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Congenital uveal disorders

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

Authoring team

Congential disorders of the uvea include:

  • albinism:
    • there is absence of pigment from the eye alone or from the whole body
    • visual acuity is poor
    • there may be nystagmus
    • referral should be made to a specialist centre

  • aniridia:
    • this is a rare genetically-determined trait
    • visual acuity is poor
    • there is a tendency to cataract formation
    • aniridia may be part of global defect in the development of the anterior eye called mesodermal dysgenesis

  • colobomata:
    • this is a developmental defect in the uvea, usually in the lower, nasal quadrant
    • large defects are associated with poor vision

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