Management overview of osteogenesis imperfecta.

The treatment of osteogenesis imperfecta varies with the age of the patients and the severity of disease. Patients with mild disease rarely develop significant deformities; therefore, the care required is mainly limited to periodic fractures or to associated problems, such as erosion of the teeth, decreased hearing, and difficulties in adjustment. It is the patient with severe disease who often requires an unusual amount of attention. Regardless of the immediate problem, the treatment program should be geared toward the long-term function of the patient; in most cases this will be best served by the prevention and/or correction of long bone deformities, and by encouraging maximum intellectual and academic development. The latter requires contact of the child with normal children by placement in regular school classes. The primary basis of a successful treatment program to maintain acceptable skeletal alignment consists of properly timed operations for insertion of extensible intramedullary rods, together with supportive splints and braces. No other method of treatment, such as the use of systemic medications, has yet been shown to benefit the patient clinically. Progressive improvement in bone strength during childhood permits better eventual function for those patients in whom good skeletal alignment has been maintained. Most patients will become productive members of society, so the combination of optimum physical and optimum academic development will offer maximum opportunity to those who enter the competitive job market.