- Premature fusion of the cranial sutures (the fibrous joints between the bones of the skull) in an infant, preventing normal growth of the baby's head. Craniosynostosis involving some but not all of the sutures causes an abnormally shaped skull. Premature closure of all of the sutures results in microcephaly (an abnormally small head) which arrests the normal growth and development of the baby's brain and may result in developmental delay and mental retardation. Early detection of the condition is therefore of great importance. Treatment is surgery designed to keep the sutures open. Craniosynostosis can occur as an isolated abnormality or as part of a syndrome. A number of craniosynostosis syndromes are now known to be due to mutations in the FGFR genes (FGFR = fibroblast growth factor receptor).
The FGFR-related craniosynostosis syndromes include established entities (the Apert, Crouzon, and Pfeiffer syndromes) and several newer entities (the Beare-Stevenson, Muenke, and Jackson-Weiss syndromes). All are inherited in an autosomal dominant manner. Affected individuals have a 50% chance of passing the mutant gene to each child. Prenatal testing is available.
* * *Premature ossification of the skull and obliteration of the sutures. The particular sutures involved determine the resultant shape of the malformed head. SYN: craniostosis.
* * *cra·nio·syn·os·to·sis -.sin-.äs-'tō-səs n, pl -to·ses -.sēz or -to·sis·es premature fusion of the sutures of the skull
* * *n.premature fusion of some of the cranial bones, usually before birth, so that the skull is unable to expand in certain directions to assume its normal shape under the influence of the growing brain. Depending on which cranial suture fuse early, the skull may become elongated from front to back, broad and short, peaked (oxycephaly or turricephaly), or asymmetrical. Compare craniostenosis.
* * *cra·nio·syn·os·to·sis (kra″ne-o-sin″os-toґsis) [cranio- + syn- + ostosis] premature closing of the sutures of the skull of an infant, causing deformities such as oxycephaly and scaphocephaly. Called also craniostosis.
Medical dictionary. 2011.
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Craniosynostosis — Classification and external resources Child with premature closure (craniosynostosis) of the lambdoid suture. Notice the swelling on the right side of the head ICD 10 … Wikipedia
craniosynostosis — or craniostosis Cranial deformity produced when the bones of the skull fuse too early. Pressure from the growing brain normally causes the skull bones to grow along the seams (cranial sutures) between them. If all the sutures fuse early, the head … Universalium
craniosynostosis — n. premature fusion of some of the cranial bones, usually before birth, so that the skull is unable to expand in certain directions to assume its normal shape under the influence of the growing brain. Depending on which cranial sutures fuse early … The new mediacal dictionary
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craniosynostosis — noun Etymology: New Latin Date: 1951 premature fusion of the sutures of the skull … New Collegiate Dictionary
craniosynostosis — noun a medical condition in which some or all of the sutures in the skull of an infant close too early, causing problems with normal brain and skull growth. Syn: cloverleaf skull, cloverleaf skull syndrome, kleeblattschadel … Wiktionary
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clinocephaly — Craniosynostosis in which the upper surface of the skull is concave, presenting a saddle shaped appearance in profile. SYN: saddle head. [clino + G. kephale, head] * * * cli·no·ceph·a·ly (kli″no sefґə le) [clino + cephaly] congenital… … Medical dictionary