先天性胆总管囊肿癌变20例报告

目的总结先天性胆总管囊肿癌变的防治经验。方法回顾性分析1981-2006年间收治的先天性胆总管囊肿癌变20例的临床资料。结果原发癌变9例,内引流术后癌变11例。临床表现为非特异性,其中15例表现为化脓性胆管炎,5例为上腹部包块,均伴乏力、消瘦。原发胆总管囊肿癌变9例中7例行ERCP检查,其中6例合并胆胰管合流异常。手术方式包括剖腹活检术4例,T管引流4例,囊肿切除加胰十二指肠切除术6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4例。癌变主要位于囊壁18例,术后病理结果16例(16/20)为腺癌。术后生存期为4~31个月,平均12.7个月。结论胆胰管合流异常是先天性胆总管囊肿癌变的病因之一,临床表现不典型,术前诊断困难,预后极差,先天性胆总管囊肿行内引流术后更易癌变,对先天性胆管囊肿病人推荐行新的预防癌变的手术方式,术中冰冻切片检查有助于确诊,对侵及胰腺的病例考虑行囊肿切除及胰十二指肠切除术。

Malignant Change in Congenital Choledochal Cyst

Objective To summarize the experience of prevention and cure of malignant change in choledochal cyst in the past 25 years.Methods The clinical data of 20 patients admitted from 1981to 2006 were analyzed retrospectively.Results 9 patients had synchronous and 11 had metachronous carcinoma lesions arising in the biliary duct cyst.The clinical presentation was non-specific,in which 15 patients had biliary tract infection,5 cases followed abdominal mass.All of the cases were accompanied body weight loss and general malaise.ERCP performed in 7 cases who had metachronous carcinoma,in which Abnormal pancreatobiliary duct junction was found in 6 patients.Operative procedures for bile duct malignancy included exploratory,laparotomy with metastatic lvmph node biopsy in 4 patients,choledochotomy with T-tube drainage in 4 patients,cyst excision and pancreatoduodenectomy in 6 cases,partial cyst excision with left lobectomy in 2 patients,cysts excision with Roux-en-Y hepaticojejunostomy in 4 patients.The site of carcinoma were cyst wall in 18 cases,the pathology of 16 cases were adenocarcinoma.Postoperature survival time ranged from 4 to 31 months with a mean of 12.7 months.Conclusion One of the aetiology in congenital choledochal cyst malignance is abnormal pancreatobiliary duct junction,The clinical symptoms of malignant change in congenital choledochal cyst were non-specific.The diagnosis in preoperative were difficult and the prognosis was poor.At present,prevention should be emphasized.A new procedure which can prevent from maligance of choledochal cysts is recommended.Intraoperative frost slice examination is helpful to comfirm diagnosis.cyst excision with pancreatoduodenectomy is the treatment of choice for this disease invading pancreatic head.