Hereditary haemorrhagic telangiectasia (Osler-Weber-Rendu syndrome): otorhinolaryngological manifestations.

Abstract

Hereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant disorder affecting blood vessels of the skin, mucous membrane and viscera. The otorhinolaryngologist is the commonest clinician involved in management as epistaxis occurs in 93% of the patients. As marked advances have recently been made regarding the pathogenesis and management of the condition, the otorhinolaryngological perspective is reviewed.