Objective: 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.
Methods: 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).
Results: 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.
Conclusions: In young patients, constipation related to IND B can be treated successfully by conservative treatment regimens, including diet, laxatives, and prokinetic drugs.