Osteogenesis Imperfecta

Osteogenesis Imperfecta is a rare affection characterized by fragility of the bones, blue sclerae, and deafness, less frequently by hypermobility of the joints. The etiology is unknown, but it appears to be a mesenchymaldefect. A cases of osteogencesis imperfecta (tarda form) in a 18 yesrs old male is presented with a review of the literatures. The chief complaints were bowing deformity of the all extremites and blue sclera. In this cases, other typical features such as deafness and hypermobility of the joints were not observed. X-ray showed multiple malunited fraeture, of all long bones of extremities except Ieft humerus. For the treatment of bowing deformity of right humerus, multiple corrective osteotomy and intramedullary nailing was performed and the result was good.