Are giant ganglia a reliable marker of intestinal neuronal dysplasia type B (IND B)?

Abstract

It has been suggested that giant ganglia are a marker for a developmental bowel disorder, intestinal neuronal dysplasia of the submucosal plexus (IND B), diagnosed in a proportion of patients with severe intractable constipation. Diagnosis of this condition, however, remains controversial with a wide variation in the frequency of diagnosis in different centres. Our aim was to assess the frequency with which giant ganglia could be found in the bowel of individuals who did not give a history of life-long constipation. We also aimed to assess the reproducibility of giant ganglia counts. For this two pathologists independently assessed pieces of normal bowel taken away from the site of the lesion in patients who had undergone surgery for colorectal carcinoma. Giant ganglia containing seven or more ganglion cells were found in 76 and 78% of subjects by each of the two pathologists. There was 1 giant ganglion per 10 ganglia counted in those patients in whom they were identified and 1 giant ganglion per 10.9 ganglia overall. Sections from eight patients in whom there was a history of constipation and/or melanosis coli did not show a greater number of giant ganglia. We conclude therefore that so-called "giant ganglia" are a common feature in the submucosa of normal bowel and that the presence of occasional giant ganglia cannot be considered diagnostic of IND B.

Quantitative morphometric analysis of the submucous plexus in age-related control groups

“…However, the existence of this disease entity is open to controversy [2,8,9] because the criteria for this diagnosis have been defined based merely upon observations in patients with bowel motility disturbances. Furthermore, no age-related reference data from unaffected controls are available.…”

A Practical Guide for the Diagnosis of Primary Enteric Nervous System Disorders

“…Some studies have challenged the dogma of giant ganglia as a diagnostic criterion: Lumb and Moore (66) have shown in an adult study the presence of up to 62% of ganglia with >7 neurons, making them a common feature. This clearly raises concerns that previous series included an unrepresentative cohort.…”

Intestinal neuronal dysplasia type B: A still little known diagnosis for organic causes of intestinal chronic constipation

“…INDB has been considered to be secondary to acquired phenomena caused by congenital obstructions or inflammation occurred during pre, peri or postnatal periods in humans [12,13,18,56,57] . Morphological findings suggestive of INDB have been observed in intestinal segments proximal to areas of intestinal atresia, rectal mucosal prolapse and ileostomy, intestinal intussusception, imperforate anus and necrotizing enterocolitis [56,58,59] . This secondary histopathologic response to a bowel obstruction has also been tested in experimental studies with conflicting results [6062] .…”