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Cecilia S. Jung, John F. Payne, Chris Bergstrom, Blaine Cribbs, Jiong Yan, G. B. Hubbard, III, Timothy W. Olsen, Steven Yeh; Multimodality Diagnostic Imaging in Unilateral Acute Idiopathic Maculopathy. Invest. Ophthalmol. Vis. Sci. 2011;52(14):2192.
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© ARVO (1962-2015); The Authors (2016-present)
Unilateral acute idiopathic maculopathy (UAIM) is a rare cause of acute, painless visual loss in young healthy patients, often following a flulike illness. This study describes UAIM's clinical features and multimodality diagnostic imaging characteristics.
Six patients diagnosed with UAIM at our institution between 2008 and 2010 were included. Clinical characteristics, funduscopic findings and imaging features were reviewed.
Four male and two female patients, with the median age of 30 years, were identified. All presented with unilateral, sudden vision loss, and the median interval between symptom onset and presentation was two weeks. Associated systemic findings included viral prodrome (50%), orchitis (33%), hand-foot-mouth disease (16%), and positive Coxsackievirus titer (33%). Five patients (83%) presented in late summer. The median presenting visual acuity was 20/240 (Range 20/50 to 2/200), which improved to 20/60 (range 20/25 to 20/400) at last follow-up. Mean follow-up was 4 weeks.Early in the disease course, patients exhibited mild retinal edema and subretinal fluid on time-domain optical coherence tomography (OCT). Fluorescein angiography demonstrated irregular early hyperfluorescence and late leakage. As the disease progressed, patients developed irregular, stippled hyperpigmentation of parafoveal retina and retinal pigment epithelium. Spectral-domain OCT showed irregularity of the inner segment-outer segment photoreceptor layer and choroidal thickening. Fundus autofluorescence (FAF) revealed granular hyper- and hypoautofluorescence of the central macula. FAF findings became more diffuse and less discrete over time. Angiography with indocyanine green showed a "moth-eaten" appearance of the choroidal vasculature, which was suggestive of choroidal inflammation.
This study highlights the unique imaging characteristics in UAIM. These findings suggest that pathologic processes of UAIM appear to involve the inner choroid, RPE and photoreceptors, some of which are reversible.
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