Craniosynostosis is the premature closure of the spaces between the 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. At birth, the bony plates are separated by slight gaps ("sutures"), which allow the head to grow in size. As a baby reaches his or her first year of life, the bony plates of the skull become closer and eventually fuse together. The size and shape of the skull approximately reflect the size and shape of the brain.
In a baby with craniosynostosis, one or more of the skull sutures closes before brain growth is complete. It can lead to constriction of the brain and deformity 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 often with a twin pregnancy or if the shape of the uterus is abnormal and constrains the growth of the baby's head. Craniosynostosis is also seen more often:
- if the mother smokes
- if the mother spends time at high altitudes while pregnant
- if the baby has Apert syndrome, a congenital condition that causes multiple abnormalities
- if the baby has Crouzon's disease, a congenital disease that causes abnormalities of the face, as well as other disorders
What can be done to prevent the disease?
There is no known prevention for craniosynostosis. Avoiding smoking and higher altitudes may decrease the risk of some forms of this condition. A couple with a family history of congenital disorders may find genetic counseling helpful.
How is the disease diagnosed?
Craniosynostosis is diagnosed initially by noting an abnormal shape to an infant's head. Cranial CT scans can define the skull shape and the involved sutures.
Long Term Effects
What are the long-term effects of the disease?
Untreated craniosynostosis leads to significant skull deformity. If only one suture is involved, there will probably be no interference with brain function. If more than one suture is involved, brain function may be impaired.
What are the risks to others?
Craniosynostosis poses no risk to others. If there is a possibility this condition was inherited, genetic counseling may help with family planning.
What are the treatments for the disease?
Surgery often involves several surgical specialties, such as plastic and neurological surgery. It is usually carried out in multiple stages, with the first surgery done sometime in the first year of life. Subsequent procedures are often needed 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
Complications of surgery to correct craniosynostosis are rare, but can include damage to the brain or eye. Surgery can also be complicated by bleeding, infection, or an allergic reaction to the anesthetic.
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 depend on the extent of the craniosynostosis and any corrective surgery. At times, further treatment and surgeries may be needed to treat deformities of the skull. Recovery from surgery may take a few days to a few weeks.
How is the disease monitored?
Regular measurements of the head circumference are important to track the child's progress. Skull X-rays are also helpful. The best view of the skull is from the top looking down (a "bird's eye" view). If the ears are asymmetrical, that is, if one ear is moved forwards or backwards, there is a need for further examination and follow-up. Any new or worsening symptoms should be reported to the healthcare provider.
Current Pediatric Diagnosis&Treatment, Hathaway, et al, 1993