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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Image from Rajan Eye Care Hospital

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