Hereditary hemorrhagic telangiectasia: a new surgical approach.

Hereditary Hemorrhagic telangiectasia (HHT), or Rendu-Osler-Weber disease, is a vascular anomaly characterized by multiple dilations of skin and mucosa capillaries and venules. It is an autosomal dominant disease, equally distributed between both genders and its incidence is of 1-2/100.000 inhabitants.1 Bleeding may occur in numerous places; however, epistaxis is the most common, present in 90% of the cases. The basic lesion is found on the vessels’ walls, with defects in the elastic and muscular layers that make them more prone to bleeding. Many treatment modalities have been used to control epistaxis; none of them have rendered entirely satisfactory result4. Options are nasal packing, hormone-therapy, vascular embolizations, fascioplasty and septodermoplasty. Young’s surgery is based on nasal occlusion, thus preventing the friction between air and the telangiectasia, precluding epistaxis episodes.