What is Craniosynostosis?

CraniosynostosisCraniosynostosis is a birth defect in which one or more of the joints between the bones of your infant’s skull close prematurely, before your infant’s brain is fully formed. When your baby has craniosynostosis, his or her brain can’t grow in its natural shape and the head is misshapen.

Symptoms:

  • A misshapen skull, with the shape depending on which of the cranial sutures are affected.
  • An abnormal feeling or disappearing “soft spot” (fontanel) on your baby’s skull.
  • Slow or no growth of the head as your baby grows.
  • Development of a raised, hard ridge along affected sutures.
  • Increased pressure within the skull (intracranial pressure).

Cause:

The cause of craniosynostosis is unknown. However, there’s a hereditary component to craniosynostosis when it occurs with certain genetic syndromes, such as Apert’s syndrome and Crouzon syndrome. Besides misshapen heads, infants with genetic syndromes may have seizures, blindness, developmental delays and mental retardation.

Complications:

Babies with craniosynostosis, particularly those with an underlying syndrome, may develop increased pressure inside the skull (intracranial pressure). Their skulls don’t expand enough to make room for their growing brains.

  • Blindness
  • Seizures
  • Brain damage
  • Death, in rare instances

Treatments:

  • Traditional surgery:
    The surgeon makes an incision in your infant’s scalp and cranial bones, then reshapes the affected portion of the skull. Sometimes plates and screws, often made of material that is absorbed over time, are used to hold the bones in place. Surgery, which is performed during general anesthesia, usually takes hours.
  • Endoscopic surgery:
    This less invasive form of surgery isn’t an option for everyone. But in certain cases, the surgeon may use a lighted tube (endoscope) inserted through one or two small scalp incisions over the affected suture. The surgeon then opens the suture to enable your baby’s brain to grow normally. Endoscopic surgery usually takes about an hour, causes less swelling and blood loss, and shortens the hospital stay, often to one day after surgery.