General Considerations

• One of a group of sclerosing bone disorders
• Rare
• Cause is unknown
• Produces thickening of the endosteum and periosteum
• Peak age of presentation is 5-20 years
• May be monostotic or polyostotic
  • May involve one entire limb
    • Usually does not involve multiple limbs
• Twice as common in lower extremities than elsewhere

Clinical Findings

• About 50% affected develop symptoms by age 20
• Adults present with
  • Pain
• Joint stiffness
• Deformity that may progress over time
• Children may present with
  • Leg length discrepancies
• Joint contractures

Imaging Findings

• Patterns described include:
  • Resembling osteoma(s)
• Candle-wax appearance (classic)
• Resembling myositis ossificans
• Resembling osteopathia striata
• Mixed
• Sclerotic lesions of cortical bones, usually in the diaphysis, that resemble “candle-wax-dripping”
• Cortical hyperostosis with an undulating appearance usually affecting one side of a bone
• Soft tissue lesions that may calcify
  • Adjacent to involved bone
• May grow to compress nerves
• Usually low signal on MRI
  • Enhance with Gadolinium
• Bone scan is markedly positive

Differential Diagnosis

• Osteopathia striata
  • Longitudinal dense striations
• Osteopoikilosis
  • Punctate, rounded bone islands surrounding joints
• Osteosarcoma
  • Bone destruction

Treatment

• Analgesia
• Nerve blocks
• Surgery directed at relieving contracture

Complications

• Associated soft tissue lesions and cutaneous lesions
  • Vascular malformations
  • Neurofibromatosis
  • Tuberous sclerosis
  • Hemangioma
• Muscle contractures
• Scoliosis

Prognosis

• Progressive disorder

 

 

Melorheostosis. Frontal and oblique radiographs of the hand shown in close-up demonstrate the
undulating cortical hyperostosis representing the classical "candle-wax" dripping appearance
involving only the radial side of the 3rd metacarpal shaft.
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