Pentosan Polysulfate Associated Maculopathy
A patient presents with a history of difficulty reading and progressive blurry vision in both eyes prior to her presentation. Patient has a significant history of interstitial cystitis with long term use of Pentosan Polysulfate Sodium for 20 years. Her exam was notable for a visual acuity of 20/25 in the right eye and 20/30 in the left eye. Slit lamp examination shows no significant abnormalities except for mild cataract with normal IOP. Posterior segment exam shows macular pigmentary mottling and RPE deposits mainly centrally located and extend slightly nasal to the disc OU. FAF shows a significant pattern of mixed hyper/hypoautofluorescent spots with areas of RPE atrophy, in addition to a hypoautofluorescent halo around the nerve OU. SD-OCT shows RPE excrescences with areas of focal atrophy OU. Genetic work up was done and revealed negative results. Diagnosis was made clinically based on medical history and exam findings. Patient was advised to stop PPS immediately with further emphasis on the possibility of disease progression even after drug cessation.
Presentation Date: 06/08/2023
Issue Date: 06/23/2023
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