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Clinical and radiographic presentation and preparation of the prototyping model for pre-surgical planning in Apert's syndrome

¹ Department of Oral and Maxillofacial Radiology, São Paulo Metodista School of Dentistry, São Paulo, Brazil; ² Department of Oral and Maxillofacial Radiology, Okayama, Japan; ³ Department of Medical and Dentistry Prototyping Rapid Models, Renato Archer Institute, Campinas, Brazil

*Correspondence to: Eduardo Kazuo Sannomiya, Department of Oral and Maxillofacial Radiology, São Paulo Metodista School of Dentistry, Av. Lacerda Franco 1180, Aclimação, São Paulo, Brazil, CEP:01536-000; Email: eduardosannomiya{at}ig.com.br

Received 17 December 2003; revised 23 May 2005; accepted 9 July 2005

Acrocephalosyndactyly, or Apert's syndrome, described nearly a century ago, is a craniofacial dysostosis, an autosomal dominant condition characterized by severe development disturbances of the craniofacial region including bilateral coronal synostosis associated with midface hypoplasia, exophthalmia, hypertelorism, symmetric syndactyly of the hands and feet, cone-shaped calvarium, pharyngeal attenuation and malocclusion. The aim of this study was to assess clinical and computed tomography (CT) imaging patterns of a non-operated patient with Apert's syndrome, correlating the cranium, face and the skull base bone abnormalities. Three-dimensional images were generated from spiral CT scans in order to produce a prototyping model in polyamide material. Clinical examination determined that syndactyly of the hands and feet, pseudocleft in the midline palate and midface hypoplasia were present. The surgical model allowed the analysis of some abnormalities regarding to calvaria morphology, nasal bones and maxilla, improving the criteria for a case diagnosis and surgical plan.

Keywords: Apert's syndrome;; acrocephalosyndactyly;; syndactyly;; craniosynostoses;; tomography; computerized X-ray