Normally, the cells lining the inside of the cornea (endothelial cells) help maintain a healthy balance of fluids within the cornea. Healthy endothelial cells prevent the cornea from swelling and help keep the cornea clear. But with Fuchs' dystrophy, the endothelial cells slowly die off, and probably do not function correctly, resulting in fluid buildup within the cornea (swelling or edema). This causes corneal thickening and blurred vision.
Fuchs' dystrophy can be inherited. The genetic basis of the disease is complex — family members can be affected to very variable degrees, and sometimes not at all.
July 22, 2014
- Facts about the cornea and corneal disease. National Eye Institute. http://www.nei.nih.gov/health/cornealdisease. Accessed Feb. 7, 2014.
- What is Fuchs' dystrophy? American Academy of Ophthalmology. http://www.geteyesmart.org/eyesmart/diseases/fuchs-dystrophy.cfm. Accessed Feb. 7, 2014.
- Patel SV, et al. Anterior corneal aberrations after Descemet's stripping endothelial keratoplasty for Fuchs' endothelial dystrophy. Ophthalmology. 2012;119:1522.
- Gipson IK. Age-related changes and diseases of the ocular surface and cornea. Investigative ophthalmology and visual science. 2013;54:ORSF48.
- Hamill CE, et al. Fuchs endothelial cornea dystrophy: A review of the genetics behind disease development. Seminars in Ophthalmology. 2013;28:281.
- Riggin EA. Decision Support System. Mayo Clinic, Rochester, Minn. Feb. 10, 2014.
- Wieben ED, et al. A common trinucleotide repeat expansion within the transcription factor 4 (TCF4, E2-2) gene predicts Fuchs corneal dystrophy. PLoS One. 2012;7:e49083. http://www.plosone.org/article/info%3Adoi%2F10.1371%2Fjournal.pone.0049083. Accessed Feb. 13, 2014.
- What is a comprehensive dilated eye exam? National Eye Institute. http://www.nei.nih.gov/healthyeyes/eyeexam.asp. Accessed Feb. 24, 2014.
- The Wills Eye Manual: Office and Emergency Room Diagnosis and Treatment of Eye Disease Gerstenblith AT, et al., eds. Disease. 6th ed. Philadelphia, Pa.: Wolters Kluwer Health Lippincott Williams & Wilkins; 2012. http://ovidsp.ovid.com/ovidweb.cgi?T=JS&CSC=Y&NEWS=N&PAGE=booktext&D=books3&AN=01626623/6th_Edition/2&XPATH=/OVIDBOOK%5b1%5d/METADATA%5b1%5d/TBY%5b1%5d/EDITORS%5b1%5d. Accessed Feb. 24, 2014.
- Robertson DM (expert opinion). Mayo Clinic, Rochester, Minn. March 5, 2014.
- Patel SV. Graft survival and endothelial outcomes in the new era of endothelial keratoplasty. Experimental Eye Research. 2012; 95:40.
- Patel SV (expert opinion). Mayo Clinic, Rochester, Minn. May 20, 2014.
- Baratz KH, et al. E2-2 protein and Fuchs's corneal dystrophy. New England Journal of Medicine. 2010;363:1016.