Hereditary Hemorrhagic Telangiectasia (HHT) also known as Rendu-Osler-Weber syndrome is a rare, autosomal dominant multisystemic disease. It is a mucocutaneous and fibrovascular dysplasia, the diagnosis of which is based on the fulfillment of the four Curaçao criteria: 1) Recurrent epistaxis 2) Dermatovascular mucosal telangiectasias at characteristic sites: skin of the face, ears, fingertips, lips, tongue, oral and nasal cavity 3) Arteriovenous malformations of visceral organs and central nervous system 4) Family history: diagnosis of HHT in a first-degree relative. HHT is a progressively evolving disease, characterized by a wide range of symptoms, which show a correlation with age, affected organ, type and extent of the attack. It is a complex disease with a multitude of clinical manifestations that require management and treatment by a multidisciplinary team including otorhinolaryngologists. Despite the rarity of Rendu-Osler-Weber syndrome, otorhinolaryngologists must be aware of the general and ENT manifestations of this syndrome so that they can suspect disease from the initial stages, document the diagnosis with the help of other specialties and treat with particular attention to recurrent epistaxis. Spontaneous recurrent epistaxis due to telangiectasias of the nasal mucosa is the most common, frequent and earliest symptom of Rendu-Osler-Weber syndrome. They first appear at a young age. Almost all patients with HHT will experience recurrent epistaxis at some point in their lives, some more frequently with several episodes per day and others more infrequently with occasional episodes over many years. In general, incidence and severity of epistaxis increase with age. In most patients, treatment goals include decreasing the frequency, volume, and severity of epistaxis and improving quality of life. Less commonly, treatment is pursued to relieve severe anemia or prevent life-threatening epistaxis. No standard treatment algorithm is available; however, a graduated treatment strategy beginning with conservative topical therapies progressing to more invasive surgical treatments is recommended. The purpose of this study is to present management and treatment of ENT manifestations of Rendu-Osler-Weber of HHT.

Eleni Litsou is MD, MSC, PhD, ENT Specialist Assistant Consultant, at University Hospital of Ioannina, Greece. She has 4 master degrees: Neoplastic Disease, Audiology and Neurotology, Thyroid and Parathyroid Gland Surgery, Basic Biomedical Sciences. She has over 75 publications, presentations and posters in national and international conferences and journals. She has been serving as a reviewer of several reputed journals.