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THE JOURNAL OF CARDIOVASCULAR SURGERY

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The Journal of Cardiovascular Surgery 2001 October;42(5):687-9

Copyright © 2009 EDIZIONI MINERVA MEDICA

language: English

A brachial aneurysm in childhood caused by Ehlers-Danlos syndrome

Sayin A. G., Bozkurt A. K., Cangel U., Köksal C., Öz B.

From the Department of Thoracic and Cardiothoracic Surgery, *Department of Pathology, University of Istanbul, Cerrahpas¸a Medical Faculty, Istanbul, Turkey


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A ­case of an 11-­year-­old ­boy ­with Ehlers-Danlos syn­drome (­EDS) ­type IV is pre­sent­ed. He ­was ­referred ­with a pul­sa­tile ­mass ­above ­the ­right ante­cu­bi­tal fos­sa ­and Doppler ­revealed a sac­cu­lar aneu­rysm. Excision ­and liga­tion of ­the bra­chi­al ­artery aneu­rysm ­was per­formed. The diag­no­sis ­was con­firmed by ­skin fibro­blast cul­tures ­and his­to­path­o­log­ic eval­u­a­tion. We con­clude ­that arter­i­og­ra­phy ­should be avoid­ed in ­EDS ­and col­or-Doppler ­should be ­the ­sole ­means of diag­no­sis. Ligation rath­er ­than arte­ri­al recon­struc­tion ­should be pre­ferred.

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