Agung P

11.023
Abdominal wall defect
- Omphalocele
- Gastroschisis
Omphalocele
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.
Pathophysiology

• Failure of the midgut to return to
abdomen by the 10th week of
gestation
Minggu 6
Minggu 10
Minggu 11
Back to normal from
herniasi
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 ,
testes/ovary
• >50% have associated defects
• Prognosis depends on theses associated anomalies
• Mortality is approximately 40%
Omphalocele
• 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
Gastroschisis
• No membrane covering
• Abdominal wall defect typically 2-4cm
diameter
• Lateral to the right side of the umbilical
cord
• Usually contains midgut and stomach
• Thickened, atretic, and possibly ischemic
bowel
• 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
Diagnosis
• 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
Treatment
• NGT to low intermittent suction
• Use of bowel bags, saran wrap
• Conservation of body heat and fluid
losses
• 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
birth
• Primary closure for smaller defects
• Delayed primary closure for large
defects
– Avoid compromised ventilation and
abdominal compartment syndrome
– Use of silo with sequential reduction of
abdominal contents
– Later fascial closure
Staged Closure