成人先天性胆管囊状扩张症的临床分析

目的:探讨成人先天性胆管囊状扩张症的诊断及治疗。方法:回顾性分析1996年1月—2012年5月收治的53例成人先天性胆管囊状扩张症患者的临床资料。结果:53例患者均行手术治疗,Ia,Ib,Ic型39例和II型4例行囊肿完整切除、肝总管空肠Roux-en-Y吻合术,另3例Ia型行囊肿大部分切除、肝总管空肠Roux-en-Y吻合术;IVa型1例行肝左外叶切除、囊肿完整切除、胆管成形、肝总管空肠Roux-en-Y吻合术,另1例行囊肿完整切除、肝总管空肠Roux-en-Y吻合术;V型1例行左肝内胆管囊肿切除术;2例癌变患者,其中1例行癌变囊肿切除、局部转移淋巴结清扫术,1例行囊肿切除、左肝内胆管肿瘤切除术。53例手术患者中获随访42例,随访时间为6个月至3年,良性患者情况良好;2例癌变患者,1例术后生存26个月,死于肿瘤复发,多系统器官功能衰竭,1例术后26个月肿瘤复发,肝转移,再次手术行左半肝切除、S5(第V段肝脏)切除、肝肠吻合术,术后2个月出现肿瘤进展,死于多系统器官功能衰竭。结论:成人先天性胆管囊状扩张症的手术方式选择与治疗效果密切相关,不同的临床分型应选择不同的手术方式。

Clinical analysis of congenital cystic dilatation of bile ducts in adults

Objective: To investigate the diagnosis and treatment of adult congenital bile duct cysts (BDC). Methods: The clinical data of 53 adult patients with congenital BDC admitted from January 1996 to May 2012 were retrospectively analyzed. Results: All patients underwent operation. Of the patients, total cyst excision with Roux-en-Y hepaticojejunostomy was performed in 39 cases with types Ia, Ib and Ic BDC as well as in 4 cases with type II BDC, while another 3 cases with type Ia BDC underwent partial cyst excision plus Roux-en-Y hepaticojejunostomy; one case with type IVa BDC underwent left lateral hepatic lobectomy, total cyst excision, hepatic ducts plasty and Roux-en-Y hepaticojejunostomy, and another case of type IVa BDC underwent total cyst excision plus Roux-en-Y hepaticojejunostomy; one case with type V BDC underwent left intrahepatic bile duct cyst excision. Of the two patients who showed malignant change, one case underwent resection of the malignant cysts and dissection of local metastatic lymph nodes, and another underwent cyst excision and left intrahepatic bile duct tumor resection. Forty-two of the 53 patients were followed-up for 6 months to 3 years. All patients with benign disease were in excellent condition after surgery. Of the two cancer patients, one case died of tumor recurrence and multi-system organ failure 26 months after peration, and the other case had tumor recurrence and liver metastasis after 26 months and then underwent re-operation that included left hemihepatectomy, S5 resection and Roux-en-Y hepaticojejunostomy, but finally died two months after re-operation due to the tumor progression and multi-system organ failure. Conclusion: For adult congenital BDC, the selection of surgical procedure may significantly influence the final results, and the different clinical types require different surgical methods.