The normal skull consists of several plates of bone that are separated by sutures. The sutures (fibrous joints) are found between the bony plates in the head. The function of the suture is to allow molding through the birth canal and adjustments for the growing brain. As the infant grows and develops, the sutures close, forming a solid piece of bone, called the skull.
What is Craniosynostosis?
Craniosynostosis (kray-nee-o-sin-os-TOE-sis) is a birth defect in which one or more of the joints between the bones of an infant's skull closes prematurely, before your baby's brain is fully formed.
As an infant's brain grows, open sutures allow the skull to expand and develop a relatively normal head shape. If one or more of the sutures has closed early, it causes the skull to expand in the direction of the open sutures. This can result in an abnormal head shape. In severe cases, this condition can also cause increased pressure on the growing brain.
Types of Craniosynostosis:
In sagittal synostosis (scaphocephaly), the sagittal suture is closed. As a result, the infant's head does not expand in width but grows long and narrow to accommodate the growing brain. The sagittal suture is the most common single suture involved in Craniosynostosis.
When the metopic suture is closed, this condition is called metopic synostosis. You may also hear the term trigonocephaly used to describe your child's head shape. The deformity can vary from mild to severe. There is usually a ridge down the forehead that can be seen or felt and the eyebrows may appear "pinched" on either side. The eyes may also appear close together.
The coronal suture goes from ear to ear on the top of the head. Early closure of one side, unilateral coronal synostosis (plagiocephaly) results in the forehead and orbital rim (eyebrow) having a flattened appearance on that side. This gives a "winking" effect. These features may also be more apparent when looking at the child in the mirror.
Both sides are fused in bicoronal synostosis (brachycephaly). In these cases, the child may have a very flat, recessed forehead. This suture fusion is most often found in Crouzon and Apert Syndromes.
How is Craniosynostosis diagnosed?
There are several clues that may cause a parent or doctor to suspect that a child has Craniosynostosis. A misshapen head is usually the first clue. The anterior fontanelle, or soft spot, may or may not be open. The suspected diagnosis is confirmed by x-rays. A CT scan is also done to make sure there are no underlying abnormalities in the brain.
Treatment:
For most babies, surgery is the primary treatment for Craniosynostosis. The type and timing of surgery depend on the type of Craniosynostosis and whether there's an underlying syndrome that needs treatment.
The purpose of surgery is to relieve pressure on the brain, create room for the brain to grow normally and improve your child's appearance. A team that includes a specialist in brain surgery (Neurosurgeon) and a specialist in surgery of the head and face (Craniofacial Surgeon) performs the procedure.
It's very important to find doctors (Pediatrician, Pediatric Neurosurgeon and Pediatric Craniofacial Surgeon) that you feel comfortable with and have confidence in.
There's a lot of information on the internet, some correct and some false (it can be overwhelming and frightening). There are also support groups available where you can get information and support from other families that have gone or are going through the same journey. Just remember every child and situation is different and it's your team of doctors that you should rely on to answer all your questions.
Newborns with this medical condition are usually kept under the wraps but by educating parents on Craniosynostosis, more infants will have a chance to live a normal life. Remember, you are your babies only advocate!!!
