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Fibrous dysplasia


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Fibrous dysplasia

  1. 1. FIBROUS DYSPLASIA Dr/ Hytham Nafady
  2. 2. Definition  replacement of the norm lam al ellar cancellous bone by abnorm fibrous tissue. al
  3. 3. Types of fibrous dysplasia
  4. 4. Demographics   Age: 3-30. Sex: F > M.
  5. 5. Natural history    P olystotic F more aggressive than m Dis onostotic F D. The lesions usually progress in number till the end of skeletal maturation, by then they become quiescent in only about 5% of cases it continue to enlarge after that.
  6. 6. Complications of fibrous dysplasia 1. 2. 3. 4. 5. Pathological fracture. Bone deformity. Massive cartilage hyperplasia. Accelerated bone growth. Sarcomatous degeneration.
  7. 7. Pathological fracture
  8. 8. Shepherd crock deformity
  9. 9. Sarcomatous degeneration  is very rear, it may occur    spontaneously or following radiation therapy. Radiological Criteria:   Cortical destruction. Extraosseous soft tissue component.
  10. 10. Radiological criteria Location :  Long bones specially the neck of femur  Skull (including calvarium, skull base, facial bones & mandible),  Pelvis  Ribs (fibrous dysplasia is the most common cause of rib expansion). Distribution:  Unilateral or rarely bilateral asymmetrical.
  11. 11. Long bone fibrous dysplasia Meta-diaphyseal. The epiphysis is usually spared.  Central  May be expansile.  Cortex thinned or scalloped with no cortical break through or periosteal reaction (smooth outer cortex). Density:  depend upon the ratio between the fibrous & osseous tissues,     Sclerotic (increased osseous content), or Lytic (increased fibrous content) or Ground glass, (relatively equal ratios between fibrous & osseous content). Margin:  Well defined sclerotic margin (Geographic bone destruction).  The sclerotic margin may be thick (rind sign).
  12. 12. Pelvis fibrous dysplasia   Expansile, lytic, bubbly lesion. The degree of expansion & the bubbly nature are quite marked in the pelvis rather than long bones.
  13. 13. Rib fibrous dysplasia  Expansile lytic lesion with no rib destruction.
  14. 14. Calvarium Widening of the diploic space. Displacement of the outer table. Sparing of the inner table. Density: Skull base Narrowing of the neural foramina. Facial deformities •Common •Lytic or mixed DDx: •Sclerotic or Ground glass, Facial bones •Rare Paget disease Skull base tumors (the inner table is involved). Fibrous dysplasia conforms to the shape of the involved bone. Ossifying fibroma
  15. 15. Special forms of fibrous dysplasia    Leontiasis ossea. Cherubism. Mc Cune Albright syndrome.
  16. 16. Leontiasis ossea  A special form of polystotic fibrous dysplasia that affects the skull & facial bones.
  17. 17. Cherubism  Familial fibrous dysplasia of the jaws.
  18. 18. Mc Cune Albright syndrome  Almost exclusively affect females.
  19. 19. Mc Cune Albright syndrome Never cross the midline and Irregular borders (coast of Maine) Neurofibromatosis Cross the midline. Smooth borders (coast of California)