PERIPAPILLARY PACHYCHOROID SYNDROME. Issue 9 (September 2018)

• Record Type: Journal Article
• Authors: Phasukkijwatana, Nopasak; Freund, K. Bailey; Dolz-Marco, Rosa; Al-Sheikh, Mayss; Keane, Pearse A.; Egan, CATHERINE A.; Randhawa, Sandeep; Stewart, Jay M.; Liu, Qingyun; Hunyor, Alex P.; KREIGER, Allan; Nagiel, Aaron; Lalane, Robert; Rahimi, Mansour; Lee, Won Ki; Jampol, Lee M.; Sarraf, David
• Is Part Of: Retina. Volume 38:Issue 9(2018)
• Publication Date: 2018-09
• Subjects: central serous chorioretinopathy -- choroidal folds -- choroidal thickness -- optic disk edema -- pachychoroid disease spectrum; Retina -- Diseases -- Periodicals; Retinal Diseases; Vitreous Body; 617.735
• DOI: 10.1097/IAE.0000000000001907
• Languages: English
• ISSNs: 0275-004X

Abstract

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. Abstract : 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.