Browse > Article
http://dx.doi.org/10.3345/kjp.2009.52.1.124

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

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)
Publication Information
Clinical and Experimental Pediatrics / v.52, no.1, 2009 , pp. 124-128 More about this Journal
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.
Keywords
Pulmonary arteriovenous malformations; Cyanosis; Dyspnea;
Citations & Related Records
연도 인용수 순위
  • Reference
1 Gossage JR, Kanj G. Pulmonary arteriovenous malformations. A state of the art review. Am J Respir Crit Care Med 1998;158:643-61   DOI   PUBMED
2 Kang SM, Kim MK, Chi JG. Pulmonary arteriovenous fistula in childhood: report of a case. Korean J Pathol 1992; 26:201-3
3 Shovlin CL, Guttmacher AE, Buscarini E, Faughnan ME, Hyland RH, Westermann CJ, et al. Diagnostic criteria for hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome). Am J Med Genet 2000;91:66-7   DOI   ScienceOn
4 Kuwon ES, Park WK, Ryu JS, Kwak SM, Lee HL, Jo CH, et al. A case of pulmonary arteriovenous fistula with a family history. Tuberc Respir Dis 1998;45:649-53   ScienceOn
5 Lee HK, Han GS. A case of transcatheter coil embolization of diffuse pulmonary arteriovenous malformation. J Korean Pediatr Soc 2001;44:1036-40
6 Hall WA. Hereditary hemorrhagic telangiectasia (Rendu- Osler-Weber disease) presenting with polymicrobial brain abscess. J Neurosurg 1994;81:294-6   DOI   PUBMED   ScienceOn
7 White RI Jr, Pollak JS, Wirth JA. Pulmonary arteriovenous malformations: diagnosis and transcatheter embolotherapy. J Vasc Inter Radiol 1996;7:787-804   DOI   ScienceOn
8 Plauchu H, de Chadarevian JP, Bideau A, Robert JM. Age- related clinical profile of hereditary haemorrhagic telangiectasia in an epidemiological recruited population. Ann J Med Genet 1989;8:345-51   DOI   ScienceOn
9 Thompson RD, Jackson J, Peters M. Doré CJ, Hughes JM. Sensitivity and specificity of radioisotope right-left shunt measurements and pulse oximetry for the early detection of pulmonary arteriovenous malformations. Chest 1999;115: 109-13   DOI   ScienceOn
10 Brydon HL, Akinwunmi J, Selway R, UI-Haq I. Brain abscesses associated with pulmonary arteriovenous malformations. Br J Neurosurg 1999;13:265-9   DOI   ScienceOn
11 Chang UG, Kim DG, Yang HJ, Lee HK, Choi KS, Han DH. Brain abscess associated with pulmonary arteriovenous malformation. J Korean Neurosurg Soc 1992;21:895-8
12 Walder LA, Anastasia LF, Spodick DH. Pulmonary arteriovenous malformations with brain abscess. Am Heart J 1994; 127:227-32   DOI   ScienceOn
13 Dines DE, Seward JB, Bernatz PE. Pulmonary arteriovenous fistulas. Mayo Clin Proc 1983;58:176-81   PUBMED   ScienceOn
14 Guttmacher AE, Marchuk DA, White RI. Hereditary hemorrhagic telangiectasia. N Engl J Med 1995;333:918-24   DOI   ScienceOn
15 Churton T. Multiple aneurysms of pulmonary artery. BMJ 1897;1:1223
16 Hewes RC, Auster M, White RI Jr. Cerebral embolism: first manifestation of pulmonary arteriovenous malformation in patients with hereditary hemorrhagic telangiectasia. Cardiovasc Intervent Radiol 1985;8:151-5   DOI   ScienceOn
17 Cottin V, Plauchu H, Dupuis-Girod S, Cordier JF. Pulmonary arteriovenous malformations in patients with hereditary hemorrhagic telangiectasia: follow-up and pathophysiologic considerations. J Vasc Interv Radiol 2007;18:938-9   DOI   ScienceOn
18 Temes RT, Parasothy P, Endara SA, Wernly JA. Sensitivity and specificity of radioisotope right-left shunt measurements and pulse oximetry for the early detection of pulmonary arteriovenous malformation by video-assisted thoracic surgery. J Thorac Cardiovasc Surg 1998;116:878-9   DOI   ScienceOn