Intrahepatic dilated bile ducts in osler-weber-rendu syndrome

Osler-Weber-Rendu syndrome, is a rare autosomal dominant disorder (incidence in the general population is 1 – 2/100,000 while the race and gender distribution are homogeneous). It is characterized by abnormal blood vessel formation in the skin, the mucous membranes and often within other organs such as the lungs, liver and brain. Epistaxis is the earliest and most frequent manifestation, followed by telangiectasias in the oral mucosa, tongue, lips, fingers and skin.