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Gastroschisis

Gastroschisis

What is gastroschisis?

Gastroschisis is a hole in the abdominal wall of the fetus that allows the intestines to protrude outside of the baby’s body, where they are exposed to the amniotic fluid. The birth defect occurs early in fetal development when the abdominal wall doesn’t close properly.

This opening is located next to the baby’s navel, typically on the right side. In severe cases, the stomach and/or liver are also outside of the baby’s body.

Treatment for gastroschisis involves surgery shortly after birth to move the intestines inside the baby’s body and close the opening in the abdominal wall. Gastroschisis is not generally associated with other abnormalities or genetic disorders.


How does gastroschisis affect my baby?

Babies with gastroschisis face risks that include:

  • Irritated, swollen intestines caused by exposure to the amniotic fluid
  • Blocked or reduced blood flow to the protruding organs or twisting of the intestines, in cases where the opening tightens up during fetal growth, causing major damage to these organs
  • Blockage or narrowing in the bowel, known as intestinal atresia
  • Lower than normal birth weight
  • Problems with feeding, digestion, dehydration and absorption of nutrients after birth
  • Preterm delivery and stillbirth

The outlook for each baby depends on the size of the opening, the abdominal organs protruding outside of the fetus, and the extent of damage to the baby’s intestines and other organs before birth.


Cause and Prevalence

Gastroschisis is believed to be caused by disruption of the blood supply to the abdominal wall during fetal development, preventing it from closing all the way.

The defect is estimated to occur in 1 in 5,000 live births. According to the Centers for Disease Control and Prevention (CDC), recent studies show the birth defect is becoming more common. The condition occurs more often among mothers in their late teens or early twenties.


Gastroschisis or Omphalocele?

Gastroschisis is one of two main types of abdominal wall defects. A similar but more serious condition is omphalocele, in which the abdominal organs protrude through the baby’s navel and are covered by a sac or membrane. Children with omphalocele often have other birth defects.

Accurate diagnosis is critical to proper prenatal care and treatment planning for your baby’s condition.


Testing and Diagnosis

Gastroschisis is usually detected during a routine prenatal blood test and ultrasound. High levels of a protein in the mother’s blood known as alpha-fetoprotein (AFP) can indicate certain fetal defects, including abdominal wall defects. An ultrasound scan can confirm the diagnosis with imaging of the organs outside the fetal body.

If gastroschisis is diagnosed during your pregnancy, you may be referred to a fetal center with specialized expertise in the care of complex and rare fetal conditions, including abdominal wall defects.

At Texas Children’s Fetal Center, we arrange for you to visit as quickly as possible to meet with a team of specialists experienced in diagnosing and treating gastroschisis, including maternal-fetal medicine (MFM) physicians, neonatologists, pediatric surgeons and fetal imaging experts. Our specialists will perform a comprehensive evaluation and additional testing to gain critical about your baby’s condition for proper treatment planning.

Testing may include:

  • Anatomy ultrasound to confirm the diagnosis, distinguishing it from omphalocele, and gain more information about your baby’s defect
  • Fetal MRI for a more detailed view to assess the severity of the condition
  • Fetal echocardiogram to evaluate your baby’s heart and look for any heart defects

Following this thorough assessment, our specialists will meet with you about your results, answer any questions your family has, and provide treatment recommendations based on your baby’s gastroschisis. 


Treatment During Pregnancy and Delivery

You and your baby will be closely monitored throughout your pregnancy, with regular ultrasounds to assess fetal growth and any changes in your baby’s condition, including damage to the intestines caused by the amniotic fluid or impaired blood flow.

Because your baby’s intestines will be exposed at birth, delivery and postnatal care should be carefully planned and coordinated with a team of maternal-fetal medicine specialists, neonatologists, and pediatric surgeons experienced in the treatment of gastroschisis.

Delivery should take place at a center equipped to address both the mother’s needs in pregnancies complicated by birth defects and the immediate surgical and intensive care needs of babies with gastroschisis, avoiding the need to transport your newborn, delay treatment and risk injury to the exposed bowel.

Babies with gastroschisis can typically be delivered vaginally. Because of the increased risk of stillbirth, in some cases delivery may be scheduled between 35 and 37 weeks of gestation, with labor induced.

At delivery, the exposed abdominal organs will be placed in a special “bag” to keep them warm, moist, and protected. Your baby will be moved to a neonatal intensive care unit (NICU) for surgery. Our gastroschisis patients are expertly cared for in Texas Children’s level IV NICU, the largest and highest-ranked NICU in the South, providing the highest-level of care available for premature and critically ill babies. 


Surgical Repair After Birth

Surgery is performed shortly after birth to move the intestines and any other protruding organs into the baby’s body and close the opening in the abdominal wall. When completed in one surgery, this is known as a “primary repair.”

In some cases, a more gradual approach may be needed to move the organs into the baby’s body. Known as a “staged repair,” this approach may be necessary when:

  • The baby’s condition is unstable
  • There is a large volume of intestines or multiple organs outside the body
  • The intestines are extremely swollen from prolonged exposure to the amniotic fluid
  • The baby’s abdominal cavity isn’t large enough immediately after birth to hold the organs

In a staged repair, a long, plastic pouch called a “silo” is placed around the exposed intestines immediately after birth and attached to the baby’s abdomen. The other end of the pouch is hung above the abdomen and the organs are gradually pushed down through the defect and into the baby’s body. The process typically takes 5 to 10 days. When all of the organs are inside the baby and any intestinal swelling has reduced, the silo is removed and surgery is performed to close the opening in the abdomen.

In addition to surgery, babies with gastroschisis typically need other treatments such as antibiotics to prevent infection in the exposed organs, an intravenous (IV) line for nutrients, and close monitoring for hypothermia, as excessive heat may be lost through the exposed organs.


After Surgery

Following surgery, your baby will remain in intensive care until the intestines begin functioning normally and they can feed by mouth and gain weight. Hospital stays after gastroschisis repair vary from several weeks to several months, depending on the extent of damage to the baby’s intestines before birth.

In rare cases, when the baby is born with severely damaged intestines that prevent the passage of food or absorption of sufficient nutrients, the child may have a prolonged hospital stay to receive the nutrition support required for normal growth. Additional surgeries may be required.    


Long-Term Outcomes

After discharge, your baby will need follow-up visits with their pediatric surgeon and neonatologist to monitor their growth and development and the health of their intestines.

While most babies do not experience long-term problems following gastroschisis repair, in severe cases children may continue to have difficulties with feeding, digestion or infection issues. Our specialists work closely with Texas Children’s Intestinal Rehabilitation Clinic to coordinate your baby’s care across a multidisciplinary team, ensuring expert care for children with gastrointestinal and nutrition problems. 


Why Texas Children’s Fetal Center?

  • A single location for expert maternal, fetal and pediatric care. At Texas Children’s Hospital, you and your baby can get the specialized care required for the diagnosis and treatment of gastroschisis all in one location, avoiding the need to transport your critically ill newborn and delay surgery. Texas Children’s Hospital is home to the first neonatal intensive care unit (NICU) to be designated by the Texas Department of State Health Services (DSHS) as a level IV NICU, the highest level of care available for premature and critically ill newborns.
  • A skilled, experienced team with proven outcomes. We have a dedicated team of maternal-fetal medicine specialists, fetal and pediatric surgeons, neonatologists, fetal imaging specialists, cardiologists, and others who work in concert to care for you and your baby every step of the way, using proven protocols we’ve developed over the years. With their combined expertise and unified approach, these physicians offer the best possible care for babies with gastroschisis.
  • We care for your child’s needs at every stage of life. Our comprehensive approach starts with your first prenatal visit for gastroschisis and continues throughout your child’s delivery, postnatal care, and childhood, as needed, thanks to one of the nation’s leading teams of fetal and pediatrics specialists for the treatment of fetal abnormalities and rare birth defects.

Patient Stories

For more information or to schedule an appointment,

call Texas Children’s Fetal Center at 832-822-2229 or 1-877-FetalRx (338-2579) toll-free.

Our phones are answered 24/7. Immediate appointments are often available.