- Discussion:
- consists of polyostotic
fibrous dysplasia, cafe'-au-lait spots of
"coast of Maine" variety, and premature menses;
- it is more common in girls than boys and accompanies approx 1 in 30
cases of polyostotic fibrous dysplasia;
- there is appreciable coincidence of other endocrinologic dz including
hyperthyroidism & Cushing's dz as well as hyperparathyroidism and
hypophosphatemic rickets;
- in polyostotic cases of fibrous dysplasia, dx is usually not difficult;
- in monostotic cases, the radiographs are frequently not diagnostic and
biopsy may be required;
- malignant transformation is seen in less than 0.5%, with osteogenic
sarcoma being the most common neoplasm;
-----------------------------
Freeman BH, Bray EW, Meyer LC:
Multiple osteotomies with Zickel nail
fixation for polyostotic fibrous dysplasia involving the proximal part of
the femur.
J Bone Joint Surg 1987;69:691-698.
Enneking WF, Gearen PF:
Fibrous dysplasia of the femoral neck: Treatment
by cortical bone grafting.
J Bone Joint Surg 1986;68A:1415-1422.
