Neurosurgery - Patient Care and Treatment

Learn about craniosynostosis causes, diagnosis and treatment from the Pediatric Neurosurgery team at Penn State Children’s Hospital.

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Why do some children have abnormal head shapes?

Quick answers from, Mark Dias, MD, Director, Pediatric Neurosurgery, Penn State Children’s Hospital. 

What is craniosynostosis?

Craniosynostosis is a congenital (present at birth) skull deformity that happens when the skull sutures (fibrous joints) between various skull bones fuse improperly and prematurely.

Skull and brain growth is limited at the fused sutures, and growth at the remaining sutures results in an abnormally shaped skull.

Craniosynostosis is usually noticed in infancy. The child may have an abnormal but characteristic head shape, depending on how many and which sutures are involved. In most cases, the problem is just cosmetic and leads to an abnormal head shape. Sometimes, the growth of the facial bones is affected as well.

When skull growth is restricted enough to cause increased pressure in the head, it can lead to:

  • Headaches
  • Vision loss
  • Developmental delays

The severity of the deformity depends on:

  • Which sutures close
  • At what point in development the closure occurred
  • The success or failure of the other sutures to compensate by expanding

What causes craniosynostosis?

Most often, there is no family history or other known cause of craniosynostosis. Sometimes craniosynostosis is inherited and can be associated with other developmental problems.

How is craniosynostosis diagnosed?

A doctor can usually tell by examining the child's head. A radiological exam is usually needed to confirm the problem, characterize the deformity and guide the corrective surgical procedure.

  • X-rays of the skull usually show the problem well.
  • Computerized tomography (CT) scans give more information about the fused sutures and the status of the underlying brain.
  • 3D CT scans may provide information to guide surgical correction.

How is craniosynostosis treated?

Craniosynostosis can be treated with endoscopic (through a small incision) or open surgery, depending on the child’s health, age, medical history and type of craniosynostosis. Surgery is generally recommended if the closed sutures have caused pressure on the brain or marked skull deformity.

  • The fused suture(s) can be removed and the skull remodeled into a more natural shape.
  • Surgery is often performed by a neurosurgeon and a craniofacial plastic surgeon working together.
  • In more severe cases, surgery may be done in stages for the best result.
  • Ideally surgery should be done before the child is one year old since the bones at that age are are well-suited for intervention.

In cases where the head shape is abnormal, but the sutures have not closed, a child’s head may be reshaped without surgery using a cranial molding helmet.

  • This specially fitted helmet uses the child's own head growth to help reshape the skull by allowing growth in areas that appear flattened and restricting growth in areas that are more prominent.
  • The helmet is usually worn almost constantly for weeks or months.
  • It’s most successful in younger infants. Once the child approaches one year of age, the rate of head growth slows and the helmet therapy is less successful.