Familial occurrence of membranous obstruction of the inferior vena cava: arguments in favor of a congenital etiology

Membranous obstruction of the inferior vena cava is a rare disease. The etiology of the membrane is believed to be thrombotic or congenital. In three of 11 siblings from a single family, symptoms of membranous obstruction of the inferior vena cava developed during early adult life. All had signs of...

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Bibliographic Details
Published in:Journal of hepatology 1995-04, Vol.22 (4), p.404-409
Main Authors: Riemens, Stephen C., Haagsma, Elizabeth B., Kok, Theo, Gouw, Annette S.H., van der Jagt, Eric J.
Format: Article
Language:English
Subjects:
Adolescent
Adult
Aged
Angiography
Angiography, Digital Subtraction
Biological and medical sciences
Budd-Chiari
Budd-Chiari Syndrome - genetics
Cardiology. Vascular system
Child
Cirrhosis
Congenital heart diseases. Malformations of the aorta, pulmonary vessels and vena cava
Familial occurrence
Female
Genetic Testing
Heart
Humans
Male
Medical sciences
Membranous obstruction inferior vena cava
Middle Aged
Phlebography
Vascular Diseases - congenital
Vascular Diseases - diagnostic imaging
Vascular Diseases - genetics
Vena Cava, Inferior - diagnostic imaging
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Summary:Membranous obstruction of the inferior vena cava is a rare disease. The etiology of the membrane is believed to be thrombotic or congenital. In three of 11 siblings from a single family, symptoms of membranous obstruction of the inferior vena cava developed during early adult life. All had signs of more long-standing disease, as judged by the presence of collaterals, cirrhosis and, in one case, hepatocellular carcinoma. On family screening no further cases of membranous obstruction of the inferior vena cava were found. There was also no evidence of inherited defects in the natural coagulation inhibitors (protein C, protein S and antithrombin III) and plasminogen deficiency. This familial occurrence of membranous obstruction of the inferior vena cava supports a congenital etiology, although a thrombotic etiology cannot be totally excluded.
ISSN:0168-8278
1600-0641
DOI:10.1016/0168-8278(95)80102-2