先天性巨结肠合伴肠神经分布异常 (附4例报告)  

HIRSCHSPRUNG'S DISEASE COMPLICATED WITH INTESTINAL NEURONAL MALFORMATIONS: A PROSPECTIVE STUDY IN 4 CASES

作　　者：施诚仁[1] 陈其民[1] 吴燕[1] 王玲华[1] 张忠德[2] 丁毅 

机构地区：[1]上海第二医科大学附属新华医院小儿外科,上海200092 [2]上海第二医科大学附属新华医院病理科,上海200092

出　　处：《大肠肛门病外科杂志》1998年第4期1-3,共3页Journal of Coloproctological Surgery

摘　　要：目的:对先天性巨结肠合伴肠神经分布异常的现象行回顾性组织学观察。方法:1991年1月至1997年12月111例先天性巨结肠根治术切除肠段标本,其远端病理证实为无神经节细胞症,拟对拖出段即切除肠管近端亦作常规H.E染色病理学回顾性观察;且配合嗜银染色法及特异性神经元烯醇酶(NSE)染色法。结果:111例先天性巨结肠中有4例合伴肠神经分布异常,其中3例合伴肠神经元性发育异常(IND),1例为神经节细胞减少症。伴发IND的例3,术后有便秘,且再作活检为同原IND病理表现相似。结论:本组观察先天性巨结肠合伴肠神经分布异常占3.6%,治疗应切除无神经节细胞肠段外,对合伴的肠神经分布异常肠段尽可能作切除,按本组4例结合型暂不能作出无神经节细胞病变长度与合伴肠神经元性发育异常之间有否关系。A prospective histologic study of 4 patients with Hirschsprung's disease (H. D) complicated with instestinal neuronal malformations is presented. Methods: From Jan-nuary 1991 to December 1997, specimens of aganglionic colon and its proximal segment were observed by H. E stain, silver stain and specific histochemistry, which is specific neuron enolase (NSE). Results: There were 4 cases of H. D with intestinal neuronal malformations in 111 consecutive patients with H. D, 3 of whom were complicated with instestinal neuronal dysplasia (IND) and another with hypogangionosis. They underwent follow-up for 3-10 months. 3 cases had constipation after duhamel procedure, but not anal stricture. Conclusions: In this study, there were 3. 6% cases complicated with intestinal neuronal malformation in 111 patients of H. D. All patients had aganglionetomy and complicated malformation segment. We cannot explain the causal relationship between the length of aganglionic segment and complicated instestinal neuronal malfomation

关 键 词：先天性巨结肠 巨结肠 肠神经分布异常 

分 类 号：R574.62[医药卫生—消化系统]