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To assess vision in pseudophakic eyes with Fuchs' endothelial corneal dystrophy (FECD) before the onset of clinically detectable corneal edema.Cross-sectional study.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.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.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.

The goal of this study was to compare outcomes of Descemet stripping endothelial keratoplasty (DSEK) in eyes with glaucoma and abnormal anatomy to eyes with Fuchs endothelial corneal dystrophy (FECD).In a retrospective interventional series of all cases of DSEK between April 1, 2006, and November 30, 2015, recipient diagnosis, preoperative glaucoma status, concurrent surgical procedures, and graft outcomes were recorded. Graft survival, risk of rejection, and subsequent glaucoma surgery were estimated by using Kaplan-Meier analysis with risk factors determined by proportional hazard models.Of 703 DSEKs in 666 eyes (509 subjects), the main recipient diagnoses were FECD (n = 496), pseudophakic corneal edema (n = 112), and failed graft (n = 83). Glaucoma was present in 150 cases before DSEK. Overall graft survival was 85%, 75%, and 71% at 5, 10, and 12 years, respectively, and for FECD without glaucoma was 95%, 89%, and 87% at 5, 10, and 12 years, respectively. Independent risk factors for graft failure included recipient diagnoses of pseudophakic corneal edema (HR = 8.3, P0.001), failed graft (HR = 6.4, P0.001), and preoperative medical glaucoma (HR = 7.1, P0.001) or surgical glaucoma (HR = 12.3, P0.001). Preoperative glaucoma treated by previous surgery resulted in graft survival of 28% at 10 years. Preoperative glaucoma was associated with an increased risk of graft rejection (HR = 6.8, P0.001) and subsequent glaucoma surgery (HR17.4, P0.001).Preoperative glaucoma increases the risk of graft failure, graft rejection, and needing subsequent glaucoma surgery in the first decade after DSEK. With previous glaucoma surgery, DSEK graft survival was more favorable compared with published reports of Descemet membrane endothelial keratoplasty.

Oleander is a poisonous plant with extensively documented systemic side effects; however, oleander's ophthalmic side effects have not been detailed in the literature. We report a case of oleander-associated keratitis with subsequent corneal edema and anterior uveitis.This is a case report and review of relevant literature.A 58-year-old woman presented with large corneal epithelial defect after being struck in the eye with an oleander leaf. Despite treatment with topical moxifloxacin, she developed severe corneal edema and anterior uveitis. A diagnosis of oleander-associated ocular inflammation with secondary corneal edema was made, given the temporal relationship, and treatment was initiated with topical prednisolone and cyclopentolate. However, the corneal edema and inflammation continued to progress until oral prednisone and topical difluprednate were initiated. Visual acuity, anterior uveitis, and corneal edema significantly improved with aggressive immunomodulation. Follow-up at 1 month confirmed complete recovery of symptoms, corneal edema and anterior uveitis.Severe corneal edema and anterior uveitis can be associated with oleander exposure. Aggressive treatment with oral and topical steroids may be required without persistent sequelae at the 5-month follow-up. Ophthalmologists should consider this inflammatory reaction if patients experience ocular exposure to oleander.

Purpose To determine if Scheimpflug tomography pachymetry map and posterior elevation map patterns, central corneal thickness (CCT), and corneal backscatter can predict the prognosis of Fuchs endothelial corneal dystrophy (FECD). Design Cross-sectional study with follow-up of outcomes. Participants Ninety-six eyes (56 subjects) with a range of severity of FECD. Methods Corneas were graded by cornea specialists according to the area and confluence of guttae and the presence of clinically definite edema. Masked and randomized Scheimpflug imaging pachymetry map and posterior elevation map patterns were assessed by 1 observer for loss of regular isopachs, displacement of the thinnest point of the cornea, and the presence of posterior surface depression. The prognosis of eyes over a 5-year (median) follow-up period was determined based on FECD progression (new onset of clinically definite edema or ≥5% increase in CCT) or intervention by endothelial keratoplasty. Cumulative probabilities of progression and intervention were estimated from survival analyses, with risk factors determined by using Cox proportional hazards models. Main Outcome Measures Pachymetry map and posterior elevation map patterns, corneal backscatter, and CCT (ultrasonic pachymetry). Results In univariate analyses, loss of regular isopachs (hazard ratio [HR], 18.00) displacement of the thinnest point (HR, 11.53), focal posterior surface depression (HR, 10.21), and anterior corneal backscatter (HR, 1.22, per 1-grayscale unit increment), were risk factors for progression or intervention (P Conclusions Three Scheimpflug tomography pachymetry map and posterior elevation map patterns can predict FECD prognosis independent of CCT. The risk of FECD progression and intervention, including after uncomplicated cataract surgery, increases according to the number of parameters present.

Expansion of CTG trinucleotide repeats (TNR) in the transcription factor 4 (TCF4) gene is highly associated with Fuchs Endothelial Corneal Dystrophy (FECD). Due to limitations in the availability of DNA from diseased corneal endothelium, sizing of CTG repeats in FECD patients has typically been determined using DNA samples isolated from peripheral blood leukocytes. However, it is non-feasible to extract enough DNA from surgically isolated FECD corneal endothelial tissue to determine repeat length based on current technology. To circumvent this issue, total RNA was isolated from FECD corneal endothelium and sequenced using long-read sequencing. Southern blotting of DNA samples isolated from primary cultures of corneal endothelium from these same affected individuals was also assessed. Both long read sequencing and Southern blot analysis showed significantly longer CTG TNR expansion (>1000 repeats) in the corneal endothelium from FECD patients than those characterized in leukocytes from the same individuals (TCF4 CTG repeat expansions in the FECD corneal endothelium are much longer than those found in leukocytes.

The purpose of this study is to quantify cancer risk in patients with Fuchs endothelial corneal dystrophy (FECD).Using the 2014 to 2016 Medicare Limited 5% Data Sets-Carrier Line File, US Medicare fee-for-service beneficiaries (aged 65 years or older) with FECD and cancer were identified through International Classification of Diseases , ninth and 10th Revision diagnostic codes from January 1, 2014, to December 31, 2016. The main outcome measures were odds ratios (ORs) of cancer at various anatomic locations in patients with versus without FECD.Of the 1,462,740 Medicare beneficiaries, 15,534 patients (1.1%) had an International Classification of Disease code for FECD. Compared with US Medicare beneficiaries without FECD, patients with FECD were at increased risk for the following malignancies: breast [OR: 1.32; 95% confidence interval (CI): 1.22-1.43; P0.001], cutaneous basal cell (OR: 1.42; 95% CI: 1.35-1.49; P0.001), cutaneous melanoma (OR: 1.20; 95% CI: 1.03-1.40; P = 0.02), cutaneous squamous cell (OR: 1.45; 95% CI: 1.38-1.53; P0.001), ovarian (OR: 1.84; 95% CI: 1.48-2.30; P0.001), and thyroid (OR: 1.32; 95% CI: 1.04-1.68; P = 0.02). By contrast, FECD cases were at lower odds of having lung (OR: 0.81; 95% CI: 0.71-0.93; P = 0.003) and prostate cancer diagnoses (OR: 0.88; 95% CI: 0.81-0.96; P = 0.002).Patients with FECD aged 65 years or older may be at increased risk for cancer at several anatomic locations. Follow-up studies are needed to further explore the association of FECD and malignancy, elucidate potential disease mechanisms, and identify genetic and/or environmental risk factors.

PURPOSE To investigate the association of body mass index (BMI) with Fuchs endothelial corneal dystrophy (FECD) severity and TCF4 CTG18.1 expansion. METHODS A total of 343 patients with FECD were enrolled from the Mayo Clinic. FECD severity was graded by slit-lamp biomicroscopy. BMI values were obtained from the electronic medical records. DNA extracted from leukocytes was analyzed for CTG18.1 expansion length, with ≥40 repeats considered expanded. Wilcoxon signed-rank tests were used to compare FECD grade and CTG18.1 expansion length in patients by BMI (

The Rho kinase inhibitor netarsudil is a recently approved therapeutic option for the management of increased intraocular pressure in the United States. Although phase 3 clinical trials noted corneal changes related to the medication-namely, nonvisually-significant corneal verticillata-descriptions of a unique form of cystic epithelial edema began to surface as netarsudil (and its sister drug ripasudil, approved in Japan) gained widespread use. This series adds 3 new cases and reviews the current literature on this unique side effect.

PURPOSE: To determine if there is an increased incidence rate of post-cataract surgery (pcs) anterior ischemic optic neuropathy (AION) compared to spontaneous AION (sAION). DESIGN: Retrospective, population-based cohort. METHODS: Patients diagnosed with AION from January 1, 1990 through December 31, 2016 while residing in Olmsted County, Minnesota. Patients with cataract surgery preceding AION were included in the pcsAION cohort defined in two ways: AION within two months and AION within one year of cataract surgery. The incidence rates of pcsAION and sAION were compared using Poisson regression models. RESULTS: During the study period, 102 residents developed AION. The median age was 65 years (range, 40-90 years), 44 (43.1%) were female. Twenty of 102 (19.6%) patients had previous cataract surgery, of which 2 and 9 developed AION within 2 months and 1 year of surgery, respectively. The annual incidence rate of pcsAION within two months of surgery (8.6 per 100,000) was not significantly greater than the annual incidence rate of sAION (6.9 per 100,000; p=0.78). However, the annual incidence rate of pcsAION within one year of surgery (38.9 per 100,000) was significantly higher than the incidence rate of sAION (6.5 per 100,000; p

Unraveling the genetic mechanisms of Fuchs endothelial corneal dystrophy has opened new possibilities for future targeted medical therapy of the disease. Until these possibilities mature, regenerative semi-surgical approaches by cell injection or cell sheet transfer could help expand the donor pool, and possibly enable autologous transplantation. Descemet membrane stripping alone and acellular Descemet membrane transfer are more immediate surgical approaches that could be temporary treatments in some patients, though there is a lack of understanding of the factors that predict success for these procedures. Regardless of approach, clinical trials will be necessary, and clinicians should therefore try to standardize their methods of assessing disease severity and the outcomes of intervention.Ein Verständnis der genetischen Grundlagen der Fuchs-Endotheldystrophie eröffnet die Möglichkeit zukünftiger gezielter, nicht operativer Therapien. Bis diese Therapien ausgereift sind, könnten neue regenerative therapeutische Ansätze wie die Injektion von Endothelzellen oder der Transfer kultivierter Endothelzellschichten helfen, die Verfügbarkeit von Donorgewebe zu verbessern und vielleicht sogar autologe Transplantationen zu ermöglichen. Eher temporäre operative Ansätze für ausgewählte Patienten sind das alleinige Stripping und der azelluläre Transfer der Descemet-Membran, obgleich unklar ist, welche Patienten am meisten von diesen Verfahren profitieren können. Für alle Behandlungsverfahren werden klinische Studien notwendig sein, weshalb Kliniker anstreben sollten, die Bestimmung des Schweregrads der Fuchs-Endotheldystrophie und der postoperativen Erfolgsparameter zu standardisieren.

Primary cultures of human corneal endothelial cells (HCECs) are an important model system for studying the pathophysiology of corneal endothelium. The purpose of this study was to identify and validate an optimal primary culture model of normal and Fuchs endothelial corneal dystrophy (FECD) endothelial cells by comparing cell morphology and marker expression under different media conditions to in vivo donor tissues. Primary and immortalized HCECs, isolated from normal and FECD donors, were cultured in proliferation media (Joyce, M4, Bartakova) alone or sequentially with maturation media (F99, Stabilization 1, M5). CD56, CD73 and CD166 expressions were quantified in confluent and matured cell lines by flow cytometry. HCECs that were allowed to proliferate in Joyce’s medium followed by maturation in low-mitogen containing media yielded cells with similar morphology to corneal endothelial tissues. Elevated expression of CD56 and CD166 and low expression of CD73 correlated with regular, hexagonal-like HCEC morphology. CD56:CD73 > 2.5 was most consistent with normal HCEC morphology and mimicked corneal endothelial tissue. Immortalization of normal HCECs by hTERT transduction showed morphology and CD56:CD73 ratios similar to parental cell lines. HCECs established from FECD donors showed reduced CD56:CD73 ratios compared to normal HCECs which coincided with reduced uniformity and regularity of cell monolayers. Overall, a dual media system with Joyce’s medium for proliferation and a low-mitogen media for maturation, provided normal cultures with regular, hexagonal-like cell morphologies consistent with corneal endothelial cells in vivo. CD56:CD73 expression ratio >2.5 was predictive of in vivo-like cellular morphology.

Purpose To determine if agreement between subjective interpretations of Scheimpflug tomography maps of corneas with Fuchs endothelial corneal dystrophy (FECD) is affected by daily and hourly changes in corneal edema. Design Reliability analysis. Methods Scheimpflug imaging pachymetry and posterior elevation maps of corneas with a range of severity of FECD were evaluated in a randomized manner for the presence of 3 tomographic features of edema. Agreement between interpretations of 1 masked observer was assessed (percentage, and κ-statistic with 95% confidence interval) for images taken within minutes on the same day, for images taken at a similar time on a different day, and for images taken over the course of a morning. Intra- and interobserver agreement was also assessed. Results Interpretations for individual tomographic features agreed for ≥88% of images (κ ≥ 0.75) taken within minutes on the same day; complete disagreement (ie, disagreement for all 3 tomographic features in an image) occurred in ≤3% of images. Interpretations agreed for ≥77% of images (κ ≥ 0.52) taken at a similar time on a different day; complete disagreement did not occur. Interpretations agreed for ≥81% of images (κ ≥ 0.61) taken over the course of a morning; complete disagreement occurred in ≤6% of images. Intraobserver agreement was ≥93% (κ ≥ 0.83) and interobserver agreement was ≥93% (κ ≥ 0.66); complete disagreement did not occur. Conclusions Subjective interpretation of Scheimpflug images in FECD is highly repeatable for disease classification. Although small variations in interpretations resulted from pathophysiologic changes in corneal hydration and other factors, clinically significant disagreements in interpretation were uncommon and therefore unlikely to affect clinical decision-making.

PReCIS:: Over 10 years in a tertiary care setting, glaucoma drainage devices (GDDs) have not increased as a reason for keratoplasty. PURPOSE The purpose of this study was to determine whether the reasons for keratoplasty have changed between 10 years in a tertiary care setting, with special attention to the rate of GDDs as a reason for keratoplasty. METHODS Patients aged 18 years or above who underwent keratoplasty at Mayo Clinic, Rochester, MN from 2005 to 2006 and 2015 to 2016 were studied. All reasons for keratoplasty performed in the study time period are assessed, including patients who previously had a GDD implanted in the same eye. After analyzing the reasons for keratoplasty, we assess whether the reasons for keratoplasty have changed between 2005 to 2006 and 2015 to 2016 in association with the increasing placement of GDDs. RESULTS The number of keratoplasty procedures performed in the 2 time periods increased by 62% from 163 (2005 to 2006) to 264 (2015 to 2016), whereas GDD placement increased by 164% from 80 GDD (2005 to 2006) to 211 GDD (2015 to 2016). Although the performance of keratoplasty increased between the 2 points in time, the frequency of each cause for keratoplasty did not change significantly. The majority of keratoplasties were performed due to corneal disease, and GDDs made up a small portion of reasons for keratoplasty (2005 to 2006, 4.29%; 2015 to 2016, 5.68%). CONCLUSIONS The frequency of GDDs as a reason for keratoplasty has not changed significantly between 10 years in this tertiary care setting. Patients with GDDs who later required keratoplasty had associated features including multiple surgical procedures and comorbid infection, pseudophakic bullous keratopathy, Fuchs dystrophy, pseudoexfoliation, uveitis, and congenital glaucoma.

PURPOSE: To investigate single nucleotide polymorphisms (SNPs) and trinucleotide repeat (TNR) expansion in the transcription factor 4 (TCF4) gene in a large cohort of German patients with Fuchs endothelial corneal dystrophy (FECD). METHODS: Genomic DNA was obtained from 398 patients with FECD and from 58 non-FECD controls. Thirty-seven previously reported SNPs were evaluated by genotyping. The 398 FECD samples were analyzed for TNR expansions by short tandem repeat (STR) assays and Southern blotting. The possible associations between TNR length and clinical parameters (age, sex, visual acuity, and central corneal thickness) were analyzed in 132 patients. RESULT: The SNPs in COL8A2, TCF8, LOXHD1, and AGBL1 showed no heterogeneity in 36 cases, although SLCA411 showed 3 nonsense mutations. SNPs were detected for TCF4 (rs613872, rs2123392, rs17089887, rs1452787, and rs1348047), but only rs613872 showed a significant association with FECD (P=9.93×10(−12)). Overall, 315/398 (79%) patients harbored TNR lengths >50, while no non-FECD control harbored TNR lengths >50. The TCF4 SNP rs613872 genotype was TT:39 (67%), TG:18 (31%), and GG:1 (2%) in non-FECD controls; TT:39 (47%), TG: 38 (46%), and GG:6 (7%) in FECD cases harboring TNR 50 (P=2.93×10(−25)). No significant association was detected between clinical parameters and TNR length. CONCLUSIONS: Our large German cohort demonstrated a significant association between the risk allele G in rs613872 and FECD, irrespective of TNR expansion, although this risk allele was more frequent in FECD cases with TNR expansion than without.

Fuchs endothelial corneal dystrophy (FECD) is a common cause for heritable visual loss in the elderly. Since the first description of an association between FECD and common polymorphisms situated within the transcription factor 4 (TCF4) gene, genetic and molecular studies have implicated an intronic CTG trinucleotide repeat (CTG18.1) expansion as a causal variant in the majority of FECD patients. To date, several non-mutually exclusive mechanisms have been proposed that drive and/or exacerbate the onset of disease. These mechanisms include (i) TCF4 dysregulation; (ii) toxic gain-of-function from TCF4 repeat-containing RNA; (iii) toxic gain-of-function from repeat-associated non-AUG dependent (RAN) translation; and (iv) somatic instability of CTG18.1. However, the relative contribution of these proposed mechanisms in disease pathogenesis is currently unknown. In this review, we summarise research implicating the repeat expansion in disease pathogenesis, define the phenotype-genotype correlations between FECD and CTG18.1 expansion, and provide an update on research tools that are available to study FECD as a trinucleotide repeat expansion disease. Furthermore, ongoing international research efforts to develop novel CTG18.1 expansion-mediated FECD therapeutics are highlighted and we provide a forward-thinking perspective on key unanswered questions that remain in the field., Highlights • FECD is a common, age-related corneal dystrophy. • The majority of cases are associated with expansion of a CTG repeat (CTG18.1). • FECD is the most common trinucleotide repeat expansion disease in humans. • Evidence supports multiple molecular mechanisms underlying the pathophysiology. • Novel CTG18.1-targeted therapeutics are in development.

Purpose To determine if Scheimpflug tomography can identify subclinical corneal edema in Fuchs endothelial corneal dystrophy (FECD), and to recommend a new classification of FECD for clinical practice and research. Design Cross-sectional study with follow-up of outcomes. Participants Ninety-three eyes from 57 subjects with a range of severity of FECD and 74 eyes from 40 subjects with normal corneas. Methods Corneas were clinically assessed for FECD and corneal edema by using slit-lamp biomicroscopy, and categorized as having clinically definite edema (obvious visible edema), being suspicious for subclinical edema (possible corneal thickening without obvious edema on slit-lamp examination), or not having edema (no clinical suspicion of edema). Tomographic pachymetry and elevation maps derived from Scheimpflug images were evaluated by 3 masked observers for specific features believed to be consistent with corneal edema. FECD clinical disease course and outcomes were reviewed from the time of Scheimpflug image acquisition to the last available follow-up. Main Outcome Measures Presence of tomographic features: (1) loss of parallel isopachs, (2) displacement of the thinnest point of the cornea, and (3) focal posterior corneal surface depression. Clinical outcomes included the change in central corneal thickness (CCT) and vision after endothelial keratoplasty (EK). Results The 3 specific tomographic features were all present in all FECD corneas with clinically definite edema (n = 15), in ≥81% of FECD corneas suspicious for subclinical edema (n = 16), in ≤42% of FECD corneas deemed not to have edema (n = 62), and in ≤5% of normal corneas (n = 74). Corneas suspicious for subclinical edema that subsequently underwent EK (n = 9) all had at least 2 of the tomographic features present before EK, and improvement in vision, CCT, and tomographic features after EK confirmed the presence of subclinical edema preoperatively. Conclusions Subclinical corneal edema in FECD can be detected by Scheimpflug tomography. We recommend classifying FECD corneas as having clinically definite edema (based on slit-lamp examination), subclinical edema (based on tomographic features without clinically definite edema), or no edema (no tomographic or slit-lamp features of edema). This classification is independent of CCT and should be considered when evaluating FECD eyes for cataract surgery or EK.

Measuring patient-reported visual disability in Fuchs' endothelial corneal dystrophy (FECD) may be helpful in determining when to intervene and for understanding the outcomes of intervention. In this study, we aimed to validate a new patient-reported visual disability questionnaire, the Visual Function and Corneal Health Status (V-FUCHS) instrument, in FECD before and after endothelial keratoplasty (EK).Cross-sectional study with instrument readministration at 6 weeks.Patients with a range of severity of FECD, including after EK, and patients with healthy corneas.The V-FUCHS instrument was developed based on patient interviews, expert consultations, and pretesting. Psychometric methods of classic test theory were applied to estimate reliability and validity, including testing V-FUCHS against Catquest-9SF, a cataract-specific visual disability questionnaire. Ordered polytomous Rasch-based partial credit models and item response theory diagnostics were used to define the response patterns.Reliability (primary) and validity (secondary).The final instrument was completed by 65% of patients with a range of severity of FECD (n = 41), after undergoing EK for FECD (n = 70), and with healthy corneas (n = 28). Participants were phakic or pseudophakic. Exploratory factor analysis among FECD and patients who underwent EK showed 2 independent factors of visual disability: 7 items related to visual acuity and 8 items related to glare or diurnal variation. Retest reliability was substantial (intraclass correlation coefficient, ≥0.8, both factors). Among all patients, the visual acuity factor was correlated with Catquest-9SF scores (r = 0.65; P0.001; n = 96). The glare or diurnal variation factor increased with FECD severity (P0.001, n = 69) and was higher in FECD than healthy participants (P 0.001). Diagnostics revealed that both factors were well calibrated and met Rasch model expectations.The V-FUCHS instrument is valid and reliable for assessing visual disability in FECD. The instrument allows for standardized, comprehensive, and rapid assessment of disease-specific visual disability and may be useful in clinical practice and research as a patient-reported outcome measure.

Purpose To determine 5-year outcomes of Descemet stripping endothelial keratoplasty (DSEK) for Fuchs' endothelial corneal dystrophy (FECD). Design Prospective cohort study. Participants Fifty-two eyes of 45 subjects with FECD undergoing primary DSEK. Methods Subjects were examined before and at fixed intervals through 60 months after DSEK. At each visit, graft survival was determined by slit-lamp examination; best spectacle-corrected visual acuity (BSCVA) was measured using the electronic Early Treatment Diabetic Retinopathy Study (ETDRS) protocol; total anterior corneal higher-order aberrations (HOAs) were derived from corneal topography; and corneal backscatter, corneal thickness, and endothelial cell density were measured from confocal microscopy images. Corneal thickness also was measured by ultrasonic pachymetry. Changes after DSEK were analyzed using generalized estimating equation models. Main Outcome Measures Best-corrected visual acuity, HOAs, endothelial cell loss, corneal thickness, and corneal backscatter. Results Complete 60-month follow-up was possible in 34 eyes. Mean BSCVA±standard deviation improved from 0.45±0.19 logarithm of the minimum angle of resolution (logMAR) (Snellen equivalent, 20/56) before DSEK to 0.09±0.13 logMAR (Snellen equivalent, 20/25) at 5 years ( P P P ≤ 0.002). Six grafts failed, of which 4 were primary (iatrogenic); mean endothelial cell loss±standard deviation was 55±15% at 5 years. Conclusions Between 1 and 5 years after DSEK, BSCVA continues to improve such that at 5 years, more than half of eyes see better than 20/25 with a mean total corneal thickness of 700 μm. Improvement in vision is accompanied by continued reduction in corneal haze and aberrations, suggesting ongoing remodeling of the cornea after restoration of endothelial function.

To assess corneal thickness (CT) and correlation with graft outcome after penetrating keratoplasty in the Cornea Donor Study.A total of 887 subjects with a corneal transplant for a moderate-risk condition (principally Fuchs dystrophy or pseudophakic corneal edema) had postoperative CT measurements throughout a 5-year follow-up time. Relationships between baseline (recipient, donor, and operative) factors and CT were explored. Proportional hazards models were used to assess the association between CT and graft failure. Relationship between CT and cell density was assessed with a longitudinal repeated measures model and Spearman correlation estimates.Higher longitudinal CT measurements were associated with the following: diagnosis of pseudophakic or aphakic corneal edema (P0.001), intraocular pressure25 mm Hg during the first postoperative month (P = 0.003), white (non-Hispanic) donor race (P = 0.002), and respiratory causes of donor death (P0.001). Among those without graft failure within the first postoperative year, the 5-year cumulative incidence (± 95% confidence interval) of graft failure was 5% ± 5% in those with a 1-year CT ≤ 500 μm, 5% ± 3% for CT 501 to 550 μm, 7% ± 4% for CT 551 to 600 μm, and 20% ± 11% for CT600 μm. In a multivariate analysis, both 1-year CT and cell density were associated with subsequent graft failure (P = 0.002 and 0.009). CT increase was modestly associated with endothelial cell loss during follow-up (r = -0.29).During the first 5 years after penetrating keratoplasty, CT can serve as a predictor of graft survival. However, CT is not a substitute for cell density measurement because both measures were independently predictive of graft failure.

Purpose To assess interobserver agreement between 2 corneal specialists grading Fuchs' dystrophy clinically and to determine if the corneal central-to-peripheral thickness ratio (CPTR) may be an alternative and objective metric of disease severity. Design Cross-sectional study. Participants Forty-five eyes (26 subjects) with mild and moderate Fuchs' dystrophy, 73 eyes (60 subjects) with advanced Fuchs' dystrophy, and 267 eyes (142 subjects) with normal corneas. Methods Corneas with Fuchs' dystrophy were graded by 2 corneal specialists based on the confluence and area of guttae and the presence or absence of edema. Central corneal thickness (CCT) and peripheral corneal thickness at 4 mm from the center (PCT 4 ) were measured by using scanning-slit pachymetry. The value of CPTR 4 was the quotient of CCT and PCT 4 . Main Outcome Measures Interobserver agreement for clinical grade and CPTR 4 . Results Interobserver agreement for clinical grading of Fuchs' dystrophy was moderate (κ = 0.32; 95% confidence interval, 0.19–0.45). In normal corneas, CCT was not correlated with age ( r = −0.10; P = 0.28; n=267), PCT 4 decreased with age ( r = −0.33; P 4 increased with age ( r = 0.59; P P 4 was higher in Fuchs' dystrophy (650±51 μm; n=107) than in normal corneas (643±43 μm; n=254; P 4 was higher in advanced Fuchs' dystrophy (1.03±0.07; n=65) than in mild and moderate Fuchs' dystrophy (0.95±0.07; n=42; age-adjusted P P 4 was highly correlated with clinical grade of Fuchs' dystrophy ( r = 0.77; P Conclusions Agreement between corneal specialists for the subjective and morphologic clinical grading of Fuchs' dystrophy is only moderate . The corneal CPTR is an objective, repeatable, and possibly functional, metric of severity of Fuchs' dystrophy that warrants further investigation to determine its role in monitoring disease progression and predicting the need for keratoplasty. Financial Disclosure(s) The author(s) have no proprietary or commercial interest in any materials discussed in this article.

To determine corneal sensitivity and evaluate corneal nerves before and after keratoplasty for Fuchs endothelial dystrophy.Central corneal sensitivity, measured by using a Cochet-Bonnet esthesiometer in 69 eyes before and after different keratoplasty procedures for Fuchs dystrophy, was compared with that of 35 age-matched normal corneas. Corneal nerves were qualitatively examined by confocal microscopy in 42 eyes before and after Descemet stripping endothelial keratoplasty (DSEK).Corneal sensitivity in Fuchs dystrophy (4.61 ± 1.42 cm) was lower than that of age-matched controls (5.74 ± 0.48 cm, P0.001). Sensitivity decreased by 1 month after DSEK (2.98 ± 2.01 cm, P0.001), returned to preoperative sensitivity by 24 months (4.50 ± 1.63 cm, n = 33, P = 0.99), but remained lower than controls at 36 months (4.50 ± 1.48 cm, n = 15, P0.001). Sensitivity at 36 months after penetrating keratoplasty (1.46 ± 1.98 cm) remained decreased compared with preoperative sensitivity (P0.001). Subbasal nerves appeared sparse with abnormal branching before and through 36 months after DSEK. Sensitivity was lower in corneas without visible subbasal nerves by confocal microscopy at 12 months after DSEK (P0.005) than in corneas with visible nerves. Stromal nerves were frequently tortuous and formed loops in Fuchs dystrophy, and this appearance persisted in some eyes at 36 months after DSEK.Corneal sensitivity is decreased in Fuchs dystrophy compared with normal and remains subnormal even at 3 years after endothelial keratoplasty. Decreased sensitivity is likely to be related to loss of subbasal nerves and abnormal nerve morphology, which persist after endothelial keratoplasty.

To evaluate the prevalence of musculoskeletal disorders among eye care physicians compared with family medicine physicians.Case control study.Ophthalmologists and optometrists at the University of Iowa and Mayo Clinic (participants) and family medicine physicians at the University of Iowa and Mayo Clinic (controls).An electronic survey was e-mailed to all subjects.The prevalence of musculoskeletal symptoms between eye care providers and family medicine physicians (control group).One hundred eight-six surveys were completed by 94 eye care physicians and 92 family medicine physicians with a response rate of 99% and 80%, respectively. There were no significant differences between the 2 groups with regard to mean age, gender, body mass index, years with current employer, or years in practice. Eye care providers, compared with their family medicine colleagues, reported a higher prevalence of neck (46% vs 21%; P0.01), hand/wrist pain (17% vs 7%; P = 0.03), and lower back pain (26% vs 9%; P0.01). A greater proportion of eye care physicians classified their job as a high-strain job (high demand, low control; 31% vs 20%) and a lower proportion classified their job as an active job (high demand, high control; 24% vs 47%; p = 0.01). Several job factors reported by eye care providers to contribute to musculoskeletal symptoms included performing the same task repeatedly, working in awkward/cramped positions, working in the same position for long periods, and bending/twisting the back (all P0.01).In this survey, the study group, composed of ophthalmologists and optometrists, had a higher prevalence of neck, hand/wrist, and lower back pain compared with family medicine physicians; repetitive tasks, prolonged or awkward/cramped positions, and bending/twisting were contributory factors. Given the ramifications of these findings, future efforts should concentrate on modifications to the eye care providers' work environment to prevent or alleviate musculoskeletal disorders and their personal and socioeconomic burden.

Purpose RNA toxicity from CTG trinucleotide repeat (TNR) expansion within noncoding DNA of the transcription factor 4 (TCF4) and DM1 protein kinase (DMPK) genes has been described in Fuchs' endothelial corneal dystrophy (FECD) and myotonic dystrophy, type 1 (DM1), respectively. We prospectively evaluated DM1 patients and their families for phenotypic FECD and report the analysis of CTG expansion in the TCF4 gene and DMPK expression in corneal endothelium. Methods FECD grade was evaluated by slit lamp biomicroscopy in 26 participants from 14 families with DM1. CTG TNR length in TCF4 and DMPK was determined by a combination of Gene Scan and Southern blotting of peripheral blood leukocyte DNA. Results FECD grade was 2 or higher in 5 (36%) of 14 probands, significantly greater than the general population (5%) (P < 0.001). FECD segregated with DM1; six of eight members of the largest family had both FECD and DM1, while the other two family members had neither disease. All DNA samples from 24 subjects, including four FECD-affected probands, were bi-allelic for nonexpanded TNR length in TCF4 (

OBJECTIVES To describe the use of the Jupiter scleral contact lens (Medlens Innovations, Front Royal, VA or Essilor Contact Lens, Inc., Dallas, TX) in the management of ocular manifestations of chronic graft versus host disease (cGVHD). METHODS This study is a retrospective case series. Five consecutive patients with severe keratoconjunctivitis sicca (KCS) associated with cGVHD that could not be adequately managed with conventional therapy were evaluated for scleral contact lens wear between January and December 2007. All patients were evaluated with lenses from the standard 18.2 mm Jupiter B diagnostic fitting set. If lenses of standard design failed to provide adequate fit, custom lenses were designed. Three outcome measures were evaluated: the patient's ability to tolerate and successfully handle the lenses, improvement in symptoms of KCS, and improvement in visual acuity. RESULTS All 5 patients (10 eyes) were successfully fit with Jupiter scleral lenses. Six eyes of 3 patients were successfully fit with lenses of standard design. Standard parameters were altered to achieve adequate fit in 4 eyes of 2 patients. All patients reported subjective improvements in comfort with Jupiter scleral lenses, and best-corrected vision improved in 7 of the 10 eyes fit within the first several months of contact lens wear. The remaining 3 eyes maintained the visual acuity measured before scleral lens wear (20/20 in 2 eyes, 20/40 in 1 eye). Duration of follow-up ranged from 4 to 14 months. CONCLUSIONS Jupiter scleral lenses can relieve symptoms of KCS and may improve vision in patients with cGVHD.

PURPOSE To describe the first-documented case of histoplasma scleritis in an immunocompetent patient. METHODS Medical and pathological records of the patient described were reviewed. RESULTS A 77-year-old immunocompetent man presented with necrotizing scleritis. Biopsy was consistent with histoplasma species, and culture results confirmed a causative agent of Histoplasma capsulatum. The patient required a long course of treatment, which included topical voriconazole and amphotericin, oral itraconazole and voriconazole, intravenous amphotericin, and periocular voriconazole and amphotericin. After 8 months of treatment, the patient's scleritis resolved, and he maintained good visual acuity. CONCLUSIONS To our knowledge, this is the second report of histoplasma scleritis in the literature and the first report in an immunocompetent patient.

To estimate sex- and age-specific incidence rates of cataract surgery in a defined United States population and evaluate the change in incidence over time.Department of Ophthalmology, Mayo Clinic College of Medicine, Rochester, Minnesota, USA.Rochester Epidemiology Project databases were used to identify all incident cataract surgeries in Olmsted County, Minnesota, residents during the 25-year period from January 1, 1980, through December 31, 2004. Annual incidence rates for each sex and 10-year age group were calculated and adjusted to the 2000 U.S. white population. Change in incidence over time was assessed by fitting generalized linear models assuming a Poisson error structure.During the study period, 10 245 cataract extractions were performed in 7141 residents of all ages. Overall, the age-adjusted cataract surgery incident rate per 100000 residents was 548 (95% confidence interval [CI], 534-561) for women, 462 (95% CI, 447-478) for men, and 511 (95% CI, 501-521) for all residents. The incidence of cataract surgery increased 500% among women and 467% among men during the study period (P.001). Overall, the incidence of cataract surgery was highest in residents 70 years and older (3538 surgeries [95% CI, 3322-3764] per 100,000 residents).This population-based study found a substantial increase in incident cataract surgery among Olmsted County residents during the 25-year study period. The rate of cataract surgery increased in a nearly linear fashion during a period when phacoemulsification replaced extracapsular cataract extraction in the community.

PURPOSE: To report logarithm of the minimum angle of resolution (logMAR) visual outcomes of the Boston Keratoprosthesis Type 1. DESIGN: Prospective, cohort study. METHODS: Pre-, intra-, and postoperative parameters of 300 eyes of 300 patients who underwent implantation of a Boston Keratoprosthesis Type I device between January 2003 and July 2008 by one of 19 surgeons at 18 medical centers were collected. RESULTS: After an average of 17.1 ± 14.8 months, visual acuity improved significantly (p

Suboptimal visual acuity after endothelial keratoplasty has been attributed to increased anterior corneal high-order aberrations (HOAs). In this study, we determined anterior and posterior corneal HOAs over a range of severity of Fuchs' endothelial corneal dystrophy (FECD).Cross-sectional study.A total of 108 eyes (62 subjects) with a range of severity of FECD and 71 normal eyes (38 subjects).All corneas were examined by using slit-lamp biomicroscopy to determine the severity of FECD versus normality. Fuchs' endothelial corneal dystrophy corneas were categorized as mild, moderate, or advanced according to the area and confluence of guttae and the presence of clinically visible edema. Normal corneas were devoid of any guttae. Wavefront errors from the anterior and posterior corneal surfaces were derived from Scheimpflug images and expressed as Zernike polynomials through the sixth order over a 6-mm diameter optical zone. Backscatter from the anterior 120 μm and posterior 60 μm of the cornea also was measured from Scheimpflug images and was standardized to a fixed scatter source. Variables were compared between FECD and control eyes by using generalized estimating equation models to adjust for age and correlation between fellow eyes.High-order aberrations, expressed as root mean square of wavefront errors, and backscatter of the anterior and posterior cornea.Total anterior corneal HOAs were increased in moderate (0.61±0.27 μm, mean ± standard deviation; P = 0.01) and advanced (0.66±0.28 μm; P = 0.01) FECD compared with controls (0.47±0.16 μm). Total posterior corneal HOAs were increased in mild (0.22±0.09 μm; P = 0.017), moderate (0.22±0.08 μm; P0.001), and advanced (0.23±0.09 μm; P0.001) FECD compared with controls (0.16±0.03 μm). Anterior and posterior corneal backscatter were higher for all severities of FECD compared with controls (P ≤ 0.02, anterior; P ≤ 0.001, posterior).Anterior and posterior corneal HOAs and backscatter are higher than normal even in early stages of FECD. The early onset of HOAs in FECD might contribute to the persistence of HOAs and incomplete visual rehabilitation after endothelial keratoplasty.

Purpose To estimate the long-term cumulative risk of retinal detachment (RD) after cataract extraction (CE). Design Retrospective cohort study and nested case–control study. Participants All residents of Olmsted County, Minnesota who had CE from 1980 through 2004 (10 256 CEs in 7137 residents) and were diagnosed subsequently with RD in the same period. Two controls chosen from the primary cataract surgery cohort were matched to each RD case by age, gender, and duration of follow-up. Methods Cases were identified through the Rochester Epidemiology Project databases. Records were reviewed to confirm case status and ascertain risk factor information. The observed probability of RD after CE was estimated using the Kaplan–Meier method. A cumulative probability ratio of RD after CE was determined by comparing the observed probability of RD and the expected probability of RD in residents without CE. Logistic regression models assessed differences between cases and controls. Main Outcome Measures Probability of and risk factors associated with RD after CE. Results Eighty-two cases of RD were identified. The cumulative probability of RD increased in a nearly linear manner over the 25-year study period. At 1, 5, 10, 15, and 20 years after extracapsular CE (ECCE) and phacoemulsification, cumulative probabilities of RD were 0.27%, 0.71%, 1.23%, 1.58%, and 1.79%, respectively. There was no significant difference in the probability of RD after ECCE when compared with phacoemulsification ( P = 0.13). The cumulative probability ratio of RD at 20 years after ECCE and phacoemulsification remained 4.0-fold (95% confidence interval, 2.6–5.4) higher than would be expected in a similar group of residents not undergoing CE ( P P Conclusions The cumulative risk of RD after ECCE and phacoemulsification is increased for up to 20 years after surgery.

PURPOSE: To estimate the cumulative probability of Nd:YAG laser posterior capsulotomy after cataract extraction in a geographically defined population. METHODS: Rochester Epidemiology Project databases were used to identify retrospectively all Nd:YAG laser posterior capsulotomies performed on Olmsted County, Minnesota, residents who had previously undergone cataract extraction between 1988 and 1996, inclusive. Demographic data and potential risk factors for laser, including age, sex, surgical technique, year of surgery, and intraocular lens material, were obtained by chart review or by retrieval from computer databases. The cumulative probability of Nd:YAG laser posterior capsulotomy was calculated by Kaplan-Meier estimates, and risk factors were analyzed using the Cox proportional hazards model. RESULTS: A total of 925 Nd:YAG laser posterior capsulotomies were identified after 3541 cataract extractions in 2718 patients. The cumulative probability of Nd:YAG laser posterior capsulotomy after cataract surgery was 6% (95% confidence interval = 5% to 7%) at 1 year, increasing to 38% (35% to 40%) at 9 years. Young age at the time of surgery (P = .02), polymethylmethacrylate intraocular lens material (P < .001), earlier year of surgery (P < .001), and extracapsular extraction (in comparison with phacoemulsification, P < .001) were found to increase significantly the risk of subsequent Nd:YAG laser posterior capsulotomy. Woman tended to have a greater probability of Nd:YAG laser posterior capsulotomy (P = .17), but this difference was not statistically significant. CONCLUSIONS: Nd:YAG laser posterior capsulotomy was common after cataract surgery but infrequent during the first postoperative year. Prolonged follow-up is necessary in investigations of the effects of new cataract surgery technologies on the probability of capsulotomy.

Primary episcleral melanoma is exceedingly rare. We report a case of primary episcleral melanoma in an eye with ocular melanocytosis.Interventional case report with presentation of clinical photographs and histopathology.A 62-year-old man with long-standing ocular melanocytosis presented with a new, raised, darkly pigmented lesion on the episclera. Clinical examination and ultrasound biomicroscopy showed no evidence of intraocular mass or nevus. Lamellar scleroconjunctivectomy of the lesion was performed, and histopathologic analysis revealed episcleral melanoma with no evidence of intra- or extra-ocular extension. He was later treated with cryotherapy to the surgical bed. Continued observation shows no evidence for intraocular tumor or recurrence of episcleral tumor.Primary episcleral melanoma is a previously unreported complication of ocular melanocytosis, and should be considered in the differential diagnosis in patients with ocular melanocytosis and pigmented nodules on the ocular surface.

To evaluate histologic signs of toxicity of the protein tyrosine kinase inhibitor, imatinib mesylate, in rabbit eyes.Twenty Dutch-belted rabbits underwent intravitreal injections of 0.1 ml solutions of imatinib mesylate. Ten rabbits were killed and enucleated 1 week after injection of imatinib mesylate (1.65 mg (four eyes), 165 microg (four eyes), and 16.5 microg (two eyes)). Ten rabbits injected with imatinib mesylate (165 microg (five eyes) and 825 microg (five eyes)) were enucleated 1 month later. Eyes were fixed in 10% formalin and stained with haematoxylin and eosin for microscopic examination.All four eyes injected with 1.65 mg of imatinib mesylate and enucleated at 1 week demonstrated ocular toxicity. All four eyes injected with 165 microg and enucleated at 1 week showed no ocular toxicity. One of the two eyes injected with 16.5 microg and enucleated at 1 week revealed focal areas of subretinal fluid and retinal undulations, suggestive of retinal oedema. None of the 10 eyes injected with imatinib mesylate at either the 165 or 825 microg dose and enucleated at 1 month showed ocular toxicity.Imatinib mesylate at 1.65 mg caused extensive retinal toxicity in rabbit eyes. In contrast, lower doses did not appear to cause toxicity, but may be associated with retinal oedema.

PURPOSE: To describe the clinical appearance of factitious (or self-inflicted) lesions on periocular skin and face. METHODS: All patients with factitious cutaneous disease who were examined at Mayo Clinic, Rochester, Minnesota, between 1985 and 1997 were identified. For patients with lesions on the face and periocular skin, the demographic features, clinical descriptive characteristics of their lesions, associated psychopathology, and treatments were ascertained. RESULTS: Of 38 patients with factitious dermatitis, 18 (47%) had facial lesions. Of these 18 patients, 15 (83%) were female. The mean age (± SD) of the patients with facial lesions was 35.2 ± 15.7 years (range, 9 to 66 years). Eight patients (44%) had neurotic excoriations, nine (50%) had dermatitis artefacta, and one (6%) had trichotillomania. The working diagnoses of five patients cared for initially in the Department of Ophthalmology were corneal epithelial and facial desquamation associated with severe pain of unknown cause, medial cicatricial ectropion of probable vasculitic cause, basal cell carcinoma of the nasojugal fold, recurrent preseptal cellulitis resistant to medical treatment, and madarosis of the upper eyelids of unknown cause. CONCLUSION: Cutaneous factitious disease may masquerade as numerous clinical entities and should be included in the differential diagnosis of lesions of the periocular skin.

Objective To estimate the incidence of rhegmatogenous retinal detachment (RD) in a geographically defined population and to compare the probability of RD in residents after cataract extraction with the probability of RD in residents who did not have cataract extraction. Design Rochester Epidemiology Project databases were used to perform a retrospective population-based incidence study of RD diagnosed between 1976 and 1995 with cohort analyses of the influence of risk factors on the occurrence of RD. Participants The population of Olmsted County, Minnesota, participated. Main outcome measure Incidence rates of RD adjusted to the age and gender distribution of the 1990 U.S. white population were measured. Results Three hundred eleven incident cases of rhegmatogenous RD were identified. The mean annual age- and gender-adjusted incidence rate of rhegmatogenous RD was 17.9 per 100,000 persons (95% confidence interval [CI], 15.9–19.9). For idiopathic rhegmatogenous RD alone, the mean annual age- and gender-adjusted incidence rate was 12.6 (95% CI, 10.9–14.3) per 100,000 persons. Ten years after phacoemulsification and extracapsular cataract extraction, the estimated cumulative probability of RD was 5.5 (95% CI, 3.4–7.6) times as high as would have been expected in a similar group of county residents not undergoing cataract surgery. Conclusions Cataract surgery is associated with a significantly elevated long-term cumulative probability of retinal detachment.

Systemic infections occasionally present with ocular involvement. Prompt diagnosis and management, aided by an internist, may lead to resolution of the infection without severe ocular sequelae. Recent literature discussing atypical ophthalmic manifestations, treatment options, or transmission risks of the following diseases is reviewed: infection with hepatitis viruses, toxoplasmosis, tuberculosis, bacteremia, and endogenous mycotic endophthalmitis.

Purpose To investigate the effects of phacoemulsification in glaucomatous eyes with functioning Baerveldt tube shunts. Setting Department of Ophthalmology, Mayo Clinic College of Medicine, Rochester, Minnesota, USA. Methods This retrospective analysis followed 9 eyes of 8 patients with functioning Baerveldt tube shunts who had clear corneal phacoemulsification. Intraocular pressure (IOP), number of glaucoma medications, corneal thickness, visual acuity, and additional glaucoma surgery were measured. Results The mean follow-up was 21 months ± 3 (SD) (range 17 to 25 months). The mean change in IOP and the number of glaucoma medications used did not significantly change at 1 month (P>.19), 1 year (P>.17), or the last follow-up (P>.17). At the last examination, 2 eyes had IOP increases of 2 to 4 mm Hg, 3 eyes had no change, and 4 eyes had decreases of 4 to 7 mm Hg. Intraocular pressure increased in 1 eye at 8 months and required a tube shunt revision. No eye developed corneal decompensation. Visual acuity improved in 8 eyes; the mean improvement was 4 ± 3 Snellen lines. Conclusion In most cases, phacoemulsification in glaucomatous eyes with a functioning Baerveldt tube shunt improved vision and maintained control of IOP.

Corneas with advanced Fuchs' endothelial dystrophy that require endothelial keratoplasty manifest anterior corneal structural and cellular abnormalities that have been associated with visual deficits before and after endothelial keratoplasty. In this study, we determined the onset of these abnormalities in the course of the disease.Cross-sectional study.Sixty-three eyes (39 subjects) with a range of severity of Fuchs' dystrophy and 25 eyes (13 subjects) with normal corneas.All corneas were examined using slit-lamp biomicroscopy, ultrasonic pachymetry, and confocal microscopy. The clinical grade of Fuchs' dystrophy was assessed according to the presence and extent of guttae and clinically evident edema and was categorized as mild (grades 1 and 2), moderate (grades 3 and 4), or advanced (grades 5 and 6). Normal corneas were devoid of any central guttae (grade 0). Corneal backscatter (haze) was measured from the confocal image light intensity profile. Stromal cell density and number and the presence of abnormal subepithelial cells were determined from confocal images. Comparisons between groups were made by using generalized estimating equation models.Anterior corneal backscatter, stromal cell density and number, presence of subepithelial cells, and central corneal thickness.Anterior corneal backscatter was 18% to 67% higher in eyes with moderate and advanced Fuchs' dystrophy compared with normal eyes (P ≤ 0.003); a similar trend was noted in mild Fuchs' dystrophy eyes compared with normal eyes (P = 0.08). Stromal cell density and the absolute number of stromal cells in the anterior 10% of the stroma were approximately 20% and 27% lower, respectively, in Fuchs' dystrophy (regardless of severity) compared with normal (P0.001). Abnormal subepithelial cells were visible in 9%, 19%, and 30% of corneas with mild, moderate, and advanced Fuchs' dystrophy, respectively. Only corneas with advanced Fuchs' dystrophy were thicker than normal (P0.001).Anterior corneal cellular and structural abnormalities begin early in the course of Fuchs' dystrophy, before the onset of clinically evident edema. The chronicity of these changes can explain their incomplete resolution after endothelial keratoplasty, and understanding the onset of these may help to determine the optimal time to intervene to achieve best outcomes.

Purpose To describe a case of congenital entropion presenting with ulcerative keratitis that was successfully treated with a single injection of botulinum toxin. Design Interventional case report. Methods A 3-week-old female infant with a corneal ulcer of the left eye since birth presented for evaluation. She was found to have entropion of the left lower lid. The pretarsal orbicularis muscle was injected with 5 units of botulinum toxin. Results Four days after treatment, the entropion had resolved and the corneal epithelial defect had healed. There was no recurrence of the entropion 7 months after botulinum toxin injection. Conclusions Injection of botulinum toxin can effectively treat certain cases of congenital entropion.

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 Editioncode 371.57), stratified by race/ethnicity. Among all Medicare beneficiaries 65 years or older, a diagnosis code for FECD was used in 1.55% (95% CI, 1.51%-1.59%) of white and 1.38% (95% CI, 1.26%-1.50%) of African American beneficiaries who had an ophthalmologist eye examination in 2014 (P = .01). Among beneficiaries who obtained medical care for FECD, keratoplasty was 1.9 times more likely in white than African American patients (4.7%; 95% CI, 4.2%-5.2% vs 2.5%; 95% CI, 1.1%-3.9%;P Conclusions and Relevance In 2014, keratoplasty was 1.9 times more likely in US Medicare fee-for-service white patients than African American patients with FECD. This might be caused by racial/ethnic differences in the biology of FECD, access to care, or other unidentified factors.

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. Steady-state corneal thickness (CTss) and standardized central corneal backscatter were derived from Scheimpflug images. Corneal swelling was induced by wearing a low-oxygen transmissible contact lens for 2 hours in the morning. De-swelling was measured over 5 hours after lens removal or until corneal thickness returned to CTss. Percent recovery per hour was 100 × (1 – e−k), where k was determined from CT(t) = (de−kt) + CTss, and where d was the initial change from CTss. Results After contact lens wear, corneas swelled by 9% (95% CI 9–10). Percent recovery per hour was 49%/h (95% CI 41–57) in controls and 37%/h in advanced FECD (95% CI 29–43, P = 0.028). Low PRPH was associated with disease severity, low ECDe, and increased anterior and posterior corneal backscatter. Anterior backscatter was associated with PRPH in a multivariable model (R2 = 0.44). Conclusions Corneal hydration control is impaired in advanced FECD and is inversely related to anterior corneal backscatter. Anterior corneal backscatter might serve as an indicator of impaired endothelium in FECD.