Unilateral acute idiopathic maculopathy (UAIM) is characterized by sudden unilateral vision loss following flulike illness.1,2 Reduced vision is caused by exudative retinal detachment overlying a thickening of the retinal pigment epithelium (RPE). Spontaneous resolution of exudative changes may occur within a few weeks, with favorable visual acuity.
Little is known about the microstructural abnormalities of UAIM.3,4 We investigated the morphologic changes of photoreceptors in association with retinal function in UAIM using images obtained by spectral-domain (SD) optical coherence tomography (OCT), fundus-monitoring microperimetry, and an original prototype of an adaptive optics scanning laser ophthalmoscopy (AO-SLO) system. Our AO-SLO system allows us to identify individual cone photoreceptors.5
Figure 1. Images of the left eye of a 33-year-old woman with best-corrected visual acuity of 20/50 who had unilateral acute idiopathic maculopathy. She had a flulike illness that preceded her visual symptoms by 7 days. Images were taken 13 days after the onset of visual symptoms. A, Fundus photograph showing graying of the retina in the macula. B, Late-phase fluorescein angiography showing irregular hypofluorescence from blockage due to retinal pigment epithelium (RPE) hyperpigmentation, encircled by hyperfluorescence from surrounding atrophy of the RPE. C, Horizontal spectral-domain optical coherence tomographic scan through the foveal center, obtained in the direction of the horizontal arrow in B, showing disruption of the photoreceptor inner segment/outer segment junction (IS/OS) (between arrows) and thickening of the RPE. D, Vertical spectral-domain optical coherence tomographic scan through the foveal center, obtained in the direction of the vertical arrow in B, also showing disruption of the IS/OS (between arrows) and thickening of the RPE.
Figure 2. Images taken 2 weeks (A-E) and 3 months (F-J) after the initial visit (27 days after the onset of visual symptoms). At 3 months after the initial visit, best-corrected visual acuity was 20/15. A, Infrared image. Arrows indicate the direction of scans C and D; box, extent of the double-headed arrows in C and D. B, Fundus-monitoring microperimetry image showing a foveal and parafoveal scotoma. C, Horizontal spectral-domain optical coherence tomographic (SD-OCT) scan through the foveal center, obtained in the direction of the horizontal arrow in A, showing a decreased thickness of the retinal pigment epithelium (RPE). The line representing the inner segment/outer segment junction (IS/OS) seems partially restored but is still irregular in the fovea and parafovea (between arrows). Note that the area of IS/OS irregularity corresponds with the area where retinal sensitivity is decreased on fundus-monitoring microperimetry. D, Vertical SD-OCT scan through the foveal center, obtained in the direction of the vertical arrow in A, showing similar findings. The line representing the IS/OS seems partially restored but is irregular in the fovea and lower side of the fovea (between arrows). E, Adaptive optics scanning laser ophthalmoscopic image of the area indicated by the box in A and the double-headed arrows in C and D; many patchy dark regions are visible, surrounded by the sparse cone mosaic. F, Infrared image. Arrows indicate the direction of scans H and I; box, extent of the double-headed arrows in H and I. G, Fundus-monitoring microperimetry image showing improved retinal sensitivity, although relative scotoma is seen in the fovea. H, Horizontal SD-OCT scan through the foveal center, obtained in the direction of the horizontal arrow in F, showing a continuous IS/OS line. I, Vertical SD-OCT scan through the foveal center, obtained in the direction of the vertical arrow in F, also showing a restored IS/OS line. J, Adaptive optics scanning laser ophthalmoscopic image of the area indicated by the box in F and the double-headed arrows in H and I; the cone mosaic is remarkably restored, but small dark lesions are still visible in the fovea (arrows). Box corresponds to the area of E.
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