Flecks! Part 2

𝘚𝘵𝘢𝘳𝘨𝘢𝘳𝘥𝘵’𝘴 𝘋𝘪𝘴𝘦𝘢𝘴𝘦 𝘐𝘯𝘷𝘦𝘴𝘵𝘪𝘨𝘢𝘵𝘪𝘰𝘯𝘴 𝘢𝘯𝘥 𝘔𝘢𝘯𝘢𝘨𝘦𝘮𝘦𝘯𝘵 ⁣

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𝙁𝙡𝙪𝙤𝙧𝙚𝙨𝙘𝙚𝙞𝙣 𝘼𝙣𝙜𝙞𝙤𝙜𝙧𝙖𝙥𝙝𝙮:⁣

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● Dark choroid sign- Not exclusive to Stargardt disease.⁣

● Due to a lack of early choroidal hyperfluorescence, which is⁣

blocked by high-grade lipofuscin accumulation in the RPE, thus improving visualization of the small retinal capillaries that become easily evident over the dark, non-fluorescent and high-contrast choroid (blockhead arrow)⁣

● Atrophic fovea- choroidal vessels become visible⁣

● Fundus flecks are seen as small irregular hyperfluorescent lesions⁣

(arrow). The clinically visible flecks do not necessarily correspond to the window-defects in the FFA.⁣




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𝙁𝙪𝙣𝙙𝙪𝙨 𝘼𝙪𝙩𝙤𝙛𝙡𝙪𝙤𝙧𝙚𝙨𝙘𝙚𝙣𝙘𝙚:⁣

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● Abnormally increased FAF represents excessive lipofuscin accumulation in the RPE.⁣

● Inversely, decreased areas of FAF relate to low level RPE metabolic activity which normally underlies local atrophy with secondary photoreceptor loss.⁣

● Abnormally high FAF intensity with all other normal parameters suggests that RPE lipofuscin deposition may be the first pathophysiological event in ABCA4-related disease.⁣

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𝙀𝙍𝙂 :⁣

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● Normal in early disease. Reduced scotopic and photopic responses in advanced cases.⁣

● Slow dark adaptation⁣

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𝙊𝘾𝙏 :⁣

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● Shows atrophic changes and disorganisation in photoreceptors⁣

● RPE and lipofuscin deposits maybe detected within the parafoveal RPE.⁣




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𝙈𝙖𝙣𝙖𝙜𝙚𝙢𝙚𝙣𝙩 :⁣

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● No proven treatment.⁣

● Avoid Vitamin A and its supplements- makes the disease worse.⁣

● PUFA such as Docosahexaenoic acid (DHA) shown to reduce toxicity of⁣

A2E ( In autosomal dominant Stargardt)⁣

● Gene therapy⁣

● RPE precursor cells derived from embryonic stem cells injected⁣

subretinally.⁣

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www.ophthalmobytes.com⁣

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