Omphalocele

What Is an Omphalocele?

A baby born with an omphalocele has an opening where the umbilical cord goes into the belly. A membrane or thin sac that covers the internal organs bulges through the opening.

An omphalocele (ahm-FAL-uh-seel) happens because the belly wall doesn't close as it should.

Some very small omphaloceles close on their own, but many need treatment, including surgery. Some kids born with omphaloceles have other health problems too.

What Causes Omphaloceles?

An omphalocele happens while a baby is developing in the womb. The baby's belly muscles don't close completely to keep the internal organs inside the belly. Doctors think this might happen because of changes in genes or chromosomes. Or, it might be a combination of genes and things going on in the environment.

A baby born with an omphalocele is more likely to have other problems, such as:

• extra chromosomes, like trisomy 13, trisomy 18, or trisomy 21 (Down syndrome) 
• genetic syndromes, like Beckwith-Wiedemann syndrome
• gastrointestinal problems
• cleft lip and palate
• heart defects

How Are Omphaloceles Diagnosed?

Sometimes an omphalocele shows up on a prenatal ultrasound or other routine tests done during pregnancy. If doctors find out that a baby has an omphalocele before birth, they may do more tests to see if the baby has another health problem.

Sometimes doctors don't know a baby has an omphalocele until birth.

How Are Omphaloceles Treated?

If a baby is diagnosed with an omphalocele before birth, the parents and care team will plan to:

• choose a hospital with an advanced neonatal intensive care unit (NICU) for the baby's birth
• decide if a vaginal delivery or cesarean section (C-section) is best for the mother and baby

If a baby is diagnosed with an omphalocele at birth, the care team will develop a plan. Treatment for an omphalocele depends on its size and whether it has ruptured (burst open).

• A small omphalocele may not need any treatment, or a surgeon may do a minor procedure to close it with stitches within a few days of birth. The baby probably will spend a few days in the hospital. After that, babies with small omphaloceles usually don't need more treatment.
• A large omphalocele (called a giant omphalocele) usually gets treated in stages. This lets the baby's belly muscles grow and form more fully, making surgery more successful. The stages of treatment are:
  • Wrap: At birth, the omphalocele is wrapped in moist gauze until a pediatric surgeon checks the baby. The surgeon then decides the best plan for treatment, including how to care for the omphalocele and when to do surgery.
  • "Paint and wait": Parents learn how to "paint" the omphalocele with an antibiotic mixture. They do this over a period of months while their baby's skin grows over the sac.
  • Surgery: Usually several months to a year after the painting process, doctors do one or more surgeries to fix the omphalocele.
• A ruptured omphalocele needs special treatment. An omphalocele can burst open (rupture) as the baby is being born. It needs special treatment because the ruptured membrane can't protect the organs inside the belly. The surgeon will replace the membrane with a patch.

Some babies with omphaloceles may need to spend extra time in the hospital if they have trouble with breathing or feeding, or other medical problems.

The care team typically includes a pediatric surgeon, a neonatologist (a doctor who specializes in complex newborn care), and NICU critical care nurses. If doctors diagnose a baby's omphalocele during pregnancy, the care team also might include a high-risk pregnancy specialist (called a maternal-fetal medicine specialist or perinatologist).

What Can Parents Do?

If your baby has an omphalocele, your care team will be there to guide you. Be sure to:

• Learn all about wound care before taking your baby home from the hospital so you can help your child heal well.
• Go to all follow-up visits with the surgeon.
• Meet with any specialists your doctor recommends (for example, a registered dietitian or genetic counselor).