Pediatric Limb Reconstruction in Osteogenesis Imperfecta

The indications for limb reconstruction surgery in children with osteogenesis imperfecta (OI) are recurrent long bone fractures or long bone deformity that interferes with function. Intramedullary rod fixation is the preferred method of stabilization, as it is load sharing and will splint the pathologic bone to reduce the incidence of fracture, progressive deformity and associated pain. Telescoping rod systems have been developed that will expand with growth and diminish the number of procedures necessary to keep the bone splinted during skeletal growth. Even with modern treatment consisting of medical management with bisphosphonates and surgical reconstruction with telescoping rods, reoperation is common. Children with OI have a higher incidence of non-union, which may be further increased by bisphosphonate therapy. Combined medical and surgical treatment lowers bone pain, fracture occurrence and can improve function.