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Pulmonary arteriovenous malformation manifesting with perioral cyanosis and dyspnea on exertion: A case report

Korean Journal of Pediatrics 2009;52(1):124-128.
Published online January 15, 2009.
Pulmonary arteriovenous malformation manifesting with perioral cyanosis and dyspnea on exertion: A case report
Yu Kyung Kim1, Jin Woo Kim1, Gun Lee2, Man Yong Han1
1Department of Pediatrics, Pochon CHA University, Seongnam, Korea
2Department of Thoracic & Cardiovascular Surgery, Pochon CHA University, Seongnam, Korea
청색증과 호흡곤란을 동반한 폐동정맥루의 1예
김유경1, 김진우1, 이건2, 한만용1
1포천중문의과대학 소아청소년과학교실
2포천중문의과대학 훙부외과학교실
Correspondence: 
Man Yong Han, Email: drmesh@gmail.com
Abstract
Pulmonary arteriovenous malformations (PAVMs) are direct communications between pulmonary arteries and pulmonary veins, resulting in right-to-left shunts that may cause cyanosis, dyspnea, and digital clubbing. Neurological complications such as intracerebral hemorrhage or brain abscess may result from cerebral thrombosis or emboli. In most cases, they remain unrecognized until the late teenage years. Here, we report a case of a 6-year-old boy who presented with perioral cyanosis, digital clubbing, and dyspnea on exertion. A plain chest X-ray showed a focal nodular opacity in the right lower lobe (RLL), and a diagnosis of a large PAVM in the RLL was confirmed by chest computed tomography. A right lower lobectomy was successfully performed without any complications. Although their incidence in children is low, PAVMs should be suspected as a possible cause of cyanosis and dyspnea of non-cardiac origin, and should be treated promptly to prevent further neurological complications.
Key Words: Pulmonary arteriovenous malformations, Cyanosis, Dyspnea


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