[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.


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.
Geisthoff
U W
UW
Klinik und Poliklinik für Hals-Nasen-Ohren-Heilkunde, Universitätskliniken des Saarlandes, Homburg/Saar. hnougei@uniklinik-saarland.de
Schneider
G
G
Fischinger
J
J
Plinkert
P K
PK
D003937:Diagnosis, Differential; D003952:Diagnostic Imaging; D004844:Epistaxis / Q000209:etiology* / Q000401:mortality; D006801:Humans; D010348:Patient Care Team; D011379:Prognosis; D015996:Survival Rate; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000175:diagnosis* / Q000235:genetics / Q000401:mortality