[MacCune-Albright syndrome. Apropos of a case]

The MacCune-Albright's syndrome as described by these authors in 1936 and 1937 is extremely rare if one considers the complete form consisting in the triad: café-au-lait spots, fibro-osseous dysplasia, endocrine disturbances. We report an additional case in a 5 year old girl with an precocious puberty. Within a 10 year follow up occurred a polyostotic fibrous dysplasia with pathologic bone fractures in cystic areas, and a right exophthalmos induced by a cyst of the large wing of the sphenoid bone. All these lesions led to orthopaedic and neural surgical procedures. The early puberty of central origin (as proved by the LH-RH test) was treated during 7 years by medroxyprogesterone acetate with an excellent result. No peripheral endocrine syndrome was evidenced. This case stresses the multiple problems arising in the disease and the necessity of a long lasting careful follow up.