Classical Triad in Pulmonary Arteriovenous Malformation: Clubbing, Cyanosis and Policytemia/Pulmoner Arteriyovenoz Malformasyonda Klasik Triad: Comak Parmak, Siyanoz ve Polisitemi

• Published in: Güncel pediatri Vol. 11; no. 2; p. 92
• Main Authors: Nacaroglu, Hikmet Tekin, Gulle, Saniye, Bag, Ozlem, Alper, Hudaver, Bak, Mustafa, Can, Demet
• Format: Journal Article
• Language: Turkish
• Published: Galenos Yayinevi Tic. Ltd 01-08-2013
• Subjects: Abnormalities; Arteriovenous malformations; Bones; Case studies; Chest; Cyanosis; Diagnosis; Diseases; Health aspects; Pediatric research; Polycythemia; Pulmonary artery; Turkey
• ISSN: 1304-9054
• DOI: 10.4274/Jcp.11.08208

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. (Journal of Current Pediatrics 2013; 11: 92-5) Key words: Arteriovenous malformation, clubbed fingers, cyanosis Pulmoner arteriyovenoz malformasyonlar (PAVM) anormal kapiller gelisimden kaynaklanan, genellikle konjenital lezyonlardir. Lezyonlar izole tek bir anomali seklinde gorulebilecegi gibi otozomal dominant gecisli herediter hemorajik telenjiektazi (Rendu-Osler-Weber sendromu; ROWS) ile birlikte cok sayida da olabilir. Bu vakalar asemptomatik olabilecekleri gibi efor dispnesi, carpinti ve cabuk yorulma da gorulebilir. Klasik radyolojik gorunumu yuvarlak, duzgun sinirli lezyonlardir. Bilgisayarli toraks tomografisi ve anjiyografi de teshis icin faydalidir. Burada comaklasma ve siyanoz ile PAVM tanisi alan 2 ve 13 yaslarinda iki kiz olgu sunulmustur. (Guncel Pediatri 2013; 11: 92-5) Anahtar kelimeler: Arteriyovenoz malformasyon, comak parmak, siyanoz