Craniofacial and dental characteristics of Kabuki syndrome: nine years cephalometric follow-up.

TitleCraniofacial and dental characteristics of Kabuki syndrome: nine years cephalometric follow-up.
Publication TypeJournal Article
Year of Publication2012
AuthorsTuna EB, Marşan G, Gençay K, Seymen F
JournalJ Clin Pediatr Dent
Volume36
Issue4
Pagination393-400
Date Published2012 Summer
ISSN1053-4628
KeywordsAbnormalities, Multiple, Anodontia, Cephalometry, Child, Preschool, Craniofacial Abnormalities, Face, Follow-Up Studies, Hematologic Diseases, Humans, Male, Malocclusion, Angle Class III, Maxillofacial Development, Open Bite, Palate, Hard, Prognosis, Tooth Abnormalities, Vestibular Diseases
Abstract

OBJECTIVE: Kabuki syndrome is a rare autosomal dominant trait with variable clinical expression. Common dental manifestations of Kabuki syndrome are high-arched palate, micrognathia, malocclusion, microdontia, small dental arches, hypodontia, severe maxillary recession and mid-facial hypoplasia. STUDY DESIGN: This report includes the oral manifestations of a Turkish patient with Kabuki syndrome with consideration of the long-term craniofacial prognosis for this patient based on the physical, clinical and radiological findings in 9 years follow-up period. General appearance of the patient was characterized by postnatal growth retardation, moderate mental retardation, peculiar face characterized by long palpebral fissures with eversion of the lateral third of the lower eyelids, prominent and cup-shaped ears, broad and depressed nasal tips, short fifth fingers, psychomotor retardation and dermatologic abnormalities. RESULTS AND CONCLUSION: Cephalometric analysis revealed skeletal open bite; periapical and panoramic radiographic examinations showed agenesis permanent teeth. A patient with Kabuki syndrome, who may lead to a better understanding of the abnormalities, playing an important role in clinical diagnosis, planning and dental management is presented.

Alternate JournalJ Clin Pediatr Dent
Citation Key1210
PubMed ID23019839