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Omphalocele repair

Omphalocele repair is surgery to repair a birth defect in the wall of the belly (abdomen) in which all or parts of the small intestine, liver, and large intestine stick out of the belly button (navel) in a thin sac.

Other birth defects are also present.

The goal of surgery is to place the abdominal organs back into the baby's body and fix the defect in the wall of the belly, if possible. Right after birth:

  • The baby's sac of exposed organs is covered with warm, moist, sterile dressings or a sterile plastic bag.
  • A nasogastric (NG) tube is inserted through the baby's nose or mouth into the stomach to keep the stomach empty. This prevents the baby from choking and breathing stomach contents into the lungs.

For infants with small omphaloceles, the surgery is done soon after birth.

If the infant has a large omphalocele, the surgery is usually delayed. The intestines and other organs can be gently squeezed into the belly with the help of a plastic pouch called a silo. This can take a few weeks. Surgery will be done right away if the sac around the organs breaks.

Before surgery, your baby will receive general anesthesia. This will make your baby asleep and stay pain-free during the operation.

The surgeon will make a cut to remove the sac around the organs. The intestines will be examined closely for signs of damage or other birth defects. Unhealthy parts will be removed. The healthy edges will be stitched together.

More surgery may be needed later to repair the muscles in your baby's belly.

Why the Procedure Is Performed

Omphalocele is a life-threatening condition that needs immediate treatment. It needs to be treated soon after birth so that the baby’s organs can develop and be protected in the belly.

Risks

Risks for any anesthesia are:

  • Allergic reactions to medicines
  • Breathing problems

Risks for any surgery are:

  • Bleeding
  • Infection
  • Blood clots

Additional risks of omphalocele repair are:

  • Breathing problems. The baby may need a breathing tube and breathing machine for a few days or weeks after surgery.
  • Inflammation of the tissue that lines the wall of the abdomen and covers the abdominal organs
  • Organ injury
  • Problems with digestion and absorbing nutrients from food, if a baby has a lot of damage to the small bowel

Before the Procedure

Omphalocele is usually seen on ultrasound before the baby is born. After it is found, your baby will be followed very closely to make sure he or she is growing.

Your baby should be delivered at a hospital that has a neonatal intensive care unit (NICU) and a pediatric surgeon. A NICU is set up to handle emergencies that occur at birth. A pediatric surgeon has special training in surgery for babies and children.

The delivery is usually planned and a cesarean section is often done.

After the Procedure

After surgery, your baby will receive care in the hospital's neonatal intensive care unit. Your baby will be placed in a special bed called an isolette. This bed has an incubator to keep your baby warm.

Your baby may need to be on a breathing machine until organ swelling has decreased and the size of their belly area has increased.

Other treatments your baby will probably need after surgery are:

  • Antibiotics
  • Fluids and nutrients given through a vein
  • Oxygen
  • Pain medicines, as needed
  • A nasogastric (NG) tube placed through the nose into the stomach to drain the stomach to keep it empty

Feedings are started through the NG tube as soon as your baby’s bowel starts functioning after surgery. Feedings by mouth will start very slowly. Your baby may eat slowly and may need feeding therapy and a lot of encouragement.

The total length of time in the hospital will vary. It will depend on whether there are other birth defects and complications. You may be able to take your baby home once he or she is taking all foods by mouth and gaining weight.

After you go home, your child may develop a bowel obstruction (a blockage in the intestines) due to a kink or scar in the intestines.

Outlook (Prognosis)

Most of the time, surgery can correct omphalocele. How well your baby does depends on how much damage or loss of intestine there was, and whether your child has other birth defects.

Some babies have gastroesophageal reflux after surgery. This condition causes food or stomach acid to come back up from the stomach into the esophagus.

Some babies with large omphaloceles may also have small lungs and may need to use a breathing machine.

All babies born with an omphalocele should have chromosome testing. This will help parents understand the risk for this disorder in future pregnancies.

References

Chung DH. Pediatric surgery. In: Townsend CM, Beauchamp RD, Evers BM, Mattox KL, eds. Sabiston Textbook of Surgery. 19th ed. Philadelphia, PA: Elsevier Saunders; 2012:chap 67.

Islam S. Advances in surgery for abdominal wall defects: Gastroschisis and omphalocele. Clin Perinatol. 2012;39:375-386.

Updated: 1/29/2013

John A. Daller, MD, PhD, Department of Surgery, Crozer-Chester Medical Center, Chester, PA. Review provided by VeriMed Healthcare Network. Also reviewed by A.D.A.M. Health Solutions, Ebix, Inc., Editorial Team: David Zieve, MD, MHA, David R. Eltz, Stephanie Slon, and Nissi Wang.


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