OMPHALOCELE

What is an omphalocele?

An omphalocele is a congenital (found at birth) malformation in which variable amounts of abdominal contents protrude into the base of the umbilical cord. As the fetus grows in pregnancy, the intestines grow and get longer and project from the abdomen into the umbilical cord. This growth is taking place from the sixth to the tenth week of pregnancy. Normally the intestines return rapidly into the abdomen by the eleventh week of pregnancy. If this fails to happen, an omphalocele is present. It is important to remember that you did not do anything to make this happen. However, more than half of all infants born with an omphalocele may have other birth defects. Some of these defects may be serious.

What does an omphalocele look like?

An omphalocele is covered by a clear sac or membrane through which the umbilical cord is inserted. The sac may contain only a small loop of bowel or most of the bowel and other abdominal organs. If the sac ruptures, the abdominal contents move through the opening in the abdominal wall. The abdomen is smaller than usual making replacement of the abdominal contents more difficult.

How is an omphalocele treated?

Surgery must be delayed until the baby is doing well if the omphalocele sac is intact. Small omphaloceles are repaired immediately to prevent infection or tissue damage. Larger omphaloceles may require gradual reduction by enlarging the abdominal cavity to accommodate the intestinal contents. A Silastic (plastic) pouch is placed over the abdominal contents to contain the bowel and aid in reduction until surgical closure is possible. When the abdominal contents have returned to the abdominal cavity with the aid of gravity, the opening is surgically closed. Returning the abdominal contents into the abdominal cavity can take up to ten days. The baby is usually on a breathing machine (or ventilator) during the time it takes for this return. Non-operative treatment involves the application of Silvadene cream to toughen the sac. Surgery is postponed in these cases for 6-12 months to allow the abdominal cavity to enlarge as the baby grows. If the omphalocele ruptures, this is a medical emergency and surgery would be performed immediately. Complications from omphalocele include infection, rupture and intestinal obstruction. Should you have any further questions, please do not hesitate to ask any member of the Pediatric Surgery Team.

Where can a parent get help and talk with other parents about omphaloceles? 

There is a new web site called www.omphalocele.com which is owned by a group called Mothers of Omphaloceles (MOOs). The site serves as a support group, a source of non-technical information, and a gateway into the personal websites of many MOOs and omphalocele kids.

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