[Budd-Chiari syndrome. Description of 2 cases]

The causes of Budd-Chiari syndrome are quickly reviewed with reference to the pathology described in series from various parts of the world. In the West the syndrome is caused by obstruction of the Inferior Vena Cava in its retrohepatic course with or without concomitant obstruction of the suprahepatic veins attributable to fibrous regression of the vessel wall and subsequent endoluminal thrombosis. In the Far East and South Africa the syndrome is caused by the presence of a thin fibrous septum obstructing the vein immediately upstream of the right atrium. Of the two cases of Budd-Chiari syndrome described, one presented high grade stenosis of the Vena Cava caused by fibrous regression of the vessel wall and the other a fibrous septum situated transversally to the lumen. The description of the first case is particularly interesting because before the clinical picture was correctly understood, a succession of major complications induced incorrect diagnosis and treatment. In contrast the second was a classic case of rapid diagnosis and successful treatment. The discussion of the two cases highlights the primary role currently played by abdominal echography with Doppler ultrasound, examination with lower cavography using a contrast medium in documenting a caval flow pathology of this kind. CAT scanning, arteriography of the Celiac tripod and closed hepatic needle biopsy appear to be much less decisive.