LIVER-TRANSPLANTATION FOR HEPATIC ARTERIOVENOUS MALFORMATION IN HEREDITARY HEMORRHAGIC TELANGIECTASIA

Symptomatic hepatic involvement is a rare complication of hereditary h emorrhagic telangiectasia and most commonly consists of fibrosis or ci rrhosis. We describe a 33-year-old woman in whom multiple hepatic arte riovenous malformations led to high output cardiac failure and liver f ailure due to biliary necrosis with refractory biliary sepsis, requiri ng orthotopic liver transplantation. Hepatic arteriovenous malformatio ns were the first manifestation of the disease and a similar asymptoma tic hepatic tumour was subsequently detected in her 60-year-old father who also had the classical cutaneous stigmata of the syndrome, Unreco gnised genetic factors may determine the clinical spectrum of heredita ry haemorrhagic telangiectasia including the hepatic manifestations.