Gastroschisis

(See also Overview of Congenital Gastrointestinal Anomalies Overview of Congenital Gastrointestinal Anomalies Most congenital gastrointestinal (GI) anomalies result in some type of intestinal obstruction, frequently manifesting with feeding difficulties, distention, and emesis at birth or within 1 or... read more .)

The estimated incidence is 1 in 2500 live births (more common than omphalocele Omphalocele An omphalocele is a protrusion of abdominal viscera from a midline defect at the base of the umbilicus. (See also Overview of Congenital Gastrointestinal Anomalies.) In omphalocele, the herniated... read more , in which the abdominal viscera protrude from a midline defect at the base of the umbilicus). In gastroschisis, unlike omphalocele, there is no membranous covering over the intestine, which is markedly edematous and erythematous and is often enclosed in a fibrin mat. These findings indicate long-standing inflammation due to the intestine being directly exposed to amniotic fluid (ie, chemical peritonitis). Infants with gastroschisis have low incidence of associated congenital anomalies (10%) other than gastrointestinal abnormalities such as malrotation Malrotation of the Bowel Malrotation of the bowel is failure of the bowel to assume its normal place in the abdomen during intrauterine development. Diagnosis is by abdominal x-ray. Treatment is surgical repair. (See... read more and intestinal atresia, which occur in about 25% of cases.

Gastroschisis can be suspected before birth by abnormally high alpha-fetoprotein levels on routine blood tests and can be detected by prenatal ultrasonography; when confirmed, delivery should take place at a tertiary care center.

At delivery, the exposed viscera should be immediately covered with a sterile, moist, nonadherent dressing (eg, medicated petrolatum gauze that can then be covered with plastic wrap) to maintain sterility and prevent evaporation. The infant should then be given IV fluids and broad-spectrum antibiotics (eg, ampicillin, gentamicin), and a nasogastric tube should be placed. The amount of fluids needed is typically significantly higher than that needed for a normal healthy neonate (1.5 to 2 times) because of excessive fluid loss from the exposed gut.

The infant is evaluated for associated anomalies before surgical repair. Primary closure is done when feasible. When a large amount of bowel is exposed, the abdominal cavity may be too small to accommodate the viscera. In this case, the viscera are covered by a pouch or silo of polymeric silicone sheeting, which is progressively reduced in size over several days as the abdominal capacity slowly increases, until all of the viscera are enclosed within the abdominal cavity. More recently, sutureless repair of gastroschisis has been done using the umbilical cord or a synthetic dressing to cover the defect. More research is required to assess the effectiveness of this approach.

It often takes several weeks before gastrointestinal function recovers and oral feedings can be given; occasionally, infants have long-term problems caused by abnormal intestinal motility.