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Vascular Ehlers–Danlos syndrome presenting as rapidly progressive multiple arterial aneurysms and dissections

Authors

  • Eduardo J. Mortani Barbosa Jr.,

    Corresponding author
    1. Department of Radiology, The Raymond and Ruth Perelman School of Medicine of the University of Pennsylvania, Philadelphia, Pennsylvania
    • Department of Radiology, Hospital of the University of Pennsylvania, 3400 Spruce Street, Philadelphia, PA 19104.
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  • Reed E. Pyeritz,

    1. Departments of Medicine and Genetics, The Raymond and Ruth Perelman University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania
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  • Harold Litt,

    1. Department of Radiology, The Raymond and Ruth Perelman School of Medicine of the University of Pennsylvania, Philadelphia, Pennsylvania
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  • Benoit Desjardins

    1. Department of Radiology, The Raymond and Ruth Perelman School of Medicine of the University of Pennsylvania, Philadelphia, Pennsylvania
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  • How to Cite this Article: Mortani Barbosa Jr. EJ, Pyeritz RE, Litt H, Desjardins B. 2011. Vascular Ehlers–Danlos syndrome presenting as rapidly progressive multiple arterial aneurysms and dissections. Am J Med Genet Part A 155: 3090–3094.

Abstract

Life expectancy in vascular Ehlers–Danlos syndrome (EDS) is shortened due to spontaneous rupture of arteries, the colon and the gravid uterus. Two adolescent males with vascular EDS illustrate rapid progression of arterial aneurysms, dissections, and rupture. Radiologic imaging played an important role in initially diagnosing and monitoring the evolution of arterial involvement. Both prophylactic and emergency management remain largely ineffective in this connective tissue disorder; however, noninvasive imaging may provide important prognostic information. © 2011 Wiley Periodicals, Inc.

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