成人多囊肝、多囊肾病外科治疗可行性的探讨

Feasible surgical treatment of autosomal dominant polycystic liver disease associated with autosomal dominant polycystic kidney disease

目的:探讨采用肝部分切除加肝囊肿广泛开窗联合肾囊肿去顶减压术治疗严重成人多囊肝、多囊肾病的效果及可行性。方法:回顾分析我院自2007年7月至10月采用肝部分切除联合囊肿广泛开窗治疗严重成人多囊肝、多囊肾病3例患者的术前症状、手术方法、手术时间、术后并发症及手术效果。结果:3例患者均顺利出院,手术时间分别为475、402、430min,3例患者均出现胸、腹腔积液,经穿刺引流后胸、腹腔积液消失。3例分别于术后9、6、5个月随访,症状消失,肾功能正常,2例术前伴高血压的患者血压恢复正常。结论:肝部分切除加囊肿广泛开窗联合肾囊肿去顶减压术治疗严重成人多囊肝、多囊肾病是一种理想安全的治疗方法,但术后并发症较多,长期疗效尚需观察。

Objective：To investigate the feasibility and outcome of partial hepatectomy and fenestration combined with renal cyst decortication for treatment of autosomal dominant polycystic liver disease（APLD）associated with autosomal dominant polycystic kidney disease（APKD）.Methods：From July 2007 to Oct.2007,three patients with APLD associated with APKD were treated by partial hepatectomy and fenestration combined with renal cyst decortication in our hospital.Their preoperative symptoms,operation procedure,operation time,complications and the clinical outcomes were retrospectively analyzed.Results：The 3 patients were successfully treated and discharged.The operation time periods of the 3 patients were 475,402,and 430 minutes.Pleural effusion and ascites occurred in all the 3 patients and disappeared after puncture and drainage.Follow-up was conducted 5,6,and 9 mouths later,the symptoms disappeared in all patients and the renal function became normal.The blood pressure of 2 patients recovered to normal level after operation.Conclusion：Partial hepatectomy and fenestration combined with renal cyst decortication is a safe and acceptable procedure for treatment APLD associated with APKD.The complication rate is comparatively more and the long-term outcomes need to be evaluated further.