Craniofacial and dental characteristics of Kabuki syndrome

K Matsune, T Shimizu, T Tohma… - American journal of …, 2001 - Wiley Online Library
K Matsune, T Shimizu, T Tohma, Y Asada, H Ohashi, T Maeda
American journal of medical genetics, 2001Wiley Online Library
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 …
Abstract
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. © 2001 Wiley‐Liss, Inc.
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