1、*先天性巨结肠先天性巨结肠Congenital megacolon定义定义 Defination 由于直肠或者结肠远端的肠管持续痉挛，粪便淤积在近端结肠，使该肠管肥厚、扩张 Rectal or colonic intestinal has persistent spasm and fecal deposition in the proximal colon results in hypertrophy and dilatation in proximal segment.Hirschsprungs Disease,HD 赫什朋氏病Aganglionosis 先天性无神经节细胞症英文名称 Eng
2、lish name概述概述 Summary 一种常见的消化道畸形 A common digestive tract malformation 有遗传倾向， 约 1.4-7.8% Genetic predisposition, about 1.4-7.8% 发病率为 1:2000-5000 Incidence rate of 1:2000-5000 男 :女 = 4:1 Male:female=4:1病 因 Etiology病变肠管肌间及粘膜下 神经节细胞缺如 ，是一种发育停顿，停顿越早，无神经节细胞段越长 .Absence of ganglion cells in muscle and sub
3、mucosa of lesion bowel. It is a development pause. The more early the pause occurs, the longer intestinal segment without ganglion cells is. 胚胎期第 5周消化道神经母细胞从头端向尾端移行 Neuroblastoma cells of digestive tract migrate from the beginning to the end In the fifth week of fetal.Normal defecation physiology 直肠
4、壶腹潴便经大脑整合，决定排便与否便意直肠肛管抑制反射肛管感受粪便性质骶髓低级中枢 肠壁感受器Absence of ganglion cells Smooth muscle of lesion intestine sustaines contraction Anorectal reflex loop is interruptedStool can not be discharged. Proximal normal intestine has compensatory dilatation and hypertrophy and forms huge expansive intestinal s
5、egment Pathology of congenital megacolon 病变肠段呈痉挛样改变 (Spasm segment）近端肠管扩张肥厚，形成巨大结肠(Enlarged segment)二者之间过度肠段呈漏斗状称移行段(Transformed segment)正常结肠Normal colon Pathological anatomy of congenital megacolon按病变长度 According to the length of lesion segment 常见型 Ordinary type (common type) megacolon (85%): Lesio
6、n is limited in rectum and sigmoid colon. 短段型 Short segment type megacolon(10%): Lesion is limited in the distal 3-4 cm of rectum. 长段型 Long segment type megacolon(10%): Lesion reaches splenic flexure, and even the entire colon. 全结肠型 Total colonic type(1%):Lesion reathes entire colon or evev terminal ileum.分型Pathologic types of congenital megacolon
