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先天性胆管囊肿这一胆管畸形病变会导致胆管系统长期处于炎症状态，因此患者并发胆管结石、囊肿癌变的风险较高。外科手术是治愈先天性胆管囊肿的唯一治疗手段，其治疗要点是尽可能彻底切除囊肿病灶以及彻底实现“胆胰分流”。非规范化的外科手术治疗会给患者带来胆肠吻合口狭窄、残余胆管囊肿甚至囊肿癌变等难以处理的术后并发症，严重影响患者生活质量，威胁患者生命。笔者团队基于东方肝胆外科医院的先天性胆管囊肿大样本回顾性研究结果，围绕先天性胆管囊肿的病理解剖学特点，提出一种新的分型系统，将胆管囊肿分为“三类五型”，即肝外胆管囊肿肝门型、主干型、末端型；中央型胆管囊肿；肝内胆管囊肿局限型、弥漫型，以期能够更为精确地指导外科治疗方案的设计和实施，并降低术后远期并发症风险。“,”Congenital bile duct cysts, which is a kind of malformation of bile duct, will lead to the inflammation in the bile duct system for a long time. Therefore, patients with cholelithiasis and cyst canceration have a risk. Surgical operation is the only treatment option for the congenital bile duct cysts patients. Resection of the cysts and complete “ biliary-pancreatic duct shunt” are the key points of the standardized treatment of congenital bile duct cysts. The non-standard surgical treatment will bring the postoperative complications such as the stricture of choledochojejunostomy, the remnant cysts and even the canceration of cysts, which will seriously affect the quality of life and threaten the health of patients. Based on the retrospective study of congenital bile duct cysts patients in Eastern Hepatobiliary Hospital, the author proposed a new classification system according to the pathological and anatomical characteristics of congenital bile duct cysts. The congenital bile duct cysts can be divided into three regions and five types, i. e. localized type and diffuse type of extrahepatic bile duct cysts of hilar, trunk and terminal type; central type; and intrahepatic bile duct cysts of limited and diffuse type. It is our hope that this typing system will accurately guide the design and implementation of surgical treatment plans for congenital bile duct cysts and reduce the risk of long-term postoperative complications for patients.