Fenestrated Sheen Macular Dystrophy

To the Editor. —We have recently had the opportunity to reexamine the family with fenestrated sheen macular dystrophy that we described in the JulyArchives(97:1292-1296,1979). In the 18 months since their initial examination by us, we have photographic evidence of progression (Fig 1 and 2) in the young affected patient and his grandfather. The visual acuity in the grandfather has slipped to 20/25 in both eyes. The results of color vision tests (100-hue) are essentially unchanged. The electroretinograms were repeated in all patients. All were found to have normal photopic and scotopic responses.