Craniosynostosis is a birth defect of the brain characterized by the premature closure of one or more of the fibrous joints between the bones of the skull (called the cranial sutures) before brain growth is complete. Closure of a single suture is most common. In contrast to normal skull growth, in which the skull expands uniformly to accommodate the growth of the brain, premature closure of a single suture restricts the growth in that part of the skull and promotes growth in other parts of the skull where sutures remain open. This results in an abnormal shape of the skull, but does not prevent the brain from expanding to a normal volume. However, when many sutures close prematurely, the skull cannot expand to accommodate the growing brain, which leads to increased pressure within the skull and impaired development of the brain. Craniosynostosis can be gene-linked, or caused by metabolic diseases, such as rickets or an overactive thyroid. Some cases are associated with other disorders such as microcephaly (abnormally small head) and hydrocephalus (excessive accumulation of cerebrospinal fluid in the brain). The first sign of craniosynostosis is an abnormally shaped skull. Other features can include signs of increased intracranial pressure, developmental delays, or mental retardation, which are caused by constriction of the growing brain. Seizures and blindness may also occur.
Is there any treatment?
Treatment for craniosynostosis generally consists of surgery to improve the symmetry and appearance of the head and to relieve pressure on the brain and the cranial nerves. For some children with less severe problems, cranial molds can reshape the skull to accommodate brain growth and improve the appearance of the head.
What is the prognosis?
The prognosis for craniosynostosis varies depending on whether single or multiple cranial sutures are involved or other abnormalities are present. The prognosis is better for those with single suture involvement and no associated abnormalities.
What research is being done?
The NINDS conducts and supports a wide range of studies that explore the complex mechanisms of early neurological development. The knowledge gained from these fundamental studies provides the foundation for understanding how this process can go awry and offers hope for new ways to treat and prevent brain birth defects, including craniosynostosis.
March of Dimes Foundation
1275 Mamaroneck Avenue
White Plains, NY 10605
Toll-free: 888.MODIMES (663.4637)
Children's Craniofacial Association
13140 Coit Road, Suite 307
Dallas, TX 75240
The Arc of the United States
1825 K Street, NW, Suite 1200
Washington, DC 20006
National Organization for Rare Disorders (NORD)
55 Kenosia Avenue
Danbury, CT 06813-1968
Voice Mail: 800.999-NORD (6673)
Source: National Institutes of Health; National Institute of Neurological Disorders and Stroke
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This information is provided by the Cleveland Clinic and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition. This document was last reviewed on: 9/16/2011...#6000