Gastroschisis and Omphalocele

An omphalocele is a defect in the umbilical ring that allows the intestines to protrude out of the abdominal cavity in a sac. A gastroschisis is a defect in the abdominal wall that allows the antenatal evisceration of abdominal structures without a sac being present. There is some controversy as to the exact embryology of the two conditions.

Omphaloceles are found in one out of 6000 to 10,000 births, while gastroschisis occurs twice as frequently in the newborn population. Omphaloceles have a higher (37 percent) incidence of associated anomalies, including chromosomal abnormalities. Three specific syndromes are associated with omphalocele: the upper midline pentalogy of Cantrell, Haller, and Ravitch (sternal, ventral, diaphragmatic, pericardial, and cardiac defects); the lower midline syndrome (vesicointestinal fissure); and the Beckwith-Wiedemann syndrome (macroglossia, visceromegaly, and hypoglycemia).

The emergency management of the two conditions is not different, especially when the sac in an omphalocele is ruptured. The eviscerated bowel should be wrapped in saline-soaked gauze and placed in a plastic bag to protect it from hypothermia and evaporative losses. A nasogastric tube should be inserted to decompress the intestines. Rapid infusion of 20 mL/kg of 5% Ringer's lactate may be necessary to restore vital signs, after which the infusion should be adjusted to maintain a urine output of at least 2 (mL/kg)/h. Intravenous antibiotics should be administered.

Primary fascial closure is the treatment of choice and is often accomplished within hours after birth. When the defect is large, a Silastic silo may be used, but survival nonetheless correlates with rapid closure and removal of the prosthesis.

Complications include gastroesophageal reflux, malabsorption, diarrhea, dehydration, and failure to thrive. The mortality of omphalocele is 25 to 30 percent, largely as a result of congenital heart disease and sepsis, while death in patients with gastroschisis is associated with intestinal atresia.

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