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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。
Objective: To retrospectively observe the abnormal distribution of enteric nerve in Hirschsprung’s disease. Methods: From January 1991 to December 1997, 111 cases of Hirschsprung’s disease were treated by radical resection of the intestine. The distal pathology was confirmed as having no ganglion cell disease. The histopathology was stained retrospectively. And silver staining and specific neuron enolase (NSE) staining were used. Results: In 111 cases of Hirschsprung ’s disease, 4 cases were associated with abnormal distribution of intestinal nerves, 3 cases were associated with abnormal development of intestinal neurons (IND) and 1 case was ganglion cell degeneration. Example 3 with IND, postoperative constipation, and then for biopsy with the original IND pathological findings similar. Conclusion: This group observed Hirschsprung’s disease associated with congenital distribution of abnormal nerve accounted for 3.6%, the treatment should be removed without ganglion cell intestinal segment, the concomitant intestinal nerve distribution of abnormal bowel resection as possible, according to this group of 4 patients Whether the length of ganglion cell-free lesions can be temporarily associated with the development of anomalous intestinal neurons may not be related to the type of binding.