Ultra-wide-field fundus autofluorescence in multiple evanescent white dot syndrome

Purpose: To observe the progression of affected lesions using ultra-wide-field fundus autofluorescence (FAF) in multiple evanescent white dot syndrome.

Design: Retrospective, observational case series.

Methods: setting: Institutional.

Patient population: 14 eyes of 13 patients (mean age, 35.8 years) with acute disease unilaterally.

Observation procedures: Patients underwent ultra-wide-field FAF, spectral-domain optical coherence tomography (SD OCT), multifocal electroretinography (mfERG), and Goldmann or automated perimetry; the best-corrected visual acuity (BCVA) and refractive error were measured.

Main outcome measure: Ability of ultra-wide-field FAF to detect lesions with greater sensitivity compared with color fundus photography.

Results: Ultra-wide-field FAF imaging enabled improved visualization of the affected lesions and showed that the core lesion was in the posterior fundus involving the peripapillary retina and posterior pole and surrounded by hyper-autofluorescent spots outside the vascular arcade. The posterior lesions expanded rapidly and peripheral spots spread farther peripherally and reached a maximal extent during the acute stage. During follow-up, the peripheral hyper-autofluorescent spots resolved and then hyper-autofluorescence of the posterior fundus gradually faded. SD OCT showed diffuse disruption of the photoreceptor inner segment/outer segment junction (IS/OS) in the posterior fundus during the acute stage. The correlation between the IS/OS abnormality and hyper-autofluorescent areas was unclear. The disrupted IS/OS was restored with normalization of the FAF.

Conclusions: Ultra-wide-field FAF showed that the lesions arise from the peripapillary retina and the posterior pole and spread peripherally in a centrifugal manner during the acute stage. The hyper-autofluorescent spots faded from the periphery in a centripetal manner.