What is craniosynostosis?

Craniosynostosis is a common craniofacial anomaly in children. It is the early closing of one or more of the sutures – soft fibrous seams – that separate the plates of the skull. When the sutures close too early, the skull cannot grow normally and an abnormal head shape results.

The shape of the skull of a baby with craniosynostosis varies depending upon which suture has closed. For example, when the central midline suture (the sagittal suture) closes too early, the skull cannot grow from side to side and overgrows from front to back. This results in a long and narrow head shape. A single suture is involved most often, though more than one can be involved.

Although craniosynostosis may be a component of an identifiable syndrome, it more commonly occurs as a single suture fusion. Infants usually have abnormal head shapes identified at or soon after birth, may have developmental delays, and may have associated eye findings. Early evaluation, diagnosis, and treatment planning leads to very good outcomes.

What causes craniosynostosis?

The reason craniosynostosis occurs is not known. Most babies with craniosynostosis have only one suture involved and have no other conditions or health problems.

Craniosynostosis can occur as part of a genetic syndrome, like Crouzon, Apert, Pfeiffer, or Saethre-Chtotzen. These children usually have more than one suture involved, and these syndromes may be passed in families between multiple generations. You may be aware of relatives who share the same condition. Your doctors will advise you if genetic testing is recommended for your child.

How is craniosynostosis treated?

For cases where the head is misshapen significantly, the treatment usually involves surgery. There are three reasons for surgery:

  1. Cosmetically, surgery will normalize the shape of the babies’ skull. Without intervention, the shape of the skull will not improve and may progress.
  2. While rare in cases of single suture craniosynostosis, pressure inside the skull can build, leading to neurologic problems. Expanding the skull treats or prevents this.
  3. There is an association between single suture craniosynostosis and developmental delays, especially in craniosynostosis of the metopic suture. There is some evidence that surgical treatment improves developmental outcomes.

There are two types of surgery to treat craniosynostosis. Both are involved and carry risks with each procedure.

  • Open Remodeling: In this procedure, the abnormally shaped skull bones are exposed and removed. Great care is taken to protect the brain and eyes. The bones are then molded to a normal shape, replaced, and secured with absorbable plates and screws to normalize the head shape. This surgery usually involves an ear-to-ear incision, cuts in the bone, and remodeling the shape. At the end of surgery, the shape is normalized; no helmet is needed afterward.
  • Endoscopic Strip Craniectomy: This procedure is most effective in infants less than 3 months of age with an involved sagittal sutures. Two small incisions are made and a special camera at the end of an instrument is used to visualize the fused sagittal suture. The suture is then cut out and removed. Benefits of this procedure include shorter surgical time and less blood loss. Skull reshaping is then reliant on the infant brain growing over the next 18 months. A full-time helmet must be worn to guide the reshaping of the skull.

Timing of surgery depends upon which suture is involved and the baby’s overall health and development. In general, most surgical interventions are done during the first year of life, while the skull bones are relatively soft and moldable. Most parents wish to have surgery performed as soon as the diagnosis is made. Surgery is safer and most successful when done at the proper age. Your surgeons will discuss optimal timing for your baby’s procedure. Most children need only one surgery.