Omphalocele

Omphalocele is a defect in the anterior abdominal wall leading to herniation of the abdominal contents (primarily intestines and liver).

The 3 layered human embryo folds centrally fuse in the first month of embryogenesis and a defective fusion leads to abdominal wall defects. In the sixth week of intrauterine life, rapid growth of the midgut causes it to herniate through the umbilical ring. The abdominal contents are in a translucent sac, which is composed of amnion, Wharton jelly, and peritoneum. The umbilical vessels radiate onto the wall of the sac.

Omphalocele must be distinguished from gastroschisis:

	Gastroschisis	Omphalocele
Covering Membrane	Absent	Present (ruptured in 10-15%)
Size of the defect	2-5 cm	2-15 cm
Umbilical cord location	Left of the defect	In the center of the membrane
Condition of the gut	Inflamed	Normal
Recovery of gut function	Late	Early
Associated anomalies	10%	60%

Most cases are repaired surgically in the early neonatal period. Appropriate management of hydration and nutritional status is required until surgery is accomplished.