Clinical diagnosis of the Budd-Chiari syndrome. A report of six cases

Six patients with the Budd-Chiari syndrome were investigated. Hepatic venography in five showed narrow or occluded hepatic veins and a distinctive pattern in adjacent vessels. In one, patent branches of a left hepatic vein were outlined after advancing a catheter through a partial occlusion of the ostium. In the same patient an injection of contrast material in the parenchyma of the right lobe demonstrated complete obstruction at the ostia of two hepatic veins. In five patients the engorged liver caused side to side narrowing of the cava. Hepatic arteriograms and scintillation scans showed abnormalities which seem to be caused by the effects of hepatic venous occlusion. Polycythaemia vera was the cause of thrombosis of the hepatic veins in three cases and no aetiology was established in the others. Autopsy in three patients confirmed the anatomic and aetiologic findings during life. Five patients responded to a modern diuretic regimen and polycythaemia vera was successfully treated in three. Exploratory surgery is not advised in the Budd-Chiari syndrome. Special roentgenologic procedures are the only means of making an accurate diagnosis.