Fibrous dysplasia
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Fibrous dysplasia

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Fibrous dysplasia Presentation Transcript

  • 1. FIBROUS DYSPLASIA Dr/ Hytham Nafady
  • 2. Definition  replacement of the norm lam al ellar cancellous bone by abnorm fibrous tissue. al
  • 3. Types of fibrous dysplasia
  • 4. Demographics   Age: 3-30. Sex: F > M.
  • 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. Complications of fibrous dysplasia 1. 2. 3. 4. 5. Pathological fracture. Bone deformity. Massive cartilage hyperplasia. Accelerated bone growth. Sarcomatous degeneration.
  • 7. Pathological fracture
  • 8. Shepherd crock deformity
  • 9. Sarcomatous degeneration  is very rear, it may occur    spontaneously or following radiation therapy. Radiological Criteria:   Cortical destruction. Extraosseous soft tissue component.
  • 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. 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. 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. Rib fibrous dysplasia  Expansile lytic lesion with no rib destruction.
  • 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. Special forms of fibrous dysplasia    Leontiasis ossea. Cherubism. Mc Cune Albright syndrome.
  • 16. Leontiasis ossea  A special form of polystotic fibrous dysplasia that affects the skull & facial bones.
  • 17. Cherubism  Familial fibrous dysplasia of the jaws.
  • 18. Mc Cune Albright syndrome  Almost exclusively affect females.
  • 19. Mc Cune Albright syndrome Never cross the midline and Irregular borders (coast of Maine) Neurofibromatosis Cross the midline. Smooth borders (coast of California)