PENTOSAN POLYSULFATE MACULOPATHY–ASSOCIATED CYSTOID MACULAR EDEMA RESPONSIVE TO INTRAVITREAL STEROIDS

Abstract

Purpose: To report a case of angiographically silent cystoid macular edema (CME) secondary to pentosan polysulfate sodium (PPS) maculopathy responsive to intravitreal steroids. Methods: Observational case report. Results: A 52-year-old female patient with a history of 4 years of PPS use for interstitial cystitis presented with PPS maculopathy that developed CME 2.5 years after drug cessation and had associated progression of pigmentary and atrophic changes. Her CME was nonresponsive to topical ketorolac and dorzolamide, but was responsive to intravitreal triamcinolone acetonide and subsequently intravitreal dexamethasone implant (Ozurdex) with reduction in central subfield thickness and improvement in visual acuity. Conclusion: Cystoid macular edema secondary to PPS maculopathy may be angiographically silent yet responsive to intravitreal steroids alone without the use of vascular endothelial growth factor agents. There is potential for both anatomic and functional improvements in such cases demonstrating the value of such treatment. Cystoid macular edema may be a delayed finding that can develop despite drug cessation. Steroid monotherapy should be further evaluated as possible first-line management for PPS maculopathy-associated CME.