Türk Nöroşirürji Dergisi 2021 , Vol 31 , Num 1
Syndromic Craniosynostoses
Abdurrahim TAŞ1,Abdurrahman AYCAN1
1Yüzüncü Yıl Üniversitesi Tıp Fakültesi, Beyin ve Sinir Cerrahisi Kliniği, Van, Türkiye Early closure or absence of one or more of the cranial sutures can be defined as craniosynostosis. It can be divided 2 groups as syndromic and non-syndromic. Syndromic craniosynostoses constitute 20-30% of all craniosynostoses. Syndromic craniosynostoses usually have problems in more than one suture. They are also hereditary forms of craniosynostosis associated with various extracranial dysformisms such as cardiac, facial, extremity, central nervous system and tracheal malformations. The genetic etiology of syndromic craniosynostoses is still only partially explained today. In addition, the diagnosis and treatment of these patients is quite complex. A multidisciplinary approach is required for an effective treatment. The most common genetic syndromes associated with craniosynostoses, their characteristics, syndrome-specific problems, and treatment recommendations are briefly summarized in the review. Anahtar Kelimeler : Craniosynostosis, Syndromic, Genetics, Children, Fibroblast growth factor receptor (FGFR), Apert syndrome, Crouzon syndrome