Intestinal atresia and stenosis: a 25-year experience with 277 cases.

OBJECTIVE To evaluate the causes, clinical presentation, diagnosis, operative management, postoperative care, and outcome in infants with intestinal atresia. DESIGN Retrospective case series. SETTING Pediatric tertiary care teaching hospital. PATIENTS A population-based sample of 277 neonates with intestinal atresia and stenosis treated from July 1, 1972, through April 30, 1997. The level of obstruction was duodenal in 138 infants, jejunoileal in 128, and colonic in 21. Of the 277 neonates, 10 had obstruction in more than 1 site. Duodenal atresia was associated with prematurity (46%), maternal polyhydramnios (33%), Down syndrome (24%), annular pancreas (33%), and malrotation (28%). Jejunoileal atresia was associated with intrauterine volvulus, (27%), gastroschisis (16%), and meconium ileus (11.7%). INTERVENTIONS Patients with duodenal obstruction were treated by duodenoduodenostomy in 119 (86%), of 138 patients duodenotomy with web excision in 9 (7%), and duodenojejunostomy in 7 (5%) A duodenostomy tube was placed in 3 critically ill neonates. Patients with jejunoileal atresia were treated with resection in 97 (76%) of 128 patients (anastomosis, 45 [46%]; tapering enteroplasty, 23 [24%]; or temporary ostomy, 29 [30%]), ostomy alone in 25 (20%), web excision in 5 (4%), and the Bianchi procedure in 1 (0.8%). Patients with colon atresia were managed with initial ostomy and delayed anastomosis in 18 (86%) of 21 patients and resection with primary anastomosis in 3 (14%). Short-bowel syndrome was noted in 32 neonates. MAIN OUTCOME MEASURES Morbidity and early and late mortality. RESULTS Operative mortality for neonates with duodenal atresia was 4%, with jejunoileal atresia, 0.8%, and with colonic atresia, 0%. The long-term survival rate for children with duodenal atresia was 86%; with jejunoileal atresia, 84%; and with colon atresia, 100%. The Bianchi procedure (1 patient, 0.8%) and growth hormone, glutamine, and modified diet (4 patients, 1%) reduced total parenteral nutrition dependence. CONCLUSIONS Cardiac anomalies (with duodenal atresia) and ultrashort-bowel syndrome (<40 cm) requiring long-term total parenteral nutrition, which can be complicated by liver disease (with jejunoileal atresia), are the major causes of morbidity and mortality in these patients. Use of growth factors to enhance adaptation and advances in small bowel transplantation may improve long-term outcomes.