Unilateral acute idiopathic maculopathy (UAIM) is a rare disorder presenting in young people with an acute onset of unilateral central visual loss often associated with a prodromal flu-like illness. The authors present the early anatomical findings of a 35-year-old man clinically diagnosed with UAIM using swept-source optical coherence tomography (SS-OCT) and SS-OCT angiography.
[Ophthalmic Surg Lasers Imaging Retina. 2016;47:180–182.]
Unilateral acute idiopathic maculopathy (UAIM) is a rare disorder presenting in young people with an acute onset of unilateral central visual loss1 often associated with a prodromal flu-like illness. Coxsackievirus2 infection is considered to be the cause of UAIM. The disease is characterized by a spontaneous recovery during the course of several weeks.3 Previous spectral-domain optical coherence tomography (SD-OCT) studies have reported abnormal heterogeneous hyperreflective thickening at the level of the outer retina and retinal pigment epithelium (RPE) in the foveal region during the acute phase and hyporeflective exudation and subretinal fluid with detachment and disruption of photoreceptor outer segment.4 However, swept-source OCT (SS-OCT) and SS-OCT angiography (SS-OCTA) (DRI OCT Triton; Topcon, Tokyo) features of the outer retina, RPE, and choriocapillaris have not been reported in UAIM so far.
A 35-year-old man clinically diagnosed with UAIM after a 7-day duration of severe flu-like symptoms with sores in the mouth and rash on the hands and feet underwent a complete ophthalmologic examination 2 days after the onset of blurred vision in his left eye. Serology was positive for immunoglobulin M against coxsackievirus. Fundus biomicroscopy revealed a yellowish lesion with an orange/dark core in the left macula (Figure). Fluorescein angiography revealed early hypofluorescence with an intense late hyperfluorescence (Figure). Indocyanine green angiography revealed a hypofluorescent lesion with some hyperfluorescence in the mid-late phase (Figure). Right eye revealed a juxtafoveal RPE defect. SS-OCT performed at presentation showed neurosensory detachment of the retina, with thickening of the outer retina due to disruption and irregularity of the photoreceptor outer segment (Figure). SS-OCTA with a 3 mm × 3 mm field of view passing through the macula showed a preserved superficial and deep retinal capillary plexus. At the level of the choriocapillaris segmentation line, there was a dark pattern compared to the right eye and to an age-matched healthy eye (Figure). After 4 days, there was partial resolution of the neurosensory detachment with a better delineation of the outer retina layers (Figure). SS-OCTA still showed a dark pattern (Figure). At 1 month there was a complete resolution of the neurosensory detachment, with gradual improvement of the outer retinal layers but a focal submacular scar, which caused a slight metamorphopsia (Figure). At this point visual acuity was 10/10. On SS-OCTA, the dark pattern still persisted, although it was slightly decreased (Figure).
Photographs of the left eye of a patient with unilateral acute idiopathic maculopathy (UAIM) at the initial visit (A–E, H), after 4 days (F, I) and at 1 month (G, L). (A) Fundus photograph showing a yellowish lesion with an orange/dark core in the left macula. (B, C) Fluorescein angiography showing central hypofluorescence with surrounding faint, ring-shaped hyperfluorescence in the initial phase (B) and marked leakages and pooling of the dye within the subretinal space in the late phase (C). (D) Indocyanine green angiography showing a hypofluorescent lesion with some hyperfluorescence in the mid-late phase. (E) Swept-source optical coherence tomography (SS-OCT) at the initial visit (E) showing neurosensory detachment of the retina with thickening of the outer retina due to disruption and irregularity of the photoreceptor outer segment. At 4 days (F), imaging showed partial resolution of the neurosensory detachment with a better delineation of the outer retina layers. (C) At 1 month (G), showed a complete resolution of the neurosensory detachment with gradual improvement of the outer retinal layers and a juxtafoveal focal retinal pigment epithelial detachment containing hyporeflective material. SS-OCT angiography photographs taken at the level of the choriocapillaris segmentation line in the study (H, I, L), fellow (M), and control healthy (N) eyes. (H, I) Photographs showing the dark pattern and the progressive reduction compared to the normal “white noise” effect of the fellow (M) and control healthy (N) eye.
Choroidal involvement during UAIM has been recently proposed.5,6 In 4 eyes with UAIM, Hashimoto et al. found increased choroidal thickness and reduced choroidal blood flow in the acute phase and a progressive decrease of choroidal thickness and increase in choroidal blood flow during follow-up. The authors concluded that patients with UAIM can have a choroidal circulation impairment due to the disease, the mechanism of which remains largely unknown. Although in our case choroidal thickness was normal, the dark pattern at the level of the choriocapillaris might confirm that a choroidal circulation impairment does develop during UAIM. OCTA is usually unable to detect the blood flow at choriocapillaris level, and the pattern is similar to the “white noise” of the analog television. SS-OCTA uses a light source centered at 1,050 nm, which, when compared with the 840-nm wavelength SD-OCT, enables deeper light penetration into the RPE and choroid. As the segmentation lines move toward the choroid, the signal becomes more evident because the blood flow is higher and choroidal vessels appear dark instead of white, probably due to the barrier of the RPE. In our case, one possible explanation for the dark pattern at the choriocapillaris level on SS-OCTA may be a transitory increase in blood flow velocity at the level of the choriocapillaris. The dark pattern of the choriocapillaris, as highlighted on SS-OCTA, together with the photoreceptor outer-segment layer elevation and disruption, confirm that a hyperperfusion of the choriocapillaris might be involved primarily in the disease process.
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