To describe a potential case of pentosan polysulfate maculopathy that seemed to manifest nearly 3 years after drug cessation.
Complete ophthalmic examination, including multimodal fundus imaging, electroretinography, automated perimetry, and molecular testing, was performed.
A 44-year-old woman with a 435 g cumulative exposure to pentosan polysulfate sodium presented 38 months after drug cessation with 6 months of worsening metamorphopsia and prolonged dark adaptation. Fundus examination and multimodal fundus imaging demonstrated characteristic features of pentosan polysulfate maculopathy, and molecular testing was unremarkable. By contrast, color fundus photographs of the same patient acquired at an outside facility 25 months before did not display features consistent with pentosan polysulfate sodium maculopathy.
This case suggests that new-onset clinically detectable pentosan polysulfate maculopathy may develop years after drug cessation. If corroborated, this finding has important ramifications for pentosan polysulfate sodium dosing and surveillance guidelines.