肝移植新术式——双多米诺供肝交叉辅助式肝移植治疗代谢性肝脏疾病

Conceptual innovation： cross-auxiliary double domino donor liver transplantation

目的总结应用肝移植新术式——双多米诺供肝交叉辅助式肝移植治疗家族性淀粉样变患者的经验及效果。方法双多米诺供肝受者为家族性淀粉样变患者。多米诺供者1为肝豆状核变性患者，多米诺供者2为鸟氨酸氨甲酰基转移酶缺乏（OTCD）患者，2例多米诺供肝患儿均接受亲属活体肝移植（左外叶供肝），切取的病肝用于双多米诺供肝肝移植。交叉辅助式双多米诺供肝肝移植的手术分为2期进行：1期手术包括多米诺肝移植受者病肝部分切除、多米诺供者1供肝切取及后台流出道重建、辅助式肝移植1期；2期手术包括多米诺供肝2的切取及血管重建，多米诺肝移植受者残余肝脏的切除、辅助式肝移植2期。结果1期术后多米诺肝移植受者肝功能恢复正常且持续稳定，血铜、尿铜水平均正常；2期术后上述指标及血氨水平正常。腹部B型超声检查提示双多米诺供肝形态及血流未见异常。结论作为肝移植概念的创新，在同一受者体内，2个不同的非硬化性肝脏代谢性疾病多米诺供肝可以互相代偿其代谢功能障碍，在克服外科技术难题获得手术成功的同时，受者可获得理想的肝脏功能和生活质量。

Objective To summarize the experience and effect of the new liver transplantation operation for FAP-Cross-Auxiliary Double Domino Donor liver Transplantation. Method The recipient of the double domino donor liver transplantation is a female, diagnosed of familial amyloid polyneuropathy （FAP）; domino liver donor-1 was diagnosed of Wilson＇s disease and domino liver donor-2 was diagnosed of Ornithine carbamyl acyltransferase deficiency （OTCD） The two domino liver donors received living donor liver transplantation from their parents （lateral left lobe graft）. Cross- Auxiliary Double Domino Donor liver Transplantation was divided into 2 stages： Right lobe resection for the FAP recipient, domino liver-1 resection, Back Table preparation of outflow tract and domino liver transplantation were completed in stage-1; domino liver-2 resection, vascular preparation and transplantation were done in stage-2. Result The recipient obtain normal liver function, ceruloplasmin oxidase activity, blood copper and urine copper level after stage-l; and also with normal blood ammonia after stage-2. Abdominal Ultrasound showed that the blood flow of artery, portal vein and hepatic vein were all normal. Conclusion As a conceptual change in a liver transplant, two domino donors from different liver-based metabolic disorder patients can implement a metabolic function complementary each other in the same recipient. The recipient can obtain ideal liver function and quality of life by overcoming the surgical technical problems.