Severe Recurrent Epistaxis - The Main Symptom of Hereditary Haemorrhagic Teleangiectasia

Abstract

Hereditary haemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber syndrome, is of dominant autosomal inheritance. Pathologic changes of vascular walls cause recurrent episodes of bleeding from many organ systems. Recurrent epistaxis is the first and the most frequent symptom of HHT. The causal therapy is not known but there are many therapeutic procedures improving the overall condition. We present a case of a 76-year-old man suffering from HHT, frequently hospitalized and treated for massive nose bleeding. In past a selective arterial embolization was performed thrice; nonetheless, the intensity and frequency of epistaxis remained unchanged. Anterior nasal package and electrocoagulation were performed repeatedly as the “first aid” treatment. In the article we also mention other therapeutic modalities for this diagnosis; unfortunately, their efficacy remains inadequate.