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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
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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
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
关键词 先天性巨结肠 巨结肠 肠神经分布异常 hischsprung's disease intestinal neuronal dysplasia hypoganglionosis
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参考文献5

  • 1Scharli A.Standardization of terminology of intestinal innervation disorders.Pediatr Surg Int,1995.10:440. 被引量:1
  • 2Pena A, Intestinal neuronal dysplasia,myth or reality?literature review, Pediatr Surg Int, 1995,10 :441. 被引量:1
  • 3Ure BM, Holschneider AM, Meier-Ruge W. Neuronal intestinal malformations. A retro and prospective study on 203 patients. Eur J Pediatr Surg, 1994, 4:279. 被引量:1
  • 4Holschneider AM,et al. Hirchsprung's disease and allied disorders A Review: Eur J Pediatr Surg, 1994, 4: 260. 被引量:1
  • 5余亚雄,格忠德.先天性巨结肠类缘病[J].中华小儿外科杂志,1991,12(6):331-333. 被引量:11

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