Cross-sectional Study of the Occurrence of Epistaxis in HHT Patients

Abstract

Hereditary hemorrhagic telangiectasia HHT , also known as Osler-Rendu-Weber disease, is an autosomal dominant disorder. Epistaxis is the most common symptom and presents in more than % of the HHT patients. However, the occurrence and frequency of epistaxis in HHT patients are not well understood in Japan.We administered an anonymous questionnaire and obtained information regarding the degree, history, and treatment of epistaxis. Thirty-six valid responders were analyzed, % of whom suffered from epistaxis more than once a week. Epistaxis appeared before the age of years in % of the responders, but the median age of the HHT diagnosis was years. Thirty % of the responders underwent otorhinolaryngological examination. About half of the responders underwent either gauze-packing or coagulation treatment. However, these patients recorded low levels of satisfaction with the otorhinolaryngological examination. About % of the responders preferred to visit an otorhinolaryngologist who was familiar with both the symptoms and diagnosis of HHT. Thus, otorhinolaryngologists are now recommended in Japan for the diagnosis and treatment of HHT.