Gastroschisis

An infant with gastroschisis. With this condition, the intestines and other internal organs push outside of the body.

What is gastroschisis?
Gastroschisis (gas-tro-ski-sis) is an opening in the abdominal wall through which the internal organs push outside of the baby's body (see illustration at right). During fetal development the abdominal wall fails to close properly leaving an opening. The opening is usually to the right of the umbilical cord. This anomaly or birth defect is not commonly associated with any other defects except bowel strictures and/or atresias (narrowing of the intestines). It occurs in approximately 1 out of every 5,000 births. The cause of gastroschisis is unknown, but it is associated with younger maternal age and almost never occurs in mothers over 30 years of age. It is not related to anything the mother ate or did during pregnancy.

Prenatal diagnosis of gastroschisis
Gastroschisis may be suspected when the alpha feto protein (AFP) blood work is elevated. This blood test may also be referred to as a "triple screen." The diagnosis is confirmed by ultrasound. With ultrasound, the bowel and perhaps the liver will be seen floating in the amniotic fluid. Your obstetrician will most likely refer you to a specialist that handles high-risk pregnancies. These doctors are called perinatologists.

How does gastroschisis affect my baby?
Gastroschisis is not commonly associated with any other birth defects. It is usually an isolated defect. Gastroschisis is usually not associated with chromosome disorders. The only problems that are common with these babies are related to their gastrointestinal system. Approximately 20 to 40 percent will have some type of gastrointestinal abnormality such as: malrotation (bowel is not in correct position), atresia (passage is blocked), volvulus (twisting of the bowel, blood supply can be cut off), or infarction (blood supply has been cut off and that area will be damaged).

Babies with gastroschisis are at an increased risk for stillbirth. The incidence of stillbirth is approximately 10 percent. These babies will often be smaller than normally expected. It is estimated that as many as 75 percent will be classified as growth restricted. This means the baby weighs less than we would expect for the gestational age.

A baby with gastroschisis will need to go to surgery soon after birth, so it is best if they are born at a hospital that can care for baby and mother.

How does the gastroschisis diagnosis affect the pregnancy?
The diagnosis of gastroschisis is confirmed by ultrasound. Because gastroschisis is usually not associated with chromosomal abnormalities, an amniocentesis is not normally necessary. However, if the liver is outside the abdominal cavity, there is a suspicion that the defect may be a ruptured omphalocele. Omphaloceles are associated with chromosomal abnormalities and an amniocentesis may be offered.

A baby with gastroschisis will often be smaller than would be expected. Therefore monthly ultrasounds will be done to assess fetal growth, and if there is a concern about the size, these may be done more frequently along with other tests to confirm the well-being of the baby.

Fetal movement counting will begin at approximately 26 weeks of gestation. You will be instructed to count your baby's movements once a day, every day, for a maximum of two hours. Your baby should move a minimum of 10 times in that two hours. If the baby moves ten times in 10 minutes you are done for that day. However if later in the day you notice your baby is not moving as much, you can always repeat the test. It is good to pick the same time each day and to do the test after eating. We often recommend a good time to do fetal movement counting is after a meal. If your baby fails this test call your doctor immediately or go directly to labor and delivery. A fetus with gastroschisis is at an increased risk for stillbirth (approximately 10 percent). Twice weekly nonstress tests will begin at around 32 weeks' gestation. Weekly biophysical profiles will begin at the same time. A biophysical profile combines the results of the nonstress test with an ultrasound.

Delivery is scheduled between 35 to 37 weeks' gestation because of the increased risk for stillbirth. Labor is usually induced. The preferred method of delivery is vaginal. This defect does not require that a Caesarean section be performed for delivery except for usual obstetrical indications.

In the Fetal Concerns Program, should you decide to deliver at Froedtert Hospital, you will deliver just an elevator ride away from where your baby will be cared for. You will receive a tour of the Birth Center before delivery. The Birth Center is actually located within CHW. All of the specialists you need to care for your baby are immediately available to you and your newborn.

If the intestines do not all fit into the body, the remainder of the exposed bowel is placed into a sac, or "silo" on the abdomen.

How is gastroschisis treated?
Because this happens very early in the pregnancy, prolonged exposure to amniotic fluid causes the exposed bowel to become thick, swollen and inflamed. Once the baby is born, the internal organs are now exposed to air and unprotected. This makes the bowel more susceptible to infection and causes the baby to lose heat and fluids. Until surgery the bowel will be covered with moist, warm, sterile gauze and the lower half of the baby's body will be placed in a bag to hold moisture and heat in.

Your baby will need surgery. During surgery the organs will be put back inside your baby's abdomen. If possible all the bowel will be put back into the abdominal cavity at this initial surgery. This is referred to as a "primary closure." If the intestines do not all fit the remainder of exposed bowel will be placed in a sac or "silo" on the abdomen, that is reduced daily or as your baby tolerates. Sometimes with this procedure babies experience more difficulty breathing as the bowel increases pressure on the diaphragm. The abdominal wall will be closed at a later time in surgery, once all the bowel is in the abdomen.

Level III institutions such as Froedtert Hospital and Children's Hospital of Wisconsin would be able to care for both mother and baby, keeping them together and avoiding the trauma of having to transport the baby after birth. It is also beneficial in reducing the time required to get the baby to surgery, and decreases the chance of injury to the exposed bowel. Babies born at Froedtert Hospital get to the operating room within two hours, while those born elsewhere average more than six hours to get to surgery.

An infant with a repaired gastroschisis.

What about after surgery?
After surgery the baby may not be able to breathe effectively on his/her own due to the increased pressure on the diaphragm from the bowels that are now in the abdominal cavity. A special tube will be placed at the time of surgery (if it was not needed earlier) to assist with breathing. This tube will stay in for a time until the baby can breath effectively on his/her own. Typically, about three days after final closure of the abdomen the baby no longer needs support from the ventilator and is extubated.

Another tube will be passed into your baby's stomach at delivery, either through the nose, which is nasal gastric (NG), or mouth, which is oral gastric (OG). Suction will be applied to this tube to keep the stomach empty so as not to cause more pressure on the diaphragm.

Your baby will not be able to eat for a time after surgery. Some special intravenous lines are put in place while the baby is in the operating room for surgery. A central IV line will be placed to so that nourishment (vitamins, minerals, calories, fat) can be given for a prolonged period of time. An arterial line will be placed in one of the arteries in the arm. IV fluid and medications can be given, blood pressure can be monitored, and blood removed through this line for lab work. Before he/she goes to surgery a peripheral IV will be put in his/her hand.

Your baby will need frequent blood draws for lab work to monitor oxygenation, electrolytes, blood count and other things. Your baby may be on a variety of medications that may include: antibiotics to fight infection and pain medication to control post operative pain. Your baby may look puffy or swollen after surgery. Especially the upper and lower legs may be swollen. This is called edema. It will go away as your baby gets stronger and his/her body adjusts to the pressure of the bowels being back in the abdomen.

Because babies with gastroschisis cannot eat for a prolonged period, special nourishment is given through the central line. Total parenteral nutrition (TPN) is a solution that contains protein, fats, sugar, vitamins and minerals. This will supply your baby with all nutritional requirements until he/she is able to take food by mouth.

The hardest part of recovery for babies with gastroschisis is learning to eat and tolerating food. Their bowels were thick, swollen and inflamed at birth and require a long time to get adjusted to being inside the body. In approximately 10 percent of infants with gastroschisis there will be bowel atresias or narrowings. These infants may then need additional surgeries to remove these narrowings to allow normal passage of food. We wait for signs from the baby that the bowels are beginning to work. These signs include bowel sounds, spontaneous passing of stool, and a decrease in the amount of drainage coming from the tube in the baby's stomach. Feedings are typically started through the tube (NG or OG) at a slow rate. This rate is gradually increased as the baby tolerates.

Will I be able to help care for my baby after surgery?
Yes! Please ask your baby's nurse about ways to interact with and care for your baby.

We encourage you to breast-feed your baby and until the baby can eat by mouth we can help you pump your breast milk. A lactation consultant can assist in getting you started with pumping and answer any questions you may have. Your milk will be frozen and stored in the Neonatal Intensive Care Unit until your baby is ready for it. The NICU has breast pumps and private rooms available to you when you are visiting. You can bring in pictures, small toys, booties, and blankets for your baby while he/she is in the NICU.

When can my baby go home?
Your baby can go home when he/she is eating and tolerating enough food to allow them to grow and gain weight. A typical baby with gastroschisis will be in the NICU for approximately 6 to 12 weeks. A lot of this time will be spent working with him/her on learning to eat. There are speech therapists available to assist in encouraging him/her to take a bottle and lactation consultants to help with breastfeeding.

About 10 percent of babies with gastroschisis will have complications such as strictures or an inability to eat by mouth that may require more surgery. If this happens the stay may be prolonged.

What is my baby's long-term prognosis?
Your baby is not expected to need any special care once you go home. He/she may go home on medications to prevent and treat acid reflux, and will likely remain on these medications for the first few months after discharge. Your baby's pediatric surgeon and your regular pediatrician will follow them.

Learn more about services at Children's Hospital of Wisconsin:
Gastroenterology Clinic
Wal-Mart/Sam's Club Feeding, Swallowing and Nutrition Center
Surgery Clinic