Last updated: 02/17/2009

An omphalocele is a defect that can be viewed on sonogram during an ultrasound performed while the mother is pregnant. At about six to eight weeks of fetal development, the abdominal contents come out of the fetus's abdomen at the base of the umbilical...

cord. They return to the inside as development continues. If this process is interrupted in some way during the seventh to tenth week of fetal development, the contents remain on the outside, and an omphalocele develops. Because the abdominal contents are now on the outside of the body, the inside cavity may not develop properly. For this reason, a large omphalocele cannot simply be placed back inside because the cavity may be too small. The internal organs will need to be protected and kept hydrated while the inside is gradually stretched. Small amounts of the omphalocele are returned at any one time to allow the cavity to gradually stretch to accommodate them. If the sac surrounding the tissue has ruptured, or broken, there is a greater risk of infection, tissue damage, loss of body temperature, and dehydration.

The repair may be performed in stages. If the omphalocele is very small, it may be possible to return all of the contents to the inside, and surgically close the opening. If the omphalocele is too large to do this all at once, some contents will remain on the outside while a sterile pouch is created to protect the tissue that remains on the outside. To be sure that the tissue does not dry out, it will be covered with warm and moist sterile dressings. The infant can lose considerable body heat through the large amount of exposed surface area, so keeping him or her warm, and closely monitoring body temperature is a high priority. An antibacterial solution may be used to decrease the risk of infection. The infant will have a tube that goes in through the nose or mouth and down into the stomach, called a nasogastric tube. Suction is used to keep the stomach empty, avoiding the chance of vomiting, or of the fluid moving from the stomach up into the lungs. The contents of the sac will be carefully examined to make sure that none of the tissue is damaged or dead, and to check for signs of intestinal birth defects before being inserted into the body.

The omphalocele repair is a surgical procedure performed under general anesthesia. The infant will receive medication to relax his or her muscles, and to help the surgery move forward without causing any pain. A large omphalocele repair may be done in stages over several weeks. The contents of the sac are often swollen, which makes it impossible to return them into the small cavity all at once. The return of the sac contents into the abdominal cavity creates intra-abdominal pressure, which may cause the infant to have difficulty breathing. To help the infant breathe, a special breathing tube may be inserted. The tube is attached to a machine that regulates the length and frequency of the breaths. When the necessary surgeries have been completed, the suturing will be done in such a way as to leave, if possible, a somewhat normal-looking belly button. A large omphalocele repair can leave a large, unsightly scar. For cosmetic purposes, the scar may be operated on at a later date to make it less noticeable. Gastroesophageal reflux, which may require additional surgery, is common in patients with a repaired omphalocele.