Updated Results on Intestinal Neuronal Dysplasia (IND B)

Abstract

The diagnosis of IND B requires that biopsies are taken proximal to the ampulla recti (about 8-10 cm above the dentate line) with a sufficient amount of submucosa. The biopsies must be cut rectangular to the surface of the mucosa. A diagnosis of IND B can be made only if, in the submucosa of 30 serial sections, 15-20 % of all ganglia are giant ganglia with more than eight nerve cells. Ganglioneuromatosis (MEN2B) must be clearly differentiated from IND. The clinical course of IND B depends on the extent of disturbed bowel innervation, the severity of motility failure, and the coexistence of MH. The conservative management of isolated IND is possible in most children. In individual cases, however, a transient enterostomy or a segmental resection is unavoidable.

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

“…However, when the quantitative criteria of MeierRuge et al [5,22] were applied, only 4 of the 13 cases sustained the INDB diagnosis. IND proximal to a segment of aganglionosis is not uncommon and has been suggested to be a possible cause of persistent bowel problems after surgery for HD.…”

“…In the several published criteria, the giant ganglia are defined by the presence of a minimum number of ganglion cells ranging from 6 to more than 10 per ganglion [6,1821] . Given this lack of diagnostic standardization, in 2004, MeierRuge et al [5,22] proposed quantitative criteria for the histopathologic diagnosis of IND-B. They defined INDB by the presence of at least 20% giant nerve ganglia in the submucosa, with more than 8 ganglion cells each, based on the examination of a minimum of 25 submucosal ganglia.…”

“…They defined INDB by the presence of at least 20% giant nerve ganglia in the submucosa, with more than 8 ganglion cells each, based on the examination of a minimum of 25 submucosal ganglia. Additionally, they used a histochemical panel in frozen sections for the analyses of lactate dehydrogenase, succinyl dehydrogenase and nitric oxide synthase [5,22] . All of these changes in the proposed histopathological criteria for the diagnosis of INDB have not only caused disparities in its definitions but also skepticism about its existence.…”

Intestinal Neuronal Dysplasia Type B: An Updated Review of a Problematic Diagnosis

“…These giant ganglia comprise only 3-5% of all ganglia seen in a given case and are usually not observed in the distal rectum (within 6-7 cm of the pectinate line). 9 Because of the latter observation, Meier-Ruge insists that diagnostic biopsies be obtained 8 to 10 cm proximal to the dentate line, 13 which is much farther from the anus than the typical biopsy used to rule out HSCR.…”

“…Although consensus does not exist for the management of patients who receive a diagnosis of IND B, Meier-Ruge and others have cautioned that many patients improve with conservative medical management and resolve their symptoms by age 4 years. 13 In some centers, most IND B patients who failed conservative management are treated successfully by anal sphincter myomectomy. It seems likely that the IND B patients evaluated by Terra et al 39 failed medical management and represent a clinically severe end of the IND B spectrum.…”