肝移植术后肝窦阻塞综合征初步诊疗经验

Preliminary clinical experiences of hepatic sinusoidal obstruction syndrome after orthotopic liver transplantation

目的总结肝移植术后肝窦阻塞综合征(hepatic sinus obstruction syndrome,HSOS)的病因、发病过程、临床表现特征、诊断和治疗的经验。方法回顾性分析陆军军医大学西南医院全军肝胆外科研究所2019年1月至2022年5月收治的3例男性肝移植术后并发HSOS受者的临床资料,分析其基线资料特征、原发疾病、起病经过、临床表现、影像学及病理学变化的特点,总结肝移植术后HSOS受者的诊断要点、治疗方案及疾病转归情况。结果肝移植术后HSOS发病率为1.7%(2/117);中位发病时间为15(13,50)d;临床表现主要有肝区疼痛、肝脏肿大、腹胀、纳差、乏力、黄疸、尿少、腹腔积液和胸腔积液等,部分合并有急性肾功能不全。3例HSOS受者的腹部超声检查结果提示,肝静脉及门静脉血流通畅,但血流减慢,肝实质呈现不均匀回声改变。腹部增强CT检查结果显示,门静脉期和平衡期呈现"花斑样""地图状"不均匀强化的特征性表现。肝穿刺活检表现为肝血窦明显扩张伴淤血,部分肝细胞肿胀、出血、坏死,肝小叶中心静脉管壁明显增厚,出现管腔狭窄或闭塞。3例受者出现HSOS后均调整了免疫抑制剂方案,将他克莫司调整为西罗莫司,并加用低分子肝素或联合利伐沙班抗凝溶栓治疗,同时给予甲泼尼龙调节免疫治疗,给予补充白蛋白、利尿、保肝、补液等对症治疗。2例受者经积极治疗后病情好转,治愈出院。1例受者因消化道出血、急性肾功能衰竭并发多器官功能衰竭而死亡。结论HSOS是肝移植术后非常罕见但致命的严重并发症,早期及时诊断和调整治疗方案可以避免出现肝肾功能衰竭等不良预后,显著改善受者的生存。

Objective To explore the etiology,pathogenesis,clinical features,diagnosis and treatment of hepatic sinus obstruction syndrome(HSOS)after orthotopic liver transplantation(OLT).Methods Clinical data were reviewed for 3 HSOS patients after OLT.Baseline profiles,primary disease,onset,clinical manifestations,abdominal imaging and pathological changes were recorded for summarizing the key points of diagnosis,treatment and outcomes of HSOS after OLT.Results HSOS was an extremely rare complication after OLT with an incidence of 2%(2/117)and a median onset of 15(13-50)days.The major clinical manifestations were hepatic pain,abdominal distension,poor appetite,fatigue,jaundice,oliguria,peritoneal effusion and pleural effusion.Some of them were complicated with acute renal insufficiency.Abdominal ultrasonography revealed that blood stream of hepatic and portal veins was smooth but rather slow and hepatic parenchyma showed uneven echo changes.Abdominal enhanced computed tomography(CT)demonstrated"mosaic"and"map-like"uneven enhancement in portal vein and balance phases.The pathological manifestations of liver biopsy included obvious dilation and congestion of hepatic sinuses,swelling and necrosis of hepatic cells,thickening of hepatic venules and luminal stenosis or occlusion.All of them received immunosuppressants.Tacrolimus was switched to sirolimus,low molecular weight heparin or plus rivaroxaban anticoagulant thrombolytic therapy,methylprednisolone regulatory immunotherapy,albumin supplementation,diuresis,hepatic protection and fluid replacement.Afterward clinical symptoms of 2 patients improved,became cured and discharged.One case died from gastrointestinal hemorrhage and acute renal failure secondary to multiple organ failure.Conclusions HSOS is an extremely rare but severe complication after OLT.Early diagnosis and fine-tuning of treatment protocols can avoid poor prognosis such as liver and kidney failure and significantly improve patient survival.