Gastroschisis

What Is Gastroschisis?

Gastroschisis is a birth defect that develops in a baby while a woman is pregnant. This condition occurs when an opening forms in the baby’s abdominal wall. The baby’s bowel pushes through this hole. It then develops outside of the baby’s body in the amniotic fluid.

The opening is most often on the right side of the baby’s belly button. It can be large or small, but is typically 1-2 inches in size. In more severe cases, the stomach and/or liver can sometimes make their way through the opening as well.

Because the bowel is outside of the baby’s body, it is unprotected. That means there is a chance it can become irritated, swollen and damaged.

This condition is relatively rare, but has seen an increase in recent years. It occurs in about one in every 2,000 babies. It develops early in pregnancy, during the fourth through eighth weeks. Gastroschisis occurs due to a weakness in the baby’s abdominal wall muscles near the umbilical cord. If your baby develops this condition during your pregnancy, you will not experience any symptoms related to it.

Gastroschisis can be repaired with surgery after your baby is born. It is usually not associated with other malformations.

What Causes Gastroschisis? 

The exact cause of gastroschisis is not known. It does not appear to be inherited. Having one baby with gastroschisis does not make it more likely that you would have another baby with the condition. 

Gastroschisis is labeled as simple or complicated. This is based on how inflamed the bowel and/or organs are that have moved through the opening.

With simple gastroschisis, only the bowel makes its way out of the abdominal opening.

With complicated gastroschisis, one or more of the following occurs:

  • The bowel outside of the baby’s body is extremely damaged, e.g., a portion of the tissue has died (called necrosis), or the bowel has become twisted or tangled.
  • Intestinal atresia, which occurs when part of the baby’s bowel doesn’t form completely, or the intestine is blocked.
  • Other organs, such as the stomach or liver, protrude out of the opening as well.

Simple cases are more common than complicated ones.

It is possible for gastroschisis to be detected in the third month of pregnancy. However, we most often perform evaluations for it at 20-24 weeks, after it has shown up on an ultrasound. It is most commonly diagnosed by ultrasound around weeks 18-20 of pregnancy.

Some women are referred to us for gastroschisis late in pregnancy. We see them within two weeks of their referral. It is important to make a diagnosis and delivery plan as early as possible.

In babies with gastroschisis, the ultrasound will show loops of bowel floating freely. This often shows up when a woman goes in for a routine ultrasound with her obstetrician (OB). It is at this point that most of our patients affected by gastroschisis are referred to the Cincinnati Fetal Center. Here, we’ll work with you to assess how severe your case is and create a plan for the remainder of your pregnancy. We will also talk to you about what to expect after delivery.

An evaluation for gastroschisis consists of the following:

  • An ultrasound (we can use an ultrasound performed within two weeks of your appointment with us, or one will be done on the day of your evaluation);
  • Possibly an MRI and/or a fetal echocardiogram to test your baby’s heart function;
  • A meeting with a nurse, social worker and genetic counselor;
  • A team meeting with a maternal-fetal medicine specialist (MFM), pediatric surgeon and neonatologist.

An important part of the evaluation is determining whether the condition is gastroschisis or omphalocele. These conditions can sometimes look similar on an ultrasound. In omphalocele, a sac from the umbilical cord covers and protects the intestines that are outside of the baby’s body.

After your tests are complete, our team of experts meets with you to discuss the extent of the baby’s condition and its impact on the rest of the pregnancy. We’ll also cover medical treatments that might be needed right after the birth of your child, and long-term prognosis of babies with gastroschisis.

For patients who are local or plan to deliver locally, we also discuss the following:

We recommend frequent ultrasounds throughout the remainder of your pregnancy. These will help to monitor your baby’s health, the severity of the gastroschisis, and how it evolves.

Compassionate, Expert Care 

The Cincinnati Fetal Center offers comprehensive diagnostic tests and the latest treatments for gastroschisis. Just as important, our team of specialists takes time to explain test results, answer questions and discuss treatment options. We understand that parents are facing unique challenges. We provide as much support as possible every step of the way. Extensive experience and expertise allow our team to treat the most complex cases of this condition. 

Planning for Delivery 

Normally we recommend our patients deliver at a level III hospital, which is one that can provide advanced trauma care. A children’s hospital should be nearby with a pediatric surgeon available to perform the surgery after your baby’s birth.

It is possible for a woman to have a vaginal delivery unless there are obstetric concerns. Your doctor may suggest a C-section at about 37 weeks of pregnancy if your baby’s lungs are mature enough.

For patients who deliver locally, your baby will be transferred to Cincinnati Children’s soon after delivery. Once you are stable, you will be given a pass from your delivery hospital to visit your baby until you are discharged. Your baby will stay in the NICU for a period of time after surgery.

There are no fetal interventions recommended for babies with gastroschisis. The condition cannot be corrected while you are pregnant. Rather, it must be treated right after your baby is born.

Any baby with gastroschisis must have surgery after birth. An infant cannot survive with his or her bowel outside of the body.

After your baby is born, doctors will assess how severe the gastroschisis is. The type of repair needed depends on how much bowel and/or organs are outside of your baby’s belly and any inflammation or damage to those tissues.

Primary Repair

With a simple gastroschisis, treatment often is what’s called a “primary repair.” This is a surgery where the bowel is placed back inside of the baby’s belly and the abdominal opening is closed. When possible, this surgery is done the day your baby is born.

This type of repair is performed when there’s relatively small amount of bowel outside of the belly, and the bowel is not overly swollen or damaged.

Staged Repair

A primary repair might not be possible if:

  • Your baby has a large amount of bowel outside the body,
  • The bowel is very swollen, or
  • The baby’s belly doesn’t have enough room to hold all of the bowel.

In these cases, several surgeries may be needed to slowly place the bowel/organs back into the belly. This is called a “staged repair.” This takes place over several days and can last up to two weeks.

With a staged repair, a plastic pouch or “silo” is placed around the bowel and attached to the belly. Every day the silo is tightened and some of the bowel is gently pushed inside. When all the bowel is inside, the silo is removed and the belly is closed. Some babies may need the help of a breathing machine for a few days after the surgery or surgeries.

Of the gastroschisis repairs performed by the surgeons we partner with at Cincinnati Children’s, about 50% are primary, and the other 50% are staged reductions.

About 10 percent of babies born with gastroschisis also have a part of the bowel that does not develop correctly. In these cases, some babies may experience:

  • Bowel resection – a surgery on the bowel needed when part of the bowel is extremely damaged
  • Colostomy – an opening to allow to allow stool to pass out of the body and into a bag
  • Short bowel syndrome – when a large portion of the intestine does not work normally
  • Intestinal transplantation – when a new intestine is needed (rare)

Your baby’s bowel has developed outside of the belly. It needs to heal and adjust to functioning normally. Because of that, babies with gastroschisis commonly have feeding challenges the first few weeks of life.

During this time, your baby will receive IV nutrition. Babies with gastroschisis often need other treatments as well, including:

  • Medications for comfort,
  • Antibiotics to prevent infection, and
  • Careful attention to control body temperature.

Once your baby’s bowel is functioning—usually after about two to three weeks—breast milk or special formula will begin.

Your baby will be discharged once he or she is doing well with feedings and the bowel appears to be functioning normally. Hospital stays can range anywhere from 30-50 days or more.

A follow-up appointment will be scheduled with your neonatologist and/or pediatric surgeon. The doctor(s) will assess your baby’s feeding, development, and surgery site, and rule out any obstruction in the belly.

Babies with gastroschisis are usually smaller than average. After birth, it can take some time for them to catch up developmentally. Long-term problems mostly occur in the very complicated cases. These can be related to feeding, bowel or infection issues.

Babies with gastroschisis can have very different experiences based on how severe each case is. They first must recover from their initial surgical repairs, become successful at feeding, and their bowel must heal. After that, most babies who had gastroschisis can go on to live a normal, healthy life without complications related to the condition.

Last Updated 05/2016