What is gastroschisis?
Gastroschisis is a condition that occurs in approximately 1 in 5,000 live births. It is one of a group of birth defects known as abdominal wall defects, which occur very early in gestation and are characterized by an opening in the abdominal wall (belly) of the fetus. This condition may be detected by an ultrasound as early as week 12 of pregnancy.
Most of the time, it is only some of the intestines that come out through the hole and are exposed to the amniotic fluid around the fetus. In more severe cases, other organs (liver, bladder, ovary or testes) can also come partially or completely out through the hole. This opening is usually found to the right of the umbilicus (belly button). The unprotected intestine may become irritated but the amniotic fluid, causing it to swell and shorten.
We do not know what causes gastroschisis. It is more common in young mothers. It does not appear to be inherited or “run in families.” Having one baby with gastroschisis does not make it more likely that you would have another baby with it.
What happens during pregnancy with gastroschisis?
Pregnancies in which the fetus has gastroschisis are at risk for certain complications such as poor fetal growth, decreased amniotic fluid volume, preterm delivery and stillbirth. Our Center routinely monitors mothers starting at 32 to 34 weeks gestation with twice-weekly non-stress tests and ultrasounds, keeping a close watch for signs of fetal distress. Normally, women with a baby with gastroschisis can have a vaginal delivery, and cesarean delivery is only performed for standard obstetrical reasons. A vaginal birth permits the mother to have a shorter and easier recovery, and does not increase the risk for complications in the baby.
During pregnancy, our nurse navigator will arrange for you to meet with some of the doctors who will take care of your baby after birth. These specialized doctors are neonatologists and pediatric surgeons. You can even tour the newborn intensive care unit (NICU) where your baby will be cared for before and after the gastroschisis repair is performed.
We usually recommend delivery a little early (around 37 weeks) for babies who are small and have gastroschisis, and moms are typically able to deliver vaginally. Babies with gastroschisis usually do not need to be delivered by C-section.
How is gastroschisis treated?
This is a condition that can’t be treated before the baby is born. You and your doctor will work closely together to monitor your pregnancy.
A team of neonatal specialists will be in the delivery room to address any problems your baby might have and will make sure that the baby’s intestines are covered, protected and kept moist with a plastic bag. A small soft tube will be placed through the baby’s mouth or nostril into baby’s stomach. This tube removes fluid and air in the baby’s stomach, so that the intestines do not become bloated. Because your baby cannot be fed by mouth, an intravenous catheter (IV) will be placed to provide nutrition and hydration. After you see your baby, the baby will be taken to the newborn intensive care unit (NICU). Neonatologists and pediatric surgeons will evaluate and care for your baby there.
Treatment to repair the gastroschisis will begin as soon as possible after birth, sometimes the same day. If the baby has a small amount of bowel outside his or her body, this might be repaired with one surgery. This is called a “primary repair.” During the surgery, the bowel is placed in the belly and the opening is closed.
If the baby has a large amount of bowel outside the body, the baby is very small, or he/she has breathing problems, the surgeon will place the bowel into a cylinder shaped clear plastic sleeve, this sleeve is called a “silo.” The silo protects the bowel from the environment and allows the team to assess the condition of the bowel until surgical closure is possible. While the silo is in place, the surgeon will slowly and gently push the bowel into the abdomen. This procedure is often done at the bedside. When this method is used it is called a “staged repair.” This process might take three to 10 days, sometimes longer. When the bowel is placed into the abdomen it creates pressure on the diaphragm and can make breathing difficult. Some babies may need the help of a breathing machine during and after the procedure.
Regardless of the type of gastroschisis repair, it takes time for the intestine to recover and for function to return. For this reason, nutrition is provided through an intravenous (IV) line. After surgery, the baby may not be able to eat for several weeks, since the intestines need time to heal. Once intestinal function returns, oral feedings or feedings via nasogastic (NG) tube are slowly started while IV feeds continue. Breastmilk is easy for babies to digest and is particularly important for these babies. Our nurses will teach you to pump and store your milk so it is ready when your baby needs it.
The length of stay in the NICU after gastroschisis repair varies greatly and is difficult to predict. Close attention will be paid to the condition of the intestine and the baby’s growth. Most babies heal well, but some babies need additional surgeries if they develop intestinal blockage. A few babies have long lasting problems with the way their intestinal system works and will require special treatment tailored to their specific needs.
Photo credit: Centers for Disease Control and Prevention, National Center on Birth Defects and Developmental Disabilities.