Neuronal intestinal dysplasia: an entity of chronic intestinal pseudo-obstruction

Abnormalities of bowel innervation and ganglion distribution are represented by a wide spectrum of conditions, including an entity known as neuronal intestinal dysplasia [NID]. NID is characterized by localized or disseminated hypertrophic and immature ganglion cells, hyperplasia of the myenteric and submucosal plexi with giant ganglia [NID type B], hypoplasia or aplasia of sympathetic innervation of the myenteric plexus [NID type A] and sometimes by the presence of ectopic neural formations in the submucosa and muscular stratum. The frequency of NID coexisting with Hirschsprung's disease has been reported to vary from 20- 66%. Special diagnostic clinical signs do not exist, while a precise correlation between histology and clinical manifestations is lacking. The precise options for therapy have not been clearly established. If aganglionosis is excluded, treatment is initially conservative, since clinical and histological improvement has been reported in cases with mild dysganglionosis, in the presence of immature ganglion cells in the myenteric plexus, in atopies of ganglions of the submucosal plexus, as well as in cases of limited areas NID type B. When symptoms can not be controled with medical treatment, a temporary loop colostomy could be undertaken. In cases with severe obstructive symptoms subtotal colectomy and ileorectal anastomosis is suggested. Key words: Constipation, pseudobstruction, neuronal intestinal dysplasia