Okuda K
Department of Medicine, Chiba University School of Medicine, Chiba, Japan.
J Gastroenterol Hepatol. 2001 Nov;16(11):1179-83. doi: 10.1046/j.1440-1746.2001.02577.x.
Confusion prevails throughout the world regarding the definition and classification of the Budd-Chiari syndrome. The original patients (Budd and Chiari) described had hepatic vein thrombosis, but this syndrome now encompasses various hepatic venous outflow blocks, of which membranous obstruction of the inferior vena cava (IVC) is the most common. This author has been suggesting that the classical Budd-Chiari syndrome or hepatic vein thrombosis and membranous obstruction of IVC or primary thrombosis of IVC at its hepatic portion are epidemiologically, pathologically and clinically different, and that they should be treated as two clinical entities that are not to be mixed. The two diseases have a different onset, different clinical manifestations and a different natural history. Whereas hepatic vein thrombosis is a severe disease with an acute onset, IVC thrombosis presents mildly at onset, but it recurs and eventually turns into a fibrous occlusion of IVC of varying thickness or stenosis of a various degree. The fibrous IVC occlusion is found as a mysterious thin membrane, but is more often much thicker than a membrane, and therefore 'membrane' is a misnomer. Although the genesis is not established, formation of a thin membrane may be an outcome of recurrent thrombosis. The past congenital vascular malformation theory no longer holds, because the disease occurs mostly in adulthood, and transformation of thrombosis into a membrane has now been well documented pathologically as well as clinically. This author suggests that a term 'obliterative hepatocavopathy' replace membranous obstruction of IVC, the term 'Budd-Chiari syndrome' be abandoned, and that primary hepatic venous outflow block be divided into primary hepatic vein thrombosis and primary IVC thrombosis (obliterative hepatocavopathy).
布-加综合征的定义和分类在全球范围内都存在混淆。最初描述的患者（布德和加综合征）患有肝静脉血栓形成，但现在该综合征涵盖了各种肝静脉流出道阻塞，其中下腔静脉膜性梗阻最为常见。本文作者一直认为，经典的布-加综合征即肝静脉血栓形成与下腔静脉膜性梗阻或肝段下腔静脉原发性血栓形成在流行病学、病理学和临床方面均有所不同，应将它们视为两种不应混淆的临床实体。这两种疾病的起病、临床表现及自然病程均不同。肝静脉血栓形成是一种起病急骤的严重疾病，而下腔静脉血栓形成起病时症状较轻，但会复发并最终发展为不同厚度的下腔静脉纤维性闭塞或不同程度的狭窄。纤维性下腔静脉闭塞表现为一层神秘的薄膜，但通常比薄膜厚得多，因此“膜”这种说法并不准确。虽然其发病机制尚未明确，但薄膜的形成可能是反复血栓形成的结果。过去的先天性血管畸形理论已不再成立，因为该疾病大多发生在成年期，而且血栓转变为膜的过程在病理及临床方面均已得到充分证实。本文作者建议用“闭塞性肝腔病变”取代下腔静脉膜性梗阻，摒弃“布-加综合征”这一术语，并将原发性肝静脉流出道阻塞分为原发性肝静脉血栓形成和原发性下腔静脉血栓形成（闭塞性肝腔病变）。
