The Budd-Chiari syndrome is the clinical manifestation of the total or partial obstruction of the hepatic veins and/or inferior vena cava. It is an infrequent cause of portal hypertension. The chronic presentation is the most frequent and is characterized by right upper quadrant pain, hepatomegaly, and ascites of slow onset. We report a case of a 26 year old woman affected by this disorder associated to a recent use of oral contraceptive and a "Lupus Anticoagulant". She subsequently developed deep venous thrombosis and pulmonary embolism. She died almost 6 years after the onset of symptoms.
PIP: A 26-year-old woman sought medical care in April 1983 because she had been experiencing pain in the right upper quadrant for 2 months, as well as an enlarged abdomen and postprandial fullness accompanied by nausea and vomiting. She had used oral contraceptives (OCs) for a period of 11 months up to 4 months before the inception of the symptoms. Examination showed normal vital functions but painful hepatomegaly. Hepatic biopsy showed dilatation of the central vein of the lobe; ultrasound of the liver showed hepatomegaly, the dilatation of hepatic veins, and suprahepatic veins; and echos of the inside were suggestive of thrombosis. The Doppler instrument revealed inversion of the hepatic flow towards the spleen and the presence of multiple collateral veins. Venocavography confirmed almost total obstruction of the inferior vena cava in its retrohepatic trajectory. Percutaneous transhepatic splenoportography demonstrated evidence of slow suprahepatic drainage with obstruction of the contrast medium in the area of the cava. The pressure in the suprahepatic vein was 43 cm of H2O. As the illness progressed, profound venous thrombosis of the left lower extremity developed, which was treated with heparin and managed with fenindione for 4 years. 5 years later, multiple pulmonary thromboembolism was confirmed by pulmonary gammagram of perfusion and digital arteriography. She received medical treatment based on low sodium and diuretic diet. Her hepatic function progressively deteriorated with increased ascites and collateral venous network. She died in December 1988.