Congenital grouped pigmentation of the retina presents an uncommon but characteristic ophthalmoscopic picture, which was well described by Stephenson1 in 1891. This author, in reporting 3 cases, said: "The changes, which occupy a sector-like portion of the fundus, consist of groups made up of black to dark chocolate-brown spots, which, as seen by the direct method, range in size from ½ to 2 mm. or more ; in general terms it may be said that the farther the spots lie from the optic disc the larger they become."
A study of the cases reported in the literature shows that the clinical picture of this entity, which has also been called melanosis of the retina and nevoid pigmentation of the fundus, includes certain characteristic findings: 1. The condition is unilateral and more frequent in males than in females. 2. There is no hereditary aspect, and consanguinity does not play a role.
PERERA CA. CONGENITAL GROUPED PIGMENTATION OF THE RETINA: REPORT OF A CASE. Arch Ophthalmol. 1939;21(1):108–110. doi:10.1001/archopht.1939.00860010124010
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