All issues > Volume 39(8); 1996

Case Report

J Korean Pediatr Soc. 1996;39(8):1151-1157. Published online August 15, 1996.

A Case of Intestinal Neuronal Dysplasia

Se Young SY Kim², Yon Ho YH Choe², Jeong Kee JK Seo², In One IO Kim¹, Chong Jai CJ Kim¹

¹Department of Radiology, College of Medicine, Seoul National University, Seoul, Korea
²Department of pediatrics, College of Medicine, Seoul National University, Seoul, Korea

Abstract

Intestinal neuronal dysplasia(IND) is a disease characterized clinically by symptoms of intestinal obstruction and pathologically by hyperplasia of the submucosal and myenteric plexuses with formation of giant ganglia. Chronic intestinal pseudo-obstruction is a clinical diagnosis, composed of myopathic form and neuropathic form, and normal intestinal histology. Intestinal neuronal dysplasia is a neuropathic form of chronic intestinal pseudo-obstruction. The clinical presentation and the course of IND is very variable. We experienced a case of intestinal neuronal dysplasia in a 5 year-old boy who had suffered from recurrent abdominal pain and vomiting for 3 years. Small bowel series showed multiple intestinal dilatations and delayed excretion of contrast media. He underwent exploratory laparotomy. However no mechanical causes for markedly dilated intestine were found and he received loop ileostomy. However, he suffered from recurrent vomiting and abdominal pain. So, he received repair-operaton. The pathology of surgical specimen showed hyperplasia of the submucosal and myenteric plexuses with giant ganglia. We report this case with a brief review of the related literatures.

Keywords :Intestinal neuronal dysplasia, Chronic idiopathic intestinal pseudo-obstruction syndrome