Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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Pulmonary arteriovenous malformation manifesting with perioral cyanosis and dyspnea on exertion: A case report

청색증과 호흡곤란을 동반한 폐동정맥루의 1예

  • Kim, Yu Kyung (Department of Pediatrics, Pochon CHA University) ;
  • Kim, Jin Woo (Department of Pediatrics, Pochon CHA University) ;
  • Lee, Gun (Department of Thoracic & Cardiovascular Surgery, Pochon CHA University) ;
  • Han, Man Yong (Department of Pediatrics, Pochon CHA University)
  • 김유경 (포천중문의과대학 소아청소년과학교실) ;
  • 김진우 (포천중문의과대학 소아청소년과학교실) ;
  • 이건 (포천중문의과대학 훙부외과학교실) ;
  • 한만용 (포천중문의과대학 소아청소년과학교실)
  • Received : 2008.07.05
  • Accepted : 2008.10.16
  • Published : 2009.01.15
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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.

폐동정맥루는 폐동맥과 폐정맥이 모세혈관 없이 바로 연결된 것으로서 우좌 단락을 형성하여 청색증, 호흡곤란과 곤봉지를 일으키는 질환이다. 또한 뇌혈전에 의하여 뇌출혈이나 뇌농양 등의 신경학적인 합병증을 일으킬 수 있다. 대부분의 경우 청소년기 후기까지 증상이 나타나지 않는 경우가 많다. 본 증례는 입주위의 청색증과 곤봉지 및 호흡곤란을 보인 6세된 남아였다. 흉부 엑스레이에서 우측하엽에 국소적인 결절의 음영이 발견되었고 흉부 단층 촬영을 통해 우측 하엽의 큰 폐동정맥루를 확진하였다. 우측 하엽의 폐절제술로 합병증 없이 성공적으로 치료하였다. 영유아시기에 폐동정맥루의 발생 빈도는 드물긴 하지만, 심혈관계 질환 없이 청색증과 호흡곤란을 보이는 드문 원인으로 폐동정맥루의 가능성을 염두에 두고 일찍 진단하고 치료함으로써 신경학적인 합병증의 발생을 예방하여야 한다.

Keywords

Acknowledgement

Supported by : Biorane Company

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