This document provides information on abdominal wall defects, specifically omphalocele and gastroschisis. It discusses the epidemiology, etiology, clinical features, diagnosis, management, and prognosis of each condition. Omphalocele is caused by failure of the midgut to return to the abdomen during development. It presents as abdominal organs herniated within a sac. Gastroschisis is caused by failure of abdominal wall closure and presents as bowel protruding through an abdominal wall defect without a sac. Management may include prenatal monitoring, temporary covering of exposed organs, surgery to repair the defect, and treatment of any associated anomalies or complications. Outcomes depend on the severity of each case and presence of other birth defects

1. ABDOMINAL WALL DEFECTS
OMPHALOCELE VS GASTROSCHISIS
Okla B. AlShammari
Pediatric Resident R2

2. OBJECTIVES
• Epidemiology
• Etiology
• Pathophysiology
• Clinical Features
• Diagnosis
• Management
• Prognosis , long term outcomes.

3. Epidemiology
Omphalocele
* Incidence - 3 per 5,000
*M:F is 1.5:1
* >70% association with
congenital anomalies such
Bowel atresia, Imperforated
anus, Trisomies 13, 18, 21,
Beckwith-Wiedemann
Syndrome
Gastroschisis
*Incidence - 4 per 10,000
*M:F is 1:1
• 10-15% association with
congenital anomalies such as
CHD(VSD), cleft palate and
intestinal atresia
• 40% are premature/SGA

4. Etiology
Omphalocele
*Congenital abdominal wall
defect with protrusion of
abdominal viscera contained
within a parietal peritoneum
and amniotic membranous
sac with Wharton’s jelly.
*Due to failure of the midgut
to return to abdomen by the
10th week of gestation
during midgut rotation.
Gastroschisis
*Congenital abdominal wall defect
towards the right side of the
umbilicus and protruded bowel is not
covered by a membrane.
* Failure of migration and fusion of the
lateral folds of the embryonic disc on
the 3rd-4th week of gestation.
* Disruption of the right
omphalomesenteric artery as midgut
returns to abdomen by the 10th week
of gestation causing ischemia of the
abdominal wall and weakness then
herniation.
* Rupture of omphalocele

5. Risk Factors
Omphalocele
• Increased maternal age
More than 40 yr
• Twins
• High gravida
• Consecutive births
Gastroschisis
• Young maternal age
• Low gravida
• Prematurity
• Low birth-weight
secondary to IUGR

6. OMPHALOCELE Vs
GASTROSCHISIS

7. GASTROSCHISIS
*Abnormal
involution of right
umbilical vein
* Rupture of a
small
omphalocoele
*Failure of
migration and
fusion of the
lateral folds of the
embryonic disc on
the 3rd-
4th week of
gestation

8. OMPHALOCELE
Failure of the
midgut to
return to
abdomen
by the 10th
week of
gestation

9. Clinical Features
GASTROSCHISIS
Defect to the right of an intact
umbilical cord allowing
extrusion of abdominal content
Umbilical cord arises from normal
place in abdominal wall
Opening <=5 cm
No covering sac (never has a sac )
Evisceration usually only contains
intestinal loops
Bowels often thickened, matted and
edematous
10-15% have associated anomalies
40% are premature/SGA
OMPHALOCELE
central defect of the abdominal wall
beneath the umbilical ring.
Defect may be 2-12 cm (Small-
<5cm)(Large>8cm)
Always covered by sac
Sac is made of amnion, Wharton’s
jelly and peritoneum
The umbilical cord inserts directly
into the sac in an apical
or lateral position.
Small one contains intestinal loops
only. Large one may involve liver,
spleen and bladder, testes/ovary
>50% have associated anomalies

10. Diagnosis
About 90% of GASTROSCHISIS and
Omphalocele diagnosed prenatally.
Maternal AFP usually elevated with fetal
gastroschisis
Alpha-feto-protein-synthesized in fetal liver and
excreted by fetal kidneys and crosses placenta
by 12weeks.
Prenatal ultrasound after 14 weeks gestation
is the confirmatory test.

11. Prenatal Ultrasound
The diagnostic
prenatal ultrasound
findings of
Gastroschisis are
extraabdominal loops
of bowel without
covering sac

12. Prenatal Ultrasound
The prenatal ultrasound
findings of Omphalocele
are abdominal organs
herniated outside the
abdominal cavity with an
abnormal insertion of
Umbilical cord
into the membrane rather
than into abdominal wall
at midline on the mass
Contents are intestinal
loops and maybe liver,
spleen and gonads.

13. Management
Gastroschisis
After delivery :-
The perfusion of the herniated contents should be carefully
evaluated . If bowel ischemia or infarction suspected >
immediate surgical consultation is indicated.
If the viscera are well perfused , it is important to next place a
clear plastic bag over the exposed bowel as a temporary
covering to minimize evaporative heat and fluid loss

14. Pre-operative Management
• ABC
• Heat Management
– Sterile wrap or sterile bowel bag
– Radiant warmer
• Fluid Management
– IV bolus 20 ml/kg LR/NS
– D10¼NS 2-3 maintenance rate
• Nutrition
– TPN (central venous line )
• Abdominal Distention
– OG/NG tube
– urinary catheter
• Infection Control
Broad-spectrum antibiotics
• Closure of the Defect

15. Surgical Management
Gastroschisis
The goal of surgical repair is safe the
reduction of the eviscerated contents and
eventual closure of the abdominal wall.
– Primary Closure
Use of own baby umbilical stump as
biological dressing to seal gastroschisis
defect without attempting a primary
fascial closure
– Staged Closure
• Staged repair using silo pouch

16. Management
Omphalocele:-
Including the evaluation for associated
anomalies and monitoring of fetal growth.
Echocardiography : high risk for CHD
Prenatal monitoring of fetal growth : high risk of
IUGR . Other specific evaluation for associated
pulmonary hypoplasia ( giant omphalocele )
Prenatal counseling about the expected hospital
course and the long term prognosis

17. Cont management
Omphalocele:
After delivery :-
The initial evaluation and resuscitation to a babies
with an Omphalocele follow same protocol and
sequences of all newborns.
Should be handled carefully to prevent the
omphalocele membrane from tearing. After initial
stabilization for the newborn with omphalocele
should be inspected to confirm that it is intact and
then covered with a nonadherent dressing to
protect the sac.

18. Cont management
Omphalocele:
Primary Closure
Small defects (<4cm)
excision of the sac and closure
of the fascia and skin over the
abdominal contents
Mesh patch
Medium defects (6-8cm)
Conservative
Large defect (10-12cm)
apply topical application -
Betadine ointment or silver
sulfadiazine to make intact sac.
till the baby is bigger and more able to tolerate major
operation

19. Long term outcomes
Gastroschisis :
-Almost always with intestinal malrotation
- Hernias at the site of repair.
- Intestinal atresia.
- Short bowel syndrome.
Omphalocele:
Small will recovery well
The outcomes determine by the severity of associated anomalies ,
so babies with giant omphalocele have increased mortality and
morbidity because of the large abdominal wall defect and
associated pulmonary hypoplasia and pulmonary hypertension
- GERD
- Hernias
- Respiratory infection
- Failure to thrive

20. Thank you