Nopasak Phasukkijwatana1,2, K Bailey Freund3,4, Rosa Dolz-Marco3,4, Mayss Al-Sheikh5,6, Pearse A Keane7, Catherine A Egan7, Sandeep Randhawa8,9, Jay M Stewart10, Qingyun Liu10,11, Alex P Hunyor12,13, Allan Kreiger1, Aaron Nagiel1, Robert Lalane1, Mansour Rahimi1, Won Ki Lee14, Lee M Jampol15, David Sarraf1,16. 1. Retinal Disorders and Ophthalmic Genetics Division, Stein Eye Institute, David Geffen School of Medicine at UCLA, Los Angeles, California. 2. Department of Ophthalmology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand. 3. Vitreous Retina Macula Consultants of New York, New York, New York. 4. Department of Ophthalmology, New York University School of Medicine, New York, New York. 5. Department of Ophthalmology, Doheny Eye Institute, David Geffen School of Medicine at UCLA, Los Angeles, California. 6. Department of Ophthalmology, University Hospital Zurich, University of Zurich, Zurich, Switzerland. 7. Medical Retina Service, Moorfields Eye Hospital NHS Foundation Trust, London, United Kingdom. 8. Associated Retinal Consultant, PC, Royal Oak, Michigan. 9. Ophthalmology, Oakland University William Beaumont School of Medicine, Royal Oak, Michigan. 10. Department of Ophthalmology, UCSF, San Francisco, California. 11. Department of Ophthalmology, Tongliao City Hospital, Tongliao, China. 12. Retina Associates, Chatswood, Australia. 13. Macular Research Unit, Save Sight Institute, University of Sydney, Sydney, Australia. 14. Ophthalmology, Seoul St. Mary's Hospital, Catholic University of Korea, Seoul, Korea. 15. Ophthalmology, Northwestern University Feinberg School of Medicine, Chicago, Illinois. 16. Greater Los Angeles, VA Healthcare Center, Los Angeles, California.
Abstract
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.
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.