쌍생아에서 발생한 Intestinal Neuronal Dysplasia
Intestinal Neuronal Dysplasia in Twins
임인숙, 정주영, 최명재, 김상우, 김홍주, 김정연,
소속 상세정보
임인숙 ( Lim In-Suk )
인제대학교 의과대학 상계백병원 소아과
정주영 ( Chung Ju-Young )
인제대학교 의과대학 상계백병원 소아과
최명재 ( Chey Myoung-Jae )
인제대학교 의과대학 상계백병원 소아과
김상우 ( Kim Sang-Woo )
인제대학교 의과대학 상계백병원 소아과
김홍주 ( Kim Hong-Ju )
인제대학교 의과대학 상계백병원 외과학교실
김정연 ( Kim Jung-Yeon )
인제대학교 의과대학 상계백병원 병리학교실
KMID : 0816120030060020202
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 differenfiated by histological findings such as hyperplasia of sutnnucosal and myenteric plexuses, giant (Tanglia, 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 a-anglionosis. But surgical biopsy specimens showed hyperganglionosis in submucosa with formation of oiant gan-lia. Both neonates suffers from several episodes of peudo-obstruction after the repair operation of colostomy.
키워드
Intestinal neuronal dysplasia;Twins
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