Abdominal Wall Defects

Priscilla Joe, MD
Childrens Hospital and Research Center at
Oakland









Omphalocele
Membrane sac arising from the umbilical cord
covers intestines
Outer membrane layer consists of amnion and
inner lining of peritoneum
Size ranging from small->giant defects containing
liver, small and large bowel, stomach, spleen,
ovaries, and testes
Associated with foreshortened bowel and
malrotation
Small abdominal cavity and pulmonary hypoplasia





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

Embryology of Omphalocele
Normally, midgut returns to the abdomen
by 10
th
week of gestation
Somatic layers of cephalic, caudal, and
lateral folds join to close abdominal wall
With omphalocele, folds fail to close


Gastroschisis and Omphalocele

Combined incidence of 1 in 2000 births
Male-to-female ratio is 1.5:1
Overall survival > 90%
Gastroschisis
Increasing incidence
Associated with young maternal age and
low gravida
Associated with prematurity and low birth
weights



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





























Beckwith-Wiedemann
Pentalogy of Cantrell
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
Mortality/Morbidity
Short gut syndrome
NEC
Gut dysmotility and prolonged ileus
Sepsis
Complications from associated
anomalies/syndromes