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Jayson D. Edwards, Sandeep Grover; Spectral-Domain Optical Coherence Tomography and Retinal Function in Pigmented Paravenous Chorioretinal Atrophy. Invest. Ophthalmol. Vis. Sci. 2011;52(14):5007.
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To describe the spectral-domain optical coherence tomography (SD-OCT) findings and retinal function in two unrelated patients with pigmented paravenous chorioretinal atrophy (PPCRA).
Two patients, one 46-year old man and one 62-year old woman with PPCRA underwent clinical evaluation, consisting of best-corrected visual acuity, complete ophthalmological examination, SD-OCT (Spectralis, Heidelberg, Germany), fluorescein angiography, fundus autofluorescence, Goldmann visual fields and electroretinography.
Visual acuity in both patients was 20/20 bilaterally. Both patients showed chorioretinal atrophy and bone-spicule pigments along the peripheral retinal veins in both eyes, although it was asymmetrical in one of the patients. Visual fields in one patient showed mild restriction with corresponding visual field defects whereas the other patient showed ‘spiral’ field pattern due to functional overlay. Fluorescein angiography showed areas of hyperfluorescence depicting ‘window-defect’ along the veins that clinically showed areas of chorioretinal atrophy. An interesting finding was the presence of hyperfluorescence even along the veins in the main arcade, which clinically looked normal. SD-OCT showed loss of outer retinal layers and retinal pigment epithelium (RPE) thinning in areas adjacent to the veins. Retinal and RPE structure was normal in the intervening areas between the veins. Electroretinography showed slightly decreased scotopic and photopic responses but still within the normal range.
This study in two patients highlights for the first time the structural abnormality along the veins in pigmented paravenous chorioretinal atrophy using SD-OCT. There are certain findings on fluorescein angiography that may add to the pathogenesis of the disease.
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