Cranio - Syno - What - O?

Pierscen won the lottery!!!

The odds? 1 in 2000

The prize? Some quality 1-on-1 time with the Craniofacial surgical team at Primary Children's Medical Center just shortly after he turns 4 months.

The reason? Baby P was born with craniosynostosis.

Uh... what is that? Craniosynostosis is where one (or more) of the sutures in an infant's skull fuses prematurely. This usually does not happen until around the age of 2 giving the brain adequate time to grow and develop. When these plates fuse prematurely it causes the head to be misshaped and may inhibit proper brain growth. There is no known cause and the only treatment is surgery.

There are several types of craniosynostosis. Below is a picture outlining the four main types: Metopic, Sagittal, Coronal, and Lambdoid. Pierscen has Unilateral Coronal Synostosis (one sided). The picture below is a perfect demonstration because it is the same side as Pierscen's.


The front right and side bones are fused near the temple. The result of this is that the right side if his forehead is recessed and the left side bulges out more. The right eye socket is also is pulled back so it is "longer" than the left side, making his right eye appear larger. It's not larger, of course. He can just open it wider. His nose is also slightly crooked. In short, this type of synostosis creates an asymmetrical look. That being said, it sure sounds like it would be obvious, right? Not really. In fact, unless you know it's there, most people don't notice a thing.


I mean, look at that cute chubby face! Not exactly what comes to mind when you mention "misshaped" and "asymmetrical."

I love this picture. Man, oh man, is he a sweet little boy. But this shows things a little bit better, though still not completely. It's far more obvious from the birds eye view. But here you can see the left forehead is bulged out a bit and the right eye looks larger than the left.


We have actually known about his condition since a few hours following birth. While Ryan was down in the nursery with him, he was feeling his head to see if he could feel all the sutures. (I personally don't know any other father that would do that... but I am grateful he did. In fact, later when I asked him why he did so [meaning, did he felt impressed to do so] he responded that he wasn't sure. Only that his first thought was to check his head before he even looked to see if he had all his fingers and toes.) Ryan noticed that he couldn't feel that particular suture. He mentioned to the nurses that he thought Pierscen's head was a little funny shaped. They laughed and said, "Honey, he was just born." He replied, "I know, I know. I am sure you get that all the time. But he was a c-section baby. It shouldn't be that bad." This gave them pause, but they still maintained that by the time we left the hospital that it would be totally normal. It was then that he told them he couldn't feel that particular suture. That got their attention. They came over and checked it and finding that they couldn't feel it either, called over the charge nurse. She too couldn't feel it. So she made a note in his chart for the pediatrician to check it when he made his rounds in the morning. Ryan came back to the room and googled "fused skull" and came up the diagnosis. We read up on the condition as well as the treatment for it and had already come to terms by the time the pediatrician came in. He told us that everything looked great. But when he paused, Ryan added, "Except his head?" To which he responded, "That's the only thing..." He ordered an x-ray, and later a CT. By the end of the day, the diagnosis was officially confirmed.


What we learned from our research is that the surgery involves opening the scalp, separating the fused plates, and then placing the child in a helmet for up to a year while the skull reshapes. We couldn't find a definite timeline for when they would operate, but we assumed they would at least wait until he was six weeks old. It was amazing to see before and after photos on some of the websites. The change in many cases was immediate! Our pediatrician told us he would send a referral to the neurosurgeon team at Primary's and they would be in touch.


The following week we called up to Neurosurgery to make sure they had received the referral. The answer was no. So we called our pediatrician and asked them to send one up. After a couple of days we called Primary's again. This time the referral had made it, but we were told that after they had reviewed his case, due to his age we would actually need to go through the Craniofacial team. They couldn't say what about his age made that determination. Just his age. Unfortunately they couldn't just send our info to the Craniofacial team. We would need to contact our pediatrician again and have him send a separate referral. Luckily we had an appointment with our doctor the next day, so we just asked him to send one when we saw him. We told him about the change of team and he didn't seem surprised. But he had no answer for the whole age thing. He seemed just as baffled by it as we had been. A few days following that appointment we called the Craniofacial surgeon's office and were able to secure an appointment the following week - and in Provo none the less. We were really excited that we wouldn't have to drive up to Salt Lake just yet. Our doctor has an office at UVRMC that he comes to once a month and we made it in just in time.


Turns out Craniofacial (which we had never heard of up to this point) is basically plastics. At first I was a little concerned about this. Considering the surgery involves cutting open the skull and all that, I thought Neurosurgery made a lot more sense. But then the more I thought about it, I realized that no - plastics is more logical. Especially for his type of synostosis. It literally is reconstructive surgery. He nose will need to be fixed, his brow straightened out, the eye socket pulled down, and of course the suture separated... yes, plastics made sense. We were told when we called the Craniofacial office that it is pretty much always the craniofacial doctor that does the surgery, but that they work hand in hand with the neurosurgeon and that they would be there as well. Age had nothing to do with it.


We met with Dr. Morales on a Wednesday morning, bright and early. The nurse that took us back told us that Morales has done hundreds of these cases and that we were in very good hands. As we first began talking, he seemed surprised to see how young Pierscen was (only two weeks) and wanted to know how we had figured out what he had so early. I explained that it was Ryan who had found it originally and had gotten the ball rolling. (The fact that he was surprised by his age confirmed to me that had Ryan not been the one to find it, there was a very good chance we would not have discovered it until a much later date. Ryan kept saying he was sure the pediatrician would have caught it the next morning if he hadn't. I am not so sure.) Dr. Morales said he assumed we had already researched it a bit if we were proactive enough to figure out the diagnosis before the clinical staff had. We said yes, and told him what we knew about the surgery and the helmet. He nodded at our telling of things, but then told us that that particular method was "kind of outdated." We were more than surprised to hear this as that was pretty much all we could find online - and that information was coming from the medical websites.


He gave us a booklet on craniosynostosis that outlined the condition, how to prepare for surgery, and what to expect from the surgery. Included were some pictures showing how they would proceed. They will have to shave a strip of hair from ear to ear. Then they will cut the scalp in a zig-zag or wavy line. This is so that when the hair grows back, it should fall over the scar in a way that hides it much better. If it was just a straight line across the scalp, it would be much harder for the scar to be hidden. Makes sense! And there is another good reason the plastic surgeon is doing it. Plastic surgeons are all about minimal scaring! Once the scalp has been pulled back, they will separate the sutures, reconstruct the brow, eye socket, and nose, and shift all the plates around where they want them. Then instead of putting the child in a helmet, they connect everything with dissolvable plates and screws. Dissolvable! They take about a year to disappear completely, but there should be no reason for a helmet (since the plates and screws are basically an internal helmet.) How amazing is that?! Modern medicine I tell you! It is simply amazing! He explained that the reason he steers away from the helmet is because there is no guarantee that it will work. You can separate the sutures and do everything else you are going to do, but there is no guarantee that the helmet will be able to get things were they need to be.



The picture above is from the booklet he gave us. You can see the wavy incision. But even more amazing is the before and after comparison. Note that the child is still on the operating table. The head shape is already fixed! That is so incredible! They say that it only takes about a week and the child is feeling as good as new. What a blessing. Children are so resilient! They spend 3-5 in the hospital split between the PICU and the Surgical Ward. It is common for one or both of the eyes to swell shut and become black and blue, but that, of course will go away with time. But once they are eating well and things are looking good, they get to go home. Dr. Morales said this surgery is typically done between 4-8 months of age. Old enough for them to be able to handle the physical maneuvering of the plates, but young enough that they aren't too mobile yet and in danger of lots of falls and head bumps. He said that we would aim for 5-6 months for Pierscen, just depending on when his schedule and the neurosurgeon's schedule would work together. In the mean time, he told us to set up an appointment with the pediatric ophthalmologist to check to see if his eye muscle is weakening. (Not vision, but eye muscle due to the elongation of the socket.) If it is, then they can fix it when they go in. They just have to know beforehand. He told us we would also need to set up one more consult with him closer to the surgery date as well as with the neurosurgeon. At these check ups they would make sure that his head is still growing and that things are on track for surgery. His secretary/assistant said that she would actually arrange these appointments for us so that we could see them on the same day up in Salt Lake and save us another trip. She said their schedules are very hectic and to save us the headache of calling back and forth between them, she would call us with the appointment dates as well as the surgery date.


It seemed like an eternity before I heard from her, but she got it all figured out. May 3rd is the surgery date. He is the first surgery of the day and she emphasized the need to keep him healthy. Even so much as a runny nose and they might not operate and then it could be months before we could get another date. May 3rd puts him at 4 months 1 week old. I was surprised at that since I wasn't expecting anything until 5 or 6 months, but we are also excited and relieved to have it done even sooner. Our consult appointments are set for March 7th. With that in mind I was able to call the ophthalmologist and set up an appointment. The doctor they referred me to is leaving the practice so the doctor taking over was pretty booked, but they were able to squeeze me in March 2nd. It will be great to have all of these appointments over (including his 2 month pediatric appointment) in the next few weeks. It is a relief to at least have everything lined up and ready so we can move forward.


So here we are. It has been an interesting adventure. Neither of us are worried about the surgery even though in reality it kind of is a big thing. (Opening the skull and playing tetris with the plates... yeah I would say that's a major surgery. Most adults are frightened by head surgery and here is this little helpless boy...) I am grateful that we both work at the hospital. I think it has definitely helped us feel confident in the medical world and the doctors that will be doing the surgery. So I don't worry about the surgery itself. I have cried thinking about the eyes swelling shut and him not being able to see mom and dad. I have cried thinking about the pain he will experience. And I have cried about losing his hair. But all of these things are so minor. Even though I had never heard of Craniosynostosis, and the resulting surgery is a bit overwhelming (especially financially... so grateful we have insurance. Could you ask for a more expensive surgical team [plastics AND neurosurgery]?! I doubt it...) in the grand scheme of things, we are really lucky. He will have the surgery, be back to his normal self within a week or two, his hair will grow back, and the plates and screws will dissolve. And that's it! It's over and done with. No one will ever know. The only lasting proof will be the scar. He could have had some disease or illness that we would be fighting his whole life. There is no limit to the possibilities of what could have happened and what would have been worse. We are lucky. And oh so blessed. We are lucky to have Primary's so close. People come from all over the states to this hospital to have this surgery done. And we just have an hour drive. Very blessed indeed.


About a month ago now, Ryan received a phone call from our neighbor who he home teaches, and who is also my visiting teaching companion. She asked if he would be willing to come over and give her and her husband a blessing as they had just learned that their youngest daughter had... you guessed it... craniosynostosis, and they were set to have surgery in 2 weeks. After learning about the condition, I had actually suspected that her daughter might have it too based on her head shape (though hers is the sagittal one - fused down the middle.) I called to talk to her, curious about how she came to find out considering her youngest is 5 months old and knowing that our surgery date was set 4 months in advance - the fact that theirs was set for 2 weeks said to me that they must have just barely found out about it. Sure enough, it wasn't until her 4 month old appointment that the doctor finally decided that the head shape wasn't going to fix itself and that there might be something wrong. He sent her to Primary's where the youngest, and the second to youngest were both diagnosed with it. The older girl is old enough now that the skull has already fused completely, so surgery is kind of out of the question unless she starts developing too much pressure on the brain. They will just watch her closely for a while. As for the younger girl, she needed the surgery. What I didn't know was that the surgery that involves the helmet is done laparoscopically. They make a couple small incisions and then use a scope to separate the fused sutures. Then they put the child in a helmet and hope that the helmet will mold the head into the correct shape. (So when my doctor said that technique was "outdated" it was really more of a preference for him because even though the technique they are going to use on Pierscen is much more "invasive" it allows them to have more control and manipulate things they way they want to.) The laparoscopic technique is typically done when the child is still pretty young. My neighbor's girl was right on the border of that time line. Her doctor felt that the laparoscopic method would likely still work, so they opted to try that first. She will be in a helmet for a year. If that doesn't fix it, then they will need to go and do the other surgery. The surgery took place almost two weeks ago now and she is doing really well. It's kind of nice to know someone personally that has gone through this so we can kind of know what to expect. It is just interesting that for the odds to be 1 in 2000, we have not one, but 3 in our ward alone - 2 of which are in the same family.


As I have researched online, it sounds like most parents don't find out about the condition until the child is a few months old. Likely it is because many pediatricians may not be familiar with it, and most feel that misshaped heads will fix themselves (as they usually do) following birth. So once again, I really don't know that Pierscen's condition would have been picked up in the hospital. I am sure we would have found out about it eventually, and our pediatrician has had other patients with it so maybe... but it was been a blessing to know from the get go. This has allowed us tons of time to research, learn and prepare ourselves.

I cannot say enough about modern medicine! I am so grateful to live in this day and age where medicinal miracles happen every day. I am so thankful to my loving Heavenly Father for leading mankind on his quest for knowledge. I am grateful to know that because of the amazing technologies that exist I will not have to worry that my son's brain will not be able to grow or that he will be ostracized my other children because he "looks different." Of course, this surgery won't shield him from a whole world of difficulties, but at least that shouldn't be one of them. And hopefully, with a little coaching from his parents, he will grow up thinking his scar is totally awesome and I am sure other kids will think so too. But most important, I want him to grow up knowing he has a Heavenly Father that loves him very much and a Brother that died for him, making it possible for us to be a family forever. I love this boy more than I can say. I am the luckiest girl in the world to have him.

I will keep you updated as things progress. I am sure it will be here and over before we know it!