Updated: 6/22/2021

Focal Fibrocartilaginous Dysplasia

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  • 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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Flashcards (2)
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(OBQ06.116) The parents of an 18-month-old toddler are concerned about bowing of the child's left lower extremity. The child began ambulating at 13 months of age and continues to ambulate with no overt signs of pain. Clinical photograph, radiographs, bone scan, CT, and histopathology are shown in Figures A through E. Which of the following is the most likely diagnosis?

QID: 302
FIGURES:
1

Focal fibrocartilaginous dysplasia

77%

(1765/2278)

2

Neurofibromatosis

9%

(212/2278)

3

Toddler's fracture

1%

(28/2278)

4

Non-ossifying fibroma

10%

(226/2278)

5

Aneurysmal bone cyst

2%

(42/2278)

L 2 D

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