Updated: 10/6/2016

Focal Fibrocartilaginous Dysplasia

Topic
Review Topic
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Questions
1
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Evidence
2
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https://upload.orthobullets.com/topic/8079/images/focal fibrocartilaginous dysplasia xray.jpg
https://upload.orthobullets.com/topic/8079/images/focal fibrocartilaginous dysplasia histology.jpg
Introduction
  • A benign dysplasia that leads to unilateral varus of the tibia (most common location)
  • May also occur in humerus, forearm, phalanx, and femur
  • Epidemiology
    • demographics
      • usually seen in infant or toddlers
  • 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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Questions (1)

(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? Review Topic

QID: 302
FIGURES:
1

Focal fibrocartilaginous dysplasia

77%

(1185/1534)

2

Neurofibromatosis

9%

(143/1534)

3

Toddler's fracture

1%

(20/1534)

4

Non-ossifying fibroma

10%

(155/1534)

5

Aneurysmal bone cyst

2%

(27/1534)

ML 2

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