Posterior corneal pigmentation in melanosis oculi.

A 5-year-old girl was seen with findings consistent with melanosis oculi and notably decreased vision secondary to dense corneal pigmentation. A penetrating keratoplasty was performed without complications. Light microscopy disclosed dense pigment granules layered in deep stroma just anterior to Descemet's membrane. Electron microscopy revealed these to be melanocytes, without evidence of phagocytic activity. The corneal pigmentation may have resulted from a misdirection of melanocytes in embryogenesis. To our knowledge, the finding of visually notable corneal pigmentation has not been previously reported in association with the syndrome of melanosis oculi.