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Question answered:12/04/07 Warning! this question is over two years old.
We found very little information on polyostotic fibrous dysplasia from our usual sources. The TRIP database did find two e-textbooks (1-2) on fibrous dysplasia. Both chapters discuss the polyostotic form throughout, however, in the first e-textbook the author discusses the polyostotic form in detail, which we have reproduced below:
“Approximately 20-30% of fibrous dysplasias are polyostotic. Polyostotic fibrous dysplasia more frequently involves the skull and facial bones, pelvis, spine, and shoulder girdle. The sites of involvement are the femur (91%), tibia (81%), pelvis (78%), ribs, skull and facial bones (50%), upper extremities, lumbar spine, clavicle, and cervical spine, in decreasing order of frequency. 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. Although the polyostotic variety tends to occur in a unilateral distribution, involvement is asymmetric and generalized when disease is bilateral.
Two thirds of patients are symptomatic before they are 10 years of age. Often, the initial symptom is pain in the involved limb associated with a limp, a spontaneous fracture, or both. In one series, pathologic fracture was present in 85% of polyostotic fibrous dysplasias. Leg-length discrepancy of varying degrees occurs in about 70% of patients with limb involvement. The structural integrity of the bone is weakened, and the weight-bearing bones become bowed. The curvature of the femoral neck and proximal shaft of the femur markedly increase because a femoral lesion commonly causes a severe coxa vara abnormality, shepherd's-crook deformity, which is a characteristic sign of the disease. Overgrowth of adjacent soft tissues may be present”.
The authors also suggest serum alkaline phosphatase levels are often elevated in polyostotic fibrous dysplasia.
http://www.emedicine.com/radio/topic284.htm http://www.emedicine.com/orthoped/topic487.htm
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