Fundus Autofluorescence Patterns in Best Vitelliform Macular Dystrophy

PURPOSE: To provide a systematic classification of fundus autofluorescence (FAF) patterns in patients affected by Best vitelliform macular dystrophy. DESIGN: Cross-sectional prospective study. METHODS: Patients affected by Best vitelliform macular dystrophy at different stages of the disease were prospectively enrolled from January 2012 to July 2013. Eighty eyes of 40 patients were included in the study. All patients underwent a complete ophthalmologic examination, including genetic characterization, short-wavelength FAF, and near-infrared FAF. Main outcome measures were the recognition of the FAF patterns in the different stages and the identification of a relationship between FAF patterns and best-corrected visual acuity (BCVA). RESULTS: Six FAF patterns for both short-wavelength and near-infrared FAF were identified, including normal, hyper-autofluorescent, hypo-autofluorescent, patchy, multifocal, and spoke-like patterns. Applying Gass's classification for defining consecutive stages of Best vitelliform macular dystrophy (namely vitelliform, pseudohypopyon, vitelliruptive, atrophic, and cicatricial) identified no pattern as stage-specific. Patchy patterns had the highest prevalence. A statistically significant difference (Kruskat-Wallis ANOVA) was. found among hyperautofluorescent, patchy, and hypo-autofluorescent patterns, both in short-wavelength (P =. 001) and near-infrared FAF (P = .001). Hyper-autofluorescent and hypo-autofluorescent patterns were associated with better and worse BCVA, respectively. CONCLUSIONS: Six main patterns on both short-wavelength and near-infrared FAF were identified in Best vitelliform macular dystrophy. No FAF pattern can be considered stage-specific. Although a difference in the BCVA among the FAF patterns was registered, only a longitudinal study designed to evaluate the clinical and FAF modifications over the follow-up will help clarify the prognostic implications of each FAF pattern. (C) 2014 by Elsevier Inc. All rights reserved.