Peripapillary pachychoroid neovasculopathy: A novel entity

Purpose: Peripapillary pachychoroid syndrome is a new variant of pachychoroid disease recently described. It is important to establish the features and pathogenic mechanisms of this novel entity and its possible association with type 1 neovascularization. The aim of this study is to report a case of peripapillary pachychoroid neovasculopathy, a novel entity of pachychoroid spectrum disease. Case report: A 51-year-old woman was referred to our macula service due to macular pigmentary changes in her right eye. On examination, best corrected visual acuity was 20/20 Snellen equivalent in both eyes. Funduscopy revealed pigmentary changes in the papillonasal bundle in her right eye with mottled autofluorescence in the same area where pigmentary changes. Spectral-domain optical coherence tomography illustrated a shallow irregular pigment epithelium detachment (PED) which appears as “double layer sign” overlying pachyvessels, with hyper-flow signal beneath it and a large network of neovascularization revealed by cross-sectional optical coherence tomography angiography. Indocyanine green angiography confirmed the pachyvessels and choroidal hyper-permeability in the peripapillary region. A diagnosis of peripapillary pachychoroid neovasculopathy was made and good visual and anatomical outcomes were achieved with a treat-and-extend regimen with intravitreal aflibercept. Conclusion: We report a novel entity within the pachychoroid spectrum disease, the peripapillary pachychoroid neovasculopathy, which could improve our understanding of the pathogenic mechanism of choroidal neovascularization in pachychoroid disorders.