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About the Author Sakshi Vasiu is a 2024 graduate of SUNY College of Optometry where she obtained her Doctor of Optometry and Master of Science in Vision Sciences degrees. She is currently completing a residency in Ocular Disease and Contact Lenses at Massachusetts Eye and Ear Infirmary.
References: Lim, Yizhe, et al. “Interstitial Cystitis/Bladder Pain Syndrome.” StatPearls, StatPearls Publishing, 7 October 2024.Marcu, Ioana et al. “Interstitial Cystitis/Bladder Pain Syndrome.” Seminars in reproductive medicine vol. 36,2 (2018): 123-135. doi:10.1055/s-0038-1676089Lee, Jongsoo et al. “Pentosan Polysulfate Sodium and Maculopathy in Patients with Interstitial Cystitis: A Systematic Review and Meta-Analysis.” The world journal
Questions for You… Do we need to consider another etiology such as infectious, malignant or other toxicity such as Vitamin A deficiency and acquire blood work testing (for TB, syphilis, Vit. A deficiency) and fluorescein angiography? Or is the current workup enough to diagnose pentosan polysulfate maculopathy?
Discussion/Takeaways: Drug induced retinopathies should not be overlooked in patients with nyctalopia.It is imperative to do an extended retinal examination of patients reporting visual distortions or difficulty in seeing in dim lighting even if VA is not reduced.Patients taking PPS should be monitored closely for retinal toxicity similar to patients taking
Back to the Patient: Treatment and Management:Discussed with PCP to stop the use of PPS, but educated retinal disease may continue to progressPt was seen in low vision – educated on proper lighting to manage night blindness symptoms. Pt also needed PALs/reading glasses as part of the blurry vision was due
Back to the Patient: Macular hyperpigmented spots with yellow-orange deposits and patchy RPE atrophy on fundus photography Densely packed clusters of hyper and hypoAF areas within the posterior pole on FAF Focal thickening of the RPE with hyperreflectance on NIR Peripapillary hypoAF halo Maximum size of the hyperAF spots
Proposed Disease Severity Stages5,8 Wang and colleaguesMild: Speckled pattern on FAF without well-demarcated atrophic lesions on FAF and no evidence of complete RPE and outer retinal atrophyModerate: Well-demarcated, nummular, and localized lesions with RPE and outer retinal atrophy; no central foveal involvementSevere: Well-demarcated lesions with associated hypoAF atrophy with RPE and outer retinal atrophy,
Proposed Diagnostic Criteria5,7 Barnes and colleaguesMacular hyperpigmented spots with yellow-orange deposits and patchy RPE atrophy on fundus photographyDensely packed clusters of hyper and hypoAF areas within the posterior pole on FAFFocal thickening of the RPE with hyperreflectance on NIRPeripapillary hypoAF haloMaximum size of the hyperAF spots of 2 venule widthsAbsence of
Clinical Findings in PPM and evaluation4 Fundus Examination – bilateral hyperpigmented spots with surrounding yellow subretinal deposits and mottled RPE appearance, densely-packed usually limited to posterior pole. FAF – densely packed hyper and hypo AF spots mainly in the posterior pole with peripapillary hypoAF halo around optic nerveOCT – nodular RPE abnormalities
Pentosan Polysulfate Maculopathy (PPM) Pathophysiology – toxicity of RPE, choroid and the retina’s extracellular matrix It is thought that PPS can displace the glycosylated proteins in the extracellular matrix or the interphotoreceptor matrix of the retina similar to how it affects the urothelium. Additionally, PPS similarity to heparin allows it to
