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

[kabuki syndrome]

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 .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 .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 .

Diseases
Validation

Diseases presenting "psychomotor retardation and dermatologic abnormalities" symptom

kabuki syndrome

You can validate or delete this automatically detected symptom