【摘要】 本文综合分析了先天性胆管扩张症并发癌变４９例的临床资料，其中作者医院收治的５例和收集国内文献报道的４４例；癌变率明显高于非扩张囊肿性胆管的癌变率；发现癌变时平均年龄为３１．５岁，早于国外６岁左右。以前接受过胆肠内引流术者占６７．３％，表示更易癌变。术前癌变确诊仅６例，比较困难。仔细的Ｂ超、ＰＴＣ、ＣＴ和ＥＲＣＰ检查对癌变诊断有较大帮助，尽量争取术中获诊。全组癌肿切除率为２９．７％，根据先天性胆管扩张症可能发生癌变因素，强调指出癌变发生前早期彻底切除扩张胆管（即囊肿），特别是内层上皮至为重要，以预防癌变。更多还原

【Abstract】 Abstract The authors collected 49 cases of bile duct carcincoma arising on the basis of congenital choledochocele from domestic literature including their own 5 cases. Among them 33 cases had received various internal drainage procedures. Correct preoperative diagnosis was made in only 6 cases (12.2%), and the tumor was surgically resected in 14 cases (29.7%). In order to prevent the canceration it is mandatory to perform primary excision of the dilated common bile duct till its confluence with the pancreatic duct and do a choledochojejunostomy.更多还原