Concept and characteristics of peripapillary pachychoroid syndrome

Peripapillary pachychoroid syndrome (PPS), a new entity in pachychoroid disease spectrum, is characterized by the thickening of the choroid in the peripapillary area.PPS is more likely to occur in middle-aged and older males, and some of the affected eyes are hyperopic and have short axial lengths.The diagnosis and differentiation of PPS depends mainly on the imaging characteristics.Optical coherence tomography shows that dilated Haller layer vessels are located in the nasal macula and associated with intraretinal fluid in the corresponding locations.Indocyanine green angiography shows dilated large choroidal vessels and choroidal hyperpermeability in the nasal maculal and peripapillary region.PPS should be differentiated from uveal effusion syndrome, central serous chorioretinopathy, and pachychoroid neovascularization.Photodynamic therapy may improve retinal effusion in patients with PPS.At present, the research on PPS is insufficient.It is still controversial whether PPS can be considered as a separate entity.The effectiveness of treatment needs more prospective studies.Further research is needed to provide new insights into the definition, diagnosis, treatment and pathogenesis of PPS.

Peripapillary pachychoroid syndrome; Pachychoroid disease spectrum; Review