Diagnosis and Management of Hereditary Hemorrhagic Telangiectasia

The expert panel recommends that patients with HHT-related epistaxis use moisturizing topical therapies that humidify the nasal mucosa to reduce epistaxis. (S, M, )

The expert panel recommends that clinicians consider the use of oral tranexamic acid for the management of epistaxis that does not respond to moisturizing topical therapies. (S, H, )

The expert panel recommends that clinicians should consider ablative therapies for nasal telangiectasias, including laser treatment, radiofrequency ablation, electrosurgery, and sclerotherapy, in patients that have failed to respond to moisturizing topical therapies. (W, M, )

The expert panel recommends that clinicians consider the use of systemic antiangiogenic agents for the management of epistaxis that has failed to respond to moisturizing topical therapies, ablative therapies, and/or tranexamic acid. (S, M, )

The expert panel recommends that clinicians consider a septodermoplasty for patients whose epistaxis has failed to respond sufficiently to moisturizing topical therapies, ablative therapies, and/or tranexamic acid. (W, L, )

The expert panel recommends that clinicians consider a nasal closure for patients whose epistaxis has failed to respond sufficiently to moisturizing topical therapies, ablative therapies, and/or tranexamic acid. (S, M, )

The expert panel recommends that physicians advise patients with HHT-related epistaxis to use agents that humidify the nasal mucosa to prevent epistaxis. (W, , III)

The expert panel recommends that clinicians refer HHT patients with epistaxis and who desire treatment to otorhinolaryngologists with HHT expertise for evaluation and treatment. (W, , III)

The expert panel recommends that when considering nasal surgery for reasons other than epistaxis, the patient and clinician obtain consultation from an otorhinolaryngologist with expertise in HHT-related epistaxis. (W, , III)

The expert panel recommends that the treatment for acute epistaxis requiring intervention include packing with material or products that have a low likelihood of causing rebleeding with removal (e.g., lubricated low-pressure pneumatic packing). (W, , III)