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Updated: Jun 22 2021

Melorheostosis

Images
https://upload.orthobullets.com/topic/8043/images/33b_moved.jpg
https://upload.orthobullets.com/topic/8043/images/300px-melorheostosis-002 ulna.jpg
https://upload.orthobullets.com/topic/8043/images/x-ray_of_hand melorheostosis.jpg
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https://upload.orthobullets.com/topic/8043/images/melorheostosis histology.jpg
  • summary
    • Melorheostosis is a rare benign painful disorder of the extremities characterized by formation of periosteal new bone. Patients typically present in the first 4 decades of life with localized pain and reduced range of motion. 
    • Diagnosis is made radiographically with a "dripping candle wax” appearance with dense hyperostosis that flows along the cortex of the bone.
    • Treatment is observation and medical management for those with mild symptoms and normal joint motion. Surgical bone resection and contracture release is indicated for those with severe symptoms and joint contractures. 
  • Epidemiology
    • Demographics
      • usually presents before age 40
      • no sex predilection
    • Anatomic location
      • more common in the lower extremities, but can occur in any bones
  • Etiology
    • Genetics
      • non-hereditary
  • Presentation
    • Symptoms
      • pain
      • reduced range of motion
      • joint contractures
    • Physical exam
      • fibrosis of the skin with significant induration and erythema is common
      • reduced range of motion
      • painful hyperostoses
  • Imaging
    • Radiographs
      • cortical hyperostosis
        • “dripping candle wax” appearance with dense hyperostosis that flows along the cortex of the bone
        • hyperostosis may flow across joints
  • Studies
    • Histology
      • normal haversian systems with enlarged bone trabeculae and without cellular atypia or mitotic figures
  • Treatment
    • Nonoperative
      • symptomatic treatment
        • indications
          • mild symptoms with adequate motion
          • bisphosphonates are shown to help with pain and swelling
    • Operative
      • hyperostotic bone resection with contracture release
        • indications
          • severe contractures, limited mobility, and pain
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