Craniosynostosis

Craniosynostosis

If your baby has been diagnosed with craniosynostosis, some of the bones in his or her skull have fused together earlier than normal. Craniosynostosis literally means "fused bones of the skull."


Children are born with joints in the cranium, known as sutures. These allow the skull to grow as the child grows. Once the child stops growing, the sutures will fuse together to form a solid bone structure. 


It is important to keep in mind that craniosynostosis occurs in varying degrees, and not all children with craniosynostosis will have complications. The concern with craniosynostosis is that it can cause problems with normal brain development and skull growth. Typically, as a baby grows, the brain rapidly increases in size, pressing on the sutures, allowing the skull to grow passively with the brain. If, however, one area of the skull is already fused, another area will take on the growth, resulting in an abnormally shaped skull. In extreme cases, the brain growth is restricted and is squeezed. This restriction can lead to localized or widespread pressure inside the skull, which can cause delays in development or permanent brain dysfunction.

Your child’s doctor may have used the terms plagiocephaly, brachycephaly, trigonocephaly, or scaphocephaly. These terms simply refer to which sutures are involved in the craniosynostosis. 

  • Plagiocephaly: Fusion of the right or left side of the coronal suture, which extends from ear to ear over the top of the head.
  • Brachycephaly: Fusion of the coronal suture on both the left and right side.
  • Trigonocephaly: Fusion of the metopic suture, which runs from the top of the head, down the middle of the forehead, toward the nose. 
  • Scaphocephaly: Fusion of the sagittal suture, which runs from the front to the back, down the middle of the head.

Causes of Craniosynostosis

As a parent, it can be very distressing to see your child’s skull growing abnormally and you will want to know why this is happening to your child.  



Research has not been able to definitively explain why craniosynostosis occurs, but rest assured there is no correlation between anything that might have happened in the pregnancy, nor could anything have been done to prevent the defect. 

In most cases, craniosynostosis happens by chance. Plagiocephaly, the most common form of craniosynostosis, occurs in 1 out of 2,500 live births. Males are affected twice as often as females.

In some families, craniosynostosis is inherited. If the defect is autosomal recessive, then both parents need to carry the gene and there is a 25 percent chance of passing the defect on to your child. If the defect is autosomal dominant, then only one gene is necessary and there is a 50 percent chance of the child acquiring craniosynostosis.

Craniosynostosis can be a feature of many different genetic syndromes. It is important for your child and family members to be examined for signs of an inherited genetic disorder in which craniosynostosis is a symptom.

It is natural to wonder if you will have another child with craniosynostosis. If craniosynostosis was not inherited, there is a very small chance of having a second child with the same birth defect. In fact, with most cases of craniosynostosis, there is only a 0 to 4 percent chance that you will a child with this defect. 

It is also natural to wonder if your child’s children will inherit the condition. If not genetically inherited, your child’s chance of having a baby with craniosynostosis is only 0 to 4 percent.

Features of Craniosynostosis

If your pediatrician suspects that your child has craniosynostosis, he or she will refer you to a craniofacial surgeon. The craniofacial surgeon will perform a clinical evaluation of your child and may also use a radiologic evaluation, such as a CT scan, for a diagnosis. 



In a clinical evaluation, some signs that our surgeons at Yale Craniofacial Program will look for in your child are:
  • Abnormally shaped head or increasing circumference
  • A small ridge of bone that runs along the skull in different locations
  • Early closure of the soft spot
  • A full or bulging soft spot 
  • Sleepiness (or less alert than usual)
  • Scalp veins that may be very noticeable
  • Increased irritability and crying
  • Poor feeding
  • Projectile vomiting
  • Seizures
  • Bulging eyes 
  • Inability to look upward with the head facing forward
  • Developmental delays
This list should be used as a guideline. Not every sign of Craniosynostosis is included. If your child has one or more of these findings, it does not mean that he or she has craniosynostosis. If you are concerned that your child might have craniosynostosis, please make an appointment with your child's doctor.

Using 3D CT imaging, we can provide a diagnosis more quickly and with more accuracy than ever before. Traditional CT scans provide black and white, one-dimensional scans, while advanced 3D imaging technology provides colored, multidimensional images that allow us to see the specific characteristics of your child’s craniofacial differences. In this way, we can better understand how your child’s birth defect will specifically impact his or her health and development for an initial evaluation.

Treatments for Craniosynostosis

We know that having a child with craniosynostosis can be distressing, and the idea of your child needing surgical treatment can be overwhelming.

Not all children with craniosynostosis will need treatment. Each of the types occurs in varying degrees. For example, in the mildest forms, only a small ridge on the skull will result in virtually no abnormal skull shape. This condition can be monitored.

If your child does need surgery, the best results are obtained when surgical treatment is performed between 3 and 6 months old. In most cases, only one surgery will be needed. Children generally recover from surgery much more quickly than adults do. In fact, most children feel only a little pain from craniosynostosis surgery, often needing no more than acetaminophen for a few days.

Our team of surgeons at the Yale Craniofacial Program offers surgical treatment for craniosynostosis with specialists from 18 clinical services. With some of the top surgeons in the world, you can expect the latest breakthroughs in treatment and among the best care your child can receive.

During the planning stage, we may advise the use of the most advanced 3D imaging techniques for a complete understanding of your child’s specific condition and how your child’s soft and bony tissues are affected. By gathering accurate data about your child’s craniofacial differences, we can:
  • Simulate the outcome of proposed surgical plans and adjust any discrepancies before surgery
  • Follow and monitor your child’s condition during the post-surgical healing process
  • Monitor your child’s long-term development