U W Geisthoff1, G Schneider, J Fischinger, P K Plinkert. 1. Klinik und Poliklinik für Hals-Nasen-Ohren-Heilkunde, Universitätskliniken des Saarlandes, Homburg/Saar. hnougei@uniklinik-saarland.de
Abstract
BACKGROUND: Hereditary hemorrhagic telangiectasia (HHT or Rendu-Osler-Weber disease) is an autosomal dominant inherited disease of the fibrovascular tissue. More than 90% of patients have recurrent nosebleeds. Therefore, ENT physicians often have a key position for diagnosis and management of the disease. Epistaxis can severely reduce the quality of life, but visceral lesions are also life threatening. METHODS: This paper provides a review of the literature about the disease, its diagnosis, preventive measures, and therapy of the different manifestations. RESULTS AND CONCLUSIONS: Physicians should be especially aware of gastrointestinal telangiectases, arteriovenous malformations (AVM) of the lungs, liver, and brain. A screening is recommended at least for pulmonary AVM.
BACKGROUND:Hereditary hemorrhagic telangiectasia (HHT or Rendu-Osler-Weber disease) is an autosomal dominant inherited disease of the fibrovascular tissue. More than 90% of patients have recurrent nosebleeds. Therefore, ENT physicians often have a key position for diagnosis and management of the disease. Epistaxis can severely reduce the quality of life, but visceral lesions are also life threatening. METHODS: This paper provides a review of the literature about the disease, its diagnosis, preventive measures, and therapy of the different manifestations. RESULTS AND CONCLUSIONS: Physicians should be especially aware of gastrointestinal telangiectases, arteriovenous malformations (AVM) of the lungs, liver, and brain. A screening is recommended at least for pulmonary AVM.