Learning Radiology xray montage
 
 
 
 
 

Paget Disease of the Rib


General Considerations

  • Multifocal chronic skeletal disease due to chronic paramyxoviral infection
  • Prevalence
    • 3% of individuals >40 years
    • 10% of persons >80 years
    • Unusual <40 years
    • M:F = 2:1
  • Pelvis most commonly involved
  • Histology
    • Increased resorption and increased bone formation
    • Newly formed bone is abnormally soft with disorganized trabecular pattern

Clinical Findings

  • Asymptomatic (1/5)
  • When symptomatic, symptoms may include
    • Fatigue
    • Enlarged hat size
    • Peripheral nerve compression
    • Neurologic disorders from compression of brainstem (basilar invagination)
    • Hearing loss, blindness
    • Facial palsy (narrowing of neural foramina) - rare
    • Pain from(a)primary disease process is rare so think of
    • Pathologic fracture
    • Malignant transformation
    • Secondary degenerative joint disease aggravated by skeletal deformity
    • High-output congestive heart failure from markedly increased perfusion (rare)
    • Increased alkaline phosphatase (increased bone formation)
    • Hydroxyproline increased (increased bone resorption)
    • Normal serum calcium + phosphorus

Imaging Findings

  • Characteristic changes of Paget’s disease of bone include
    • Thickening of the cortex
    • Coarsening of the trabecular pattern, and
    • Increased size of the bone

Differential Diagnosis

  • Metastatic disease
  • Fibrous dysplasia

 

Paget Disease, right 6th rib

Paget Disease, right 6th rib. The rib is enlarged. The cortex is thickened (white arrows) but the medullary space remains intact (red arrow) with no evidence of infiltration.