July 2019
Volume 60, Issue 9
Open Access
ARVO Annual Meeting Abstract  |   July 2019
Topography Shows Limited Ellipsoid Zone Recovery from Mild Hydroxychloroquine Toxicity
Author Affiliations & Notes
  • Brandon Pham
    Byers Eye Institute at Stanford, Palo Alto, California, United States
  • Luis De Sisternes
    Carl Zeiss Meditec, Dublin, California, United States
  • Mary K Durbin
    Carl Zeiss Meditec, Dublin, California, United States
  • Michael F Marmor
    Byers Eye Institute at Stanford, Palo Alto, California, United States
  • Footnotes
    Commercial Relationships   Brandon Pham, None; Luis De Sisternes, Carl Zeiss Meditec (E); Mary Durbin, Carl Zeiss Meditec (E); Michael Marmor, None
  • Footnotes
    Support  Retina Research Foundation award to MFM
Investigative Ophthalmology & Visual Science July 2019, Vol.60, 4998. doi:
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      Brandon Pham, Luis De Sisternes, Mary K Durbin, Michael F Marmor; Topography Shows Limited Ellipsoid Zone Recovery from Mild Hydroxychloroquine Toxicity. Invest. Ophthalmol. Vis. Sci. 2019;60(9):4998.

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

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Purpose : While severe hydroxychloroquine (HCQ) retinopathy progresses relentlessly, milder damage stabilizes clinically, raising the possibility of limited anatomic recovery after drug cessation. Scattered anecdotal reports have indicated some improvement, but the consistency and extent has been unclear.

Methods : Our recent report on long-term progression after HCQ cessation included 5 patients with early-moderate toxicity without RPE damage that remained stable with respect to foveal thickness, ellipsoid zone (EZ) line integrity, and visual acuity for up to 9 years after HCQ cessation. To assess more critically the possibility of cellular recovery, we have used an advanced automated segmentation paradigm to define the EZ boundary (pixel by pixel) from SD-OCT volume scans and produce topographic maps of EZ integrity and loss. These images and measurements of EZ area were compared to sequential visual fields (when available), fundus autofluorescence (FAF), and SD-OCT cross-sections obtained at the time of HCQ cessation, and 2 and 7 years later.

Results : Topographic maps obtained from 4 of the 5 patients (one was excluded due to poor OCT resolution from myopia) illustrate the anatomic extent of EZ line loss at our 3 time points. One patient had minimal EZ line abnormalities, one showed a 30% decrease in the area of EZ loss over 7 years, one showed small but focal improvements, and one remained stable during the observation period. Sequential visual fields in 2 patients showed no clear changes beyond field variability. FAF hyperfluorescence rarely followed EZ changes. Matching SD-OCT cross-sections to corresponding topographic maps showed that improvement occurred irregularly in areas where the EZ line was thinned or fragmented. However, areas of complete EZ line loss or markedly thinned ONL showed no significant improvement. Other areas of damage deepened locally over time.

Conclusions : The potential for limited anatomic recovery related to the initial extent of EZ line damage at the time of drug cessation. Regions of complete EZ line absence showed essentially no improvement, while other regions of mild EZ line disruption, where the ONL was mostly intact, could be repaired (though functional improvement was likely rather minimal). Efforts to detect subtle signs of retinopathy earlier might allow for more functional recovery but may carry risk of inappropriately stopping a valuable drug for ambiguous findings.

This abstract was presented at the 2019 ARVO Annual Meeting, held in Vancouver, Canada, April 28 - May 2, 2019.


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