Orthopaedic treatment of osteogenesis imperfecta.

Orthopaedic management of patients with OI is best organized through an OI Service so that families are well informed about the disease. The orthopaedic treatment is greatly facilitated by close association between the orthopaedic surgeon and other members of the service as well as with the local family doctor. The severity of the deformities and skeletal fragility vary considerably. Fractures in mild to moderately severe cases of OI type I are treated using standard methods as used in patients without OI. Severely affected patients with OI type I and many cases of OI type III will require additional orthopaedic care. Realignment of the deformed bones that are fracturing frequently followed by external or internal support are commonly used. We have favoured lightweight orthoses in the upper limb rather than fragmentation and rodding. In the lower limb we undertake fragmentation and rodding in bones that are deformed and repeatedly fracturing. It is important in selecting various modes of treatment to consider the natural history of the particular type of OI and to set realistic goals. This particularly important when considering mobility and walking. It is to be hoped that improved knowledge of the classification, pathogenesis and prognosis of OI will provide better guidelines that will be of practical value to the orthopaedic surgeon.