Craniosynostosis
 
 




Craniosynostosis is a congenital birth defect that causes one or more of the skull’s sutures to close prematurely. This restricts head and brain growth and surgery is required to open the fused suture. If it is not surgically repaired, it could result in developmental delays, facial abnormalities, seizures and many other long term health problems. Craniosynostosis occurs in approximately 1 in 2,000 live births.


How is Craniosynostosis Diagnosed?

A Pediatric Craniofacial Plastic Surgeon or Pediatric Neurosurgeon usually diagnoses Craniosynostosis. A 3D CT Scan will provide a definitive answer as to whether or not your child has Craniosynostosis. X-rays can be preformed, however they can also provide a false negative and are usually not very useful. After your child has been diagnosed with Craniosynostosis, your child may be referred to a Pediatric Ophthalmologist who will check your child’s eyes for pappillaedema on the optic nerve. If this is present, it is a sign that the Craniosynostosis is causing pressure on the brain and would require more urgent surgery to release the sutures.


Oftentimes, Craniosynostosis is overlooked by Pediatricians. You may hear that your child’s abnormal head shape may be due to: birth trauma, positional issues, or that head and facial asymmetries are normal. If you are concerned with your child’s head shape or appearance, ask to be referred to a Craniofacial Plastic Surgeon in your area, or get a 2nd opinion.


How is Craniosynostosis Treated?

Craniosynostosis is treated with surgery. Depending on the type of Craniosynostosis and/or severity, either an Endoscopic approach or CVR (Cranial Vault Reconstruction) is done. The optimal time to have the Endoscopic procedure done is typically before 3 months of age, whereas the CVR is typically done between 6 months and a year of age.


It is crucial to be diagnosed as early as possible in order to receive the best possible treatment.


Surgery is done by a team which includes both a Craniofacial Plastic Surgeon and a Neurosurgeon. For a Cranial Vault Reconstruction (CVR ) surgery, the head is cut in a zig-zag pattern from ear-to-ear (which allows for the scar to be more easily covered with hair in the future). The skull bone is then cut to release the fused suture. After the suture is released, the skull is then stabilized with dissolvable plates and screws. 


Subsequent surgeries are sometimes needed. Patients are followed for years to ensure there is no increased pressure on the brain and to ensure the skull is growing normally.


What are the Types of Craniosynostosis?

Click on the type of Craniosynostosis below to learn more about the different types of Craniosynostosis:

Coronal

Metopic

Sagittal

Lambdoid


What causes Craniosynostosis?

The cause of Craniosynostosis can be genetic, however usually the cause is unknown and there is no family history of Craniosynostosis.


NOTE: This site is not intended to diagnose or give medical advice.  If you suspect your child has Craniosynostosis, please contact your Pediatrician, a Craniofacial Plastic Surgeon or a Pediatric Neurosurgeon in your area.

 

Types of Craniosynostosis

Right Coronal

Left Coronal

Metopic

Sagittal

Lambdoid

Multiple Fused Sutures

What is Craniosynostosis?

Characteristics of craniosynostosis

may include:

Abnormal Head Shape

Facial Asymmetry

Absence of Soft Spot

Small Soft Spot

Triangular Head Shape

Elongated Head Shape

Narrow Head Shape

Ridge on Head

Flattening on Forehead

Increased Forehead Height

Bulging Eye

Widening of Skull

Recession of Eye

Frontal Bossing