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Fibrous Dysplasia : McCune-Albright Syndrome

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McCune-Albright syndrome consists of polyostotic fibrous dysplasia, endocrinopathy causing precocious puberty (usually in girls), and café-au-lait skin pigmentation. Other endocrinopathies such as hyperthyroidism, growth hormone-secreting pituitary adenomas, and primary adrenal hyperplasia may be seen. The bone lesions are often unilateral and the skin pigmentation is limited to the same side of the body.

Case History (Images 3-5): 18 y/o male with spine deformity. The thoracolumbar spine shows an S-shaped scoliosis convex to the right, centered at the T3/4 level, and convex to the left at the T10/11 level. The bones are diffusely sclerotic, with loss of corticomedullary differentiation, and with bony expansion. BONE AGE: The chronological age is 18 years. The bone age corresponds to a male aged 15 years 6 months. The bones of the hand have a ground glass appearance. This patient had established McCune-Albright Syndrome with polyostotic fibrous dysplasia and extensive skeletal changes (the next two images belong to the same case).

Case courtesy of A.Prof Frank Gaillard, Radiopaedia.org. From the case rID: 19348

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