Pulmoner Arteriyovenöz Malformasyonda Klasik Triad: Çomak Parmak, Siyanoz ve Polisitemi

Yıl 2013, Cilt: 11 Sayı: 2, 92 - 95, 01.09.2013

Hikmet Tekin Nacaroğlu Saniye Gülle Özlem Bağ Hüdaver Alper Mustafa Bak Demet Can

https://doi.org/10.4274/Jcp.11.08208

Öz

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

Anahtar Kelimeler

Arteriyovenöz malformasyon, çomak parmak, siyanoz

Classical Triad in Pulmonary Arteriovenous Malformation: Clubbing, Cyanosis and Policytemia

Öz

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

Anahtar Kelimeler

Arteriovenous malformation, clubbed fingers, cyanosis

Kaynakça

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