Epistaxis in Rendu-Osler-Weber disease treated with selective embolization--case report
Hereditary hemorrhagic telangiectasia (HHT), known as well as Osler-Weber-Rendu syndrome (ORW disease) is autosomal dominant inheritance disease with the worldwide prevalence of 1 case per 5000-10000 population. The pathophysiology of the disease consists of disorders in the growth and migration of endothelial cells, which leads to telangiectasias and arterio-venous malformations (AVM) development. Vascular abnormalities can form in various organs. The most frequent locations are nose and mouth mucous membranes, as well as the rest of GI tract, skin, lungs, urinary system and central nervous system. The most common symptom is reccurent epistaxis (80-90% of patients). Advanced stage disease can result in extensive bleeding with dicrease in hemoglobin levels. Unfortunately, the only available treatment options for Osler-Weber-Rendu syndrom fight the symptoms, not the essential cause, and because of the rarity of the disease there are no guidelines for effective therapy. We are presenting a case of a patient suffering from recurrent episodes of nose bleeding due to hereditary hemorrhagic telangiectasia, who was successfully treated using low-invasive, intravascular arterial embolisation in interventional radiology department.