01/26/2025先天性巨结肠Congenitalmegacolon第一页，共四十四页。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•Rectalorcolonicintestinalhaspersistentspasmandfecaldepositionintheproximalcolonresultsinhypertrophyanddilatationinproximalsegment.第二页，共四十四页。Hirschsprung’sDisease,HD赫什朋氏病Aganglionosis先天性无神经节细胞症Hirschsprung’sDisease,HD赫什朋氏病Aganglionosis先天性无神经节细胞症英文名称Englishname第三页，共四十四页。概述Summary•一种常见的消化道畸形•Acommondigestivetractmalformation•有遗传倾向，约1.4-7.8%•Geneticpredisposition,about1.4-7.8%•发病率为1:2000-5000•Incidencerateof1:2000-5000•男:女=4:1•Male:female=4:1第四页，共四十四页。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.•Absenceofganglioncellsinmuscleandsubmucosaoflesionbowel.Itisadevelopmentpause.Themoreearlythepauseoccurs,thelongerintestinalsegmentwithoutganglioncellsis.第五页，共四十四页。•胚胎期第5周消化道神经母细胞从头端向尾端移行•NeuroblastomacellsofdigestivetractmigratefromthebeginningtotheendInthefifthweekoffetal.第六页，共四十四页。NormaldefecationphysiologyNormaldefecationphysiology直肠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第七页，共四十四页。Absenceofganglioncells•Smoothmuscleoflesionintestinesustainescontraction•AnorectalreflexloopisinterruptedStoolcannotbedischarged.ProximalnormalintestinehascompensatorydilatationandhypertrophyandformshugeexpansiveintestinalsegmentPathologyofcongenitalmegacolon第八页，共四十四页。病变肠段呈痉挛样改变(Spasmsegment〕近端肠管扩张肥厚，形成巨大结肠(Enlargedsegment)二者之间过度肠段呈漏斗状称移行段(Transformedsegment)正常结肠NormalcolonPathologicalanatomyofcongenitalmegacolon第九页，共四十四页。按病变长度Accordingtothelengthoflesionsegment•常见型Ordinarytype(commontype)megacolon(85%):Lesionislimitedinrectumandsigmoidcolon.•短段型Shortsegmenttypemegacolon(10%):Lesionislimitedinthedistal3-4cmofrectu...
