Gastroschisis

Excerpt

Gastroschisis is a paraumbilical, full-thickness abdominal wall defect associated with protrusion of the bowel through the defect. It is rarely associated with genetic conditions. A membrane does not cover the bowel exposed in utero and, as a result, may be matted, dilated, and covered with a fibrinous inflammatory rind. Infants have a high proportion of intrauterine growth restriction. Diagnosis is often made on the 20-week ultrasound with free-floating bowel loops in the uterine cavity. Maternal serum alpha-fetoprotein (AFP) is elevated in pregnancies with gastroschisis. Compared with other abdominal wall defects diagnosed prenatally, such as omphalocele, only 10 percent of cases with gastroschisis are associated with malformations outside of the gastrointestinal tract. Additional gastrointestinal problems occur in up to a quarter of cases. Infants can be classified and simple or complex, which can help stratify outcomes and care for infants born with gastroschisis. Complexity is based on the absence or presence of intestinal atresia, stenosis, bowel perforation, necrosis, malrotation, or volvulus. Infants may benefit from delivery in a facility with resources such as high-risk obstetrics, neonatology, and neonatal intensive care unit, and a pediatric surgeon—a trial of labor rather than scheduled cesarean birth for most patients. Spontaneous delivery usually occurs between 37 to 38 weeks gestation. A trial of spontaneous vaginal delivery is supported. The exposed infant bowel is protected following birth, an orogastric tube is placed, as are peripheral IVs. The airway is stabilized. Gastroschisis closure can be performed operatively or through slow bowel reductions utilizing a spring-loaded silo to contain the bowel. While a small percentage of infants have intestinal atresia, bowel loss, and prolonged hospitalizations, the overall survival is greater than 90%.