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先天性胆总管囊肿穿孔是胆总管囊肿的严重并发症，是小儿急性腹膜炎的重要原因，除一般临床症状，全身中毒症状重，引起严重后果。由于临床症状缺乏特异性，常致漏诊、误诊发生。常用辅助检查包括腹腔穿刺抽液，B超、C T等。目前常用治疗手段为手术，1期根治术：胆总管囊肿切除加胆道重建术，即胆总管-空肠R-Y吻合术；分期手术：1期行引流术，3～6个月后再行2期根治术；使用腹腔镜Ⅰ期行引流术，可为二期根治术创造更好解剖条件。“,”Congenital bile duct cysts perforation is a common bile duct cyst of serious complications, is the important reason of children acute peritonitis. Due to the lack of specific clinical symptoms often cause of missed diagnosis and misdiagnosis. Commonly used auxiliary examination include:abdominal puncture pumping fluid, B ultrasonic, CT, etc. At present commonly used treatment for surgery. 1 radical prostatectomy:common bile duct cyst resection with biliary revascularization. Drainage, staging surgery:1 line again 2) 3~6 months after radical prostatectomy. Using laparoscopic drainage, can create better for the second phase of radical dissection.