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

Year 2013, Volume: 11 Issue: 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

Abstract

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

Keywords

Arteriyovenöz malformasyon, çomak parmak, siyanoz

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

Abstract

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

Keywords

Arteriovenous malformation, clubbed fingers, cyanosis

References

• 1. Shields TW. Congenital vascular lesions of the lungs. General thoracic surgery. 5th ed. Lippincot Williams & Wilkins 2000;975-87.
• 2. Liptay MJ, Ujiki MB, Locicero J. Congenital vascular lesions of the lungs. In: Shields WT (ed). General Thoracic Surgery. 6th ed. Philadephia: 2004:1144-52.
• 3. Coley SC, Jackson JE. Review pulmonary arteriovenous malformations. Clinical Radiology 1998;53:396-404.
• 4. Guttmacher AE, Marchuk DA, White RI Jr. Hereditary hemorrhagic telengiectasia. N Engl J Med 1995;339:918-24.
• 5. Borrero CG, Zajko AB. Pulmonary arteriovenous malformations: clinical features, diagnosis and treatment. J Radiol Nurs 2006;25:33-7.
• 6. Cottin V, Plauchu H, Bayle JY, Barthelet M, Revel D, Cordier JF. Pulmonary arteriovenous malformations in patients with hereditary hemorrhagic telangiectasia. Am J Respir Crit Care Med 2004;169:994-1000.
• 7. Gossage JR, Kanj G. Pulmonary arteriovenous malformations. A state of the art review. Am J Respir Crit Care Med 1998;158:643-61.
• 8. Dines DE, Seward JB, Bernatz PE. Pulmonary arteriovenous fistulas. Mayo Clin Proc 1983;58:176-81.
• 9. Hoffman WS, Weinberg PM, Ring E, Edmunds LH Jr. Massive hemoptysis secondary to pulmonary arteriovenous fistula. Treatment by a catheterization procedure. Chest 1980;77:697- 700.
• 10. Ference BA, Shannon TM, White RI Jr, Zawin M, Burdge CM. Life-threatening pulmonary hemorrhage with pulmonary arteriovenous malformations and hereditary hemorrhagic telangiectasia. Chest 1994;106:1387-90.
• 11. White RI Jr. Pulmonary arteriovenous malformations: how do I embolize? Tech Vasc Interv Radiol 2007;10:283-90.
• 12. Remy J, Remy-Jardin M, Wattinne L, Deffontaines C. Pulmonary arteriovenous malformations: evaluation with CT of the chest before and after treatment. Radiology 1992;182:809-16.
• 13. M. Puvanesary. 3 D gadolinium-enhanced MR angiography of pulmonary AVM. Australasian Radiology 2002;46:189-93.
• 14. Vrachliotis TG, Bis KG, Kirsch MJ, Shetty AN. Contrastenhanced MRA in pre-embolization assessment of a pulmonary arteriovenous malformation. J Magn Reson Imaging 1997;7:434-6.
• 15. Mann MJ, Kramer MJ, Hall TS, Anastassiou P, Katz J, Golden J, et al. Isolated pulmonary arteriovenous malformations requiring anatomic resection. J Thorac Cardiovasc Surg 2004;127:574-