1 minggu
2. Sub akut : gejala-gejala terjadi antara 1-2
minggu
3. Kronik : gejala-gejala yang sudah berlang
sung lebih 2 minggu.

b. Atas dasar penampilan gejala :
Sven Berghdahl at al, membagi intussussepsi menjadi
2 macam :
1. Intussussepsi yang typis, yakni intussussepsi
dengan gejala yang khas yang mudah
dikenal antara lain muntah-muntah, nyeri perut,
massa abdomen dan perdarahan perectal.
2. Intussussepsi yang atypis, golongan ini
menampakkan gejala-gejala yang bervariasi dari
gambaran diatas, sehingga kadang kadang
terlambat didiagnosa. Golongan ini baik dengan
operatif maupun dengan ba-enema mudah
tereduser secara spontan, terdapat lesi spesifik,
mudah rekurens dan sering menjadi kronis.

c. Klasifikasi atas dasar lokalisasi :
Ellis menggolongkan intussussepsi dalam :
1. Enteric : adalah intussussepsi usus kecil ke
usus kecil.
2. Colic : adalah invaginasi colon ke colon
(colo-colic).
.

3. Entero-colic : adalah invaginasi ileum kedalam
colon yang terdiri dari :
a. Ileocaecal : intusssussepsi dimana valvula
ileocaecal merupakan apek dari intussussep
tum
b. Ileocolic : intussussepsi ileum terminal dima
na apek melewati valvula ileocaecal ma
suk kedalam colon.
c. Ileo-ileo caecal : intussusseposi ileo-ileal, yang
mendesak kedalam valvula ileocaecal dimana
valvula ileocaecal berupa apek dari intussu
sseptum.

4. Multiple intussussepsi dan retrograde
5. Appendicular ( jarang )
6. Gastroduodenal.
7. Yeyunogastrik.

Insiden tertinggi intussussepsi adalah type :
enterocolic/ileosekalis,




Mencapai 95 % dari kasus ( Mark, 1979 ).
PENYEBAB

Pada anak-anak: Umumnya tak diketahui
penyebabnya sehingga ada yang menggolongkan
idiopatik atau primer.

Orloff meneliti dari 1424 kasus pada anak:
95 % kausa tak diketahui
5 % divertikel Meckels, polip, duplikasi usus.



Faktor pemyebab dari golongan idiopatik ini antara
lain:

Motilitas coecum yang berlebihan,

Perkembangan usus besar yang lebih cepat dari
usus kecil selama masa kanak-kanak

Pembesaran lymphe follikel ileum distal

Lymphadenitis mesenterial
Pada orang dewasa : Kebanyakan disebabkan oleh lesi
organis (pada umumnya tumor), tapi tidak berarti
kasus idiopatik tidak ada. Donhauser dan Kelly
mengemukakan penyebab intussussepsi orang dewasa
seperti tabel dibawah ini :

Jenis lesi Jumlah %

Neoplasma benigna 213 44 %
Neoplasma maligna 123 26 %
Divertikel Meckels 53 9 %
Gastroenteeeerostomy 27 6 %
Ulcus usus 13 3 %
Tidak diketahui 59 12 %



Dasar : - anamnesa yang baik
- pemeriksaan fisisk yang cermat
- penunjang : radiologi dan USG
Anamnesis : - Anak sehat , menangis, serangan
nyeri perut (sifat kolik), gelisah

- Bayi . nyeri perut mungkin tak nyata
. pendiam
. muntah
. pucat dan berkeringat
- Anak >2 th keluhan nyeri perut
lebih jelas

DIAGNOSIS
Defekasi:
Tahap awal normal / diare
3-6 jam setelah serangan : lendir campur darah.

Trias invaginasi (Farid Nur , 1993) :
- nyeri yang bersifat kolik
- teraba massa tumor
- BAB lendir darah

Pemeriksaan fisik
Stadium dini : fungsi vital masih baik
6-8 jam setelah serangan sakit pertama :
Bila perut makin kembung :
Muntah Dehidrasi Demam Takicardi

bakteremia

Bila perut tak kembung:
Palpasi dinding abdomen : Dinding abdomen
supel, tidak nyeri tekan. teraba massa berbentuk
lonjong sosis sign
Dance sign : pada perut kanan bawah teraba
kosong karena sekum dan colon ascenden bergerak
keatas mengikuti proses invaginasi

Colok dubur : - lendir darah
- pseudo portio (+) bila invaginatum
masuk jauh sampai rektum
18-24 jam sejak serangan sakit :
-ada tanda-tanda obstruksi sal cerna
-muntah warna hijau
-dehidrasi
-syok
bila invaginasi lanjut tjd nekrosis perforasi
peritonitis
RADIOLOGI

Plain foto abdomen :
Gambaran obstruksi saluran cerna
Distribusi udara yang tidak merata
Perselubungan pada daerah perut kanan bawah,
tengah dan atas
Udara hanya menempati perut kiri atas
Pada keadaan lanjut tanda obstruksi berupa
multiple air fluid level

Barium enema

Untuk diagnosis sekaligus terapi
Gambaran foto radiologis
Gambaran obstruksi , aliran barium terhenti
pada daerah distal invaginasi
Suatu gambaran berbentuk mangkok pada
barium yang terhenti ( Cupping appeareance)
Gambaran yang berbentuk lingkaran pir, dari
barium yang terdifusi diantara celah invaginasi
(coil spring appearance)

Ultrasonografi
Pada potongan longitudinal tampak masa
berbentuk tubuler
Pada potongan melintang tampak gambaran
target appearance atau doughnut appearance
Terapi
sebelum dilakukan tindakan definitif
1. resusitasi
2. Pasang NGT
3. Pasang kateter
4. Periksa lab darah
5. persiapan donor darah untuk transfusi
6. injeksi antibiotik

Pembedahan dapat dilakukan bila :
1. Produksi urin 0,5-1 ml/kgBB/jam
2. suhu tubuh < 38
o
C
3. nadi < 120 kali/menit
4. RR < 40 kali/menit
5. kesadaran baik

CARA PENGELOLAAN

1. Tanpa operasi :
Reduksi dengan barium enema, untuk mengerjakan
tindakan ini kamar operasi harus disiapkan, bila terjadi
kegagalan reposisi langsung dikerjakan operasi

Indikasi: semua kasus kecuali ada kontra indikasi
Kontra indikasi:
1. Strangulasi
2. KU jelek
3. Demam tinggi
4. Dehidrasi
5. Perforasi
6. Intussusepsi yang rekuren

Keuntungan:
1. terhindarnya dari stress operasi
2. terhindarnya bahaya adesi dan strangulasi dikemudian
hari

Komplikasi :
1. perforasi kolon
2. kolon yang tereduksi mungkin tidak viable lagi

Penilaian keberhasilan reduksi:
a. terjadi pembebasan dari intussusepsi
b. terlihat cairan barium masuk kedalam ileum terminal
c. adanya flatus bersama keluarnya cairan barium
bersama feses
d. tidak ditemukanya lagi massa di abdomen

2. Dengan operasi

Laparotomi Eksplorasi
Irisan tranversal diatas/dibawah umbilikus
Reduksi dikerjakan dengan manual, distal
intussusepsi dilakukan milking dengan udara usus
sebagai pendorong
Bila terjadi reposisi nilai viabilitas usus, bila usus
nekrosis lakukan reseksi dan anatomose end to end
bila kondisi memungkinkan
Bila reduksi gagal lakukan reseksi dan anastomose
end to end


Duodenal Atresia
Incidence--1 in 5,000 to 10,000 live
births
75% of stenoses and 40% of atresias are
found in Duodenum
Multiple atresias in 15% of cases
50% pts are LBW and premature
Polyhydramnios in 75%
Bilious emesis usually present
Duodenal Atresia Cont
Associated Anomalies
Downs (30%)
Malrotation
Congenital Heart Disease
Esophageal Atresia
Urinary Tract Malformations
Anorectal malformations
VACTERL
Duodenal Atresia Diagnosis
Radiographs
Double-Bubble
Pyloric dimple sign
Absence of beak sign seen in pyloric
obstruction
Workup of potential associated
anomalies
ECHO, abd US, possible VCUG

Double Bubble
Duodenal Atresia Treatment
Nasogastric decompression, hydration
Surgery
Double diamond duodenoduodenostomy
Cont prolonged NG decompression,
sometimes more than 2 weeks needed
Common Disorders
NEC
Duodenal Atresia
Small Bowel Atresia
Malrotation
Hirschsprungs
Small Bowel Atresia
Jejunal is most common, about 1 per
2,000 live births
Atresia due to in-utero occlusion of all or
part of the blood supply to the bowel
Classification--Types I-IV
Presents w/bilious emesis, abd
distension, failure to pass meconium
(70%)
Intestinal Atresia Classification
Small Bowel Atresia Cont
Associated Anomalies
other atresias
Hirschsprungs
Biliary atresia
polysplenia syndrome (situs inversus,
cardiac anomalies, atresias)
CF (10%)
Atresia--Diagnosis and Treatment
Plain films show dilated loops small bowel
Contrast enema shows small unused colon
UGI/SBFT shows failure of contrast to pass
beyond atretic point
Treatment is surgical
tapered primary anastamosis
check for other atresias/associated anomalies
Common Disorders
NEC
Duodenal Atresia
Small Bowel Atresia
Malrotation/Volvulus
Hirschsprungs
Malrotation
1 per 6,000 live births
can be asymptomatic throughout life
Usually presents in first 6 months of life
18% children w/short gut had malrotation with
volvulus
Etiology
physiologic umbilical hernia--4th wk gestation
Reduction of hernia 10th - 12th wks of gestation
Normal Embryology
Malrotation Classification
Nonrotation
when neither duodenojejunal or cecocolic
limbs undergo correct rotation
Abn Rotation of Duodenojejunal limb
causes Ladds bands to form across
duodenum
Abn rotation of Cecocolic limb
cecum lies close to midline, narrow
mesenteric base
Abnormal Rotation/Fixation
Malrotation Diagnosis
Varying symptoms from very mild to
catastrophic
**Bilious emesis is Volvulus until proven
otherwise**
Bilious emesis, bloody diarrhea, abd
distension, lethargy, shock
UGI shows abnormal position of
Duodenum
if Volvulus, see birds beak in duodenum
Malrotation UGI
Intraop Volvulus
Bowel Necrosis--Volvulus
Malrotation--Treatment
Surgical--Ladds Procedure
Evisceration
Untwisting of volvulus (counterclockwise)
Division of Ladds Bands
Widening mesenteric base
Relief of Duodenal obstruction
Appendectomy
Recurrence 10% after Ladds
Common Disorders
NEC
Duodenal Atresia
Small Bowel Atresia
Malrotation
Hirschsprungs
Hirschsprungs Disease
Migratory failure of neural crest cells
Incidence 1 in 5,000 live births, males
affected 4:1 over females
90% of pts w/Hsprungs fail to pass
meconium in first 24-48 hrs
Abd distension, bilious emesis,
obstructive enterocolitis
Hirschsprungs Diagnosis
Barium Enema
Transition zone
Anorectal Manometry
shows failure of reflexive relaxation
not very helpful in infants, young children
Rectal Biopsy
Absence of Ganglion cells and hypertrophy
of nerves
Transition Zone on BE
Hirschsprungs Treatment
In neonates, can do primary pull-
through--bringing normal colon down to
anorectal junction
In older infants, may need diverting
colostomy first to decompress
May need prolonged dilatations and
irrigations
Pull-Through Procedure