Lernau O Z, Udassin R, Vinograd I, Stamler B, Nissan S
Am J Gastroenterol. 1982 Jun;77(6):374-6.
A case of a 35-yr-old woman with the characteristics of pseudo-Hirschsprung's disease is presented. The diagnosis was based on the following criteria: a history of chronic constipation since early infancy, a largely distended abdomen and an adynamic distal colon. The patient had a normal anosphincteric relaxation reflex and normal rectal myenteric ganglion cells. Resection of the distended large bowel failed to relieve the symptoms and was followed by distention of the entire small bowel. Complete cure was achieved after a Duhamel procedure. This patient is an adult, unlike the previously reported cases. Of special interest is the occurrence of true Hirschsprung's disease in one of her children.
本文报告了一例具有假性先天性巨结肠病特征的35岁女性病例。诊断基于以下标准：自幼即有慢性便秘史、腹部明显膨隆以及远端结肠动力不足。患者肛门括约肌松弛反射正常，直肠肌间神经节细胞正常。切除扩张的大肠未能缓解症状，随后整个小肠出现扩张。采用杜哈梅尔手术（Duhamel procedure）后实现了完全治愈。与先前报道的病例不同，该患者为成年人。特别值得关注的是，她的一个孩子患有真性先天性巨结肠病。
