Intestinal neuronal dysplasia: Results of treatment in 33 patients

Purpose: Intestinal neuronal dysplasia (IND) is a disease of the enteric ne rvous system, which clinically resembles Hirschsprung's disease. The author s reviewed their experience of IND over an 8-year period. Methods: Between 1992 and 1999, 418 patients underwent rectal suction biops y for persistent constipation. Thirty-three (7.8%) patients had histologic evidence of IND. There were 26 boys and 7 girls (age range, 1 week to 10 ye ars). The diagnosis of IND was based on the presence of hyperganglionosis o f the submucous plexus and giant ganglia and at least one of the following features in rectal biopsies: (1) ectopic ganglia, (2) increased acetylcholi nesterase (AChE) activity in the lamina propria, and (3) increased AChE ner ve fibers around the submucosal blood vessels. All patients were started on laxatives with or without enemas after the diagnosis was made. Patients ha ve been followed up from 1 to 8 years (mean, 2.4 years). Results: Twenty-one (64%) patients had a good response to conservative mana gement and currently have normal bowel habits. Twelve patients (36%) underw ent internal sphincter myectomy after failed conservative management. Seven of these patients now have normal bower habits. Two patients are able to s tay clean with regular enemas. Three patients who continued to have persist ent constipation after myectomy and underwent resection of redundant and di lated sigmoid colon now have normal bowel habits. Conclusion: The majority of patients with IND can be treated successfully w ith conservative treatment. J Pediatr Surg 36:777-779. Copyright (C) 2001 b y W.B. Saunders Company.