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Updated: Jun 21 2021

Osteofibrous Dysplasia

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  • summary
    • Osteofibrous Dysplasia is a rare benign tumor-like condition that primarily affects the cortices of the tibia.
    • The condition typically presents in patients less than 10 years of age with painless anterior shin swelling. 
    • Diagnosis is made by a combination of radiographs showing an anterior eccentric lytic tibial lesion and biopsy showing fibroblast proliferation surrounding islands of woven bone with osteoblastic rimming.
    • Treatment is usually observation alone as lesions usually regress and do not cause problems in adulthood.
  • Epidemiology
    • Demographics
      • usually found in younger children (< 10 years old)
      • males>females
    • Anatomic location
      • primarily in anterior tibia
  • Etiology
    • Genetics
      • trisomy 7, 8, 12, 22 have been reported
      • does NOT have Gs alpha activating mutation like fibrous dysplasia has
  • Presentation
    • Symptoms
      • may be asymptomatic
      • painless swelling
      • anterior or anterolateral bowing of the tibia
      • pseudoarthrosis develops in 10-30% of patients
    • Physical exam
      • may have local tenderness over the tibia
  • Imaging
    • Radiographs
      • recommended views
        • AP and lateral of affected area
      • findings
        • anterior eccentric lytic tibial lesion in child that often leads to tibial bowing
        • usually diaphyseal
        • no periosteal reaction
        • confined to anterior cortex
      • radiographic differential includes adamantinoma
  • Studies
    • Histology
      • histology similar to fibrous dysplasia EXCEPT osteoblastic rimming is present
        • fibroblast proliferation surrounding islands of woven bone with osteoblastic rimming
        • mitotic figures are common
        • may have giant cells
  • Differential
    • Adamantinoma 
      • Osteofibrous Dysplasia Differential 
      • Tibial diaphysis lesion
      • Treatment is Observation alone 
      • Osteofibrous dysplasia
      • o
      • o
      • Fibrous dysplasia
      • o
      • Adamantinoma
      • o
      • Eosinophilic granuloma
      • o
      • Enchondroma / Olliers / Maffucci's
      • o
      • Osteochondroma / MHE
      • o
      • NOF /Jaffe-Campanacci syndrome
      • o
      • Paget's
      • o
  • Treatment
    • Nonoperative
        • indication
          • first line of treatment
          • alone is the treatment for most patients
      • bracing
        • indication
          • if deformity significant and interfering with walking
    • Operative
      • deformity correction with osteotomy
        • indication
          • rarely needed
          • significant deformity
          • perform after skeletal maturity
  • Prognosis
    • Lesions usually regress and do not cause problems in adulthood
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