Craniosynostosis

Craniosynostosis is the premature closure of the spaces betweenthe bones that make up the skull.

What is going on in the body?

In the growing child, the skull is made up of a number of bony plates.The bony plates are separated by sutures. As a baby reaches his or her firstyear of life, the bony plates of the skull become closer and eventually fusetogether. The size and shape of the skull more or less reflect the size andshape of the brain.In a baby with craniosynostosis, one or more of the skull sutures stop growingbefore brain growth is complete. It can lead to constriction of the brain anddeformity of the skull and facial structures.

What are the causes and risks of the disease?

Craniosynostosis occurs in about 1 in 2000 live births. It occurs more oftenwith a twin pregnancy or if the shape of the uterus is abnormal and constrainsthe growth of the baby’s head. Craniosynostosis is also seen more often:

• if the mother smokes
• if the fetus is exposed to high altitude while in the womb
• if the baby has Apert syndrome, a congenital condition that causesmultiple abnormalities
• if the baby has Crouzon’s disease, a congenital disease that causesabnormalities of the face, as well as other disorders

What are the signs and symptoms of the disease?

Craniosynostosis is usually identified when a parent or healthcare providersee an asymmetry in the shape of the baby’s skull. When symptoms do occur, theymay include:

• early closure of the soft spot at the front top of the head
• a ridge in one of the suture lines that can be seen or felt
• vision and hearing problems
• slow growth of head circumference

How is the disease diagnosed?

Craniosynostosis isdiagnosed initially by noting an abnormal shape to an infant’s head. Cranial CT scans can define the skullshapeand the involved sutures.

What can be done to prevent the disease?

There is noknown prevention for craniosynostosis. Avoiding smokingand higher altitudes may decrease the risk of some forms of this condition. Acouple with a family history of congenital disorders may find genetic counseling helpful.

What are the long-term effects of the disease?

Untreatedcraniosynostosis leads to significant skull deformity. If only one suture isinvolved, there will probably be no interference with brain function. If morethan one suture is involved, brain function may be impaired.

What are the risks to others?

Craniosynostosis poses no riskto others. If there is a possibility this condition was inherited, genetic counseling may help with familyplanning.

What are the treatments for the disease?

Surgery often involves severalsurgical specialties, such as plastic and neurological surgery. It is usuallydone in multiple stages, with the first surgery done sometime in the first yearof life. Subsequent procedures are often done later in childhood.

The goals of surgery are to:

• keep the pressure inside the brain normal
• allow normal brain and skull growth
• improve vision, voice, and dental functions
• minimize cosmetic defects

What are the side effects of the treatments?

Complications of surgery to correct craniosynostosis are rare, but can include damage to the brain or eye. Surgery can also cause bleeding, infection, or allergic reaction to anesthesia.

What happens after treatment for the disease?

Follow-up will dependon the extent of the craniosynostosis and any corrective surgery. At times, further treatment and surgeries may beneeded to treat deformities of the skull. Recovery from surgery may take a few days to a few weeks.

How is the disease monitored?

Any new or worsening symptoms should be reported to the healthcare provider.