BACKGROUND: Hereditary hemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber syndrome, is an autosomal dominant disease that leads to multiregional angiodysplasia. The presence of telangiectasias in nasal mucosa leads to recurrent epistaxis that affects up to 96% of patients but with unpredictable severity. Some authors have previously explained that endonasal morphology and distribution of telangiectasias can be variable too. The purpose of this study was to evaluate any possible relationship between the severity of epistaxis and the different morphology and distribution of nasal telangiectasias in HHT patients. METHODS: A review was performed of nasal endoscopy records of 76 consecutive HHT patients treated for epistaxis between 2003 and 2007 at our institution. An evaluation was performed of severity of epistaxis in the same patient group using a questionnaire and considering frequency, intensity, duration of nosebleeds, and need for blood transfusions. Comparison of data collected on morphology and distribution of nasal telangiectasias with data collected on severity of epistaxis was performed. RESULTS: Morphology and distribution of nasal telangiectasias showed a statistically significant correlation with frequency and intensity of epistaxis. Presence of telangiectasias endoscopically appearing as large and prominent correlates with higher frequency of epistaxis. An increase in number of nasal subsites involved correlates with higher intensity of nosebleeds. CONCLUSION: Our data suggest that to reduce frequency and intensity of epistaxis in HHT patients, treatments should be directed also at lesions located in the posterior part of nasal fossae and especially on telangiectasias endoscopically appearing as large and prominent.
BACKGROUND:Hereditary hemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber syndrome, is an autosomal dominant disease that leads to multiregional angiodysplasia. The presence of telangiectasias in nasal mucosa leads to recurrent epistaxis that affects up to 96% of patients but with unpredictable severity. Some authors have previously explained that endonasal morphology and distribution of telangiectasias can be variable too. The purpose of this study was to evaluate any possible relationship between the severity of epistaxis and the different morphology and distribution of nasal telangiectasias in HHTpatients. METHODS: A review was performed of nasal endoscopy records of 76 consecutive HHTpatients treated for epistaxis between 2003 and 2007 at our institution. An evaluation was performed of severity of epistaxis in the same patient group using a questionnaire and considering frequency, intensity, duration of nosebleeds, and need for blood transfusions. Comparison of data collected on morphology and distribution of nasal telangiectasias with data collected on severity of epistaxis was performed. RESULTS: Morphology and distribution of nasal telangiectasias showed a statistically significant correlation with frequency and intensity of epistaxis. Presence of telangiectasias endoscopically appearing as large and prominent correlates with higher frequency of epistaxis. An increase in number of nasal subsites involved correlates with higher intensity of nosebleeds. CONCLUSION: Our data suggest that to reduce frequency and intensity of epistaxis in HHTpatients, treatments should be directed also at lesions located in the posterior part of nasal fossae and especially on telangiectasias endoscopically appearing as large and prominent.
Authors: F Haubner; A Schneider; H Schinke; M Bertlich; B G Weiss; M Canis; F Kashani Journal: Orphanet J Rare Dis Date: 2021-04-17 Impact factor: 4.123