肝移植术后肝窦阻塞综合征的单中心诊疗经验

Diagnosis and treatment of hepatic sinusoidal obstruction syndrome after liver transplantation:a single-center experience

目的总结肝移植术后肝窦阻塞综合征(HSOS)的发病情况和诊疗经验。方法回顾性分析4例肝移植术后HSOS患者的临床资料。收集肝移植术后HSOS的发病情况、临床表现、影像学及病理学特点,分析HSOS患者的治疗方式及转归情况。结果肝移植术后HSOS的发生率为0.8%(2/239),中位发病时间为肝移植术后4.5(1.7,9.0)个月。HSOS的临床表现主要包括腹胀、腹腔积液、肝肿大、胆红素升高,部分伴有肾功能不全。4例HSOS患者的腹部增强CT均呈“花斑样”不均匀强化;肝组织病理学表现主要为肝窦扩张伴淤血。4例患者均给予调整免疫抑制剂,将他克莫司(Tac)转换为环孢素,并加用华法林抗凝治疗,其中1例患者接受经颈静脉肝内门体静脉分流术(TIPS)治疗。3例患者经治疗后症状完全缓解,1例患者治疗无效死亡,1例患者在治疗好转后因肺部感染和消化道出血死亡。结论HSOS是肝移植术后少见但致命的并发症,及时的诊断和治疗可以避免移植物衰竭,改善患者预后。

Objective To summarize the incidence,diagnostic and therapeutic experience of hepatic sinusoidal obstruction syndrome(HSOS)after liver transplantation.Methods Clinical data of 4 patients with HSOS after liver transplantation were retrospectively analyzed.The incidence,clinical manifestations,imaging and pathological characteristics of HSOS after liver transplantation were collected,and the treatment methods and clinical outcomes of patients with HSOS were analyzed.Results The incidence of HSOS after liver transplantation was 0.8%(2/239),and the median time of onset was 4.5(1.7,9.0)months after liver transplantation.The clinical manifestations of HSOS mainly included abdominal distension,ascites,hepatomegaly,increased bilirubin,and renal insufficiency in partial cases.Enhanced abdominal CT scan of 4 patients with HSOS showed uneven spot-like enhancement and the liver histopathological examination mainly showed the signs of hepatic sinusoidal dilatation complicated with congestion.Four patients were administered with an adjusted regime of immunosuppressant by replacing tacrolimus(Tac)with ciclosporin and adding anticoagulant therapy with warfarin.One patient received transjugular intrahepatic portosystemic shunt(TIPS).After treatment,the symptoms of 3 patients were completely relieved,and 1 patient died.One of the 3 surviving patients died from pulmonary infection and gastrointestinal bleeding.Conclusions HSOS is a rare and fatal complication after liver transplantation.Timely diagnosis and treatment can avoid the incidence of graft failure and improve clinical prognosis of the patients.