Imaging in the Diagnosis and Management of Acute Idiopathic Maculopathy

Abstract

Acute idiopathic maculopathy (AIM), originally termed unilateral AIM, is a rare inflammatory disorder that typically affects young adults (mean age, 32 y; range, 15 to 45 y) and is characterized by acute, severe, unilateral vision loss preceded by a flu-like illness. Men and women are affected equally. Patients generally present with visual acuity of r20/200 and complain of central metamorphopsia and/or scotoma formation that is attributable to an exudative macular detachment. There may also be hypopigmentation of the retinal pigment epithelium, which may simulate the appearance of choroidal neovascularization (CNV) (Fig. 1). 1 Papillitis, a few vitreous cells, subretinal exudation, and bilaterality have also been observed in AIM, whereas eccentric macular lesions have been noted in cases with milder vision loss (20/60 to 20/100). 2 Most cases resolve spontaneously with near-complete recovery of vision (20/25 or better) over a period of 3 weeks to 6 months. After resolution of the macular detachment, a ''bull's-eye'' pattern composed of retinal pigment epithelial (RPE) hyperpigmentation surrounded by hypopigmented atrophic changes develops at the site of the antecedent neurosensory detachment (Fig. 2). To date, there has been only 1 reported case with recurrent episodes of AIM. 3 Rarely, secondary CNV has occurred after resolution of AIM. The authors have seen a case of secondary CNV after AIM that responded well to intravitreal anti-vascular endothelial growth factor therapy.The flu-like illness that precedes AIM suggests a viral cause. The coxsackie virus has been implicated in the pathogenesis of AIM,

Atypical findings in unilateral Acute Idiopathic Maculopathy: a case report

“…In conclusion, a unilateral acute idiopathic maculopathy case associated with more severe features than previously reported, including cystoid edema of the macula, flame shaped hemorrhages in the retina, and subretinal exudation, was reported. Depicting the unusual and rare features associated with a condition is necessary to help avoid misdiagnosis and unnecessary treatment as has been previously reported in cases of acute idiopathic maculopathy [7].…”

Correspondence

“…In our recent literature review, we have found that BALAD was commonly seen during the early stages of acute idiopathic maculopathy and additional cases have been reported since then. 2,4–14 I wonder whether the 4 cases that displayed serous retinal detachment at presentation also had BALAD on multimodal imaging.…”