Gastroschisis

Overview

In gastroschisis, the muscles of the abdominal wall do not close correctly while the fetus is developing. Part of the stomach or intestines protrudes outside the fetus's body, floating in the amniotic fluid. The defect is similar to omphalocele; however, in omphalocele, the organs are contained in a thin, membranous sac instead of floating in the amniotic fluid.

The amount of abdominal contents protruding outside of the fetus varies from case to case. Some are very small — just a few loops of bowel — while others can be quite large, involving most of the intestine and stomach.

Most babies with gastroschisis are born to mothers in their late teens or early twenties. The cause of the defect is unknown.

Outcomes

Gastroschisis ranges in severity, depending on the condition of the intestine. Fortunately, most fetuses with gastroschisis do not have severe damage to the intestine before birth. The relatively normal intestine can be returned to the abdomen and the defect closed with one or two surgeries shortly after birth. These babies will still be in the intensive care nursery for several weeks before the intestines work well enough to allow for feeding and discharge home. However, these babies eventually feed and grow normally.

Ten to twenty percent of fetuses with gastroschisis have significant damage to the intestine that greatly complicates their course after birth and, occasionally, can be fatal. Babies born with damaged intestine can have a very difficult and prolonged stay in the intensive care nursery. These babies often require several operations to return the intestine to the abdomen using a plastic silo and eventual closure of the abdominal wall. The bowel can be so damaged that parts of it have to be removed.

In the worst cases, there may not be enough bowel left to absorb food. The most severely affected babies may not survive, and others may be left with "short bowel syndrome." At the very least, these babies will require nutritional support in the nursery for many months.

Diagnosis

An ultrasound can be used to accurately diagnose gastroschisis and distinguish it from similar conditions, such as omphalocele. However, it can't determine the severity of the bowel damage. It may be necessary to perform ultrasounds every few weeks to see if the bowel outside the fetus's body becomes dilated, develops a thick wall or loses some blood flow.

Since eight of 10 babies with gastroschisis don't have damaged bowel and do fine after birth, it is important to identify the two babies who will have badly damaged bowel and may benefit from fetal intervention before birth. At UCSF, we follow all fetuses with a careful ultrasound examination every week or two to look for any change in the bowel.

Treatment

Monitoring the Baby and Preparing for Delivery

Babies with gastroschisis should be carefully monitored throughout the pregnancy for intrauterine growth retardation — not growing enough while in the womb — and for damage to the intestines. Intestines can be damaged by exposure to the amniotic fluid or by impaired blood flow to the exposed intestine.

Since most fetuses with gastroschisis do well with regular ultrasound observation and near-term delivery at an appropriate hospital, the most important decisions relate to where to deliver and the medical team who will care for your baby before and after birth.

Babies with gastroschisis should be delivered at a center where the intestines can be immediately covered and kept warm and moist until surgical repair or silo placement can be performed. The biggest threat to the baby and to the intestine's condition is to have to transport the baby to another medical center or in any way delay the repair.

Therefore, delivery plans should be coordinated with your perinatologist (an obstetrician who specializes in high-risk pregnancies), neonatologist (a pediatrician who specializes in caring for newborns) and pediatric surgeon.

Contrary to previous belief, babies with gastroschisis do not have to be delivered via Caesarean section. Delivering your baby vaginally will not harm you or your baby.

Treatment After Birth

Your baby should be born at a hospital with an intensive care nursery and a pediatric surgeon available. Soon after birth, your child will have surgery to close the opening in the abdominal wall and return the organs to the abdomen. The pediatric surgeon will attempt to close the opening at the time of surgery, but sometimes this is not possible.

If the gastroschisis is too large, a silo is placed. A silo is a covering placed over the abdominal organs on the outside of the baby. Gradually, the organs are squeezed by hand through the silo into the opening and returned to the body. This method can take up to a week.

Babies with gastroschisis can spend anywhere from two weeks up to three to four months in the hospital. Because your baby's intestine will have been floating in amniotic fluid for months, it will be swollen and will not function well. The function of the gastrointestinal tract and the baby's ability to tolerate feedings will determine the length of the hospital stay. Babies are discharged from the hospital when they are taking all their feedings by mouth and gaining weight.

After discharge from the hospital, your baby has a small risk for developing bowel obstruction due to scar tissue or a kink in a loop of bowel. Symptoms of bowel obstruction include:

• Bilious (green) vomiting
• Bloated stomach
• No interest in feeding

If any of these symptoms occur, contact your pediatrician immediately.