OSLI Retina

January 2021

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January 2021 · Vol. 52, No. 1 13 ■ C L I N I C A L S C I E N C E ■ New Insights Into Pentosan Polysulfate Maculopathy Michelle M. Abou-Jaoude, MD; Alexander M. Davis, MS; Claire E. Fraser, MD, PhD; Monique Leys, MD; David Hinkle, MD; J. Vernon Odom, PhD; Ramiro S. Maldonado, MD BACKGROUND AND OBJECTIVE: To provide new in- sights into toxic maculopathy secondary to pento- san polysulfate (PPS) utilizing multimodal testing. PATIENTS AND METHODS: Retrospective case-series of four patients from two academic centers evalu- ated with multimodal imaging, electrophysiology, dark adaptometry (DA), and genetic testing. RESULTS: Median age was 58 years, exposure to PPS was 18.5 years, and cumulative dose of was 2,025 grams. Seven of eight eyes had visual acuity of 20/40 or better. Optical coherence tomography (OCT) an- giography demonstrated increased choriocapillaris flow voids (54.25%) in cases compared to controls (13.2%). Two subjects had abnormal foveal avascu- lar zone configurations. Two subjects demonstrated collapse of the retinal pigment epithelium nodular excrescences and progressive retinal thinning over 4 to 5 years on OCT. Electrophysiology was normal (3/3 patients), but DA was delayed (2/2 patients). CONCLUSIONS: The authors describe novel findings of PPS maculopathy, including flow voids in the choriocapillaris. Progressive retinal thinning may suggest a secondary retinal effect. These findings may improve understanding of the pathophysiology. [Ophthalmic Surg Lasers Imaging Retina. 2021;52:13-22.] INTRODUCTION Macular toxicity secondary to pentosan polysul- fate sodium (PPS) (Elmiron; Janssen Pharmaceuticals, Beerse, Belgium) was first reported in 2018. 1 PPS is the only U.S. Food and Drug Administration (FDA)- approved oral treatment for interstitial cystitis (IC), a painful urological condition 2 affecting approximately 45 in 100,000 women and eight in 100,000 men glob- ally. 3 Pearce et al. initially described six patients with PPS exposure (approximately 400 mg/day, average of 15 years) presenting with pigmentary maculopathies characterized by paracentral hyperpigmentation and nodular excrescences on optical coherence tomogra- phy (OCT) at the level of the retinal pigment epithe- lium (RPE) with negative genetic testing. 1 Subsequent articles investigated the strength of the association, 4 described the phenotypic range of presentation, 5,6 and generated screening recommen- dations. 7 Diagnostic criteria described include: 1) bi- lateral pathology centered on the fovea, 2) paracen- tral macular hyperpigmented spots, 3) dense array of hyper- and hypoautofluorescent spots and reticular fundus autofluorescence (FAF) abnormalities, and 4) foci of nodular RPE enlargement on OCT correspond- ing to hyperreflectance on near-infrared reflectance imaging. Humphrey visual fields (HVF) ranged from normal to dense paracentral to central scotomas cor- relating with the degree of atrophy 5 and analysis of electroretinography (ERG) revealed normal to diffuse- ly dampened amplitudes. 1,4-6 One study described findings on OCT angiography (OCTA) in a case with a choroidal neovascular mem- From University of Kentucky College of Medicine, Department of Ophthalmology and Visual Sciences, Lexington, Kentucky (MMAJ, AMD, CEF, RSM); and West Virginia University Eye Institute, Department of Ophthalmology, Morgantown, West Virginia (ML, DH, JVO). © 2021 Abou-Jaoude, Davis, Fraser, et al.; licensee SLACK Incorporated. This is an Open Access article distributed under the terms of the Creative Com- mons Attribution-NonCommercial 4.0 International (https://creativecommons.org/licenses/by-nc/4.0). This license allows users to copy and distribute, to remix, transform, and build upon the article non-commercially, provided the author is attributed and the new work is non-commercial. Originally submitted June 29, 2020. Revision received September 19, 2020. Accepted for publication November 9, 2020. Preliminary data presented at ISGEDR Ophthalmic Genetics Club Annual Meeting, October 2019, San Francisco, California. Support for genetic testing from Spark Therapeutics and "My RetinaTracker" from the Foundation for Fighting Blindness. Neither source had any involve- ment in study design, collection, analysis, interpretation of data, manuscript writing, or the decision to submit for publication. The authors report no relevant financial disclosures. Address correspondence to Ramiro S. Maldonado, MD, University of Kentucky, Department of Ophthalmology, 110 Conn Terrace, Suite 550, Lexington, KY 40508; email: Ramiro.maldonado@uky.edu. doi: 10.3928/23258160-20201223-04

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