Craniosynostosis

What is Craniosynostosis?
The skull is made up of five major bones, or plates, that are separated by spaces called sutures. These sutures are flexible joints that allow infants to grow and develop normally, eventually closing to permanently shape the skull and protect the brain.

Craniosynostosis (craniostenosis) occurs when the sutures close too early, so the skull can no longer stretch and expand to allow for the young child's brain to grow.  This premature condition causes infants to have an abnormally shaped head and can possibly lead to developmental problems.

What Causes Craniosynostosis?
We don't know why some children have craniosynostosis (craniostenosis), but this condition occurs in approximately one out of every 2,000 children and is twice as common in boys than girls.  However, most children with this condition do not have complex cases and have only one suture affected.  After the treatment, most of these children resume normal development.

Sometimes, craniosynostosis occurs as part of a genetic syndrome.  A syndrome is a disorder that has more than one identifying feature or symptom.  Some of these syndromes, such as Crouzon, Apert, Pfeiffer and Saethre-Chotzen syndromes include craniosynostosis as part of the condition.

What Does a Baby with Craniosynostosis Look Like?
Babies with craniosynostosis (craniostenosis) generally have abnormally shaped skulls.  The shape depends on which sutures are affected.  If you are worried that your baby has an abnormal head shape, you should raise your concerns with your pediatrician.  Infants with craniosynostosis may also be very irritable, feed poorly and have other developmental delays.

In general, abnormally shaped heads are caused by craniosynostosis, or another condition called deformational plagiocephaly, a common condition that is not harmful to your child.  It is caused by infants who sleep on their backs and flatten the back of their heads.  This condition does not cause pressure on the brain and will not affect their brain development.  This is much more common than craniosynostosis and your child's pediatrician will be able to help make the diagnosis.  If they are unsure, your child may be referred to a craniofacial surgeon.  A physical exam and a computerized tomography scan (CT) can diagnose craniosynostosis.

How is Craniosynostosis Treated?
Surgery to reshape the skull is the most common treatment.  The goal is to reduce the pressure in the head and correct any abnormalities of the face or skull bones.  Infants with craniosynostosis (craniostenosis) are referred to a multidisciplinary team of doctors for further evaluation. A CT scan is done to evaluate the skull structure and the brain.  If there is no evidence of increased pressure on the scan or by physical exam, surgery will be done when the child is closer to one year of age.

When it is time for surgery, the neurosurgeon on the team removes the affected suture and the craniofacial plastic surgeon reshapes the skull bones into a more normal shape.  Special plates and screws are used to hold the shape while the bone heals and will dissolve in one or two years.

Only one incision is needed, which is made in a zigzag line from just above one ear to just above the other.  The scar will blend in very well and is usually covered completely by the hair within months of the surgery.

After the operation, you child will be cared for in the intensive care unit (ICU) for one night and then will be monitored in the neuroscience unit for three to four more days.

The surgery is typically performed before the baby is one year old, but sometimes doctors suggest just watching a child with mild craniosynostosis to see how the skull will grow.

The treatment of simple craniosynostosis may require only one surgery before the baby's first birthday.  Children with craniofacial syndromes that include craniosynostosis often need a number of surgeries to correct the problem.

Posted: April 23, 2007 For more information please contact: Neuroscience Institute , 866.705.8279