Omphalocele, (or omphalocoele), is a rare abdominal wall defect in which the intestines, liver, and occasionally other organs are outside of the abdomen in an amnionic sac.  This occurs  because of failure of normal return of intestines and other contents back to abdominal cavity during around ninth week of intrauterine development.

Omphalocele occurs in 1/4,000 births and unlike gastroschisis this condition is associated with a high rate of mortality (25%) and other congenital malformations(30%), such as cardiac anomalies (50%), neural tube defects(40%), exstrophy of bladder and Beckwith Wiedemann syndrome. Approximately 15% of live-born infants with omphalocele have chromosomal abnormalities.

Treatment ranges from primary closure of the defect with or without mesh to delayed closure after treating the amnion with a drying agent. 

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