Intestinal neuronal dysplasia involving whole alimentary tract in children: a report of 8 cases

Abstract

Objective To explore an efficient management for children with Intestinal neuronal dysplasia(IND) involving whole alimentary tract. Methods All cases diagnosed of intestinal neuronal dysplasia involving whole alimentary tract with definite pathologic results between January 2007 and December 2012 were retrospectively analyzed. The ages of initial onset of symptom such as vomiting, abdominal bloating and constipation were between 11 days and 12 months. And the imaging features, pathological findings, surgical approaches and follow-up outcomes were analyzed. Results There was no specific findings on plain abdominal radiograph or barium enema examination. Rectosphincteric reflex on balloon dilatation could be observed in 4 cases except for a delayed return to base line. Enterostomy, Rehbein operation and Bishop enterostomy were performed. And conservative treatments included enemas, laxatives and traditional acupuncture stimulations. During a follow-up period of 3-45 months, symptomatic relief was offered as needed. Conclusions Despite a low incidence, IND involving whole alimentary tract has intractable symptoms with a high mortality rate. As surgery is not curative, a combination of conservative measures and operation are essential for its optimal management. At the same time, individualized diet control and prevention from enteritis should be implemented. Key words: Intestinal neuronal dysplasia; Enterostomy; Retrospective studies