Lens Cone
๐๐ง๐ญ๐๐ซ๐ข๐จ๐ซ ๐๐๐ง๐ญ๐ข๐๐จ๐ง๐ฎ๐ฌโฃ
โ The anterior surface of the lens assumes a conical/ spherical shape and protrudes axially into the anterior chamberโฃ
โ Characteristically associated with Alportโs syndromeโฃ
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โ Bilateral in 75% of the casesโฃ
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โ Usually absent at birth and appears during second or third decadeโฃ
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โ โOil dropletโ reflex on retinoscopy/ slit lamp examination in early stagesโฃ
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โ The site of the cone shows thin basement membrane (capsule)โฃ
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โ Decrease in the number of nucleated cellsโฃ
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โ Irregular myopic lenticular astigmatismโฃ
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โ Can be associated with cataractโฃ
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โ Spontaneous rupture of the capsule can occurโฃ
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โ Caution should be taken during cataract surgery as lens capsule can be fragile especially during capsulorhexis and IOL implantationโฃ
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๐๐ฅ๐ฉ๐จ๐ซ๐ญโ๐ฌ ๐๐ฒ๐ง๐๐ซ๐จ๐ฆ๐โฃ
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โ Commonly X Linked inheritanceโฃ
โ Predominantly affects boysโฃ
โ Mutations in Type IV collagenโฃ
โ Hemorrhagic nephritis - abnormal glomerular basement membraneโฃ
โ Progressive sensorineural deafnessโฃ
โ Ophthalmic manifestations - anterior lenticonus, spherophakia, anterior polar cataract, retinal flecks, dot and fleck maculopathy, retinal and iris neovascularization, posterior polymorphous corneal dystrophyโฃ
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Image from Rajan Eye Care Hospital
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