CraniosynostosiS
It's a word we hoped we wouldn't have to hear in relation to the birth and growth of our son. Unfortunately, we heard that word from a pediatrician 2 days into our son, Dexter's, life.
Before we go any further, let's explain what craniosynostosis is.
Craniosynostosis is a big, scary, medical term for the premature fusing of the sutures in the skull. When you are born there are 4 sutures in your skull that separate the bones into plates; the metopic, coronal, sagittal, and lambdoidal. These sutures allow for the plates to move around. This allows for the baby to be born naturally (it would be hard to push a 13in. circle through a 10cm hole without the 13in. circle being able to be made smaller!) It also allows for the proper growth and development of the brain (as the brain grows the skull needs to expand to make room for it). With craniosynostosis, one or more of the sutures prematurely fuses causing the skull to grow incorrectly as the brain grows. The way in which the skull grows is determined by which sutures have fused. These sutures can fuse at anytime, most of the time it is within the first year of life. But wait, you say, don't these sutures fuse anyway, what's the problem? The problem is that the metopic suture closes between 3 and 9 months and the coronal, sagittal, and lambdoidal fuse between 22 and 39 years of age. Think about what would happen to a brain if an adult had a head the size of a one year old, it wouldn't be good. So what do you do about this? The answer is almost always surgery. There are 2 surgeries available, open vault and endoscopic. With both surgeries a section of skull over the fused suture is removed. With open vault, the surgeon will manually move the plates around until the desired skull shape is achieved. With endoscopic, the child wears a helmet to reshape the skull.
Before we go any further, let's explain what craniosynostosis is.
Craniosynostosis is a big, scary, medical term for the premature fusing of the sutures in the skull. When you are born there are 4 sutures in your skull that separate the bones into plates; the metopic, coronal, sagittal, and lambdoidal. These sutures allow for the plates to move around. This allows for the baby to be born naturally (it would be hard to push a 13in. circle through a 10cm hole without the 13in. circle being able to be made smaller!) It also allows for the proper growth and development of the brain (as the brain grows the skull needs to expand to make room for it). With craniosynostosis, one or more of the sutures prematurely fuses causing the skull to grow incorrectly as the brain grows. The way in which the skull grows is determined by which sutures have fused. These sutures can fuse at anytime, most of the time it is within the first year of life. But wait, you say, don't these sutures fuse anyway, what's the problem? The problem is that the metopic suture closes between 3 and 9 months and the coronal, sagittal, and lambdoidal fuse between 22 and 39 years of age. Think about what would happen to a brain if an adult had a head the size of a one year old, it wouldn't be good. So what do you do about this? The answer is almost always surgery. There are 2 surgeries available, open vault and endoscopic. With both surgeries a section of skull over the fused suture is removed. With open vault, the surgeon will manually move the plates around until the desired skull shape is achieved. With endoscopic, the child wears a helmet to reshape the skull.
Craniosynostosis is a fairly common disorder with 1 in every 2,500 births worldwide being diagnosed. However, not much is known about the disorder, including the cause.
Craniosynostosis can also be a symptom of a larger syndrome. The most common syndrome being Crouzon's Syndrome, which has been shown to have a genetic component.
Okay, your science lesson is over for today, now onto the more human part of our story...
Craniosynostosis can also be a symptom of a larger syndrome. The most common syndrome being Crouzon's Syndrome, which has been shown to have a genetic component.
Okay, your science lesson is over for today, now onto the more human part of our story...
We Start at the very beginning...
It's a very good place to start. My name is Erika and I am married to the most amazing man, Eric. We were married about a year and a half before we decided we wanted to expand our family. Eric was diagnosed with craniosynostosis when he was born and we were concerned about having a baby. We didn't want to bring a baby into the world with the disorder if we could help it. We went to my OBGYN and asked her if she knew anything about craniosynostosis. She checked with a geneticist who told her that there was no known genetic cause of craniosynostosis (we later found this to not be true). With this information, we decided to go ahead and try and start a family. We found out we were having a child on November 4th, 2013 and were beyond excited. Of course we were still slightly nervous about the possibility of our child having the disorder.
I had a healthy and normal pregnancy. No problems and our baby (a boy!!!) was growing and developing great! We were both anxiously awaiting the day our little Dexter would be born.
When it came to looking for pediatricians, we always asked the same question, "Have you dealt with craniosynostosis and what are the steps you would take to diagnose it?" Every answer was the same, "Yes" and "We would refer you to a specialist at Children's Hospital." We ended up deciding on a pediatrician that had rights to the hospital we would be delivering at. We wanted someone who knew our history to see our baby as soon as possible after birth in order to determine if he had craniosynostosis or not.
When it came to looking for pediatricians, we always asked the same question, "Have you dealt with craniosynostosis and what are the steps you would take to diagnose it?" Every answer was the same, "Yes" and "We would refer you to a specialist at Children's Hospital." We ended up deciding on a pediatrician that had rights to the hospital we would be delivering at. We wanted someone who knew our history to see our baby as soon as possible after birth in order to determine if he had craniosynostosis or not.
Dexter was due on July 15th, 2014. He was 3 days late and I went into labor early on Friday, July 18th, 2014. It was a pretty rough labor and I ended up having a C-section due to failure to progress. Dexter was born at 8:32pm a healthy 8lbs 13oz and 20in. long. His head shape was slightly abnormal, but the nurses said his head shape would look more normal in a few days and was due to his failure to progress.
Saturday a pediatrician came in and didn't mention Dexter's head shape at all. We thought maybe he was fine. The next day another pediatrician came in and said that dreaded word, "craniosynostosis" and on top of that she said another word that we weren't expecting "Crouzon's." She left those with us and said we should keep an eye on his head shape. We didn't see our pediatrician until Monday and he told us we should get an appointment scheduled with the neurosurgery team at Children's within the next week.
It was all I could do to look at my perfect little 3 day old and not cry. All I could think about was the possible surgeries he would have to have within the next few months and how much pain he would be in, it was (still is) horrifying. I couldn't even let him get circumcised without balling, how was I going to make it through a 3 to 5 hour skull surgery?
We made it home and the next couple weeks were a whirlwind of doctors' appointments. We ended up with the diagnosis of Sagittal Craniosynostosis with possible Crouzon's Syndrome, a genetic disorder with craniosynostosis as one of the symptoms. We have scheduled surgery for November 6th, 2014.
Saturday a pediatrician came in and didn't mention Dexter's head shape at all. We thought maybe he was fine. The next day another pediatrician came in and said that dreaded word, "craniosynostosis" and on top of that she said another word that we weren't expecting "Crouzon's." She left those with us and said we should keep an eye on his head shape. We didn't see our pediatrician until Monday and he told us we should get an appointment scheduled with the neurosurgery team at Children's within the next week.
It was all I could do to look at my perfect little 3 day old and not cry. All I could think about was the possible surgeries he would have to have within the next few months and how much pain he would be in, it was (still is) horrifying. I couldn't even let him get circumcised without balling, how was I going to make it through a 3 to 5 hour skull surgery?
We made it home and the next couple weeks were a whirlwind of doctors' appointments. We ended up with the diagnosis of Sagittal Craniosynostosis with possible Crouzon's Syndrome, a genetic disorder with craniosynostosis as one of the symptoms. We have scheduled surgery for November 6th, 2014.
I am writing all of this and the subsequent blog mainly as a way to cope with all of this. If anybody reads this; I hope that you can learn more about this disorder and if you are going through the same thing, I hope it can bring you comfort and support.
We will be updating our timeline and blog as things progress. Thank you for following our journey!
~ Erika, Eric & Dexter
We will be updating our timeline and blog as things progress. Thank you for following our journey!
~ Erika, Eric & Dexter