Rav-Acha M, Gur C, Ilan Y, Verstandig A, Eid A
Department of Internal Medicine A, Hadassah-Hebrew University Medical Center, Jerusalem, Israel.
Harefuah. 2004 May;143(5):372-6, 389.
Budd-Chiari Syndrome (BCS) refers to hepatic venous outflow obstruction, resulting in simultaneous occurrence of hepatic congestion and portal hypertension, leading to a typical clinical triad consisting of right upper quadrant pain, hepatomegaly and ascites. Contrary to Asia and Africa, where BCS is caused primarily by an obstructing membranous web, BCS in the western world is considered a thrombotic complication of an underlying hypercoagulable state. Recognition of the contribution of hypercoagulability as a causative factor in BCS, has led to acknowledgement of the importance of anti-coagulant therapy in BCS. Indeed, a conservative approach consisting of diuretics and anti-coagulant therapy is considered an appropriate treatment strategy for the BCS patient, in the absence of significant hepatic insult. However, once disease progression is noted, based on clinical symptoms, hepatic laboratory disturbance or histological evidence of irreversible hepatic damage, a definite invasive treatment should be applied. The specific procedure to be used is dependent upon the extent of hepatic insult and the anatomical characteristics of the venous obstruction in any individual patient. In the absence of significant hepatic damage, one may employ surgical shunting or invasive roentgenic measures, such as TransJugular Intrahepatic Porto-Systemic Shunt procedure, for the decompression of the portal system. Alternatively, in cases of a single localized obstruction, one may use balloon angioplasty with stent insertion. In contrast, upon evidence of significant hepatic damage, liver transplantation becomes necessary. To date, numerous studies report excellent results regarding the success of liver transplantation for patients with advanced BCS disease accompanied by significant hepatic damage.
布加综合征（BCS）是指肝静脉流出道梗阻，导致肝淤血和门静脉高压同时出现，进而引发由右上腹疼痛、肝肿大和腹水组成的典型临床三联征。与亚洲和非洲不同，在亚洲和非洲BCS主要由阻塞性膜状蹼引起，而在西方世界，BCS被认为是潜在高凝状态的血栓形成并发症。认识到高凝状态作为BCS致病因素的作用，使得人们承认抗凝治疗在BCS中的重要性。事实上，在没有严重肝损伤的情况下，由利尿剂和抗凝治疗组成的保守方法被认为是BCS患者的合适治疗策略。然而，一旦根据临床症状、肝脏实验室指标异常或不可逆肝损伤的组织学证据发现疾病进展，就应采取明确的侵入性治疗。具体采用的手术方法取决于任何个体患者的肝损伤程度和静脉阻塞的解剖特征。在没有严重肝损伤的情况下，可以采用手术分流或侵入性放射学措施，如经颈静脉肝内门体分流术，来减轻门静脉系统压力。或者，对于单一局限性阻塞的病例，可以采用球囊血管成形术并置入支架。相反，一旦有明显肝损伤的证据，肝移植就成为必要。迄今为止，许多研究报告了晚期BCS疾病伴有严重肝损伤患者肝移植成功的优异结果。
