[Syndrome of craniosynostosis with syn- or polydactylia]

Since the first description of the acrocephalosyndactyly syndrome, type Apert, in 1906, numerous entities belonging to this group of disorders have been defined. Recently several families with various types of craniosynostosis have been published. Moreover, most of the types of acrocephalosyndactylia have been observed with polydactyly, rendering exact diagnosis more and more difficult. Hence it seems necessary to revise the classification of these disorders. The paper gives a survey of the craniosynostosis syndromes and a classification for genetic counseling is proposed.