July 2019
Volume 60, Issue 9
Open Access
ARVO Annual Meeting Abstract  |   July 2019
Multimodal Adaptive Optics Imaging of the Cellular Status and Progression of the Outer Retinal Complex in Bietti Crystalline Dystrophy
Author Affiliations & Notes
  • Johnny Tam
    National Eye Institute, National Institutes of Health, Bethesda, Maryland, United States
  • Laryssa Huryn
    National Eye Institute, National Institutes of Health, Bethesda, Maryland, United States
  • Zhuolin Liu
    U.S. Food and Drug Administration, Center for Devices and Radiological Health, Silver Spring, Maryland, United States
  • Jianfei Liu
    National Eye Institute, National Institutes of Health, Bethesda, Maryland, United States
  • HaeWon Jung
    National Eye Institute, National Institutes of Health, Bethesda, Maryland, United States
  • Nancy Aguilera
    National Eye Institute, National Institutes of Health, Bethesda, Maryland, United States
  • Daniel Hammer
    U.S. Food and Drug Administration, Center for Devices and Radiological Health, Silver Spring, Maryland, United States
  • Wadih M Zein
    National Eye Institute, National Institutes of Health, Bethesda, Maryland, United States
  • Tao Liu
    National Eye Institute, National Institutes of Health, Bethesda, Maryland, United States
  • Footnotes
    Commercial Relationships   Johnny Tam, None; Laryssa Huryn, None; Zhuolin Liu, None; Jianfei Liu, None; HaeWon Jung, None; Nancy Aguilera, None; Daniel Hammer, None; Wadih Zein, None; Tao Liu, None
  • Footnotes
    Support  Intramural Research Program of the National Eye Institute, National Institutes of Health; Alcon Research Institute
Investigative Ophthalmology & Visual Science July 2019, Vol.60, 6098. doi:https://doi.org/
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      Johnny Tam, Laryssa Huryn, Zhuolin Liu, Jianfei Liu, HaeWon Jung, Nancy Aguilera, Daniel Hammer, Wadih M Zein, Tao Liu; Multimodal Adaptive Optics Imaging of the Cellular Status and Progression of the Outer Retinal Complex in Bietti Crystalline Dystrophy. Invest. Ophthalmol. Vis. Sci. 2019;60(9):6098. doi: https://doi.org/.

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

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Abstract

Purpose : To evaluate and longitudinally follow the cellular status of the outer retinal complex (photoreceptor, retinal pigment epithelial cells (RPE), and choriocapillaris (CC)) in Bietti Crystalline Dystrophy (BCD) using multimodal adaptive optics (AO) imaging.

Methods : Four eyes from two unrelated patients (P1: 52, P2: 43 years old) with molecularly-confirmed mutations in the CYP4V2 gene and clinical phenotypes consistent with BCD were imaged using high resolution imaging including color fundus photography, spectral-domain (SD) optical coherence tomography (OCT), and a custom-built multimodal AO imager incorporating AO-SDOCT and indocyanine green (ICG) imaging capabilities (Comm Biol 1:189,2018) to reveal the cone photoreceptors, RPE cells, and CC.

Results : Discrete crystals found in non-atrophic macular areas of the retina were mostly localized in the outer retina near the RPE layer (SDOCT and AO-SDOCT). Near the fovea, contiguous mosaics of intact cone photoreceptors were observed above enlarged RPE and intact CC, including above crystals. In patient P1 (advanced BCD), quantitative morphological measurements of cones, RPE, and CC were significantly different from normal (p<0.05) with the greatest degree of change observed in the RPE and CC. Intact CC was observed with an abrupt loss at the edge of atrophy. In patient P2 (early BCD), cone spacing and CC flow voids were within or near normal limits, but RPE cells were significantly enlarged (Table 1). Follow-up imaging performed 7 months afterwards revealed a highly dynamic process of disappearance of old crystals and formation of new crystals with a dramatic redistribution of RPE cell fluorescence that mirrored the redistribution of crystals. Interestingly, RPE cells associated with crystals were largely hypofluorescent on late phase AO-ICG imaging. Despite the rapid reconfiguration of crystals and RPE, there was no difference in cone spacing attributed to the loss of a crystal (p=0.7, paired two-tailed t-test, n=10 pairs of regions containing a single crystal in visit 1 with no crystal in visit 2). Longitudinal assessment of the CC showed relative preservation of flow voids despite the overlying changes in the RPE.

Conclusions : Our data suggests that the RPE (and crystals associated with RPE) undergoes dramatic remodeling early in BCD, with photoreceptor and CC degeneration occurring as secondary events.

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

 

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