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CASE REPORT
Year : 2011  |  Volume : 2  |  Issue : 3  |  Page : 211-214

Crouzon's syndrome: A review of literature and case report


1 Departments of Pedodontics and Preventive Children Dentistry, Vyas Dental College and Hospital, Jodhpur, Rajasthan, India
2 Department of Pedodontics and Preventive Dentistry, A.B Shetty Memorial Institute of Dental Sciences, India

Correspondence Address:
Vivek Padmanabhan
Department of Pedodontics and Preventive Children Dentistry, Vyas Dental College and Hospital, Jodhpur, Rajasthan
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0976-237X.86464

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Crouzon's syndrome is an autosomal dominant disorder with complete penetrance and variable expressivity. Described by a French neurosurgeon in 1912, it is a rare genetic disorder. Crouzon's syndrome is caused by mutation in the fibroblast growth factor receptor 2 (FGFR2) gene. Normally, the sutures in the human skull fuse after the complete growth of the brain, but if any of these sutures close early then it may interfere with the growth of the brain. The disease is characterized by premature synostosis of coronal and sagittal sutures which begins in the first year of life. Case report of a 7 year old boy is presented with characteristic features of Crouzon's syndrome with mental retardation. The clinical, radiographic features along with the complete oral rehabilitation done under general anesthesia and preventive procedures done are described.


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