Fibrous dysplasia

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[edit] Discussion

  • Defect in osteoblastic differentiation and maturation resulting in replacement of medullary bone with fibrous tissue
  • Virtually any bone in the body can be affected.
  • It is a nonhereditary disorder of unknown cause.

[edit] Monostotic

  • Accounts for approx 70-80% of fibrous dysplasias.
  • Location in decreasing order of frequency:
    • Rib (28%)
    • Femur (23%)
    • Tibia
    • Craniofacial bones (10-25%)
    • Humerus
    • Vertebrae
  • The degree of bone deformity of the monostotic form is relatively less severe than that of the polyostotic type.

[edit] Polyostotic form

  • Account for approximately 20-30% of fibrous dysplasias.
  • Sites of involvement are:
    • Femur (91%)
    • Tibia (81%)
    • pelvis (78%)
    • Ribs
    • Other locations include skull and facial bones, upper extremities, lumbar spine, clavicle, and cervical spine.
  • The dysplasia may be unilateral or bilateral, and it may affect several bones of a single limb or both limbs with or without axial skeleton involvement.

[edit] Other features

[edit] McCune-Albright syndrome

  • Sexual precocity in girls, with polyostotic fibrous dysplasia and cutaneous pigmentation (cafe-au-lait spots).

[edit] Mazabraud's syndrome

  • Intramuscular myxomas associated with fibrous dysplasia of bone (written boards question)

[edit] Imaging Findings

[edit] Images

Patient #1: Polyostotic fibrous dysplasia

Patient #2: McCune-Albright Syndrome

Patient #3: Shepard's crook deformity

[edit] See Also

[edit] External Links

[edit] References

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