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Apert syndrome

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Author(s): Premalatha | Kannan V | Madhu

Journal: Journal of the Indian Society of Pedodontics and Preventive Dentistry
ISSN 0970-4388

Volume: 28;
Issue: 4;
Start page: 322;
Date: 2010;
Original page

Keywords: Craniosynostosis | fibroblast growth factor receptor 2 gene | midface hypoplasia | synda

ABSTRACT
Apert syndrome (acrocephalosyndactyly) is a rare developmental malformation characterized by craniosynostosis, mid-face hypoplasia, symmetrical syndactyly of hands and feet. The prodromal characteristics for the typical cranio-facial appearance are early craniosynostosis of the coronal suture, cranial base and agenesis of the sagittal suture. The purpose of this paper is to report a case of Apert syndrome with emphasis on craniofacial and oral features in an eighteen-month-old male child. The patient presented with several craniofacial deformities, including brachycephaly, midface hypoplasia, flat face, hypertelorism, ocular proptosis, downslanting palpebral fissures. Syndactylies with osseous fusion of the hands and feet were also observed. Intraoral findings included delayed eruption of teeth, high arched palate with pseudo cleft in the posterior one third.
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