齐鲁医学先天性巨结肠与胆道剖析

2021/7/27星期二先天性巨结肠Congenitalmegacolon1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.22021/7/27星期二Hirschsprung’sDisease,HD赫什朋氏病Aganglionosis先天性无神经节细胞症英文名称Englishname32021/7/27星期二概述Summary一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=4:142021/7/27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.Absenceof ganglioncellsinmuscle and submucosaoflesionbowel.Itisadevelopment pause. Themoreearlythepauseoccurs,thelongerintestinalsegmentwithoutganglioncellsis.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Neuroblastoma cellsofdigestivetractmigrate fromthebeginningtotheendInthefifthweekoffetal.62021/7/27星期二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72021/7/27星期二Absenceof ganglioncellsSmoothmuscle oflesionintestinesustainescontractionAnorectal reflex loop isinterruptedStool cannotbedischarged. Proximal normal intestinehas compensatorydilatation and hypertrophyandformshugeexpansiveintestinalsegmentPathology ofcongenitalmegacolon82021/7/27星期二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NormalcolonPathological anatomyofcongenitalmegacolon92021/7/27星期二按病变长度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 segmenttypemegacolon(10%):Lesionreachessplenicflexure, andeven theentirecolon.全结肠型Totalcolonic type(1%):Lesionreathesentire colonor evevterminalileum.分型Pathologictypesofcongenitalmegacolon102021/7/27星期二临床表现Clinicalmanifestations新生儿期Theneonatalperiod: acutelow incompleteintestinalobstructionDelayoffetal dischargeAbdominaldistention andvomitingConstipationWasting andmalnutrition112021/7/27星期二Clinicalmanifestations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Progressiveabdominaldistension发育迟缓，营养不良Growthretardation,malnutrition122021/7/27星期二并发症Complications多在2个月内发生Occurring within2months肠梗阻Intestinalobstruction小肠结肠炎Enterocolitis肠穿孔，腹膜炎Bowelperforationandperitonitis继发败血症，肺炎Secondarysepsisandpneumonia132021/7/27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Accessoryexamination142021/7/27星期二152021/7/27星期二162021/7/27星期二钡灌肠X表现BariumenemaX-ray葛X，2y，长段型巨结肠，soave172021/7/27星期二182021/7/27星期二Anorectalmanometry: reflection ofnormalanorectal  reflexdisappears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Intestinal mucosalglands酶阳性神经Enzyme positivenerve正常人Normalchild巨结肠患儿Megacolonchild212021/7/27星期二诊断Diagnosis1、病史Medicalhistory2、钡灌肠Bariumenema3、直肠肛管测压力Anorectalmanometry3、直肠黏膜组织活检Rectalmucosal biopsy222021/7/27星期二1、胎粪性便秘Meconiumconstipation2、新生儿肠闭锁Neonatal intestinalatresia3、特发性巨结肠Idiopathic megacolon4、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Differentialdiagnosis232021/7/27星期二先天性巨结肠Congenital megacolon继发性巨结肠Secondarymegacolon242021/7/27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:Operation treatment.Resectionofthelesion boweland dilatation bowel.252021/7/27星期二术前准备（保守治疗）Preoperativepreparation (conservativetreatment)1、洗肠Intestinallavage2、括肛、通便 Enlarge anusandinducingdefeation3、缓泻药ApplicationofLaxativedrug262021/7/27星期二手术方式Operation methodsSewnon’soperationSoave’soperationDuhamel’soperationRehbein’soperationMartin’soperation272021/7/27星期二Swenson改良术：结肠经直肠内拖出（Pull-through）切除术282021/7/27星期二Soaveprocedure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Anastomotic infectionandleakage尿潴留Retentionofurine小肠结肠炎Enterocolitis吻合口狭窄，便秘复发Anastomoticstenosisandrecurrentconstipation肛门内括约肌损伤Internalanal sphincterinjury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Clinicalcharacteristicsand diagnosisofmegacolon4、治疗原则Thesurgicalprinciplesofmegacolon322021/7/27星期二肛门直肠畸形的分类Classification ofanorectalmalformation332021/7/27星期二没有X影像学资料前不要轻易手术Weshould notdooperationeasilywithoutX-ray342021/7/27星期二MR352021/7/27星期二针形电刀Needle typeelectric knife电刺激仪Electricalstimulation instrument362021/7/27星期二先天性胆道闭锁Congenitalbiliaryatresia372021/7/27星期二DiagnosisanddifferentialdiagnosisMRCP,laparoscopic exploration andintraoperativecholangiographyCholestasis syndrome382021/7/27星期二Operation treatment ofcongenitalbiliaryatresia纤维三角板的切除Excisionoffibrous triangle plate肝门部与肠吻合Anastomosisofjejunum andHilar392021/7/27星期二胆道闭锁术后用药Medicationafter biliaryatresiaoperation激素应用Hormonedrugs抗生素应用Antibiotics利胆药应用Cholagogue402021/7/27星期二先天性胆总管囊肿Congenital choledochalcyst412021/7/27星期二MRCP422021/7/27星期二治疗:囊肿切除，Roux-en-Y吻合，抗反流瓣Treatment: cystexcision, Roux-en-Yanastomosis, antireflux valve432021/7/27星期二