Atlantoaxial Rotatory Fixation/Subluxation

Ok, so I wanted to post a bit about this new diagnosis I am learning about! My 6 year old daughter, Kimber, came home from school on Wednesday the 3rd (?) saying she hurt her neck in gym. She couldn't tell me how, just said it started hurting while she was walking around. By 8 pm she was in a pretty funny position, with her shoulders uneven like in scoliosis, her head was tilted towards one of her shoulders and she had a nasty lump on the stretched side of her neck. Maybe, if she hadn't of had the funny lump I would have just said she pulled a muscle.

But, lump in mind, she and I went to the ER at 9 pm and were there until 2 am. The did x-rays and said she had Torticollis. This condition involves spasms of the neck muscles on one side of the neck making the individual turn the neck to the opposite side. In most cases it will resolve on it's own over days to weeks. The ER physician called the neurosurgeon on call at 1 am to make sure there were no considerations that needed to be taken related to her possible EDS. Of course he said no, but that we were to follow up with him Thursday.
Really? A neurosurgeon for torticollis? Unless the torticollis is cause by something underlying.

Yes. The neurosurgeon told me she was on her way to Atlantoaxial Rotatory Fixation. Wow. That's a mouth full.



This happens when C1 rotates funny on C2, causing pain, muscle spasm, and the funny head tilt she had. He said she was not at the place of being totally fixated yet, and our goal is to avoid that.

Total fixation would mean that C1 would not move back into position and it would require surgical intervention. The firt being something minor like a halo with traction, moving to further surgeries if it doesn't move back into position on its own.

So, we are trying a week of low dose Valium (muscle relaxer) to relax the neck muscles around the C1-C2 to see if it goes back into place on its own.

Her movement is much improved from Wednesday. Sometimes she has full ROM, sometimes she can't move certain ways, like tilting her head backward. Her neck still hurts a great deal. I am hoping to take her to the orthopedist I work with. She still has a funny lump on the back of her neck, up high around the C1-C2 area. And it's not the muscle anymore like originally thought. Her muscles are relaxed from the Valium.


I have asked several physicians if this is related to the EDS, but have gotten a no from all of them. I don't understand how it could not be related. It involves instability of a joint in the spine, subluxation of that joint. I know in Down's syndrome it is a common complication from joint instability. I don't understand why it wouldn't be considered in EDS. Makes sense right? No, instead they'd rather relate it to a cold she doesn't have or trauma that never happened... Sometimes I don't understand doctors.

Anyway, she is feeling better, just the pain and somewhat limited ROM and that lump. Thanks for all the prayers for her!