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J Neurosurg Pediatr. 2009 Jul;4(1):21-5. doi: 10.3171/2009.3.PEDS08355.

Evolution in the frequency of nonsyndromic craniosynostosis.

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Craniofacial Surgery Unit, Department of Pediatric Neurosurgery, Hôpital Necker-Enfants Malades, University Paris V, Paris, France.



The prevalence of the different subtypes of craniosynostosis varies greatly. The aim of this study was to analyze the prevalences of the different subtypes of craniosynostosis at a single major craniofacial center and their changes during a 20-year period.


The medical charts of 2808 children hospitalized between 1988 and 2007 for a true craniosynostosis were retrospectively reviewed. Patients were divided according to their subtype of craniosynostosis (sagittal, plagiocephaly, brachycephaly, metopic, oxycephaly, syndromic cases, or unclassifiable).


The total number of patients according to their craniosynostosis subtype were as follows: 1224 cases of scaphocephaly, 315 cases of plagiocephaly, 598 cases of trigonocephaly, 105 cases of brachycephaly, 69 cases of oxycephaly, 396 syndromic cases, and 101 unclassifiable cases. The prevalences of these craniosynostoses evolved differently over time. The number of children hospitalized each year increased over time from 106 to 181 cases, but this increase varied according to the subtype of craniosynostosis. Cases of scaphocephaly, plagiocephaly, brachycephaly, and syndromic cases increased similarly (170% increase on average), whereas trigonocephalies dramatically increased (420% increase).


The evolution observed in the prevalence of craniosynostosis might result from several factors. An increased recruitment of patients at the center and an improvement in the diagnosis of craniosynostosis might explain the overall increase. However, other mechanisms should be examined to explain the great increase in the number of cases of trigonocephaly (environmental or pharmacological).

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