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Woman presents with gradually progressive decrease in vision
Over the course of 3 years, the patient’s vision, especially the central acuity, worsened.
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A 51-year-old phakic woman presented for complaints of decreased vision in both eyes, gradually progressing over the last 3 years. She especially noted decreased central acuity, complaining specifically of seeing blind spots centrally. She had no prior ocular surgery in either eye and no contributory family history of ocular disease. Medical history was significant for systemic lupus erythematosus.
Andrew A. Moshfeghi
Visual acuity measured 20/30-1 in the right eye and 20/20-1 in the left eye. IOP was 16 mm Hg in the right eye and 15 mm Hg in the left eye. Mild corticonuclear cataracts were noted on slit-lamp examination of the anterior segment, which was otherwise normal. Fundus examination (Figure 1a, top) demonstrated retinal pigment epithelium (RPE) mottling in the parafoveal region encircling the fovea, crystalline appearance of the temporal macula, and right-angled venules in the temporal macula bilaterally. No subretinal hemorrhage was appreciated. Fundus autofluorescence images (Figure 1a, bottom) demonstrated subtle areas of hypoautofluorescence in the parafoveal region encircling the fovea. Spectral-domain optical coherence tomography images showed focal areas of loss of the ellipsoid zone, the photoreceptors and RPE (temporally worse than nasally) in both eyes (Figure 1b, top). A 10-2 automated visual field test with white target and reliable indices (Figure 1b, bottom) revealed subtle paracentral defects in the left eye but was essentially full in the right eye.
In 2008, the patient had been followed for a known diagnosis of idiopathic juxtafoveal retinal telangiectasia (Gass-Blodi type 2) in both eyes. At that time, visual acuity measured 20/20 in each eye. Color fundus photos (Figure 2, top) demonstrated a crystalline appearance temporal to the fovea in both eyes as well as the presence of right-angled venules in this same location. Fluorescein angiogram (Figure 2, bottom) demonstrated leakage in the temporal macula in both eyes without evidence of choroidal neovascularization. In addition, the patient had been treated with hydroxychloroquine 200 mg twice daily for lupus since 2007. She was initially observed without signs of choroidal neovascularization or hydroxychloroquine toxicity and was subsequently followed by her local ophthalmologist for the next 4 years.
Figure 1a. Top: Fundus exam demonstrated RPE mottling in the parafoveal region encircling the fovea, crystalline appearance of the temporal macula, and right-angled venules in the temporal macula bilaterally. Bottom: Fundus autofluorescence images demonstrated subtle areas of hypoautofluorescence in the parafoveal region encircling the fovea.
Images: Moshfeghi AA
Figure 1b. Top: SD-OCT revealed focal areas of retinal and RPE atrophy temporally and nasally, creating a ‘flying saucer sign’ in the ellipsoid region centrally. Bottom: A 10-2 automated visual field test with white target and reliable indices revealed subtle paracentral defects in the left eye but was essentially full in the right eye.
Figure 2. Top: Color fundus photos 4 years before presentation demonstrated a crystalline appearance temporal to the fovea in both eyes as well as the presence of right-angled venules in this same location. Bottom: Fluorescein angiogram 4 years before presentation demonstrated leakage in the temporal macula in both eyes without evidence of choroidal neovascularization.
Discussion
This patient received 1,022 cumulative grams of hydroxychloroquine over 7 years of therapy. Both the cumulative dose and duration of exposure to hydroxychloroquine put this patient at a relatively high risk of macular toxicity. In addition to the suggestive clinical observations of an early bull’s-eye appearance to the macula clinically and on autofluorescence images, the SD-OCT scans demonstrated the early beginnings of the so-called “flying saucer sign” for hydroxychloroquine toxicity. Perimetric findings in this case, however, were subtle and nonspecific.
This patient represented a bit of a clinical challenge insofar as she had macular telangiectasia as a known ocular comorbidity. Therefore, determining which new findings were due to the macular telangiectasia and which were due to possible hydroxychloroquine toxicity was not a straightforward task, especially because some of the findings can be present in both conditions. A bull’s-eye macula and the development of a “flying saucer” sign on SD-OCT are not typically seen in macular telangiectasia. It should be acknowledged that the ultrastructural damage observed on SD-OCT was primarily localized to the temporal macula, which was the area predominantly involved by the macular telangiectasia, although more subtle changes were also observed centrally and nasally. Despite the possibility that the constellation of these findings may have been due to the macular telangiectasia alone, I opted to have the patient, in coordination with her rheumatologist, discontinue the hydroxychloroquine immediately.