Ota syndrome (nevus of ota; oculodermal melanocytosis; nevus fuscoceruleus-ophthalmomaxillaris syndrome)

General: Affects mainly black and Japanese populations; female preponderance (4:1); mode of transmission unknown; most frequently unilateral; pigmentary changes frequently spread during puberty, but no malignant transformation occurs; malignant transformation to melanoma in the uvea and orbit has been reported.

Ocular: Congenital benign periorbital pigmentation of brown, slate to bluish-black coloration, involving area of first and second (rarely third) division of trigeminal nerve; unilateral hyperchromic heterochromia iridis; possible scleral and conjunctival pigmentation; trabeculae heavily pigmented; slate-gray hyperpigmentation of fundus; optic disk pigmentation (occasionally).

Clinical: Pigmentation of temples, nose, forehead, and malar region; 'Mongolian spot' in sacral area (present at birth but usually disappears after puberty).

Bordon AF, et al. Choroidal malignant melanoma in association with oculodermal melanocytosis in a black patient. Br J Ophthalmol 1995; 79:191-l92.

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

Cowan TH, Balistocky M. The nevus of Ota or oculodermal melanocytosis: the ocular changes. Arch Ophthalmol 1961; 65:483.

Fraunfelder FT, Roy FH. Current Ocular Therapy, 5th ed. Philadelphia: WB Saunders, 2000.

Ota M. Nevus fusco-caeruleus ophthalmomaxillaris. Tokyo Med J 1939; 63:1243.

Seregard S, et al. Multiple melanocytic tumors in a case of ocular melanocytosis. Acta Ophthalmol 1993; 71:562-565.