多发性节段型肠无神经节细胞症的临床分析  

Clinicai study of multiple zonal aganglionosis in long segment Hirschsprung′s disease

作　　者：杨合英[1] 刘秋亮[1] 王家祥[1] 许华峰[1] 

机构地区：[1]郑州大学第一附属医院小儿外科河南省临床医学重点学科开放实验室,450052

出　　处：《中华医学杂志》2005年第39期2772-2774,共3页National Medical Journal of China

摘　　要：目的探讨多发性节段型肠无神经节细胞症的病因、诊断和治疗。方法回顾性分析1987至2005年间我院收治的3例患儿,年龄5d、29d、18个月,均为男性。病理检查HE染色。行全结肠切除回肠脱出巨结肠根治术。结果3例患儿均于术前诊断先天性巨结肠,术中探查回肠末段和升结肠起始段狭窄,病理切片证实狭窄段肠壁内无神经节细胞。结论长段型先天性巨结肠患儿应探查全结肠及回肠,以免漏诊;先天性巨结肠的发病原因不仅是胚胎早期由神经嵴的神经母细胞向肠管移行过程中胚胎发育停顿引起,也可能是神经节细胞发育成熟过程中不同节段的神经节细胞发育障碍引起。Objective To discuss pathogeny and diagnosis and management of multiple zonal aganglionosis in Hirschsprung＇s disease. Methods Records of 3 children, aging 5 days, 29 days and 18 months, 3 boys, with multiple zonal aganglionosis in long segment Hirschsprung＇s disease between 1987 - 2005 were reviewed retrospectively. Total colectomy and Soave＇s operations were performed. Results 3 children were diagnosed HD before surgery , but the convulsive stenosis in distal ileum and proximal ascending colon were detected during surgery. The aganglionic cells in the stenosis gut were confirmed by pathologic diagnosis. Conclusions The total colon and ileum should be detected carefully during surgery in chirldren with long segment Hirschsprung＇s disease. It is believed not sound that the neuroblastic cells stop moving from neuroectoderm to gut in early gestation in HD, but it is also believed that some other causes in the course of gestation might interfere the normal growth of the ganglionic cells.

关 键 词：先天性巨结肠 多发性节段型无神经节细胞症 

分 类 号：R726.5[医药卫生—儿科]