Omphalocele is an abdominal wall defect at the base of the umbilical cord (umbilicus); the infant is born with a 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.

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.

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.

The infant is cared for post-operatively in a neonatal intensive care unit. The baby is placed in an isolette (incubator) to keep warm and prevent infection. Oxygen is given and mechanical ventilation is often required. Intravenous fluids, antibiotics, and pain medications will be given. A nasogastric 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.