International Journal of Oral & Maxillofacial Surgery
Volume 39, Issue 6 , Pages 622-625, June 2010

Extreme oral manifestations in a Marfan-type syndrome

  • R.H. Khonsari

      Affiliations

    • Service de Chirurgie Maxillo-Faciale and Stomatologie, CHU de Nantes, France
    • Corresponding Author InformationAddress: Roman Hossein Khonsari, Service de Chirurgie Maxillo-Faciale and Stomatologie, CHU de Nantes, 1 place Alexis Ricordeau, 44000 Nantes, France. Tel.: +44 7595420252; Fax: +33 145245998.
    • ,
  • P. Corre

      Affiliations

    • Service de Chirurgie Maxillo-Faciale and Stomatologie, CHU de Nantes, France
    • ,
  • Z. Boukerma-Vernex

      Affiliations

    • Service de Chirurgie Maxillo-Faciale and Stomatologie, CHU de Nantes, France
    • ,
  • J. Schmidt

      Affiliations

    • Service de Chirurgie Maxillo-Faciale and Stomatologie, CHU de Nantes, France
    • ,
  • K. Renaudin

      Affiliations

    • Service d’Anatomie and Cytologie Pathologiques, CHU de Nantes, France
    • ,
  • C. Frayssé

      Affiliations

    • Département d’Odontologie Pédiatrique, CHU de Nantes, France
    • ,
  • M. Gayet-Delacroix

      Affiliations

    • Service de Radiologie and d’Imagerie Médicale, CHU de Nantes, France
    • ,
  • P. Khau Van Kien

      Affiliations

    • Laboratoire de Génétique Moléculaire, CHU de Montpellier and INSERM U827, France
    • ,
  • A. David

      Affiliations

    • Département de Génétique Médicale, CHU de Nantes, France

Accepted 12 January 2010. published online 08 February 2010.

Abstract 

A 12-year-old girl with an otherwise typical Marfan syndrome (Ghent criteria fulfilled) presented with highly unusual oral manifestations consisting of supernumerary teeth and severe dental crowding. Pathological examination of the supernumerary teeth revealed an elevated number of pulpoliths. No mutation in the FBN1, TGFBR1 and TGFBR2 genes was identified despite exhaustive screening, suggesting that another gene defect could explain this association of marfanoid features with dental abnormalities.

Key words: marfan syndrome, Loeys–Dietz syndrome, dental crowding, supernumerary teeth, pulpoliths, FBN1, TGF-beta sub-unit receptors

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PII: S0901-5027(10)00009-3

doi:10.1016/j.ijom.2010.01.006

International Journal of Oral & Maxillofacial Surgery
Volume 39, Issue 6 , Pages 622-625, June 2010

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