Your Questions

Thanks so much for the emails, comments, and calls about Wil. We appreciate the prayers and concern so much. I want to take this opportunity to answer a few of your questions regarding AAI/AOI.

1. What exactly is AAI and why was Wil tested for it?

AAI, Atlano-axial instability, is an instability between the first two cervical vertebrae, C1 and C2. All kids with Down syndrome have loose joints as exibited by this photo. Ouch!
This is due in part to low muscle tone and weak ligaments. However, about 10-30% of kids with Ds have increased instability in the atlanto-axial joint. Health care guidelines for kids with Ds recommend an x-ray of the neck at age three to rule out AAI.

2. Is surgery the only option?

A diagnosis of AAI does not always lead to surgery. Many kids with Ds are diagnosed with AAI but because the gap between C1 and C2 is not causing harm to their spinal cord surgery is not indicated. A typical person may have a gap at the AA joint of 2.5 mm. A kid with Ds will normally have a larger gap - if it is too large an MRI is done to see if surgery is necessary. Wil is measuring 7 mm at the AA joint. His neurosurgeon feels this leaves him at risk of spinal injury.

3. What will the surgery involve?

It is a 3-4 hour surgery in which C1 and C2 are fused together with bone taken from a bone bank or from the patient's hip (this is determined by the orthopedic surgeon who is part of the surgical team). Then some type of cord is wrapped around the fused vertebrae. The fusion will have no impact on range of motion once healed.

4. Is a halo the only option during recovery? Why not a neck brace?

We are told the halo is a must for proper healing. A brace does not stabilize the neck enough. The halo does not come off the entire recovery period. We have been told 3 months by the neurosurgeon here but have heard 6 months from another hospital that specializes in spinal fusion. We will obviously want some clarity on this.

5. What is AOI and why does the doctor think Wil has that?

AOI is Atlanto-occipital instability and is the joint between the occipital bone (base of the skull) and C1. Frankly I am not sure why Dr. Smith is preparing us for this. He admitted that the xray did not capture that joint and could not say based on xray. So, he either senses something based on his experience and his observation of Wil (the way he holds his head, his lateness in walking, his balance, his lack of reflexes) or he is just preparing us for the worst so that he doesn't have to come back later with bad news. I don't know.

What I do know is that AOI is far more involved and would add a few more hours to surgery, require metal plates be placed on each side of the spine with rods going down to C3. This would permanently change Wil's range of motion. He would have to rotate from his torso to look around rather than his neck. The recovery is the same - a halo.

6. What will the MRI and CT scan tell the doctors?

The MRI looks at soft tissue and will let them know if the spine is being compressed or pinched or has any damage. They will be able to see where it is in the vertebral column and if it is compromised. The CT scan gives a good picture of bone. It will give them a good structural look at things and reveal whether or not the gap between the occipital bone and C1 is dangerous. Also, kids with Ds can have a malformed C2. If if is not formed properly then surgery is an absolute, the CT will show this.

7. Will you have the surgery there?

Yes. We feel comfortable with the neurosurgeon and the orthopedic surgeon performing the surgery. However, we want many more voices involved in making the decision to go to surgery. We have been encouraged by another doctor to get many opinions, that is what we will do. Our current plan is to get a second opinion from Shriners Children's Hospital in PA (they specialize in spinal fusion), Shriner's in Greenville, SC (they specialize in orthopedics), UF-Shands (we know a general orthopedic surgeon there that we trust), and MUSC in Charleston, SC. When Wil receives his MRI and CT we will request copies, mail them to the appropriate physician at each of these hospitals where they will review them and phone us with their opinion.

8. When will the surgery be?

If the consensus is to move forward with surgery we will discuss the timing with those involved. While we don't want to do anything to endanger Wil by holding off on surgery we also want to make sure that we are not rushing surgery.

Wil is at a developmental stage that will make the recovery very difficult and cause him to regress in his gross motor development. Obviously we would face these challenges if putting off surgery puts him at high risk of permanent spinal injury.

9. What can we do for you?

Pray boldly.

That's what I know now. If there are any medical folks out there reading forgive my layman's explanation - hopefully I got most of it right!

We do have a date for the MRI and CT scan. Thursday, June 12. We will update you once we get some more answers.

Again, many thanks for your prayers and concern for Wil.


Mommy to those Special Ks said...

Just to let you know, Kennedy will not have rods put in for her surgery. While her range of motion will be limited, she will be able to move her neck some. I'm so glad you are going to talk to Shriners! Let me know if I can help!

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