ÀA congenital malformation that results from a failure of the return of the bowel to the abdomen following herniation of the abdominal contents into the base of the umbilical cord during the fifth week of fetal development.
A type of abdominal wall defect in which the intestines and liver remain outside of the abdomen in a sac because of a defect in the development of the muscles of the abdominal wall.
The herniated tissue is covered by the peritoneal membrane.
Most common in children of mothers at the extremes of reproductive ages.
Can be identified during a second trimester ultrasound or when the herniated sac is seen at birth.
Associated anomalies include the Beckwith-Wiedemann syndrome and the Pentalogy of Cantrell.
The herniated sac may be identified by ultrasound during pregnancy.
Serum alpha-fetoprotein (AFO) elevated in70% of cases.
Treatment is by surgical reduction of the herniated contents back to the abdomen.