(Also see Overview of Congenital GI Anomalies.)

An omphalocele is a protrusion of abdominal viscera from a midline defect at the base of the umbilicus.

In omphalocele, the herniated viscera are covered by a thin membrane and may be small (only a few loops of intestine) or may contain most of the abdominal viscera (intestine, stomach, liver). Immediate dangers are drying of the viscera, hypothermia and dehydration due to evaporation of water from the exposed viscera, and infection of the peritoneal surfaces. The estimated incidence is 1 in 3000 live births. Infants with omphalocele have a very high incidence of other congenital anomalies (up to 70%), including

Omphalocele can be detected by routine prenatal ultrasonography; if the disorder is present, delivery should be at a tertiary care center by personnel experienced in dealing with this disorder and the other associated congenital anomalies.

At delivery, the exposed viscera should be immediately covered with a sterile, moist, nonadherent dressing (eg, medicated petrolatum gauze that can then be covered with plastic wrap) to maintain sterility and prevent evaporation. The infant should then be given IV fluids and antibiotics.

The infant is evaluated for associated anomalies before surgical repair of the omphalocele. Primary closure is done when feasible. With a large omphalocele, the abdominal cavity may be too small to accommodate the viscera. In this case, the viscera are covered by a pouch or silo of polymeric silicone sheeting, which is progressively reduced in size over several days as the abdominal capacity slowly increases, until all of the viscera are enclosed within the abdominal cavity.