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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