Giant canine with dentine anomalies in oculo-facio-cardio-dental syndrome

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• 作者：Matthieu Larhant^a ; Sophie Sourice^b ; Fanny Grimaud^a ; Luis Cordoba^c ; Sophie Leveau^d ; Pascal Huet^e ; Pierre Corre^a ; Roman Hossein Khonsari^a ; ^{roman.khonsari@kcl.ac.uk" class="auth_mail}
• 关键词：Macrodontia ; Radiculomegaly ; Oculo-facio-cardio-dental syndrome ; BCOR ; Dentine ; Pulp stone
• 刊名：Journal of Cranio-Maxillofacial Surgery
• 出版年：June 2014
• 年：2014
• 卷：42
• 期：4
• 页码：321-324
• 全文大小：1757 K

文摘

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.