Do 10-25% of those with Classical EDS who tested negative for Vascular EDS have vascular issues?
||I am not aware of an absolute increased risk for a "vascular issue" in
the other forms of
EDS. One "vascular concern" that has been evaluated in individuals with
classical EDS and hypermobility EDS is the issue of aortic diameter.
After identifying a small number of individuals with classical EDS who
have a larger than normal aortic diameter, researchers in Cincinnati
and the NIH evaluated the aorta of 72 individuals with EDS and found
aortic root dilation (ARD) in 20 individuals; 14 of 42 with classical
EDS and 6 of 29 with hypermobile EDS. Any aortic diameter greater than
2 standard deviations beyond the mean (95%tile) was recorded as
"dilated". Now, as only one ultrasound evaluation was done on each
patient, we do not know if and how the size of the aorta might change
with time in EDS individuals. Longitudinal studies are necessary to
answer this question. Prevalence of aortic root dilation in the
(Wenstrup RJ, et.al.Genet Med. 2002; 4(3): 112-7) Answered by: Melanie Pepin, MS,CGC.