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目的:明确先天性胆管囊肿非合理诊疗导致不良临床后果的主要类型。方法:回顾性分析东方肝胆外科医院2010年1月至2016年12月间收治的先天性胆管囊肿患者资料。归纳分析既往因未能接受合理治疗导致继发不良后果的患者囊肿类型、既往治疗方案和病程特点等临床信息。总结胆管囊肿继发不良治疗后果的主要类型和临床表现特征。结果:筛查875例先天性胆管囊肿资料后共有268例患者纳入本研究，其中男性83例，女性185例，年龄(34.0±16.0)岁。268例中有18例(6.7%)患者既往仅采取临床观察或非手术治疗方案；250例(93.3%)患者既往曾接受胆道外科手术治疗和/或内镜逆行胰胆管造影等介入治疗，其中有58例(23.2%)既往曾接受多次外科手术治疗和/或内镜逆行胰胆管造影介入治疗，仍需实施胆管囊肿切除术；173例(64.6%)患者既往接受过胆肠吻合术，其中57例(32.9%)患者既往未施行胆管囊肿切除术；在116例(43.3%)既往接受胆管囊肿切除、胆肠吻合术的患者中，残留胆管囊肿57例(49.1%)，胆肠吻合口狭窄28例(24.1%)，同时发生残留胆管囊肿及胆肠吻合口狭窄者30例(25.9%)；胆管囊肿癌变46例(17.1%)，胆肠吻合口癌变4例(1.5%)。结论:胆管囊肿残留和胆肠吻合口狭窄及并发的胆管炎和胆管结石是胆管囊肿非合理治疗继发不良后果的主要类型。“,”Objective:To identify adverse clinical outcomes after unreasonable treatments of congenital bile duct cyst.Methods:Data on patients with congenital bile duct cysts treated at the Eastern Hepatobiliary Surgery Hospital between January 2010 and December 2016 were retrospectively reviewed. The clinical data on cyst type, previous treatments and disease courses in patients who had previously been treated inappropriately with adverse treatment outcomes were analyzed.Results:Of 268 patients who were included in the study after screening 875 patients with congenital bile duct cysts, there were 83 males and 185 females, with a Mean±SD age of (34.0±16.0) years. There were 18 patients (6.7%) who were previously treated by clinical observation or non-surgical treatment. Of 250 patients (93.3%) who had undergone surgical and/or intervention treatments, 58 patients (23.2%) underwent multiple surgical and/or ERCP treatments but the cysts were still there. For the 173 patients (64.6%) who had undergone choledochojejunostomy, 57 (32.9%) still had an intact cyst of the 116 patients (43.3%) who had undergone choledochotomy and choledochostomy, 57 patients (49.1%) had residual cysts, 28 patients (24.1%) developed choledochostomy stenosis, and 30 patients (25.9%) had a combination of residual cyst and choledochostomy stenosis. Malignancies developed in 46 patients (17.1%) in the bile duct cysts and at the choledochojejunostomy anastomosis in 4 patients (1.5%).Conclusions:Residual bile duct cyst, stricture of choledochojejunostomy, cholangitis and cholelithiasis were the main adverse outcomes after unreasonable treatments of choledochocyst.