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OA

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.