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Authors: Nopasak Phasukkijwatana
K. Bailey Freund
Rosa Dolz-Marco
Mayss Al-Sheikh
Pearse A. Keane
Catherine A. Egan
Sandeep Randhawa
Jay M. Stewart
Qingyun Liu
Alex P. Hunyor
Allan Kreiger
Aaron Nagiel
Robert Lalane
Mansour Rahimi
Won Ki Lee
Lee M. Jampol
David Sarraf
NYU School of Medicine
University of California, San Francisco
UniversitatsSpital Zurich
Moorfields Eye Hospital NHS Foundation Trust
Vitreous Retina Macula Consultants of New York
Save Sight Institute
Northwestern University Feinberg School of Medicine
Oakland University
Faculty of Medicine, Siriraj Hospital, Mahidol University
David Geffen School of Medicine at UCLA
The Catholic University of Korea
Alzheimer's Greater Los Angeles
Tongliao City Hospital
Retina Associates
Keywords: Medicine
Issue Date: 1-Sep-2018
Citation: Retina (Philadelphia, Pa.). Vol.38, No.9 (2018), 1652-1667
Abstract: 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.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.
ISSN: 15392864
Appears in Collections:Scopus 2018

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