Omphalocele

(See also Overview of Congenital Gastrointestinal Anomalies.)

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 2 to 3 in 10,000 live births (1). Infants with omphalocele have a very high incidence of other congenital anomalies (up to 70%), including

• Bowel atresia

• Chromosomal abnormalities (eg, trisomy 18, trisomy 13, Down syndrome [trisomy 21])

• Cardiac anomalies and renal anomalies

• Beckwith-Wiedemann syndrome

Omphalocele can be detected by routine prenatal ultrasonography (2); if the disorder is present, delivery should be at a tertiary care center with personnel experienced in dealing with this disorder and the other associated congenital anomalies.

Omphalocele

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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.