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Saethre-Chotzen syndrome (SCS), also known as acrocephalosyndactyly type 3 (ACS III) and Chotzen syndrome,[1] is a very rare autosomal dominant[2] congenital disorder characterized by acrocephalosyndactyly, craniosynostosis (premature closure of one or more of the sutures between the bones of the skull). It is caused by mutations in the TWIST transcription factor (TWIST) gene.[2][3]