自体肝脏移植治疗终末期肝泡型包虫病合并继发性门静脉海绵样变

Ex vivo liver resection and autotransplantation in treating end-stage hepatic alveolar echinococcosis combined with secondary cavernous transformation of portal vein

目的探索自体肝脏移植治疗终末期肝泡型包虫病合并继发性门静脉海绵样变的可行性及安全性。方法对1例诊断为终末期肝泡型包虫病合并继发性门静脉海绵样变的患者,术前经过CT、MRI及超声检查评价肝脏占位病灶大小、范围及血管侵犯情况,使用IQQA肝脏系统(EDDA Technology,Inc.USA)重建肝脏模型,测量并记录剩余肝脏体积及第1肝门侵犯程度,经多学科协作讨论后拟采用自体肝脏移植对其进行治疗。结果经过充分术前评估,明确患者肝内病灶147 mm×134 mm×165 mm大,严重侵犯肝后下腔静脉及肝右和肝中静脉并造成三者闭塞,肝左静脉部分受侵,伴有门静脉海绵样变,剩余肝脏体积约912 m L,剩余肝脏体积与标准肝脏体积比为0.81,术前肝脏功能Child-Pugh评分为A级。按预期方案行自体肝脏移植术,术中应用患者自身大隐静脉做移植物重建部分流入及流出道,下腔静脉未予以重建,术后患者恢复良好并于术后第20天顺利出院。结论自体肝脏移植可成功治疗终末期肝泡型包虫病合并继发性门静脉海绵样变,其中充分的术前评估和第一肝门的处理是手术的关键。

Objective To explore feasibility and safety of ex vivo liver resection and autotransplantation in treating end-stage hepatic alveolar echinococcosis combined with secondary cavernous transformation of portal vein. Methods The patient was diagnosed with the end-stage hepatic alveolar echinococcosis combined with secondary cavernous transformation of portal vein. The ultrasonography, computed tomography, and magnetic resonance imaging were used to access the characteristics of the lesions and the extent of involvement of the portal vein and its branches. The liver model was reconstructed using a three-dimensional imaging data analysis system （EDDA Technology, Inc. USA）, the remnant liver volume and the extent of involvement of the first hepatic hilum were recorded. Then the multidisciplinary team repetitively discussed the risks and procedures involved in the surgery. Finally, the ex vivo liver resection and autotransplantation was proposed. Results The preoperative evaluation showed the patient had a large intrahepatic lesion which severely invaded the retrohepatic inferior vena cava, the right hepatic vein, and the middle hepatic vein and were completely occluded, the left hepatic vein was partially invaded, and the portal vein was spongiform. The remnant liver volume was 912 mL, the ratio of residual liver volume to standard liver volume was 0.81. The preoperative liver function Child-Pugh score was grade A. The ex vivo liver resection and autotransplantation was successfully managedaccording to the expected schedule. The autografts （made by patient＇s great saphenous vein） were used to reconstruct the hepatic vein and portal vein, and the retrohepatic inferior vena cava was not reconstructed. The patient recovered well and was discharged on day 20 after the operation. Conclusions Ex vivo liver resection and autotransplantation could successfully be applied in treating patient with end-stage hepatic alveolar echinococcosis combined with secondary cavernous transformation of portal vein. Adequate preoperative assessment and management of the first hepatic hilum are key to this operation.