The LESION of congenital melanosis oculi consists of a large number of dendritic melanocytes of normal appearance similar to those usually found in the uvea. The melanosis is most frequently located in the episclera, sclera, and uvea, occasionally involving the optic nerve, meninges, and orbital tissue.1 According to Reese, melanocytes of congenital melanosis often become malignant. He reported that malginant melanoma of the choroid developed in 13% to 25% of cases of melanosis oculi.2 For instance, eight out of 27 was the incidence in one of his surveys.3 Hogan and Zimmerman, however, stated that melanosis oculi rarely became malignant.1 Recent communication indicates that only 12 cases of malignant melanomas secondary to melanosis oculi (of which this is one) have been registered at the Armed Forces Institute of Pathology.4 The melanosis oculi is typically congenital and occasionally hereditary. Malignant changes take place late in life at
Sabates FN, Yamashita T. Congenital Melanosis Oculi: Complicated by Two Independent Malignant Melanomas of the Choroid. Arch Ophthalmol. 1967;77(6):801–803. doi:10.1001/archopht.1967.00980020803018
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