Abdominal Wall Defects

Gastroschisis

Abdominal wall defect typically on the right side of the umbilicus. Failure to complete formation of the ventral wall. Intestinal contents lack a protective cover and the small intestine is eviscerated. (Sometimes can include the stomach, colon, and gonads)

Not usually associated with syndromic abnormalities

–Mothers < 20 years of age
–Singleton pregnancies
–Teratogen exposures (nicotine, cocaine, ephedrine, nitrosamines, NSAIDs)

Diagnosis:
–20-week prenatal ultrasound
–Elevated serum alpha-fetoprotein level
–Growth restriction or preterm delivery in 30%
–GI complications: Intestinal atresia, stenosis, perforation, necrosis in 25%

Management:
–Prenatal diagnosis should allow planning for delivery at a level for NICU and coordination with neonatologist and pediatric surgeon
–Ensure immediate coverage of exposed intestines
–Primary closure for small defects
–Larger defects require creation of silo which is slowly reduced as intestine is returned to the abdominal cavity

Good long-term outcomes, low risk of volvulus
–Risk of adhesions and obstructions

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Omphalocele

–Midline defect of the rectus muscles, evisceration of abdominal contents into protective sac (peritoneum, Wharton jelly, amnion)
–Contents fail to rotate and return to abdominal compartment
–Advanced maternal age
–Twin births
–Prenatal diagnosis with ultrasound and elevated alpha-fetoprotein
–Associated with syndromes and chromosomal abnormalities (beckwith-Weidemann: High risk of embryonal tumors during the first 8 years including: Hepatoblastoma, Wilms tumor, neuroblastoma) also Cantrell and CHARGE syndrome
–Higher risk of volvulus with omphalocele