Let's hop in the way back Machine 😆
Last year I took Jaylia to her normal dentist appointment and the hygienist did her normal dental x-rays. She was shocked to see the two teeth on either side of her two front teeth were turned 90°, the baby teeth had not fallen out yet, this was all seen on the x-ray. She suggested we get checked out by our orthodontist asap to get an action plan.
So off to the orthodontist we went. He took this really cool x-ray you see here of her profile.
When he looked at this he said, to paraphrase, That doesn't look right to me. He said he sees a lot of skulls, but this was not something he was familiar with seeing.
He recommended we go to the cranial specialists at our local Children's hospital. So off we went!
The head of their cranial facial department did not expect to find anything, but when he viewed her CT scan, he said, "Oh, there is something there!". And by something he meant, not what was supposed to be there.
Jaylia was then diagnosed with Multi-suture craniosynostosis. There are 4 major sutures of the skull, all of hers are fused, something that typically doesn't happen until adulthood. This has caused her brain to pulse with every heartbeat and imprint into the inner layer of her skull, giving it that rippled or "copper beaten" appearance on our first orthodontic x-ray. If you Google a typical skull profile x-ray it looks smooth, and you may even be able to see the sutures. For a bit of perspective, I saw my oldest child's x-ray when she got her braces off at the end of summer and I could still see a suture on the x-ray of her 14yr old skull, and no dimpling.
Jay is a bit of an enigma because she has very few visible symptoms of intracranial pressure. But she does have some. The appearance of her skull is one, and another is that her skull is thinner than expected. Another is her optic nerve sheaths are measuring bigger than they should according to one specialist.
Because she is older, she may not have any issues from her craniosynostosis, but she could grow and her brain try to grow and it not have room. This could cause harm to her optic nerve which would result in loss of vision or blindness.
The plastic surgeon we consulted with first wanted to take a conservative watch and see approach. This didn't sit well with my instincts.
With the help of some wonderful parents and the CAPPS foundation we were able to pursue a second and third opinion. Our third opinion doctor took a middle of the road approach and said that the "Gold Standard" of treatment would be to do what is called intracranial pressure monitoring with a bolt monitor to see what is going on inside her skull to the best of our ability.
The plan is that on March 7th she will be having a surgery to insert an ICP monitor and do an OCT scan (ultrasound) to check her eyes. They'll decide in surgery one of four possible options.
1. No concerning ICP- remove the monitor and send us home
2. Questionable- leave ICP monitor in place and wake her up in the ICU and see how the pressure
fluctuates during a "normal" day.
3. Medium pressure- convert to a posterior CVR , which is a skull enlarging surgery using plates to expand the back of her skull.
4. High pressure- convert to a posterior skull enlargement via the use of distractors. Basically a palate expander only for her skull.
There are positives and negatives to the two different enlarging surgeries which is a topic for another day.
But this is the plan and this is how we got here.
