Clinical History
epigastric pain and weight loss. Physical examination showed a distended abdomen. Laboratory tests revealed a total red blood cell mass of 67ml/kg and abnormal liver tests. Iliac crest biopsy was performed and was suggestive for polycythemia vera. On ultrasonography ascites and hepatomegaly were demonstrated. Subsequently contrast enhanced CT scan of the abdomen, Doppler ultrasonography, and hepatic angiography were carried out.
Imaging Findings
A 62-year-old woman consulted with epigastric pain and weight loss. Physical examination showed a distended abdomen. Laboratory tests revealed a total red blood cell mass of 67ml/kg and abnormal liver tests. Iliac crest biopsy was performed and was suggestive for polycythemia vera. On ultrasonography ascites and hepatomegaly were demonstrated. Subsequently contrast enhanced CT scan of the abdomen, Doppler ultrasonography, and hepatic angiography were carried out.
Clinical data and imaging findings indicate Budd-Chiari syndrome caused by polycythemia vera.
Discussion
Budd-Chiari syndrome includes a variety of conditions with obstruction of the hepatic venous outflow, at the level of either the large hepatic veins or the suprahepatic segment of the inferior vena cava. Depending on the cause and pathophysiologic manifestations, distinction is made between primary and secondary forms.
In the primary type, there is total or incomplete membranous obstruction of the hepatic veins. Although uncommon in Europe and Northern America, this is the most common cause of Budd-Chiari syndrome in Japan and India. Secondary Budd-Chiari syndrome occurs in the setting of polycythemia vera, paroxysmal nocturnal hemoglobinuria, myeloproliferative disorders, thrombocytosis and Behcet's disease. A variety of hepatic and extrahepatic masses can also compress the inferior vena cava and/or hepatic veins. The clinical abnormalities are hepatomegaly, abdominal pain and ascites. The latter develops abruptly and is accompanied by liver dysfunction. Splenomegaly may arise from persistent portal hypertension and is seen in chronic cases.
Histologically, centrizonal sinusoidal distension and blood pooling in the sinusoids is seen. In chronic cases the affected areas become fibrosed and shrink in size.
Treatment of patients with Budd-Chiari syndrome depends on the condition of the patient, extent of disease and underlying cause. Some patients undergo an initial trial of thrombolytic therapy with streptokinase or urokinase before surgery is considered.
Differential Diagnosis List
