Omphalocele

Overview

What is an omphalocele?

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.

How big is an omphalocele?

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.

What other complications might babies with an omphalocele have?

Babies born with an omphalocele frequently have other complications including:

  • Poor lung development
  • Intestines that are slow to handle food
  • Heart malformations (20 percent)
  • Beckwith-Wiedeman Syndrome (a condition typified by a large tongue, high insulin and low blood sugar)
  • Chromosomal abnormalities

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.

When does an omphalocele develop?

An omphalocele develops in the womb, before birth:

  • Six to 10 weeks in the womb: In early pregnancy, the baby’s intestines bulge into the umbilical cord as they grow. This push is normal.
  • By 11 weeks in the womb: Normally, the intestines return to the abdomen. If that doesn’t happen, an omphalocele develops.

How common is an omphalocele?

Approximately 1 in every 4,200 babies in the United States is born with an omphalocele.

Symptoms and Causes

What are the signs of omphalocele?

Providers typically diagnose an omphalocele during a prenatal ultrasound. They can see the organs developing outside the abdominal wall.

What causes an omphalocele?

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.

Who is at risk for developing an omphalocele?

Researchers are studying risk factors for omphalocele. These factors may affect your risk of having a baby with an omphalocele:

  • Consuming alcohol and tobacco.
  • Taking certain medications, including selective serotonin-reuptake inhibitors (SSRIs), during pregnancy.
  • Carrying extra weight before pregnancy.

Diagnosis and Tests

How is an omphalocele diagnosed?

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 the baby is born.

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 my provider detected an omphalocele, what tests will my baby and I need?

If your provider diagnoses your baby in the womb, you might have more tests for the rest of your pregnancy. These tests may include:

  • Blood test: Providers check your blood for high levels of alpha-fetoprotein. A baby’s liver produces this protein, which then passes into the mother’s blood. High levels can show that a baby needs further evaluation.
  • Ultrasound: Frequent ultrasounds help your provider check your baby’s growth. The provider can also make sure the cover over the organs remains unbroken.
  • MRI: You may need this imaging to check on your baby’s heart, lungs and other organs.
  • Amniocentesis: During an amniocentesis, your provider removes fluid from inside the uterus. The fluid gets check for chromosomal problems.

What tests will my baby need after birth for an omphalocele?

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.

Management and Treatment

How is an omphalocele treated?

Treatment for an omphalocele will depend on many things, including

  • Gestational age of your baby, or the week of pregnancy your baby was born.
  • Likelihood your baby can tolerate medications.
  • Overall health of your baby.
  • Severity of the condition.
  • Your preferences.

What happens after my baby is born with an omphalocele?

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.

How is a giant omphalocele treated?

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:

  • It’s safest. The baby’s abdomen is too small and not developed enough to hold all the organs at once. The organs also won’t get the blood flow they need in a small space. Waiting allows the abdomen to grow.
  • It may give time for the sac covering the organs to turn into regular skin. Even so, the care team may need to stretch the skin of the abdomen to cover the opening. In some cases, your baby might need a skin flap to cover the area. A skin flap is when surgeons move tissue from one part of the body to another part.

Will my baby need a ventilator?

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.

Prevention

Can I prevent an omphalocele?

Researchers are studying possible ways to prevent omphaloceles. It’s unclear if there’s anything a mother 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:

  • Avoid alcohol.
  • Quit smoking.
  • Maintain a healthy weight.

Outlook / Prognosis

What’s the outlook for a baby with an omphalocele?

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.

Living With

How do I deliver a baby with an omphalocele?

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.

If my baby has a giant omphalocele, can they eat while waiting for surgery?

Babies continue to eat and grow during the months it may take to put all their organs back in.

Will my baby eat after surgery?

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.

Are there activity restrictions after surgery?

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.

When should I call the healthcare provider?

Call your provider after surgery if you notice:

  • Drainage from the wound.
  • Fever.
  • Wound redness.
  • Vomiting, especially if it’s green (may indicate an intestinal blockage).

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.

References

  • American Pediatric Surgical Association. . Accessed 5/24/2021.Omphalocele (https://apsapedsurg.org/parents/learn-about-a-condition/f-o/)
  • Centers for Disease Control and Prevention. . Accessed 5/24/2021.Birth Defects: Omphalocele (https://www.cdc.gov/ncbddd/birthdefects/omphalocele.html)
  • Merck Manual. . Accessed 5/24/2021.Abdominal Wall Defects (Omphalocele and Gastroschisis) (https://www.msdmanuals.com/home/children-s-health-issues/birth-defects-of-the-digestive-tract/abdominal-wall-defects-omphalocele-and-gastroschisis)

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