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Dysplasia Epiphysealis Hemimelica (Trevor's Disease)

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Topic updated on 11/13/13 3:29pm
Introduction
  • A form of bone dysplasia caused by development of a benign cartilage lesion in the epiphysis
    • osteochondroma that occurs in the epiphysis
  • Epidemiology
    • incidence
      • extremely rare
      • incidence is estimated at ~ 1:1,000,000
    • demographics
      • recognised male predilection
      • usually presents in young children
    • location
      • most common in knee or ankle
      • usually only involves one joint
  • Pathophysiology
    • caused by a defect in the Goove of Ranvier
    • asymmetrical limb deformity due to localized overgrowth of cartilage
  • Genetic
    • non hereditary
  • Prognosis
    • may lead to early osteoarthritis of affected joint
Presentation
  • Symptoms
    • asymmetrical limb deformity
    • joint symptoms
  • Physical exam
    • decreased motion
    • joint line tenderness
Imaging
  • Radiographs
    • may see calcification at the joint line
  • CT
    • useful for assessing cortical continuity with the epiphysis  
Studies
  • Histology
    • similar to osteochondroma 
    • diagnosis of DEH is made with the location of the osteochondroma, specifically located in the epiphysis of the involved bone
Treatment
  • Operative
    • excision of overgrowth
      • indications
        •  symptomatic early disease
    • osteotomies
      • indications
        • symptomatic late stage disease
Complications
  • Recurrence
    • a common complication following excision

 

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