Extremity Surgery in Adults with Osteogenesis Imperfecta

Abstract

Osteogenesis imperfecta (OI) requires surgical intervention also in adulthood. Although it is less frequent than in childhood, surgery in adult OI patients has its own characteristic considerations. They still have a greater risk of fracture than normal populations and most of the fractures require surgical intervention. Pre-existing deformity of the long bone and subsequent joint derangement also require surgical intervention. Mild residual deformity persistent from childhood or device implanted in childhood may become symptomatic in middle age or older and also require surgical intervention. Intramedullary rod remains as the mainstay of fixation, while plate and screw is indicated only in limited and special circumstances. Tension band fixation is sometimes useful for fixing metaphyseal fracture or deformity correction. Degenerative joint disease may develop in later life from traumatic arthritis or from malalignment of the limb; joint replacement surgery is technically challenging and its longevity is much shorter than in normal populations.