Agung P

Abdominal wall defect
- Omphalocele
- Gastroschisis
birth defect in which the infant's
intestine or other abdominal organs
stick out of the belly button (navel). In
babies with an omphalocele, the
intestines are covered only by a thin
layer of tissue and can be easily seen.

• Failure of the midgut to return to
abdomen by the 10th week of
Minggu 6
Minggu 10
Minggu 11
Back to normal from
Clinical Findings
• central defect of the abdominal wall beneath the umbilical ring.
• Defect may vary from 2-10 cm

• Always covered by sac
• The sac may be intact or ruptured
• Sac is composed of amnion, Wharton’s jelly and peritoneum
• The umbilical cord inserts directly into the sac in an apical or occasionally
lateral position.

• Sac contains intestinal loops, liver, spleen and bladder ,
• >50% have associated defects
• Prognosis depends on theses associated anomalies
• Mortality is approximately 40%
• Incidence has remained constant
• Increased risk with advanced maternal age
• Probable genetic predisposition
• Associated syndromes and anomalies (45-55%):
- gastrointestinal
- cardiac
- trisomy 13, 18, 21
- OEIS complex (omphalocele, bladder extrophy,
imperforate anus, spinal defects
- Beckwith-Wiedemann
- pentalogy of Cantrell
- cleft palate
- pulmonary hypoplasia
• May be associated with maternal use of valproic acid
• No membrane covering
• Abdominal wall defect typically 2-4cm
• Lateral to the right side of the umbilical
• Usually contains midgut and stomach
• Thickened, atretic, and possibly ischemic
• Associated with malrotation
Embryology of Gastroschisis

• Failure of vascularization of the
abdominal wall due to abnormal
involution of the right umbilical vein or
a vascular accident of
omphalomesenteric artery causes
abdominal wall weakness and
subsequent rupture
• Rupture of a small omphalocele with
absorption of the sac and growth of a
skin bridge between the abdominal wall
defect and umbilical cord
Clinical Findings
• 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

• Infants have better prognosis than those with an omphalocele

(Mortality is approximately 10% )

• 10-15% have associated anomalies (intestinal atresia)

• 40% are premature/SGA
• AFP synthesized in fetal liver and excreted
by fetal kidneys and crosses placenta by
12 weeks
• Elevated maternal MSAFP in neural tube
defects, abdominal wall defects, duodenal
or esophageal atresia
• 40% false positive rate
• Fetal ultrasound after 14 weeks gestation
• Amniocentesis and fetal echocardiography
• NGT to low intermittent suction
• Use of bowel bags, saran wrap
• Conservation of body heat and fluid
• Antibiotics
• Careful positioning to avoid kinking
of mesenteric vessels
• 1.5 times maintenance fluids with
isotonic fluids
Surgical Management
• Operative repair within 2-4 hours of
• Primary closure for smaller defects
• Delayed primary closure for large
– Avoid compromised ventilation and
abdominal compartment syndrome
– Use of silo with sequential reduction of
abdominal contents
– Later fascial closure
Staged Closure