A case of atypical McCune-Albright syndrome requiring optic nerve decompression.
McCune-Albright syndrome (MAS) is a disease of noninheritable, genetic origin defined by the triad of café-au-lait pigmentation of the skin, precocious 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 such as the proximal aspects of the femur and the pelvis, the craniofacial region is frequently involved. A male patient with MAS presented with juvenile 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 deterioration and its surgical management are presented.
Yavuzer
R
R
Institute for Craniofacial and Reconstructive Surgery, Southfield, MI, USA.
Khilnani
R
R
Jackson
I T
IT
Audet
B
B
D000293:Adolescent; D019299:Decompression, Surgical; D005359:Fibrous Dysplasia, Polyostotic / Q000150:complications* / Q000000981:diagnostic imaging; D006801:Humans; D008297:Male; D009408:Nerve Compression Syndromes / Q000209:etiology*; D020221:Optic Nerve Injuries / Q000209:etiology*; D011859:Radiography