Hikmet Tekin NACAROĞLU, Mustafa BAK, Hüdaver ALPER, Özlem BAĞ, Demet CAN, Saniye GÜLLE

Pulmoner arteriyovenöz malformasyonda klasik triad: Çomak parmak, siyanoz ve polisitemi

Pulmoner arteriyovenöz malformasyonlar (PAVM) anormal kapiller gelişimden kaynaklanan, genellikle konjenital lezyonlardır. Lezyonlar izole tek bir anomali şeklinde görülebileceği gibi otozomal dominant geçişli herediter hemorajik telenjiektazi (Rendu-Osler-Weber sendromu; ROWS) ile birlikte çok sayıda da olabilir. Bu vakalar asemptomatik olabilecekleri gibi efor dispnesi, çarpıntı ve çabuk yorulma da görülebilir. Klasik radyolojik görünümü yuvarlak, düzgün sınırlı lezyonlardır. Bilgisayarlı toraks tomografisi ve anjiyografi de teşhis için faydalıdır.Burada çomaklaşma ve siyanoz ile PAVM tanısı alan 2 ve 13 yaşlarında iki kız olgu sunulmuştur.

Classical triad in pulmonary arteriovenous malformation: Clubbing, cyanosis and policytemia

Pulmonary arteriovenous malformations (PAVM) are generally congenital lesions that results from an abnormal capillary development. Lesions can be presented as an isolated single anomaly, or may be multiple when accompanying with autosomal dominant hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber Syndrome; ROWS). These cases may be asymptomatic, but exertional dyspnea, palpitations and easy fatigability may also be seen. The classic radiological appearance is a round, well-circumscribed lesions. Computed tomography of thorax and angiography are also useful for diagnosis. Herein, we present 2 and 13 years old girls with the diagnosis of PAVM with clubbing and cyanosis.

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