目的　研究儿童及成人先天性胆总管囊肿的不同特点 ,提高对先天性胆总管囊肿的诊治水平。方法　回顾分析 3 2例先天性胆总管囊肿的诊治资料 ,将其分为少儿 ( <14岁 )和成人( >14岁 )发病组 ,分析两者不同的临床表现、诊断和治疗特点。结果　少儿组 17例 ,临床表现为持续性黄疸、肝肿大、腹部包块 ,术前一般情况差 ,保守治疗 6例 ,其中 2例因肝硬化腹水 ,肝功能衰竭死亡 ,1例未愈出院 ,3例延期手术 ;手术 14例 ,13例行肝管空肠Roux -Y吻合 ,1例行肝管十二指肠吻合术。成人组 15例 ,以间歇性右上腹疼痛、畏寒发热、黄疸首次就诊 ,分别行囊肿切除肝管空肠吻合术 ( 12例 )、肝管十二指肠吻合术 ( 1例 )、肝左叶并囊肿切除肝管空肠吻合术 ( 2例 )。结论　先天性胆总管囊肿发病患儿起病急 ,病情发展快 ,易合并肝脏损害 ,应加强围手术期支持治疗 ;成人囊肿切除胆道重建术后 ,应密切随访 ,防治胆管炎及胆道癌变 Objective To study the different characteristics of congenital choledochal cyst in children and adults and to improve the diagnosis and treatment of congenital choledochal cyst. Methods Retrospective analysis of 32 cases of congenital choledochal cyst diagnosis and treatment of data, divided into children (<14 years old) and adults (> 14 years) incidence of group, analysis of the two different clinical manifestations, diagnosis and treatment characteristics. Results There were 17 cases in children group. The clinical manifestations were persistent jaundice, hepatomegaly and abdominal mass. Preoperative general conditions were poor. Six cases were conservatively treated. Two cases died of liver cirrhosis and liver failure, and one case was cured , 3 cases of delayed surgery; surgery in 14 cases, 13 cases of hepatic duct jejunum Roux-Y anastomosis, 1 case of hepatic duct duodenal anastomosis. In the adult group, 15 cases were treated with intermittent right upper quadrant pain, chills and fever and jaundice for the first time. All patients underwent cyst excision of hepaticojejunostomy (12 cases), duodenal anastomosis (1 case), left hepatic lobe Cyst excision of hepatic jejunum anastomosis (2 cases). Conclusions Children with congenital choledochal cyst should have acute onset, rapid progression and complicated with liver damage. Perioperative supportive treatment should be strengthened. Adult cystic resection and biliary reconstruction should be closely followed to prevent and treat cholangitis and biliary tract cancer