Abstract
Oral management of two sisters out of three who were affected amelogenesis imperfecta with dentin dysplasia for 12 years.
At the time of the first visit, the elder sister was 7 years,2 months old and the younger sister was 2 years,5 months old. The chief complaint was the abnormal shape of their tooth crowns. No abnormality was discovered in their general physical status. All of the teeth of their father had been affected with hypoplasia and he had been wearing full dentures both on the maxilla and mandible.
At the first oral examination for the elder sister,13 primary and 8 permanent teeth had erupted and the teeth showed yellow discoloration. The tooth crowns of the 8 primary teeth and one mandibular first molar were severely destroyed and not be able to restored. At the first oral examination of the younger sister, all of the primary teeth had erupted and the dentin of all of the teeth were exposed, with yellow-brown discoloration being observed. The exposed dentin surfaces were smooth. The permanent enamel was thin, rough but hard and showed hypoplasia. Spacing between the teeth and attrition were also observed. Both for their primary and permanent teeth, with increase in age a highly narrowing or closing of the pulp chamber was obseved on the x-ray films. Closed pulp chambers were especially obvious for the permanent teeth of the younger sister. With histo-pathologic observation of the primary teeth, small number of dentinal tubles and irregularity of the dentin calcification were evident and dentin dysplasia was diagnosed.
Soon after the eruption of the teeth, all of the teeth were first restored with glass ionomer cement, and then the teeth were restorative with adhesive composite resin. Finally, all of the primary incisors were restored with restorative composite resin crowns and all of the primary canines and primary molars were restored with steel crowns. All of the permanent incisors were first restored with restorative composite resin crowns and finally restored with prosthetic composite crowns. All of the permanent molars and premolars were first restored with steel crowns and finally restored with metal full cast crowns. The adhesiveness of the restorative materials such as the bonding resins and glass ionomer cements to the enamel and dentin was low, and the peeled off with loss of the restoration often occuring.
With cephalometric analysis of the elder sister at 18 years 9 months of age and the younger sister at 14 years 6 months of age, no disorder was obtained except for the shallow over bite of the younger sister.
These cases might be classified into autosomal dominant amelogenesis imperfecta combined with dentin dysplasia.
