Omphalocele

What is an omphalocele?

An omphalocele is a birth defect in which an abnormal opening in your baby's abdominal wall allows the stomach, liver or intestines to protrude outside the body in a membrane sac. It is not known why this happens, but an omphalocele occurs when the abdominal wall muscles don't form correctly during fetal development. The size of the omphalocele varies from very small - about the size of a golf ball - to giant omphaloceles that can be as big as grapefruit.

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Prenatal diagnosis and fetal treatment

You probably will not experience any unusual symptoms during the pregnancy but an ultrasound will show a bump on the outside of the abdominal wall. The ultrasound will show the size of the defect and identify the affected organs.

In order to determine the severity of your fetus's omphalocele, a thorough evaluation should be performed. This evaluation will include:

• an ultrasound
• a fetal echocardiogram, which is a special ultrasound to look at the baby's heart
• amniocentesis, a test of a sample of amniotic fluid drawn from your uterus. An amniocentesis will show whether or not there is a problem with the chromosomes.

Time intervals for ultrasound studies are decided on an individual basis. Your perinatologist, an obstetrician who specializes in maternal and fetal medicine, will discuss recommendations for your delivery. Babies with large omphaloceles often are delivered by cesarean section to minimize the risks to the baby. You and your obstetrician will determine your delivery plan.

For more information on prenatal diagnosis and fetal treatment visit Fetal Treatment Center: Omphalocele

What will happen after my baby is born?

Delivery should be at a hospital with an intensive care nursery (ICN) and a pediatric surgeon available because your child will need surgery soon after birth to close the abdominal wall and return the organs to the abdomen. If the omphalocele is too large to be closed, a covering called a silo is placed over the abdominal organs. Gradually, the organs are squeezed through the silo into the opening and returned to the body.

How does this squeezing occur?

This method can take up to a week. Extremely large omphaloceles are not surgically repaired until the baby grows. Until then, they are treated by applying painless drying agents to the omphalocele membrane.

What does drying accomplish?

Babies born with omphaloceles can stay in the hospital from a week to a month after surgery, depending on the defect size. Hospital discharge occurs when the baby can take all feedings by mouth and is gaining weight. Most babies with omphaloceles don't have any long-term problems but they may be at risk for bowel obstruction due to scar tissue or a kink in a loop of bowel caused by the operation. Symptoms of bowel obstruction include:

• Bilious or green vomiting
• Bloated stomach
• No interest in feeding

If any of these symptoms occur, contact your pediatrician immediately.