医学专题一先天性巨结肠与胆道剖析.ppt

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1、2023/2/3先天性巨结肠(jichng)(jichng)Congenitalmegacolon第一页，共四十四页。定义定义(dngy)(dngy)(dngy)(dngy)Defination由于直肠或者结肠(jichng)远端的肠管持续痉挛，粪便淤积在近端结肠(jichng)，使该肠管肥厚、扩张Rectalorcolonicintestinalhaspersistentspasmandfecaldepositionintheproximalcolonresultsinhypertrophyanddilatationinproximalsegment.第二页，共四十四页。Hirschspru

2、ngsHirschsprungs Disease,HDDisease,HD 赫什朋氏病赫什朋氏病AganglionosisAganglionosis先天性无神经节细胞先天性无神经节细胞(xbo)(xbo)症症英文名称 English name第三页，共四十四页。概述概述(i sh)(i sh)(i sh)(i sh)Summary 一种常见的消化道畸形Acommondigestivetractmalformation有遗传(ychun)倾向，约1.4-7.8%Geneticpredisposition,about1.4-7.8%发病率为1:2000-5000Incidencerateof1:2

3、000-5000男:女=4:1Male:female=4:1第四页，共四十四页。病 因Etiology病变(bngbin)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.Absenceofganglioncellsinmuscleandsubmucosa of lesion bowel.It isadevelopmentpause.Themoreearlythepauseoccurs,thelongerintestinal segment without ganglion cells is.第五页，共四十四页。胚胎(piti)期第5周消化道神经母细胞从头端向尾端移

4、行NeuroblastomacellsofdigestivetractmigratefromthebeginningtotheendInthefifthweekoffetal.第六页，共四十四页。NormaldefecationphysiologyNormaldefecationphysiology 直肠(zhchng)壶腹潴便经大脑整合(zhn h)，决定排便与否便意直肠肛管抑制反射肛管感受(gnshu)粪便性质骶髓低级中枢肠壁感受器第七页，共四十四页。AbsenceofganglioncellsAbsenceofganglioncells Smoothmuscleoflesionintes

5、tinesustainescontraction AnorectalreflexloopisinterruptedStoolcannotbedischarged.Proximalnormalintestinehascompensatorydilatationandhypertrophyandformshugeexpansiveintestinalsegment Pathologyofcongenitalmegacolon 第八页，共四十四页。病变(bngbin)肠段呈痉挛样改变(Spasm segment）近端肠管扩张肥厚(fi hu)，形成巨大结肠(Enlarged segment)二者之间

6、过度(gud)肠段呈漏斗状称移行段(Transformed segment)正常结肠Normalcolon Pathologicalanatomyofcongenitalmegacolon第九页，共四十四页。按病变长度(chngd)Accordingtothelengthoflesionsegment常见型Ordinarytype(commontype)megacolon(85%):Lesionislimitedinrectumandsigmoidcolon.短段型Shortsegmenttypemegacolon(10%):Lesionislimitedinthedistal3-4cmofr

7、ectum.长段型Longsegmenttypemegacolon(10%):Lesionreachessplenicflexure,andeventheentirecolon.全结肠型Totalcolonictype(1%):Lesionreathesentirecolonorevevterminalileum.分型Pathologictypesofcongenitalmegacolon第十页，共四十四页。临床表现Clinicalmanifestations 新生儿期新生儿期Theneonatalperiod:acutelowincompleteintestinalobstructionDe

8、layoffetaldischargeAbdominaldistentionandvomitingConstipationWastingandmalnutrition第十一页，共四十四页。Clinicalmanifestations婴幼儿期：慢性(mnxng)低位肠梗阻Infantandchildhood:subacuteorchroniclowincompleteintestinalobstruction 1.反复便秘Recurrentconstipation2.进行性腹胀Progressiveabdominaldistension3.发育迟缓，营养不良Growthretardation,m

9、alnutrition第十二页，共四十四页。并发症并发症 Complications多在2个月内发生Occurringwithin2months肠梗阻Intestinalobstruction小肠(xiochng)结肠炎Enterocolitis肠穿孔，腹膜炎Bowelperforationandperitonitis继发败血症，肺炎Secondarysepsisandpneumonia 第十三页，共四十四页。1、肛门指诊Rectaltouch2、钡灌肠Bariumenema3、直肠(zhchng)肛管测压Anorectalmanometry4、直肠活检Biopsy5、肌电图Electromy

10、ogram 辅助辅助(fzh)检查检查 Accessoryexamination 第十四页，共四十四页。第十五页，共四十四页。第十六页，共四十四页。钡灌肠钡灌肠(gun(gun(gun(gunchng)chng)chng)chng)X X表现表现 Barium enema X-ray葛X，2y，长段型巨结肠(jichng)，soave第十七页，共四十四页。第十八页，共四十四页。Anorectalmanometry:reflectionofnormalanorectalreflexdisappearsAnorectalmanometry:reflectionofnormalanorectalre

11、flexdisappears 正常直肠正常直肠(zhchng)肛管反射肛管反射第十九页，共四十四页。第二十页，共四十四页。肠壁粘膜肠壁粘膜(zhn m)腺体腺体Intestinalmucosal glands酶阳性酶阳性(yngxng)神经神经Enzymepositive nerve 正常人正常人Normal child巨结肠巨结肠(jichng)患儿患儿Megacolon child第二十一页，共四十四页。诊 断 Diagnosis1、病史 Medical history2、钡灌肠 Barium enema 3、直肠肛管测压力Anorectalmanometry3、直肠黏膜组织(zzh)活检

12、 Rectal mucosalbiopsy 第二十二页，共四十四页。1、胎粪性便秘 Meconiumconstipation2、新生儿肠闭锁Neonatalintestinalatresia3、特发性巨结肠Idiopathicmegacolon4、巨结肠类缘病Neuronalintestinaldysplasia5、肛门(gngmn)内括约肌失弛症 Internal analsphincterachalasia6、继发性巨结肠Secondarymegacolon7、内分泌性Endocrinedisease8、乙状结肠过长症Redundantsigmoidcolon 鉴别鉴别(jinbi)(ji

13、nbi)诊断诊断 Differential diagnosis第二十三页，共四十四页。先天性巨结肠(jichng)Congenitalmegacolon 继发性巨结肠(jichng)Secondary megacolon 第二十四页，共四十四页。治疗Treatment治疗原则(yunz):手术治疗,切除病变肠段以及扩张肥厚的肠管Treatmentprinciple:Operationtreatment.Resectionofthelesionbowelanddilatationbowel.第二十五页，共四十四页。术前准备（保守治疗）Preoperativepreparation(conserv

14、ativetreatment)1、洗肠Intestinallavage2、括肛、通便(tnbin)Enlargeanusandinducingdefeation3、缓泻药ApplicationofLaxativedrug第二十六页，共四十四页。手术手术(shush)(shush)(shush)(shush)方式方式 Operationmethods SewnonsoperationSoaves operationDuhamels operationRehbeins operationMartins operation第二十七页，共四十四页。Swenson Swenson 改良术：改良术：结肠结

15、肠(jichng)(jichng)经直肠内拖出经直肠内拖出（Pull-throughPull-through）切除术）切除术第二十八页，共四十四页。Soave procedure手术手术(shush)(shush)的基本步骤的基本步骤第二十九页，共四十四页。Duhameloperation第三十页，共四十四页。术后并发症Postoperativecomplications 吻合口感染、泄漏Anastomoticinfectionandleakage尿潴留Retentionofurine小肠结肠炎Enterocolitis吻合口狭窄，便秘复发(f f)Anastomotic stenosis a

16、ndrecurrentconstipation肛门内括约肌损伤Internalanalsphincterinjury远期生活质量下降Declinedqualityoflifeinlong-term第三十一页，共四十四页。要点(yodin)(yodin)Keypoints1、概念Conceptofmegacolon2、分型Pathologicaltypeofmegacolon3、临床特征(tzhng)Clinicalcharacteristicsanddiagnosisofmegacolon4、治疗原则Thesurgicalprinciplesofmegacolon 第三十二页，共四十四页。肛门

17、直肠肛门直肠(zhchng)(zhchng)(zhchng)(zhchng)畸形的分类畸形的分类Classificationofanorectalmalformation 第三十三页，共四十四页。没有没有X X影像学资料影像学资料(zlio)(zlio)(zlio)(zlio)前不要轻易手术前不要轻易手术WeshouldnotdooperationeasilywithoutX-ray 第三十四页，共四十四页。MRMR第三十五页，共四十四页。针形电刀Needletypeelectricknife电刺激(cj)仪Electricalstimulationinstrument第三十六页，共四十四页。

18、先天性胆道闭锁先天性胆道闭锁(b su)(b su)Congenitalbiliaryatresia 第三十七页，共四十四页。Diagnosisanddifferentialdiagnosis MRCP,laparoscopicexplorationandintraoperativecholangiography Cholestasissyndrome第三十八页，共四十四页。Operationtreatmentofcongenitalbiliaryatresia 纤维(xinwi)三角板的切除 Excisionoffibroustriangleplate 肝门部与肠吻合 Anastomosis

19、ofjejunumandHilar第三十九页，共四十四页。胆道闭锁胆道闭锁(b su)(b su)术后用药术后用药 Medication afterbiliaryatresiaoperation激素激素(j s)应用应用Hormonedrugs抗生素应用抗生素应用Antibiotics利胆药应用利胆药应用Cholagogue第四十页，共四十四页。先天性胆总管囊肿先天性胆总管囊肿(nngzhng)(nngzhng)Congenitalcholedochalcyst 第四十一页，共四十四页。MRCP第四十二页，共四十四页。治疗治疗:囊肿囊肿囊肿囊肿(nngzhng)(nngzhng)切除，切除，切

20、除，切除，Roux-en-YRoux-en-Y吻合，抗反流瓣吻合，抗反流瓣吻合，抗反流瓣吻合，抗反流瓣Treatment:cystexcision,Roux-en-YTreatment:cystexcision,Roux-en-Yanastomosis,anti,antirefluxvalverefluxvalve 第四十三页，共四十四页。内容(nirng)总结2022-02-27。2022-02-27。Male:female=4:1。Anorectalreflexloopisinterrupted。Swenson改良(giling)术：结肠经直肠内拖出（Pull-through）切除术。Needletypeelectricknife。MRCP第四十四页，共四十四页。