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Omphalocele Treatment at the Center for Maternal Fetal Health

Omphalocele (sometimes called exomphalos) is a rare birth abnormality in which the abdominal wall does not close properly and one or more organs remain outside the abdomen, housed in a sac. This condition is similar to gastroschisis, but treatment and long-term outlooks differ for each condition. The pediatric surgeons of Rocky Mountain Pediatric Surgery are highly experienced in treating this condition. Additionally, our facility is uniquely equipped with staff and specialized equipment to handle all aspects of a safe pregnancy, delivery and surgery for a baby diagnosed with omphalocele.

About Omphalocele

It is normal for the organs of a fetus to develop outside the abdomen until about week 10 of gestation. Omphalocele (pronounced um-fal-eh-seal) is a condition in which the organs remain outside the body after this stage of gestation. This type of hernia affects an estimated 1 in 6,000 live births. It differs from gastroschisis, another abdominal wall defect, in that these patients have a translucent membrane housing the organs that have developed outside the body. Omphalocele can range in size and severity. One or more abdominal organs, including the intestine, liver or spleen may be displaced into the base of the umbilical cord. Risks of the condition include infection, injury of the organs, an underdeveloped abdomen and/or underdeveloped lungs. Babies with this defect also may have heart, spine and digestive organ issues. An estimated 30 percent of omphalocele patients have a genetic abnormality.

Diagnosis

Omphalocele often is diagnosed during a routine prenatal ultrasound in the second or third trimester. An echocardiogram (ultrasound of the heart) usually is ordered to detect any heart abnormalities. Typically, additional ultrasounds are conducted during the remainder of the pregnancy to gather more information and create a plan of treatment after birth.

Treatment

If your baby is diagnosed with omphalocele, it is important to work with a perinatologist such as those at the Center for Maternal Fetal Health, to plan your delivery at a hospital offering a Level IV NICU as well as highly trained pediatric surgeons, neonatologists, experienced pediatric staff and specialized pediatric equipment. Surgery is the recommended treatment for omphalocele. Babies with small omphalocele typically can be carried to term and do not require a Cesarean section birth. Surgery is performed in the days or weeks following birth. Small omphalocele patients will undergo a primary repair operation in which the organs are placed back into the abdominal cavity and the abdominal wall defect is closed.

Babies with large or giant omphalocele require more attention. A Cesarean birth may be necessary to protect the sac during delivery, and a specialized team of respiratory therapists should be on hand to help evaluate breathing and offer assistance as giant omphalocele babies often have smaller lungs.  These babies may require a staged repair in which the sac is protected with medication while the belly is given time to grow enough to accommodate the organs. When it is more developed, an operation to place the organs back into the body takes place.

Long-Term Outlook

After a repair surgery, babies will need to have regular follow ups and continued care. Some babies will have feeding difficulty, breathing problems, growth delays and/or reflux. Babies born with omphalocele may also be more prone to sickness. With special care and attention, many babies with omphalocele go on to live happy, healthy lives.

To find a maternal fetal medicine surgeon in Denver experienced in Omphalocele treatment, contact our program care coordinator at 720-754-4902.