Arnold-Chiari Malformation

The routine brain MRI Will had done showed an Arnold-Chiari Type I malformation. Ever heard of this? I hadn't heard of this. Initially the developmental pediatrician called me with the results and said she didn't believe it was of significance but asked us to see a neurosurgeon so he could explain it and take a good look. That appointment was two weeks out and I had plenty of time to use my Web-MD mom-ster skills and I immersed myself in learning about this condition. For those two weeks I had a lot of hope and was so sure this surgeon was going to tell us our son would be a candidate for surgery and this could be the answer to all his mysteries. That's not how it went down. Not at all.

First, what is a Chiari malformation? It is a disorder where the cerebellum, the lower part of the brain, protrudes out of the skull and into the spinal canal. Here's a diagram:

There can be many, many different symptoms associated with Chiari. The main symptoms are pain and headaches. We were told as many as 1 out of 3 people have a Chiari malformation and most do not know they have it. It is a fairly-common condition. But for the adults and children who do have symptoms, their lives are a living hell. I also read on one parent's blog a list of possible issues associated with Chiari and this includes (but not limited to): Visual disturbances, impaired fine motor skills, clumsiness, poor motor skills, insomnia, intolerance to loud sounds, difficulty swallowing, poor depth perception, unusual pupil size, poor muscle tone, and developmental delays. I read this list and you can imagine how I felt. I was just so sure getting this thing taken care of would be the answer! I jumped-the-gun way too soon and told everyone who would listen I thought this was exactly what Will's problem was.  

I'll just show myself out!

People who are candidates for the surgery I speak of can have a decompression surgery. It is a craniotomy which is supposed to make room for the protruding cerebellum and would relieve pressure on the brain as well as restore normal flow of cerebrospinal fluid.

We met with the neurosurgeon and he blatantly said this was not causing Will's issues. We had a few questions for him, but he didn't really feel anything as drastic as surgery would make any difference. The one thing that bothered me about this was he asked if Will has headaches. Will doesn't talk and doesn't understand what we are asking him if he's in pain. He can't point to his head and say or signal in some way that he has a headache. He at this point was starting up with his behavior issues and I said, well he's really unhappy most of the time. I had tons of examples, but this doctor just didn't agree. I can't make a doctor agree to brain surgery!

Sometimes people with Chiari can have a condition called Syringomyelia. This is a collection of the abnormally-flowing fluid that is building up because of the Chiari and creates a cavity of fluid called a syrinx down into the spine and can cause many problems including (but, again, not limited to) pain in the extremities, numbness, weakness, loss of normal gait, and dizziness. This is a really serious condition and tends to get worse over time. The neurosurgeon ordered another MRI of Will's spine this time to look for a syrinx and, if there was one, he would revist the surgery idea. Will didn't have one. Here's a pic of a syrinx:

This doctor told us to follow up in a year with another MRI to see if anything had changed. That's coming up towards the end of this year. He essentially told us if there was no change, then not to pursue this further. I guarantee I will fight to have this condition monitored at least every couple of years... And if there is any indication of pain, we will go back immediately.

The back of a child's head after decompression surgery.

We also ended up seeing a neurologist to talk about Will overall and also the Chiari malformation. She agreed with the neurosurgeon, but also did a wonderful job explaining things to me in a way I could understand.

We decided to also get a second opinion from a different neurosurgeon from a different hospital. I was starting to be suspicious that maybe Children's Hospital had some kind of standard thing they told parents about Chiari malformations and I really just wanted to see what someone not associated with them thought. This isn't really rational thinking, but we did it to cover all bases. This new neurosurgeon was a great lady and she is famous in the community of neurosurgery. I appreciated her taking the time to talk with us and look at Will's MRIs. In the end, she agreed with the other doctors. We decided at that point to take a break from all this Chiari business and revamp our focus more on helping Will through his various therapies. I think we made the right decision.

I still have a bit of a fire under my feet, though, about Chiari. I joined several message boards and groups on Facebook for families affected by Chiari. I will tell you there are some really incredible families, amazing warrior mothers in particular, and super-hero children out there. Some of these children have had multiple brain surgeries sometimes before they are even 2 or 3 years old and are fighting for their lives... They live with pain and endure many visits to various specialists and doctors and therapists. The mothers are some of the smartest and most educated people I've ever encountered and they always are willing to answer questions or lend an ear. I feel very lucky to know them. It feels good to be informed of something I never knew existed before. It also makes me feel blessed for all the things we really do have.