The spine and surgical treatment in osteogenesis imperfecta

In the osteogenesis imperfecta patient, spinal deformity should be expected, particularly in the severely affected nonambulator with chest deformity. Curves develop early (age five to six) and generally progress rapidly. Early bracing, although somewhat effective, may well compress the soft osteoporotic rib cage without controlling the spinal curvature. The pulmonary compromise created by the scoliosis is compounded by the chest cage deformity secondary to bracing. In the patient with severe disease (thin bones and numerous fractures), posterior correction and fusion, with or without Harrington instrumentation, is the preferred approach. This should be done early as the osteoporotic bone does not tolerate the hook forces well; the correction is correspondingly limited. The use of methylmethacrylate bone cement around the hook provides redistribution of forces and more stable fixation. In the patient with mild disease (thick bones and few fractures) treatment should be similar to that of patients with idiopathic scoliosis. The chest cage should be carefully observed to avoid deformity from placement of the lateral or posterior pad.