Mar 22, 2014

Craniofacial & Eyelid Update

Last week we had two important appointments with the craniofacial clinic and the occuloplastics surgeon. I've been wanting to blog but we had another round of illness hit our family (with all 3 kids missing school at various times), and I have been taking photos for Evie's school as well as adjusting to a new nurse (to replace the one who was fired in February for hitting her Ainsley in the classroom in front of a teacher and students) as well as dealing with important issues that have come up with the school . Unfortunately (or fortunately) I am not compelled to stay up late blogging like I used to. I function better when I get decent sleep.

Ainsley has had a bump on top back part of her skull, almost like a goose egg from a fall, except it isn't. She also has a depression at her left temple. Both areas have gotten worse as she's gotten older, especially in the past few months. This wasn't the first time I'd mentioned them to the surgeon. I've been concerned and was really half expecting that she would need surgery. Thankfully she doesn't. I found out that any minor bumps are unlikely to cause pressure or trauma to the brain (the brain is squishable like jello, it takes more than a small irregularity to cause a problem), and are likely irregular bone growth due to the fact that Ainsley's skull was cut apart and pieced back together like a jigsaw puzzle when she was a baby. The bone is all in different places than it was and likely the irregularity is caused by that.  Really it's amazing she looks as good as she does when you see the imaging.

They told us it is important to have her eyes dilated yearly to check for papilledema, or pressure on the eyes from the brain being under pressure. Good to know. Thankfully the ophthalmologist did that in January and her pressures were good. While I was there I discussed Ainsley's eye appearance and he could see my concern about the pinching in the corner of her left eye. We've been seeing more discharge from that eye for awhile, and the usual fix, tweezing out the inwardly rotated eyelash, didn't help.  I mentioned it again at the craniofacial appointment and it was agreed something should be done so we saw the occuloplastic surgeon. He agreed to do surgery to the left eye.

Then Tuesday night I found a large quantity of discharge that seemed to out come from behind her eyeball. I've never seen anything like it, ever. The next day it appeared she was far more able to open her eye. I have no idea for sure what was going on but suspect there must have been a foreign object in there for quite some time. It is hard to see because the opening of her eyes was made overly small during surgery (her palprebal fissure was shortened). None of the doctors fully looked inside her eye despite my report of unusual discharge, I presume because of how difficult it is to pry them open. Now I am concerned that the surgery we have planned may "over-do" things and I feel like it would be smart to go back to the surgeon to give him the opportunity to see her again before planning the surgery. I tried to write an e-mail to both surgeons and ended up spending several hours watching videos about the structure of the skull and looking at her imaging. I had no idea the skull was so complex. It left me feeling overwhelmed with even more questions. I learned that the sphenoid bone connects to the nasal cavity, and that makes me wonder if perhaps that may contribute to Ainsley's breathing difficulties since there was an abnormality of her sphenoid bone.  I decided to wait, and finish this blog post and the e-mail over the weekend. Sometimes you just have to preserve your state of mind.

Ainsley was said to be born with absence of the greater sphenoid wing bone (in her skull, but not the entire bone, just part of it) which was found when they took the MRI's to prepare for the cranial surgery. Absense of the greater wing of the sphenoid is extremely rare. However, by the time she was ready to have her second craniofacial surgery the bone had started to grow. In hindsight I believe that the sagittal craniosynostosis caused the sphenoid fontanelle to be larger than normal, just like it did the anterior fontanelle.  As you can see in the imaging below, a few months following her first surgery to open the sagittal suture all the fontanelles look quite a lot smaller. During the second craniofacial surgery bone was placed behind her eyes to keep her eyes from touching the dura around her brain. After this surgery her eyes appeared slanted and were very closed. This is likely due to the fact that bone was moved and her skin had been covering a skull that was differently shaped. Even though Ainsley' eyelid tissue was insufficient prior to surgery she was able to compensate and use her brow to fully open her eyes. After the surgery she was no longer able to.

 Sphenoid fontanelles and absence of the greater sphenoid wing.

 The sphenoid fontanelle after sphenoid growth. (Not surgically altered.)

My hope has always been that the surgeons could figure out a way to fix the results of that second craniofacial surgery. They have tried. She had brow prosthetics placed in Jan. 2010, a tarsal switch and canthopexy surgery in Dec. 2010, and a frontalis sling in Jun. 2012. The surgeries improved things some. Ainsley can function better, but her appearance is still not normal. I believe that if her eyes appeared now as they did when she was born, people would have a different impression of Ainsley and treat her differently.

In January when I was at Jack's funeral I was talking with another mom. Her son has had 30 craniofacial surgeries and was trached as well.  When I told her my child had sagittal craniosynostosis and showed her a picture she asked if she'd had surgery yet. It was a heartbreaking realization, that even an experienced craniofacial mom who knows what it is to have a child who looks different (her son was born with a hole in his face) cannot see that her work is finished, and in fact thinks it hasn't even started. It isn't a matter of her being insensitive as the surgeon suggested when I told him. It is the reality of the results from her surgeries. They do say that she has some extra skin on her eyelids that can be removed, but not until she is 14. How do I know that will fix things? I don't. How do I know that the surgery they propose doing now, won't look funny after the skin is removed 7-10 years from now? I don't.

I think that one of the hardest things about being a parent of a medically complex special needs child is making decisions for which there are no easy answers. You have to educate yourself and advocate for your child to get them the best care possible. I know many parents who have pushed doctors to perform surgery past what they were comfortable with (sometimes switching surgeons), and the outcome was exactly what their child needed.  But there is always the fear of making the wrong decision since there are no guarantees in surgery or medicine. The eyes are delicate and very important. They say the eyes are the windows to the soul. I just want to make it easier for people to see Ainsley's beauty like I do, and for her to be able to live the best life possible. That is what I always try to do. I will let you know if the surgery is going to happen or not as soon as we know.


  1. Hi Susan! I totally agree that one of the hardest things about being a parent of a medically complex special needs child is making decisions for which there are no easy answers. Careful decision making is crucial. Nonetheless, Ainsley's beauty shines and it is because of her loving family.

    Victor Peterson@


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