先天性胆总管囊肿ppt课件

先天性胆总管囊肿(Congenitalcholedochalcyst),1,图例,2,概述,Vater1723年首次报告Todd1818年作了描述Douglas1852年首次命名McWhorter1924年首次行囊肿切除手术,3,概述,又称先天性胆管囊状扩张症。东方民族多见，日本、中国发病率高。幼儿、儿童期发现者80%男:女=1:45,4,病因,先天性胰胆管合流异常；2.胆道上皮增生不平衡；3.病毒感染。,5,病理,Alonso-Lej将其分为：1.囊性扩张型：球状，梭形，少数圆柱状（86.7%）；2.憩室型（3.1%)；3.胆总管口囊性脱垂(5.6%)；4.混合型:肝内、肝外(2.6%)；5.Caroli病(1958)(肝内),6,病理,囊肿容量数毫升至数千毫升。世界报告最大的胆总管囊肿容量分别为5800ml,8000ml。反复感染，致壁厚2-5mm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。,7,Thedrawingshowsthenormalarrangementofthegallbladderandbileductsbelowtheleveloftheliver.,8,Thesearethefivecommontypesofcholedochalcystmalformations.Theabilitytoshowtheseclearlybycholangiography(injectingdyeintothebileducts)hasmadeitpossibletoplanappropriateoperativetreatment,9,临床表现,腹痛肿块:(90%)黄疸:(70%)“三联症”，同时出现率仅20%-30%。发热，呕吐，T38-39。尿:色深，粪便淡，灰白。囊肿穿孔：剧烈腹痛，呕吐，腹肌紧张，胆汁性腹膜炎。,10,11,诊断,病史，体征生化检查：胆红素，AKP,沉粉酶，肝功,血，尿，粪常规。B超。X-ray,平片，上消化道气钡造影CTPTCMRIERCP放射性核素扫描选择性腹腔动脉造影,12,DiagnosticsonogramdemonstratingatypeIcholedochalcystina4-month-oldchildpresentingwithelevatedhyperbilirubinemiaandhepatictransaminaselevels.,13,CholedochalcystidentifiedonCTscan.Thebileduct(whitewitharrows)shouldbelessthanonetenthaslargeasitis.,14,Operativecholangiogramdelineatingcholedochalcystandpertinentassociatedbiliaryanatomy.,15,Surgicalspecimen.,16,Nuclearmedicinescanofacholedochalcyst.Earlyimageshowsmostoftheradionuclideintheliver.,Nuclearmedicinescanofacholedochalcystmidwaythroughthestudywithbetterfillingofthecystbytheradionuclide.,17,鉴别诊断,胆道闭锁肝包虫慢性肝炎右肾盂积水腹部肿瘤,18,治疗,症状发作期间的治疗禁食解痉药物抗生素保持水、电解质、酸碱平衡有黄疸者用VitK，纠正凝血功能障碍症状缓解后择期手术,19,治疗,手术治疗囊肿切除胆道重建（Lilly.1978)肝总管空肠Roux-en-y式吻合术肝总管空肠襻式吻合术空肠间置、肝总管十二指肠吻合术（加防反流瓣）阑尾代胆道肝总管十二指肠吻合囊肿、胃吻合囊肿、十二指肠吻合术囊肿外引流术,20,Thisdrawingshowsthevariousoptionsavailableforthesurgicalmanagementofcholedochalcyst.Themethodshownontheright,completecystremovalwithdrainageintoaspeciallyconstructedlimbofintestineispreferred,21,切断囊肿下端,保留囊肿后壁的囊肿切除,（一）先天性胆总管囊肿切除术,22,切除囊肿，上提空肠袢,空肠胆管Y式吻合,23,间置空肠胆管十二指肠吻合,24,(二）先天性胆总管囊肿十二指肠吻合术,显露胆总管囊肿,吻合口后壁浆肌层间断缝合,25,切开胆总管囊肿和十二指肠肠壁,后壁全层间断缝合,前壁全层间断缝合,前壁浆肌层缝合,26,（三）先天性胆总管囊肿空肠吻合术（Y形吻合）,27,28,术中注意事项,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，显露胆总管开口，扩大的胆总管如球状，肝总管比正常增粗数倍）,29,术后并发症,吻合口漏出血（腹腔、消化道）感染胰腺炎逆行感染吻合口狭窄残留囊肿恶性变,30,预后,囊肿切除胆道重建者预后好内引流者差囊肿癌变率较正常人大20倍,31,Type1choledochalcystina25-year-oldwoman.MRcholangiopancreatogramdemonstratesfusiformdilatationofthesuprapancreaticportionoftheextrahepaticbil