Intestinal Neuronal Dysplasia in Twins

쌍생아에서 발생한 Intestinal Neuronal Dysplasia

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.

장신경 이형성증은 만성 가성 폐색 증후군의 드문 원인질환으로 임상 증상이 선천성 거대결장증과 유사하여 진단이 늦어지는 경우가 많기 때문에 임상적인 의심이 필요하다. 저자들은 쌍생아에서 신생아기에 태변이 배출되지 않고 복부 팽만을 보여 선천성 거대결장증이 의심되었으나 임상경과와 조직검사로 진단된 장신경 이형성증을 보고하는 바이다.