辅助性原位尸肝部分移植治疗Wilson病的疗效初探

Cadaveric auxiliary orthotopic partial liver transplantation: case report

目的:总结我院2002年7月19日施行的辅助性原位尸体肝部分移植治疗Wilson病(肝豆状核变性)病例,探讨该移植技术的可行性和初步临床疗效。方法:通过双灌注快速取肝法获取血型为A型的供肝;在整型台上用CUSA沿肝脏镰状韧带的左侧劈离肝脏,取第Ⅱ、Ⅲ段为重230g的部分移植供肝,仔细结扎断面的管道系统,历时185min。受体女性,22岁,血型A型,因锥体外系症状逐渐加重而具肝移植指征,切除受体病肝左外侧叶(Ⅱ、Ⅲ段),将供肝(Ⅱ、Ⅲ段)原位植入。用5鄄0Prolene缝线分别对供体鄄受体的肝左静脉和门静脉左支行端端连续吻合,均预留“增宽因子”,于受体胃十二指肠动脉水平与供肝的左肝动脉以8鄄0Prolene缝线间断吻合。胆道对合取左肝管鄄空肠Roux鄄en鄄Y式吻合。术中予甲基泼尼松龙静脉注射1000mg,术后采用激素、FK506和骁悉联合免疫抑制治疗。结果:热缺血时间为6min,冷缺血时间15.5h,手术时间6.5h,术中输血1400ml。术后第3天开始进食和离床活动,至今病人已生存16个月,完全恢复正常生活,血清铜和铜蓝蛋白水平恢复正常,移植肝脏体积无明显萎缩,手足震颤明显减轻。结论:辅助性原位尸体部分肝移植是治疗Wilson病的良好方法,同时可避免活体部分肝移植给供体带来的风险。

Objective To explore the feasibility and the outcome of auxiliary orthotopic partial liver transplantation (APOLT) from a cadaveric donor for the treatment of Wilson's disease. Methods The donor liver was rapidly procured from a type A cadaver using the double perfusion technique. At the back-table, the liver was split by CUSA along the falciform ligament. Segmeat II and III weighing 230g were acquired with the tubular system carefully ligated. The duration was 185 min. The recipient was a 22-year-old girl with consistent severe symptoms of Wilson's disease. The left lateral lobe of the recipent was removed, and the donor left lateral lobe (230g) was orthotopically grafted in place. The donor's left hepatic vein and left branch was end-to-end anastomosed to the recipient's left portal vein by 5-0 Prolene. Arterial anastomosis was made between the recipient's gastro-duodenal artery and left heptatic artery with interrupted 8-0 Prolene. Biliary duct reconstruction was made by Roux-en-Y anastomosis of the left hepatic duct and the jejunum. FK506, adrenocortical hormones and MMF were administered after operation. Results The warm ischemic time was 6 min and cold ischemic time 15.5 h. The whole operation on the recipient took 6.5 h; 1 400 ml of blood was transfused during operation. The diet and activity was resumed at the 3rd post-operative day. The patient has now survived 16 months with a normal copper-protein level, mitigated extrapyramidal symptoms and a larger grafted liver. Conclusions APOLT is a feasible procedure for late-stage Wilson's disease and possess the advantage of not running the risk for a living donor.