IMPROVED LONG-TERM OUTCOME FOR PATIENTS WITH JEJUNOILEAL APPLE PEEL ATRESIA

Background/Purpose: Although apple peal intestinal atresia is a rare l esion associated with significant morbidity and high mortality, the au thors have seen no deaths since 1983. Similar success has rarely been reported, and there are no reports of long-term follow-up. This study examines the short-term and long-term complications and outcome for th ese children, critiques our evolution in care, and gives current recom mendations for therapy. Methods: A retrospective review of 12 patients over 11 years was conducted. Perinatal history and operative and peri operative management were examined and end results and complications u sing different management plans compared. Long-term outcome was determ ined through clinic follow-up. Results: Mean follow-up was 5.1 years. Children had a mean 61.4 cm of total small bowel. Seven patients under went a primary anastomosis and five had enterostomies. The proximal je junum was tapered, plicated, resected or left intact. Eleven children required gastrostomy tubes. All children required total parenteral nut rition. Full enteral feeding was achieved in all children, but three r equired gastrostomy supplementation. Three patients who had enterostom ies suffered bowel obstruction, two with dilated, dysmotile proximal j ejunum required subsequent tapering. Eight children maintained a growt h curve between the 5th and 50th percentile. None have short bower phy siology, and all have achieved acceptable bowel function. Conclusions: (1) Total parenteral nutrition is essential for initial nutritional m anagement. (2) Use of an enterostomy leads to an increased incidence o f complications. (3) The dilated proximal bowel should be resected, ta pered, or plicated, and a primary anastomosis should be performed. (4) Gastrostomy tubes are necessary for initial management, (5) Early mor bidity is common, though excellent long-term outcome and normal growth and development are expected. Copyright (C) 1997 by W.B. Saunders Com pany.