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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:

References

Freund KB, Sarraf D, Mieler WF, Yannuzzi LA. Hereditary chorioretinal dystrophies. In: Freund KB, Sarraf D, Mieler WF, Yannuzzi LA, eds. The Retinal Atlas. 2nd ed. Philadelphia, PA: Elsevier; 2017:chap 2.

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.

Klufas MA, Kiss S. Wide-field imaging. In: Schachat AP, Sadda SVR, Hinton DR, Wilkinson CP, Wiedemann P, eds. Ryan's Retina. 6th ed. Philadelphia, PA: Elsevier; 2018:chap 5.

Noble KG. Fluorescein angiography of the hereditary chorioretinal dystrophies. In: Tasman W, Jaeger EA, eds. Duane's Foundations of Clinical Ophthalmology. 2013 ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2013: vol 2, chap 113B.

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  • 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 - 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

 

Review Date: 12/2/2017

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

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