ACQUIRED VITELLIFORM LESIONS: A Novel Finding in Eyes with Peripapillary Pachychoroid Syndrome

To describe a novel finding of acquired vitelliform lesions in eyes with peripapillary pachychoroid syndrome.

A retrospective, multicenter, observational case series including seven patients with peripapillary pachychoroid syndrome and concomitant acquired vitelliform lesions. Medical records and multimodal imaging findings were comprehensively reviewed.

Six of seven patients were men, with a mean age of 72.6 years. Mean baseline best-corrected visual acuity was 0.11 ± 0.07 (Snellen equivalent 20/25), and all patients presented with baseline best-corrected visual acuity of 20/30 or better. Mean follow-up time was 24.4 months (range 1-54 months). At last follow-up, best-corrected visual acuity was stable in 13/14 eyes, and deteriorated in one eye because of subfoveal atrophy.Fundus examination revealed peripapillary retinal pigment epithelium alterations and pigment migration in all eyes. Fundus autofluorescence showed mottled hyperautofluorescence and hypoautofluorescence in the peripapillary region, and hypoautofluorescent lesions corresponding to the pigmentary changes. Optical coherence tomography showed nasal choroidal thickening and pachyvessels with overlying retinal pigment epithelium irregularity. All eyes presented with peripapillary intraretinal fluid. Subretinal fluid was present in five eyes of three patients. Acquired vitelliform lesions were present in 11 eyes.

The novel association between peripapillary pachychoroid syndrome and acquired vitelliform lesions is characterized by the classic findings of both entities, and visual prognosis is accordingly good.