A 57-year-old woman was referred for a routine screening exam without visual complaints. She had no family history of retinal disease. Visual acuity was 20/20 OU. Multimodal imaging depicted bilateral pathology.

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DIAGNOSIS

Diagnosis: Pentosan polysulfate maculopathy

This patient was a chronic user of pentosan polysulfate and was referred for a routine screening exam. On examination, findings of pentosan maculopathy were found and cessation of the medication was advised. Her exam has remained stable with preserved 20/20 OU visual acuity. Pentosan polysulfate is a commonly prescribed medication for interstitial cystitis. Recent studies have demonstrated an associated unique maculopathy related to increased dosage and duration of usage. Many patients can be asymptomatic, however symptoms of nyctalopia and blurred vision often arise. The clinical picture often consists of paracentral macular hyperpigmentation and yellow deposits. Autofluoresence demonstrates a clustering of hyper- and hypopigmented spots which can extend outside of the arcades. OCT demonstrates hyperreflective nodules at the level of the RPE. Currently there are no recommended guidelines for screening. There is no known treatment for pentosan maculopathy besides cessation of the medication. Affected patients should undergo continued monitoring to evaluate for associated CME or CNV.

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