Omphalocele repair

Definition

Omphalocele repair corrects a birth defect in which all or parts of the small intestine, liver, and large intestine are on the outside of the abdomen and enclosed in a membranous sac. This procedure is similar to gastroschisis repair.

Omphalocele is frequently associated with other birth defects, such as heart defects, imperforate anus, urinary problems, and some genetic conditions. Omphalocele is similar to gastroschisis, except that the protruding organs are enclosed in a sac.

Gastroschisis and omphalocele are frequently diagnosed before birth by ultrasound examinations.

Description

Surgical repair of abdominal wall defects involves placing the abdominal organs back into the abdomen through the defect and repairing the defect if possible. If immediate replacement is not possible, a sterile pouch is created to protect the intestines while they are gradually pushed back into the abdomen over a period of time.

Immediately after delivery, the baby’s exposed organs are covered with warm, moist, sterile dressings. A nasogastric (NG) tube is inserted through the baby’s nose or mouth into the stomach to keep the stomach empty. This prevents Choking on or breathing stomach contents into the lungs (aspiration). The surgery is done as soon as the infant is stable.

While the baby is under general anesthesia, an incision is made to remove the sac membrane. The intestine is examined closely for signs of damage or additional birth defects. Damaged or defective portions are removed and the healthy edges are stitched together.

A tube may be inserted into the stomach (gastrostomy tube) and out through the skin.

If possible, the organs are replaced into the abdominal cavity and the incision closed. If the abdominal cavity is too small or the protruding organs are too swollen to allow the skin to be closed, a pouch will be made from a sheet of plastic (silo) to cover and protect the organs. Complete closure may be done over a few weeks.

Surgery may be necessary to repair the abdominal muscles at a later time.

The baby’s abdominal cavity may be smaller than normal. Placing the abdominal organs into the abdomen increases the pressure within the abdominal cavity and can cause breathing difficulties. The infant may require the use of a ventilator for a few days or weeks until the swelling of the organs has decreased and the size of the abdomen has increased.

Indications
Omphalocele is a life-threatening event requiring immediate intervention. The infant may be born underweight (small for gestational age) due to stress from this condition before birth.

Risks
Risks for any anesthesia include the following:

     
  • Reactions to medications or the anesthetic  
  • Problems breathing due to anesthesia

Risks for any surgery include the following:

     
  • Bleeding  
  • Infection

Additional risks of omphalocele repair include the following:

     
  • Injury to organs  
  • Breathing difficulties (may occur as a result of increased pressure in the abdomen when the omphalocele is closed)  
  • Peritonitis  
  • Temporary paralysis of the small bowel (paralytic ileus)

If the baby has breathing difficulties, mechanical ventilation may be necessary. If a large portion of the small bowel is damaged, the infant may have problems digesting and absorbing feedings.

Expectations after surgery
This defect can be corrected with surgery in most cases. The outcome depends on the amount of damage to or loss of intestine. Omphalocele is often associated with other congenital (present from before birth) defects that may influence the patient’s prognosis.

Convalescence

The infant will be cared for postoperatively in a neonatal intensive care unit (NICU). The patient is placed in an isolette (incubator) to provide warmth and prevent infection. Oxygen is given and mechanical ventilation is often required. Intravenous fluids, antibiotics, and pain medications will be given as needed.

An NG tube will be in place to keep the stomach emptied of gastric secretions. Feedings are started by nasogastric tube as soon as bowel function resumes. Feedings are started very slowly, and often infants are reluctant to feed. These babies may need feeding therapy and lots of encouragement.

Johns Hopkins patient information

Last revised: December 8, 2012
by Armen E. Martirosyan, M.D.

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