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Fuchs' corneal dystrophy

Lorena Sepsakos, Kevin Shah, T Peter Lindquist, William Barry Lee, Edward Holland
PURPOSE: To evaluate the rate of rejection after Descemet stripping automated endothelial keratoplasty (DSAEK) in patients with Fuchs endothelial dystrophy (ED) and to analyze potential risk factors for rejection. METHODS: An observational retrospective chart review of 400 patients with Fuchs ED was performed. The primary outcome was the rate of endothelial graft rejection. Secondary outcomes included graft clarity after rejection, medications at the time of rejection, and preoperative graft thickness...
October 5, 2016: Cornea
Sotiria Palioura, Kathryn Colby
PURPOSE: To evaluate the feasibility of Descemet stripping endothelial keratoplasty using grafts preloaded by an eye bank in a commercially available insertion device. METHODS: In this retrospective case series, a series of 35 eyes in 34 consecutive patients who underwent Descemet stripping endothelial keratoplasty for Fuchs endothelial dystrophy or previously failed full-thickness grafts at a single tertiary care center from March 2013 to March 2014 was included...
October 12, 2016: Cornea
Muhammad Rizwan, Gary S Peh, Khadijah Adnan, Sacha L Naso, Alon R Mendez, Jodhbir S Mehta, Evelyn K F Yim
A common indication for corneal transplantation, which is the most transplanted tissue, is a dysfunctional corneal endothelium due to Fuchs' endothelial dystrophy (FED). FED is diagnosed by the presence of in vivo pathological microtopography on the Descemet membrane, which is called corneal guttata. Minimally invasive corneal endothelial cell regenerative procedures such as endothelial cell injection therapy and Rho kinase inhibitor pharmacotherapy have been proposed as alternatives to conventional corneal transplantation for FED patients...
October 4, 2016: Advanced Healthcare Materials
Thomas M Müller, Robert M Verdijk, Itay Lavy, Marieke Bruinsma, Jack Parker, Perry S Binder, Gerrit R J Melles
PURPOSE: To describe the histologic features of postmortem eyes after Descemet membrane endothelial keratoplasty (DMEK) and their potential clinical implications. DESIGN: Histopathologic study. PARTICIPANTS: Eleven postmortem DMEK corneas of 8 patients who underwent surgery for Fuchs endothelial dystrophy, with an average postoperative time of 4±1.9 years (range, 7 months-6.5 years). METHODS: Eleven corneas transplanted with a DMEK graft were procured after death and processed for light microscopy evaluation...
September 21, 2016: Ophthalmology
Katrin Wacker, Jay W McLaren, Katrina M Kane, Keith H Baratz, Sanjay V Patel
Purpose: To assess corneal hydration control across a range of severity of Fuchs' endothelial corneal dystrophy (FECD) by measuring the percent recovery per hour (PRPH) of central corneal thickness after swelling the cornea and to determine its association with corneal morphologic parameters. Methods: Twenty-three corneas of 23 phakic FECD patients and 8 corneas of 8 healthy control participants devoid of guttae were graded (modified Krachmer scale). Effective endothelial cell density (ECDe) was determined from the area of guttae and local cell density in confocal microscopy images...
September 1, 2016: Investigative Ophthalmology & Visual Science
Korine van Dijk, Marina Rodriguez-Calvo-de-Mora, Hilde van Esch, Laurence Frank, Isabel Dapena, Lamis Baydoun, Silke Oellerich, Gerrit R J Melles
PURPOSE: To monitor refractive changes after Descemet membrane endothelial keratoplasty (DMEK) and to determine what may influence these changes and the time point of stabilization. METHODS: From 67 pseudophakic DMEK eyes operated on for Fuchs endothelial dystrophy at a tertiary referral center, biomicroscopy, visual acuity, subjective refraction, and Scheimpflug-based corneal tomography data were obtained before and up to 2 years postoperatively. Visual acuity and changes in spherical equivalent (SE), mean anterior and posterior simulated keratometry (Km), and central pachymetry were analyzed...
September 21, 2016: Cornea
Mor M Dickman, Pieter J Kruit, Lies Remeijer, Jeroen van Rooij, Allegonda Van der Lelij, Robert H J Wijdh, Frank J H M van den Biggelaar, Tos T J M Berendschot, Rudy M M A Nuijts
OBJECTIVE: To compare visual acuity, refraction, endothelial cell density (ECD), and complications after Descemet stripping automated endothelial keratoplasty (DSAEK) and ultrathin DSAEK (UT-DSAEK). DESIGN: A multicenter, prospective, double-masked, randomized, controlled clinical trial. PARTICIPANTS: From 66 patients with irreversible corneal endothelial dysfunction dues to Fuchs' dystrophy who enrolled from 4 tertiary medical centers in the Netherlands, 66 eyes were studied...
September 19, 2016: Ophthalmology
Kishore Reddy Katikireddy, Thore Schmedt, Marianne O Price, Francis W Price, Ula V Jurkunas
Human corneal endothelial cells are derived from neural crest and because of postmitotic arrest lack competence to repair cell loss from trauma, aging, and degenerative disorders such as Fuchs endothelial corneal dystrophy (FECD). Herein, we identified a rapidly proliferating subpopulation of cells from the corneal endothelium of adult normal and FECD donors that exhibited features of neural crest-derived progenitor (NCDP) cells by showing absence of senescence with passaging, propensity to form spheres, and increased colony forming efficacy compared with the primary cells...
October 2016: American Journal of Pathology
Maryam Ali, VijayKrishna Raghunathan, Jennifer Y Li, Christopher J Murphy, Sara M Thomasy
The posterior face of the cornea consists of the corneal endothelium, a monolayer of cuboidal cells that secrete and attach to Descemet's membrane, an exaggerated basement membrane. Dysfunction of the endothelium compromises the barrier and pump functions of this layer that maintain corneal deturgesence. A large number of corneal endothelial dystrophies feature irregularities in Descemet's membrane, suggesting that cells create and respond to the biophysical signals offered by their underlying matrix. This review provides an overview of the bidirectional relationship between Descemet's membrane and the corneal endothelium...
September 14, 2016: Experimental Eye Research
Bia Z Kim, Jay J Meyer, Nigel H Brookes, S Louise Moffatt, Helen C Twohill, David G Pendergrast, Trevor Sherwin, Charles N J McGhee
AIMS: To report the 25-year longitudinal trends in indications and corneal transplantation techniques in New Zealand. METHODS: Statistical analysis of prospectively acquired New Zealand National Eye Bank (NZNEB) electronic database from 1991 to 2015 inclusive. Subjects were recipients of corneal transplants in 62 centres supplied by the NZNEB. Main outcome measures were indications, recipient age and transplantation techniques. RESULTS: From January 1991 to December 2015, NZNEB supplied tissue for 5574 corneal transplants, increasing annually from 89 (1991) to 290 (2015)...
September 15, 2016: British Journal of Ophthalmology
Sarah B Weissbart, Kristin M Hammersmith, Brandon D Ayres, Christopher J Rapuano, Parveen K Nagra, Irving M Raber, Amir A Azari
PURPOSE: Long-standing corneal edema can lead to anterior stromal scarring that may limit visual acuity following Descemet stripping endothelial keratoplasty (DSEK). The ideal time to perform DSEK to prevent permanent changes is unclear. Our aim is to determine whether earlier DSEK is associated with improved visual outcomes. DESIGN: Cohort study. METHODS: Setting: Institutional. STUDY POPULATION: 120 eyes of patients who underwent DSEK for corneal edema following cataract surgery (CE)...
September 12, 2016: American Journal of Ophthalmology
Yoshinori Oie, Shinya Watanabe, Kohji Nishida
Fuchs endothelial corneal dystrophy (FECD) is a bilateral corneal endothelial dystrophy characterized by the deposition of extracellular matrix (guttae), thickening of Descemet membrane, and progressive loss of corneal endothelial cells. Progressive endothelial cellular loss leads to corneal edema and impairs visual function. In eyes with FECD, corneal edema begins in the central cornea and expands into the periphery. FECD is the most common dystrophic reason for corneal transplantation in many countries. Some patients with FECD report visual discomfort despite the absence of corneal edema...
November 2016: Cornea
Itay Lavy, Robert M Verdijk, Marieke Bruinsma, Hein F Sleddens, Silke Oellerich, Perry S Binder, Gerrit R J Melles
PURPOSE: To identify the origin of corneal endothelial cells (host or donor) present on grafts at various time points after Descemet membrane endothelial keratoplasty (DMEK), using fluorescence in situ hybridization (FISH) of sex chromosomes on post mortem corneas with sex mismatch between the donor and host. METHODS: Corneoscleral buttons of 6 post mortem DMEK eyes of 4 patients, operated for Fuchs endothelial dystrophy, with an average postoperative time of 2...
September 8, 2016: Cornea
Julia M Weller, Ursula Schlötzer-Schrehardt, Friedrich E Kruse, Theofilos Tourtas
PURPOSE: In Descemet membrane endothelial keratoplasty (DMEK), lamellar splitting of the Descemet's membrane (DM) may occur during stripping of host's DM, leaving residual DM on the recipient's DMEK interface. The purpose of this study was to determine the incidence rate of lamellar splitting of DM during DMEK and to describe the ultrastructure of DM in these eyes. DESIGN: Retrospective consecutive case series METHODS: Setting: Institutional, single-center PATIENT POPULATION: 664 eyes with Fuchs endothelial corneal dystrophy (FECD) scheduled for primary DMEK...
September 5, 2016: American Journal of Ophthalmology
Liyo Kao, Rustam Azimov, X M Shao, Ricardo F Frausto, Natalia Abuladze, Debra Newman, Anthony J Aldave, Ira Kurtz
Congenital hereditary endothelial dystrophy (CHED), Harboyan syndrome (CHED with progressive sensorineural deafness), and potentially a subset of individuals with late onset Fuchs endothelial corneal dystrophy (FECD) are caused by mutations in the SLC4A11 gene that results in corneal endothelial cell abnormalities. Originally classified as a borate transporter, the function of SLC4A11 as a transport protein remains poorly understood. Elucidating the transport function(s) of SLC4A11 is needed to better understand how its loss results in the aforementioned posterior corneal dystrophic disease processes...
August 31, 2016: American Journal of Physiology. Cell Physiology
Sampath K Loganathan, Hans-Peter Schneider, Patricio E Morgan, Joachim W Deitmer, Joseph R Casey
SLC4A11, a member of the SLC4 family of bicarbonate transporters, is a widely expressed integral membrane protein, abundant in kidney and cornea. Mutations of SLC4A11 cause some cases of the blinding corneal dystrophies, congenital hereditary endothelial dystrophy and Fuchs endothelial corneal dystrophy. These diseases are marked by fluid accumulation in the corneal stroma, secondary to defective fluid reabsorption by the corneal endothelium. The role of SLC4A11 in these corneal dystrophies is not firmly established, as SLC4A11 function remains unclear...
August 24, 2016: American Journal of Physiology. Cell Physiology
Michael A Mahr, Keith H Baratz, David O Hodge, Jay C Erie
Importance: Fuchs endothelial corneal dystrophy (FECD) is the most common indication for corneal transplant in the United States. The association between race/ethnicity and incidence of advanced FECD, defined by a need for endothelial or penetrating keratoplasty, has not been investigated. Observations: The 2014 US Medicare Limited Data Set (5% sample of 27 163 740 fee-for-service Medicare patients) was analyzed for rate of keratoplasty performed for FECD (International Classification of Diseases, Ninth Edition code 371...
October 1, 2016: JAMA Ophthalmology
J Jankowska-Szmul, D Dobrowolski, K Krysik, J Kwas, M Nejman, E Wylegala
BACKGROUND: The purpose of this work was to study the evolving trends in techniques and indications for corneal transplantation in Poland. METHODS: This retrospective, descriptive analysis of corneal transplantations was performed at the Ophthalmology Department of Saint Barbara Hospital, Trauma Center, Sosnowiec, Poland, between 1988 and 2014. Structure of indications and surgery type rates over 26 years were tabulated by means of 5-year intervals. RESULTS: Between 1989 and 2014, 1762 corneal transplantations were performed, including 1375 (78%) cases of penetrating keratoplasty, 137 (8%) lamellar keratoplasty, 112 (6%) patch grafts, and 138 (8%) keratolimbal allografts...
June 2016: Transplantation Proceedings
Bernhard Steger, Elinor Curnow, Robert Cheeseman, Vito Romano, Abigail Kaye, Mark Jones, Stephen Kaye
PURPOSE: To investigate graft survival and rejection following sequential bilateral corneal transplantation. DESIGN: Retrospective cohort study. METHODS: The study included patients with Fuchs endothelial dystrophy (FED), pseudophakic bullous keratopathy (PBK), or keratoconus who had undergone a penetrating keratoplasty (PK), endothelial keratoplasty (EK), or deep anterior lamellar keratoplasty (DALK) between 1999 and 2012. The main cohort included patients who had received a first transplant in both eyes for the same indication and a control cohort patients who had undergone a unilateral first corneal transplant...
October 2016: American Journal of Ophthalmology
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