MONSTROUS ASCITES IN HEREDITARY HEMORRHAGIC TELANGIECTASIA

Background: Hepatic involvement in hereditary haemorrhagic telangiecta sia (HHT) consisting of fibrosis, telangiectases, and cirrhosis, has b een reported as a relatively frequent finding. Case: A 50-year-old man with HHT presented with monstrous ascites. Liver biopsy demonstrated multiple dilated sinusoids but not cirrhosis. There were no findings i ndicative of portal hypertension or malignant disease. Portal pressure , recorded in hepatic vein wedge position, was normal. Arteriography s howed numerous hypervascular lesions throughout the liver. The clinica l course has been stable for more than 2 years. Conclusion: No other r eason for the monstrous ascites could be found. We thus hypothesize th at this case of monstrous ascites is due to hepatic involvement in HHT , presenting as numerous vascular lesions throughout the liver.