Potential New Phenotypic Presentation of Pentosan Polysulfate Sodium Maculopathy

Purpose: To describe a potential new phenotypic presentation of pentosan polysulfate sodium maculopathy. Methods: An observational case was analyzed. Results: A 55-year-old woman with interstitial cystitis who was treated with oral pentosan polysulfate sodium for 10 years presented with reports of decreased vision and metamorphopsia that had worsened over the past 3 months. On funduscopic examination, there were bilateral areas of retinal pigment epithelial hyperplasia inferior to the fovea with a surrounding halo of drusenoid material. Neurosensory retinal detachments with drusenoid deposition at the edges of the detachment were seen on spectral-domain optical coherence tomography. Conclusions: These findings could be mistaken for age-related macular degeneration, central serous retinopathy, Best disease, or adult vitelliform dystrophy. In this case, a review of pertinent medications assisted in making the diagnosis of toxic maculopathy. Proper diagnosis and prompt discontinuation of the inciting medication are necessary to avoid the risk for potentially irreversible retinal damage and vision loss.