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Fuchs corneal dystrophy

MedGen UID:
4800
•Concept ID:
C0016781
•
Disease or Syndrome
Synonyms: Dystrophy, Fuch's Endothelial; Dystrophy, Fuchs' Endothelial; Endothelial Dystrophy, Fuch's; Endothelial Dystrophy, Fuchs'; Fuch Endothelial Dystrophy; Fuch's Endothelial Dystrophy; Fuchs Atrophy; Fuchs Corneal Dystrophy; Fuchs Dystrophy; Fuchs Endothelial Corneal Dystrophy; Fuchs Endothelial Dystrophy; Fuchs' Endothelial Dystrophy
SNOMED CT: Fuchs corneal dystrophy (193839007); Fuchs' corneal dystrophy (193839007); Fuchs' endothelial dystrophy (193839007); Fuch's endothelial corneal dystrophy (193839007)

Definition

An autosomal dominant, bilateral, slowly progressive degeneration of corneal endothelial cells with thickening of Descemet''s membrane and accumulation of excrescences. It results in corneal edema and loss of vision. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVFuchs corneal dystrophy

Professional guidelines

PubMed

An update on ripasudil for the treatment of glaucoma and ocular hypertension.
Testa V, Ferro Desideri L, Della Giustina P, Traverso CE, Iester M
Drugs Today (Barc) 2020 Sep;56(9):599-608. doi: 10.1358/dot.2020.56.9.3178110. PMID: 33025953

Recent clinical studies

Etiology

Cataract surgery and intraocular lens placement in patients with Fuchs corneal dystrophy: a review of the current literature.
Moshirfar M, Huynh R, Ellis JH
Curr Opin Ophthalmol 2022 Jan 1;33(1):21-27. doi: 10.1097/ICU.0000000000000816. PMID: 34743088
Long-term Outcomes in Fellow Eyes Comparing DSAEK and DMEK for Treatment of Fuchs Corneal Dystrophy.
Weisenthal RW, Yin HY, Jarstad AR, Wang D, Verdier DD
Am J Ophthalmol 2022 Jan;233:216-226. Epub 2021 Jun 19 doi: 10.1016/j.ajo.2021.06.013. PMID: 34157279
Omics Biomarkers in Ophthalmology.
Lauwen S, de Jong EK, Lefeber DJ, den Hollander Al
Invest Ophthalmol Vis Sci 2017 May 1;58(6):BIO88-BIO98. doi: 10.1167/iovs.17-21809. PMID: 28525563
Corneal Astigmatism Stability in Descemet Membrane Endothelial Keratoplasty for Fuchs Corneal Dystrophy.
Yokogawa H, Sanchez PJ, Mayko ZM, Straiko MD, Terry MA
Cornea 2016 Jul;35(7):932-7. doi: 10.1097/ICO.0000000000000882. PMID: 27191667
3-D characterization of the corneal shape in Fuchs dystrophy and pseudophakic keratopathy.
Brunette I, Sherknies D, Terry MA, Chagnon M, Bourges JL, Meunier J
Invest Ophthalmol Vis Sci 2011 Jan 5;52(1):206-14. doi: 10.1167/iovs.09-4101. PMID: 21169529

Diagnosis

CTG18.1 Expansion in TCF4 Among African Americans With Fuchs' Corneal Dystrophy.
Eghrari AO, Vahedi S, Afshari NA, Riazuddin SA, Gottsch JD
Invest Ophthalmol Vis Sci 2017 Dec 1;58(14):6046-6049. doi: 10.1167/iovs.17-21661. PMID: 29196769Free PMC Article
Long-term evaluation of endothelial cell changes in Fuchs corneal dystrophy: the influence of phacoemulsification and penetrating keratoplasty.
Kim YW, Kim MK, Wee WR
Korean J Ophthalmol 2013 Dec;27(6):409-15. Epub 2013 Nov 15 doi: 10.3341/kjo.2013.27.6.409. PMID: 24311925Free PMC Article
Prevalence and severity of fuchs corneal dystrophy in Tangier Island.
Eghrari AO, McGlumphy EJ, Iliff BW, Wang J, Emmert D, Riazuddin SA, Katsanis N, Gottsch JD
Am J Ophthalmol 2012 Jun;153(6):1067-72. Epub 2012 Feb 8 doi: 10.1016/j.ajo.2011.11.033. PMID: 22321803Free PMC Article
Combined granular and Fuchs' corneal dystrophy diagnosed by confocal microscopy after total anterior lamellar keratoplasty.
John T, Pullos A
Ann Ophthalmol (Skokie) 2009 Fall-Winter;41(3-4):179-83. PMID: 20214052
A morphologic study of Fuchs dystrophy and bullous keratopathy.
Yuen HK, Rassier CE, Jardeleza MS, Green WR, de la Cruz Z, Stark WJ, Gottsch JD
Cornea 2005 Apr;24(3):319-27. doi: 10.1097/01.ico.0000148288.53323.b2. PMID: 15778606

Therapy

Phase 2, Randomized, Open-Label Parallel-Group Study of Two Dosing Regimens of Netarsudil for the Treatment of Corneal Edema Due to Fuchs Corneal Dystrophy.
Lindstrom RL, Lewis AE, Holland EJ, Sheppard JD, Hovanesian JA, Senchyna M, Hollander DA
J Ocul Pharmacol Ther 2022 Dec;38(10):657-663. Epub 2022 Nov 3 doi: 10.1089/jop.2022.0069. PMID: 36327101Free PMC Article
An update on ripasudil for the treatment of glaucoma and ocular hypertension.
Testa V, Ferro Desideri L, Della Giustina P, Traverso CE, Iester M
Drugs Today (Barc) 2020 Sep;56(9):599-608. doi: 10.1358/dot.2020.56.9.3178110. PMID: 33025953
Association of common variants in TCF4 and PTPRG with Fuchs' corneal dystrophy: a systematic review and meta-analysis.
Lau LC, Ma L, Young AL, Rong SS, Jhanji V, Brelen ME, Pang CP, Chen LJ
PLoS One 2014;9(10):e109142. Epub 2014 Oct 9 doi: 10.1371/journal.pone.0109142. PMID: 25299301Free PMC Article
Endothelial keratoplasty for Fuchs' dystrophy with cataract: complications and clinical results with the new triple procedure.
Terry MA, Shamie N, Chen ES, Phillips PM, Shah AK, Hoar KL, Friend DJ
Ophthalmology 2009 Apr;116(4):631-9. Epub 2009 Feb 8 doi: 10.1016/j.ophtha.2008.11.004. PMID: 19201480
Cataract surgery in patients with Fuchs' corneal dystrophy: expanding recommendations for cataract surgery without simultaneous keratoplasty.
Seitzman GD, Gottsch JD, Stark WJ
Ophthalmology 2005 Mar;112(3):441-6. doi: 10.1016/j.ophtha.2004.10.044. PMID: 15745771

Prognosis

CTG18.1 Expansion in TCF4 Among African Americans With Fuchs' Corneal Dystrophy.
Eghrari AO, Vahedi S, Afshari NA, Riazuddin SA, Gottsch JD
Invest Ophthalmol Vis Sci 2017 Dec 1;58(14):6046-6049. doi: 10.1167/iovs.17-21661. PMID: 29196769Free PMC Article
Omics Biomarkers in Ophthalmology.
Lauwen S, de Jong EK, Lefeber DJ, den Hollander Al
Invest Ophthalmol Vis Sci 2017 May 1;58(6):BIO88-BIO98. doi: 10.1167/iovs.17-21809. PMID: 28525563
Long-term evaluation of endothelial cell changes in Fuchs corneal dystrophy: the influence of phacoemulsification and penetrating keratoplasty.
Kim YW, Kim MK, Wee WR
Korean J Ophthalmol 2013 Dec;27(6):409-15. Epub 2013 Nov 15 doi: 10.3341/kjo.2013.27.6.409. PMID: 24311925Free PMC Article
Mathematical projection model of visual loss due to fuchs corneal dystrophy.
Hatou S, Shimmura S, Shimazaki J, Usui T, Amano S, Yokogawa H, Kobayashi A, Zheng X, Shiraishi A, Ohashi Y, Inatomi T, Tsubota K
Invest Ophthalmol Vis Sci 2011 Oct 7;52(11):7888-93. doi: 10.1167/iovs.11-8040. PMID: 21873666
Cataract surgery in Fuchs corneal dystrophy.
Eghrari AO, Daoud YJ, Gottsch JD
Curr Opin Ophthalmol 2010 Jan;21(1):15-9. doi: 10.1097/ICU.0b013e328333e9d6. PMID: 19910792

Clinical prediction guides

Phase 2, Randomized, Open-Label Parallel-Group Study of Two Dosing Regimens of Netarsudil for the Treatment of Corneal Edema Due to Fuchs Corneal Dystrophy.
Lindstrom RL, Lewis AE, Holland EJ, Sheppard JD, Hovanesian JA, Senchyna M, Hollander DA
J Ocul Pharmacol Ther 2022 Dec;38(10):657-663. Epub 2022 Nov 3 doi: 10.1089/jop.2022.0069. PMID: 36327101Free PMC Article
Omics Biomarkers in Ophthalmology.
Lauwen S, de Jong EK, Lefeber DJ, den Hollander Al
Invest Ophthalmol Vis Sci 2017 May 1;58(6):BIO88-BIO98. doi: 10.1167/iovs.17-21809. PMID: 28525563
Retroillumination Photography Analysis Enhances Clinical Definition of Severe Fuchs Corneal Dystrophy.
Eghrari AO, Garrett BS, Mumtaz AA, Edalati AE, Meadows DN, McGlumphy EJ, Iliff BW, Gottsch JD
Cornea 2015 Dec;34(12):1623-6. doi: 10.1097/ICO.0000000000000656. PMID: 26488628Free PMC Article
RNA toxicity and missplicing in the common eye disease fuchs endothelial corneal dystrophy.
Du J, Aleff RA, Soragni E, Kalari K, Nie J, Tang X, Davila J, Kocher JP, Patel SV, Gottesfeld JM, Baratz KH, Wieben ED
J Biol Chem 2015 Mar 6;290(10):5979-90. Epub 2015 Jan 15 doi: 10.1074/jbc.M114.621607. PMID: 25593321Free PMC Article
Prevalence and severity of fuchs corneal dystrophy in Tangier Island.
Eghrari AO, McGlumphy EJ, Iliff BW, Wang J, Emmert D, Riazuddin SA, Katsanis N, Gottsch JD
Am J Ophthalmol 2012 Jun;153(6):1067-72. Epub 2012 Feb 8 doi: 10.1016/j.ajo.2011.11.033. PMID: 22321803Free PMC Article

Recent systematic reviews

Association of common variants in TCF4 and PTPRG with Fuchs' corneal dystrophy: a systematic review and meta-analysis.
Lau LC, Ma L, Young AL, Rong SS, Jhanji V, Brelen ME, Pang CP, Chen LJ
PLoS One 2014;9(10):e109142. Epub 2014 Oct 9 doi: 10.1371/journal.pone.0109142. PMID: 25299301Free PMC Article

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