齐鲁医学先天性巨结肠与胆道剖析.pptx
2021/7/27 星期二先天性巨结肠Congenitalmegacolon1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Rectalorcolonicintestinalhaspersistentspasmandfecaldepositionintheproximalcolonresultsinhypertrophyanddilatationinproximalsegment.22021/7/27 星期二HirschsprungsDisease,HDHirschsprungsDisease,HD 赫什朋氏病赫什朋氏病AganglionosisAganglionosis先天性无神经节细胞症先天性无神经节细胞症英文名称 English name32021/7/27 星期二概述概述 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1:2000-5000Incidencerateof1:2000-5000男:女=4:1Male:female=4:142021/7/27 星期二病 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.Absenceofganglioncellsinmuscleandsubmucosa of lesion bowel.It isadevelopmentpause.Themoreearlythepauseoccurs,thelongerintestinal segment without ganglion cells is.52021/7/27 星期二胚胎期第5周消化道神经母细胞从头端向尾端移行NeuroblastomacellsofdigestivetractmigratefromthebeginningtotheendInthefifthweekoffetal.62021/7/27 星期二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72021/7/27 星期二AbsenceofganglioncellsAbsenceofganglioncells Smoothmuscleoflesionintestinesustainescontraction AnorectalreflexloopisinterruptedStoolcannotbedischarged.Proximalnormalintestinehascompensatorydilatationandhypertrophyandformshugeexpansiveintestinalsegment Pathologyofcongenitalmegacolon 82021/7/27 星期二 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Normalcolon Pathologicalanatomyofcongenitalmegacolon92021/7/27 星期二 按病变长度Accordingtothelengthoflesionsegment常见型Ordinary type(commontype)megacolon(85%):Lesionislimitedinrectumandsigmoidcolon.短 段 型 Shortsegment type megacolon(10%):Lesionislimitedinthedistal3-4cmofrectum.长 段 型 Longsegment type megacolon(10%):Lesionreachessplenicflexure,andeventheentirecolon.全 结 肠 型 Totalcolonictype(1%):Lesionreathesentirecolonorevevterminalileum.分型Pathologictypesofcongenitalmegacolon102021/7/27 星期二临床表现Clinicalmanifestations 新生儿期新生儿期Theneonatalperiod:acutelowincompleteintestinalobstructionDelayoffetaldischargeAbdominaldistentionandvomitingConstipationWastingandmalnutrition112021/7/27 星期二Clinicalmanifestations婴幼儿期：慢性低位肠梗阻Infantandchildhood:subacuteorchroniclowincompleteintestinalobstruction 1.反复便秘Recurrentconstipation2.进行性腹胀Progressiveabdominaldistension3.发育迟缓，营养不良Growthretardation,malnutrition122021/7/27 星期二并发症并发症 Complications多在2个月内发生Occurringwithin2months肠梗阻Intestinalobstruction小肠结肠炎Enterocolitis肠穿孔，腹膜炎Bowelperforationandperitonitis继发败血症，肺炎Secondarysepsisandpneumonia 132021/7/27 星期二1、肛门指诊Rectaltouch2、钡灌肠Bariumenema3、直肠肛管测压Anorectalmanometry4、直肠活检Biopsy5、肌电图Electromyogram 辅助检查辅助检查 Accessoryexamination 142021/7/27 星期二152021/7/27 星期二162021/7/27 星期二钡灌肠钡灌肠X X表现表现 Barium enema X-ray葛X，2y，长段型巨结肠，soave172021/7/27 星期二182021/7/27 星期二Anorectalmanometry:reflectionofnormalanorectalreflexdisappearsAnorectalmanometry:reflectionofnormalanorectalreflexdisappears 正常直肠肛管反射正常直肠肛管反射192021/7/27 星期二202021/7/27 星期二肠壁粘膜腺体肠壁粘膜腺体Intestinalmucosal glands酶阳性神经酶阳性神经Enzymepositive nerve 正常人正常人Normal child巨结肠患儿巨结肠患儿Megacolon child212021/7/27 星期二诊 断 Diagnosis1、病史 Medical history2、钡灌肠 Barium enema 3、直肠肛管测压力Anorectalmanometry3、直肠黏膜组织活检 Rectal mucosalbiopsy 222021/7/27 星期二1、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、肛门内括约肌失弛症Internalanalsphincterachalasia6、继发性巨结肠Secondarymegacolon7、内分泌性Endocrinedisease8、乙状结肠过长症Redundantsigmoidcolon 鉴别诊断鉴别诊断 Differential diagnosis232021/7/27 星期二先天性巨结肠Congenitalmegacolon 继发性巨结肠Secondary megacolon 242021/7/27 星期二治疗Treatment治疗原则:手术治疗,切除病变肠段以及扩张肥厚的肠管Treatmentprinciple:Operationtreatment.Resectionofthelesionbowelanddilatationbowel.252021/7/27 星期二术前准备（保守治疗）Preoperativepreparation(conservativetreatment)1、洗肠Intestinallavage2、括肛、通便Enlargeanusandinducingdefeation3、缓泻药ApplicationofLaxativedrug262021/7/27 星期二手术方式手术方式 Operationmethods SewnonsoperationSoaves operationDuhamels operationRehbeins operationMartins operation272021/7/27 星期二Swenson Swenson 改良术：改良术：结肠经直肠内拖出结肠经直肠内拖出（Pull-throughPull-through）切除术）切除术282021/7/27 星期二Soave procedure手术的基本步骤手术的基本步骤292021/7/27 星期二 Duhameloperation302021/7/27 星期二 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 Anastomotic stenosis andrecurrentconstipation肛门内括约肌损伤Internalanalsphincterinjury远期生活质量下降Declinedqualityoflifeinlong-term312021/7/27 星期二要点Keypoints1、概念Conceptofmegacolon2、分型Pathologicaltypeofmegacolon3、临床特征Clinicalcharacteristicsanddiagnosisofmegacolon4、治疗原则Thesurgicalprinciplesofmegacolon 322021/7/27 星期二肛门直肠畸形的分类肛门直肠畸形的分类Classificationofanorectalmalformation 332021/7/27 星期二没有没有X X影像学资料前不要轻易手术影像学资料前不要轻易手术WeshouldnotdooperationeasilywithoutX-ray 342021/7/27 星期二MRMR352021/7/27 星期二针形电刀Needletypeelectricknife电刺激仪Electricalstimulationinstrument362021/7/27 星期二先天性胆道闭锁先天性胆道闭锁Congenitalbiliaryatresia 372021/7/27 星期二Diagnosisanddifferentialdiagnosis MRCP,laparoscopicexplorationandintraoperativecholangiography Cholestasissyndrome382021/7/27 星期二Operationtreatmentofcongenitalbiliaryatresia 纤维三角板的切除 Excisionoffibroustriangleplate 肝门部与肠吻合 AnastomosisofjejunumandHilar392021/7/27 星期二胆道闭锁术后用药胆道闭锁术后用药 Medication afterbiliaryatresiaoperation激素应用激素应用Hormonedrugs抗生素应用抗生素应用Antibiotics利胆药应用利胆药应用Cholagogue402021/7/27 星期二先天性胆总管囊肿先天性胆总管囊肿Congenitalcholedochalcyst 412021/7/27 星期二MRCP422021/7/27 星期二治疗治疗:囊肿切除，囊肿切除，囊肿切除，囊肿切除，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-Yanastomosis,antiTreatment:cystexcision,Roux-en-Yanastomosis,antirefluxvalverefluxvalve 432021/7/27 星期二
