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Observations: An 86-year-old male presented with bilateral hand motion visual acuity after 26 years of PPS use (~3769 g cumulative dose) for interstitial cystitis; imaging showed severe outer retinal disorganization, widespread retinal atrophy, extensive RPE loss, and negative exclusion of key retinal genetic diagnoses. Conclusions and importance: The case highlights dose-dependent PPS toxicity with potential irreversible vision loss, supporting periodic multimodal retinal monitoring and cumulative exposure threshold screening in long-term therapy.",{"@graph":14,"@context":73},[15,34,56],{"@type":16,"itemListElement":17},"BreadcrumbList",[18,23,27,31],{"item":19,"name":20,"@type":21,"position":22},"https://docshare.wps.com","Home","ListItem",1,{"item":24,"name":25,"@type":21,"position":26},"https://docshare.wps.com/document/","Document",2,{"item":28,"name":29,"@type":21,"position":30},"https://docshare.wps.com/document/healthcare/","Healthcare",3,{"item":32,"name":10,"@type":21,"position":33},"https://docshare.wps.com/document/severe-bilateral-retinal-degeneration-following-a-twenty-six-year-pentosan-polysulfate-sodium-exposure-a-case-report/432163/",4,{"url":32,"name":10,"@type":35,"image":36,"author":41,"headline":10,"publisher":44,"fileFormat":47,"inLanguage":8,"description":12,"dateModified":48,"datePublished":49,"encodingFormat":47,"isAccessibleForFree":50,"interactionStatistic":51},"DigitalDocument",{"url":37,"@type":38,"width":39,"height":40},"https://docshare.wps.com/thumbnails/severe-bilateral-retinal-degeneration-following-a-twenty-six-year-pentosan-polysulfate-sodium-exposure-a-case-report/432163.png","ImageObject",300,407,{"name":42,"@type":43},"Dipper","Person",{"url":19,"name":45,"@type":46},"DocShare","Organization","application/pdf","2026-11-02","2026-09-29",true,{"@type":52,"interactionType":53,"userInteractionCount":55},"InteractionCounter",{"@type":54},"ViewAction",8,{"@type":57,"mainEntity":58},"FAQPage",[59,65,69],{"name":60,"@type":61,"acceptedAnswer":62},"What is the reported clinical context of the case?","Question",{"text":63,"@type":64},"The report describes severe bilateral vision loss in an 86-year-old male with a 26-year history of pentosan polysulfate sodium (PPS) use for interstitial cystitis/bladder pain syndrome.","Answer",{"name":66,"@type":61,"acceptedAnswer":67},"What ocular findings and imaging results were observed?",{"text":68,"@type":64},"Fundus examination showed widespread retinal degeneration with near confluent nummular patches of atrophy; OCT revealed severe outer retinal disorganization, and fundus autofluorescence showed extensive RPE loss with absence of the Robson Holder ring.",{"name":70,"@type":61,"acceptedAnswer":71},"What conclusions does the case support about PPS toxicity?",{"text":72,"@type":64},"It supports the dose-dependent nature of PPS toxicity, indicating that high cumulative exposure can cause extensive retinal degeneration beyond typical macular involvement, emphasizing the need for periodic multimodal retinal evaluation and screening guidance.","https://schema.org",{"og:url":32,"og:type":75,"og:title":10,"og:site_name":45,"og:description":12},"article",{"robots":77,"canonical":32},"index,follow",{"doc_id":79,"site_id":7},432163,1791234985,{"code":4,"msg":82,"data":83},"success",[84,88,92,96,101,106,110,114,119,122,126],{"id":22,"doc_module":4,"doc_module_name":25,"category_name":85,"show_sort_weight":86,"slug":87},"Story & Novel",90,"story-novel",{"id":26,"doc_module":4,"doc_module_name":25,"category_name":89,"show_sort_weight":90,"slug":91},"Literature",80,"literature",{"id":33,"doc_module":4,"doc_module_name":25,"category_name":93,"show_sort_weight":94,"slug":95},"Exam",70,"exam",{"id":97,"doc_module":4,"doc_module_name":25,"category_name":98,"show_sort_weight":99,"slug":100},5,"Comic",60,"comic",{"id":102,"doc_module":4,"doc_module_name":25,"category_name":103,"show_sort_weight":104,"slug":105},6,"Technology",50,"technology",{"id":107,"doc_module":4,"doc_module_name":25,"category_name":29,"show_sort_weight":108,"slug":109},7,40,"healthcare",{"id":55,"doc_module":4,"doc_module_name":25,"category_name":111,"show_sort_weight":112,"slug":113},"Research & Report",30,"research-report",{"id":115,"doc_module":4,"doc_module_name":25,"category_name":116,"show_sort_weight":117,"slug":118},9,"Religion & Spirituality",20,"religion-spirituality",{"id":117,"doc_module":4,"doc_module_name":25,"category_name":120,"show_sort_weight":117,"slug":121},"World Cup","world-cup",{"id":123,"doc_module":4,"doc_module_name":25,"category_name":124,"show_sort_weight":123,"slug":125},10,"Lifestyle","lifestyle",{"id":127,"doc_module":4,"doc_module_name":25,"category_name":128,"show_sort_weight":97,"slug":129},19,"General","general",{"code":4,"msg":82,"data":131},{"doc_id":79,"user_id":132,"nickname":42,"user_avatar":133,"doc_module":4,"category_id":107,"category_name":29,"doc_title":10,"doc_description":12,"doc_content":134,"file_id":135,"file_url":136,"file_type":137,"file_size":138,"view_count":55,"is_deleted":4,"is_public":22,"is_downloadable":22,"audit_status":22,"page_count":97,"language":139,"language_code":8,"site_id":7,"html_lang":8,"table_of_contents":140,"faqs":141,"seo_title":142,"seo_description":12,"update_tm":143,"read_time":144,"preview_page_count":4},1374404997633,"https://ap-avatar.wpscdn.com/davatar_a8503ba1806abce46bf441b54a3ca4cd","American Journal of Ophthalmology Case Reports 41 (2026) 102497  \nContents lists available at ScienceDirect  \nAmerican Journal of Ophthalmology Case Reports  \njournal [homepage:](homepage: www.ajocasereports.com/)[ www.ajocasereports.com/](homepage: www.ajocasereports.com/)  \n| Severe bilateral retinal degeneration following a twenty-six-year pentosan polysulfate sodium exposure: a case report\u003Cbr>Aaron T. Zhao a , Turner D. Wibbelsman b, Martin Calottib, Collin J. Richards b, Tamara R. Vrabecc,* \u003Cbr>a Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA b Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA, USAc Geisinger Eye Institute, Geisinger Medical Center, Danville, PA, USA |  |  |\n| --- | --- | --- |\n| A R T I C L E I N F O |  | A B S T R A C T |\n| Keywords:\u003Cbr>Pentosan polysulfate sodium PPS\u003Cbr>Retinopathy Interstitial cystitis Retinal degeneration |  | Purpose: To describe an advanced case of pentosan polysulfate sodium (PPS)-associated retinopathy with extensive macular and peripheral retinal involvement following prolonged high-dose exposure.\u003Cbr>Observations: An 86-year-old male presented with bilateral hand motion visual acuity and a 26-year history of PPS use (400mg daily, ~3769g cumulative dose) for treatment of interstitial cystitis. Fundus examination revealed widespread retinal degeneration characterized by confluent nummular patches of retinal atrophy in a radial distribution from the optic nerves. Ocular coherence tomography (OCT) demonstrated severe outer retinal disorganization, and fundus autofluorescence (FAF) showed extensive RPE loss and absence of the Robson Holder ring. Genetic testing excluded choroideremia and late-onset retinal dystrophy, identifying only variants of uncertain significance in candidate genes DNAJC17 and SPP2. The patient had a history of excessive coffee consumption, which may have exacerbated his interstitial cystitis and potentially contributed to retinal vascular compromise.\u003Cbr>Conclusions and importance: This case supports the dose-dependent nature of PPS toxicity and demonstrates that high cumulative doses can lead to widespread retinal degeneration beyond the typical macular involvement. The potential severity of irreversible vision loss highlights the importance of periodic retinal evaluation using multimodal imaging to facilitate early recognition of PPS toxicity in patients on long-term PPS therapy and supports ongoing efforts to establish widely accepted, comprehensive screening guidelines and cumulative dose thresholds for patients on long-term PPS therapy, similar to protocols for other potentially toxic drug-related retinopathies. |\n\n1. Introduction  \nPentosan polysulfate sodium (PPS) is commonly prescribed for the treatment of interstitial cystitis (IC)/bladder pain syndrome.1 An association has been described between prolonged PPS use and the development of progressive maculopathy, characterized by bilateral retinal pigment epithelium (RPE) atrophy and hyperpigmented macular spots, referred to as PPS-associated maculopathy.2,3 A dose-dependent relationship has been recognized, with greater lifetime cumulative exposure increasing risk of vision impairment.4 Here, we report the clinical findings and multimodal imaging features of severe macular and peripheral retinal deterioration in a patient with exceptionally high cumulative PPS exposure during prolonged therapy for refractory IC  \nsymptoms.  \n2. Clinical course  \nAn 86-year-old white male was referred for evaluation of severe, bilateral vision loss. Ocular history was significant for “dry macular degeneration” with visual acuity of 20/30 OU in Year 1. During the subsequent 11 years, records from another provider revealed visual acuity worsened to 20/200 OU. He was subsequently lost to follow up until Year 16. The patient denied a family history of any ocular conditions including vision loss and retinitis pigmentosa.  \nMedical history was significant for atrial fibrillation, ","cbCaitGNH3mSyTYJ","https://ap.wps.com/l/cbCaitGNH3mSyTYJ","pdf",2282989,"English","# Introduction\n# Clinical course","[{\"question\":\"What is the reported clinical context of the case?\",\"answer\":\"The report describes severe bilateral vision loss in an 86-year-old male with a 26-year history of pentosan polysulfate sodium (PPS) use for interstitial cystitis/bladder pain syndrome.\"},{\"question\":\"What ocular findings and imaging results were observed?\",\"answer\":\"Fundus examination showed widespread retinal degeneration with near confluent nummular patches of atrophy; OCT revealed severe outer retinal disorganization, and fundus autofluorescence showed extensive RPE loss with absence of the Robson Holder ring.\"},{\"question\":\"What conclusions does the case support about PPS toxicity?\",\"answer\":\"It supports the dose-dependent nature of PPS toxicity, indicating that high cumulative exposure can cause extensive retinal degeneration beyond typical macular involvement, emphasizing the need for periodic multimodal retinal evaluation and screening guidance.\"}]","Severe bilateral retinal degeneration following a twenty-six-year pentosan polysulfate sodium exposure - a case report | PDF",1790658337,13]