What is Craniosynostosis?

What causes craniosynostosis?

Craniosynostosis occurs in one out of 2,200 live births and affects males slightly more often than females.

Craniosynostosis is most often sporadic (occurs by chance). In some families, craniosynostosis is inherited. Craniosynostosis is a feature of many different genetic syndromes that have a variety of inheritance patterns and chances for reoccurrence, depending on the specific syndrome present.

It is important for the child as well as family members to be examined carefully for signs of a syndromic cause (inherited genetic disorder) of craniosynostosis such as limb defects, ear abnormalities, or cardiovascular malformations.

What is Craniosynostosis?

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 and act as expansion joints to accommodate the growing brain. As the infant grows and develops, the sutures close, forming a solid piece of bone, called the skull.

Craniosynostosis is a condition in which the sutures close too early, causing problems with normal brain and skull growth. Premature closure of the sutures may also cause the pressure inside of the head to increase and the skull or facial bones to change from a normal, symmetrical appearance.

Diagnosis of Craniosynostosis

What are the symptoms of craniosynostosis?

In infants with this condition, the most common signs are changes in the shape of the head and face. The appearance of the child's face may not be the same when compared to the other side.

How is craniosynostosis diagnosed?

Craniosynostosis may be congenital (present at birth) or may be observed later, during a physical examination. The diagnosis is made after a thorough physical examination and after diagnostic testing. During the examination, Dr Kelly will obtain a complete prenatal and birth history of your child. He may ask if there is a family history of craniosynostosis or other head/face abnormalities. He will also ask about developmental milestones since craniosynostosis can be associated with other neuromuscular disorders. Developmental delays may require further medical follow up for underlying problems.
During the examination, a measurement of the circumference of your child's head is taken and plotted on a graph to identify normal and abnormal ranges.

Diagnostic tests that may be performed to confirm the diagnosis of craniosynostosis include:

• x-rays of the head
• computed tomography scan (also called CT or CAT scan) of the head –A CT scan shows detailed images of any part of the body, including the bones, muscles, fat, and organs. CT scans are more detailed than general x-rays.

What are the different types of craniosynostosis?

There are numerous types of craniosynostosis. Different names are given to the various types, depending on which suture, or sutures, are involved, including the following:

Plagiocephaly

Anterior plagiocephaly involves fusion of either the right or left side of the coronal suture that runs from ear to ear. This is called coronal synostosis and it causes the normal forehead and the brow to stop growing. Therefore, it produces a flattening of the forehead and the brow on the affected side, with the forehead tending to be excessively prominent on the opposite side. 

Trigonocephaly

Trigonocephaly is a fusion of the metopic (forehead) suture. This suture runs from the top of the head down the middle of the forehead, toward the nose.
Early closure of this suture may result in a prominent ridge running down the forehead. Sometimes, the forehead looks quite pointed, like a triangle, with closely placed eyes (hypotelorism).

Scaphocephaly

Scaphocephaly is an early closure or fusion of the sagittal suture. This suture runs front to back, down the middle of the top of the head. 
This fusion causes a long, narrow skull. The skull is long from front to back and narrow from ear to ear.

Treatment Options

Management of craniosynostosis:

Specific treatment for craniosynostosis will be determined by :

  • your child's age, overall health, and medical history
  • extent of the craniosynostosis
  • type of craniosynostosis (which sutures are involved)
  • expectations for the course of the craniosynostosis
  • your opinion or preference

Surgery is typically the recommended treatment. The goal of treatment is to reduce the pressure in the head and correct the deformities of the face and skull bones. In general, the optimal time to perform surgery is before the child is 1 year of age since the bones are still very soft and easy to work with. Surgery may be necessary at a much earlier age depending upon the severity of the condition.

Before surgery, Dr Kelly will explain the operation as well as the risks of surgery to you. If you would like to speak to parents of children who have had similar operations , this can be arranged.
After surgery, most children are nursed in the Intensive Care Unit for a few days before being transferred to a general paediatric ward. After 24- 36 hours your baby’s eyes may swell closed , this is not a reason for concern but a normal response to the operation.

The health care team will inform you after surgery on how to best care for your child at home, and outline specific problems that require immediate medical attention. Read more about Craniosynostosis surgery.