先天性胆总管囊肿

图例Р概述РVater 1723年首次报告РTodd 1818年作了描述РDouglas 1852年首次命名РMc Whorter 1924年首次行囊肿切除手术Р概述Р又称先天性胆管囊状扩张症。Р东方民族多见,日本、中国发病率高。Р幼儿、儿童期发现者80%Р男:女=1:4-5Р病因Р先天性胰胆管合流异常;Р2.胆道上皮增生不平衡;Р3.病毒感染。Р病理РAlonso-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)Р病理Р囊肿容量数毫升至数千毫升。Р世界报告最大的胆总管囊肿容量分别为5800ml,8000ml。Р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。РThe drawing shows the normal arrangement of the gallbladder and bile ducts below the level of the liver.РThese are the mon 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 treatmentР临床表现Р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。