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Fibrous Dysplasia

 

 

  • Etiology unknown

  • Most common 3-15 years

  • Fundamental abnormality is replacement of medullary bone by fibrous tissue

  • Clinically

    • Deformities

      • Shepherd’s crook deformity of femur

    • Bone pain

  • Most commonly involved bones are pelvis, femora

  • In widespread disease, the skull and jaw are almost always involved

  • X-ray

    • Endosteal scalloping

    • Cortical thinning

    • Ground-glass appearance

    • May have matrix calcification

  • Prone to fracture

  • Growth of lesions usually stops when epiphyses close

  • DDX:

    • Brown tumor

    • Unicameral bone cyst

  • Albright’s Syndrome

    • Polyostotic

    • Sexual precocity

    • Skin pigmentation

    • Almost always in a female

  • Polyostotic form is usually bilateral

 

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