Granero_2007_Cir.Pediatr_20_166

Isolated Intestinal neuronal dysplasia is a disease characterized by symptoms of diarrhea or intestinal obstruction along with histopathologic diagnosis based on hyperplasia of submucous plexus with giant ganglia containing more than seven nerve cells, increased acetylcholinesterase activity in the lamina propria and around the submucosal blood vessels and heterotopic ganglia in the lamina propria. The aims of this study have been to determine the incidence of the congenital malformations associated with the isolated intestinal dysplasia type B (not associated to another anomaly of the enteric nervous system) and to correlate them with the severity of the symptoms and their possible familial involvement. We have retrospectively reviewed all the patients diagnosed with IND type B nonassociated to Hirschsprung's disease in our hospital from 1981 to 2002. Our serie consists of 44 cases, 1 for every 7500 newborn. 35% of the patients presented associated congenital anomalies, with digestive malformations being the most commonly found (20% of the total). 40% of the patients studied had previous history of constipation in the family. Onset of symptoms was detected in 75% of children during newborn period.