Grus Tomáš, Lambert Lukáš, Grusová Gabriela, Banerjee Rohan, Burgetová Andrea
2nd Department of Surgery - Department of Cardiovascular Surgery, First Faculty of Medicine, Charles University and General University Hospital in Prague, Prague, Czech Republic.
Department of Radiology, First Faculty of Medicine, Charles University and General University Hospital in Prague, Prague, Czech Republic.
Prague Med Rep. 2017;118(2-3):69-80. doi: 10.14712/23362936.2017.6.
Budd-Chiari syndrome (BCS) is a rare disease with an incidence of 0.1 to 10 per million inhabitants a year caused by impaired venous outflow from the liver mostly at the level of hepatic veins and inferior vena cava. Etiological factors include hypercoagulable conditions, myeloprolipherative diseases, anatomical variability of the inferior vena cava, and environmental conditions. Survival rates in treated patients range from 42 to 100% depending on the etiology and the presence of risk factors including parameters of Child-Pugh score, sodium and creatinine plasma levels, and the choice of treatment. Without treatment, 90% of patients die within 3 years, mostly due to complications of liver cirrhosis. BCS can be classified according to etiology (primary, secondary), clinical course (acute, chronic, acute or chronic lesion), and morphology (truncal, radicular, and venooclusive type). The diagnosis is established by demonstrating obstruction of the venous outflow and structural changes of the liver, portal venous system, or a secondary pathology by ultrasound, computed tomography, or magnetic resonance. Laboratory and hematological tests are an integral part of the comprehensive workup and are invaluable in recognizing hematological and coagulation disorders that may be identified in up to 75% of patients with BCS. The recommended therapeutic approach to BCS is based on a stepwise algorithm beginning with medical treatment (a consensus of expert opinion recommends anticoagulation in all patients), endovascular treatment to restore vessel patency (angioplasty, stenting, and local thrombolysis), placement of transjugular portosystemic shunt (TIPS), and orthotopic liver transplantation as a last resort rescue treatment.
布加综合征（BCS）是一种罕见疾病，每年发病率为百万分之0.1至10，主要由肝脏静脉流出道受阻引起，多发生于肝静脉和下腔静脉水平。病因包括高凝状态、骨髓增殖性疾病、下腔静脉解剖变异以及环境因素。根据病因和危险因素的存在情况，包括Child-Pugh评分参数、血钠和肌酐水平以及治疗选择，接受治疗患者的生存率在42%至100%之间。未经治疗，90%的患者会在3年内死亡，主要死于肝硬化并发症。BCS可根据病因（原发性、继发性）、临床病程（急性、慢性、急性或慢性病变）和形态学（主干型、分支型和静脉闭塞型）进行分类。通过超声、计算机断层扫描或磁共振成像显示静脉流出道梗阻以及肝脏、门静脉系统的结构改变或继发性病变来确诊。实验室和血液学检查是综合检查的重要组成部分，对于识别血液学和凝血障碍非常重要，在高达75%的BCS患者中可能会发现这些障碍。推荐的BCS治疗方法基于逐步算法，首先是药物治疗（专家意见共识建议对所有患者进行抗凝），然后是恢复血管通畅的血管内治疗（血管成形术、支架置入和局部溶栓），放置经颈静脉肝内门体分流术（TIPS），最后是作为挽救治疗的原位肝移植。
