Dentinogenesis Imperfecta
A genetic disorder affecting dentin formation, causing translucent, discolored teeth.
Dentinogenesis imperfecta (DI) is an inherited dentin formation disorder affecting approximately 1 in 6,000-8,000 people. Teeth appear translucent blue-gray or amber-brown. The dentin is abnormally soft, causing teeth to wear rapidly, chip easily, and break. Three types exist: Type I (associated with osteogenesis imperfecta), Type II (isolated dental condition, most common), and Type III (rare, 'shell teeth'). Radiographically, teeth show bulbous crowns, short roots, and obliterated pulp chambers. Management focuses on preventing wear and maintaining function. Treatment includes stainless steel crowns on primary teeth, overlay dentures for severe cases, and full-mouth rehabilitation with crowns in adulthood. Early intervention is crucial to prevent progressive tooth structure loss.