(BMJ) – A 55-yo man presented w/ recurrent epistaxis. No risk factors or family hx were reported. Exam: extensive telangiectasia of the lips and septum (A), polyp (B), and lateral wall (C) in both nasal cavities. What is the diagnosis?
CREST syndrome
Allergic rhinitis
Osler-Weber-Rendu syndrome
Ataxia-telangiectasia
Essential telangiectasia
You are correct. Hereditary hemorrhagic telangiectasia, or Osler-Weber-Rendu syndrome, is an uncommon cause of epistaxis w/ serious implications. This autosomal dominant condition is strongly associated w/ AVMs and aneurysms of the brain and lung. A CT pulmonary angiogram in this pt showed a 2-cm left lower lobe AVM. Embolization was planned and he was advised of the need for antibiotics for any dental work.
By whadikin, 21 November, 2014

HF