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MACULAR DYSTROPHY

Originally posted on @retina.rocks 07/29/2021

This 74YO female came in to see us for a second opinion for age-related macular degeneration. She was visually asymptomatic and denied prior Elmiron (pentosan polysulfate sodium, PPS) use. Vision was 20/30 OU.

Bilateral drusenoid/fleck-like yellow-orange subretinal lesions are scattered throughout the macula and peripapillary regions. These are variably hyper- or hypofluorescent. OCT scanning shows variable outer retinal atrophy without drusen.

Learning Points:
We believe these findings likely represent a type of macular dystrophy. However, she declined genetic testing.

PPS, a medication used to treat interstitial cystitis, can also mimic the findings in age-related macular degeneration and macular dystrophies. Peripapillary hypoautofluorescence, more densely packed macular autofluorescent changes, and earlier central macular involvement suggest PPS toxicity over other causes (see Barnes et al, Ophthalmology Retina 2020;4:1196-1201). Thus, it is important to perform thorough history-taking and document all medications the patient is taking.