Inflammatory changes secondary to postoperative complications of Hirschsprung’s disease as a cause of histopathologic changes typical of intestinal neuronal dysplasia

Presented at the 50th Annual Congress of the British Association of Paediatric Surgeons, Estoril, Portugal, July 15–18, 2003.
https://doi.org/10.1016/j.jpedsurg.2003.10.008Get rights and content

Abstract

Purpose

The aim of this study was to clarify the pathogenesis of intestinal neuronal dysplasia (IND).

Methods

The bowel habits of 36 postoperative HD patients were assessed retrospectively. Twenty-five had no complaints. Seven had persistent enterocolitis and were the focus of our study. They were divided into group A (n = 2) if they were severe and had associated postoperative surgical complications, and group B (n = 5) if they were mild. The histological changes were assessed.

Results

The 7 patients who had persistent enterocolitis postoperatively had no AchE activity in the mucosa, and there was normal distribution of submucosal and myenteric ganglia in the proximal resection margin. Rectal biopsies performed postoperatively for investigation of persistent enterocolitis in group A showed inflammatory changes and typical histopathologic features of IND such as abundant acetylcholinesterase (AchE)-positive nerve fibers in the lamina propria associated with giant submucosal ganglia and hyperganglionosis, and in group B there was increased AchE activity without hyperganglionosis or giant ganglia.

Conclusions

This is the first report of histopathologic changes typical of IND occurring in response to persistent enterocolitis related to postoperative complications of surgery for HD.

Section snippets

Materials and methods

From 1995 through 2002, 36 cases of HD were diagnosed and treated at the Department of Pediatric Surgery at Juntendo University School of Medicine. Twenty-five patients were boys and 11 were girls. Their age at the time of diagnosis ranged from 4 days to 4 years.

Thirty one had rectosigmoid HD, and 5 had long-segment HD. Two had Down’s syndrome. H&E staining of frozen sections obtained at the time of definitive surgery confirmed there was normal distribution of ganglion cells at the proximal

Results

The most proximal margin of all resected specimens was examined, and all but 2 were confirmed to have normal histology on H&E staining and AchE immunohistochemistry. Both had typical findings of IND.

Discussion

Unlike HD, which is a multigenic disorder, the pathogenesis of IND remains unclear. Its etiology is unresolved, with diverse histopathologic mechanisms including developmental disturbances, reactive changes, or inflammatory diseases being discussed.

Data about combined malformations involving IND are limited, and the pathogenesis of IND is not yet understood fully, so an analysis of associated malformations and morbidity may shed light on how and why intestinal dysganglionosis develops. On

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