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Introduction
  • A condition characterized by idiopathic inflammatory disease of the skeleton that meets the following criteria
    • multiple sites of apparent osteomyelitis
    • pathology and cx’s are negative
    • no response to antibiotics
  • Epidemiology
    • demographics
      • primarily occurs in children/adolescents
      • peak age of onset is 10 years old
      • girls more common than boys
    • location
      • the tubular long bones and clavicle are most frequently affected
  • Pathophysiology
    • poorly understood
  • Associated conditions
    • Pustulosis palmoplantaris syndrome
      • a rare chronic relapsing condition causing red patches and pustules on the soles of the feet and palms of the hands
      • considered a rheumatologic condition and no infectious agents have been identified
      • following the outbreak o the lesions, desquamation occurs several days later
      • associated with CRMO
    • SAPHO Syndrome
      • CRMO associated with: synovitis, acne, pustulosis, hypersotosis, osteitis
  • Prognosis
    • natural history
      • characterized by periods of remission and exacerbation
      • typically resolves in 3-5 years
    • prognosis
      • traditionally thought as having a relatively benign sequelae
      • several case reports of growth disturbance have been reported.
Presentation
  • Symptoms
    • episodic fever
    • malaise
    • localized pain, swelling
  • Physical exam
    • focal tenderness in region of involvement
Imaging
  • Plain radiography
    • eccentric metaphyseal lesions with sclerosis and new bone formation  
  • Bone scan
    • Can help determine other sites of disease
  • MRI
    • If concerned for malignancy, MRI can help determine presence or absence of soft tissue involvement
Studies
  • Laboratory values
    • WBC typically normal
    • ESR and CRP may be elevated
  • Biopsy
    • biopsy and bone cultures negative
    • important to establish diagnosis which is one of exclusion
Treatment
  • Nonoperative
    • symptomatic treatment with NSAIDS and pamidronate
      • indications
        • treat during exacerbations
      • medications
        • pamidronate can provide symptomatic improvement and stimulate vertebral remodling
Complications
  • Growth disturbance
    • rare but may occur
 

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