Paget's disease of the bone

Question 1

Describe Paget’s disease of bone?

Answer 1

• Increased bone turnover
• Disorganiesd bone remodelling
  
  • => Bone enlargement, deformity and weakness

Question 2

Pathophysiology of Paget’s disease of the bone?

Answer 2

• Increased osteoclastic bone resorption
• Bone marrow fibrosis

Question 3

Describe the clinical features of Paget’s disease of bone?

Answer 3

• Asymptomatic
• Mostly axial skeleton involved
  
  • Pelvis, femur, lumbar spine, skull
• Deep, boring pain
• Bone deformity and enlargement
• Cranial nerve defects, spinal cord compression

Question 4

What are some of the complications of Paget’s disease of bone?

Answer 4

• Hypercalcaemia
• Pathological fractures
• Osteoarthritis, Osteosarcoma
• Nerve compression due to bone overgrowth

Question 5

Investigations into Paget’s disease of bone?

Answer 5

• X-ray
  
  • Enlargement
  • Patchy cortical thickenin
• ALP markedly raised in isolation
• Radionucleotide bone scan
  
  • Identify areas of involvement
• Bone biopsy may be required to exclude metastases

Question 6

Describe the treatment for paget’s disease of bone?

Answer 6

• If analgesia fails:
  
  • Alendronic acid
    
    • Reduce pain +/- deformity
  • Calcitonin

Question 7

What is pictured here?

Answer 7

• Osteomalacia
  
  • Cortical bone lucency
  • Looser’s zones seen in both forearms

Question 8

What is pictured here?

Answer 8

• Rickets
  
  • Ragged metaphyseal surfaces in the knee and ankle
  • Bowing of the long bones

Question 9

What is pictured here?

Answer 9

• Paget’s disease of bone
  
  • Sabre tibia with multiple sclerotic lesions

Question 10

Case history for presentation of Paget’s disease of bone

Answer 10