Korean J Pediatr Gastroenterol Nutr.
2003 Sep;6(2):202-207.
Intestinal Neuronal Dysplasia in Twins
- Affiliations
-
- 1Department of Pediatrics, College of Medicine, Inje University, Seoul, Korea. pedchung@sanggyepaik.ac.kr
- 2Department of Pediatrics, College of Medicine, Inje University, Seoul, Korea.
- 3Department of Diagnostic Pathology, College of Medicine, Inje University, Seoul, Korea.
Abstract
- Intestinal neuronal dysplasia (IND) is a disorder of abnormal intestinal innervation resulting in dysfunctional colonic motility. IND shares clinical features with Hirschsprung's disease but differentiated by histological findings such as hyperplasia of submucosal and myenteric plexuses, giant ganglia, ectopic ganglion cell and increased acetylcholinesterase activity in lamina propria. Although IND may exist as an isolated condition, more commonly, it occurs in association with Hirschsprung's disease. We report a case of twins affected with IND. Both children manifested with delayed passage of meconium and severe abdominal distention after birth. Barium enema in both patients showed microcolon. They underwent emergency ileostomy under the impression of total aganglionosis. But surgical biopsy specimens showed hyperganglionosis in submucosa with formation of giant ganglia. Both neonates suffers from several episodes of peudo-obstruction after the repair operation of colostomy.
