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Omphalocele

Our long experience with prenatal diagnosis and planning for optimum care allows us to offer the highest quality treatment whether intervention is indicated before or after birth.

Under the direction of Dr. Michael Harrison, the Fetal Treatment Center was the first institution to develop fetal surgery techniques. The first open fetal surgery in the world was performed at UCSF over 2 decades ago. We presently have more experience with fetal surgery and endoscopic fetal intervention (FETENDO fetal surgery) than any other institution in the world. We are dedicated to omphalocele research and treatment innovation.

What is omphalocele?

Omphalocele is an abdominal wall defect like gastroschisis in which the anterior abdomen does not close properly allowing the intestines to protrude outside the fetus. An omphalocele is different in that the organs are contained in a thin membranous sac instead of floating in the amniotic fluid. While the fetus is growing, the muscles of the abdominal wall do not form correctly. This allows some of the organs (stomach, liver, intestine) of the abdomen to protrude on the outside of the fetus's body through the umbilical cord.

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What is the outcome for a fetus with omphalocele?

The outcome depends on the size of omphalocele and the presence of other defects, particularly chromosome abnormalities. Fetuses with small or medium-sized omphaloceles and no other problems do extremely well with surgical repair after birth. Fetuses with other abnormalities, particularly chromosomal defects, do poorly due almost entirely to the associated anomaly, and not necessarily the omphalocele itself. Fetuses with very large or so-called 'giant' omphaloceles, even when not associated with other problems, have a very difficult time after birth. Most will survive with a series of difficult operations after birth to return the organs to the abdomen and close the abdominal wall. This can be done with a series of operations in the neonatal period (placement of a silo and staged reduction). Or, the repair can be delayed months or years if an appropriate covering of skin can be arranged. A few of these babies will be left with life-long problems including breathing and feeding difficulties.

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How serious is my fetus’s omphalocele?

In order to determine the severity of your fetus's condition it is important to gather information from a variety of tests and determine if there are any additional problems. These tests along with expert guidance are important for you to make the best decision about the proper treatment.

This includes:

1. The type of defect—distinguishing it from other similar appearing problems.
2. The severity of the defect—is your fetus’s defect mild or severe.
3. Associated defects—is there another problem or a cluster of problems (syndrome).

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What are my choices during this pregnancy?

For small omphaloceles with no other defects, the baby can be delivered normally at term in a center with pediatric surgeons available. Babies with very small omphaloceles can be safely transported for repair elsewhere, but most will benefit by being repaired shortly after birth in the same unit.

Large and giant omphaloceles should be delivered at a high level tertiary center with pediatric surgery expertise and very good neonatology support. These babies often require prolonged respiratory support during reconstruction. Often, babies with large omphaloceles are delivered by Cesarean section. This is done to minimize the risks to the baby and the mother. You and your obstetrician will determine your delivery plans.

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What will happen after birth?

Your baby should be born at a hospital with an intensive care nursery and experienced pediatric surgeons. Soon after birth, your child will have surgery to close the opening in the abdominal wall and return the organs to the abdomen. The pediatric surgeon attempts to close the hole at the time of the surgery, but sometimes this is not possible. If the omphalocele is large, a silo is placed. A silo is a covering placed over the abdominal organs on the outside of the baby. Gradually, the organs are squeezed through the silo into the opening and returned to the body. This method can take up to a week. Extremely large omphaloceles are not surgically repaired until the baby grows.

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