Obstruction of the Hepatic Veins (Chiari??s Disease)

Armstrong, Charles D.; Carnes, William H.

doi:10.1097/00000441-194410000-00005

Cited by 25 publications

(5 citation statements)

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“…At Stanford University, hepatic vein thrombosis was found in 5 of 11,979 consecutive autopsies (0.042%). 70 Other figures reported in the United States vary from 0.005% 71 to 0.05% among consecutive autopsies. 72 In Japan, the incidence of MOVC was 0.15% among 4,523 patients admitted to Kanazawa University Hospital.…”

Section: Epidemiological Aspectsmentioning

confidence: 98%

Proposal of A New Nomenclature for Budd–Chiari Syndrome: Hepatic Vein Thrombosis Versus Thrombosis of the Inferior Vena Cava At Its Hepatic Portion

Okuda

Kage

Shrestha³

1998

Hepatology

185

150

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Budd-Chiari syndrome (BCS) was initially defined as a symptomatic occlusion of the hepatic veins, but subsequent reports on various obliterative changes that occur in the hepatic portion of the inferior vena cava (IVC) and hepatic vein orifices have resulted in a broadened and ambiguous definition. Membranous obstruction of the inferior vena cava has been regarded by many as a congenital vascular malformation, but its relation to the classical BCS has remained obscure. With modern imaging and recent histological study of new cases, membranous obstruction of the IVC is now considered to be a sequela to thrombosis. How to classify various forms of occlusion and stenosis of the IVC and hepatic vein ostia is a major challenge. In this review, we emphasize that primary hepatic vein thrombosis (classical Budd-Chiari) and an obliterative disease predominantly affecting the hepatic portion of the IVC, both of which account for most patients with venous outflow block, are clinically quite different. In the West, the former is more common than the latter, which constitutes the vast majority of cases of outflow block in developing countries such as Nepal, South Africa, China, and India. 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. Markedly enlarged subcutaneous veins over the body trunk characterize the latter. We propose that these two disorders be clinically distinguished with a suggested term ''obliterative hepatocavopathy'' for the latter against classical BCS. (HEPATOLOGY 1998;28:1191-1198.)

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Section: Epidemiological Aspectsmentioning

confidence: 98%

Proposal of A New Nomenclature for Budd–Chiari Syndrome: Hepatic Vein Thrombosis Versus Thrombosis of the Inferior Vena Cava At Its Hepatic Portion

Okuda

Kage

Shrestha³

1998

Hepatology

185

150

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“…When Parker 7 analyzed autopsy records in London in 1959, there were 18 symptomatic cases of BCS among 29,920 consecutive necropsies (0.061%). At Stanford University, 17 hepatic vein thrombosis was found in 5 of 11,979 consecutive autopsies (0.042%). In Japan, the incidence of MOVC was 0.15% among 4,523 patients admitted to Kanazawa University Hospital.…”

Section: Membranous Obstruction Of the Ivcmentioning

confidence: 99%

Inferior Vena Cava Thrombosis at Its Hepatic Portion (Obliterative Hepatocavopathy)

Okuda¹

2002

Semin Liver Dis

107

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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.

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“…Based on autopsy findings, the estimated incidence varies between 0.005% and 0.061% in Western countries [1][2][3]. Thrombosis of the hepatic veins was first reported by Budd and later confirmed by Chiari [4,5].…”

Section: Introductionmentioning

confidence: 99%

Ultrasonography in patients with Budd–Chiari syndrome – Diagnostic signs and prognostic implications

Boozari¹,

Bahr²,

Kubicka³

et al. 2008

Journal of Hepatology

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Obstruction of the Hepatic Veins (Chiari??s Disease)

Cited by 25 publications

References 0 publications

Proposal of A New Nomenclature for Budd–Chiari Syndrome: Hepatic Vein Thrombosis Versus Thrombosis of the Inferior Vena Cava At Its Hepatic Portion

Proposal of A New Nomenclature for Budd–Chiari Syndrome: Hepatic Vein Thrombosis Versus Thrombosis of the Inferior Vena Cava At Its Hepatic Portion

Inferior Vena Cava Thrombosis at Its Hepatic Portion (Obliterative Hepatocavopathy)

Ultrasonography in patients with Budd–Chiari syndrome – Diagnostic signs and prognostic implications

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