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Craniofacial and dental characteristics of Kabuki syndrome: nine years cephalometric follow-up.
J Clin Pediatr Dent. 2012 Summer; 36(4):393-400.JC

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.

Authors+Show Affiliations

Department of Pedodontics, Istanbul University, Faculty of Dentistry, Istanbul, Turkey. ebtuna@istanbul.edu.trNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

23019839

Citation

Tuna, Elif Bahar, et al. "Craniofacial and Dental Characteristics of Kabuki Syndrome: Nine Years Cephalometric Follow-up." The Journal of Clinical Pediatric Dentistry, vol. 36, no. 4, 2012, pp. 393-400.
Tuna EB, Marşan G, Gençay K, et al. Craniofacial and dental characteristics of Kabuki syndrome: nine years cephalometric follow-up. J Clin Pediatr Dent. 2012;36(4):393-400.
Tuna, E. B., Marşan, G., Gençay, K., & Seymen, F. (2012). Craniofacial and dental characteristics of Kabuki syndrome: nine years cephalometric follow-up. The Journal of Clinical Pediatric Dentistry, 36(4), 393-400.
Tuna EB, et al. Craniofacial and Dental Characteristics of Kabuki Syndrome: Nine Years Cephalometric Follow-up. J Clin Pediatr Dent. 2012;36(4):393-400. PubMed PMID: 23019839.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Craniofacial and dental characteristics of Kabuki syndrome: nine years cephalometric follow-up. AU - Tuna,Elif Bahar, AU - Marşan,Gülnaz, AU - Gençay,Koray, AU - Seymen,Figen, PY - 2012/10/2/entrez PY - 2012/10/2/pubmed PY - 2012/10/25/medline SP - 393 EP - 400 JF - The Journal of clinical pediatric dentistry JO - J Clin Pediatr Dent VL - 36 IS - 4 N2 - 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. SN - 1053-4628 UR - https://www.unboundmedicine.com/medline/citation/23019839/Craniofacial_and_dental_characteristics_of_Kabuki_syndrome:_nine_years_cephalometric_follow_up_ L2 - http://jocpd.org/doi/10.17796/jcpd.36.4.u021164272805116?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub=pubmed DB - PRIME DP - Unbound Medicine ER -