The spine in osteogenesis imperfecta: a review of treatment.

Progressive spinal deformity can be an anathma for indivudials with osteogenesis imperfecta. Scoliosis or khyphosis develop indolently, being less dramatic than long bone fractures, but once significant deformities evolve, they tend to remain progressive on into adulthood. State of the art spinal fixation systems are of little help in correcting such deformities due to poor bone stock. However, most curves can be arrested by posterior spinal fusion, performed either in situ, or by utilizing basic Harrington type instrumentation with methylmethacylate supplemtation for the hook sites, along with Drummond wires where feasible. Platybasia is yet another issue involving the spine which may be complicated by neurologic deterioration. It has been posulated as a cause of death, but can respond to shunting and brain stem decompression when recognized.