1、先天性胆总管囊肿(Congenital choledochal cyst)1ppt课件图例2ppt课件概述mVater 1723Vater 1723年首次报告年首次报告mTodd 1818Todd 1818年作了描述年作了描述mDouglas 1852Douglas 1852年首次命名年首次命名mMc Whorter 1924Mc Whorter 1924年首次行囊肿切除手术年首次行囊肿切除手术3ppt课件概述n又称先天性胆管囊状扩张症。又称先天性胆管囊状扩张症。n东方民族多见，日本、中国发病率高。东方民族多见，日本、中国发病率高。n幼儿、儿童期发现者幼儿、儿童期发现者80%80%n男男:女女
2、=1:4=1:45 54ppt课件病因n先天性胰胆管合流异常；先天性胰胆管合流异常；n2.2.胆道上皮增生不平衡；胆道上皮增生不平衡；n3.3.病毒感染。病毒感染。5ppt课件病理nAlonso-LejAlonso-Lej将其分为：将其分为：1.1.囊性扩张型：球状，梭形，少数圆柱状（囊性扩张型：球状，梭形，少数圆柱状（86.7%86.7%）；）；2.2.憩室型（憩室型（3.1%)3.1%)；3.3.胆总管口囊性脱垂胆总管口囊性脱垂(5.6%)(5.6%)；4.4.混合型混合型:肝内、肝外肝内、肝外(2.6%)(2.6%)；5.Caroli5.Caroli病病(1958)(1958)(肝内肝内
3、)6ppt课件病理n囊肿容量数毫升至数千毫升。囊肿容量数毫升至数千毫升。n世界报告最大的胆总管囊肿容量分别为世界报告最大的胆总管囊肿容量分别为5800ml,8000ml5800ml,8000ml。n反复感染，致壁厚反复感染，致壁厚2-5mm2-5mm不等，色黄褐，质硬韧，纤维组织不等，色黄褐，质硬韧，纤维组织增生，平滑肌稀少，有时无上皮覆盖。增生，平滑肌稀少，有时无上皮覆盖。7ppt课件The drawing shows the normal arrangement of the gallbladder and bile ducts below the level of the liver.8
4、ppt课件These are the five common types of choledochal cyst malformations.The ability to show these clearly by cholangiography(injecting dye into the bile ducts)has made it possible to plan appropriate operative treatment9ppt课件临床表现n腹痛腹痛n肿块肿块:(90%):(90%)n黄疸黄疸:(70%)“:(70%)“三联症三联症”，同时出现率仅，同时出现率仅20%-30%20%
5、-30%。n发热，呕吐，发热，呕吐，T38-39T38-39。n尿尿:色深，粪便淡，灰白。色深，粪便淡，灰白。n囊肿穿孔：剧烈腹痛，呕吐，腹肌紧张，胆汁性腹膜炎。囊肿穿孔：剧烈腹痛，呕吐，腹肌紧张，胆汁性腹膜炎。10ppt课件11ppt课件诊断n病史，体征病史，体征n生化检查：胆红素，生化检查：胆红素，AKP,AKP,沉粉酶，肝功沉粉酶，肝功,血，尿，粪常规。血，尿，粪常规。nB B超。超。nX-ray,X-ray,平片，上消化道气钡造影平片，上消化道气钡造影nCTCTnPTCPTCnMRIMRInERCPERCPn放射性核素扫描放射性核素扫描n选择性腹腔动脉造影选择性腹腔动脉造影12ppt课
6、件Diagnostic sonogram demonstrating a type I choledochal cyst in a 4-month-old child presenting with elevated hyperbilirubinemia and hepatic transaminase levels.13ppt课件Choledochal cyst identified on CT scan.The bile duct(white with arrows)should be less than one tenth as large as it is.14ppt课件Operati
7、ve cholangiogram delineating choledochal cyst and pertinent associated biliary anatomy.15ppt课件Surgical specimen.16ppt课件Nuclear medicine scan of a choledochal cyst.Early image shows most of the radionuclide in the liver.Nuclear medicine scan of a choledochal cyst midway through the study with better
8、filling of the cyst by the radionuclide.17ppt课件鉴别诊断n胆道闭锁胆道闭锁n肝包虫肝包虫n慢性肝炎慢性肝炎n右肾盂积水右肾盂积水n腹部肿瘤腹部肿瘤18ppt课件治疗n症状发作期间的治疗症状发作期间的治疗禁食禁食解痉药物解痉药物抗生素抗生素保持水、电解质、酸碱平衡保持水、电解质、酸碱平衡有黄疸者用有黄疸者用VitKVitK，纠正凝血功能障碍，纠正凝血功能障碍症状缓解后择期手术症状缓解后择期手术19ppt课件治疗n手术治疗手术治疗囊肿切除胆道重建（囊肿切除胆道重建（Lilly.1978)Lilly.1978)l肝总管空肠肝总管空肠Roux-en-yRo
9、ux-en-y式吻合术式吻合术l肝总管空肠襻式吻合术肝总管空肠襻式吻合术l空肠间置、肝总管十二指肠吻合术（加防反流瓣）空肠间置、肝总管十二指肠吻合术（加防反流瓣）l阑尾代胆道肝总管十二指肠吻合阑尾代胆道肝总管十二指肠吻合囊肿、胃吻合囊肿、胃吻合囊肿、十二指肠吻合术囊肿、十二指肠吻合术囊肿外引流术囊肿外引流术20ppt课件This drawing shows the various options available for the surgical management of choledochal cyst.The method shown on the right,complete cys
10、t removal with drainage into a specially constructed limb of intestine is preferred21ppt课件切断囊肿下端切断囊肿下端保留囊肿后壁的囊肿切除保留囊肿后壁的囊肿切除（一）先天性胆总管囊肿切除术（一）先天性胆总管囊肿切除术22ppt课件切除囊肿，上提空肠袢切除囊肿，上提空肠袢空肠胆管空肠胆管Y Y式吻合式吻合23ppt课件间置空肠胆管十二指肠吻合间置空肠胆管十二指肠吻合 24ppt课件(二）先天性胆总管囊肿十二指肠吻合术二）先天性胆总管囊肿十二指肠吻合术 显露胆总管囊肿显露胆总管囊肿 吻合口后壁浆肌层间断缝合吻合
11、口后壁浆肌层间断缝合 25ppt课件切开胆总管囊肿和十二指肠肠壁切开胆总管囊肿和十二指肠肠壁 后壁全层间断缝合后壁全层间断缝合 前壁全层间断缝合前壁全层间断缝合 前壁浆肌层缝合前壁浆肌层缝合 26ppt课件（三）先天性胆总管囊肿空肠吻合术（三）先天性胆总管囊肿空肠吻合术（Y Y形吻合）形吻合）27ppt课件Roux-en-Y hepaticojejunostomy to Roux-en-Y hepaticojejunostomy to restore biliary-enteric continuity restore biliary-enteric continuity following
12、resection of choledochalfollowing resection of choledochal cystcyst 28ppt课件 术中注意事项术中注意事项 胆总管囊肿的辨认胆总管囊肿的辨认（图示上翻的肝脏和胃体，部分切除胰腺，剖开十二指肠降部，显露（图示上翻的肝脏和胃体，部分切除胰腺，剖开十二指肠降部，显露胆总管开口，扩大的胆总管如球状，肝总管比正常增粗数倍）胆总管开口，扩大的胆总管如球状，肝总管比正常增粗数倍）29ppt课件术后并发症n吻合口漏吻合口漏n出血（腹腔、消化道）出血（腹腔、消化道）n感染感染n胰腺炎胰腺炎n逆行感染逆行感染n吻合口狭窄吻合口狭窄n残留囊肿恶性
13、变残留囊肿恶性变30ppt课件预后n囊肿切除胆道重建者预后好囊肿切除胆道重建者预后好n内引流者差内引流者差n囊肿癌变率较正常人大囊肿癌变率较正常人大2020倍倍31ppt课件 Type 1 choledochal cyst in a 25-year-old woman.MR cholangiopancreatogram demonstrates fusiform dilatation of the suprapancreatic portion of the extrahepatic bile duct(arrow),indicative of a type 1 choledochal cyst.An anomalous union of the bile and pancreatic ducts is also seen(arrowhead).32ppt课件33ppt课件
