Shanil Dhanji1,2, Thomas Groeneveld1,2, Hira Saud2, Maria Korsakova2, Nonna Saakova2, Haipha Ali2, Clare Fraser1,2, Robyn Jamieson2, John Grigg1,2
Purpose: This study aims at determining whether fundus autoﬂuorescence (FAF) patterns correlate with Best disease staging according to Gass’s classi- ﬁcation (pre-vitelliform, vitelliform, pseudohypop- yon, vitelliruptive and atrophic). It also assesses how FAF compares with Gass’s classiﬁcation, opti- cal coherence tomography (OCT), electroretinogra- phy (ERG) and electrooculography (EOG) in predicting age of onset and visual acuity.
Method: We performed a retrospective review of patients, from 2009 to 2017 at the Save Sight Insti- tute, who were diagnosed with Best disease based on characteristic bilateral macular lesions and a nor- mal full ﬁeld ERG.
Results: 28 eyes with Best disease were examined, mean age 30 16 years. FAF patterns included hyper- (n = 10), ring- (n = 9), patchy- (n = 5), hypo- FAF (n = 2) and normal (n = 2). The normal FAF pattern occurred in 33% of patients with pre- vitelliform lesions (n = 2). Hyper-, patchy- and ring- FAF patterns occurred in a combination of pre-vitel- liform, vitelliform, pseudohypopyon and vitellirup- tive stages. Hypo-FAF existed only in atrophic macular lesions (n = 2).
A younger age of onset and better visual acuity was associated with a hyper-FAF pattern (20.64 11.02 years and 0.9 0.2), greater macular thickness on OCT and increased pERG 15deg (20deg) P50 and N95
amplitudes (P-value < 0.05). There was no correlation with the Arden ratio. Conclusion: FAF patterns correlated with Gass’s classiﬁcation for early and late stages with normal and hypo-FAF occurring exclusively in pre- vitelliform and atrophic lesions respectively. In other stages a hyper-FAF pattern was associated with a better visual acuity. FAF adds a further struc- tural outcome measure in monitoring Best disease.