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Writer's pictureMadhuvanthi Mohan

Lens cone!


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ā— The anterior surface of the lens assumes a conical/ spherical shape and protrudes axially into the anterior chamberā£




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ā— Characteristically associated with Alportā€™s syndromeā£

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ā— Bilateral in 75% of the casesā£

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ā— Usually absent at birth and appears during second or third decadeā£

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ā— ā€˜Oil dropletā€™ reflex on retinoscopy/ slit lamp examination in early stagesā£

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ā— The site of the cone shows thin basement membrane (capsule)ā£

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ā— Decrease in the number of nucleated cellsā£

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ā— Irregular myopic lenticular astigmatismā£

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ā— Can be associated with cataractā£

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ā— Spontaneous rupture of the capsule can occurā£

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ā— Caution should be taken during cataract surgery as lens capsule can be fragile especially during capsulorhexis and IOL implantationā£

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š€š„š©šØš«š­ā€™š¬ š’š²š§šš«šØš¦šžā£

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ā— Commonly X Linked inheritanceā£

ā— Predominantly affects boysā£

ā— Mutations in Type IV collagenā£

ā— Hemorrhagic nephritis - abnormal glomerular basement membraneā£

ā— Progressive sensorineural deafnessā£

ā— Ophthalmic manifestations - anterior lenticonus, spherophakia, anterior polar cataract, retinal flecks, dot and fleck maculopathy, retinal and iris neovascularization, posterior polymorphous corneal dystrophyā£

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