Osteogenesis imperfecta and dentinogenesis imperfecta: Clinical features and dental management

Abstract

Osteogenesis imperfecta (OI) is a rare congenital condition, marked by fragile bones, skeletal deformities and additional extra-skeletal symptoms. Depending upon the degree of seriousness, affected people can either carry on with a mostly unrestricted, independent life, or their mobility is severely affected, making them dependent on others for support. Despite that, there is no effect on intellectual capacities. The medical and surgical treatments of OI are directed towards improving the patient's quality of life, mobility and functional independence. Because of the close biochemical relationship that exists between collagen and dentine, the teeth are affected in certain patients, leading to dentinogenesis imperfecta (DI) which is described by the appearance of opalescent teeth. To enable preventive intervention and effective dental treatment, it is essential that the correct diagnosis of DI is done at an early stage.

Keywords

• dentinogenesis imperfecta
• mutations
• osteogenesis imperfecta