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Gastrointestinal Tract | Abdominal wall defect - Gastroschisis

In babies with gastroschisis, the abdominal wall does not form completely, allowing the bowel (intestines) to pass outside of the fetal abdomen. Regular ultrasounds during pregnancy are needed to monitor fetal health.

Babies with gastroschisis need to be born in a hospital which can deliver specialized newborn care. Labour is usually induced a little before term and they are usually birthed vaginally at approximately 37 weeks gestation following induction of labour, and will usually require surgery relatively soon after birth. There is no need for routine Caesarean section for these babies.

Essential Information

  • In gastroschisis, there is a small opening in the fetal abdominal wall, usually just to the right of the umbilical cord, that allows the bowel to pass outside the abdomen, into the amniotic fluid.
  • Some fetuses will have only a small amount of bowel outside the body, while others may have most of their bowel outside, along with the stomach.
  • Gastroschisis occurs early in pregnancy and can usually be detected on ultrasound by 12-13 weeks gestation.
  • For most babies with gastroschisis, the long-term prognosis is very good.
  • Gastroschisis occurs in about 1 in every 5,000 births. It is more common in younger women. We do not yet fully understand the causes. It is not usually seen as part of a chromosome or genetic problem.

Overview

Gastroschisis is usually diagnosed during a routine pregnancy ultrasound in the second trimester. In gastroschisis, the fetal bowel is outside the body, in the amniotic fluid.

In later pregnancy, the acidity of the amniotic fluid can irritate the bowel, causing it to swell and thicken. This can increase the chance of the bowel becoming damaged or blocked. Sometimes there may be an intestinal atresia (bowel blockage), meaning that a portion of the bowel did not develop properly and is causing a blockage. As the fetus grows, the small opening in the abdominal wall may decrease the blood supply to the bowel. The bowel may also twist around itself. Both of these problems can cause damage to the bowel.

Regular ultrasounds are needed to monitor fetal health. Growth will be measured and the bowel will be monitored for increased size or thickening. The amount of amniotic fluid will be measured. Ultrasounds may be scheduled once or twice a week starting at approximately 30-34 weeks and continued until delivery.

Babies with gastroschisis do not need to be birthed by caesarean section. Induction of labour, with the goal of having a normal birth will occur at approximately 37 weeks gestation. Babies with gastroschisis need to be born in a hospital with access to a neonatologist (specialist in newborn babies) and a paediatric surgeon. All babies with gastroschisis will need to have surgery relatively soon after birth.

Treatment

When your baby is born, the bowel will be wrapped in gauze and plastic for protection, to keep the area clean and prevent moisture loss. The baby will be transferred to SickKids hospital. The paediatric surgeon will determine how soon after birth the baby will have surgery to close the abdomen. This may occur in one operation or in a staged procedure over several days. A silicone pouch, called a “silo”, is placed around the exposed bowel. Every day the silo is tightened to gradually ease more of the bowel into the baby’s abdomen. The silo is then removed and the skin on the baby’s abdomen is closed. This usually occurs within the first week of life.

Until the bowels have recovered from surgery, your baby will be unable to eat, and will be nourished intravenously. You can still breastfeed your baby, just not right away. If you wish to breastfeed, it is important to begin expressing or “pumping” milk as soon as possible after birth. Once the surgeon is satisfied that your baby’s bowels are working properly, your baby will be fed your milk. Sometimes it can take a few weeks before a baby with gastroschisis can take all of their nutrition in the form of milk.

Some babies who have damage to the bowel may need to have some portions of bowel removed surgically and very rarely, some babies can develop a “short gut” syndrome. Symptoms of this condition include diarrhea, slow weight gain, problems with absorption of water, vitamins and other nutrients from food. These babies may require longer hospital stays with IV nutrition called total parenteral nutrition (TPN). Long term care with TPN may also have some complications.

Referral Information (for physicians)

All fetuses with suspected gastroschisis should be referred to a tertiary care centre, such as Mount Sinai Hospital, for evaluation, monitoring and delivery.