Children born with Omphalocele have a type of hernia that allows the intestines, liver, and sometimes other organs to protrude through the belly button area of the stomach. In most cases of omphalocele, a thin tissue covering protects the protruding organs. The intestine is most often associated with Omphalocele, but the spleen and liver can protrude is severe cases. The reason for an omphalocele is a problem with the development of the abdominal muscles.

An omphalocele is often detected through blood screening during pregnancy such as  AFP screening or a detailed fetal ultrasound. Genetic counseling and genetic testing such as amniocentesis is usually offered during the pregnancy because an omphalocele can be associated with certain genetic conditions such as Trisomy 18 (Edward syndrome) and Trisomy 13 (Patau syndrome).

Surgical repair may seem simple, but Omphalocele develops in-utero. If the organs are not in the abdominal cavity during fetal growth, there is no room for them after birth. Surgeons must fit the intestines back into the abdominal cavity without putting pressure on any other organs or causing a constriction of the bowel.

In some cases, surgeons will reinsert a portion of the intestines back into the abdomen and wait until the cavity stretches a bit before attempting to complete the Omphalocele repair. The outlook will depend upon other congenital conditions that may be present with Omphalocele.