Fibrous dysplasia is a common benign skeletal lesion that may involve one
bone (monostotic) or multiple bones (polyostotic) and occurs throughout the
skeleton with a predilection for the long bones, ribs, and craniofacial
bones.
The etiology of fibrous dysplasia has been linked to an activating mutation
in the gene that encodes the a subunit of stimulatory G protein
(Gsa) located at 20q13.2-13.3.
Most lesions are monostotic, asymptomatic, and identified incidentally and
can be treated with clinical observation and patient education.
Bisphosphonate therapy may help to improve function, decrease pain, and
lower fracture risk in appropriately selected patients with fibrous
dysplasia.
Surgery is indicated for confirmatory biopsy, correction of deformity,
prevention of pathologic fracture, and/or eradication of symptomatic lesions.
The use of cortical grafts is preferred over cancellous grafts or bone-graft
substitutes because of the superior physical qualities of remodeled cortical
bone.