title: Craniofacial and dental characteristics of Kabuki syndrome: nine years cephalometric follow-up.
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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Pedodontics, Istanbul University, Faculty of Dentistry, Istanbul, Turkey. ebtuna@istanbul.edu.tr
authors.forename: Elif Bahar
authors.initials: EB
authors.lastname: Tuna
authors.forename: Gülnaz
authors.initials: G
authors.lastname: Marşan
authors.forename: Koray
authors.initials: K
authors.lastname: Gençay
authors.forename: Figen
authors.initials: F
authors.lastname: Seymen
mesh_terms: D000015:Abnormalities, Multiple / Q000473:pathology*; D000848:Anodontia / Q000473:pathology; D002508:Cephalometry / Q000379:methods*; D002675:Child, Preschool; D019465:Craniofacial Abnormalities / Q000473:pathology*; D005145:Face / Q000002:abnormalities / Q000473:pathology; D005500:Follow-Up Studies; D006402:Hematologic Diseases / Q000473:pathology*; D006801:Humans; D008297:Male; D008313:Malocclusion, Angle Class III / Q000473:pathology; D008445:Maxillofacial Development / Q000502:physiology; D024343:Open Bite / Q000473:pathology; D021362:Palate, Hard / Q000002:abnormalities; D011379:Prognosis; D014071:Tooth Abnormalities / Q000473:pathology*; D015837:Vestibular Diseases / Q000473:pathology*