Craniofacial and dental characteristics of Kabuki syndrome

• Published in: American journal of medical genetics Vol. 98; no. 2; p. 185
• Main Authors: Matsune, K, Shimizu, T, Tohma, T, Asada, Y, Ohashi, H, Maeda, T
• Format: Journal Article
• Language: English
• Published: United States 15.01.2001
• Subjects: Adolescent; Adult; Bone and Bones - abnormalities; Cephalometry - methods; Child; Developmental Disabilities - diagnosis; Face - abnormalities; Facies; Female; Humans; Intellectual Disability - diagnosis; Male; Radiography; Skin Abnormalities - diagnosis; Syndrome; Tooth Abnormalities - diagnosis; Tooth Abnormalities - diagnostic imaging
• ISSN: 0148-7299
• DOI: 10.1002/1096-8628(20010115)98:2<185::AID-AJMG1029>3.0.CO;2-M

We describe oral manifestations in six patients (three females and three males aged 6 to 24 years) with Kabuki syndrome (KS), based on their physical, orthopantomographic, and cephalometric findings. All six patients had a high-arched palate, malocclusion, most commonly unilateral posterior cross-bite (5/6), severe maxillary recession and mid-facial hypoplasia. Other frequently observed oral manifestations included small dental arch and hypodontia. Three patients lacked permanent teeth, mostly the central/lateral incisors. Both tooth size (in primary and permanent teeth) and dental arch (in length and width) tended to be small. We would like to stress that oral care and management is a must for the well-being of KS patients.