Omphalocele

Clinical features: defect covered by amnion, with cord attachment to apex of defect; prematurity and intrauterine growth retardation less common than in gastroschisis

Herniation through defect: any abdominal organ, but usually the large or small intestine, liver, stomach, gall bladder, urinary bladder, pancreas, spleen, or internal genitalia

Associated anomalies in 67% of affected newborns: trisomy 13, 18, or 21 syndrome, congenital heart disease (15% to 25%), gastrointestinal anomalies (midgut volvulus, Meckel's diverticulum, intestinal atresia and duplication, imperforate anus, colonic agenesis), and neurologic and renal anomalies (20%)

Overall mortality: ∼30%, related to the presence of major structural and chromosomal anomalies, and to intractable respiratory insufficiency in some newborns with large omphaloceles; with no associated anomalies, minimal mortality

Gastroschisis

Clinical features: no sac covering the defect; defect in abdominal wall positioned to right of umbilicus; cord attachment to abdominal wall to left of defect; prematurity and intrauterine growth retardation more common than in omphalocele

Herniation through defect: usually limited to small intestine and ascending colon, with thickened and matted appearance of intestine

Associated anomalies: primarily intestinal atresia

Overall mortality: ∼10%, mostly from complications of prematurity and intestinal complications such as severe short gut syndrome

Management of defects

General measures for initial stabilization and evaluation: application of warm, fluid-impermeable dressing over defect; placement of orogastric tube to decompress stomach and prevent further distension; aggressive fluid resuscitation to compensate for ongoing fluid losses from exposed viscera; blood culture followed by broad-spectrum antibiotic (ampicillin or gentamicin [Garamycin])

Immediate consultation with pediatric surgeon