Rosen & Barkin's 5-Minute Emergency Medicine Consult (503 page)

• Unknown
  
• Genetic component:
  
  • SQSTM1 mutation seen in many but not all cases
    
• Environmental influences may also play a role
  
  • Presence of nucleocapsids from measles, canine distemper, paramyxovirus, or respiratory syncytial virus may implicate viral cause
    
  • Possible association with rural life and close contact with farm animals
    
• May represent vascular hyperplasia with subsequent inflammation
  
• Increased nucleoli and intranuclear inclusion bodies seen in osteoclasts on microscopy
  

Generally not seen in children

DIAGNOSIS

• Many patients are asymptomatic, with disease discovered by incidental radiographs or elevated alkaline phosphatase levels
  
• Deep, aching bone pain occurs late in the clinical course
  
• Pain with weight bearing if femur or tibia involvement
  
• Pain worse with rest in nonweight-bearing bones
  
• Acute (resorptive/osteolytic) phase:
  
  • Pathologic fractures
    
  • Pain from acute lysis, fracture, or resultant arthritis
    
  • Hypercalcemia or renal stones
    
  • Hypervascularity may result in significant bleeding if affected bone is fractured
    
  • Widespread disease:
    
    • Increased vascularity and blood flow may result in high-output cardiac failure
      
• Secondary (sclerotic/osteoplastic) phase:
  
  • Long-bone involvement may present with swelling or deformity and gait abnormality
    
  • Skull involvement may cause headaches or abnormal skull shape (change in hat size)
    
  • Severe skull or spine involvement may result in CNS compression
    
  • Hearing loss may result from nerve compression or ossicle involvement
    

• Diagnosis usually suggested by radiographs
  
• Thorough neurologic exam must be documented, especially with vertebral or pelvis involvement
  

• Alkaline phosphatase is the most dramatic marker of disease activity
  
• Calcium and phosphate levels should be checked as well, but are usually normal
  
• Hypercalcemia seen in immobilization or presence of a fracture, but if elevated in an ambulatory patient, suspect hyperparathyroidism
  
• EKG if suspect hypercalcemia and CXR with evidence of high-output cardiac failure
  
• Increased bone formation may lead to elevations in urine hydroxyproline or serum osteocalcin or procollagen fragments
  
• Alterations in parathyroid hormone (PTH) levels occur as secondary changes during resorptive/osteolytic phase (low PTH) and sclerotic/osteoplastic phase (high PTH)
  

• Plain x-rays:
  
  • During resorptive phase, lytic lesions are often not seen, except in skull, where lesions are well demarcated (osteoporosis circumscripta)
    
  • Bowing of long bones may occur with resorption and strength loss
    
  • New bone initially appears irregular and spotty, and later becomes homogeneous and dense (“ivory pattern”)
    
  • Excess bone may be deposited along stress lines, leading to cortical irregularities and thickening
    
• CT or MRI defines margins and helps evaluate for neoplasm or hematoma:
  
  • Spiral CT to detect renal calculi
    
• Radionuclide bone scans useful to evaluate extent and activity of disease
  
• Plain films are usually all that is needed in acute setting to identify/manage fractures
  

• Primary hyperparathyroidism
  
• Multiple myeloma
  
• Hodgkin variants
  
• Acromegaly
  
• Osteosarcoma
  

TREATMENT

• Pre-hospital personnel should obtain information about mechanism of injury or social factors that suggest pathologic fracture
  
• Adequate immobilization can limit excessive bleeding around fracture site
  

• Airway management and resuscitation, as indicated
  
• High-output cardiac failure should be treated as outlined in CHF chapter
  
• Prompt immobilization of fractures will limit excessive bleeding around fracture site
  

• Analgesia for pain of lytic lesions, fractures, or arthritis includes acetaminophen and narcotics
  
• Fracture treatment is often more conservative, owing to difficulties with bleeding during operative repair
  
• Orthopedic consultation for severe arthritis and definitive fracture management
  
• Hypercalcemia may be treated with IV fluids, calcitonin, and/or bisphosphonates
  
• CNS compression requires emergent neurosurgical consultation and possible decompression
  

Treatment indicated in patients with symptomatic disease or asymptomatic disease located in areas where complications can occur

• Nitrogen-containing bisphosphonates:
  
  • Pamidronate: 30 mg IV daily × 3 consecutive days; infuse over 4 hr
    
  • Alendronate: 40 mg PO daily for 6 mo
    
  • Risedronate: 30 mg PO daily for 2 mo
    
  • Zoledronic acid: 5 mg IV × 1; infuse over at least 15 min
    

• Simple bisphosphonates and calcitonin:
  
  • Etidronate: 5 mg/kg PO daily for 6 mo
    
  • Tiludronate: 400 mg PO daily for 3 mo
    
  • Calcitonin: 50–100 U SC as tolerated; not for >6 mo
    
• Chemotherapy and simple bisphosphonates no longer recommended
  
• Use of calcitonin and simple bisphosphonates are limited to patients who cannot tolerate or who are allergic to the nitrogen-containing bisphosphonates
  
• Side effects of bisphosphonates include influenza like syndrome and jaw osteonecrosis
  
• Often need supplemental vitamin D and Ca to maintain normal Ca levels during treatment
  

FOLLOW-UP

• Admission as indicated for major trauma or injury, or excessive bleeding
  
• Orthopedic procedures
  
• Hypercalcemia
  
• CNS compressive symptoms, nerve entrapment requiring surgery
  

• No evidence of significant bleeding, neurologic compromise, or hypercalcemia, and adequate pain control
  
• Appropriate fracture immobilization and orthopedic follow-up
  

• Referral is based upon any acute injuries
  
• May also consider referral to endocrinologist within 1–2 wk of discharge
  

• Follow-up is generally driven by the acute injury that led to the radiographs on which the diagnosis of Paget disease was made
  
• Response to pharmacologic treatment aimed at correction of serum alkaline phosphatase levels
  
• Consider repeat pharmacologic treatment if rise in serum alkaline phosphatase, return of symptoms, or disease progression seen radiographically
  

• The diagnosis of Paget disease is usually made as an incidental finding on radiographic imaging
  
• Prompt immobilization of fractures will limit excessive bleeding around fracture site
  
• Consider Paget disease if elevation of alkaline phosphatase is present without any other explanation
  

• Cundy T, Reid IR. Paget’s disease of bone.
  Clin Biochem.
  2012;45:43–48.
  
• Lojo Olivieria L, Torrijos Eslava A. Treatment of Paget’s disease of bone.
  Reumatol Clin.
  2012;8:220–224.
  
• Ralston SH, Langston AL, Reid IR. Pathogenesis and management of Paget’s disease of bone.
  Lancet
  . 2008;372:155–163.
  
• Whyte MP. Clinical practice. Paget’s disease of bone.
  N Engl J Med
  . 2006;355:593–600.
  

See Also (Topic, Algorithm, Electronic Media Element)

Specific Orthopedic Injuries

CODES

731.0 Osteitis deformans without mention of bone tumor