Chapter 119

1. Definition. Gastroschisis is a centrally located, full-thickness abdominal wall defect with two distinctive anatomic features.

1. The extruded intestine never has a protective sac covering it.

1. The umbilical cord is an intact structure at the level of the abdominal skin, just to the left of the defect. Typically, the opening in the abdominal wall is 2–4 cm in diameter, and the solid organs (the liver and spleen) reside in the peritoneal cavity.

2. Pathophysiology. Exposure of unprotected intestine to irritating amniotic fluid in utero results in its edematous, indurated, foreshortened appearance. Because of these intestinal abnormalities, development of appropriate peristalsis and effective absorption is significantly delayed, usually by several weeks. Fortunately, associated congenital anomalies are rare in patients with gastroschisis.

3. Clinical presentation. The infant is born with varying amounts of extruded intestine via the defect. The intestine is swollen and friable. There is an association with atresias from blood supply disruption in utero.

4. Diagnosis. The key differential diagnosis is ruptured omphalocele, although the diagnosis is readily apparent in most cases. Increasingly, prenatal ultrasonography identifies gastroschisis.

5. Management

1. General considerations. All agree that infants with gastroschisis should be delivered at a neonatal center equipped and staffed to provide definitive care. Less certain is the recommended mode of delivery. Some experts argue that an abdominal wall defect is an indication for cesarean delivery. However, other investigators note that, in the absence of other factors, vaginal delivery does not increase the mortality, morbidity, or length of hospital stay for newborns with gastroschisis.

1. Specific measures

1. Temperature regulation. Immediate attention should be directed toward maintenance of normal body temperature. The tremendous intestinal surface area exposed to the environment puts these infants at great risk for hypothermia.

1. Protective covering/position. It is best not to keep replacing moist, saline-soaked gauze over the exposed intestine because doing so promotes evaporative heat loss. It is better to apply a dry (or moist) protective dressing and then wrap the abdomen in layers of cellophane. A warm, controlled environment should be provided. The infant should be laid on its side so the vascular pedicle of the intestine is not "kinked" while awaiting surgical intervention.

1. Broad-spectrum antibiotic coverage is appropriate, given the unavoidable contamination.

1. Total parenteral nutrition. A protracted ileus is to be expected, and appropriate intravenous nutritional support must be provided.

1. Surgical correction. As soon as the infant's condition permits, operative correction should be undertaken. Complete reduction of herniated intestine, with primary closure of the abdominal wall, or placement of unreduced intestine in a protective prosthetic silo, with subsequent staged reduction over 7–14 days, is usually performed. Gastroschisis is associated with long (weeks) periods of ileus so central venous access is also usually part of the surgical intervention.

1. Definition. An omphalocele is a herniation of abdominal contents into the base of the umbilical cord. The gross appearance of omphalocele differs from that of gastroschisis in two important respects:

1. A protective membrane encloses the malpositioned abdominal contents (unless rupture has occurred, eg, during the birth ...

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