[Epistaxis in patients with hereditary hemorrhagic teleangiectasia]

Abstract

Introduction: Spontaneous recurrent epistaxis is the most common clinical manifestation of hereditary hemorrhagic teleangiectasia (HHT). It occurs in more than 90% of HHT patients and is the most distressing symptom. Nasal teleangiectasias tend to increase with age both in size and number, so epistaxis is heavier and more frequent. For patients with mild to moderate disease, there are many adequate treatment options. For those with severe disease, most treatments offer just a hemorrhage-free interval. Experienced otorhinolaryngologists who treat epistaxis in these patients often use the adage "to do as little as possible for as long as possible".

Management of acute epistaxis: The recommendations for the management of acute epistaxis include: compression, use of topical antifibrinolytics, laser therapy, argon plasma coagulation therapy, fibrin sealant spray or gelatin sponge soaked in adrenaline. In cases of heavy acute epistaxis, an epistaxis balloon combined with artery ligation and/or embolization is the most effective treatment. Nasal packing and electrocauterisation should be avoided to prevent further trauma to the blood vessels.

Treatment of recurrent epistaxis: Management of recurrent epistaxis includes topical application of laser energy (argon, Nd:YAG, KTP/532 and diode, not CO2), argon plasma coagulation in combination with 0.1% estriol ointment, caustics, antifibrinolytics, bleomycin and sclerosing substances. Systemic estrogen-progesterone at doses used for oral contraception may eliminate bleeding in women with heavy epistaxis. Systemic antifibrinolitics (used with extreme precaution) and septal dermoplasty give good results. The only method which successfully and permanently solves the problem of severe refractory epistaxis in hereditary hemorrhagic teleangiectasia is closure of the nasal cavities.