A case of atypical McCune-Albright syndrome requiring optic nerve decompression

McCune-Albright syndrome (MAS) is a disease of noninheritable, genetic orig in defined by the triad of cafe-au-lait pigmentation of the skin, precociou s puberty, and polyostotic fibrous dysplasia, This syndrome, which affects young girls primarily, has also been reported with other endocrinopathies, and rarely with acromegaly and hyperprolactinemia. The fibrous dysplasia in MAS is of the polyostotic type and, apart from the characteristic sites su ch as the proximal aspects of the femur and the pelvis, the craniofacial re gion is frequently involved. A male patient with MAS presented with juvenil e gigantism, precocious puberty, pituitary adenoma-secreting growth hormone and prolactin, hypothalamic pituitary gonadal and thyroid dysfunction, and polyostotic fibrous dysplasia causing optic nerve compression. Visual dete rioration and its surgical management are presented.