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Choriocapillaris flow loss in center-involving retinitis pigmentosa: a quantitative optical coherence tomography angiography study using a novel classification system

  • Retinal Disorders
  • Published:
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Abstract

Purpose

Choriocapillaris insufficiency may play a role in centripetal retinitis pigmentosa (RP) progression involving the fovea. However, the relationship between choriocapillaris integrity and foveal damage in RP is unclear. We examined the relationship between choriocapillaris flow and the presence of foveal photoreceptor involvement in RP.

Methods

We categorized the severity of central involvement in RP by the occurrence of foveal ellipsoid zone (EZ) disruption: present (severe RP) or absent (mild RP). Using optical coherence tomography angiography (OCTA, AngioVue, Optovue) in cases and unaffected age-matched controls, we compared vessel density (VD) between the groups using the generalized linear mixed model, controlling for age, gender, and scan quality.

Results

Fifty-seven eyes (20 severe RP, 18 mild RP, and 19 controls) were included. Foveal and parafoveal mean outer retinal thickness (µm) were lower in severe RP (fovea: 101.3 ± 14.5; parafovea: 68.4 ± 11.7) than controls (fovea: 161.2 ± 8.9; parafovea: 142.1 ± 11.8; p ≤ 0.001) and mild RP (fovea: 162.0 ± 14.7; parafovea: 116.8 ± 29.4; p ≤ 0.0001). Foveal choriocapillaris VD (%) was lower in severe RP (56.7 ± 6.8) than controls (69.9 ± 4.6; p = 0.008) and mild RP (65.3 ± 5.3; p = 0.01). The parafoveal choriocapillaris VD was lower in severe RP than controls (64.4 ± 5.9 vs. 68.3 ± 4.1; p = 0.04) but no different than in mild RP (p = 0.4).

Conclusion

Choriocapillaris flow loss was associated with fovea-involving photoreceptor damage in RP. Further research is warranted to validate this putative association and clarify causation. Choriocapillaris imaging using OCTA may provide information to supplement structural OCT findings when evaluating subjects with RP in neuroprotective or regenerative clinical trials.

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Funding

This study was funded by a Foundation Fighting Blindness Career Development Award CD-RM-0918–0749-JHU (MSS), the Hartwell Foundation, the Shulsky Foundation, the Joseph Albert Hekimian Fund, Research to Prevent Blindness (unrestricted grant to the Wilmer Eye Institute), and the Wilmer Biostatistics Core Grant EY01765. The sponsors or funding organizations had no role in the design or conduct of this research.

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Authors and Affiliations

  1. Wilmer Eye Institute, Johns Hopkins Hospital, Johns Hopkins University School of Medicine, 600 N Wolfe St, Baltimore, MD, 21287, USA

    Sally S. Ong, T. Y. Alvin Liu & Mandeep S. Singh

  2. Department of Ophthalmology, Wake Forest School of Medicine, Medical Center Blvd, Winston-Salem, NC, 27157, USA

    Sally S. Ong

  3. Department of Biostatistics, Wilmer Biostatistics Center, Johns Hopkins Bloomberg School of Public Health, Baltimore, MD, USA

    **min Li

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  1. Sally S. Ong
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Correspondence to Mandeep S. Singh.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of Johns Hopkins University and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. This article does not contain any studies with animals performed by any of the authors.

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Ong, S.S., Liu, T.Y.A., Li, X. et al. Choriocapillaris flow loss in center-involving retinitis pigmentosa: a quantitative optical coherence tomography angiography study using a novel classification system. Graefes Arch Clin Exp Ophthalmol 259, 3235–3242 (2021). https://doi.org/10.1007/s00417-021-05223-y

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