An unusual presentation of peripapillary pachychoroid syndrome

Peripapillary pachychoroid syndrome (PPS) is a recently described entity of the pachychoroid disease spectrum and is characterized by thickening of the nasal choroid and peripapillary fluid pockets. This case illustrates the remarkable natural history of this recently described disorder.

This case report describes a patient with PPS who presented with severe cystoid macular edema (CME) that spontaneously resolved without treatment.

This report indicates that PPS can exhibit significant fluctuation of CME and a favorable natural history in some cases. It also suggests that observation may be a viable option in the initial management of this finding if vision is not significantly unaffected.