Rendu-Osler-Weber syndrome presenting with pulmonary arteriovenous fistula.
A pulmonary arteriovenous fistula is an abnormal connection between pulmonary arteries and veins. Patients with Rendu-Osler-Weber syndrome may present with this vascular malformation, which is a typical finding of the disease. Approximately 5-15% of Rendu-Osler-Weber syndrome patients have pulmonary arteriovenous malformations (AVM) and there is usually a family history of AVM in these patients. The malformations are usually located in the lower lobes. In this paper, I describe a 49-year-old male patient with dyspnoea, cough, haemoptysis and epistaxis. Physical examination showed nasal telangiectasias, cyanosis of the lips and nails, and a systolic bruit over the left lung. Chest X-ray revealed a 5-cm mass in the left lower lobe and after magnetic resonance examination, together with 3-D magnetic resonance angiography, it was demonstrated to be a pulmonary arteriovenous fistula. The history of a niece with a similiar history of suspected pulmonary arteriovenous fistula led me to consider the possibility of Rendu-Osler-Weber syndrome presenting with a pulmonary arteriovenous fistula.
Halefoglu
A M
AM
Department of Radiology, Sisli Etfal Training and Research Hospital, Istanbul, Turkey. halefoglu@hotmail.com
D001164:Arteriovenous Fistula / Q000175:diagnosis*; D001165:Arteriovenous Malformations / Q000175:diagnosis; D003287:Contrast Media; D019786:Gadolinium DTPA; D006801:Humans; D008168:Lung / Q000098:blood supply*; D018810:Magnetic Resonance Angiography; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D011651:Pulmonary Artery / Q000002:abnormalities; D011667:Pulmonary Veins / Q000002:abnormalities; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000175:diagnosis*
D003287:Contrast Media; D019786:Gadolinium DTPA