Pulmonary telangiectasia

Pulmonary telangiectasia is an uncommon developmental anomaly characterized by minute arteriovenous fistulae disseminated throughout both lungs. It should be suspected in patients with cyanosis and digital clubbing who do not have chronic lung disease or a cyanotic cardiac anomaly. The presence of telangiectases of skin or mucous membranes in the patient or members of his family is an important diagnostic clue. The chest film usually shows a coarse spidery appearance of the peripheral vascular markings in the lungs. More specific findings are obtained in the pulmonary angiogram where the normally invisible capillary phase is replaced by irregular vascular channels bridging the peripheral branches of pulmonary arteries and veins. The intrapulmonary right-to-left shunt may be confirmed by pulmonary scintigraphy using technetium-labeled albumin. Two cases are presented with a brief review of the literature.
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				M. E. Faughnan, Y. W. Lui, J. A. Wirth, R. A. Pugash, D. A. Redelmeier, R. H. Hyland, and R. I. White Jr. Diffuse Pulmonary Arteriovenous Malformations* : Characteristics and Prognosis Chest, January 1, 2000; 117(1): 31 - 38. [Abstract] [Full Text] [PDF]