肝移植治疗布加综合征九例

Treating Budd-Chiari syndrome with liver transplantation., report of 9 cases

目的总结原位肝移植术治疗布加综合征的经验和体会。方法回顾性分析2003年12月至2010年4月9例布加综合征患者接受肝移植治疗的临床资料，总结术前影像学评估和手术经验，并观察术后并发症的发生情况及受者存活情况。结果术前9例患者均经腹部CT强化扫描及血管重建等检查明确诊断，并行腔静脉造影明确梗阻情况及分型。9例患者中，接受尸体供肝移植者8例，均采用经典非转流术式；接受亲属活体右半供肝移植1例。术后免疫抑制方案为他克莫司＋吗替麦考酚酯＋皮质激素。9例受者术后平均随访时间为32．8个月（13-61个月），期间出现癫痫发作1例，十二指肠乳头及功能不良1例，小肝综合征1例，以及肺部感染4例；1例接受了中度脂肪肝供肝的受者于术后12d因原发性移植肝无功能行再次肝移植；1例受者于术后6个月因发生胆道并发症导致移植肝功能丧失而行再次肝移植，该例患者术后35个月死于肝脏肿瘤复发。所有患者未发生流出道梗阻及布加综合征复发。9例受者术后1和2年存活率为100％（9／9），术后3年存活率为88．9oA（8／9）。结论通过准确的影像学评估和选择适宜的手术方式，肝移植已成为治疗布加综合征的重要手段，受者预后良好。

Objective To investigate the experience of treating Budd-Chiari syndrome through orthotopic liver transplantation. Methods The clinical data of LTx performed on 9 patients with Budd-Chiari syndrome from December 2003 to April 2010 were retrospectively analyzed. We summarize the preoperative image and surgical experience, and observe the occurrence of postoperative complications and survival. Results Budd-Chiari syndrome was diagnosed in 9 patients by the preoperative abdominal CT enhancement and vascular reconstruction, and cavity venography was done to observe obstruction and sub-type of CAVA vein. All 9 patients were subjected to cadaveric liver transplantation. Eight cases accepted classic non-bypass type, and one accepted living related right lobe liver transplantation. Postoperative triple immunosuppressive regimen included tacrolimus, mycophenolate mofetil, and hormone. The average follow-up periods for all these 9 patients were 32. 8 months （13 to 61 months）. One patient died from the tumor recurrence at 35th month after the operation. Two patients received re-transplantation for the lost of the graft. One recipient received the donor liver with medium steatosis, and the re-transplantation was performed on the 12th day after the first transplantation due to the primary non-function of the graft. The other one received the secondary liver transplantation at 6th month after the first transplantation due to the biliary complication and died from the liver tumor recurrence. Among all the 9 cases, seizure disorder （1 case）, dysfunction of duodenal papillary muscle （1 case） and small-for-size syndrome （one case） occurred after the operation. Pulmonary infection occurred in 4 cases： 3 cases due to the bacterial infection and 1 due to the fungal infection. Neither outflow obstruction nor the recurrence of the Budd-Chiari syndrome occurred in this study. The 1- and 2-year survival rate after the operation was both 100%, and 3-year survival rate post-transplantation was 88. 9% （8/9）. Conclusion Liver transplantation can be the ideal treatment to the Budd-Chiari syndrome based on the definite clinical diagnosis, accurate imaging evaluation and eligible modus operandi.