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Oculo-facio-cardio-dental (OFCD) syndrome

Oculo-facio-cardio-dentales (OFCD) Syndrom

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Abstract

The association of facial dismorphy, congenital cataracts, microphthalmia, heart disease, and dental radiculomegaly is very rare. We describe a girl with atrial septal defect, unilateral congenital cataract, unilateral microphthalmia, radiculomegaly of incisor and canine teeth with open apices and other dental crown anomalies. This combination of symptoms clearly represents a distinct syndrome and has recently been described as oculo-facio-cardio-dental (OFCD) syndrome.

Zusammenfassung

Die Kombination von fazialer Dysmorphie, kongenitalen Katarakten, Mikrophthalmie, Herzfehler und Zahnwurzelmegalie ist sehr selten. Wir beschreiben ein Mädchen mit Vorhofseptumdefekt, einseitiger kongenitaler Katarakt, einseitiger Mikrophthalmie, verlängerten Schneide-und Eckzahnwurzeln sowie verzögertem Wurzelbildungsabschluß und weiteren Zahnkronenanomalien. Diese Kombination ist als oculo-facio-cardio-dentales (OFCD) Syndrom seit kurzem bekannt geworden.

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Authors and Affiliations

  1. Center of Dental Medicine, Department of Orofacial Orthopedics and Orthodontics, University Clinic Charité, Medical Faculty of the Humboldt-University, Schumannstraße 20/21, D-10117, Berlin, Germany

    Charlotte Opitz, Tatjana Dimitrova & Karin Fasmers-Henke

  2. Institute for Human Genetics, University Clinic Charité, Medical Faculty of the Humboldt-University, D-10117, Berlin, Germany

    Denise Horn

  3. Department of Radiology, Neuroradiology Unit, University Clinic Charité, Medical Faculty of the Humboldt-University, D-10117, Berlin, Germany

    Rüdiger Lehmann

Authors
  1. Charlotte Opitz
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  2. Denise Horn
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  3. Rüdiger Lehmann
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  4. Tatjana Dimitrova
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  5. Karin Fasmers-Henke
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Opitz, C., Horn, D., Lehmann, R. et al. Oculo-facio-cardio-dental (OFCD) syndrome. J Orofac Orthop/Fortschr Kieferorthop 59, 178–185 (1998). https://doi.org/10.1007/BF01317179

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  • DOI: https://doi.org/10.1007/BF01317179

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