[Hereditary hemorrhagic telangiectasia (Osler's disease). An interdisciplinary challenge].

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.