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Lens cone!

𝐀𝐧𝐭𝐞𝐫𝐢𝐨𝐫 𝐋𝐞𝐧𝐭𝐢𝐜𝐨𝐧𝐮𝐬⁣

● The anterior surface of the lens assumes a conical/ spherical shape and protrudes axially into the anterior chamber⁣

● Characteristically associated with Alport’s syndrome⁣

● Bilateral in 75% of the cases⁣

● Usually absent at birth and appears during second or third decade⁣

● ‘Oil droplet’ reflex on retinoscopy/ slit lamp examination in early stages⁣

● The site of the cone shows thin basement membrane (capsule)⁣

● Decrease in the number of nucleated cells⁣

● Irregular myopic lenticular astigmatism⁣

● Can be associated with cataract⁣

● Spontaneous rupture of the capsule can occur⁣

● Caution should be taken during cataract surgery as lens capsule can be fragile especially during capsulorhexis and IOL implantation⁣

𝐀𝐥𝐩𝐨𝐫𝐭’𝐬 𝐒𝐲𝐧𝐝𝐫𝐨𝐦𝐞⁣

● Commonly X Linked inheritance⁣

● Predominantly affects boys⁣

● Mutations in Type IV collagen⁣

● Hemorrhagic nephritis - abnormal glomerular basement membrane⁣

● Progressive sensorineural deafness⁣

● Ophthalmic manifestations - anterior lenticonus, spherophakia, anterior polar cataract, retinal flecks, dot and fleck maculopathy, retinal and iris neovascularization, posterior polymorphous corneal dystrophy⁣

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