Figure 1. This ultra-widefield fundus photograph of the right eye shows a well-demarcated hypopigmented region at the macula, with RPE mottling at the fovea.
Figure 2. A fundus autofluorescence image of the right eye shows hypoautofluorescence surrounding central hyperautofluorescence.
Figure 3. This fluorescein angiography of the right eye shows stippled staining at the fovea.
Figure 4. Spectral-domain optical coherence tomography of the right eye demonstrates attenuation of the central ellipsoid zone.
Figure 5. An OCT-A image shows the right eye with a central hypopigmented region surrounded by hyperpigmentation at the macula.
A 37-year-old woman presented for evaluation of a central gray spot in her vision and soreness in her right eye for the prior 6 days. She denied diplopia, metamorphopsia, photophobia or flashes of light. Her medical history was significant for possible hand-foot-and-mouth disease contracted 1.5 weeks earlier, and a diagnosis of breast cancer, with a negative mammography at the latest visit. Her medical history was negative for ocular disease or medication use.
On examination, her Snellen visual acuity was 20/300, and 20/20 in the right and left eyes respectively. Intraocular pressures were 23 mm Hg, and 21 mm Hg in the right and left eye respectively. Anterior segment examination was normal, and pupils were equally reactive to light. Ocular movements and visual fields by finger confrontation were normal. A dilated fundus exam of the right eye showed a 1.5 DD, round, well-demarcated, hypopigmented region at the macula, with RPE mottling at the fovea, and absence of subretinal fluid/heme (Figure 1). Left fundus exam was unremarkable.
Fundus autoflourescence (FAF) exhibited hypoautofluoresence surrounding a region of hyperautofluorescence at the macula (Figure 2). Fluorescein angiography (FA) showed stippled staining at the fovea in the right eye, with normal vascular filling patterns in both eyes (Figure 3). Optical coherence tomography (OCT) of the right eye demonstrated attenuation of the ellipsoid zone centrally, with no loss of foveal contour (Figure 4).
At the second visit, her visual acuity had improved to 20/70, and IOP was 21 mm Hg in her right eye. OCT demonstrated an improvement in outer retinal haze and infrared hyperreflectivity.
By the third visit, visual acuity had stabilized to 20/70 and IOP was 16 mm Hg in her right eye. The OCT showed some atrophy of the photoreceptor layer, but with an overall improvement.
Structural enface angiograms of the retina were acquired using widefield swept-source OCT angiography (OCT-A) which showed a central hypopigmented region surrounded by hyperpigmentation at the macula in the right eye (Figure 5). Lab results were negative for Lyme IgG, FTA, RPR, Coxsackie IgG, HLA-B27, ACE, lysozyme and Quantiferon gold. ANCA, ANA and CRP were also negative.
Unilateral acute idiopathic maculopathy (UAIM) was first described as a syndrome in 1991, but its mechanism has not been clearly elucidated.1 The condition is characterized by inflammatory changes in the outer retina and retinal pigment epithelium (RPE), as seen in...
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Unilateral acute idiopathic maculopathy (UAIM) was first described as a syndrome in 1991, but its mechanism has not been clearly elucidated.1 The condition is characterized by inflammatory changes in the outer retina and retinal pigment epithelium (RPE), as seen in our case. It has commonly been linked to Coxsackie-virus infection, which causes hand-foot-and-mouth disease, or follows a flu-like illness.2-4
This condition is usually unilateral, involving the macula. The characteristic macular appearance may show a transient neurosensory detachment, or hypopigmentation at the macula, with pigmentary disturbances of the RPE.4 It generally affects young, healthy individuals and patients typically report a loss of central vision, which recovers spontaneously in a few weeks.3,4 These clinical findings are substantiated in our case.
UAIM is a clinical diagnosis, based on fundoscopy, and supplemented by imaging. Spectral domain OCT demonstrates a disruption of the photoreceptor inner segment/outer segment junction (ellipsoid zone).3,4 FA predominantly shows hyperfluorescence due to staining, which is believed to arise from the RPE layer. Etiology may be determined by performing serum titers of Coxsackie virus antibody, which have been found to be elevated in several reports.2 In our case, Coxsackie labs were negative.
UAIM is a self-resolving condition; steroids may be used for severe or nonresolving cases.4,5 The lesion may leave behind a bull’s-eye macular scar, but vision closely correlates improvement in OCT, and usually returns to normal.2 In our case, vision initially improved in the first week, after which it stabilized. However, OCT continues to show gradual improvement. There was no sign of a choroidal neovascular membrane (CNVM).
This case was submitted by Dr Arora, an ophthalmologist from Sri Ramachandra Institute of Higher Education and Research, Chennai, India, and Dr Hoyek, a postdoctoral research fellow in ophthalmology at Massachusetts Eye and Ear and Harvard Medical School, Boston. It was clinically edited by Dr Patel, an assistant professor of ophthalmology at Massachusetts Eye and Ear and Harvard Medical School and director of Pediatric Retina at Boston Children’s Hospital, Boston.
1. Yannuzzi LA. Unilateral acute idiopathic maculopathy. Arch Ophthalmol. 1991;109(10):1411. doi:10.1001/archopht.1991.01080100091049
2. Beck AP, Jampol LM, Glasser DA, Pollack JS. Is Coxsackievirus the cause of unilateral acute idiopathic maculopathy? Arch Ophthalmol. 2004;122(1):121-123. doi:10.1001/archopht.122.1.121
3. Hughes EH, Hunyor AP, Gorbatov M, Ho I van. Acute idiopathic maculopathy with coxsackievirus infection. Retin Cases Brief Rep. 2012;6(1):19-21. doi:10.1097/ICB.0b013e3181f7f7ee
4. Reich M, Cakir B, Cvetkoski S, et al. Acute unilateral maculopathy associated with adult onset of hand, foot and mouth disease: a case report. BMC Ophthalmology. 2019;19(1):104. doi:10.1186/s12886-019-1111-4
5. Agrawal R, Bhan K, Balaggan K, Lee RW, Pavesio CE, Addison PK. Unilateral acute maculopathy associated with adult onset hand, foot and mouth disease: case report and review of literature. J Ophthalmic Inflamm Infect. 2015;5(1):2. doi:10.1186/s12348-015-0034-3