Fibrous Dysplasia

Overview

Fibrous dysplasia is a developmental abnormality that results in abnormal growth, pain and deformity of the affected bones.

The condition is subdivided into three types:

• Monomelic: This is the least complicated form of fibrous dysplasia. It only affects one bone, most often the femur (thigh), tibia (shin), rib cage or one of the facial bones.
• Polyostotic: This form of fibrous dysplasia, which affects a younger age group than the monolemic form, can involve numerous bones, sometimes more than half of all bones in the skeletal system.
  • In addition to bone deformity, this condition can lead to fractures, skin lesions and legs of different lengths.
• McCune-Albright syndrome: This form of fibrous dysplasia, the most complex, is associated with abnormalities of the endocrine system. Some or all of the following problems can be involved:
  • polyostotic fibrous dysplasia
  • skin pigmentation
  • premature puberty
  • acromegaly
  • hyperparathyroidism
  • vitamin D-resistant rickets
  • hyperthyroidism
  • Cushing Syndrome
  • short stature because of the premature closing of growth plates in the affected bones

Treatment with medication?

There are early studies that suggest that drugs that alter bone resorption may help the pain of fibrous dysplasia and may strengthen the bone. At this time, this therapy is not of proven value, but it does hold promise for the future. Researchers at Boston Children's Hospital are continuously investigating new forms of treatment.