What Is Craniosynostosis?

What is Craniosynostosis

(Crane -- ee--oh--sin--oh---stow--sis)

When I first began my own research just the sight of that big long word scared the hell out of me ~ how could my child have something I couldn't even pronounce? Its great you found my website...I will try to teach you what you don't already know.

Craniosynostosis happens one in every 2000 births.

 Many pediatricians and even parenting magazines quickly try to dismiss Craniosynostosis as a rare condition. I can, however break it down for you in a way that makes it appear more common.

Approximately 216000 babies are born every day in the world

There are 24 hours in a day

= 9000 babies born hourly

Now take into account one in 2000 born are affected with Craniosynostosis

In a 24 hour period 108 babies could be born with Craniosynostosis

= approximately 5 babies born hourly with craniosynostosis

So, if five babies born every hour of every day could have been born with Craniosynostosis, why is this condition not more actively warned about and talked about within the medical community?  This is the same question many parents, such as myself, have asked over and over again. There are still too many stories like my own where more than one doctor ignored a parent's concerns on their infant's head shape. It still seems as though not enough attention is paid to this condition on the professional side. I have seen some improvements over the last few years and much more knowledge is available to you parents on the web then what was available to us nine years ago. We have taken a few huge steps for the sake of awareness and knowledge but we are still miles away from the finishing line.

Definition of Craniosynostosis:

Craniosynostosis is the premature closing(fusing) of one or more of the boney gaps in an infant's skull. These fibrous boney gaps are known as sutures.
In a normal infant who is not affected by Craniosynostosis these sutures remain open throughout the first two years of life to allow proper brain growth. As 80% of the brain growth occurs rapidly in the first 18 months of life.

Is Craniosynostosis Cosmetic?

No! You might hear this word mentioned by others that have not researched craniosynostosis and might not understand why an abnormal head shape is such a big concern. To understand why Craniosynostosis is worrisome you need to know possible lifetime consequences to not correcting craniosynostosis.

• Learning delays can result from intracranial pressure(ICP) i
• Eyesight problems
• Hearing delays,
• Mouth, speech, and feeding issues
• Hydrocephalous(an abnormal collection of fluid on the brain which can lead to pressure if untreated.)
• SeizuresChiari Malformation
• Profound psychological effects in childhood and through adulthood due to looking different
• Death(rare but in cases of severe ICP it is a possibility)

If a doctor or insurance company tries to throw the term cosmetic your way rember one thing: According to the American Medical Association the definition of a cosmetic procedure is one that changes a normal structure of the body in order to improve appearance. While a reconstructive procedure is performed on an abnormal structure of the body to improve function, or return it to normal.

What are the warning signs of Craniosynostosis:

The most common sign of craniosynostosis is an irregularly shaped head. Although, many infants are born with an abnormal head shape, due to the trip through the narrow birth canal, most will correct themselves within six weeks following the birth. When an abnormal head shape persists or is not noticed until after six weeks, it is important to determine the cause.  Another sign of  craniosynostosis is a palpable ridge along the skull where the suture has closed.(Ridging and/or an abnormal skull could be positional plagiocephly or simply a natural ridging.) If a single suture is fused your only symptom might be the irregularly shaped head.

However, Craniosynostosis is usually more severe when more than one suture closes too early. This significantly restricts the skull's ability to expand as the brain grows. Intracranial pressure becomes worrisome with multi-suture closure. Warning signs of pressure can include Vomiting, becoming sluggish, sleeping more, and playing less. Irritability because of head pain. Developing swollen eyes or problems moving the eyes or following objects. Problems hearing. Breathing noisily or have periods of not breathing (apnea). When the pressure is very severe, it may cause brain damage and other problems, including seizures, blindness, and developmental delays. Untreated craniosynostosis may cause lasting disabilities.

"I think My Child has Craniosynostosis, what should I do?"

Many professionals don't give enough credit to mother's for their intuition. If your gut is telling you something just isn't right then more than likely something isn't.  First step is to discuss with your child's physician your concerns. With most insurances you will need a referral to see the proper specialist. However, your child's physician is not trained specifically to diagnose either craniosynostosis nor positional plagiocephaly. 

How Is Craniosynostosis Diagnosed?

An appointment with an Experienced Craniofacial Specialist needs to be made. More than likely CT Scans & MRIs need to be ordered. However, as more is becoming understood about Craniosynostosis some Craniofacial specialist can tell upon physical assessment if your child's condition is in fact craniosynostosis.
"Wait MRIs? CT scans? I discussed my concerns with my child's physician and they want to send for an x-ray."
Although X-rays will show if sutures are present they will not show the suture in enough clarity to catch if fusion has begun or not. Only a craniofacial specialist should be allowed to diagnose or dismiss craniosynostosis.

X-rays don't always catch the fused suture only a CT scan read by a craniofacial professional can determine if the sutures are fused or open.

"My Child Has Craniosynostosis, What's the Treatment?"

I hated reading this when my own daughter was first diagnosed but I must say it....  

 Surgery is the only cure for craniosynostosis.

Some mild cases of craniosynostosis are monitored and may never need surgery. However, Craniosynostosis is a progressive deformity(which means the awkward head shape will only get worse as the suture continues to fuse and the brain continues to grow.)
Surgery on your child is a very scary concept but, honestly, these children bounce back fast. Some children only need one initial surgery while other children could require more. It all depends on the severity of the suture fusion and how your child grows.

Go HERE to read about Types of Craniosynostosis

Go HERE to read about types of Surgeries for Craniosynostosis