Clinical Relevance of Hirschsprung-Associated Neuronal Intestinal Dysplasia (HANID)

Abstract

The rate of Hirschsprung-associated neuronal intestinal dysplasia (HANID) is reported to be as high as 20-75% but no report deals with its sequelae. The aim of our study was to evaluate the influence of the retained neuronal dysplastic segment in patients with Hirschsprung's disease (HD). We report on 47 patients with HD including 11 cases with HANID (23%). All 47 children had a Duhamel procedure subsequent to colostomy and in none of the 11 cases with HANID was the neuronal dysplastic segment resected. There was no significant difference of early and late complications in both groups and the results of the mean follow-up of 5 years after the Duhamel procedure show that the patients with HANID did as well as the patients with isolated HD. It is suggested therefore that HANID may be a distinct disease compared to isolated NID and that the NID attained segment may be retained without increased risks or morbidity.

Histopathological criteria for intestinal neuronal dysplasia of the submucosal plexus (type B)

“…These observations support the hypothesis that IND B, hypoganglionosis of the myenteric plexus and aganglionosis, which are often combined, are developmental abnormalities with an identical pathogenesis [7,13,45]. This, however, contradicts the idea of Hanimann et al that the combination of aganglionosis and IND B (which they called "HANID") is a separate disease-entity [17], different from isolated Hirschsprung's disease and isolated IND B.…”

“…IND B combined with aganglionosis [5,10,15,17,19,25,34] is not morphologically different from isolated IND B. In 64 patients with Hirschsprung's disease and IND B, the biopsy diagnosis was generally made at 12_+6 months in comparison to 4+2 months in isolated Hirschsprung patients.…”

“…However, low or absent propulsive activity in the first months of life due to IND B in the proximal colon causes fairly late obstruction (12.+6 months instead of 4.+6 months in Hirschsprung's disease). If the IND B segment matures and normal propulsive activity develops, no major problems are to be expected after resection of the aganglionic segment [17,52,[53][54].…”

Hirschsprung's disease and intestinal neuronal dysplasia - a frequent association with implications for the postoperative course

“…Fadda et al [1] reported motility disorders in one-third of children with HaIND and Kobayashi et al [8] found defecation disorders in all of their patients with HaIND. Hanimann et al [3] described constipation in the early postoperative course in 18.2% of their patients with HaIND and 8.3% with isolated HD. All of their patients underwent a Duhamel procedure, without total resection of the IND segment if one was found.…”

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

“…1 IND can occur as an isolated form or in association with HD proximal to the aganglionic segment. A close association between IND and HD is recognized and was termed Hirschsprung-associated IND by Hanimann et al 2 The reported incidence of isolated IND varies from 0.3% to 62% of all rectal suction biopsies. 3 On the contrary, 25% to 35% of patients with HD have associated IND, frequently related to postoperative complications after definitive surgery for HD.…”