What is craniosynostosis?
Craniosynostosis is a term used to describe the premature fusion of one or more of the sutures that separate the bones of the skull. The skull of the newborn is made up of six major plates of bone that are separated by sutures. Sutures are joints between the bones where much of the skull growth occurs in response to the growing brain.
Typically, the sutures naturally close over time as skull growth is completed. In the condition known as craniosynostosis, one or more sutures may close early. Early closing of a suture causes the baby to have an abnormally shaped head. An atypical head shape occurs because the skull is unable to expand perpendicularly to the closed suture and makes up for growth in an alternative direction.
Who gets craniosynostosis?
Craniosynostosis occurs in 1 out of every 2,500 newborn babies. It is not yet known what causes non-syndromic craniosynostosis; however, it typically occurs in a non-inherited fashion.
There is a 0-4% chance that a subsequent sibling of a child with craniosynostosis will be affected. A similar risk exists for children of parents with craniosynostosis. A referral to a geneticist at Children's Hospital Colorado can help your family understand the cause and future risks related to the diagnosis of craniosynostosis.
What are the symptoms of craniosynostosis?
There are several different types of craniosynostosis. The particular head shape and diagnosis is based on which suture is involved:
- Sagittal craniosynostosis (scaphocephaly): Results in an elongated and narrow head shape. The sagittal suture runs along the top of the head from front to back and, when prematurely closed, may result in a ridge along the top of the head. The child may have a prominent forehead and occiput. This is the most common type of non-syndromic craniosynostosis, occurring in 1 of 4,200-5,000 newborn babies with a 3:1 male to female ratio.
- Unicoronal craniosynostosis (anterior plagiocephaly): Results in flattening of the affected side's upper orbit and forehead. The eye on this side may appear wider. The unaffected forehead may appear more prominent. The coronal sutures run from ear to ear and, when one side is prematurely closed, may result in a ridge along the top of the head. This type of craniosynostosis occurs in 1 in 10,000 newborn babies with a 1:2 male to female ratio.
- Metopic craniosynostosis (trigonocephaly): Results in a triangularly shaped forehead and a decreased distance between the eyes. The metopic suture runs from the top of the head down to the nose and, when prematurely closed, may result in a ridge along the forehead. This type of craniosynostosis occurs in 1 of 15,000 newborn babies with a 3:1 male to female ratio.
- Lambdoid Craniosynostosis (posterior plagiocephaly): Results in flattening of the affected side's occiput (back of head). The ear on the affected side may be displaced further back and down than the other ear. This is the least common type of non-syndromic craniosynostosis.
How is craniosynostosis diagnosed?
A clinician is often able to diagnose craniosynostosis by history and examination, but a CT scan or MRI may be ordered to confirm the diagnosis and aid in treatment planning.
How is craniosynostosis treated?
When treatment is necessary, surgery is usually the recommended approach for craniosynostosis. If your child's condition is very mild, then monitoring by our craniofacial surgery team may be recommended. In most cases, the goal of surgery is to reestablish the typical head shape. Under rare circumstances, surgery may be required to treat or prevent elevations in intracranial pressure.
The timing of surgery depends on your child's particular diagnosis and clinical picture. The most common types of craniosynostosis involve only one cranial suture.In the case of sagittal craniosynotosis, surgery may be recommended after 3 months of age. In the case of coronal or metopic craniosynostosis, surgery may be recommended between 4-9 months of age.
In some cases, craniosynostosis may be corrected by our specialists using a minimally invasive endoscopic technique, which requires two short incisions behind the patient's hairline. In these cases, we recommend surgery at 2-3 months of age.
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