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Virtual Pediatric Hospital: Paediapaedia: Fibrous Dysplasia, Polyostotic Paediapaedia: Musculoskeletal Diseases

Fibrous Dysplasia, Polyostotic

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed

Clinical Presentation:
Starts in the first two decades of life. There is sexual precocity in 23% of females with polyostotic fibrous dysplasia (McCune-Albright Disease and cafe-au-lait spots may be present.

Extensive proliferation of fibrous tissue which destroys and replaces normal bone.

Not applicable

Imaging Findings:
A single bone is involved. A cystic lesion can be found in the shafts of the tubular, flat, and round bones. The lesion has a ground glass appearance due to calcification of the tumor matrix. The cortex can be expanded and thin, and the spongiosa can be sclerotic. There is no periosteal reaction unless there is a pathological fracture. Skull involvement is characterized by unilateral or predominantly unilateral hyperostotic reaction with diffuse sclerosis of the skull base and facial bones with the paranasal sinuses usually obliterated.


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