Multimodality Diagnostic Imaging in Unilateral Acute Idiopathic Maculopathy

PURPOSE: To report multimodality diagnostic imaging in a case of unilateral acute idiopathic maculopathy. CASE SUMMARY: A 32-year-old woman with reduced vision in the right eye had experienced fatigue and flu-like symptoms, including sore throat and fever a few days before. Her best corrected visual acuity (BCVA) was 1.0 in the right eye. There were no cells in the anterior chamber and vitreous. Fundus photographs of the right eye on presentation showed gray-white thickening of the fovea and retinal hemorrhage next to the fovea. Fluorescein angiography demonstrated ring-shaped mottled hyperfluorescence in the early phase and dye pooling in the late phase. Spectral-domain optical coherence tomography (OCT) showed abnormal hyper-reflective thickening at the level of the outer retina and retinal pigment epithelium (RPE) and detachment of the neurosensory retina in the foveal lesion. The inner segment/outer segment junction and photoreceptor elevation/disruption was noted. Nineteen months after onset, the BCVA of the right eye was 1.0 and fundus photographs showed increased retinal pigment hyperplasia, and residual RPE changes resembling a bull's eye maculopathy. The OCT of the right macula showed that the inner segment/outer segment junction elevation/disruption almost completely regressed. The patient was diagnosed with unilateral acute idiopathic maculopathy. CONCLUSIONS: We report a typical case with flu-like symptoms of unilateral acute idiopathic maculopathy. It is a very rare macular disease and a case such as ours with long-term follow-up using multimodality diagnostic imaging has not been previously reported.

Unilateral Acute Idiopathic Maculopathy: A Case of Atypical Presentation

Unilateral acute idiopathic maculopathy(UAIM)is a rare, self-limiting disorder that affects healthy young adults who experience sudden, severe visual loss. It is characterized by an exudative maculopathy with irregular margins and yellowish-white thickening at the level of the retinal pigment epithelium(RPE). The fluorescein angiographic findings characteristically demonstrate early irregular hyperfluorescence and hypofluorescence at the level of RPE lesion, followed by complete staining of the overlying neurosensory retinal detachment in the late phase. A characteristic bulls-eye pattern pigmentary changes may occur in the macula after resolution of the acute phase of the disease. We report a patient with atypical presentation of UAIM in which multifocal leaks at the RPE level on the fluorescein angiography was observed.