History of present illness
A 59-year-old woman presented with a 4-year history of decreased vision and progressive nyctalopia. Past medical history was remarkable for depression, chronic fatigue, fibromyalgia and interstitial cystitis (IC).
Ocular examination findings
Snellen visual acuities were 20/20 in the right eye (OD) and 20/30 in the left eye (OS). Intraocular pressures were normal. External and anterior segment examinations were within normal limits in both eyes (OU). Dilated fundus examination showed retinal pigment epithelium (RPE) mottling and RPE clumps in the perifoveal region OU.
Imaging
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Ultrawide-field (UWF) fundus photography (California, Optos) demonstrated pericentral RPE mottling OU with a normal peripheral retina OU ( Fig. 56.1 A, B).
Fig. 56.1
Ultrawide-field (UWF) color (A and B) and autofluorescent (C and D) imaging of a 59-year-old patient with interstitial cystitis and pentosan polysulfate exposure (cumulative dosage 1606 g). Color UWF (A and B) demonstrates retinal pigment epithelial alterations in the macula in both eyes (OU). UWF autofluorescence (C and D) shows a speckled pattern of hyper- and hypoautofluorescence centered around the fovea with normal peripheral retina OU.
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Spectralis multicolor photography (Heidelberg Engineering, Inc.) and near-infrared reflectance (NIR) showed punctate perifoveal hyperreflective lesions OU ( Fig. 56.2 A–D).
Fig. 56.2
Spectralis multicolor photography (A and B), near-infrared reflectance (NIR) (C and D), and fundus autofluorescence (E and F) for the right and left eyes in the same patient with pentosan-associated maculopathy. Multicolor and NIR images show a symmetrical pattern of hyperreflective lesions centered around the fovea in each eye. Fundus autofluorescence (30-degree) shows a speckled network of hyper- and hypoautofluorescent lesions centered on the fovea in both eyes. Focal areas of hypoautofluorescent retinal pigment epithelial atrophy are also noted in each eye.
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Fundus autofluorescence (FAF) 55 degrees ( Fig. 56.3 ) and UWF FAF ( Fig. 56.1 C, D) illustrated a speckled network of hyperautofluorescent and hypoautofluorescent lesions centered around the fovea OU. These lesions corresponded to focal areas of RPE thickening evident on cross-sectional spectral domain optical coherence tomography (OCT) and en face OCT OU.
Fig. 56.3
Fundus autofluorescence images (55-degree) show a speckled network of hyper- and hypoautofluorescent lesions centered on the fovea in both eyes.
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OCT angiography excluded macular neovascularization ( Fig. 56.4 ).
Fig. 56.4
En face (3 x 3 mm) optical coherence tomography (OCT) angiography (A and B), centered on the fovea, and corresponding en face OCT (C and D) and OCT B-scans (E–H) from the right and left eyes. Note the absence of macular neovascularization with en face OCT angiography (outer retina slab) in each eye (A and B). En face OCT (C and D), segmented at the retinal pigment epithelium, and cross-sectional OCT B-scans (E–H) show the characteristic hyperreflective RPE lesions associated with pentosan polysulfate sodium maculopathy that are differentiated from macular drusen.
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