PROPOSAL OF A NEW NOMENCLATURE FOR BUDD-CHIARI-SYNDROME - HEPATIC VEIN-THROMBOSIS VERSUS THROMBOSIS OF THE INFERIOR VENA-CAVA AT ITS HEPATIC PORTION

Citation
K. Okuda et al., PROPOSAL OF A NEW NOMENCLATURE FOR BUDD-CHIARI-SYNDROME - HEPATIC VEIN-THROMBOSIS VERSUS THROMBOSIS OF THE INFERIOR VENA-CAVA AT ITS HEPATIC PORTION, Hepatology, 28(5), 1998, pp. 1191-1198
Citations number
105
Language
INGLESE
art.tipo
Review
Categorie Soggetti
Gastroenterology & Hepatology
Journal title
ISSN journal
0270-9139
Volume
28
Issue
5
Year of publication
1998
Pages
1191 - 1198
Database
ISI
SICI code
0270-9139(1998)28:5<1191:POANNF>2.0.ZU;2-4
Abstract
Budd-Chiari syndrome (BCS) was initially defined as a symptomatic occl usion of the hepatic veins, but subsequent reports on various oblitera tive changes that occur in the hepatic portion of the inferior vena ca va (IVC) and hepatic vein orifices have resulted in a broadened and am biguous definition. Membranous obstruction of the inferior vena cava h as been regarded by many as a congenital vascular malformation, but it s relation to the classical BCS has remained obscure. With modern imag ing and recent histological study of new cases, membranous obstruction of the IVC is now considered to be a sequela to thrombosis. How to cl assify various forms of occlusion and stenosis of the IVC and hepatic vein ostia is a major challenge. In this review; we emphasize that pri mary hepatic vein thrombosis (classical Budd-Chiari) and an obliterati ve disease predominantly affecting the hepatic portion of the IVC, bot h of which account for most patients with venous outflow block, are cl inically quite different. In the West, the former is more common than the latter, which constitutes the vast majority of cases of outflow bl ock in developing countries such as Nepal, South Africa, China, and In dia. The latter is frequently complicated by hepatocellular carcinoma (HCC), and primary hepatic vein thrombosis is not. The major cause of thrombosis is a hypercoagulable state in hepatic vein thrombosis, but more of the latter cases are idiopathic. The clinical presentation of the latter is milder, and onset is frequently inapparent, whereas the former is more severe, sometimes causing acute hepatic failure. Marked ly enlarged subcutaneous veins over the body trunk characterize the la tter, We propose that these two disorders be clinically distinguished with a suggested term ''obliterative hepatocavopathy'' for the latter against classical BCS.