Craniosynostosis can be define as a disease that affects the growth of infant cranial structures, causing permanent disfigurement. The disease prematurely fuses sutures (joints) in the skull, thereby preventing growth in certain directions. This results in abnormal growth patterns, which can have serious effects on the shape of the head and facial features, and in severe cases on the development of the brain. The limitations on the growth of the cranium in specific directions results in compensatory growth in the allowed directions, i.e. the head will grow more in the unrestricted directions in an attempt to allow sufficient space for brain growth. However, this is not always successful and the growth restrictions caused by Craniosynostosis are sometimes so severe that they cause permanent eye damage and reduced intelligence and mental ability.
Affects and Causes
Craniosynostosis affects around 0.05% of new borns, approximately 10% of which suffer from Complex Craniosynostosis – in which more than one suture is affected. Some studies have found that a restriction on space inside the womb is a significant factor in future cranial bone growth. Furthermore, smoking and the use of recreational drugs during pregnancy increase the risk of the child developing Craniosynostosis. The disease also has a strong hereditary factor, with 8% of the children affected having parents with the same condition.
Surgery is currently the only treatment option available for patients suffering from Craniosynostosis. The aim of surgical intervention is to remove as many restrictions on the growth of the cranium as possible. This is done by excising the defective sutures and repairing the damage from any uneven growth that has already taken place. In order to minimize deformity, the surgery should take place as soon as the disease has been diagnosed, ideally when the child is only a few months old. At this stage the cranium is still flexible and can be remoulded by the surgeons. The older the child gets, the more inelastic and fragile the bone diseases becomes, rendering the surgery more complicated and dangerous.