Dec 8, 2008
External herniation of abdominal viscera through a central defect into the base of the umbilical cord.It is also called as Exomphalos.It can be minor exomphalos or major exomphalos according to defect size & contents.Umbilical cord is inserted into the crown of covering or sac.Incidence is 1-5,000-10,000 live births.
Because of failure of migration and fusion of cranial, caudal and/or lateral folds because of which there is failure of intestines to return into abdominal cavity.
Associated Congenital Anomalies: 75-80%
trisomy 13, 15, 21
Pentalogy of Cantrell
Survival: 20% with heart disease
70% without heart disease
1.surgical repair of defect in minor type after repositioning of abdominal contents
inside the cavity.
2.In major type more conservative approach towards slow and continuous
reduction of contents by traction.