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

J Pediatr Surg. 2004 Feb;39(2):152-6; discussion 152-6. doi: 10.1016/j.jpedsurg.2003.10.008.

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.

MeSH terms

  • Acetylcholinesterase / analysis*
  • Autonomic Nervous System Diseases / etiology*
  • Autonomic Nervous System Diseases / pathology
  • Child, Preschool
  • Constipation / etiology
  • Enteric Nervous System / enzymology
  • Enteric Nervous System / pathology*
  • Enterocolitis / complications
  • Enterocolitis / pathology*
  • Female
  • Ganglia, Autonomic / enzymology
  • Ganglia, Autonomic / pathology*
  • Hirschsprung Disease / surgery*
  • Humans
  • Infant
  • Infant, Newborn
  • Intestinal Mucosa / innervation*
  • Male
  • Nerve Tissue Proteins / analysis*
  • Postoperative Complications / pathology*
  • Retrospective Studies

Substances

  • Nerve Tissue Proteins
  • Acetylcholinesterase