Omphalocele, or exomphalos, is a problem a baby is born with. The abdominal organs develop outside the belly. Babies with an omphalocele may also have other health conditions. Omphalocele treatment repairs the opening and puts the organs back in the abdomen. A large omphalocele may need surgery in several stages.
An omphalocele (uhm-FA-lo-seal), also known as exomphalos, is a congenital (present at birth) abnormality in which the organs of the abdomen stick out through an opening in muscles in the area of the umbilical cord. These organs are covered by a transparent membrane called the peritoneum.
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The omphalocele may be small, with only a portion of the intestine sticking out of the abdominal cavity, or large, with most of the abdominal organs (including intestine, liver and spleen) outside. Healthcare providers refer to such cases as giant omphaloceles. More than two-thirds of babies with omphalocele have abnormalities of other organs or body parts, most commonly the spine, digestive system, heart, urinary system and limbs.
Babies born with an omphalocele frequently have other complications including:
Infections may develop as well, especially if the lining covering the organ breaks. If an organ becomes pinched or twisted while outside the body, its blood flow can get blocked. The lack of blood flow might cause organ damage.
Healthcare providers usually spot an omphalocele during pregnancy. They’ll discuss the condition with you, including the treatment your baby will need after birth. Right after delivery, the care team takes steps to protect and treat your baby.
An omphalocele develops in the uterus, before birth:
Approximately 1 in every 4,200 babies in the United States is born with an omphalocele.
Providers typically diagnose an omphalocele during a prenatal ultrasound. They can see the organs developing outside the abdominal wall.
It is not known what causes an omphalocele, or whether the mother can do anything during pregnancy to prevent it. Between the 6th and the 10th weeks of pregnancy, the intestines actually bulge into the umbilical cord as they are growing. By the 11th week of development, the intestines should return to the abdomen. When this fails to happen, an omphalocele occurs.
Researchers are studying risk factors for omphalocele. These factors may affect your risk of having a baby with an omphalocele:
An omphalocele is often detected during the second and third trimesters of pregnancy using an ultrasound. Once discovered, a fetal echocardiogram (ultrasound of the heart) is frequently ordered to check for heart abnormalities before birth.
Sometimes an omphalocele isn’t found during pregnancy. If that happens, the problem will be evident soon after birth. Your care team will take immediate steps to protect your baby’s health.
If your provider diagnoses a developing fetus, you might have more tests for the rest of your pregnancy. These tests may include:
After your baby is born, your healthcare provider will examine the omphalocele and order X-rays. These scans check your baby’s organs, as well as other parts of your baby’s body.
Treatment for an omphalocele will depend on many things, including:
In the case of a small omphalocele, an operation will be done shortly after birth to return the organs to the abdomen and to close up the opening in the abdominal wall to prevent infection or any tissue damage.
For a large omphalocele that involves several organs, the surgery is often done in stages, moving the organs back into the baby's body over a period of several days or weeks. Between the surgeries, the care team places a sterile, protective sheet over the organs. It helps prevent infection. Surgeons use this approach for a giant omphalocele because:
Babies with omphalocele who have underdeveloped abdominal cavities often have breathing difficulties and may need the help of a ventilator until they can breathe on their own.
Researchers are studying possible ways to prevent omphaloceles. It’s unclear if there’s anything you can do during pregnancy to avoid an omphalocele. In general, living a healthy lifestyle reduces the chances of complications for your pregnancy and newborn. Try to:
The outlook, or prognosis, depends on the size of the omphalocele. It also depends on whether the baby has other health conditions.
If the omphalocele is the only health problem, the baby will likely make a full recovery. Omphaloceles often come with other birth defects, though. These conditions may continue to affect your baby’s health. Your baby’s healthcare provider will discuss the prognosis with you.
It’s best to have your baby in a hospital with the specialists and equipment needed to provide care right away. Your provider will discuss your delivery options with you. A vaginal delivery can injure the organs that are sticking out of the belly. Your provider will probably recommend a cesarean section (C-section), especially if the baby has a giant omphalocele.
Babies continue to eat and grow during the months it may take to put all their organs back in.
Once your baby is ready to go home, they’re usually eating normally. If they have other complications, such as heart or lung problems, they may need a feeding tube. Most babies don’t need this help, though.
Your baby can do appropriate activities for their age and developmental stage. If your child had a giant omphalocele, the care team at the hospital gives you a protective covering for the wound.
Call your provider after surgery if you notice:
A note from Cleveland Clinic
An omphalocele is a condition your baby is born with. Certain organs sit outside the abdomen, or belly, instead of inside. If your baby has an omphalocele, they will undergo surgery to move the organs back in and close the opening. For a small omphalocele, your baby may only need one surgery. For a giant omphalocele, full repair may take a few months. After surgery, many babies live healthy lives.
Last reviewed by a Cleveland Clinic medical professional on 05/24/2021.
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