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Keywords:

  • vascular Ehlers–Danlos syndrome;
  • arterial aneurysm;
  • arterial dissection;
  • vascular imaging

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.