Surgical management of paget's disease

Abstract

Orthopedic complications of Paget's disease wcur 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 loowning 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. (J Bone Miner Res 1999;14, suppl. 2:%38)

Cementless total hip arthroplasty in Paget’s disease of bone: a retrospective review

“…First, hypervascularity significantly increases the risk for blood loss and may compromise cement fixation of the implants. Prior to surgery, the use of potent bisphosphonates has been advocated, although no controlled study is available [13]. The goal of such a recommendation is to reduce bone hypervascularity and therefore to decrease intraoperative blood loss.…”

Total shoulder arthroplasty in a patient with Paget’s Disease: case report and review of the literature

“…Another consideration is whether to use cemented or uncemented components; in the hip arthroplasty literature, this issue is controversial. 8,9,12,17,18,22,25,28,35 Based on our experience, we recommend cemented humeral components, as a cemented component allows for improved fixation in a potentially voluminous and pathologic humeral intramedullary canal. The final consideration is whether or not to use prophylaxis for heterotopic ossification.…”

“…Disease activity can be monitored with serum alkaline phosphatase and urine hydroxyproline levels. 9,11,[30][31][32][33] Bisphosphonate treatment can reduce disease activity and, in turn, reduce the amount of hypervascular bone present. By treating patients with bisphosphonates until laboratory values have normalized, a surgeon can minimize the risk for excessive intraoperative blood loss.…”

“…As mentioned above, this risk can be minimized with preoperative bisphosphonates; however, it is prudent to have blood products readily available, along with a blood salvage system. 8,9,12,18,22,25 Additionally, the treating surgeon must be prepared to address any deformity present as a result of the patient's Paget's Disease. Extra-articular deformity may require the use of a corrective osteotomy for a successful arthroplasty.…”

Hüftendoprothetik bei Deformitäten des proximalen Femurs

“…Bei einem sogenannten aktiven pagetoiden Prozess werden in der Literatur in Assoziation mit einer lokalen Hypervaskularisierung ein verstärkter Blutverlust, eine Zunahme des Knochenschmerzes, eine perioperative systemische Hyperkalzämie, eine progressive Knochenresorption sowie das Risiko einer erhöhten Resorption des Knochens periimplantär berichtet [20,30,31,49]. Im Rahmen der Operationsplanung sollte daher der Aktivitätsgrad des Morbus Paget mittels Knochenszintigrafie bzw.…”