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

Eduardo Kazuo Sannomiya, S. A B Reis, Jun-Ichi Asaumi, J. V L Silva, A. S. Barbara, K. Kishi

Research output: Contribution to journalArticle

11 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)119-124
Number of pages6
JournalDentomaxillofacial Radiology
Volume35
Issue number2
DOIs
Publication statusPublished - Mar 2006
Externally publishedYes

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Acrocephalosyndactylia
Anatomic Models
Skull
Syndactyly
Foot
Hand
Craniofacial Dysostosis
Nasal Bone
Hypertelorism
Synostosis
Three-Dimensional Imaging
Malocclusion
Palate
Spiral Computed Tomography
Nylons
Skull Base
Maxilla
Tomography
Bone and Bones

Keywords

  • Acrocephalosyndactyly
  • Apert's syndrome
  • Craniosynostoses
  • Syndactyly
  • Tomography, computerized X-ray

ASJC Scopus subject areas

  • Otorhinolaryngology
  • Radiology Nuclear Medicine and imaging
  • Dentistry(all)
  • Radiological and Ultrasound Technology

Cite this

Clinical and radiographic presentation and preparation of the prototyping model for pre-surgical planning in Apert's syndrome. / Sannomiya, Eduardo Kazuo; Reis, S. A B; Asaumi, Jun-Ichi; Silva, J. V L; Barbara, A. S.; Kishi, K.

In: Dentomaxillofacial Radiology, Vol. 35, No. 2, 03.2006, p. 119-124.

Research output: Contribution to journalArticle

Sannomiya, Eduardo Kazuo ; Reis, S. A B ; Asaumi, Jun-Ichi ; Silva, J. V L ; Barbara, A. S. ; Kishi, K. / Clinical and radiographic presentation and preparation of the prototyping model for pre-surgical planning in Apert's syndrome. In: Dentomaxillofacial Radiology. 2006 ; Vol. 35, No. 2. pp. 119-124.
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