Dental evaluation of Kabuki syndrome patients.

TitleDental evaluation of Kabuki syndrome patients.
Publication TypeJournal Article
Year of Publication2009
AuthorsTeixeira CS, Silva CRL, Honjo RS, Bertola DR, Albano LMJ, Kim CA
JournalThe Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial Association
Date Published2009 Nov
KeywordsAbnormalities, Multiple, Adolescent, Anodontia, Child, Cleft Palate, Craniofacial Abnormalities, Dental Caries, Diagnosis, Differential, Female, Humans, Male, Phenotype, Radiography, Panoramic, Syndrome

Kabuki syndrome is a genetic disorder of unknown etiologyi characterized by mental retardation, growth deficiency, and peculiar face (i.e., long palpebral fissuresi, eversioni of the laterali third of the lower eyelids, prominent ears, and broad and depressed nasali tip). Oral manifestations commonly observed in Kabuki syndrome may comprise cleft lip/palate, bifidi tongue and uvulai, malocclusioni, and dental abnormalities. We evaluated the dental findings of eight patients with Kabuki syndrome. One presented cleft palatei; three presented cariesi; and seven had missing teeth, with the upper lateral incisorsi and inferior central incisors being the most commonly absent. All missing teeth were permanent, and there was no alteration of dental chronology or morphology. Because most patients had mixed dentition, the presence or absence of primary teeth was assessed through the parents' reports. One patient presented an absent upper canine, which had not been reported previously in the literature. Dental findings may be helpful for clinical diagnosis, or they may be an additional finding to substantiate the diagnosis of Kabuki syndrome in children with mild phenotypei.

Alternate JournalCleft Palate Craniofac. J.
Citation Key398