Craniosynostosis-Revisited

• Main Authors: Sunanda Bhatnagar, Vasavi Krishnamurthy, Sonal Vahanwala, C D Nayak, S S Pagare
• Format: Article
• Language: English
• Published: Wolters Kluwer Medknow Publications 2007-01-01
• Series: Journal of Indian Academy of Oral Medicine and Radiology
• Subjects: Craniosynostosis - FGFR gene; Syndactily; Polydactily
• Online Access: http://www.jiaomr.in/article.asp?issn=0972-1363;year=2007;volume=19;issue=4;spage=482;epage=491;aulast=Bhatnagar;type=0
• ISSN: 0972-1363; 0975-1572

We all take special care when holding a tiny baby. This is partly because we know that "babies" head is particularly vulnerable, as it is still ′soft′ and the protective skull is yet forming. Skull growth continues until late adolescence and its proper functioning is crucial. Craniosynostosis, an inherited genetic condition, is characterized by the premature closure of sutures of the skull with effects that are wide - ranging and potentially devastating. Normally sutures and fontanelles allow the bones of the cranial vault to overlap during birth thus acting as an expansion joint, enabling the bone to enlarge evenly as the brain grows resulting in a symmetrically shaped skull. However, craniosynostosis occurs due to mutation in Homeobox gene - MSX2 and ALX4 or Fibroblast growth factor receptors (FGFR 1,2,3) gene, thus explaining for its association with Apert, Crouzon, Chotzen, Pteiffers and carpenter syndromes.