Okuda Kunio
Department of Medicine, Chiba University School of Medicine, 1-8-1 Inohana, Chuo-ku, Chiba, Japan 260-8677.
Semin Liver Dis. 2002 Feb;22(1):15-26. doi: 10.1055/s-2002-23203.
The Budd-Chiari syndrome was primarily described as hepatic vein thrombosis within the liver, but it now includes inferior vena cava (IVC) thrombosis and other conditions that cause hepatic vein outflow obstruction. This author and several others maintain that primary hepatic vein thrombosis and primary IVC thrombosis represent two different clinical disorders. Primary thrombosis of the IVC most commonly occurs in its hepatic portion, which seems to be predisposed to thrombosis and has been called membranous obstruction of IVC, because the thrombus organizes into a fibrous and frequently membranous occlusion of the IVC. The hepatic vein orifices are affected to varying degrees, resulting in congestive liver damage. The cause of IVC thrombosis may be a hypercoagulable state such as coagulation factor deficiency and myeloproliferative disorders, but is more often idiopathic. In Nepal, it is endemic with a suspected association with infections. To consider IVC thrombosis and the congestive liver damage as a disease entity, this author proposes the term obliterative hepatocavopathy, separate from hepatic vein thrombosis. Clinically obliterative hepatocavopathy is less severe in its acute phase compared with hepatic vein thrombosis, but it aggravates occlusion of hepatic vein orifices with recurrent thrombosis. Primary hepatic vein thrombosis and obliterative hepatocavopathy display different hemodynamics of the hepatic veins, IVC, and portal vein; dilatation of the subcutaneous veins in the body trunk is more pronounced in obliterative hepatocavopathy because the ascending lumbar vein becomes the major collateral route. Congestive liver cirrhosis develops after a long clinical course that may be complicated by hepatocellular carcinoma.
布加综合征最初被描述为肝脏内的肝静脉血栓形成，但现在包括下腔静脉血栓形成以及其他导致肝静脉流出道梗阻的情况。本文作者和其他一些人认为，原发性肝静脉血栓形成和原发性下腔静脉血栓形成代表两种不同的临床疾病。下腔静脉原发性血栓形成最常发生在其肝段，该部位似乎易发生血栓形成，并且已被称为下腔静脉膜性梗阻，因为血栓会机化形成下腔静脉的纤维性且常为膜性的闭塞。肝静脉开口受到不同程度的影响，导致充血性肝损伤。下腔静脉血栓形成的原因可能是高凝状态，如凝血因子缺乏和骨髓增殖性疾病，但更常见的是特发性的。在尼泊尔，它呈地方性流行，疑似与感染有关。为了将下腔静脉血栓形成和充血性肝损伤视为一种疾病实体，本文作者提出了闭塞性肝腔病这一术语，以区别于肝静脉血栓形成。临床上，闭塞性肝腔病在急性期比肝静脉血栓形成症状较轻，但它会因反复血栓形成而加重肝静脉开口的闭塞。原发性肝静脉血栓形成和闭塞性肝腔病在肝静脉、下腔静脉和门静脉的血流动力学方面表现不同；在闭塞性肝腔病中，躯干皮下静脉扩张更为明显，因为腰升静脉成为主要的侧支循环途径。充血性肝硬化在漫长的临床病程后发生，可能并发肝细胞癌。
