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Updated: Jun 22 2021

Focal Fibrocartilaginous Dysplasia

Images fibrocartilaginous dysplasia xray.jpg fibrocartilaginous dysplasia histology.jpg
  • summary
    • Focal Fibrocartilaginous Dysplasia is a rare benign bone dysplasia that leads to unilateral varus of the tibia, most commonly seen in infants. 
    • Diagnosis is made with radiographs showing an abrupt varus deformity at the metaphyseal–diaphyseal junction of the tibia with cortical sclerosis at the medial cortex.
    • Treatment is usually observation as the majority of lesions spontaneously correct with normal proximal tibial physeal growth. Rarely, surgical deformity correction is indicated in the presence of deformity progression. 
  • Epidemiology
    • Demographics
      • usually seen in infant or toddlers
    • Anatomic location
      • most common location is tibia
      • may also occur in humerus, forearm, phalanx, and femur
  • Etiology
    • Pathophysiology
      • etiology and the pathogenesis of the deformity are unknown
    • Associated conditions
      • infantile tibia vara
        • important to recognize this variation of infantile tibia vara as it can resolve without surgery
  • Presentation
    • Physical exam
      • unilateral tibia vara
      • knee hyperextension with lateral thrust can be seen
  • Imaging
    • Radiographs
      • abrupt varus at the metaphyseal–diaphyseal junction of the tibia
      • cortical sclerosis at the medial cortex
      • radiolucency may appear just proximal to area of cortical sclerosis corresponding to the fibrocartilaginous tissue
  • Studies
    • Histology
      • prominent layers of collagenic fibrous tissue with thick dense fibrocartilaginous tissue
  • Treatment
    • Nonoperative
      • observation
        • indications
          • majority spontaneously correct because of the normal proximal tibial physeal growth
    • Operative
      • deformity correction
        • indications
          • may be necessary if the deformity progresses or fails to resolve during a period of observation or orthotic management
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