[A case of the Hallermann-Streiff syndrome]

The Hallermann-Streiff Syndrome has been characterized and established according to 7 positive and 5 negative signs, which were described by François. We encountered an 11 year, 5 months old boy who had 7 positive symptoms of this syndrome in our clinic. In this study, we reported on this typical patient focusing on his dental view. 1) Prolonged retention of the primary teeth which involved microdontia were noted. Congenitally missing teeth were also seen. 2) The occlusal relationship indicated open bite, and also the mandibular function was impaired. 3) The measurements of the length and width of the dental arch were smaller than that of a normal subject, and the dental arch of the maxilla was V-shaped. 4) According to X-ray cephalometric analysis, (a) the dental calculus and the alveolar bone absorption were very evident. (b) abnormal morphologic of the glenoid fossa, mandibulars condyle and the neck of mandibula were seen. These conditions were very evident on the left side. 5) The growth obstade of the maxilla and mandibla and the left shift of the mandible were found. 6) According to histological study, enamel hypoplasia was noted.