title: Giant canine with dentine anomalies in oculo-facio-cardio-dental syndrome.
abstract: Radiculomegaly affecting incisors, canines or premolars is a rare radiological finding (Maden et al., 2010) but is pathognomomic of a rare x-linked dominant syndrome called oculo-facio-cardio-dental syndrome (OFCDS). As this syndrome includes cardiac malformations and can lead to blindness due to congenital glaucoma, oral and maxillofacial surgeons should be aware of the somatic anomalies potentially associated with radiculomegaly. We report a typical case of OFCDS and provide the first description of the microscopic dental anomalies associated with this syndrome.
authors.affiliation: Service de Chirurgie Maxillo-Faciale et Stomatologie, Centre Hospitalier Universitaire Hôtel-Dieu, Nantes, France.
authors.forename: Matthieu
authors.initials: M
authors.lastname: Larhant
authors.affiliation: Laboratoire d'Ingéniérie Ostéo-Articulaire et Dentaire, INSERM U791, Nantes, France.
authors.forename: Sophie
authors.initials: S
authors.lastname: Sourice
authors.affiliation: Service de Chirurgie Maxillo-Faciale et Stomatologie, Centre Hospitalier Universitaire Hôtel-Dieu, Nantes, France.
authors.forename: Fanny
authors.initials: F
authors.lastname: Grimaud
authors.affiliation: Laboratoire de Physiopathologie de la Résorption Osseuse et Thérapie des Tumeurs Osseuses Primitives, INSERM U957, Nantes, France.
authors.forename: Luis
authors.initials: L
authors.lastname: Cordoba
authors.affiliation: Service de Chirurgie Maxillo-Faciale, Clinique Jules-Vernes, Nantes, France.
authors.forename: Sophie
authors.initials: S
authors.lastname: Leveau
authors.affiliation: Service de Chirurgie Maxillo-Faciale, Nouvelles Cliniques Nantaises, Nantes, France.
authors.forename: Pascal
authors.initials: P
authors.lastname: Huet
authors.affiliation: Service de Chirurgie Maxillo-Faciale et Stomatologie, Centre Hospitalier Universitaire Hôtel-Dieu, Nantes, France.
authors.forename: Pierre
authors.initials: P
authors.lastname: Corre
authors.affiliation: Service de Chirurgie Maxillo-Faciale et Stomatologie, Centre Hospitalier Universitaire Hôtel-Dieu, Nantes, France. Electronic address: roman.khonsari@kcl.ac.uk.
authors.forename: Roman Hossein
authors.initials: RH
authors.lastname: Khonsari
mesh_terms: D000328:Adult; D000848:Anodontia / Q000000981:diagnostic imaging; D001641:Bicuspid / Q000002:abnormalities; D002386:Cataract / Q000151:congenital* / Q000473:pathology; D054893:Cone-Beam Computed Tomography / Q000379:methods; D003481:Cuspid / Q000002:abnormalities* / Q000000981:diagnostic imaging; D003743:Dental Enamel / Q000002:abnormalities; D003804:Dentin / Q000002:abnormalities*; D005260:Female; D006343:Heart Septal Defects / Q000473:pathology*; D006801:Humans; D007180:Incisor / Q000002:abnormalities; D008850:Microphthalmos / Q000473:pathology*; D019227:Tooth Apex / Q000002:abnormalities; D014092:Tooth Root / Q000002:abnormalities* / Q000000981:diagnostic imaging
keywords: BCOR; Dentine; Macrodontia; Oculo-facio-cardio-dental syndrome; Pulp stone; Radiculomegaly