Amelogenesis imperfecta & Dentinogenesis imperfecta - Click here

Amelogenesis Imperfecta (AI) is a group of inherited developmental disorders affecting enamel formation in both primary and permanent dentitions. It results from mutations in genes involved in enamel matrix formation and mineralization. Clinically, AI presents with thin, hypoplastic, soft, or poorly mineralized enamel, leading to tooth sensitivity, discoloration, rapid wear, and esthetic concerns. Radiographically, enamel thickness and density are altered depending on the subtype.

Dentinogenesis Imperfecta (DI) is an inherited disorder of dentin formation, commonly associated with mutations in the DSPP gene. Teeth exhibit a blue-gray or amber discoloration, bulbous crowns, cervical constriction, and early pulpal obliteration. Enamel fractures easily due to defective dentin support, causing rapid attrition.

Management of both conditions is multidisciplinary, focusing on early diagnosis, prevention of tooth wear, restoration of function and esthetics, and long-term follow-up.