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Omphalocele is a fetal abnormality where part or all of the baby's abdominal organs are contained in a membrane that protrudes outside the baby's abdomen at the naval. It is similar to a condition called gastroschisis, but differs in that the abdominal organs are covered in a membrane sac. Babies born with an omphalocele require omphalocele surgery to return the abdominal organs to the abdomen and close the opening. Omphalocele is often associated with other birth defects and chromosomal abnormalities. Sanford Fetal Care Center physicians provide omphalocele diagnosis and omphalocele treatment in coordination with our Neonatal Intensive Care Units and a team of highly trained specialists.
There are two types of omphaloceles: small – where only the intestines are involved, and giant – where the liver and other organs are involved. The omphalocele size and the presence or absence of other birth defects determines the long-term outlook.
The exact cause is unknown. During week six to 10 of pregnancy, the fetus's intestines are outside the fetal abdomen; by week 11, they should return to the abdomen. With omphalocele, the abdominal organs do not return. Some people have a genetic trait causing omphaloceles to run in families. However, if the baby has no other abnormalities, the chances of having a second child with an omphalocele are 1 in 100. The occurrence of small omphaloceles is 1 in 5,000 and giant omphaloceles is 1 in 10,000.
Omphalocele often occurs with other chromosomal abnormalities and birth defects including Trisomy 13 and 18, Beckwith-Wiedermann Syndrome, Pentalogy of Cantrell, and heart, genitourinary, neural tube and gastrointestinal defects. Nearly 50% of babies born with giant omphalocele will have other birth defects. Omphaloceles affect more boys than girls.
An omphalocele diagnosis is made during a routine prenatal ultrasound and may also be detected through AFP chromosomal screening. The mother usually has no signs or symptoms of the condition.
If diagnosed, your physician will recommend an amniocentesis to check for chromosomal abnormalities. A fetal echocardiogram may be done to evaluate the fetus's heart. A detailed ultrafast fetal MRI may be done to assess the omphalocele and the condition of the organs within it.
Typically, you will undergo monthly ultrasounds to monitor the fetus's condition. Delivery should take place in a hospital equipped with a Level III Neonatal Intensive Care Unit as well as a specialized team of neonatal surgeons. If the omphalocele is small, the baby may be delivered vaginally with omphalocele repair surgery scheduled immediately after delivery. If the omphalocele is giant (involving the liver), surgery for the omphalocele may need to be delayed. Your Sanford Fetal Care team will help you plan for your delivery and treatment options.
Babies born with a small omphalocele often have omphalocele surgery after birth. The surgeon will return the abdominal contents to the abdomen and close the opening. Most babies with small omphaloceles do very well. Babies with giant omphaloceles will be delivered via Cesarean section. The membrane sac will be covered with a protective wrap and the abdominal contents will be returned via gravity to the abdomen over a period of several days to weeks. Because the baby's abdomen may not have grown large enough to hold the abdominal organs, time is often needed for it to grow. The opening may not be surgically closed for several months to a year and often repeated surgeries are required. During this time there is a risk for complications including respiratory compromise, infection and feeding intolerance.
Babies with omphaloceles may be hospitalized for weeks to months, depending on the size of the omphalocele, any additional birth defects, and their ability to gradually tolerate oral feedings, gain weight and have normal bowel function. The survival rate for babies without any other birth defects is 90%. When other birth defects are present, the survival rate is 70%. Additionally, babies who have omphalocele repair surgery are at an increased risk for feeding difficulties, bowel obstruction and gastroesophageal reflux.
Talk to your Sanford Fetal Care team to learn more about Omphalocele and how it may affect your baby. Our genetic counselors are also here to help families learn more about genetic conditions.