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.
Vascular Ehlers–Danlos syndrome presenting as rapidly progressive multiple arterial aneurysms and dissections†
Version of Record online: 7 NOV 2011
Copyright © 2011 Wiley Periodicals, Inc.
American Journal of Medical Genetics Part A
Volume 155, Issue 12, pages 3090–3094, December 2011
How to Cite
Mortani Barbosa, E. J., Pyeritz, R. E., Litt, H. and Desjardins, B. (2011), Vascular Ehlers–Danlos syndrome presenting as rapidly progressive multiple arterial aneurysms and dissections. Am. J. Med. Genet., 155: 3090–3094. doi: 10.1002/ajmg.a.34332
- Issue online: 21 NOV 2011
- Version of Record online: 7 NOV 2011
- Manuscript Accepted: 7 SEP 2011
- Manuscript Received: 3 JAN 2011
- vascular Ehlers–Danlos syndrome;
- arterial aneurysm;
- arterial dissection;
- vascular imaging
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.