Congenital uveal disorders

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