Constipation and intestinal neuronal dysplasia type B: a clinical follow-up study.

Abstract

Intestinal neuronal dysplasia type B (IND B) is one of the gastrointestinal motility disorders with a defined malformation of the parasympathetic submucous and myenteric ganglia. The clinical presentation of IND B is variable, ranging from intestinal obstruction in the neonatal period to acute or chronic constipation in childhood. Between 1993 and 1996, 105 patients (49 females and 56 males) were treated for constipation, and in all of them an IND type B was confirmed histopathologically. Twenty-two neonates, 42 infants to 6 months of age (38% of them were premature, and 5% had additional malformations), and 41 patients to the age of 4 years were included in this study. All 105 patients had been treated conservatively. Treatment consisted of diet in all patients, cisapride in 70% of them, laxatives in 52%, and repeated anal dilatations in 12% of the patients. The mean duration of their treatment lasted from 3 months to 10 months (mean, 6 months). The clinical follow-up 5 to 9 years later in 89 of the 108 (85%) patients showed daily defecation in 80% of them and every second day in 14% of them. Only 5 (6%) patients experience recurrent constipation, which responds well to diet and laxatives. In young patients, constipation related to IND B can be treated successfully by conservative treatment regimens, including diet, laxatives, and prokinetic drugs.