June 2020
Volume 61, Issue 7
ARVO Annual Meeting Abstract  |   June 2020
Pentosan Polysulfate Maculopathy: Comprehensive Functional Analysis and Structure-Function Correlation
Author Affiliations & Notes
  • Riley Lyons
    Emory University School of Medicine, Atlanta, Georgia, United States
  • Adam Hanif
    Department of Internal Medicine, Virginia Mason Medical Center, Seattle, Washington, United States
  • Nieraj Jain
    Department of Ophthalmology, Emory University School of Medicine, Atlanta, Georgia, United States
  • Footnotes
    Commercial Relationships   Riley Lyons, None; Adam Hanif, None; Nieraj Jain, Foundation Fighting Blindness Career Development Award CD-C-0918-0748-EEC (NJ) (F)
  • Footnotes
    Support  None
Investigative Ophthalmology & Visual Science June 2020, Vol.61, 1068. doi:
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      Riley Lyons, Adam Hanif, Nieraj Jain; Pentosan Polysulfate Maculopathy: Comprehensive Functional Analysis and Structure-Function Correlation. Invest. Ophthalmol. Vis. Sci. 2020;61(7):1068.

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      © ARVO (1962-2015); The Authors (2016-present)

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Purpose : Individuals with pentosan polysulfate (PPS) maculopathy commonly report symptoms of prolonged dark adaptation and difficulty reading. We hypothesize that PPS maculopathy results in degradation of visual function that is not adequately captured with visual acuity testing. We performed a prospective study to comprehensively evaluate the functional impact of PPS maculopathy and then correlated functional results with retinal structure.

Methods : Fourteen patients (28 eyes) with PPS maculopathy prospectively underwent multimodal evaluation of retinal structure and function. Structural changes were graded as either moderate or severe based on fundus autofluorescence imaging findings. Subjective visual function was assessed with the National Eye Institute Visual Function Questionnaire 25 (NEI VFQ-25) and Low Luminance Questionnaire (LLQ). Objective evaluations included best corrected visual acuity (BCVA), Pelli-Robson contrast sensitivity, dark adaptometry (MacuLogix), and microperimetry (CenterVue). Results of functional testing were correlated with structural disease category using a Mann-Whitney U Test.

Results : Functional evaluation of the 28 affected eyes demonstrated noteworthy functional deficits despite a median logMAR BCVA of 0.10 (Snellen equivalent, 20/25; range, -0.1 - 1.5). Results were markedly abnormal when compared to previously reported normative data (Table 1). Median NEI VFQ-25 and LLQ composite scores were 65 (range, 33 - 88) and 40 (range, 20 - 92), respectively. Median contrast sensitivity was 1.65 (range, 0.15 - 1.95), and median microperimetry average thresholds and percent reduced thresholds were 26 (range, 0.4 - 28.6) and 22 (range, 0 - 100), respectively.
Eyes with severe structural abnormalities were associated with significantly worse function as compared to those with moderate structural abnormalities (Table 1). Microperimetry average threshold and percent reduced thresholds for severe disease were markedly worse than those in prior studies of nonexduative AMD.

Conclusions : PPS maculopathy causes considerable degradation in visual function that is not fully captured with BCVA testing. There was good correlation between other subjective and objective measures of visual function and structural abnormalities. These findings deepen our concern regarding this major patient safety issue and support the implementation of screening programs to promote early detection.

This is a 2020 ARVO Annual Meeting abstract.



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