先天性巨结肠与胆道课件

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1、2021/9/18先天性巨结肠Congenitalmegacolon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 intestinalhaspersistent spasmandfecal depositioninthe proximalcolonresultsinhypertrophyand dilatationinproximalsegment.Hirschsprung’sDisease,HD赫什朋氏病Aganglionosis先天性无神经节细胞症英文名称Englishname概述Summary一

2、种常见的消化道畸形Acommondigestive tractmalformation有遗传倾向，约1.4-7.8%Geneticpredisposition, about1.4-7.8%发病率为1:2000-5000Incidencerateof1:2000-5000男:女=4:1Male: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.Absenceof ganglioncellsinmuscleandsubmucosaoflesionbowel.Itisadevelopment pause. Themoree

3、arlythepauseoccurs,thelongerintestinalsegmentwithoutganglioncellsis.胚胎期第5周消化道神经母细胞从头端向尾端移行Neuroblastomacellsofdigestivetractmigrate fromthebeginningtotheendInthefifthweekoffetal.Normal defecationphysiology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Absenceof ganglioncellsSmoothmuscle oflesioni

4、ntestinesustainescontractionAnorectalreflex loop isinterruptedStool cannotbedischarged. Proximal normal intestinehas compensatorydilatation and hypertrophyandformshugeexpansiveintestinalsegmentPathology ofcongenitalmegacolon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(

5、Transformedsegment)正常结肠NormalcolonPathological anatomyofcongenitalmegacolon按病变长度Accordingtothelengthoflesionsegment常见型Ordinarytype (common type)megacolon(85%):Lesionis limitedin rectumandsigmoidcolon.短段型Short segmenttypemegacolon(10%):Lesionis limitedinthe distal3-4 cm ofrectum.长段型Long segmenttyp

6、emegacolon(10%):Lesionreachessplenicflexure, andeven theentirecolon.全结肠型Totalcolonic type(1%):Lesionreathesentire colonorevevterminalileum.分型Pathologictypesofcongenitalmegacolon临床表现Clinicalmanifestations新生儿期Theneonatalperiod: acutelow incompleteintestinalobstructionDelayoffetal dischargeAbdominal

7、distention andvomitingConstipationWasting andmalnutritionClinicalmanifestations婴幼儿期：慢性低位肠梗阻Infantand childhood:subacuteorchronic low incompleteintestinalobstruction反复便秘Recurrent constipation进行性腹胀Progressiveabdominaldis