Peripapillary pachychoroid syndrome

Peripapillary pachychoroid syndrome (PPS) is a recently described condition, classified within the pachychoroid disease spectrum characterized by focal or diffuse thickening of the choroid due to dilation of choroidal vessels in the Haller’s layer (pachyvessels), thinning of the choriocapillaris and the Sattler’s layer, and accompanied by increased choroidal permeability and damage to the retinal pigment epithelium. Unlike other pachychoroid diseases that involve changes in the central retina, PPS presents with choroidal thickening and intra- or subretinal fluid located nasally in the macular region, near the optic disc. This review aims to summarize and analyze current data on the clinical features, pathogenesis, and treatment options for PPS found in the literature. Differentiating PPS is crucial, as its similarity to other conditions with overlapping features may lead to misdiagnosis and ineffective or inappropriate treatment.