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Choroidal dystrophies

Choroideremia; Gyrate atrophy; Central areolar choroidal dystrophy

 

Choroidal dystrophy is an eye disorder that involves a layer of blood vessels called the choroid. These vessels are between the sclera and retina.

In most cases, choroidal dystrophy is due to an abnormal gene, which is passed down through families. It most often affects males, starting in childhood.

The first symptoms are peripheral vision loss and vision loss at night. An eye surgeon who specializes in the retina (back of the eye) can diagnose this disorder.

Exams and Tests

 

The following tests may be needed to diagnose the condition:

  • Electroretinography
  • Fluorescein angiography
  • Genetic testing

 

 

References

Genead MA, Fishman GA, Gover S. Hereditary choroidal diseases. In: Ryan SJ, Sadda SR, Hinton DR, Schachat AP, et al, eds. Retina. 5th ed. Philadelphia, PA: Elsevier Saunders; 2013:chap 43.

Grover S, Fishman GA, Ganead MA. Choroidal dystrophies In: Yanoff M, Duker JS, eds. Ophthalmology. 4th ed. Philadelphia, PA: Elsevier Saunders; 2014:chap 6.15.

 
  • External and internal eye anatomy

    External and internal eye anatomy - illustration

    The cornea allows light to enter the eye. As light passes through the eye the iris changes shape by expanding and letting more light through or constricting and letting less light through to change pupil size. The lens then changes shape to allow the accurate focusing of light on the retina. Light excites photoreceptors that eventually, through a chemical process, transmit nerve signals through the optic nerve to the brain. The brain processes these nerve impulses into sight.

    External and internal eye anatomy

    illustration

    • External and internal eye anatomy

      External and internal eye anatomy - illustration

      The cornea allows light to enter the eye. As light passes through the eye the iris changes shape by expanding and letting more light through or constricting and letting less light through to change pupil size. The lens then changes shape to allow the accurate focusing of light on the retina. Light excites photoreceptors that eventually, through a chemical process, transmit nerve signals through the optic nerve to the brain. The brain processes these nerve impulses into sight.

      External and internal eye anatomy

      illustration

    Tests for Choroidal dystrophies

     

       

      Review Date: 11/4/2015

      Reviewed By: Franklin W. Lusby, MD, ophthalmologist, Lusby Vision Institute, La Jolla, CA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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