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. Th e 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 lif e. All had signs of more long-standing disease, as judged by the prese nce of collaterals, cirrhosis and, in one case, hepatocellular carcino ma. On family screening no further cases of membranous obstruction of the inferior vena cava were found. There was also no evidence of inher ited defects in the natural coagulation inhibitors (protein C, protein S and antithrombin III) and plasminogen deficiency, This familial occ urrence of membranous obstruction of the inferior vena cava supports a congenital etiology, although a thrombotic etiology cannot be totally excluded.