25/1/26先天性巨结肠先天性巨结肠(jiécháng)(jiécháng)CongenitalmegacolonCongenitalmegacolon第一页，共四十四页。•由于直肠或者结肠(jiécháng)远端的肠管持续痉挛，粪便淤积在近端结肠(jiécháng)，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(xìbāo)症Hirschsprung’sDisease,HD赫什朋氏病Aganglionosis先天性无神经节细胞(xìbāo)症英文名称Englishname第三页，共四十四页。•一种常见的消化道畸形•Acommondigestivetractmalformation•有遗传(yíchuán)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(bìngbiàn)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(pēitāi)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。NormaldefecationphysiologyNormaldefecationphysiologyNormaldefecationphysiologyNormaldefecationphysiology直肠(zhícháng)壶腹潴便经大脑整合(zhěnɡhé)，决定排便与否便意直肠肛管抑制反射肛管感受(gǎnshòu)粪便性质骶髓低级中枢肠壁感受器第七页，共四十四页。•Smoothmuscleoflesionintestinesustainescontraction•AnorectalreflexloopisinterruptedStoolcannotbedischarged.ProximalnormalintestinehascompensatorydilatationandhypertrophyandformshugeexpansiveintestinalsegmentPathologyofcongenitalmegacolon第八页，共四十四页。病变(bìngbiàn)肠段呈痉挛样改变(Spasmsegment）近端肠管扩张肥厚(féihòu)，形成巨大结肠(Enlargedsegment)二者之间过度(guòdù)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...
