Acute Idiopathic Maculopathy: A Proposed Disease Staging Based on Multimodal Imaging.

PURPOSE To describe the clinical course and the multimodal imaging of acute idiopathic maculopathy (AIM). METHODS Medical records and multimodal imaging including color fundus photography, optical coherence tomography (OCT) and fundus autofluorescence (FAF) were retrospectively reviewed. Recognition of the FAF patterns and their relationship with the disease duration, best-corrected visual acuity (BCVA) and OCT features represented the main outcome measures. RESULTS Seventeen eyes of 16 patients (7 females; mean age 29.9 years) with a mean follow-up of 23.9 months were included. Mean BCVA at presentation was 0.63±0.54 logarithm of the minimum angle of resolution (Snellen equivalent, 20/85). All but one patient had BCVA recovery to 20/20.Four sequential patterns of FAF corresponding to four proposed stages of disease were observed. Patterns 1 (central hypo-autofluorescence with surrounding hyper-autofluorescence) and 2 (stippled hyper- and hypo-autofluorescence) were found at presentation. Patterns 3 (central hyper-autofluorescence surrounded by hypo-autofluorescence) and 4 (hypo-autofluorescence) were observed during the disease course and/or at the last follow-up visit. Duration of the disease was significantly different between patterns at baseline and last visit. Pattern 1 significantly related to the presence of subretinal detachment (Fisher's Exact Test; P =.003) on OCT in comparison to pattern 2. Pattern 4 showed a unique homogeneously decreased autofluorescence with correspondent attenuation of RPE and restored outer retinal layers on OCT. CONCLUSIONS A sequential disease staging based on multimodal imaging for AIM is proposed. The recognition of the observed imaging patterns may help clinicians in the correct diagnosis and patient counselling.