Surgical management of life-threatening epistaxis in Osler-Weber-Rendu disease.

Abstract

Hereditary hemorrhagic telangiectasia is a systemic disorder of blood vessels. It often leads to severe epistaxis that is refractory to conventional therapy. Three patients with severe epistaxis caused by hereditary hemorrhagic telangiectasia unresponsive to laser coagulation and to septal dermoplasty and requiring multiple transfusions underwent extirpation of nasal mucosa through a rhinotomy approach, with a median forehead flap and split-thickness skin graft coverage of the mucosal defects over the floor and lateral walls of the nose. All patients had reduced frequency and severity of bleeding without any need for transfusions during follow-up periods of 6 months, 3 years, and 5 years, respectively. Forehead flaps resulted in minimal morbidity. Partial nasal obstruction and a forehead scar were the main complaints. Surgical technique, complications, and alternative therapy are discussed.