Dentin is a major structural component of tooth and serves as the framework upon which enamel and cementum form.
Dentin supports enamel via mechanical stress distribution through well‑organized dentinoenamel junctions.
Heritable dentin defects are rare diseases that affect primarily the organic dentin matrix. They have been classified into three types of dentinogenesis imperfecta (DI-I, DI-II and DI-III), and two types of dentin dysplasia (DD-I and DD-II) both of which present autosomal dominant transmission that affects the primary and permanent dentition.
According to Shields et al. (1973), the DI type I is associate with osteogenesis imperfecta.
DI type II is found in patients with dentition abnormalities alone and no bone disease. DI type III is the Brandywine form, named for the city Brandywine, Maryland, where there was a large population of patients with this disorder.
DI has a reported incidence range from 1:6000 to 1:8000 (for all types of DI) in the United States2. Clinically, the color of affected teeth ranges from gray to brownish-blue with a translucent opalescence.
Radiographically, the teeth show bulbous crowns, cervical constriction and short roots. In DI type II the pulp chambers and root canals are partial or completely obliterated, while in DI type III the teeth present viable pulp chambers leading to what is referred to as "shell teeth".
Due to the lack of support of the poorly mineralized underlying dentin, the enamel frequently fractures leading to rapid wear and attrition of the teeth.
In general, patients with DI require complex treatment that should take into account the degree of tooth destruction, age and cooperation of the patient.
°Revista de Odontologia da UNESP
°Raquel Mantuaneli Scarel-Caminaga, Lícia Bezerra Cavalcante; Livia Sertori Finoti; Maria Cristina Leme Godoy dos Santos; Maria Flávia Konishi; Lourdes Aparecida Martins dos Santos-Pinto