Gastroschisis

What is gastroschisis?

Gastroschisis is a birth defect of the abdominal (belly) wall. Birth defects are structural changes present at birth that affect almost any part of the body. They may affect how the body looks, works or both. Birth defects can cause problems in overall health, how the body develops or how the body works. About 1,871 babies are born with gastroschisis each year in the United States. Several studies show that gastroschisis has become more common in recent years.

Gastroschisis is a type of abdominal wall defect. These conditions develop as a baby grows inside the womb. Normally, the intestines, stomach, liver, bladder and other organs grow outside your baby’s body at first. The organs usually move inside the body before the baby is born. The intestines are long tubes that are part of your digestive system. The digestive system helps your body break down food, take in nutrients and remove waste.

Gastroschisis happens during early pregnancy when the muscles that make up a baby’s abdominal wall don’t connect properly. A hole in the abdominal wall forms beside the belly button and the intestines and other organs, like the stomach or liver, can poke through the hole and out of the body. The hole can be small or large but is usually 1 to 2 inches in size.

In a baby with gastroschisis, there is no membrane to cover and protect the organs, so they are exposed to amniotic fluid (the fluid that surrounds a baby in the womb). A membrane is a thin layer of tissues or cells that acts like a barrier. It allows some things to pass through, but stops other things. Amniotic fluid can irritate the intestines and other organs. This can cause them to swell, shorten or twist. The organs may be covered by thick skin that looks like leather (also called a peel).

How do you know if your baby has gastroschisis?

Gastroschisis can be found during pregnancy or after your baby is born.

Diagnosing gastroschisis during pregnancy

You may find out your baby has gastroschisis from a routine prenatal test (medical tests you get during pregnancy) that checks your baby for birth defects. Your health care provider also may check your baby for gastroschisis if you have a condition called polyhydramnios during pregnancy. Polyhydramnios is when you have too much amniotic fluid.

Your provider may use these tests to diagnose gastroschisis during pregnancy:

• Fetal echocardiogram (also called fetal echo). This test uses ultrasound to make a picture of your baby’s heart while still in the womb. Some babies with gastroschisis also have heart problems.
• Maternal blood screening (also called quad screen). This screening test is called a quad screen because it measures four substances in your blood: alpha-fetoprotein (also called AFP), estriol, human gonadotropin (also called hCG) and inhibin A. The test is done at 15 to 22 weeks of pregnancy. If you have high levels of AFP, your baby may have gastroschisis.
• Magnetic resonance imaging (also called MRI). This is a medical test that uses a large magnet and radio waves to make a detailed picture of the inside of the body.
• Ultrasound. This test uses sound waves and a computer screen to show a picture of your baby inside the womb. If your baby has gastroschisis, an ultrasound may show the bowels floating outside of your baby’s belly. Health care providers may find gastroschisis as early as 10 weeks of pregnancy on ultrasound, but it’s most often diagnosed between 18 and 20 weeks. You may need several ultrasounds during your pregnancy so your provider can monitor the intestines and other organs as your baby grows, checking for any changes to them.

Diagnosing gastroschisis after your baby is born

If gastroschisis isn’t found during pregnancy, your provider can diagnose it at birth.

Do babies with gastroschisis need special medical care?

Yes. Gastroschisis can’t be treated during pregnancy. Soon after your baby is born, he needs surgery to put his organs back inside the body. Your baby can’t survive without surgery.

Your health care provider may recommend you give birth at a hospital that has a newborn intensive care unit (also called NICU). The NICU is the nursery in a hospital where sick newborns get medical care. Most of the time, gastroschisis can be fixed with one or two surgeries. After recovering from surgery, most babies with gastroschisis live normal lives. Some children may have problems with digestion later in life.

What health care providers treat gastroschisis?

You and your baby may have a team of providers who work together to treat gastroschisis and related complications. This team may include:

Doctors, including:

• A maternal-fetal medicine specialist (also called MFM) is a doctor with special education and training to take care of women who have high-risk pregnancies
• A neonatologist is a pediatrician who has special medical training to take care of sick newborns, including preterm babies and babies with birth defects. Preterm babies are born too early, before 37 weeks of pregnancy. They may have more health problems and may need to stay in the hospital longer than babies born later.
• A pediatrician treats babies and children
• A pediatric gastroenterologist treats the digestive system in babies and children
• A pediatric surgeon has special training doing surgery on babies and children
• A urologist treats problems of the urinary tract (kidneys, bladder, ureters and urethra)

Other kinds of providers, including:

• A social worker helps people solve problems and make their lives better. Most hospitals have a social worker who can help connect you with the resources you need to care for your baby.
• A genetic counselor is trained to help you understand about genes, birth defects and other medical conditions that run in families. A genetic counselor can help you learn about how these things can affect your health and your baby's health.
• An occupational therapist is trained to help people learn how to do everyday activities. For children, this may be things like brushing teeth, getting dressed, putting on shoes or learning to use a pencil. If your baby has feeding problems, she may need treatment from an occupational therapist.
• A physical therapist creates exercise programs to help improve strength and movement.

How is gastroschisis treated?

Gastroschisis treatment depends on your baby’s health at birth. There are two types of treatment:

1. Primary gastroschisis repair. If only some of the intestine is outside of your baby’s belly and it’s not very swollen, your baby has surgery soon after birth to put it back into the belly and close the hole. This is called primary gastroschisis repair. If possible, your baby has surgery on the day he’s born. Right after birth, your baby’s surgeon places the intestine that’s outside the belly in a special plastic pouch called a silo. The silo protects the intestine from infection, dehydration and any additional damage. Dehydration is not having enough water in the body. Your baby gets general anesthesia, medicine that puts your baby to sleep so he doesn’t feel pain during surgery. Your baby’s surgeon examines your baby’s intestine closely for signs of damage or other birth defects. She removes unhealthy parts of the intestine and stiches together the healthy parts. The surgeon puts the intestine back into the belly and closes the hole in the belly wall.
   
2. Staged repair. The repair of your baby’s gastroschisis defect may be done slowly, in stages, if many organs are outside the belly or your baby isn’t healthy enough for primary repair. Your baby also may have staged repair if the intestine is very swollen, there’s a large amount of intestine outside the body or his belly isn’t big enough to hold all of the intestine.

Your baby needs time to heal after surgery. He gets fed through a needle in the vein (also called IV) at first. Once his intestines begin to work properly (usually in 2 to 3 weeks), he may be fed by mouth or through a nasogastric tube (also called an NG tube). An NG tube goes through your baby’s nose, down the esophagus and into your baby’s stomach.

You may choose to pump and freeze breast milk for feedings. Over time, your baby can do more feedings by mouth. This is a slow process, and your baby may need to stop during feedings to rest his intestine. In most cases, your baby can go home in 1 or 2 months, when he can take full feedings and has gained some weight.

Your baby may need more surgery later to repair the muscles in his belly. About 1 in 10 babies (10 percent) born with gastroschisis also have a part of the bowel that does not develop correctly. In these cases, your baby also may need:

• A bowel resection. This is surgery on a very damaged bowel to remove part or all of the intestine.
• A colostomy. This is a surgery that brings one end of the large intestine our through an opening (also called a stoma) made in the belly wall. Stools move through the intestine drain through the stoma into a bag that’s attached to the belly.
• An intestine transplant. This is surgery to put in a new intestine. This surgery is rare.

What are some common complications in babies with gastroschisis?

Babies with gastroschisis may be born prematurely or born small due to slow growth in the womb before birth. Babies with gastroschisis may have complications, including:

Breathing problems and heart problems. Your baby may need a breathing tube and breathing machine (also called a ventilator) for a few days or weeks after surgery.

Feeding and digestion problems. A baby with gastroschisis may have scar tissue or an intestinal blockage that affects digestion. An intestinal blockage is when food or stool can’t move through the intestines. Some organs may have been damaged when your baby’s provider squeezed them through the hole in the belly. This can decrease blood flow to the intestines and kidneys, causing your baby to have digestion problems after birth.

In rare cases, babies with gastroschisis have a part of the intestine that is thinner than normal, are missing a part of the intestine or have a twisted intestine. These defects can damage the intestine and cause problems with digestion and absorbing nutrients from food. This condition is called short bowel syndrome.

Infections. About 1 in 3 babies with gastroschisis develops necrotizing enterocolitis (also called NEC). NEC happens when the tissue of the intestine is damaged and dies. In some cases, a hole may form in the intestine (also called an intestinal perforation). If this happens, waste and bacteria from inside your baby’s intestine can get into the blood or the abdominal cavity. The abdominal cavity is the area between your baby’s belly and spine (backbone).

How are gastroschisis complications treated?

Your baby may lose a lot of fluids and have a low temperature because some of his organs are outside of his body. Babies with gastroschisis may need:

• Antibiotics to prevent infection. These are medicines that kills certain infections.
• An NG tube to drain his stomach and keep it empty
• A warming lamp to raise his body temperature
• Fluids and nutrients given through an IV to prevent dehydration
• Oxygen to help him breathe
• Pain medications to make him comfortable
• Support to lay in a way that makes sure his intestines get enough blood flow

What causes gastroschisis?

We don’t know what causes gastroschisis, but some things may make you more likely to have a baby with gastroschisis than others. These are called risk factors. Having a risk factor doesn’t mean for sure that your baby will be affected. But it may increase your chances. Talk to your provider about things you can do to help reduce your risk for gastroschisis.

Risk factors for gastroschisis may include:

• Age. Teen mothers are more likely to have a baby with gastroschisis than mothers who are 20 years old or older.
• Drinking alcohol or smoking during pregnancy. Don’t drink alcohol or smoke if you’re pregnant or trying to get pregnant.
• Genes changes (also called mutations). Genes are the parts of your body’s cells that store instructions for the way your body grows and works. Genes are passed from parents to children. A gene can change on its own, or it can be passed from parents to children. Changes in genes are sometimes linked to gastroschisis. If gastroschisis runs in your family, you may want to talk to a genetic counselor. Gastroschisis usually happens in babies with no family history of the condition.
• Poor nutrition. Women who don’t eat enough fruits and vegetables may be at increased risk of having a baby with gastroschisis. Eat healthy foods, including lots of fruits and vegetables, to help you have a healthy pregnancy and a healthy baby.
• Taking opioids during pregnancy. Prescription opioids are painkillers often used for pain after an injury, surgery or dental work. They include codeine, morphine and oxycodone. Ask your provider if there’s a safer medicine you can switch to during pregnancy to manage your pain. Don’t stop taking an opioid without talking to your provider first. Quitting suddenly can cause severe problems for your baby. 

More information

• Centers for Disease Control and Prevention
• Avery’s Angels Gastroschisis Foundation

Last reviewed: June, 2019