Omphalocele

Omphalocele is a birth defect in a baby’s abdominal (belly) wall that develops before they are born. It is a rare condition causing an infant's intestines or other abdominal organs, such as the liver and spleen, to stick outside of the belly through the umbilical cord. These organs are covered with a thin, nearly transparent sac, which is one of the ways omphalocele differs from gastroschisis, a similar defect.

OmphaloceleTypically, during week 6 to 10 of pregnancy, a baby’s intestines get longer and push out from the belly into the umbilical cord. The intestines then go back into the belly by week 11. If this does not happen, an omphalocele occurs. An omphalocele can be small, with only some of the intestines outside of the belly, or it can be large or "giant", with many organs outside of the belly.

At the SSM Health Cardinal Glennon St. Louis Fetal Care Institute, we understand the anxiety and worry you may have about a diagnosis of omphalocele. Our doctors are specially trained to help you and your baby, providing the advanced monitoring and treatment you need to give your baby the best start in life.

Currently, there is no prenatal treatment for omphalocele. However, we monitor babies closely throughout gestation and provide surgical treatment for omphalocele after your baby is born.

How is an Omphalocele Diagnosed During Pregnancy?

An omphalocele is typically diagnosed during a routine ultrasound as early as week 12. When you are referred to the Cardinal Glennon St. Louis Fetal Care Institute, our team of specialists will perform an in-depth evaluation to determine the size and severity of your baby’s omphalocele and screen for the possibility of any other birth defects. This evaluation may include:

  • Detailed ultrasound: provides a visual evaluation of your growing baby’s anatomy, your womb and blood flow
  • Fetal MRI: used in addition to an ultrasound to gather more focused images of your growing baby
  • Fetal echocardiogram (echo): an ultrasound that assesses the function of your baby’s heart
  • Karyotype: a chromosome analysis to determine a possible genetic cause
  • Blood or serum screenings

This comprehensive evaluation will help doctors determine the best treatment for your baby.

Throughout your pregnancy, your nurse coordinator will arrange for you to meet with team of specialists who will monitor the health of you and your baby and plan the safest birthing and treatment plan. This team will include physicians, such as a maternal fetal medicine specialist, neonatologist and pediatric surgeon, along with a genetic counselor, social worker and sonographer. You will also learn more about our neonatal intensive care unit (NICU), where your baby will be cared for after delivery, and surgical repair of the omphalocele.

What Are The Treatment Options For Omphaloceles?

For small and medium-sized omphaloceles, a single operation to close the abdominal wall is typically effective. During this procedure, the pediatric surgeon carefully places the organs back into the abdominal cavity, then closes the opening by bringing the muscles together. Sometimes a synthetic patch is required to close the hole in the abdominal wall.

In cases of a large omphalocele, the procedure is more complex. Often there is no space in the abdomen to replace the organs. As a result, the omphalocele sac is dressed like a wound and allowed to heal, becoming covered with skin over time. Once covered with skin, the healing process to close the omphalocele can be a long one, requiring a few operations over several months to years. This allows your baby to grow and heal between surgeries.

How Will An Omphalocele Affect My Baby After Delivery And Surgery?

Depending on the severity of the omphalocele, your baby may experience complications ranging from mild to severe. If there are no other defects and the omphalocele is small, your baby is likely to do very well after surgery and develop normally. However, if the omphalocele is also accompanied by other birth defects, complications are more likely to occur.

About 30% of babies with omphalocele have a genetic defect. This can affect a baby’s long-term development. Many also have heart defects, which may require surgery. Babies with large omphaloceles will likely have other challenges after birth, including difficulties with lung development and feeding. These problems can cause a need for mechanical ventilation, tube feeding and a longer hospitalization.

From your first appointment to your last, our fetal and baby specialists at SSM Health are available to care for you and your baby’s complex needs. Our multi-disciplinary team collaborates across our hospital to provide your baby the specialized care they require.

We understand that omphalocele can be a scary diagnosis. That’s why we’re available to help 24 hours a day, 7 days a week. For more information or to schedule an appointment, call us at 314-268-4037 or toll free at 877-SSM-FETL (877-776-3385).

While we can’t change the diagnosis, we can provide you expert care and support, helping your baby get the most out of treatment and life.

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