Intestinal neuronal dysplasia: results of treatment in 33 patients.

PURPOSE: Intestinal neuronal dysplasia (IND) is a disease of the enteric nervous system, which clinically resembles Hirschsprung's disease. The authors reviewed their experience of IND over an 8-year period.

METHODS

Between 1992 and 1999, 418 patients underwent rectal suction biopsy 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 years). The diagnosis of IND was based on the presence of hyperganglionosis of the submucous plexus and giant ganglia and at least one of the following features in rectal biopsies: (1) ectopic ganglia, (2) increased acetylcholinesterase (AChE) activity in the lamina propria, and (3) increased AChE nerve fibers around the submucosal blood vessels. All patients were started on laxatives with or without enemas after the diagnosis was made. Patients have been followed up from 1 to 8 years (mean, 2.4 years).

RESULTS

Twenty-one (64%) patients had a good response to conservative management and currently have normal bowel habits. Twelve patients (36%) underwent internal sphincter myectomy after failed conservative management. Seven of these patients now have normal bowel habits. Two patients are able to stay clean with regular enemas. Three patients who continued to have persistent constipation after myectomy and underwent resection of redundant and dilated sigmoid colon now have normal bowel habits.

CONCLUSION

The majority of patients with IND can be treated successfully with conservative treatment.