Background/Aims To analyse the long-term anatomic and visual outcomes of patients with peripapillary pachychoroid syndrome (PPS), a recently described entity in the pachychoroid disease spectrum.
Methods This study retrospectively included patients from several retina centres worldwide. Visual acuity (VA), retinal thickness and choroidal thickness at baseline, 6 months and final follow-up were assessed. Temporal trends in VA and anatomic characteristics were evaluated. Visual and anatomic outcomes in eyes that were observed versus those that were treated were analysed.
Results Fifty-six eyes of 35 patients were included with mean follow-up of 27±17 months. Median VA was 20/36 at baseline and remained stable through follow-up (p=0.77). Retinal thickness significantly decreased subfoveally (p=0.012), 1.5 mm nasal to the fovea (p=0.002) and 3.0 mm nasal to the fovea (p=0.0035) corresponding to areas of increased thickening at baseline. Choroidal thickness significantly decreased subfoveally (p=0.0030) and 1.5 mm nasal to the fovea (p=0.0030). Forty-three eyes were treated with modalities including antivascular endothelial growth factor injection, photodynamic therapy, and others. VA remained stable in treated eyes over follow-up (p=0.67). An isolated peripapillary fluid pocket in the outer nuclear layer was characteristic of PPS.
Conclusion Patients with PPS experienced decreased retinal oedema and decreased choroidal thickening throughout the course of disease. While some patients experienced visual decline, the overall visual outcome was relatively favourable and independent of trends in retinal or choroidal thickening.
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Contributors The research design was created by DX, EG, NP, WKL, and DS. All members participated in data collection, data analysis was performed by DX, EG, and DS. All members participated in drafting and critical revisions of the manuscript.
Funding SS: Heidelberg Engineering, Carl Zeiss Meditec, Optos, Centervue, Novartis, Bayer, Boeheringer, Allergan, Alcon, Topcon, Nidek. KBF and DS: Macula Foundation Inc, New York NY.
Competing interests NP: speaker for Allergan, Bayer, Novartis and Optovue. SL: consultant for Novartis, personal fees and non-financial support from Heidelberg Engineering and Carl Zeiss Meditec, personal fees from Novartis, Allergan and Bayer. WKL: consultant for Bayer Healthcare, Novartis, and Santen Pharmaceutical. SS: consultant for Heidelberg Engineering, Zeiss Meditec, Optovue, Optos, Centervue, Nidek, Novartis, Bayer. KBF: consultant for Zeiss, Optovue, Novartis, Allergan, and Heidelberg Engineering. DS: Consultant for Amgen, Bayer, Genentech, Heidelberg, Novartis, Optovue, Topcon, Regeneron.
Patient and public involvement statement Not commissioned; externally peer reviewed.
Patient consent for publication Not required.
Ethics approval The study was approved by the Institutional Review Board (IRB) of the University of California Los Angeles and adhered to the tenets of the Declaration of Helsinki. IRB approval was obtained by the coauthors at their respective institutions. The study complied with the Health Insurance Portability and Accountability Act.
Data availability statement Data are available upon reasonable request.