Craniofacial and dental characteristics of Kabuki syndrome

We describe oral manifestations in six patients (three females and three ma les aged 6 to 24 years) with Kabuki syndrome (KS), based on their physical, orthopantomographic, and cephalometric findings. All six patients had a hi gh-arched palate, malocclusion, most commonly unilateral posterior cross-bi te (5/6), severe maxillary recession and mid-facial hypoplasia, Other frequ ently observed oral manifestations included small dental arch and hypodonti a. Three patients lacked permanent teeth, mostly the central/lateral inciso rs. Both tooth size (in primary and permanent teeth) and dental arch (in le ngth 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. (C) 2001 Wiley- Liss, Inc.