Snapshot A newborn infant boy is evaluated in the delivery room after delivery via cesarean section. He was born at 36 weeks of gestation to a 24-year-old G1P1 mother. On physical examination, the infant is found to have a full-thickness abdominal wall defect at the umbilicus with herniated bowel contained in a fluid-filled sac. A prenatal ultrasound from the mother's appointment at 15 weeks gestation is shown in the image. Introduction Overview omphalocele and gastroschisis are congenital abdominal wall defects omaphlocele is a central umbilical defect intestines herniate through the abdominal defect at the umbilicus into a sac covered by peritoneum and amniotic membrane gastroschisis is a small paraumbilical defect with associated intestinal abnormalities exposed intestines herniate through the abdominal wall defect next to the umbilicus with no covering sac ETIOLOGY Pathophysiology omphalocele ventral wall defect at the umbilical ring causes persistent midline herniation of intestines into the umbilical cord gastroschisis ventral wall defect causes intestines to extrude through the abdominal folds, usually to the right of the umbilicus Associated conditions omphalocele associated with congenital anomalies (i.e. trisomy 13, trisomy 18, and beckwith-Wiedemann syndrome) and structural abnormalities (i.e. neural tube defects) gastroschisis no associated chromosomal abnormalities Epidemiology Incidence omphalocele: 2.1 per 10,000 live births gastroschisis: 4.3 per 10,000 live births Demographics male-to-female ratio is approximately 1.5:1 Presentation Physical exam polyhydramnios in utero herniation of intestines through abdominal wall Imaging Ultrasonography indications performed in utero to observe structural defects in infants with a chromosomal abnormality findings herniation of abdominal contents through ventral abdominal wall defect Studies Maternal serum α-fetoprotein (MSAFP) ↑ levels associated with abdominal wall defects Differential Congenital umbilical hernia differentiating factor herniation of the intestines due to failure of umbilical ring to close small defects typically close spontaneously Treatment Surgical omphalocele cesarean delivery can prevent sac rupture surgical repair of abdominal wall defect after delivery intermittent nasogastric suction to prevent abdominal distention until surgical repair gastroschisis emergency surgical repair Complications Rupture of the omphalocele sac in utero or during delivery incidence 10-20% of cases risk factors large omphalocele treatment broad-spectrum antibiotics surgical closure Prognosis Mortality of omphaloceles is 8 times higher relative to gastroschisis Adverse factors affecting prognosis prematurity low birth weight hypothermia dehydration in utero growth restriction injury to the intestines during delivery