Cystinosis:

• The eyes are the second most commonly affected extra-renal organ, with cystine crystal deposits in the corneas.
• Cystine crystals can be  observed in the cornea after age 1.5-2 years and are diagnostic of cystinosis.
• Pigmentary retinopathy consisting of patches of depigmentation may sometime be present as an early ocular finding and can result in impaired color vision and impaired night vision.
• Progressive retinopathy and band keratopathy occur later in life in patients not treated with cysteamine eye drops.

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