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Gastroschisis is an opening in the abdominal wall (muscles and skin of the abdomen) that appears during fetal development. The opening, which is usually less than two inches in size, occurs almost always to the right of the umbilical cord. As a result, the stomach, small and large intestines are not enclosed in the abdominal wall and appear outside of the fetus's body, leaving the intestine exposed to amniotic fluid, which contains the baby's urine. This contact may irritate the bowel, causing it to swell and shorten.
As the fetus grows, the opening may become smaller, and may tighten around the intestine, or the bowel could twist around itself. Both of these problems may lead to poor function of the bowel after delivery. This may lead to long term feeding problems.
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Gastroschisis is seen in about 1 in every 5,000 births, making it an uncommon birth defect. It is rarely associated with other chromosomal or structural abnormalities, although about 10% of infants have an intestinal atresia in which a portion of the intestine does not develop.
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Normally, during early development, the intestines, stomach and liver protrude to the outside of the body. As the fetus grows, these organs are "pulled in" and the abdominal wall forms around them. This does not occur in gastroschisis. As with many birth defects, the reason this occurs is unknown.
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Gastroschisis is often diagnosed prenatally. If your maternal alpha-fetoprotein (AFP) levels are high, your obstetrician may want you to have a detailed ultrasound. The ultrasound will show loops of bowel (intestines) floating in amniotic fluid. Because injury of the bowel can occur later on in pregnancy more frequent ultrasounds are recommended to closely monitor the fetus. For these, you will probably be referred to a neonatal surgeon.
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It is best that the baby be delivered in a hospital with a neonatal intensive care unit that specializes in the care of very sick babies. It may be advisable to deliver the baby at about 36 weeks of pregnancy if the lungs are mature, and, in this case, a Caesarian delivery may be optimal. IVs and tubes will be placed to give the baby fluids. The baby's intestines will be placed in a see through surgical plastic bag and the baby will be brought to the neonatal intensive care unit. Surgery will take place within 12-24 hours. This will help prevent dehydration (fluid loss), low temperature, and infection.
In a small or medium size gastroschisis, one staged repair includes returning the bowel contents into the abdomen and closing the skin. If the gastroschisis is large, or there are other problems, it may need to be closed in a staged procedure over 3-5 days. A silastic sheet (silo) is placed around the exposed bowel. Every day, the silo is tightened to push more bowels into the baby's abdomen. The silo is then removed and the skin on the baby's abdomen is closed.
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As the baby is recovering and waiting for the intestines begin to function normally, IV nutrition is given to provide calories and fluid. Once the intestines function, feedings of breast milk or formula by mouth or by a tube placed through the nose into the stomach will begin. The baby will be observed closely in the hospital until he/she can take in calories to gain weight, before going home. At discharge, there will be communication with your baby's pediatrician to update him on his hospital stay, and progress over the following months.
The prognosis for these babies is very good. However, some babies who have sustained injuries to the bowel, either due to direct contact between the intestines and amniotic fluid, or because the intestine was twisted or damaged in some way, may develop a condition known as "short gut." This condition is characterized by diarrhea, very slow weight gain, and deficiencies of important vitamins and minerals.
These babies will be followed closely by the surgeon, pediatric gastroenterologist and primary care doctor and they may require a prolonged course of intravenous nutrition (TNP) delivered either at the hospital or at home.
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Gastroschisis
