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
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.