Clinical Long-Term Follow-Up Results in Intestinal Neuronal Dysplasia (IND)

Abstract

Commonly available information on intestinal neuronal dysplasia (IND) is sparse. Especially well documented long-term courses are lacking. The aim of this study was to correlate defecation as a clinical parameter of the long-term course in malformations of the enteric nervous system with the morphological diagnosis. 57 children with intestinal neuronal dysplasia (IND) or aganglionosis with cranial intestinal dysganglionosis (agIND), diagnosed between 1983 and 1992, were analysed including histomorphological classification, collection of clinical data and evaluation of the defecation mode by questionnaire as a parameter of the long-term course. Of 29 dysganglionic (IND) patients, 9/29 cases (31 %) had been treated conservatively, 18/29 cases surgically (62.1%), in two children (6.9%) no therapy had been necessary. All 28 patients with Hirschsprung's disease and cranial IND (agIND) underwent resection. 46 of the children could be followed up 3.64 years after the end of the main therapeutic period and with a mean age of 6.7 years at the time of follow-up; 43.5% of the analysed children still showed severe constipation. 23.9% only were really cured; 15.2% had normal defecation still using conservative treatment and 17.4% had diarrhea. No significant difference was found between both groups, IND and agIND, and the results were independent of treatment modality. The results were much worse than in idiopathic constipation as reported in the literature and even worse in comparison to unselected Hirschsprung collectives. It has to be concluded that in IND with chronic constipation intensive long-term care is necessary and it is crucial that treatment algorithms should be outlined urgently together by pediatric gastroenterologists and pediatric surgeons. AgIND seems to need more extended resection following an exact histomorphological mapping by biopsies taken during enterostomy procedure.

Abnormalities of the enteric nervous system in heterozygous endothelin B receptor deficient (spotting lethal) rats resembling intestinal neuronal dysplasia

“…5 6 IND has been reported as an enteric neuropathology underlying severe chronic constipation and intestinal pseudo-obstruction in children and adults. [31][32][33] Controversies concerning the reliability of the diagnosis (for example, histopathological criteria, morphometric techniques) and clinical management (for example, conservative versus surgical therapy) have questioned IND as a clinical-histopathological entity. 34 35 However, morphometric analysis of the ENS from +/sl rats carried out in the present study clearly revealed alterations similar to those found in IND-for example, hyperganglionosis of the submucous plexus and hypertrophy of nerve fibre strands.…”

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

“…The results obtained with these different types of treatment are very discordant. Longterm followup studies are lacking and the available studies involve limited numbers of patients [32,57,75] . Thus, the available data nowadays still remains too scarce to establish a therapeutic guideline for INDB [32,57] .…”

“…Thus, the available data nowadays still remains too scarce to establish a therapeutic guideline for INDB [32,57] . On the other hand, there is a real disease, with its own clinical manifestations and can not be classified only as an histopathological entity [75] . The several types of clinical manifestations directly influence in the treatment.…”

“…Hyperplasia of the submucosal nerve plexuses is the morphological finding that defines INDB but that is characterized in different manners according to the adopted criterion [5] . Some authors emphasize the need for the presence of a minimum number of ganglion cells per ganglion or a minimum number of ganglia with these characteristics among the analyzed ganglia for a diagnosis of plexuses hyperplasia [16,22,75,90] (Figure 1). …”

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

“…3,[5][6][7][8] Modified criteria evolved gradually, primarily because of a series of papers coauthored by Meier-Ruge in the mid-1990s that clarified in detail how enzyme histochemistry should be performed and ganglion cells counted. [7][8][9][10][11][12] During this period, Meier-Ruge and colleagues reported that abnormal AChE-positive innervation is an unreliable diagnostic feature after age 9 months, emphasized that submucosal ganglion cell hyperplasia was the key diagnostic feature of IND B, and commented that:…”