Dentinogenesis Imperfecta

Dentinogenesis Imperfecta (DI) is a genetically heritable developmental defect of the dentin. There are three types of this disease classically characterized as a Shields type. Shields Type I occurs along with osteogenesis imperfecta (“brittle bone disease”), Shields Type II (Hereditary opalescent dentin) occurs alone, without osteogenesis imperfecta, and Shields Type III (Brandywine tri-racial isolate population). Both primary and permanent dentitions are affected. The teeth present with an amber or reddish-brown to blue-gray opalescent color and bulbous crowns with short, thin roots. The enamel over the poorly formed dentin can easily fracture in these teeth, often leading to severe wear of the dentition. Type I and Type II DI teeth usually present with pulpal obliteration radiographically. The rare Type III presents with characteristic bell-shaped crowns and shell-teeth radiographically due to severely enlarged pulp chambers and minimal dentin present (see x-ray).

Clinical and Radiographic presentations of severe Shields Type III Dentinogenesis Imperfecta