BACK TOTOP Browse A-ZSearchBrowse A-ZABCDEFGHIJKLMNOPQRSTUVWXYZ0-9 E-mail FormEmail ResultsName:Email address:Recipients Name:Recipients address:Message: Print-FriendlyBookmarksbookmarks-menuChoroidal dystrophiesChoroideremia; Gyrate atrophy; Central areolar choroidal dystrophyChoroidal dystrophies are eye disorders that involve a layer of blood vessels called the choroid. These vessels are between the sclera (the white of the eye) and retina (the black of the eye). ChoroidThe choroid is the layer of blood vessels and connective tissue between the white of the eye and retina (at the back of the eye). It is part of the ...ImageRead Article Now Book Mark Article ScleraThe sclera is the white outer coating of the eye. It is tough, fibrous tissue that extends from the cornea (the clear front section of the eye) to t...ImageRead Article Now Book Mark Article RetinaThe retina is the light-sensitive layer of tissue at the back of the eyeball. Images that come through the eye's lens are focused on the retina. Th...ImageRead Article Now Book Mark Article In most cases, a 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 doctor 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 ElectroretinographyElectroretinography is a test to measure the electrical response of the eye's light-sensitive cells, called rods and cones. These cells are part of ...ImageRead Article Now Book Mark Article Fluorescein angiography Fluorescein angiographyFluorescein angiography is an eye test that uses a special dye and camera to look at blood flow in the retina and choroid. These are the two layers ...ImageRead Article Now Book Mark Article Genetic testingGenetic testingThe genes in our cells play important roles. They affect hair and eye color and other traits passed on from parent to child. Genes also tell cells ...Read Article Now Book Mark Article Treatment Clinical trials have been underway to try to slow the disease progression by injecting a form of the missing genetic material under the retina.Open ReferencesReferencesFreund 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.Fleckenstein M, Schmitz-Valckenberg S, Holz FG. Autofluorescence imaging. In: Schachat AP, Sadda SVR, Hinton DR, Wilkinson CP, Wiedemann P, eds. Ryan's Retina. 7th ed. Philadelphia, PA: Elsevier; 2023:chap 5.Lam BL, Davis JL, Gregori NZ. Choroideremia gene therapy. Int Ophthalmol Clin. 2021;61(4):185-193. PMID: 34584056 pubmed.ncbi.nlm.nih.gov/34584056/.Rahmani S, Schmitt M, Stepien KE. Inherited retinal diseases. In: Yanoff M, Duker JS, eds. Ophthalmology. 6th ed. Philadelphia, PA: Elsevier; 2023:chap 6.13.Yanoff M, Sassani JW. Uvea. In: Yanoff M, Sassani JW, eds. Ocular Pathology. 8th ed. Philadelphia, PA: Elsevier; 2020:chap 9.AllVideoImagesTogExternal 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 anatomyillustrationExternal 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 anatomyillustrationRelated Information Choroid(Special Topic)Sclera(Special Topic)Retina(Special Topic) Review Date: 11/8/2023 Reviewed By: Franklin W. Lusby, MD, Ophthalmologist, Lusby Vision Institute, La Jolla, CA. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team. 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Choroidal dystrophiesChoroideremia; Gyrate atrophy; Central areolar choroidal dystrophyChoroidal dystrophies are eye disorders that involve a layer of blood vessels called the choroid. These vessels are between the sclera (the white of the eye) and retina (the black of the eye). ChoroidThe choroid is the layer of blood vessels and connective tissue between the white of the eye and retina (at the back of the eye). It is part of the ...ImageRead Article Now Book Mark Article ScleraThe sclera is the white outer coating of the eye. It is tough, fibrous tissue that extends from the cornea (the clear front section of the eye) to t...ImageRead Article Now Book Mark Article RetinaThe retina is the light-sensitive layer of tissue at the back of the eyeball. Images that come through the eye's lens are focused on the retina. Th...ImageRead Article Now Book Mark Article In most cases, a 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 doctor 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 ElectroretinographyElectroretinography is a test to measure the electrical response of the eye's light-sensitive cells, called rods and cones. These cells are part of ...ImageRead Article Now Book Mark Article Fluorescein angiography Fluorescein angiographyFluorescein angiography is an eye test that uses a special dye and camera to look at blood flow in the retina and choroid. These are the two layers ...ImageRead Article Now Book Mark Article Genetic testingGenetic testingThe genes in our cells play important roles. They affect hair and eye color and other traits passed on from parent to child. Genes also tell cells ...Read Article Now Book Mark Article Treatment Clinical trials have been underway to try to slow the disease progression by injecting a form of the missing genetic material under the retina.Open ReferencesReferencesFreund 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.Fleckenstein M, Schmitz-Valckenberg S, Holz FG. Autofluorescence imaging. In: Schachat AP, Sadda SVR, Hinton DR, Wilkinson CP, Wiedemann P, eds. Ryan's Retina. 7th ed. Philadelphia, PA: Elsevier; 2023:chap 5.Lam BL, Davis JL, Gregori NZ. Choroideremia gene therapy. Int Ophthalmol Clin. 2021;61(4):185-193. PMID: 34584056 pubmed.ncbi.nlm.nih.gov/34584056/.Rahmani S, Schmitt M, Stepien KE. Inherited retinal diseases. In: Yanoff M, Duker JS, eds. Ophthalmology. 6th ed. Philadelphia, PA: Elsevier; 2023:chap 6.13.Yanoff M, Sassani JW. Uvea. In: Yanoff M, Sassani JW, eds. Ocular Pathology. 8th ed. Philadelphia, PA: Elsevier; 2020:chap 9.