Omphalocele repair

Alternative names:

omphalocele closure

Definition:

A surgery to correct a congenital 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 at birth. It is similar to gastroschisis repair.

Omphalocele is an abdominal wall defect at the base of the umbilical cord (umbilicus); the infant is born with sac protruding through the defect which contains small intestine, liver, and large intestine. Omphalocele is frequently associated with other birth defects, such as heart defects, imperforate anus, urinary problems, and genetic defects. Omphalocele is very similar to gastroschisis, except that the organs are enclosed in a sac.

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

Description:

Surgical repair of abdominal wall defects involves replacing the abdominal organs back into the abdomen through the abdominal wall defect, repairing the defect if possible, or creating a sterile pouch to protect the intestines while they are gradually pushed back into the abdomen.

Immediately after delivery, the exposed organs are covered with warm, moist, sterile dressings. A tube is inserted into the stomach (nasogastric tube, also called NG tube) to keep the stomach empty to prevent choking on or breathing in (aspiration) stomach contents into the lungs. The surgery is done as soon as the infant is stable.

While the baby is deep asleep and pain-free (under general anesthesia) an incision is made to remove the sac membrane. The bowel is examined closely for signs of damage or additional birth defects. Damaged or defective portions are removed and the healthy edges stitched together. A tube is inserted into the stomach (gastrostomy tube) and out through the skin. The organs are replaced into the abdominal cavity and the incision closed, if possible.

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 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 infant's abdomen 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 breathing tube and machine (ventilator) for a few days or weeks until the swelling of the abdominal 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.