先天性巨结肠合伴肠神经分布异常（附4例报告）

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，拟对施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

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

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