Omphalocele refers to an opening, or hole, in the abdominal wall. During prenatal development, the bowel and liver protrude through the hole and remain outside of the abdomen. These organs are covered by a thin layer of tissue known as a sac. The umbilical cord inserts into the sac. The size of the hole can range from small to very large. Omphalocele occurs in about 1 in every 5,000 births, and more often in mothers over the age of 35 years.
Diagnosis of omphalocele
Babies with an omphalocele often have other problems. Heart defects and chromosome abnormalities are the most common of these, occurring in 30% of children with omphalocele.Because of this, an ultrasound of the baby’s heart (fetal echocardiogram) and an amniocentesis are recommended. Babies with a giant omphalocele often have very small lungs and may need a fetal MRI to measure their lung size.
Management of pregnancy
Regular ultrasounds are scheduled to monitor the baby’s growth during the pregnancy. Additional testing may be used based the individual mother’s and baby’s needs.
Delivery of a baby with omphalocele
It is recommended that the delivery of a baby with an omphalocele be planned at a hospital that is prepared for high-risk births including a newborn intensive care unit and pediatric surgical services. This will simplify communication between the teams caring for the baby and also limit the separation between mother and baby. Often, a Cesarean delivery is required for an infant with a large omphalocele. Many smaller defects can deliver vaginally. Each birth is planned based on the individual situation.
University of Michigan is one of only a few centers nationwide at which the birth center is co-located within a comprehensive children’s hospital. This unique setting allows for seamless integration between our private-room birth center, state-of-the-art newborn intensive care unit (NICU), and access to around-the-clock pediatric surgical services in one convenient location.
Treatment of omphalocele
There is no fetal treatment for omphalocele, although fetal diagnosis is an important part of planning for the birth and immediate evaluation after the baby’s birth. The neonatal team will check the baby’s breathing and heart. Then, the surgical team will then come to assess the omphalocele.
Babies with a small omphalocele can usually have one surgical procedure to remove the sac and close the hole in the abdominal wall. A slightly larger defect may need a patch to help close the opening.Giant omphaloceles present additional challenges. They can be associated with a small chest resulting in small lungs. This means the baby may need to be on a breathing machine (ventilator) for a long time.; A large abdominal opening means the abdomen is too small to hold all the abdominal organs.
The first step in the treating giant omphaloceles involves putting antibiotic ointment on the sac. The sac is then wrapped with gentle pressure. This helps to the increase the space in the abdomen while slowly moving the organs back inside. Over time, the sac develops a skin-type coating that protects the organs.
This is sometimes called the “paint and wait” method.The baby can be discharged home while the family continues with the dressing change.
Gradually, the abdomen becomes big enough to hold all the organs. Surgery to close the close the hole can be done later when the baby is older. This approach may avoid multiple operations and an extended stay in the hospital after birth.
Babies with an omphalocele may have other health problems, especially if there is a genetic cause. There could be issues with the heart, lung, bowel, kidneys or brain. Some may be related to the size of the defect, while others may be related to the time spent in the hospital. The doctors will assess each baby to decide what testing is needed.
Babies with larger defects often have some degree of feeding problems. Sometimes it is because they have been on the breathing machine for a long time and may need help with oral skills. Others may have trouble accepting breast milk or formula and have poor weight gain. Many of these babies have reflux problems, particularly after surgery. Medicine is often effective for managing the problem, but a few may need an additional surgery.
Future Pregnancy Risk
It is important to know if the cause of the omphalocele is related to a chromosome abnormality, or a genetic condition. This will help your doctors determine the chance of the same problem happening in another pregnancy. Prenatal testing may be available for some of these conditions. An omphalocele in a pregnancy with no other physical findings is considered to a sporadic event. This type of event has a very low risk of happening again. Your doctor and a genetic counselor will review the risk in your family.
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