- 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
- ref: Images in Clinical Medicine. Albright's Hereditary Osteodystrophy
Multiple osteotomies with Zickel nail fixation for polyostotic fibrous dysplasia involving the proximal part of the femur.
Fibrous dysplasia of the femoral neck. Treatment by cortical bone-grafting.
Images in clinical medicine. Albright's hereditary osteodystrophy.
Original Text by Clifford R. Wheeless, III, MD.
Last updated by Data Trace Staff on Tuesday, November 29, 2016 4:53 am