Dental management in osteogenesis imperfecta

Osteogenesis imperfecta is a very rare heterogeneous genetic disorder associated with the development of connective tissue resultingin fragile bones and frequent fractures. More than 50% of patients aff ected with osteogenesis imperfecta have a hereditary developmentaldisorder known as dentinogenesis imperfecta. Dentinogenesis imperfecta is caused by irregularities in the formation,composition and organization of dentin matrix during tooth development. It is caused by mutations of the genes that encode basicproteins of the organic matrix, collagens and phosphoproteins. The purpose of this review is to describe the histopathologic andclinical features of teeth typical of dentinogenesis imperfecta type I, which occurs within osteogenesis imperfecta, with specialemphasis on targeted dental treatment to achieve optimal rehabilitation of the masticatory system.Key words: developmental bone disease; dentinogenesis imperfecta; rehabilitation

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