Craniofacial and Dental Features in Kabuki Syndrome Patients.

TitleCraniofacial and Dental Features in Kabuki Syndrome Patients.
Publication TypeJournal Article
Year of Publication2011
AuthorsSobral SP, Leite A, Figueiredo PT, Ferrari I, Safatle HPN, Córdoba MS, Versiani BR, Acevedo AC, Mestrinho HD
JournalCleft Palate Craniofac J
Date Published2011 Oct 24
ISSN1545-1569
AbstractAbstract Objective: To describe oral manifestations in Brazilian individuals with Kabuki Syndrome (KS), a multiple congenital anomaly/mental retardation syndrome. Study design: Sixteen KS individuals aged between 8 to 24 years of both sexes were referred by the Department of Clinical Genetics, for oral treatment and follow-up, to the Oral Care Center for Inherited Diseases, University Hospital of Brasília, Brasilia, Brazil. Each individual underwent complete physical examination, as well as intra-oral and radiographic examinations. Results: Craniofacial and dental alterations were observed in all KS patients examined. Besides, atypical shape of the molar's crowns, occlusal convergence of premolar's crowns, and root dilaceration were also observed. Enamel diffuse opacities were observed in permanent dentition (n=10). Conclusion: A great clinical heterogeneity was observed in KS individuals in line with other previous studies in the literature. Further clinical and molecular studies are necessary in order to better understand the presence of dental anomalies in this syndrome. Keywords: Kabuki Syndrome, Craniofacial Anomalies, Dental Anomalies.
DOI10.1597/11-052
Alternate JournalCleft Palate Craniofac. J.
Citation Key1101
PubMed ID22023252