Surgical management of Paget's disease

Orthopedic complications of Paget's disease occur commonly and arise as a result of chronically accelerated bone remodeling in focal regions of the skeleton. Complications include pathologic fractures with delayed union, progressive skeletal deformity, chronic bone pain and pagetic arthritis. The new bisphosphonates have transformed the treatment of Paget's disease in the past decade but have not yet been studied in depth for their ability to prevent orthopaedic complications. Although few patients with Paget's disease ever require surgical intervention, successful operative management of orthopaedic complications has dramatically improved the quality of life for many sufferers. Selected modalities with promising results include total hip replacement for end-stage pagetic arthritis of the hip, total knee replacement for end-stage pagetic arthritis of the knee, proximal tibial osteotomy for painful malalignment of the knees, internal fixation for pathological fractures, and decompressive laminectomy for spinal stenosis. Complications of surgery on pagetic bone include hemorrhage, infection, pathologic fracture, delayed union, nonunion, and aseptic loosening of hardware. Medical, surgical, and rehabilitative modalities provide a wide array of options in managing orthopaedic complications of Paget's disease and are useful in improving quality of life for sufferers of the condition. Prospective studies are needed to assess the ability of antipagetic medications to prevent severe long-term complications such as deformity, arthritis, and malignancy. Localization of susceptibility genes for Paget's disease may accelerate identification of targets for gene therapy and disease prevention.