Non-syndromic craniosynostosis

Abstract Craniosynostosis is defined as the premature fusion of a skull suture and has an incidence of approximately 1:2000 live births. Historically, craniosynostosis was subdivided into primary and secondary synostosis. Further subclassifications included syndromic/non-syndromic and single/multisuture. Multisuture synostoses also give rise to characteristic head shapes, which, with experience, are readily identifiable by experienced clinicians using clinical examination alone. While in the past, single-suture craniosynostosis was classified as ‘non-syndromic’, advances in genetic analysis has shown that this may not be the case in a proportion of these cases, just as some multisuture presentations may also be non-syndromic. This particularly applies to unicoronal craniosynostosis along with some metopic and bicoronal synostosis. Given the higher rate of syndromic association in these patients, along with the tendency to require late surgery for facial scoliosis in unicoronal and unilambdoid patients, this cohort should be regarded as a more complex subset.