先天性胆总管囊肿课件

先天性胆总管囊肿 (Congenital choledochal cyst),图例,概述,Vater 1723年首次报告 Todd 1818年作了描述 Douglas 1852年首次命名 Mc Whorter 1924年首次行囊肿切除手术,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,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。,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),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。,The drawing shows the normal arrangement of the gallbladder and bile ducts below the level of the liver.,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 treatment,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。,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 CT PTC MRI ERCP 放射性核素扫描 选择性腹腔动脉造影,Diagnostic sonogram demonstrating a type I choledochal cyst in a 4-month-old child presenting with elevated hyperbilirubinemia and hepatic transaminase levels.,Choledochal cyst identified on CT scan. The bile duct (white with arrows) should be less than one tenth as large as it is.,Operative cholangiogram delineating choledochal cyst and pertinent associated biliary anatomy.,Surgical specimen.,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 filling of the cyst by the radionuclide.,鉴别诊断,胆道闭锁 肝包虫 慢性肝炎 右肾盂积水 腹部肿瘤,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,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,This drawing shows the various options available for the surgical management of choledochal cyst. The method shown on the right, complete cyst removal with drainage into a specially constructed limb of intestine is preferred,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,切除囊肿，上提空肠袢,空肠胆管Y式吻合,间置空肠胆管十二指肠吻合,(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,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,（三）先天性胆总管囊肿空肠吻合术（Y形吻合）,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）,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,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,Type 1 choledochal cyst in a 25-year-old woman. MR cholangiopancreatogram demonstrates fusiform dilatation of the suprapancreatic portion of the extrahepatic bile duct