Related Experiment Videos
Neuronal intestinal dysplasia: quantitative diagnostic criteria and clinical management
E Simpser1, E Kahn, K Kenigsberg
1Department of Pediatrics, North Shore University Hospital, Cornell University Medical College, Manhasset, NY 11030.
Insights
Neuronal intestinal dysplasia (NID), a condition mimicking Hirschsprung's disease, involves nerve cell hyperplasia. This study shows medical management, including saline irrigations, successfully treated an infant with NID, normalizing manometry and biopsy findings.
Area of Science:
- Pediatric Gastroenterology
- Surgical Pathology
- Intestinal Motility Disorders
Background:
- Neuronal intestinal dysplasia (NID) presents clinically similar to Hirschsprung's disease but is marked by nerve cell hyperplasia, not absence, in the intestinal wall.
- Surgical intervention is a common treatment for NID, but medical management options are less explored.
Abstract:
Neuronal intestinal dysplasia (NID) clinically resembles Hirschsprung's disease but is characterized by hyperplasia rather than aganglionosis of the intramural plexus. Surgical intervention is common. We report the 5-year follow-up of an infant with the mixed form of NID managed medically and a method by which NID can be quantified histologically. Hyperganglionosis was determined by counting the number of ganglia per high-power field and the number of ganglion cells per ganglia from at least two biopsy specimens. The patient's biopsies and biopsies from "normal" and "inflamed" patients were compared. Normals contained 0.68 +/- 0.28 (mean +/- SD) ganglia per high-power field and 2.16 +/- 0.31 ganglion cells per ganglion. The inflamed biopsies were similar, 0.69 +/- 0.38 ganglia per high-power field and 2.63 +/- 0.40 ganglion cells per ganglion. The patient's initial rectal biopsy revealed 7.6 ganglia per high-power field and 3.8 ganglion cells per ganglion. Management of the patient included saline colonic irrigations and hyperalimentation with gradual reinstitution of breast-feeding. Clinical improvement was associated with normalization of manometry and biopsy findings, a phenomenon not documented previously in the literature. Irrigations were stopped at age 9 months, and the child is now asymptomatic.