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Phasukkijwatana, Nopasak, PhD, MD*,†; Freund, K. Bailey, MD‡,§; Dolz-Marco, Rosa, MD, PhD‡,§; Al-Sheikh, Mayss, MD¶,**; Keane, Pearse A., MD††; Egan, CATHERINE A., MD††; Randhawa, Sandeep, MD‡‡,§§; Stewart, Jay M., MD¶¶; Liu, Qingyun, MD¶¶,***; Hunyor, Alex P., MD†††,‡‡‡; KREIGER, Allan, MD*; Nagiel, Aaron, MD, PhD*; Lalane, Robert, MD*; Rahimi, Mansour, MD*; Lee, Won Ki, MD§§§; Jampol, Lee M., MD¶¶¶; Sarraf, David, MD*,****

doi: 10.1097/IAE.0000000000001907
Original Study

Purpose: To describe the features of peripapillary pachychoroid syndrome (PPS), a novel pachychoroid disease spectrum (PDS) entity.

Methods: Medical records of 31 eyes (16 patients) with choroidal thickening associated with intraretinal and/or subretinal fluid in the nasal macula extending from the disk were reviewed (patients with PPS). Choroidal thickness was compared with 2 age-matched cohorts: typical PDS (17 eyes with central serous chorioretinopathy or pachychoroid neovasculopathy) and 19 normal eyes.

Results: The patients with PPS were 81% men aged 71 ± 7 years. Peripapillary pachychoroid syndrome eyes displayed thicker nasal versus temporal macular choroids, unlike PDS eyes with thicker temporal macular choroids (P < 0.0001). Peripapillary intraretinal and/or subretinal fluid was often overlying dilated Haller layer vessels (pachyvessels). Fundus autofluorescence and fluorescein angiography illustrated peripapillary pigmentary mottling without focal leakage. Most PPS eyes (70%) exhibited other PDS findings including serous pigment epithelial detachment or gravitational tracks. Indocyanine green angiography illustrated dilated peripapillary pachyvessels and choroidal hyperpermeability. The disk was usually crowded, with edema noted in 4/31 (13%) eyes and mild late fluorescein disk leakage identified in half of the cases. Choroidal folds (77%), short axial lengths (39% less than 23 mm), and hyperopia (86%) were common.

Conclusion: Peripapillary pachychoroid syndrome is a distinct PDS variant, in which peripapillary choroidal thickening is associated with nasal macular intraretinal and/or subretinal fluid and occasional disk edema. Recognition of PPS is important to distinguish it from disorders with overlapping features such as posterior uveitis and neuro-ophthalmologic conditions.

Peripapillary pachychoroid syndrome is a novel pachychoroid disease spectrum variant, in which peripapillary choroidal thickening is associated with intraretinal and/or subretinal fluid. Optic nerve head leakage or edema occurs in some eyes. Peripapillary choroidal congestion exhibiting a compartment syndrome–like effect is proposed as an etiologic mechanism for the peripapillary fluid.

*Retinal Disorders and Ophthalmic Genetics Division, Stein Eye Institute, David Geffen School of Medicine at UCLA, Los Angeles, California;

Department of Ophthalmology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand;

Vitreous Retina Macula Consultants of New York, New York, New York;

§Department of Ophthalmology, New York University School of Medicine, New York, New York;

Department of Ophthalmology, Doheny Eye Institute, David Geffen School of Medicine at UCLA, Los Angeles, California;

**Department of Ophthalmology, University Hospital Zurich, University of Zurich, Zurich, Switzerland;

††Medical Retina Service, Moorfields Eye Hospital NHS Foundation Trust, London, United Kingdom;

‡‡Associated Retinal Consultant, PC, Royal Oak, Michigan;

§§Ophthalmology, Oakland University William Beaumont School of Medicine, Royal Oak, Michigan;

¶¶Department of Ophthalmology, UCSF, San Francisco, California;

***Department of Ophthalmology, Tongliao City Hospital, Tongliao, China;

†††Retina Associates, Chatswood, Australia;

‡‡‡Macular Research Unit, Save Sight Institute, University of Sydney, Sydney, Australia;

§§§Ophthalmology, Seoul St. Mary's Hospital, Catholic University of Korea, Seoul, Korea;

¶¶¶Ophthalmology, Northwestern University Feinberg School of Medicine, Chicago, Illinois; and

****Greater Los Angeles, VA Healthcare Center, Los Angeles, California.

Reprint requests: David Sarraf, MD, Retinal Disorders and Ophthalmic Genetics Division, Stein Eye Institute, David Geffen School of Medicine at UCLA, Los Angeles, CA 90095; e-mail:

Supported by The Macula Foundation, Inc, New York, NY.

K. B. Freund is a consultant for Optovue (Fremont, California), Optos (Dunfermline, Scotland), Heidelberg Engineering (Heidelberg, Germany), Genentech (South San Francisco, California), and GrayBug Vision (Redwood City, California) and receives research support from Genentech/Roche (Basel, Switzerland). P. A. Keane is an advisory board member for Heidelberg, Allergan (Dublin, Republic of Ireland), Bayer Healthcare (Leverkusen, Germany), Topcon (Tokyo, Japan), Haag-Streit (Köniz, Switzerland), and Novartis (Basel, Switzerland), a consultant for Google DeepMind (London, UK) and Optos, and receives Clinician Scientist award (CS-2014-14-023) from the National Institute for Health Research. The views expressed in this publication are those of the author(s) and not necessarily those of the NHS, the National Institute for Health Research or the Department of Health. A. P. Hunyor is an advisory board member for Bayer Healthcare, Novartis and Alcon (Fort Worth, Texas). A. Nagiel is a consultant for Allergan. W. K. Lee is a consultant for Bayer Healthcare, Novartis, and Santen Pharmaceutical (Osaka, Japan) and receives research support from Allergan. D. Sarraf is a consultant for Amgen, Bayer Healthcare, Genentech, Novartis, and Optovue and receives research or financial support from Allergan, Genentech, Heidelberg, Optovue, and Regeneron (Tarrytown, New York). The remaining authors have no conflicting interests to disclose.

© 2018 by Ophthalmic Communications Society, Inc.