The surgical management of a patient with Fuchs endothelial dystrophy and cataracts.

Objective: To report the two different surgical approaches in the case of a patient with Fuchs endothelial dystrophy with low endothelial cell count and advanced cataracts. Methods: The chosen surgical approach differed between eyes, with the right eye undergoing a combined approach consisting of cataract surgery, intraocular lens implantation, and penetrating keratoplasty in 2022. One year later, for the left eye, a different approach was decided: cataract surgery followed by Descemet membrane endothelial keratoplasty (DMEK). The Descemet membrane graft was prepared by the surgeon using the liquid bubble technique. AS-OCT was used to monitor the patient before and after surgery. Results: Visual recovery was excellent for both eyes, however, visual acuity improved quickly in the left eye (DMEK), while, in the right eye (PK), the best corrected visual acuity was reached after several months post-surgery. Conclusion: Advanced stages of Fuchs dystrophy patients will most likely need corneal transplantation. Each type of corneal transplantation procedure comes with unique challenges, both intraoperative and postoperative. DMEK is a very good treatment option for patients with Fuchs endothelial dystrophy, with excellent visual recovery and good graft survival at the 10-year mark. Abbreviations: DMEK = Descemet membrane endothelial keratoplasty, PK = penetrating keratoplasty, AS-OCT = anterior segment optical coherence tomography, FECD = Fuchs endothelial corneal dystrophy, BCVA = best corrected visual acuity, US = ultrasound, CDE = cumulative dissipated energy, IOL = intraocular lens.

Risk factors for early graft detachment requiring rebubbling in Descemet membrane endothelial keratoplasty with imported pre-cut donor tissues.

Graft detachment is a common and significant complication in Descemet membrane endothelial keratoplasty (DMEK). We investigated the risk factors of graft detachment requiring rebubbling after DMEK using imported pre-cut donor tissues. The medical records of 48 patients who underwent DMEK for Fuchs' endothelial dystrophy (FED) or bullous keratopathy (BK) at Seoul National University Hospital were retrospectively reviewed. Donor, recipient, and surgical factors were evaluated using univariate and multivariate logistic regression models. Graft detachment requiring rebubbling occurred in 17 (32.7%) eyes. The detachment group exhibited older recipient age (p = 0.006), higher prevalence of diabetes (p = 0.001), and a higher proportion of FED (65%, p = 0.003). Notably, the detachment group demonstrated a significantly lower postoperative 2-h intraocular pressure (IOP) (p = 0.002) and a greater proportion of eyes with IOP <20 mmHg (p < 0.001). Older recipient age (OR 1.08, 95% CI 1.02-1.17), diabetes (OR 23.8, 95% CI 2.61-217), FED surgical indication (OR 6.19, 95% CI 1.74-22.0), lower postoperative 2-h IOP (OR 1.21, 95% CI 1.06-1.38), and postoperative 2-h IOP <20 mmHg (OR 14.0, 95% CI 1.64-119) were associated with increased odds of graft detachment. According to multivariate logistic regression, lower postoperative 2-h IOP (OR 1.23, 95% CI 1.02-1.47) and postoperative 2-h IOP <20 mmHg (OR 25.1, 95% CI 1.05-602) increased the risk of graft detachment. Lower postoperative 2-h IOP, particularly below 20 mmHg, may increase the risk of graft detachment, and diabetes in recipients may pose a higher risk of graft detachment after DMEK.

Traducción y validación del cuestionario Visual Function and Corneal Health Status (V-FUCHS) al idioma castellano / Translation and validation of the Visual Function and Corneal Health Status (V-FUCHS) questionnaire into Spanish language

Objetivo: Traducir y validar al castellano el cuestionario V-FUCHS en una población de pacientes que padecen distrofia endotelial de Fuchs (DEF).MétodosEl V-FUCHS consta de 15 preguntas, que valoran aspectos visuales de la calidad de vida en los pacientes con DEF, las cuales se pueden agrupar en 7 que valoran el factor «dificultad visual» y 8 que valoran el factor «deslumbramiento». Para la traducción y adaptación se siguieron las normas estandarizadas, destacando, una traducción, una retro-traducción y una aplicación en pacientes con DEF.ResultadosEn una primera fase se llegó al consenso de la traducción al castellano del V-FUCHS. Posteriormente, se incluyeron 25 pacientes para realizar la fase pre-test con el objetivo de valorar la aplicabilidad y la viabilidad del test. La puntuación de las mismas obtuvo un valor mínimo de −0,88 y un valor máximo de +2,44, según la escala probabilística de Rasch. El valor medio obtenido de las preguntas que conforman el factor dificultad visual fue de 0,61 (±0,71) y la media del factor deslumbramiento (Glare) fue de 0,41 (±0,51).ConclusiónLa validación del cuestionario V-FUCHS, tras su traducción y adaptación al castellano, demostró ser una herramienta útil para la valoración de la calidad visual de los pacientes con DEF. Los pacientes con un estado más avanzado de la enfermedad presentaron una mayor severidad en el resultado de la prueba. Asimismo, el factor deslumbramiento se correlaciona mejor con el aumento paquimétrico que con la agudeza visual del paciente. (AU)

Purpose: To translate and validate the V-FUCHS questionnaire into Spanish in a population of patients with Fuchs endothelial dystrophy (DEF).MethodsThe V-FUCHS consists of 15 short, easily understandable questions that assess visual aspects of quality of life in patients with DEF, which can be gathered into a group of seven that assess the “Visual Difficulty” factor and another group of eight that assess the “Glare Factor”. For the translation and cultural adaptation, the standardized norms for this process were followed, among other phases, a translation, a back-translation and an application in patients with DEF.ResultsIn the first phase, consensus was reached on the Spanish translation of the V-FUCHS. Subsequently, 25 patients were included to carry out the pre-test phase with the aim of assessing the applicability and feasibility of the test. The score obtained a minimum value of −0.88 and a maximum value of +2.44, according to the Rasch probabilistic scale. The mean value obtained from the Visual Difficulty factor was 0.61 (±0.71), while the mean for the Glare factor was 0.41 (±0.51).ConclusionThe validation of the V-FUCHS questionnaire, after its translation and adaptation into Spanish, proved to be a useful tool for assessing the visual quality of patients with DEF. Patients with a more advanced stage of the disease presented a greater severity in the test result. Likewise, the Glare factor (Glare) correlates better with the pachymetric increase than with the visual acuity of the patient. (AU)

Translation and validation of the Visual Function and Corneal Health Status (V-FUCHS) questionnaire into Spanish language.

PURPOSE: To translate and validate the V-FUCHS questionnaire into Spanish in a population of patients with Fuchs endothelial dystrophy (DEF). METHODS: The V-FUCHS consists of 15 short, easily understandable questions that assess visual aspects of quality of life in patients with DEF, which can be gathered into a group of seven that assess the "Visual Difficulty" factor and another group of eight that assess the "Glare Factor". For the translation and cultural adaptation, the standardized norms for this process were followed, among other phases, a translation, a back-translation and an application in patients with DEF. RESULTS: In the first phase, consensus was reached on the Spanish translation of the V-FUCHS. Subsequently, 25 patients were included to carry out the pre-test phase with the aim of assessing the applicability and feasibility of the test. The score obtained a minimum value of -0.88 and a maximum value of +2.44, according to the Rasch probabilistic scale. The mean value obtained from the Visual Difficulty factor was 0.61 (±0.71), while the mean for the Glare Factor was 0.41 (±0.51). CONCLUSION: The validation of the V-FUCHS questionnaire, after its translation and adaptation into Spanish, proved to be a useful tool for assessing the visual quality of patients with DEF. Patients with a more advanced stage of the disease presented a greater severity in the test result. Likewise, the Glare Factor (Glare) correlates better with the pachymetric increase than with the visual acuity of the patient.

Phacoemulsification, pinhole pupilloplasty, and pre-Descemet's endothelial keratoplasty for keratoconus with Fuchs' endothelial dystrophy.

The comorbidity of keratoconus with Fuchs' endothelial dystrophy with cataract is a rare clinical combination. We present an amalgamation of surgical techniques to manage the above clinical conditions and its complications in single setting. The modified triple procedure, namely, the phacoemulsification, pinhole pupilloplasty, and pre-Descemet's endothelial keratoplasty (PDEK) in the order of description is followed in single sitting. Lens removal by phacoemulsification, correction of irregular astigmatism by pinhole pupilloplasty (pinhole optics), and exchanging the endothelial layer for PDEK forms the main segments of the triple procedure. This combination of techniques may decrease the risk of multiple surgeries and its related complications. Moreover, it will allow the patient for faster visual rehabilitation by improving the uncorrected visual acuity and visual quality.

Unmasking of subclinical keratoconus with Descemet membrane endothelial keratoplasty in Fuchs endothelial dystrophy.

We report a case of Fuchs endothelial corneal dystrophy (FECD) with concurrent forme fruste keratoconus (KCN) that was unmasked with Descemet membrane endothelial keratoplasty (DMEK) in the right eye, but not with Descemet-stripping automated endothelial keratoplasty (DSAEK) in the left eye. The patient was a 65-year-old female with FECD who underwent uncomplicated combination cataract surgery and DMEK in the right eye. She subsequently developed intractable monocular diplopia associated with inferior displacement of the thinnest point of the cornea and subtle steepening noted on posterior corneal curvature on Scheimpflug tomography. The patient was diagnosed with forme fruste KCN. Altering the surgical plan to combine cataract surgery and DSAEK in the left eye successfully circumvented the development of symptomatic visual distortion. This is the first case providing comparable data from contralateral eyes in the same patient regarding the outcome of DMEK versus DSAEK in eyes with concurrent forme fruste KCN. DMEK appeared to unmask posterior corneal irregularities and resulted in visual distortion, whereas DSAEK did not. The additional stromal tissue in DSAEK grafts appears to help normalize alterations of the posterior corneal curvature and may be the preferred endothelial keratoplasty for patients with concurrent mild KCN.

Evaluation of scleral thickness in patients with Fuchs endothelial dystrophy.

PURPOSE: To evaluate scleral thickness using anterior segment-optical coherence tomography (AS-OCT) in Fuchs endothelial dystrophy (FED) and compare the results with healthy individuals. METHODS: Thirty-two eyes of 32 patients with FED and 30 eyes of 30 age, gender, spherical equivalent and axial length matched healthy participants were included. All subjects underwent a detailed ophthalmological examination including endothelial cell density and central corneal thickness (CCT) measurements. Scleral thickness was measured by AS-OCT (Swept Source-OCT, Triton,Topcon,Japan) in 4 quadrants (superior, inferior, nasal, temporal) from 6 mm posterior to the scleral spur. RESULTS: The mean ages were 62.5 ± 13.2 (33-81) for FED group; 64 ± 8.1 (48-81) for control group. CCT was significantly greater in FED group than in the control group (586.8 ± 33.1 (514-635) vs 545.0 ± 20.7 (503-587), respectively)(p = 0.000). In FED group, mean scleral thickness in the superior, inferior, nasal and temporal quadrants were 434.0 ± 30.6 (371-498), 442.8 ± 27.6 (395-502), 447.7 ± 31.4 (382-502), 443.4 ± 30.3 (386-504) µm, respectively. In control group, the mean scleral thickness in the superior, inferior, nasal and temporal quadrants were 381.3 ± 20.0 (341-436), 383.2 ± 16.0 (352-436), 389.2 ± 21.0 (353-440), 383.2 ± 19.2 (349-440) µm, respectively. The mean scleral thickness was significantly higher in all quadrants in FED group than in control group (p = 0.000). CONCLUSION: In patients with FED, scleral thickness was significantly higher. FED is a progressive corneal disease that results in the accumulation of extracellular material in the cornea. These findings suggest that the accumulation of extracellular deposits may not be limited to the cornea. Due to their functional similarity and anatomical proximity, sclera may also be affected in FED.

Outcomes of Descemet's membrane endothelial keratoplasty performed in combination with, before, or after cataract surgery in Fuchs' endothelial dystrophy: A review of the literature and meta-analysis.

This review aimed to compare the outcomes of Descemet's membrane endothelial keratoplasty (DMEK) in combination with (category 1), before (category 2), or after cataract surgery (category 3) in patients with Fuchs' endothelial dystrophy (FED). Primary outcome was gain in best-corrected log of minimum angle of resolution (logMAR) visual acuity (BCVA). Secondary outcomes were graft detachment, rebubbling rates, rejection, failure, and endothelial cell loss (ECL). In category 1, 2, and 3, 12 studies (N = 1932) were included (five in category 1 [n = 696], one in category 2 [n = 286], and two in category 3 [n = 950], and the remaining four compared between two of the three categories). At 6 months, the gain in BCVA was 0.34 ± 0.04, 0.25 ± 0.03, and 0.38 ± 0.03 logMAR in category 1, 2, and 3, respectively. The difference was significant between categories 1 and 2 (Chi2 = 11.47, P < 0.01) and categories 2 and 3 (Chi2 = 35.53, P < 0.01). At 12 months, the gain in BCVA was 0.52 ± 0.05 and 0.38 ± 0.06 logMAR in categories 1 & 3 (Chi2 = 14.04, P < 0.01). The rebubbling rates were 15%, 4%, and 10% (P < 0.01) and the graft detachment rates were 31%, 8%, and 13% (P < 0.01) in categories 1, 2, and 3, respectively. However, graft rejection, survival rates, and ECL at 12 months were not different between categories 1 and 3. There is low certainty evidence that gain in BCVA in category 1 was comparable to category 3 at 6 months; however, it was significantly better with category 3 at 12 months. Although rebubbling and graft detachment rates were highest in category 1, there was no significant difference in graft rejection, survival rates, and ECL. Further high-quality studies are likely to change the effect estimate and have an impact on the confidence of the estimate.

Fuchs Dystrophy and Cataract: Diagnosis, Evaluation and Treatment.

Corneal endothelium plays an important role in maintaining hydration homeostasis and clarity of the cornea. Fuchs endothelial corneal dystrophy (FECD) affects the corneal endothelium resulting in edema and characteristic excrescences on the Descemet's membrane known as corneal guttae. Descemet membrane endothelial keratoplasty (DMEK) has evolved to become the standard of care for patients with FECD with excellent visual acuity outcomes. Patients with FECD may have coexisting cataracts and therefore may require a cataract surgery, which increases the risk of corneal decompensation. The presence of FECD may not only influence the choice of intraocular lens but vision outcomes can also be affected by the corneal condition. The ability to combine the surgeries further raises important considerations regarding the timing and sequence of DMEK and cataract extraction for patients with FECD. This review provides a guide for corneal surgeons in choosing between endothelial keratoplasty and cataract surgery-alone, in combination or sequential-in their management of patients with FECD.

The soil and the seed: The relationship between Descemet's membrane and the corneal endothelium.

Descemet's membrane (DM), the basement membrane of the corneal endothelium, is formed from the extracellular matrix (ECM) secreted by corneal endothelial cells. The ECM supports the growth and function of the corneal endothelial cells. Changes to DM are central to the diagnosis of the most common corneal endothelial disease, Fuchs endothelial corneal dystrophy (FECD). Changes in DM are also noted in systemic diseases such as diabetes mellitus. In FECD, the DM progressively accumulates guttae, "drop-like deposits" that disrupt the corneal endothelial cell monolayer. While the pathophysiologic changes to corneal endothelial cells in the course of FECD have been well described and reviewed, the changes to DM have received limited attention. The reciprocity of influence between the corneal endothelial cells and DM demands full attention to the latter in our search for novel treatment and preventive strategies. In this review, we discuss what is known about the formation and composition of DM and how it changes in FECD and other conditions. We review characteristics of guttae and the interplay between corneal endothelial cells and guttae, particularly as it might apply to future cell-based and genetic therapies for FECD.

The effect of anterior segment parameters on surgical success of Descemet membrane endothelial keratoplasty: a prospective study.

PURPOSE: To investigate how anterior segment parameters affect the success of Descemet membrane endothelial keratoplasty (DMEK) in patients with endothelial insufficiency without stromal opacities and to identify factors predisposing them to repeat keratoplasty. METHODS: Our prospective observational study included patients who underwent primary DMEK for Fuchs' endothelial dystrophy and pseudophakic bullous keratopathy between March 2019 and March 2020. After a detailed ophthalmologic examination, corneal topographic analysis, anterior segment optical coherence tomography (AS-OCT) scans, endothelial cell density (ECD) and axial length (AL) measurements were performed. Surgical success was defined as no endothelial dysfunction during follow-up. Changes in the parameters and how preoperative parameters affect surgical success were investigated. RESULTS: In 43 eyes of 43 patients (mean age: 68.58 ± 8.51 years), mean visual acuity was 1.73 ± 0.74 LogMAR before DMEK and 0.31 ± 0.29 LogMAR in the final visit. Central corneal thickness (CCT, p = 0.026) and mean anterior chamber depth (ACD, p = 0.001) affected surgical success. Preoperative CCT values greater than 770 µm increased the risk of repeat keratoplasty by 8.75-fold, whereas preoperative ACD values less than 2.5 mm increased the risk by 2.92-fold. CONCLUSION: Preoperative higher CCT and lower ACD values were associated with surgical failure after DMEK. Early surgery may be considered for more successful results. TRIAL REGISTRATION: This prospective study has been registered on the ClinicalTrials.gov system with the registration number NCT04420429 on 06/06/2020.

Predictive factors of graft detachment and rebubbling after descemet membrane endothelial keratoplasty.

PURPOSE: To identify risk and predictive factors associated with the need of rebubbling in the eye of patients who underwent a descemet membrane endothelial keratoplasty (DMEK). METHODS: The records of patients who underwent DMEK were retrospectively analyzed. Data regarding comorbidities, intraoperative characteristics, and postoperative treatments or complications were collected. The central corneal thickness (CCT) was measured by optical coherence tomography before and the day after DMEK. Univariate and multivariate analyses were performed. RESULTS: Of the 333 DMEK, rebubbling was performed in 119 cases (36%). Preoperative subepithelial fibrosis and a history of penetrating keratoplasty (PK) were associated with significantly more graft detachment [OR of 3.55 (2.02-6.32; P < 0.001) and 5.89 (2.00-21.86; P = 0.003), respectively]. A decreased CCT the day after surgery reduced by 5.7-fold the risk of rebubbling (sensitivity/specificity of 0.42/0.93). Conversely, a 20% increase in the CCT the day after surgery increased by 4.5-fold the risk of rebubbling (sensitivity/specificity of 0.42/0.91). CONCLUSION: Variation of the CCT could be used as a predictive factor of rebubbling after DMEK. Patients with a 20% increase of CCT the day after surgery are at higher risk of graft detachment. Conversely, a reduced CCT the day after the surgery is associated with a reduced risk of rebubbling. Subepithelial fibrosis and history of PK were also identified as risk factors for rebubbling. Those predictive factors may help develop a customized approach for patients undergoing DMEK surgery.

Trial-based cost-effectiveness analysis of Descemet membrane endothelial keratoplasty (DMEK) versus ultrathin Descemet stripping automated endothelial keratoplasty (UT-DSAEK).

PURPOSE: To evaluate the cost-effectiveness of Descemet Membrane Endothelial Keratoplasy (DMEK) versus Ultrathin Descemet Stripping Automated Endothelial Keratoplasty (UT-DSAEK). METHODS: A cost-effectiveness analysis using data from a multicenter randomized clinical trial was performed. The time horizon was 12 months postoperatively. Patients with Fuchs' endothelial dystrophy were randomized to DMEK (n = 29) or UT-DSAEK (n = 24). Relevant resources from healthcare and societal perspectives were included in the cost analysis. Quality-Adjusted Life Years (QALYs) were determined using the Health Utilities Index Mark 3 (HUI3) and the EuroQol EQ-5D-5L questionnaires. The main outcome was the incremental cost-effectiveness ratio (ICER; incremental societal costs per QALY). RESULTS: Societal costs averaged €8851 (US$11 406) for DMEK and €8320 (US$10 722) for UT-DSAEK. Higher costs in the DMEK group were mainly caused by higher rebubbling and regraft rates (21% and 7%, vs. 4% and 0% in the UT-DSAEK group). HUI3 QALYs were 0.70 (DMEK) and 0.79 (UT-DSAEK). EQ-5D-5L QALYs were 0.83 (DMEK) and 0.86 (UT-DSAEK). The ICER indicated DMEK was dominated by UT-DSAEK in both analyses. The cost-effectiveness probability for DMEK ranged from 21% to 5% (HUI3 QALYs) and 27%-14% (EQ-5D-5L QALYs), assuming the maximum acceptable ICER ranged from €2500 to €80.000 (US$3222-US$103 093) per QALY. CONCLUSION: The base case cost-effectiveness analysis favoured UT-DSAEK over DMEK, as costs of DMEK were higher while QALYs were lower. Further studies are required to assess long-term rebubbling and regraft rates and graft survival.

Descemet's Stripping Automated Endothelial Keratoplasty Using Donor Tissue from an 83-Day-Old Infant.

Purpose: To report a case of Descemet stripping automated endothelial keratoplasty (DSAEK) combined with phacoemulsification in an adult recipient using endothelial graft from an 83-day-old infant donor. Methods: A corneoscleral button was obtained from an infant donor and a DSAEK graft was prepared using a microkeratome. In comparison to the standard technique of DSAEK graft preparation some modifications were made in order to avoid inadvertent perforation, as the donor cornea had a very spherical shape, probably due to the very young age of the donor. The DSAEK graft was transplanted to the left eye of a 68-year-old woman suffering from Fuchs' endothelial dystrophy. Her preoperative best-corrected visual acuity (BCVA) in that eye was 20/100 and central corneal thickness 831 µm. Results: An uneventful DSAEK combined with phacoemulsification was performed. The main complication noted was detachment of the peripheral part and contraction of the corneal graft, observed two months after the procedure. The implanted tissue remained centrally attached with a BCVA of 20/40, 3 years postoperatively. Conclusion: This case report highlights the difficulties emerging from preparation and implantation using an endothelial graft tissue from the youngest ever reported donor.

Clinical profile and demographic distribution of Fuchs' endothelial dystrophy: An electronic medical record-driven big data analytics from an eye care network in India.

Purpose: To describe the demographics and clinical profile of Fuchs' endothelial corneal dystrophy (FECD) in patients presenting to a multi-tiered ophthalmology hospital network in India. Methods: This cross-sectional hospital-based study included 3,082,727 new patients presenting between August 2010 and December 2021. Patients with a clinical diagnosis of FECD in at least one eye were included as cases. The data were collected using an electronic medical record system. Results: Overall, 2570 (0.08%) patients were diagnosed with FECD. The majority of the patients were female (65.53%) and were predominantly adults (99.92%). The most common age group at presentation was during the seventh decade of life with 867 patients (33.74%). The overall prevalence was higher in patients from a higher socioeconomic status (0.1%) presenting from the urban geography (0.09%) and in retired individuals (0.4%). About half of the 5,140 eyes had mild or no visual impairment (< 20/70) in 2643 eyes (51.42%) followed by moderate visual impairment (>20/70 to 20/200) in 708 eyes (13.77%). The average logMAR was 0.61 ± 0.81 at presentation. The most documented corneal signs were guttae (76.63%), corneal scar (23%) and stromal edema (21.73%). The most associated ocular comorbidity was cataract (47.32%) followed by glaucoma (5.39%). More than a tenth of the affected eyes required a surgical intervention of endothelial keratoplasty (15.58%). Conclusion: FECD more commonly affects females presenting during the seventh decade of life. Majority of the eyes had mild or no visual impairment and endothelial keratoplasty is warranted in a tenth of the affected eyes.

The Sociodemographic and Risk Factors for Fuchs' Endothelial Dystrophy: A Nationwide, Matched Case-Control Study in Taiwan.

This nationwide, population-based, retrospective, matched case-control study included 4334 newly diagnosed Fuchs' endothelial dystrophy (FED) patients who were identified by the International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM), code 371.57, and selected from the Taiwan National Health Insurance Research Database. The age-, sex-, and index-date-matched control group included 4334 non-FED controls selected from the Taiwan Longitudinal Health Insurance Database 2000. Ocular allergic conditions and sociodemographic conditions were examined using univariate logistic regression analyses and paired t-test was used for continuous variables. Adjusted logistic regression was used to compare the odds ratio (OR) of the FED development. Patients with ocular allergic conditions were more likely to have FED than the controls (OR = 25.50, 95% CI = 12.58-51.68, p < 0.0001) even after conditional logistic regression was conducted (adjusted OR = 25.26, 95% CI = 11.24-56.77, p < 0.0001). Regarding the sociodemographic factors, we found that more than half of the FED patients in Taiwan were aged ≥45 years old, there was an equal female-to-male ratio (1.06:1), and patients with a lower income and living in northern Taiwan had higher odds of developing FED. The results strongly support an association between ocular allergic conditions, geographic region, residential status, income, and FED.

Visual Function in Pseudophakic Eyes with Fuchs' Endothelial Corneal Dystrophy.

PURPOSE: To assess vision in pseudophakic eyes with Fuchs' endothelial corneal dystrophy (FECD) before the onset of clinically detectable corneal edema. DESIGN: Cross-sectional study. METHODS: Sixty-one otherwise healthy pseudophakic eyes of 38 subjects with FECD (without clinically detectable edema) and 17 otherwise healthy pseudophakic eyes of 9 subjects with normal corneas. Subjects underwent clinical examination to determine the morphologic distribution of guttae (severity grading). Standardized best-corrected high-contract and low-contrast (photopic and mesopic) visual acuity (HCVA, LCVA) and straylight were measured. Scheimpflug tomography posterior elevation and pachymetry maps were interpreted for 3 tomographic features of subclinical edema: loss of regular isopachs, displacement of the thinnest point of the cornea, and presence of posterior surface depression. RESULTS: In FECD without tomographic features of edema (ie, normal tomography patterns), HCVA, LCVA, and straylight did not differ from that of eyes with normal corneas (P ≥ .09); these eyes encompassed the full range of severity grading of guttae. In FECD with all 3 tomographic features of edema, the same parameters were worse compared with eyes with normal corneas (P ≤ .02). In FECD with 1 or 2 tomographic abnormalities, mesopic LCVA (P = .04) and straylight (P = .003) were worse compared with eyes with normal corneas. CONCLUSIONS: Impairment of vision was associated with the presence of tomographic edema in eyes with FECD. When tomography patterns were normal in FECD (ie, guttae were present without tomographic edema), visual acuity and straylight were normal, and therefore corneal surgical intervention would not typically be indicated to improve vision.

Influence of time to procurement, incubation and release of organ cultured donor corneas on graft failure after Descemet Stripping Automated Endothelial Keratoplasty.

PURPOSE: The aim of the present study was to investigate whether the time from death to procurement, to preservation or the storage time of donor corneas preserved in organ culture influenced the clinical outcome of patients undergoing Descemet stripping automated endothelial keratoplasty (DSAEK) for Fuchs endothelial keratoplasty. METHODS: We conducted a registry-based study on 776 patients undergoing DSAEK. Data on time from donor death to cornea retrieval (DRT), time from death to preservation (DPT), the preservation time and donor cornea characteristics: age, sex and endothelial cell density (ECD) at the time of release for surgery, were extracted from The Danish Cornea Bank Registry. Data on recipient follow-up were collected from a corneal graft registry. The primary outcome was presence of graft failure within a period from 2 months to 2 years after surgery. Secondary outcomes were DRT, DPT, ECD ≤2300 and gender mismatch between donor and recipient. RESULTS: Graft failure occurred in 26 patients. The mean preservation time for failed grafts was 34.1 ± 10.0 days (mean ± SD) and 27.3 ± 10.6 days (mean ± SD) for the clear, functional grafts at the 2-year follow-up. A preservation time of >29 days compared with ≤29 days was associated with a lower survival (HR 2.33, 95% CI on 1.06-5.14, p = 0.036) and an increased risk of graft failure (RR 1.53, 95% CI on 1.11-2.10, p = 0.009). For the secondary outcome variables, no difference in the risk of graft failure was observed and did not appear to impact the survival rate of DSAEK patients. CONCLUSION: Preservation time of donor cornea was associated with graft survival and a prolonged preservation time of more than 4 weeks seemed to lower the 2-year survival.

Translational and Regulatory Challenges of Corneal Endothelial Cell Therapy: A Global Perspective.

Cell therapies are emerging as a unique class of clinical therapeutics in medicine. In 2015, Holoclar (ex vivo expanded autologous human corneal epithelial cells containing stem cells) gained the regulatory approval for treating limbal stem cell deficiency after chemical eye burn. This has set a precedent in ophthalmology and in medicine, reinforcing the therapeutic promise of cell therapy. However, to generalize and commercialize cell therapies on a global scale, stringent translational and regulatory requirements need to be fulfilled at both local and international levels. Over the past decade, the Singapore group has taken significant steps in developing human corneal endothelial cell (HCEnC) therapy for treating corneal endothelial diseases, which are currently the leading indication for corneal transplantation in many countries. Successful development of HCEnC therapy may serve as a novel solution to the current global shortage of donor corneas. Based on the experience in Singapore, this review aims to provide a global perspective on the translational and regulatory challenges for bench-to-bedside translation of cell therapy. Specifically, we discussed about the characterization of the critical quality attributes (CQA), the challenges that can affect the CQA, and the variations in the regulatory framework embedded within different regions, including Singapore, Europe, and the United States. Impact statement Functional corneal endothelium is critical to normal vision. Corneal endothelial disease-secondary to trauma, surgery, or pathology-represents an important cause of visual impairment and blindness in both developed and developing countries. Currently, corneal transplantation serves as the current gold standard for treating visually significant corneal endothelial diseases, although limited by the shortage of donor corneas. Over the past decade, human corneal endothelial cell therapy has emerged as a promising treatment option for treating corneal endothelial diseases. To allow widespread application of this therapy, significant regulatory challenges will need to be systematically overcome.

Impact of DMEK on visual quality in patients with Fuchs' endothelial dystrophy.

PURPOSE: To investigate short-term (3 months follow-up) changes in visual quality following Descemet membrane endothelial keratoplasty (DMEK) for Fuchs endothelial dystrophy (FED). METHODS: In this prospective institutional case series, 51 patients that underwent DMEK for FED were included. Assessment included the Quality of Vision (QoV) questionnaire preoperatively, at 1 month, and 3 months after surgery. Secondary outcome measures were anterior segment parameters acquired by Scheimpflug imaging, corrected distance visual acuity (CDVA), and endothelial cell density (ECD). RESULTS: Glare, hazy vision, blurred vision, and daily fluctuation in vision were the symptoms mostly reported preoperatively. All symptoms demonstrated a significant reduction of item scores for severity, frequency, and bothersome in the course after DMEK (P < 0.01). Glare and fluctuation in vision remained to some extent during the follow-up period (median score = 1). Preoperatively, corneal densitometry correlated moderately to weakly with severity of hazy vision (rs = 0.39; P = 0.03) and frequency (rs = 0.26; P = 0.02) as well as severity (rs = 0.27; P = 0.03) of blurry vision. CDVA and central corneal thickness (CCT) did not correlate with visual complains. CONCLUSIONS: Following DMEK for FED, patient-reported visual symptoms assessed by the QoV questionnaire represent a useful tool providing valuable information on the impact of DMEK on visual quality that cannot be directly estimated by morphological parameters and visual acuity only.