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[Hereditary hemorrhagic telangiectasia (Osler's disease). An interdisciplinary challenge]
U W Geisthoff1, G Schneider, J Fischinger
1Klinik und Poliklinik für Hals-Nasen-Ohren-Heilkunde, Universitätskliniken des Saarlandes, Homburg/Saar. hnougei@uniklinik-saarland.de
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.