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Spherophakia!



-Spherophakia is a rare congenital bilateral eye disorder.

-Usually patients present in adolescence or young adulthood.

-Presents with weak zonules around a more spherical crystalline lens with an increased anteroposterior thickness of the lens, and highly myopic eye.

-The lens zonules are developmentally hypoplastic and abnormally weak and due to non-attachment of the posterior zonules to the equatorial zone of the lens, the lens changes its normal shape to spherical.

-The lens may undergo subluxation or dislocation from the patellar fossa, leading to defective accommodation.

-The disease can present as an isolated condition or may run in families (due to consanguinity).

-Subluxation of lens may occur anteriorly, inferiorly, or posteriorly and may lead to pupillary block glaucoma.


FEATURES:

-Spherical lens.

-Iridodonesis.

-Axial myopia.

-Glaucoma.


MANAGEMENT:

-Lensectomy for managing the dislocated lens.

-Visual rehabilitation becomes important, with options being aphakic spectacles, contact lenses, and IOLs.


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