Bilateral asynchronous acute idiopathic maculopathy with disease recurrence

Abstract

Purpose: Acute idiopathic maculopathy (AIM) is a posterior segment disorder that involves the neurosensory retina, retinal pigment epithelium, and choroidal circulation. When first described, the disease was classified as unilateral, but subsequent reports have found bilateral presentations. There is growing evidence to suggest the coxsackie virus as the etiology, however, other pro-inflammatory conditions have also been temporally linked to fundoscopic findings. We report on a young male without viral prodrome and without known contact with viral illnesses who experienced bilateral recurrent acute idiopathic maculopathy. Observations: A 35-year-old male presented with retinal whitening within the macula of the right eye. Multimodal imaging demonstrated a bacillary layer detachment, late leakage, and disruption of the choriocapillaris. Laboratory workup for inflammatory and infectious etiologies was negative. A diagnosis of AIM was made. The findings resolved with a steroid course. Nine months after initial presentation, the patient developed similar symptoms, fundoscopic, and imaging findings in the left eye, which again resolved with a steroid course. Three years after initial presentation, he developed symptoms in the right eye. Fundoscopy and imaging demonstrated a larger affected area compared to the prior episode. At his last follow up, the visual acuity, fundoscopy, and imaging findings had returned to baseline with no residual acuity deficit. Conclusions and importance: The patient presented has recurrent, bilateral AIM. Bilateral presentations and recurrent presentations have previously been reported, but this case is unique as it meets both criteria. Based on multimodal imaging throughout each episode, we hypothesize that AIM may be on the white dot disease spectrum.

Keywords

• White dot syndrome
• Spectrum
• APMPPE
• Relentless placoid
• Acute idiopathic maculopathy