The spine in osteogenesis imperfecta.

A high incidence of spinal curvature, approaching 80 per cent in patients more than twelve years old, was found in a large series of patients with osteogenesis imperfecta. Curves showed early progression and a predictable pattern. In the age range between one and five years the incidence of scoliosis was 26 per cent; thereafter, it rose precipitously. The degree of curvature was greater in the older children. The Milwaukee brace was ineffective in the treatment of the scoliosis and the complications of its use were detrimental. In several patients it deformed the rib cage. It had little or no effect on the progression of the curve. The incidence and degree of curvature was related to the type of bone present. Patients with chest deformities and those who were not ambulatory seemed to have a predilection for scoliosis.