Aygün DİNDAR, Kemal NİŞLİ, Yakup ERGÜL

Osler-Weber-Rendu sendromu ile ilişkili pulmoner arteriyovenöz malformasyon

Osler-Weber-Rendu sendromu çocuklarda göreceli sık görülen; cilt, mukoza ve iç organlarda telanjiektazi ve arteriyovenöz malformasyonlarla seyreden vasküler bir displazidir. Bu yazıda akut romatizmal kardit atağı sırasında santral siyanoz, çomak parmak, yüzde, burun mukozasında telanjiektaziler ve sağ akciğerde arteriyovenöz malformasyonu saptanan ve Osler-Weber-Rendu sendromu tanısı alan dokuz yaşında bir erkek olgu sunuldu. Akut romatizmal kardit atağı geçtikten sonra olguya kalp kateterizasyonu yapılarak arteriyovenöz malformasyona “coil” embolizasyonu uygulandı. (Türk Ped Arfl 2011; 46: 264-6)

Pulmonary arteriovenous malformation associated with Osler-Weber-Rendu syndrome

Osler-Weber-Rendu syndrome is a relatively common vascular displasia of children presented with telengiectasias of skin, mucosa, and visceral organs and arteriovenous malformations. We reported a 9-year-old boy diagnosed as having Osler-Weber Rendu syndrome with his central cyanosis, clubbing, facial and nasal mucosal telengiectasias and right pulmonary arteriovenous malformation during an attack of acute rheumatic carditis. After relief of the acute rheumatic carditis attack, his arteriovenous malformation was treated with coil embolisation by cardiac catheterization. (Turk Arch Ped 2011; 46: 264-6)

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