Abstract

“OSTEOGENESIS imperfecta is a disorder of bone formation in which increased fragility is the most important manifestation.” This is the definition given by Fleming, Radasch, and Williams (1). The name osteogenesis imperfecta congenita was coined by Vrolik (2) in 1845, and described by Knaggs (3) as, “A disease which is characterized by a congenital defect in the evolution of the osteoblast, and recognized clinically by defective ossification of the cranium and multiplicity of fractures resulting from trivial causes.” The first case in the literature was reported by Armand (4) in 1716. According to von Recklinghausen (5), 30 cases of osteogenesis imperfecta (osteopsathyrosis congenita or fragilitas ossium congenita) were known in the literature to 1910. This number has been greatly increased since the utilization of the roentgen rays in diagnosis. A thorough clinical and probably the first anatomical description was made by von Lobstein (6) in 1833. The term “Lobstein's disease” is still encountered, to wh...

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