Fuchs endothelial corneal dystrophy: current perspectives

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Abstract

Fuchs endothelial corneal dystrophy (FECD) is the most common corneal dystrophy and frequently results in vision loss. Hallmarks of the disease include loss of corneal endothelial cells and formation of excrescences of Descemet’s membrane. Later stages involve all layers of the cornea. Impairment of endothelial barrier and pump function and cell death from oxidative and unfolded protein stress contribute to disease progression. The genetic basis of FECD includes numerous genes and chromosomal loci, although alterations in the transcription factor 4 gene are associated with the majority of cases. Definitive treatment of FECD is corneal transplantation. In this paper, we highlight advances that have been made in understanding FECD’s clinical features, pathophysiology, and genetics. We also discuss recent advances in endothelial keratoplasty and potential future treatments.

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Most cited references 129

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The endoplasmic reticulum and the unfolded protein response.

Jyoti Malhotra, Randal Kaufman (2007)

The endoplasmic reticulum (ER) is the site where proteins enter the secretory pathway. Proteins are translocated into the ER lumen in an unfolded state and require protein chaperones and catalysts of protein folding to attain their final appropriate conformation. A sensitive surveillance mechanism exists to prevent misfolded proteins from transiting the secretory pathway and ensures that persistently misfolded proteins are directed towards a degradative pathway. In addition, those processes that prevent accumulation of unfolded proteins in the ER lumen are highly regulated by an intracellular signaling pathway known as the unfolded protein response (UPR). The UPR provides a mechanism by which cells can rapidly adapt to alterations in client protein-folding load in the ER lumen by expanding the capacity for protein folding. In addition, a variety of insults that disrupt protein folding in the ER lumen also activate the UPR. These include changes in intralumenal calcium, altered glycosylation, nutrient deprivation, pathogen infection, expression of folding-defective proteins, and changes in redox status. Persistent protein misfolding initiates apoptotic cascades that are now known to play fundamental roles in the pathogenesis of multiple human diseases including diabetes, atherosclerosis and neurodegenerative diseases.

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Descemet-stripping automated endothelial keratoplasty.

Alexey Gorovoy (2006)

To evaluate the speed of visual recovery in 16 consecutive patients with corneal endothelial dysfunction who received Descemet-stripping automated endothelial keratoplasty (DSAEK). This is a retrospective study of a novel method for small-incision endothelial transplantation (DSAEK). Endothelial replacement was accomplished with Descemet stripping of the recipient and insertion of a posterior donor tissue that had been prepared with a microkeratome. Best spectacle-corrected visual acuity (BSCVA) by manifest refraction, endothelial counts, and dislocation rates were measured up to 12 months after DSAEK. Sixteen consecutive patients underwent uncomplicated DSAEK. Three patients had known optic nerve or macular disease precluding vision better than 20/200. Of the remaining 14 patients, 11 had BSCVA of 20/40 by postoperative week 12 (7 by week 6). The remaining 2 were 20/50 by weeks 6 and 12. All 14 patients were 20/40 or better at 1 year. One patient had a primary graft failure, and surgery was repeated with 20/40 BSCVA at 1 year. The dislocation rate was 25%. The average cell count between 7 and 10 months was 1714. The average pachymetry was 682. DSAEK surgery allows rapid, excellent BSCVA visual recovery. The rate of visual recovery is more rapid than usually found with penetrating keratoplasty.

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A surgical technique for posterior lamellar keratoplasty.

G Melles, F Eggink, F Lander … (1998)

To design a surgical technique for transplantation of posterior corneal tissue, while leaving the recipient anterior cornea intact. In human cadaver eyes, and in a cat and monkey model, recipient eyes had an 8.0-mm limbal incision made with a diamond blade set to 50% of central pachymetry. A stromal pocket was created across the cornea, and a 6.0-mm diameter posterior lamellar disc was excised. A donor posterior disc was implanted into the recipient opening, and the limbal incision was sutured. The procedure was evaluated with keratometry, biomicroscopy, endothelial (supra)vital staining, and light microscopy. In human cadaver eyes, post-operative astigmatism averaged 1.2 D (SD, +/- 0.6 D). Posterior transplants showed an intact endothelial cell layer with 1.0% (SD, +/- 1.2%) of cell death. In the animals, six (75%) eyes had clear transplants 2 weeks after surgery; one of these eyes later developed an allograft rejection. Two (25%) eyes showed corneal decompensation, because of inverted implantation of the donor disc. Microscopy showed minimal scarring at the donor-to-host interface and a normal wound-healing response at the posterior stromal wound edges. In experimental models, posterior lamellar keratoplasty can be performed through a limbal incision and a mid-stromal pocket. The procedure may be a potential alternative in the surgical management of corneal endothelial disorders.

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Author and article information

Journal

Journal ID (nlm-ta): Clin Ophthalmol

Journal ID (iso-abbrev): Clin Ophthalmol

Journal ID (publisher-id): Clinical Ophthalmology

Title: Clinical Ophthalmology (Auckland, N.Z.)

Publisher: Dove Medical Press

ISSN (Print): 1177-5467

ISSN (Electronic): 1177-5483

Publication date Collection: 2016

Publication date (Electronic): 18 February 2016

Volume: 10

Pages: 321-330

Affiliations

Wilmer Eye Institute, Johns Hopkins Medical Institutions, Baltimore, MD, USA

Author notes

Correspondence: Albert S Jun, Wilmer Eye Institute, Johns Hopkins Hospital, 400 N Broadway, Smith 5011, Baltimore, MD 21231, USA, Tel +1 410 955 5494, Fax +1 410 502 3526, Email aljun@ 123456jhmi.edu

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Publisher ID: opth-10-321

DOI: 10.2147/OPTH.S83467

PMC ID: 4762439

PubMed ID: 26937169

SO-VID: 4d3a5424-0768-4de1-bf0e-1199b1bbb6dd

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The full terms of this license are available at https://www.dovepress.com/terms.php and incorporate the Creative Commons Attribution – Non Commercial (unported, v3.0) License ( http://creativecommons.org/licenses/by-nc/3.0/). By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed.

Fuchs endothelial corneal dystrophy: current perspectives

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Most cited references 129

The endoplasmic reticulum and the unfolded protein response.

Descemet-stripping automated endothelial keratoplasty.

A surgical technique for posterior lamellar keratoplasty.

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