Chapter 11

The orthopedic surgeon often manages the complications of Paget’s disease, especially when they manifest as arthritis, skeletal deformity, pathologic fracture, or malignant degeneration. The proper care of these patients involves a multidisciplinary approach, which may include primary care doctors, rheumatologists, physiatrists, physical therapists, and social workers. Indications for surgery in Paget’s disease are few and include unstable fractures, symptomatic arthritis, severe limb malalignment, and malignant degeneration. This chapter focuses on the treatment of orthopaedic complications of Paget’s disease requiring the use of internal fixation.1,2

GENERAL TREATMENT CONSIDERATIONS

Few who have Paget’s disease will require treatment. Nonsteroidal anti-inflammatory medications have an important role in treating the symptomatic arthritis associated with Paget’s disease (Table 11–1). Newer cox-2 inhibitors are likely to provide more specific anti-inflammatory relief with fewer gastrointestinal side effects. Narcotic agents should be avoided, if possible, particularly in the older population.^1,2

A cane can be a very important therapeutic device for patients with Paget’s disease of the lower limbs. A cane provides increased stability, prevention of falls, and load-sharing capability. The use of a cane makes good sense and should be standard treatment for the elderly patient who has Paget’s disease involving the lower limbs.

Indications for surgical intervention in Paget’s disease include unstable fractures, malignant transformation, and severe arthritis that is refractory to medical treatment.3,4 Relative indications for surgery include malalignment of a major weight-bearing bone or impending fracture, even though these may be treated with an orthosis. Spinal decompression for spinal stenosis is an important consideration, but there are few data on the management of this complication.^1,2,5,6

Surgical intervention in Paget’s disease is most often sought when degenerative arthritis of the hip or knee produces severe pain with movement. Anti-inflammatory agents usually produce little relief of symptoms in this setting. Diagnostic intraarticular injections with a local anesthetic often confirm that the pain is primarily articular, rather than osseous. Total hip replacement is highly effective in relieving hip pain and restoring mobility. Tibial osteotomy is equally effective in relieving knee pain in patients who have severe tibial bowing if the associated articular degeneration is not far advanced. The Ilizarov external fixator has been used successfully in limited application for treatment of deformities in Paget’s disease.⁷

Before any operative procedure is performed, it is desirable, if possible, to reduce disease activity by drug therapy to prevent excessive blood loss.^1,2,5,6 Preoperative medical treatment with bisphosphonates or calcitonin decreases intraoperative bleeding. A reduction in serum alkaline phosphatase activity to approximately 50% of pretreatment levels is probably adequate preoperative control. In elective cases it is desirable, therefore, to begin antipagetic medication at least 6 weeks before surgery. When osteotomy followed by bone healing is planned, bisphosphonates are the drugs of choice.

When total joint replacement is performed, long-term suppression of disease activity through use of bisphosphonates may be desirable to diminish excessive bone-remodeling activity and to prevent loosening of prosthetic components. In all circumstances requiring bone surgery, the patient must be aware that delayed bone healing may occur and that a lengthy rehabilitation program may be necessary.

FRACTURE FIXATION

Pathological fractures are a common complication of Paget’s disease.^8–14 Painful fissure fractures or pseudofractures and completed pathological fractures occur in areas of high mechanical stress, particularly in the weight-bearing bones of the lower limbs. Fracture healing is often complicated by delayed union.¹¹ Complete immobilization of pagetic bone should be avoided because intercurrent osteopenia of immobilization further imperils the structural integrity of bone already weakened by Paget’s disease.

Exacerbation of pain in pagetic bone should raise suspicion of a pathological fracture, leading to prompt roentgenographic evaluation. Accelerated metabolic activity of active pagetic bone poses added complications when fractures occur, although none of the complications are specific to Paget’s disease (Table 11–2). In addition to following basic principles of fracture management, attention should be directed at decreasing metabolic activity in pagetic bone. Prolonged immobilization can further exacerbate osteopenia and provoke metabolic complications of hypercalcemia and hypercalciuria. Functional fracture bracing may be necessary to supplement open reduction and internal fixation.

Several series report the treatment of femur fractures in patients who have Paget’s disease. The use of modern intramedullary devices has yielded more predictable results.¹⁵ Several types of fractures, including proximal femoral fractures involving the subtrochanteric region, have been identified as problem fractures compared to subcapital, intertrochanteric, and femoral shaft fractures.^3,9

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Norian SRS Resorbable Cement for Augmentation of Internal Fixation of Hip Fractures Basic Principles and Techniques of Internal Fixation in Osteoporotic Bone An Injectable Cementing Screw for Fixation in Osteoporotic Bone Improving the Distal Fixation of Intramedullary Nails in Osteoporotic Bone Internal Fixation of Osteoporotic Long Bone Enhanced Stability of External Fixation with Hydroxyapatite-Coated Pins

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