Peripapillary pachychoroid syndrome: Clinical and imaging features, diagnostic differentiation and therapeutic...

Peripapillary pachychoroid syndrome (PPS) is a recently recognized member of the pachychoroid disease spectrum characterized by peripapillary choroidal thickening, pachyvessels, intra- and subretinal fluid and associated serous pigment epithelial detachment. We summarize current knowledge on its clinical presentation, multimodal imaging features, differential diagnosis, and available treatment strategies, with emphasis on reported outcomes. PPS predominantly affects older, hyperopic men with short axial lengths and may coexist with other pachychoroid entities, focal choroidal excavation, chorioretinal folds, or secondary choroidal neovascularization (CNV). Multimodal imaging, particularly enhanced-depth imaging optical coherence tomography and indocyanine green angiography, is essential for distinguishing PPS from optic disc pit maculopathy, tractional macular schisis, and inflammatory or neovascular disorders. Anti-vascular endothelial growth factor therapy has shown limited benefit in the absence of CNV, whereas photodynamic therapy appears to achieve anatomical improvement in a substantial proportion of persistent or refractory cases, albeit with variable functional gains. PPS exhibits a variable natural history, and treatment outcomes remain heterogeneous. Well-designed prospective studies are required to refine therapeutic algorithms and identify imaging biomarkers predictive of response, enabling more tailored management of this emerging chorioretinal disorder.