ABSTRACT
Since 1985 we have used the early surgical treatment of osteogenesis imperfecta described by Sofield, even in the most severe forms of the disease. Twenty-six patients who had reached adult age have been reviewed. Whilst the functional prognosis is relatively favourable in Sillence type I cases, it is not the same for severe type III cases. In these forms, the spine and chest deformities can affect the prognosis for life. The functional state of these patients in adult life is disappointing and many of them subside progressively into the comfort of a wheelchair. Nevertheless, this assessment at the end of growth has confirmed the principles of a surgical approach, even if, at the present time, our attitude is more reserved in severe cases. Very considerable progress in treatment has resulted from telescopic rods. The improvement in prognosis seems now to depend on taking account of the spinal problem in osteogenesis imperfecta. Early surgery on a spine that is, as yet, slightly deformed should be considered in severe forms of the disease.
