Craniosynostosis

Craniosynostosis results from the premature closing of one or more of the sutures of bones in the skull. CT scans can help determine if the abnormal skull shape is craniosynostosis, or simply a result of fetal head position or birth trauma.

Children with craniosynostosis may have the following problems:

• Abnormal skull shape
• Abnormal forehead
• Asymmetrical eyes and or ears
• Intracranial pressure (pressure inside the skull) which can cause delays in development or permanent brain damage if not corrected

Types

There are four types of craniosynostosis:

Scaphocephaly

The most common type of craniosynostosis, scaphocephaly is caused by the fusion of the sagittal suture, which runs from front to back down the middle of the top of the skull. Characteristics include a long, narrow shape from front to back, narrow from ear to ear, and a boat-shaped appearance.

Trigonocephaly

Trigonocephaly is caused by fusion of the metopic suture, which runs from the top of the head down to the middle of the forehead and towards the nose. Characteristics include triangular-shaped forehead and eyes that are abnormally close together.

Plagiocephaly

Anterior plagiocephaly is the premature fusion of one of the coronal sutures, which extend from ear to ear and over the top of the head. The forehead and brow appear pushed backwards, and the eye on the affected side is shaped differently than the eye on the unaffected side.

• Poster plagiocephaly is a form of craniosynostosis that causes flatness on the affected side of the head. As a result, it is often confused with deformation plagiocephaly – a condition that does not require surgical treatment. For more information on differentiating the conditions, see "Craniosynostosis and Deformational Plagiocephaly: How to Differentiate the Conditions."

Brachycephaly

Brachycephaly results when both sides of the coronal sutures fuse prematurely. Characteristics include a wide-shaped head with a short skull. The fusion prevents the entire forehead from growing in a forward direction, creating a tall and flattened appearance.

Treatment

With the exception of very mild cases, babies born with craniosynostosis require surgery between four and eight months of age. Only one surgery is usually required to separate the sutures, reshape the bones and place them in the proper position. About 10 percent of children will need a second surgery. Prior to surgery, a comprehensive CT scan in our Advanced Imaging Center helps surgeons plan the reconstruction and explain the procedure with the child’s family.

Genetic Considerations

In some families, craniosynostosis appears to be an inherited trait caused by a genetic mutation. So far, however, research cannot confirm this. The likelihood that a second child will also have the condition is slim – about four percent at the highest. There is also a small chance that a child born with craniosynostosis would have children with the condition.

When craniosynostosis is a part of Crouzon Syndrome (www.crouzon.org) or Apert Syndrome (www.apert.org), both congenital conditions, there is a 50 percent chance the condition – and its associated craniosynostosis – will pass from parent to child.

 

Also see: Center for Craniofacial Services | Craniofacial Surgery