Characteristics of Dental Crown and Palatal Vault in Kabuki Syndrome

抄録

Kabuki syndrome (KS) was first described independently in 1981 by Niikawa et al. and Kuroki et al., and was then defined in detail by Niikawa et al, who performed clinical, genetic, and epidemiological studies. Oral anomalies are common in KS (over 60%) and include abnormal dentition, widely spaced teeth, cleft palate or lip, high palate, hypodontia, conical incisors, screw driver-shaped incisors and ectopic upper 6-year molars. To assist in diagnosis of KS, the detailed character of molars and palatal vaults of 5 KS patients, who were the same patients Matsune reported on, were examined. The area and circumstance length of occlusal view and table view of permanent molars in the KS group were smaller than the molars of the healthy control group. The ratios of occlusal view areas to occlusal table areas of permanent molars in the KS group were significantly smaller than control group molars, but on the other hand no difference was seen in primary molars. All contour line lengths of occlusal view and occlusal table in permanent molars of KS group were significantly smaller than those in the control group, however, no significant differences were found in primary molars between the KS and control group. The pattern of the occlusal view was hexangular or round, and the occlusal table was almost quadrangular or hexangular in permanent molars in the KS and control group, respectively. No strong significant difference was found in the size of the palatal vault between the KS group and the control group. The characteristics difference between two groups was clarified that the shapes of palatal vault at the canine and first molar regions were of a V and U character in the KS and control group, respectively. These detailed molar and palatal vault characteristics of the KS group will help the identification of KS patients as an added clinical diagnosis.