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𝘐𝘳𝘪𝘴 𝘢𝘵𝘳𝘰𝘱𝘩𝘺⁣

⁣Specific conditions:⁣ ⁣ Age related⁣ ● Atrophy of both iris stroma and pigment epithelial layer occurs as a natural aging process⁣ ● Bilateral, symmetric iris stromal atrophy.⁣ ● Normal pupillary contour with increased prominence of the pigment epithelium and⁣ the pupillary sphincter- tan rings⁣ ⁣ Viral Uveitis⁣ ● Chronic inflammation can lead to characteristic iris stromal atrophy⁣ ● VZV generally produces sector iris atrophy due to a vascular⁣ occlusive vasculitis, whereas HSV usually produces patchy iris atrophy⁣ ⁣ Essential iris atrophy⁣ ● ICE syndrome, unilateral⁣ ● Primary defect is believed to be a cellular membrane secreted by the abnormal endothelial cells which covers the iris and the drainage angle of the eye- contraction leads to pupillary changes and formation of PAS - angle closure glaucoma⁣ ● Displaced or distorted pupil, patchy areas of iris atrophy and holes in iris.⁣ ⁣ Trauma⁣ Post cataract surgery due to trauma to iris can lead to iris atrophy after many years.⁣ ⁣ Pigment Dispersion Syndrome⁣ ● Pigment granules settle on iris surface⁣ ● Posterior bowing of mid peripheral iris⁣ ● Classic mid peripheral,radial slit like transillumination defects in iris best⁣ seen on retroillumination.⁣ ⁣ PXF syndrome⁣ ● Particulate pigment deposition on sphincter and peripheral iris⁣ ● Moth-eaten transillumination defects occur at the sphincter⁣ ⁣ Post acute congestive glaucoma⁣ ● Partial/total absence of pupillary ruff⁣ ● Sphincter atrophy⁣ ● Whorling of the iris radial pattern⁣ ● Sectoral iris atrophy⁣ ⁣ Fuch’s Uveitis⁣ ● Diffuse atrophy of stroma and posterior pigment epithelium of iris.⁣ ● Determines the quality of heterochromia iridis.⁣ ⁣ Albinism⁣ ● Tyrosinase-negative: Lack of melanin⁣ ● Iris is diaphanous and translucent giving rise to a ‘pink-eyed appearance’⁣ ● Tyrosinase-positive: synthesise variable amounts of melanin.⁣ ● Iris may be blue or dark-brown, variable translucency⁣ ⁣ Axenfeld Rieger Syndrome⁣ ● Iris strands adherent to the posterior embryotoxon can range from fine threadlike strands to broad bands of iris tissue.⁣ ● Stroma - generalized atrophy with corectopia, ectropion uveae

www.ophthalmobytes.com Image from Rajan Eye Care Hospital #ophthalmology #ophthal #doctor #health #medical #vision #education #optometry #medicalstudent #optometrist #medicine #eye #ophtho #ophthalmologist #ophthalmo #med #medicaleducation #ophthalmologyresident #ophthalmologyresidency #iris #irisatrophy #atrophy #herpesuveitis #herpeszoster #herpessimplex Causes from entokey.com

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𝘚𝘵𝘢𝘳𝘨𝘢𝘳𝘥𝘵’𝘴 𝘋𝘪𝘴𝘦𝘢𝘴𝘦 𝘐𝘯𝘷𝘦𝘴𝘵𝘪𝘨𝘢𝘵𝘪𝘰𝘯𝘴 𝘢𝘯𝘥 𝘔𝘢𝘯𝘢𝘨𝘦𝘮𝘦𝘯𝘵 ⁣

𝙁𝙡𝙪𝙤𝙧𝙚𝙨𝙘𝙚𝙞𝙣 𝘼𝙣𝙜𝙞𝙤𝙜𝙧𝙖𝙥𝙝𝙮:⁣

● 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.⁣

𝙁𝙪𝙣𝙙𝙪𝙨 𝘼𝙪𝙩𝙤𝙛𝙡𝙪𝙤𝙧𝙚𝙨𝙘𝙚𝙣𝙘𝙚:⁣

● 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.⁣

𝙀𝙍𝙂 :⁣

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

● Slow dark adaptation⁣

𝙊𝘾𝙏 :⁣

● Shows atrophic changes and disorganisation in photoreceptors⁣

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

𝙈𝙖𝙣𝙖𝙜𝙚𝙢𝙚𝙣𝙩 :⁣

● 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⁣



Image from Rajan Eye Care Hospital #ophthalmology#ophthal#doctor#health#medical#vision#education#optometry#medicalstudent#optometrist#medicine#eye#ophtho#ophthalmologist#ophthalmo#med#medicaleducation#ophthalmologyresident#ophthalmologyresidency#stargardtsdisease#maculardystrophy#retina#macular#retinaldystrophy

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Sashwanthi & Madhuvanthi Mohan


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