Most patients with hereditary hemorrhagic telangiectasia (HHT) have nosebleeds, but otolaryngologists do not always treat these patients appropriately. HHT is understood to be a cause of nosebleeds, but few otorhinolaryngologists consider this disease during urgent treatment of epistaxis. Therefore, I will describe several tips for otolaryngologists to identify HHT and the essentials of dealing with nosebleeds.
HHT is often overlooked due to a lack of understanding of the diagnostic criteria, a lack of history taking, and inconsistent acquisition of endoscopic findings. The four Curaçao criteria are used for diagnosis. In patients with epistaxis, if two criteria in addition to epistaxis are satisfied, diagnosis of HHT is confirmed. A patient with recurrent epistaxis in whom vasodilatation is found via endoscopy fulfills two criteria, which meets the requirement for a suspected case. If a patient has a family history of nosebleeds or a history of pulmonary vascular embolization or cerebral infarction, HHT diagnosis is definite because three criteria are present. Thus, diagnosis of HHT is straightforward. Macroscopic vascular lesions vary widely and change, so if the lesions are mild in patients with severe nosebleeds, it is better to observe the lesions at different times.
Because most bleeding occurs from the anterior nasal mucosa, the thumb should be pressed against the alar for several minutes. If the bleeding does not stop, the otolaryngologist may stop the flow of blood by inserting calcium alginate loosely until the blood does not seep. At present, there is no definitive treatment for nosebleeds, so the aim of treatment is to prevent bleeding and alleviate symptoms. For this reason, strengthening the blood vessel wall and its surrounding connective tissue and reducing stimulation is recommended. The recommended first-line treatment is application of Vaseline to nostrils, timolol nose drops, oral tranexamic acid, and oral selective estrogen receptor modulators (SERMs) for peri- and postmenopausal women.
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