The clinical pathological analysis of intestinal neuronal dysplasia type B

Abstract

Objective To investigate the clinicopathological features and diagnosis of intestinal neuronal dysplasia type B. Methods Between January 2004 and August 2014, 9 patients(5 males and 4 females) were treated for constipation and abdominal distention, and in all of them an intestinal neuronal dysplasia type B was confirmed histopathologically.The age of 9 patients ranged from 3 months to 1 year old(mean 7.8 months). The specimen of 9 patients was routinely taken by paraffin-embedded full-circumference sections of lesional bowel, hematoxylin and eosin and immunohistochemical staining were carried out on the specimen.The pathological morphology and quantitative of intestinal neurons and ganglia were retrospectively analyzed. Results Total of the 9 patients, the number and density of myenteric ganglia increased significantly increased in the lesional bowel, the pathological findings included giant nerve plexus, isolated and ectopic ganglia.In the proximal bowel, the number and density of myenteric ganglia were observed abnormal no giant nerve plexus, isolated and ectopic ganglia was observed.Combinated these pathological findings and symptoms, intestinal neuronal dysplasia type B was diagnosed. Conclusion The diagnosis of intestinal neuronal dysplasia type B relies on typical presentations of allied disorders of Hirschsprung's disease, giant ganglia, isolated and ectopic ganglia, and increasing the density of giant submucosal ganglia of pathologic morphology, meanwhile, excluding others primary etiologies. Key words: Intestinal neuronal dysplasia; Allied disorders of hirschsprung disease; Pathology; Diagnosis