Introduction:
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.
Etiology:
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
cardiac anomalies
craniofacial
gastrointestinal
Beckwith-Wiedeman Syndrome
Pentalogy of Cantrell
Epigastric omphalocoele
Sternum cleft
Diaphragmatic defect
Ectopia cordis
Cardiac anomaly
Survival: 20% with heart disease
70% without heart disease
Treatment:
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.
