Purpose: To discuss the clinical findings in a unique case of acute macular neuroretinopathy with a focus on the pathophysiology of this rare entity.

Methods: The patient's clinical course was documented with color fundus photography and spectral domain ocular coherence tomography registered to infrared reflectance imaging. The visual field was assessed using the Amsler grid testing and Humphrey visual field 24-2.

Results: Initial fundus photography showed cotton wool spots and slight darkening of the central macula in each eye. Optical coherence tomography showed initial hyperreflective plaques at the level of the outer plexiform layer/outer nuclear layer junction with subsequent thinning of the outer nuclear layer and corresponding disruption of the ellipsoid and outer segment/retinal pigment epithelium. Infrared reflectance imaging revealed perifoveal hyporeflective lesions in each eye with corresponding visual field defects on the Amsler grid and visual field testing. The hyporeflective infrared lesions became more discreet during the ensuing weeks and remained stable beyond 11 weeks.

Conclusion: The authors present the case of a 15-year-old girl diagnosed with acute macular neuroretinopathy. This case is notable in that she presented with cotton wool spots and intraretinal fluid, both of which are unusual for acute macular neuroretinopathy. The authors suggest that the presence of cotton wool spots and several small foci of intraretinal fluid seen in their patient may lend support to the ischemic hypothesis described by Sarraf et al. The optical coherence tomography images obtained in this case have the typical wedge-shaped or petaloid configuration, and the authors suggest that the shape of the lesions themselves also lends support to a vascular mechanism.

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