成人先天性胆总管囊肿的诊断与治疗

Diagnosis and treatment of adult patients with congenital choledochal cysts

目的探讨成人先天性胆总管囊肿的诊断及治疗。方法回顾性分析近10余年来共收治的78例成人先天性胆总管囊肿患者临床资料。结果 78例患者中,接受手术治疗手术治疗74例,其中术式包括：（1）囊肿完整切除、肝总管空肠端侧吻合52例;（2）囊肿大部分切除、肝总管空肠端侧吻合9例;（3）胰十二指肠切除术2例（其中1例为胆总管下端囊肿癌变）;（4）11例癌变患者1例行胰十二指肠切除术,7例行胆管癌根治术,3例行姑息性外引流术;（5）1例患者因合并胆总管囊肿破裂出血并胆汁性腹膜炎而行单纯外引流术。74例手术患者中获随访54例,随访率为73.0%,随访2个月至7年,平均39个月,良性患者情况良好。癌变患者中,1例行胰十二指肠切除术患者失访,7例行胆管癌根治术患者5例得到随访,生存期2~5年,中位生存时间2.3年,3例行姑息性手术患者生存期2~16个月,中位生存时间7.8个月。结论成人先天性胆总管囊肿易发生癌变,早期诊断、早期治疗是先天性胆总管囊肿诊治的关键所在,其治疗以手术切除囊肿及胆-肠重建为基本原则。

Objective To investigate the diagnosis and treatment of congenital choledochal cysts in adults.Methods The records of 78 cases of congenital choledochal cyst in adults seen in the last 10 years were retrospectively analysed.Results Among the 78 cases,74 patients under went operation：（1）52 cases had total cyst excision and Roux-en-Y hepatojejunostomy;（2）9 cases had partial cyst excision and Roux-en-Y hepatojejunostomy;（3）2 cases had duodenopancreatectomy;（4）Among 11 cases with cancer,1 case underwent duodenopancreatectomy,7 cases had radical resection of cholangiocarcinoma and 3 cases had palliative external drainage;（5）1 case had external drainage for bleeding and rupture of choledochal cyst.Among the 74 cases,54 patients were followed-up,the rate of follow-up was 73.0%.Follow-up period was 2 months to 7 years,mean 39 months.Patients with benign disease were in excellent condition.Among malignant disease patients,1 case had duodenopancreatectomy,was lost to follow-up;5 of the 7 cases with radical correction of cholangiocarcinoma were followed up and the survival time was 2-5 years,with the median survival time of 2.3 years.Three cases,who had palliative external drainage,had a survival of 2-16 months,with the median survival time of 7.8 months.Conclusions Adult congenital choledochal cyst is prone to develop cancer.Early diagnosis and early treatment of congenital choledochal cyst is the key,and surgical excision of the cyst and bilioenterostomy are the basic principles of treatment.