Improved long-term outcome for patients with jejunoileal apple peel atresia

Background/Purpose: Although apple peal intestinal atresia is a rare lesion associated with significant morbidity and high mortality, the authors 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 these children, critiques our evolution in care, and gives current recommendations for therapy. Methods: A retrospective review of 12 patients over 11 years was conducted. Perinatal history and operative and perioperative management were examined and end results and complications using different management plans compared. Long-term outcome was determined 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 underwent a primary anastomosis and five had enterostomies. The proximal jejunum was tapered, plicated, resected or left intact. Eleven children required gastrostomy tubes. All children required total parenteral nutrition. Full enteral feeding was achieved in all children, but three required gastrostomy supplementation. Three patients who had enterostomies suffered bowel obstruction, two with dilated, dysmotile proximal jejunum required subsequent tapering. Eight children maintained a growth curve between the 5th and 50th percentile. None have short bowel physiology, and all have achieved acceptable bowel function. Conclusions: (1) Total parenteral nutrition is essential for initial nutritional management. (2) Use of an enterostomy leads to an increased incidence of complications. (3) The dilated proximal bowel should be resected, tapered, or plicated, and a primary anastomosis should be performed. (4) Gastrostomy tubes are necessary for initial management. (5) Early morbidity is common, though excellent long-term outcome and normal growth and development are expected.