Management of gastroschisis with concomitant jejuno-ileal atresia

Background: The incidence of jejuno-ileal atresia in neonates concomitantly found to possess gastroschisis has been reported to be 5% to 25%. Initial treatment for this condition has not been well established. Methods: Thirteen newborns with gastroschisis and coexisting jejuno-ileal atresia, were identified and treated at our institution over the past 16 years (1978 through 1996). Patient characteristics at presentation, surgical therapy, and complications at extended follow-up were reviewed. Results: All neonates were preterm (mean gestational age, 35.2 ± 2.0 weeks) and of low birth weight (2.1 ± 0.4 kg). Atresia types II, IIIa, IIIb, and IV were identified at the initial surgical procedure in one, eight, one, and three patients respectively; however, one synchronous small bowel atresia went unrecognized. A primary anastomosis was fashioned in 8 of 13 newborns, the creation of which did not influence length of hospitalization, length of total parenteral nutrition (TPN) requirement, complication rate, or survival; however, reoperation was required in two of eight patients to mediate anastomotic complications. Atresia associated with intestinal gangrene or perforation was treated by primary anastomosis in three of six patients and the remaining three by enterostomy. Primary abdominal wall closure was possible in 10 of 13 patients; a Silon pouch was required in three. All nine survivors (69%) displayed protracted small bowel dysfunction requiring TPN (mean TPN duration of 3.6 ± 3.0 months; range, 1 to 11). Mortality in four patients was a consequence of severe prematurity, Silon pouch wound sepsis, or TPN-induced cirrhosis. Conclusions: When technically feasible, restoration of intestinal continuity by primary anastomosis is a reasonable treatment option in patients with coexisting gastroschisis and jejuno-ileal atresia. Favorable outcome is as much a function of supportive care and parenteral nutrition as the type of surgical repair performed for either the intestinal or the abdominal wall defect.