Sunday, December 4, 2022

William’s Syndrome case

Bryan Rammell

I recently had a 20 y/o patient with William’s Syndrome.
Hello all!

I recently had a 20 y/o patient with William’s Syndrome.  What I know so far is
that it’s a gentic disorder that has eliminated the “elastin gene”.  I contacted
the William’s Syndrome foundation and looked on the web sites but see nothing to
very little in the way of what is done for them orthopedically.  He’s had an
achilles lengthening years ago b/c bone growth out grew the length that the
achilles was attaining.  He’s now at 90 degrees with a straight knee (B) but
externally rotates not only his feet but then externally twists on the ball of
his feet once the heel lifts off.  No midfoot break.  Very short stride
lengths.  He has pes cavus with heavy callusing on the lateral plantar aspect of
each foot.  With all of this he obviously is dealing with severe pain in the ML
arch.  (In the eloquent words of our illustrious celebrities…”Duh”)  The doc
requested articulated AFOs.  I’ve since contacted him twice for his goals but he
hasn’t returned my request.  The mother stated that he had to have these
“articulated afos” so that he doesn’t have to have sx again.

So, my question is this… (I feel like I’m showing ignorance to my colleagues) 
I could see putting him in an articulated AFO and limit ROM – even a solid ankle
with rocker soles to block further planar flexion.  But he’s not getting
dorsiflexion ROM anyway to even allow for dorsiflexion in the AFO.    What do
you know about William’s Syndrome muscles?   

Do my old friends at Shriners have an answer for me…other than “a fire
extinguisher” for “burning through shoes”?

Thanks in advance,
– Bryan Rammell, CO
Northern Prosthetics, ME


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