Albright syndrome

General: Etiology unknown; disease rare; manifested in children and young adults; found predominantly in females.

Ocular: Unilateral proptosis; papilledema; optic atrophy; lacrimal fossa mass; acute or chronic monocular visual loss.

Clinical: Medullary structures replaced by fibrous dysplasia; pelvic bones and lower extremities most frequently involved (spontaneous fractures); brown pigmented areas of skin, from small, freckle-like dots to large, flat patches on thighs, sacrum, upper spine, neck, and scalp; endocrine dysfunction (precocious puberty in females) with early menarche, adolescent external genitalia, and breast enlargement; loss of hearing; convulsions; mental retardation.

Albright F, et al. Syndrome characterized by osteitis fibrosa disseminata, areas of pigmentation and endocrine dysfunction, with precocious puberty in females. N Engl J Med 1937; 16:727.

Arroyo JG, et al. Steroid induced visual recovery in fibrous dysplasia. J Clin Neuro-Ophthalmol 1991; 11:259-261.

Bland LI, et al. Acute monocular blindness secondary to fibrous dysplasia of the skull: a case report. Ann Ophthalmol 1992; 24:263-266.

Collins JF. Handbook of Clinical Ophthalmology. New York: Masson, 1982:260.

Lods F. Ophthalmologic localization of an unusual disease: Jaffe-Lichtenstein syndrome or an approximate form of Albright's syndrome. Bull Soc Ophthalmol Fr 1970; 70:1220.

McCluskey P, et al. Monostotic fibrous dysplasia of the orbit: an unusual lacrimal fossa mass. Br J Ophthalmol 1993; 77:54-56.