Near-total intestinal aganglionosis: Long-term follow-up of a morbid condition

Purpose: The aim of this study was to understand the long-term outcome of n ear-total intestinal aganglionosis (NTIA). Methods: The study was an institutional review of ail patients with NTIA be tween 1974 and 1999, inclusive. Seven full-term babies were identified (4 b oys, 3 girls). Mean birth weight was 3.2 kg (range, 2.7 to 4.1). Patients i ncluded 1 pair of siblings and 1 Down's syndrome baby. All 7 had obstructio n within 5 days of life. One patient experienced bowel perforation. All 7 h ad NTIA diagnosed within the first 2 months of life. In 2 of 7, aganglionos is began 10 cm distal to the ligament of Treitz (LOT). The longest segment of normal small bowel was 130 cm from the LOT. Five of 7 babies underwent a stoma between day of life (DOL) 1 and 15. Results: Two of three "long-term" survivors (>2 years) had corrective surge ry by 13 months. The first received extended jejuno-ileal myotomy and myect omy with multiple postoperative problems. She died at 8 years of age of com plications of total parenteral nutrition (TPN). The second underwent myotom y, resection and patch graft to jejunum, and several jejunostomy revisions. All of the involved bowel eventually was resected. The 3 of 7 who lived mo re than 1 year all took some nutrition orally. Three died, between 3 months and 8 years of age, of complications of TPN. There are 2 survivors at 3 an d 7 years of age. Survival has not correlated with the length of aganglioni c bowel. Conclusions: Aganglionosis involving most of the bowel has a high morbidity and mortality rate. Since 1990 a more aggressive surgical approach has res ulted in improved survival rates but with significant morbidity. For childr en surviving beyond 3 months of age, outcome was less dismal. Some patients may benefit from extended jejunal myotomy or myectomy. However, postoperat ive complications are the rule, not the exception. Copyright (C) 2000 by W. B. Saunders Company.