What is Gastroschisis?

Having a baby is an exciting time! The 20-week ultrasound is a chance for parents to get a glimpse of their little one, find out the gender of the baby and make sure everything is developing as it should. Sometimes this is not the case, and the family finds out that their baby will need the special attention by a team of surgeons, doctors and nurses after delivery. One condition that occurs while the baby is developing that requires this expert care as soon as the baby is delivered is called gastroschisis.

Gastroschisis is a birth defect of the abdominal wall. This defect causes the intestines (and sometimes stomach and/or liver) to exit the abdomen from a small hole, usually to the right of the umbilical cord, where the abdominal muscles and skin did not form.

The exact cause of this defect is unknown, but it is rarely associated with a genetic abnormality (not inherited). The bowel is exposed to the amniotic fluid while in utero and can become inflamed, swollen, and sometimes stuck together. The bowel occasionally does not develop properly and can have an atresia or stenosis (obstruction). Very rarely, the defect can start to close and can restrict or completely cut off blood flow to the intestines prior to delivery (vanishing gastroschisis).

Incidence of Gastroschisis

The incidence of infants born with gastroschisis in the United States is approximately 1 per every 2,000 live births. Over 95% of these infants are born to mothers younger than 35 years of age. There are theories that environmental exposures also play a significant role on incidence.

Treatment

If a baby is diagnosed with gastroschisis during pregnancy, the mother is referred to specialists to be educated on and prepare for delivery and NICU admission. Delivery is typically planned to occur within or near a high level NICU. After delivery, the infant is placed on a warming bed and is placed in a plastic bag up to their armpits to prevent the intestines from becoming “dried out”. IV fluids are given, and the surgeon evaluates the defect and the amount of bowel involved.

There are different techniques used to repair the defect. Primary repair can occur if the intestines are not swollen and can be easily replaced back into the abdomen and the defect stitched closed. A silo (spring loaded plastic bag) is utilized for other closure approaches. After the intestines are placed in the silo bag, the bag is suspended vertically above the infant. This allows the gradual replacement of intestines back into the abdomen. Typically, the surgeons will assist with the reductions with gentle milking of the bag (think of a toothpaste tube) and then placing a tie on the bag to keep the intestines in place.

After the intestines have been reduced to skin level there are two different methods for abdominal closure, the first being surgical closure in the operating room. The second is a non-surgical approach, sutureless closure, and uses the umbilical cord to cover the defect. The belly is covered with a large bandage to allow gradual healing of the defect. All methods require the baby to be monitored in the NICU and given nutrition through an IV line. The average length of admission in the NICU is approximately 1 to 1.5 months, however, complications can lead to a longer stay.

Outcomes

Generally, the survival of infants with gastroschisis is greater than 90%, with good overall health. This survival rate is largely dependent on the health of the bowel at the time of birth. There are potential complications such as infection, feeding difficulties, and bowel obstruction that can cause long-term difficulties and affect the overall health of the baby. For these reasons, the infants are followed by the surgical team as and other specialties as needed in outpatient clinics (developmental team, feeding team, gastrointestinal specialists) to ensure that they have the best outcomes possible.