Clinical long-term follow-up results in intestinal neuronal dysplasia (IND)

Commonly available information on intestinal neuronal dysplasia (IND) is sp arse. 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 morphol ogical 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 p arameter 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 wit h 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 mai n 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 conservati ve treatment and 17.4% had diarrhea. No significant difference was found be tween both groups, IND and agIND, and the results were independent of treat ment modality. The results were much worse than in idiopathic constipation as reported in the literature and even worse in comparison to unselected Hi rschsprung collectives. It has to be concluded that in IND with chronic con stipation intensive long-term care is necessary and it is crurical that tre atment algorithms should be outlined urgently together by pediatric gastroe nterologists and pediatric surgeons. AgIND seems to need more extended rese ction following an exact histomorphological mapping by biopsies taken durin g enterostomy procedure.