Gastroschisis

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What is gastroschisis?

Gastroschisis is an opening in the abdominal wall that allows the stomach and small and large intestines to extend outside the body. This occurs during fetal development. The opening almost always occurs to the right of the navel (umbilicus) and is usually less than two inches in size. With the abdominal contents outside of the body and no protective sac covering these contents, the intestine is exposed to amniotic fluid, which may irritate the bowel, causing it to swell and shorten. The damage becomes more severe with prolonged exposure to amniotic fluid.

As the fetus grows, the small tight opening may decrease the blood supply to the intestine and/or the bowel may twist around itself. Both of these problems can result in poor bowel function after the baby is born. This can lead to long-term feeding problems.

Gastroschisis is an uncommon birth defect, seen in about 1 of 5,000 births. Although about 10% of infants with gastroschisis have an intestinal atresia in which a portion of the intestine does not develop, the defect is rarely associated with other genetic abnormalities.

 

What causes gastroschisis?

The cause of gastroschisis is unknown. It occurs early in fetal development. Rather than developing normally, the abdominal wall tears on the right side of the navel. As a result, the intestine extends through the opening and outside the body of the fetus.

 

How is gastroschisis diagnosed?

Gastroschisis is often diagnosed prenatally and can be seen on ultrasound as early as the 14th week of pregnancy. When maternal alpha-fetoprotein (AFP) levels are elevated, obstetricians look for defects by having the expectant mother undergo a detailed prenatal ultrasound. With gastroschisis, this test will show loops of bowel (intestines) floating freely in amniotic fluid. More frequent ultrasounds are generally recommended to continue monitoring the fetus. The mother is usually referred to a pediatric surgeon for consultation and counseling.

 

Making arrangements for delivery

Once the diagnosis is made, you might want to plan for your baby to be delivered in a hospital with a neonatal intensive care unit, where he / she can receive the special care that will be required. If ultrasound tests indicate that the baby’s lungs are mature, you may be advised to have a cesarean section at about 36 weeks of pregnancy. Surgery for the gastroschisis will be done as soon as the infant’s condition is stabilized, usually within 12 to 24 hours after delivery.

 

How is gastroschisis treated?

We favor a staged approach to return of the intestines to abdomen and closure of the abdominal wall defect. Shortly after delivery, in the NICU, a plastic pouch is first placed over the baby’s exposed bowel and a spring loaded ring retains the device in the abdomen below the defect. Each day, the pouch is reduced , returning the intestine back into the abdominal cavity. Once the intestine is back in its proper place within the abdomen, the pouch is removed and the opening is surgically closed in the operating room. We believe this approach is the least stressful for your newborn child. Some babies may need the help of a breathing machine (ventilator) during this time.

 

What is the long-term prognosis?

In most babies, the long-term prognosis is good. Nevertheless, some babies will sustain injuries to the bowel due to direct contact between the intestines and amniotic fluid or twisting resulting in loss of blood flow resulting in a blockage (atresia) or significant loss of bowel length during fetal development. In severe instances the damage to the intestines is so great that the baby has inadequate intestine to absorb nutrients and must be fed by vein. This is known as short gut syndrome. Diarrhea, slow weight gain and deficiencies of essential vitamins and minerals are symptoms of this condition. Babies with short gut syndrome may require a prolonged course of intravenous nutrition (TPN) delivered at the hospital or at home, and will need to be closely monitored by your pediatric surgeon, pediatric gastroenterologist and primary care physician.

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