Omphalocele repair is a procedure done on an infant 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.
Abdominal wall defect repair - omphalocele; Exomphalos repair
The goal of the procedure is to place the organs back into the baby's belly and fix the defect. Repair may be done right after the baby is born. This is called primary repair. Or, the repair is done in stages. This is called staged repair.
Surgery for primary repair is most often done for a small omphalocele.
Staged repair is done when your baby isn't stable enough for primary repair. Or, it is done if the omphalocele is very large and the organs can't fit into the baby's belly. The repair is performed the following way:
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 for anesthesia and surgery in general are:
Risks for omphalocele repair are:
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 omphalocele are delivered by cesarean section.
After the Procedure
After surgery, your baby will receive care in the NICU. Your baby will be placed in a special bed 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:
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.
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.
Chung DH. Pediatric surgery. In: Townsend CM Jr, Beauchamp RD, Evers BM, Mattox KL, eds. Sabiston Textbook of Surgery: The Biological Basis of Modern Surgical Practice. 20th ed. Philadelphia, PA: Elsevier; 2017:chap 66.
Islam S. Congenital abdominal wall defects. In: Holcomb GW, Murphy JP, Ostlie DJ, eds. Ashcraft's Pediatric Surgery. 6th ed. Philadelphia, PA: Elsevier Saunders; 2014:chap 48.
Review Date: 1/31/2017
Reviewed By: Mary C. Mancini, MD, PhD, Department of Surgery, Louisiana State University Health Sciences Center-Shreveport, Shreveport, LA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
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