Dental aberrations in children and adolescents with osteogenesis imperfecta.

The aim of this investigation was to study dental aberrations in a large sample of unrelated patients with different types and forms of osteogenesis imperfecta (OI). Sixty-eight non-related index patients aged 0.3 to 20 years (mean, 10 years) were examined clinically. Panoramic radiographs from 49 patients were analyzed. Dentinogenesis imperfecta (DI) type I was found in 27 of 65 patients and was significantly more common in OI type III than in types I and IV and in patients with a severe form of the disease. The presence or absence of DI showed almost complete accordance between affected parents and children and between affected siblings. Moreover, agenesis was found in 11 of 49 patients, apically extended pulp chambers in 20 of 48 patients, and impaction of second permanent molars in 7 of 19 patients older than 15 years. The percentage of patients with no apparent dental aberrations was approximately the same in patients with OI type I and type III and in patients with mild and severe form of the disease. The high prevalence of dental aberrations in OI stresses the importance of clinical and radiographic odontologic examination as part of the clinical investigation. In patients with mild forms of the disease, in whom the medical diagnosis is uncertain, demonstration of disturbances in dental development can be crucial for establishing the OI diagnosis. C