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Omphalocele repair

Definition

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

Other birth defects may also be present.

Description

The goal of surgery is to place the abdominal organs back into the baby's body and fix the defect, if possible.

If your baby has a small omphalocele, surgery is done soon after birth.

If your baby has a large omphalocele, surgery is usually done at a later time. While waiting for surgery, a plastic pouch called a silo will be used to return most of your baby's intestines and other organs into the belly. This can take a few weeks. Surgery will be done right away if the sac around the organs breaks.

The surgery is done in the following way:

  • Your baby receives general anesthesia.This is medicine that allows your baby to sleep and be pain-free during the operation.
  • The surgeon makes a cut (incision) to remove the sac around the organs.
  • The intestines are examined closely for signs of damage or other birth defects. Unhealthy parts are removed. The healthy edges are stitched together.

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

Why the Procedure Is Performed

Omphalocele is a life-threatening condition. 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 anesthesia and surgery in general are:

  • Allergic reactions to medicines
  • Breathing problems
  • Bleeding
  • Infection

Risks for 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. Most babies who have gastroschisis are delivered by cesarean section.

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.

After the Procedure

After surgery, your baby will receive care in the NICU. 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 the 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
  • A nasogastric (NG) tube placed through the nose into the stomach to drain the stomach and keep it empty

Feedings are started through the NG tube as soon as your baby's bowel starts working after surgery. Feedings by mouth will start very slowly. Your baby may eat slowly and may need feeding therapy, lots of encouragement, and time to recover after a feeding.

How long your baby stays in the hospital depends 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.

Outlook (Prognosis)

After you go home, your child may develop a blockage in the intestines (bowel obstruction) due to a kink or scar in the intestines. The doctor can tell you how this will be treated.

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

Islam S. Advances in surgery for abdominal wall defects: Gastroschisis and omphalocele. Clin Perinatol. 2012;39:375-386. PMID: 22682386 www.ncbi.nlm.nih.gov/pubmed/22682386.

Klein MD. Congenital defects of the abdominal wall. In: Coran AG, ed. Pediatric Surgery. 7th ed. Philadelphia, PA: Elsevier Saunders; 2012;chap 75.


Review Date: 3/13/2015
Reviewed By: Debra G. Wechter, MD, FACS, general surgery practice specializing in breast cancer, Virginia Mason Medical Center, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.
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