• Chronic localized bone remodeling disorder characterized by increased bone resorption, bone formation, and remodeling, which may lead to major long bone and skull deformities.
  • Majority of patients are asymptomatic. Symptomatic patients typically experience pain localized to the bone or joint, either from the pagetic lesion, secondary osteoarthritis, deformity, or pathological fracture.
  • Neurological symptoms due to bone overgrowth with consequent nerve impingement may include hearing loss, chronic facial pain, hydrocephalus, peripheral nerve entrapment, and spinal stenosis. Very rarely, high-output cardiac failure caused by high blood flow to metabolically active bone sites may occur.
  • Diagnosis is incidental in the majority of cases, with an elevated serum alkaline phosphatase raising suspicion for disease. Radiographs have classical appearance. Bone biopsy is the only confirmatory diagnostic test, but is rarely indicated.
  • If treatment is indicated, bisphosphonates are the first-line therapy to retard excessive osteoclastic activity. Adjunctive therapy includes physical therapy, orthoses, and walking and hearing aids. Analgesics are indicated for pain and inflammatory symptoms.

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