Abstract
Purpose
To describe a unique case of unilateral benign yellow dot maculopathy.
Observations
A 25-year-man was evaluated after incidental finding of yellow dots in the right macula. The findings of examination and multimodal imaging were in keeping with a diagnosis of benign yellow dot maculopathy.
Conclusions and importance
Benign yellow dot maculopathy is a recently described entity with either a sporadic or dominant inheritance pattern. This is the first known report of the characteristic findings of this phenotype presenting unilaterally.
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Introduction
Yellow macular dots have an extensive differential diagnosis including drusen, inherited maculopathies, and fleck retinopathy. Inherited macular dystrophies are a group of heterogenous disorders that lead to macular changes and vision loss. Fleck retinopathy encompasses a large number of diseases with characteristic white-yellow retinal flecks. These two groups of disorders are typically associated with vision changes and possible systemic abnormalities.
Recently, a novel macular phenotype termed ‘benign yellow dot maculopathy’ has been described by Dev Borman et al. To date, reports of this phenotype describe non-progressive bilateral macular yellow dots with normal visual acuity and color vision. Herein, we present the first known case of unilateral benign yellow dot maculopathy.
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Case presentation
A 25-year-old Caucasian male of Dutch and mixed European ancestry was referred for assessment of white-yellow dots in the macula of his right eye (OD). He was asymptomatic. His past ocular history was significant for trauma to the left eye (OS) from a paintball approximately 10 years prior. This injury led to angle recession in the left eye, but no glaucoma. The patient was otherwise healthy and not taking any medications. There was no travel history or exposure to animals. The patient denied smoking and recreational drug use. There was no history of macular degeneration in the patient’s family.
On presentation, best corrected visual acuity was 20/20 bilaterally and intraocular pressures were normal. Pupils were equal and reactive to light, and no afferent pupillary defect was present. Farnsworth D15 color vision testing was normal. Examination of the anterior segment was unremarkable OD and revealed a small traumatic cataract OS. In both eyes, there was no evidence of cell or flare in the anterior chamber and the vitreous was clear with no cell or haze. Optic nerves were normal and symmetric.
Retinal examination revealed intraretinal diffuse fine yellow dots in the posterior pole OD, predominantly located circumferentially around the fovea and also extending into the temporal macula ( Fig. 1 A). The mid and far periphery of the retina were normal with no evidence of the yellow dots. The retinal vessels appeared normal. Examination of the left retina was normal, with no yellow dots observed ( Fig. 1 B).
The scattered yellow dots that were appreciated clinically were hyperautofluorescent on fundus autofluorescence imaging ( Fig. 2 ). Subtle abnormalities were noted on spectral-domain optical coherence tomography (OCT) of the macula OD at the level of the retinal pigment epithelium (RPE) (Heidelberg Engineering, Dossenheim, Germany) ( Fig. 3 A and B). The yellow dots appeared hyper-reflective on near-infrared imaging ( Fig. 3 C). There were no abnormalities appreciated on OCT OS. Macular OCT thickness maps appeared normal in both eyes, with central subfield foveal average thickness measuring 285 μm OD and 273 μm OS. Goldmann kinetic perimetry showed no abnormalities in either eye. Electrophysiologic tests were also performed. The pattern visual evoked potentials were in the normal range with no significant interocular difference. Multifocal electroretinogram (ERG) revealed a slight decrease in peak signal intensity OD, although foveal activity with implicit times were in the normal range in both eyes ( Fig. 4 ).
