Omphalocele

Alexander K. C. Leung, MD

,
Andrew L. Wong, MD

A mass covered by a translucent membrane was found in the umbilical area of an infant born by cesarean section at 38 weeks' gestation to a 26-year-old gravida 3 para 2 mother.

A mass covered by a translucent membrane was found in the umbilical area of an infant born by cesarean section at 38 weeks' gestation to a 26-year-old gravida 3 para 2 mother.

This was an omphalocele-a herniation of intra-abdominal contents through the umbilical ring into the umbilical cord. An omphalocele results from a failure of the viscera at the 10th week of embryonic life to return from its normal herniation into the umbilical cord and a failure of the lateral embryonic folds to close at that site. The translucent sac is composed of an outer layer of amnion and an inner layer of peritoneum.

An omphalocele occurs in about 1 in 6,000 live births. It may be an isolated lesion but may also signal chromosomal aberrations and concurrent cardiac, gastrointestinal, and genitourinary defects. It is often a presenting feature of Beckwith-Wiedemann syndrome.

Emergency treatment consists of covering the lesion with warm, moist saline gauze and decompressing the neonate's stomach by nasogastric suction.

Prophylactic parenteral broad-spectrum antibiotics should be administered. Small defects can be managed by excision of the sac and primary closure of fascia and skin. Larger defects may require a staged approach: a silo of Silastic material sutured to the fascia permits gradual reduction of the omphalocele into the abdomen until definitive closure is possible, usually within 10 days.