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Abstract #54033 Published in IGR 15-2
Giant canine with dentine anomalies in oculo-facio-cardio-dental syndrome
Larhant M; Sourice S; Grimaud F; Cordoba L; Leveau S; Huet P; Corre P; Khonsari RHJournal of cranio-maxillo-facial surgery : official publication of the European Association for Cranio-Maxillo-Facial Surgery 2014; 42: 321-324
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.
Service de Chirurgie Maxillo-Faciale et Stomatologie, Centre Hospitalier Universitaire Hôtel-Dieu, Nantes, France.
Full articleClassification:
9.4.15 Glaucoma in relation to systemic disease (Part of: 9 Clinical forms of glaucomas > 9.4 Glaucomas associated with other ocular and systemic disorders)
9.1.4 Other (Part of: 9 Clinical forms of glaucomas > 9.1 Developmental glaucomas)
