儿童复杂肝肿瘤离体肝切除联合自体肝移植术：1例报告及8年随访

Ex situ liver resection with autotransplantation for a complex hepatic tumor in a child: a case report and 8-year follow-up

摘要:

背景与目的 侵犯主肝静脉汇入下腔静脉处及肝后段下腔静脉的复杂肝肿瘤，常因解剖位置深在及血管重建困难而难以行常规原位切除。离体肝切除联合自体肝移植术（ELRA）为此类患者提供了根治性切除机会，但儿童病例报道极少。本文总结1例儿童ELRA治疗复杂肝肿瘤的诊治过程，探讨其技术可行性、适应证及远期并发症。方法 回顾性分析中国人民解放军火箭军特色医学中心收治的1例接受ELRA治疗的儿童患者临床资料，总结术前评估、手术过程、术后恢复及8年随访结果。结果 患儿为6岁女性；术前影像学检查提示肝脏巨大占位性病变，肿瘤累及第一、二肝门及肝后下腔静脉，常规手术切除困难。经充分评估后行ELRA。术中采用同种异体髂总静脉血管移植物替代重建肝后下腔静脉，并采用“Y”形血管重建方式恢复肝静脉流出道。手术无肝期300 min，冷缺血时间260 min。术后病理检查证实为肝炎性肌纤维母细胞瘤。术后发生肝创面胆汁漏，经保守治疗后愈合。随访期间患儿肝功能逐渐恢复，肝功能Child-Pugh分级维持A级；术后9个月出现食管静脉曲张及脾大，术后12个月出现区域性门静脉海绵样变。术后8年，患儿无肿瘤复发，肝功能正常，门静脉高压相关表现保持稳定。结论 ELRA可作为部分儿童复杂肝肿瘤的根治性治疗选择，但其技术复杂、适应证严格，远期可能发生流出道障碍及门静脉高压相关并发症。该术式应在具备成熟肝移植经验的中心，由多学科团队严格筛选病例并进行长期随访。

Abstract:

Background and Aims Complex hepatic tumors involving the confluence of the major hepatic veins and the retrohepatic inferior vena cava are extremely challenging to treat with conventional in situ resection. Ex situ liver resection with autotransplantation (ELRA) provides a potential curative option for selected patients; however, its application in children remains rarely reported. This study reports a pediatric ELRA case and discusses its technical feasibility, indications, and long-term complications.Methods The clinical data of a child who underwent ELRA at the PLA Rocket Force Characteristic Medical Center were retrospectively analyzed. Preoperative evaluation, surgical procedures, postoperative recovery, and 8-year follow-up outcomes were reviewed.Results A 6-year-old girl presented with a large hepatic mass. Preoperative imaging revealed tumor involvement of the first and second hepatic hila and the retrohepatic inferior vena cava, making conventional resection technically difficult. After multidisciplinary evaluation, ELRA was performed. The retrohepatic inferior vena cava was reconstructed using an allogeneic common iliac vein graft, and a Y-shaped vascular reconstruction was performed to restore hepatic venous outflow. The anhepatic phase and cold ischemia time were 300 min and 260 min, respectively. Postoperative pathological examination confirmed the diagnosis of inflammatory myofibroblastic tumor of the liver. A postoperative biliary fistula occurred and was successfully managed conservatively. During follow-up, liver function gradually recovered, with Child-Pugh class A maintained. Esophageal varices and splenomegaly developed at 9 months after surgery, followed by regional portal cavernous transformation at 12 months. After 8 years of follow-up, no tumor recurrence was observed, liver function remained normal, and portal hypertension-related manifestations remained stable.Conclusion ELRA is technically feasible for highly selected pediatric patients with complex hepatic tumors. Nevertheless, strict indications, multidisciplinary expertise, and lifelong surveillance are essential because of the risk of long-term outflow obstruction and portal hypertension-related complications.