63 Dental aspects in osteogenesis imperfecta type IV

Abstract

Abstract Background The term “dentinogenesis imperfecta” includes dental anomalies quite similar to hereditary opalescent dentin and osteogenesis imperfecta. We present dental findings in a boy with osteogenesis imperfecta type IV Case Presentation A young boy was referred to the pediatric department for management of vertebral compression and a recurrent femoral fracture without obvious trauma. The appearance of his teeth with the overall assessment of this patient reveals dentinogenesis imperfecta compatible with osteogenesis imperfecta type IV, associated with major vertebral involvement Discussion Osteogenesis imperfecta (OI), also known as also known as « brittle bone disease » is a heterogeneous group of rare genetic diseases involving connective tissue. A reduction in the amount of normal collagen leads to Type I, while qualitative and quantitative abnormalities in collagen synthesis lead to Types II, III and IV of OI. This type IV is subdivided into groups A and B: the group B depicting the characteristic dentinogenesis imperfecta like in our patient. Conclusion Dentinogenesis imperfecta is a major sign of osteogenesis imperfecta. Skeletal phenotype, scleral colour and deafness complete the picture allowing the final classification of this inherited disorder The peculiar bone lesions with normal dental aspects are highly suggestive of the extreme rare osteogenesis imperfecta type IV.