NORD gratefully acknowledges Robert F. Keating, MD, Professor and Chief, Department of Neurosurgery; President, Medical Staff, Children's National Medical Center, Washington, DC, for assistance in the preparation of this report.
Primary craniosynostosis is a general term for the improper development of the bones of the skull, which can result in an abnormal head shape in affected individuals. Craniosynostosis refers to the premature fusion of the fibrous joints (sutures) between certain bones of the skull. The severity of primary craniosynostosis can vary from one person to another. Raised intracranial pressure is unlikely with single suture abnormalities and thus intelligence is usually unaffected. Primary craniosynostosis may occur as an isolated finding or as part of a syndrome. Patients with syndromic conditions generally have more than one suture involved. Not surprisingly, the therapeutic options and outcomes are dependent upon the degree of suture involvement. The main treatment for primary craniosynostosis is surgery, but not all affected children will require surgery. The exact cause of primary craniosynostosis is unknown, although the skull abnormalities may result from the abnormal hardening (ossification) of the cranial sutures. Primary craniosynostosis is distinguished from secondary craniosynostosis, which occurs because of a primary failure in brain growth which in turn may lead to abnormalities in head shape, occasionally mimicking craniosynostosis.
Please note that some of these organizations mat provide information concerning certain conditions potentially associated with this disorder.
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