先天性胆总管囊肿癌变的防治经验

目的：总结先天性胆总管囊肿癌变的防治经验。方法；对20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。囊肿部分发除，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。术中冰切片检查有助确诊，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。

Canceration of congenital choledochal cyst:reports of 16 cases

ObjectiveTo summarize the experience in diagnosis and treatment of malignant change in choledochal cyst patients in the past 20 years. MethodsThe clinical data of 16 patients admitted from 1980 to 2000 were analyzed retrospectively. Results9 patients had had a previous internal drainage procedure,12 patients had biliary tract infection, 4 cases presented with abdominal masses. All suffered body weight loss and general malaise. ERCP was performed in 5 cases with no previous operation, in which abnormal pancreatobiliary duct junction was found in 4 patients. Laparotomy plus metastatic lymph node biopsy was performed in 4 patients, choledochotomy with T-tube drainage for 4 patients, cyst excision and pancreatoduodenectomy for 3 cases, partial cyst excision with left lobectomy for 2 patients, cyst excision with Roux-en-Y hepaticojejunostomy for 3 patients. Pathology proved carcinoma was located in cyst wall in most cases.Postoperative survival time ranged from 4 to 31 months with a mean of 12.7 months. ConclusionsThe clinical symptoms of malignant change in congenital choledochal cyst were non-specific. The preoperative diagnosis for canceration was difficult, and the prognosis was poor.Total extrahepatic choledochocele resection should be adopted for the prevention of canceration.Intraoperative frozen section is helpful to confirm diagnosis. Cyst excision with pancreatoduodenectomy is the treatment of choice for carcinoma invading pancreatic head.