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目的总结先天性胆总管囊肿癌变的防治经验。方法对 2 0年间收治的 16例先天性胆总管囊肿癌变患者的临床资料进行回顾性分析。结果 7例既往未做过手术 ,9例为内引流术后癌变。临床表现为非特异性 ,其中 12例表现为化脓性胆管炎 ,4例为上腹部包块 ,16例均伴乏力、消瘦。 7例既往无手术史的癌变患者中 5例行ERCP检查 ,其中 4例合并胆胰管合流异常。手术方式包括剖腹探查术 4例 ,T管引流术 4例 ,囊肿切除加胰十二指肠切除术 3例 ,囊肿部分切除 ,肝左外叶切除 2例 ,囊肿切除、肝管空肠Roux en Y吻合术 3例。癌变主要位于囊壁者 14例 ,术后病理结果腺癌占多数 (13例 )。术后生存期为 4～ 31个月 ,平均 12 7月。结论先天性胆总管囊肿癌变的临床表现不典型 ,术前诊断困难 ,预后极差 ,行内引流术后更易癌变。应以预防为主 ,对先天性胆管囊肿患者推荐行囊肿完全切除术。术中冰冻切片检查有助于确诊 ,对浸润至胰腺的病例应考虑行囊肿切除及胰十二指肠切除术
Objective To summarize the prevention and treatment of congenital choledochal cyst. Methods The clinical data of 16 patients with congenital choledochal cyst cancers admitted in 20 years were analyzed retrospectively. Results None of the 7 patients had previous surgery, and 9 had cancer after internal drainage. Clinical manifestations of non-specific, of which 12 cases showed purulent cholangitis, 4 cases of upper abdominal mass, 16 cases were accompanied by fatigue, weight loss. ERCP was performed in 5 of 7 cancer patients who had no prior history of surgery, 4 of whom had abnormal cholangiopancreatography. Surgical methods included laparotomy in 4 cases, T-tube drainage in 4 cases, cyst excision plus pancreatoduodenectomy in 3 cases, partial cyst excision, left hepatic resection in 2 cases, cyst excision and hepatic jejunum Roux en Y Anastomosis in 3 cases. Fourteen cases of cancers were mainly located in the cyst wall, and postoperative pathological findings accounted for the majority of adenocarcinomas (13 cases). Postoperative survival was 4 to 31 months, an average of 12 July. Conclusions The clinical manifestations of congenital choledochal cyst carcinogenesis are not typical, the diagnosis is difficult before operation, the prognosis is very poor, and it is more likely to become cancerous after drainage. Should be based on the prevention of cysts in patients with congenital choledochal cysts recommended complete resection. Intraoperative frozen section examination helps to confirm the diagnosis of infiltration into the pancreas should consider cyst excision and pancreatoduodenectomy