3.4 Rendu-Osler-Weber disease

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This is a congenital familial disease (autosomal dominant with a prevalence of 1 to 2 in 10,000), in which arterio-venous shunts develop in the liver. This can cause local ischemia in the liver, mainly in the bile ducts and gallbladder. The large AV shunts in the liver can also lead to a 'high output failure' of the heart.

The disease is characterized by frequent nosebleeds and the characteristic vascular abnormalities of the lips, nose and fingertips.

In case of liver-biliary ischemia and/or to prevent heart failure, a liver transplant is indicated.

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