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Ocular Melanocytosis
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     A nine-year-old girl presented for evaluation of hyperpigmentation of her left eye (Panel A). The condition had been present since birth. The visual acuity was 20/20 in each eye. Slit-lamp examination revealed patchy, brownish discoloration of the left episclera (Panel B). The right iris appeared normal (Panel C). The left iris had a dark velvety, nodular appearance with ectropion uveae and loss of iris crypts (Panel D). The fundus of the right eye (Panel E) was lighter than the fundus of the left eye (Panel F); both fundi were otherwise normal. The periocular skin, pupillary responses, and intraocular pressures were normal and symmetric. Congenital ocular melanocytosis is characterized by diffuse melanocytic hyperplasia of the episclera and uvea and holds the potential for the development of melanoma of the uvea, orbit, or meninges, as well as glaucoma. The diagnosis should prompt periodic ophthalmologic examinations for early recognition of related ophthalmic disorders.

    James Chodosh, M.D.

    Russell D. Burris

    Dean McGee Eye Institute

    Oklahoma City, OK 73104