What is Craniosynostosis?
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.
Organizations
March of Dimes Foundation
1275 Mamaroneck Avenue
White Plains, NY 10605
askus@marchofdimes.com
www.marchofdimes.com
Tel: 914.428.7100 888.MODIMES (663.4637)
Fax: 914.428.8203
Children's Craniofacial Association
13140 Coit Road, Suite 307
Dallas, TX 75240
www.ccakids.com
Tel: 800.535.3643 214.570.9099
Fax: 214.570.8811
The Arc of the United States
1010 Wayne Avenue, Suite 650
Silver Spring, MD 20910
Info@thearc.org
www.thearc.org
Tel: 301.565.3842
Fax: 301.565.3843 or -5342
National Organization for Rare Disorders (NORD)
P.O. Box 1968
(55 Kenosia Avenue)
Danbury, CT 06813-1968
orphan@rarediseases.org
www.rarediseases.org
Tel: 203.744.0100 Voice Mail 800.999-NORD (6673)
Fax: 203.798.2291
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: 9/16/2008...#6000
