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. METHODS: Medical records and multimodal imaging including color fundus photography, optical coherence tomography, and fundus autofluorescence were retrospectively reviewed. Recognition of the fundus autofluorescence patterns and their relationship with the disease duration, best-corrected visual acuity, and optical coherence tomography features represented the main outcome measures. RESULTS: Seventeen eyes of 16 patients (7 women; mean age 29.9 years) with a mean follow-up of 23.9 months were included. The mean best-corrected visual acuity at presentation was 0.63 ± 0.54 logarithm of the minimum angle of resolution (Snellen equivalent, 20/85). All but one patient had the best-corrected visual acuity recovery to 20/20. Four sequential patterns of fundus autofluorescence corresponding to 4 proposed stages of disease were observed. Patterns 1 (central hypoautofluorescence with surrounding hyperautofluorescence) and 2 (stippled hyperautofluorescence and hypoautofluorescence) were found at presentation. Patterns 3 (central hyperautofluorescence surrounded by hypoautofluorescence) and 4 (hypoautofluorescence) 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 =0.003) on optical coherence tomography in comparison with Pattern 2. Pattern 4 showed unique homogeneously decreased autofluorescence with corresponding attenuation of retinal pigment epithelium and restored outer retinal layers on optical coherence tomography. CONCLUSION: A sequential disease staging based on multimodal imaging for acute idiopathic maculopathy is proposed. The recognition of the observed imaging patterns may help clinicians in the correct diagnosis and patient counseling.

ACUTE IDIOPATHIC MACULOPATHY COMPLICATED BY CHOROIDAL NEOVASCULARIZATION: NEW INSIGHTS INTO MULTIMODAL RETINAL IMAGING.

PURPOSE: To report a case of acute idiopathic maculopathy complicated by choroidal neovascularization (CNV) in which multimodal imaging was used to confirm the presence of CNV and follow the response to anti-vascular endothelial growth factor treatment. METHODS: A 39-year-old man was referred to our clinic for the evaluation of maculopathy in the right eye. Multimodal imaging was performed including fundus photography, fundus autofluorescence, infrared fundus reflectance (IR), optical coherence tomography, fluorescein angiography, indocyanine green angiography, and optical coherence tomography angiography. RESULTS: On the first examination, best-corrected visual acuity was 20/63 in the right eye and 20/20 in the left eye. A diagnosis of acute idiopathic maculopathy in the right eye was made on the basis of clinical presentation and multimodal imaging. After 3 months, best-corrected visual acuity had decreased to 20/100. Fluorescein angiography and indocyanine green angiography suggested the presence of a CNV. Optical coherence tomography angiography confirmed the presence of the CNV and well outlined the extension of the neovascular network. The patient underwent 2 monthly bevacizumab intravitreal injections. One month after the second injection, best-corrected visual acuity improved to 20/25, and optical coherence tomography angiography showed regression of the neovascular network. Best-corrected visual acuity remained stable during 20 months of follow-up. CONCLUSION: Our case reports the uncommon association of acute idiopathic maculopathy complicated by CNV. Multiimaging approach allowed to document the features observed in this disease. Optical coherence tomography angiography was particularly useful in showing the extension of the neovascular network and response to treatment.