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Revista da Associacao Paulista de Cirurgioes Dentistas

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Abstract

GONCALVES, Alcides Ricardo et al. Oral and craniofacial characteristics Apert syndrome: a case report. Rev. Assoc. Paul. Cir. Dent. [online]. 2015, vol.69, n.2, pp. 142-147. ISSN 0004-5276.

The Apert syndrome, also known as acrocephalosyndactyly, is a rare genetic condition characterized by the premature fusion of the cranial sutures (craniosynostosis), hypoplasy of the middle third of the face and syndactyly of the hands and feet. This study aimed to demonstrate the main oral and craniofacial characteristics of the Apert syndrome through a case report. A 4-year-old leucoderma male patient, with a diagnosis of Apert syndrome, was referred to the Clinic of Dentistry Specialization for Special Needs Patients at the Dental Research Center São Leopoldo Mandic, in Campinas, São Paulo, Brazil. On physical examination there have been noticed the presence of syndactyly of the toes and hands, craniofacial abnormalities, hypoplasy of the maxilla, inverted V shaped of the palate, with pseudocleft midline, swellings in the palatine processes and absence of the lower incisors. The results indicated that the oral craniofacial characteristics of the patient are in accordance with those reported in the literature for the Apert syndrome, and a dentist is required to identify them.

Keywords : acrocephalosyndactylia; disabled persons; pediatric dentist.

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