Associations Between Visual Functions and Severity Gradings, Corneal Scatter, or Higher-Order Aberrations in Fuchs Endothelial Corneal Dystrophy.

Purpose: The purpose of this study was to investigate the associations between visual function and severity grading, corneal scatter, or higher-order aberrations (HOAs) in patients with Fuchs endothelial corneal dystrophy (FECD). Methods: This observational case series study included 49 eyes of 27 patients with FECD and 10 eyes of 10 healthy individuals. We evaluated corrected distance visual acuity (CDVA) using Landolt-C and Early Treatment Diabetic Retinopathy Study charts and contrast sensitivity using the CSV-1000E chart and CSV-1000RN letter chart. We analyzed the associations between visual function and explanatory variables, including age, modified Krachmer grade, central corneal thickness (CCT), anterior segment optical coherence tomography (AS-OCT)-based grade, HOAs, intraocular straylight, and corneal densitometry. We additionally conducted receiver operating characteristic (ROC) analysis to identify the corneal densitometry thresholds for decreased visual function. Results: There were significant associations between visual function and the modified Krachmer grade, CCT, AS-OCT-based grade, HOAs, intraocular straylight, and corneal densitometry. A modified Krachmer grade ≥ 3 was identified as a threshold for decreased visual function. Multivariate analysis showed that corneal densitometry was significantly associated with all visual function parameters, and HOAs were significantly associated with CDVA but not with contrast sensitivity. ROC analysis revealed that corneal densitometry of the posterior layer at 0 to 2 mm ≥ 10 grayscale units (GSU), was identified as a threshold for decreased visual function. Conclusions: HOAs, forward and backward light scatter affected visual function, with backward light scatter being the most influential. In patients with FECD, modified Krachmer grade ≥ 3 and corneal densitometry ≥ 10 GSU were thresholds for visual disturbance.

Continuous Corneal Endothelial Damage by Chlorhexidine Alcohol Used for Disinfection in Nonophthalmic Surgery.

PURPOSE: To report a case of corneal endothelial damage caused by alcohol-containing chlorhexidine gluconate (CG-A) and its progression over time. METHODS: This was a case report. RESULTS: A 22-year-old man underwent neurosurgery under general anesthesia. CG-A (1%) was used for disinfection after the application of corneal protection tape. Postoperatively, the patient presented with hyperemia and swelling of the left conjunctiva and was referred to our department. Initial examination revealed left corneal epithelial erosion and corneal edema, which improved on postoperative day 14. The corneal endothelial cell density (ECD) was 3,345 cells/mm 2 on day 14, decreased rapidly to 2,090 cells/mm 2 on day 42, and slowly reduced to 1,122 cells/mm 2 on day 168. Thereafter, no decrease in ECD was observed. CONCLUSIONS: CG formulations can lead to a persistent decrease in ECD over several months, even after improvement of acute corneal edema.

Comparison of Scheimpflug and Anterior Segment Optical Coherence Tomography Imaging Parameters for Japanese Patients With Fuchs Endothelial Corneal Dystrophy With and Without TCF4 Repeat Expansions.

PURPOSE: The aim of this study was to investigate the association between cytosine-thymine-guanine trinucleotide repeat (TNR) expansion in TCF4 and the clinical phenotypes of corneal densitometry or anterior segment morphology in Fuchs endothelial corneal dystrophy. METHODS: This retrospective cross-sectional study included 150 eyes from 75 Japanese consecutive patients with Fuchs endothelial corneal dystrophy. Cytosine-thymine-guanine repeat expansion of leukocyte-derived genomic DNA was analyzed through fragment analysis using polymerase chain reaction and triplet repeat primed polymerase chain reaction. Scheimpflug-based densitometry and anterior segment optical coherence tomography were applied. Corneal densitometry, and corneal and anterior segment morphology parameters were compared between patients with and without TNR expansion of 50 or more (expansion and nonexpansion groups, respectively) using a mixed model. RESULTS: The average age of the patients was 66.8 ± 13.0 years, and the modified Krachmer grading scale was 1, 2, 3, 4, 5, and 6 for 7, 32, 28, 51, 6, and 18 eyes, respectively. Sixteen patients (21%) exhibited ≥50 TNR expansion. No significant differences in sex, age, history of keratoplasty, modified Krachmer grade, and corneal densitometry in either diameter or depth were observed between the 2 groups. No significant differences in anterior segment morphology, including the anterior chamber depth and anterior chamber angle width parameters, were observed using a univariate mixed model, except for central corneal thickness (P = 0.047). However, according to the multivariate mixed model, repeat expansion was not significantly associated with central corneal thickness (P = 0.27). CONCLUSIONS: No significant differences in clinical phenotypes were found between Japanese patients having Fuchs endothelial corneal dystrophy with and without TNR expansion.

Long-Term Corneal Refractive Power Changes Two Decades After Radial Keratotomy With Microperforations.

ABSTRACT: We retrospectively examined corneal refractive power in three patients who had been followed up for more than 20 years after radial keratotomy (RK) with microperforations (MPs). All patients underwent RK in both eyes and were referred to our clinic because of postoperative decreased vision. MP was observed in five of the six eyes at the initial visit. The corneal refractive power of the anterior and posterior surfaces of the 6-mm-diameter cornea was examined using Fourier analysis based on corneal shape analysis using anterior segment optical coherence tomography. The spherical components decreased in all three cases. The asymmetry and higher-order irregularity components and fluctuations in corneal refractive power were markedly greater in the two cases with MP in both eyes. Fluctuations in corneal refractive power were observed at more than 20 years after RK with MP. Therefore, careful observation is necessary, even after a long-term postoperative follow-up period.

Comparison of Ocular Wavefront Aberration Measurements Obtained Using Two Hartmann-Shack Wavefront Aberrometers.

OBJECTIVES: To assess agreement between measurements of ocular wavefront aberrations obtained using the Pentacam AXL Wave (Oculus Optikgeräte GmbH) (Aberrometer A) and KR-1W (Topcon Corp) (Aberrometer B), both of which are based on the Hartmann-Shack principle. METHODS: In this prospective case-control study, ocular wavefront aberrations measurements were obtained using both aberrometers in patients with keratoconus (KC) and control participants. Ocular wavefront aberrations were measured through the natural pupil without dilation using both devices in a dark room. For both aberrometers, accommodation was inhibited by automatically adding fogging. The individual Zernike coefficients from the second to fourth order were compared between the two aberrometers for a 4-mm pupil diameter. RESULTS: Twenty-six KC and 29 control eyes were assessed. Statistically significant correlations ( P <0.05) were observed for all Zernike coefficients, except for Z 4-2 in the control group. Bland-Altman analysis indicated good agreement between aberrometers and no statistically significant differences in the control group. However, in the KC group, patterns of proportional error were observed in vertical coma Z 3-1 (r=0.338, P =0.008), trefoil Z 4-4 (r=0.701, P =0.003), secondary astigmatism Z 4-2 (r=0.348, P =0.025), and spherical aberrations Z 40 (r=0.407, P =0.012). CONCLUSIONS: The Zernike coefficient values measured by the two aberrometers were well correlated in the control and KC groups. However, in eyes with KC, Aberrometer B tended to present greater values in several Zernike coefficients than Aberrometer A, suggesting that wavefront measurements obtained using the two aberrometers are not interchangeable in patients with KC.

Assessment of the corneal biomechanical features of granular corneal dystrophy type 2 using dynamic ultra-high-speed Scheimpflug imaging.

PURPOSE: To evaluate the corneal biomechanical features of eyes with granular corneal dystrophy type 2 (GCD2) by analyzing corneal biomechanical indices obtained using a Corvis ST (CST) dynamic ultra-high-speed Scheimpflug imaging device. METHODS: In this retrospective case-control study, 35 CST parameters were compared in normal eyes (control) and eyes of patients with GCD2 treated at Osaka University Hospital, Osaka, Japan. The parameters included the Corvis Biomechanical Index (CBI), which is important in differentiating eyes with keratoconus from normal eyes. We measured the deposition rates of lesions in the central 7-mm region of the eye and assessed the correlation between the deposition rate and the CBI. RESULTS: Twenty-one eyes with GCD2 and 23 control eyes were analyzed. Eyes with GCD2 showed significantly less corneal stiffness in 15 CST parameters than did control eyes. In particular, the CBI was remarkably higher in eyes with GCD2 than in control eyes (P = 0.000006). Additionally, the deposition rate and the CBI were positively correlated. CONCLUSIONS: GCD2 eyes had softer corneas than did control eyes in most biomechanical CST parameters, and one of the parameters (the CBI) was linked to the rate of deposited lesions. Since IOP may be underestimated in GCD2 eyes, management should be especially careful in GCD2 cases complicated by glaucoma.

Learning to listen: A complementary approach to informed consent for patients with visual impairments.

This forum describes an exploratory approach for assisting individuals with visual impairment during the informed consent (IC) process to participate in a cutting-edge trial. Our approach has been developed to focus on potential participants' preparedness to give IC, along with the creation of supporting audio material.

A Case of Clinically Atypical Gelatinous Drop-like Corneal Dystrophy With Unilateral Recurrent Amyloid Depositions.

PURPOSE: The purpose of this article was to describe the successful diagnosis and management of clinically atypical, unilateral, gelatinous drop-like corneal dystrophy (GDLD) in a pediatric patient. METHODS: This study was a case report. RESULTS: A 7-year-old Japanese girl was referred to our clinic with right corneal opacity for over 3 years. Slitlamp examination revealed a white, protruding, paracentral corneal opacity with an irregular surface and tiny stromal lattice figures with subepithelial opacities. No trichiasis or epiblepharon was observed, and the patient's right corrected distance visual acuity (CDVA) was 18/20. The contralateral cornea was intact but demonstrated fluorescein uptake. After 8 months, the right CDVA worsened from 18/20 to 6/20, and corneal epithelial scraping was performed. Histopathological analysis revealed amyloid nodules in the subepithelial layer and in the anterior corneal stroma stained with Congo red, which reoccurred 2 months after the procedure, and corneal dystrophy was suspected. Isolation and sequencing of the genomic DNA revealed a homozygous p.Gln118Ter. mutation in TACSTD2 in the patient and heterozygous p.Gln118Ter. mutations in both parents. GDLD was diagnosed; bilateral use of therapeutic soft contact lenses was prescribed after the first corneal scraping. No additional surgical intervention was required for the right eye for 4.5 years. CDVA of the contralateral left eye has been successfully maintained at 30/20 over this period, without emergence of nodular lesions or corneal opacities. CONCLUSIONS: We encountered a patient with early, atypical GDLD, who was definitively diagnosed using genomic DNA sequencing. GDLD should be a part of the differential diagnosis in patients presenting with unilateral, recurrent amyloid deposition.

Long-term survival in non-human primates of stem cell-derived, MHC-unmatched corneal epithelial cell sheets.

When corneal epithelial stem cells residing in the corneal limbus become dysfunctional, called a limbal stem cell deficiency (LSCD), corneal transparency is decreased, causing severe vision loss. Transplantation of corneal epithelial cell sheets (CEPS) derived from stem cells, including induced pluripotent stem cells, is a promising treatment for LSCD. However, the potential effect of human leukocyte antigen (HLA) concordance on CEPS transplantation has not been addressed. Here, we show that there is no difference in the immune response to CEPS between HLA-matched and -unmatched peripheral blood mononuclear cells in mixed lymphocyte reactions. CEPS transplantation in cynomolgus monkeys revealed that the immune response to major histocompatibility-unmatched CEPS was not strong and could be controlled by local steroid administration. Furthermore, programmed death ligand 1 was identified as an immunosuppressive molecule in CEPS under inflammatory conditions in vitro. Our results indicate that corneal epithelium has low immunogenicity and allogeneic CEPS transplantation requires mild immunosuppression.

Treatment of Descemet's membrane detachment after primary Descemet's stripping automated endothelial keratoplasty during surgery using intraoperative optical coherence tomography.

Purpose: To present a novel microscope-integrated optical coherence tomography (iOCT)-guided surgical technique wherein Descemet's membrane detachment (DMD), occurring during vitrectomy, was treated intraoperatively in a patient who had previously undergone Descemet's stripping automated endothelial keratoplasty (DSAEK). Observations: The surgical technique was performed on a 75-year-old man with a history of DSAEK to intraoperatively treat DMD, which occurred during vitrectomy in the left eye. A fine needle mounted on a syringe was inserted into the supra-Descemet's space under iOCT guidance. The location of the needle was easily identified by its high reflection. The interface fluid was safely aspirated under excellent visualization of the needle tip and the interface. Successful aspiration of the interface fluid was confirmed via iOCT imaging at the end of the surgery. The graft has remained well attached to the cornea throughout the one-year postoperative follow-up. Conclusion and importance: iOCT-guided surgical interventions provide a safe and accurate approach for treating intraoperative complications in eyes with a history of DSAEK.

Inferior Removal of Dislocated Polymethyl Methacrylate Intraocular Lens and Scleral Refixation in Glaucomatous Eyes.

INTRODUCTION: The aim of this study was to report a technique for the removal of dislocated polymethyl methacrylate (PMMA) intraocular lenses (IOLs) in glaucomatous eyes. METHODS: Dislocated PMMA IOLs were removed from the inferior sclerocorneal incision, and sutureless intrascleral fixation of each IOL was performed to preserve the intact superior conjunctiva and sclera for future trabeculectomy or to maintain a functional filtering bleb of trabeculectomy. RESULTS: In two cases, the condition of the bleb did not change, while the intraocular pressure improved or did not change after the procedures. IOL fixation was stable with no complications, such as tilt, decentration, or extrication of the IOL haptics. CONCLUSION: This procedure of preserving the superior conjunctiva and sclera can maintain the function of the bleb, superior cornea and sclera and may contribute to the success of future trabeculectomy.

Intraocular Endoscopy Resolved Tube Occlusion of an Ahmed Glaucoma Valve.

We report a case in which intraocular endoscopy clarified the cause of Ahmed glaucoma valve (AGV) failure with a cloudy cornea. A 42-year-old patient with glaucoma underwent AGV implant surgery to treat secondary glaucoma due to chronic iridocyclitis in his left eye. After AGV, he developed bullous keratopathy (BK) in that eye. After Descemet stripping automated endothelial keratoplasty (DSAEK) was performed to treat BK, the intraocular pressure (IOP) increased and early failure of the DSAEK resulted again in a cloudy cornea. We could not precisely detect any cause of AGV failure with ordinary imaging instrumentation. An intraocular endoscope was used to determine that cause, and we found that the fibrous tissue occluded the tube of the AGV. The IOP decreased soon after the tissue was removed. We conclude that intraocular endoscopy was useful for diagnosing AGV failure with BK.

Fusarium infection complicating rheumatic keratitis that acutely progressed to endophthalmitis during regular infusion of tocilizumab: a case report.

BACKGROUND: Filamentous fungi are ubiquitous in plants, water, and soil. The predominant fungi that infect the human cornea include Fusarium and Aspergillus species. The onset of fungal endophthalmitis is indolent, and typically takes weeks to months to develop after corneal infection. We report a case of Fusarium infection complicating rheumatic keratitis that acutely progressed to endophthalmitis during intravenous tocilizumab therapy. CASE PRESENTATION: A 65-year-old female patient was referred to our department due to pain and decreased vision in her left eye. Slit-lamp examination showed a white focus on the upper peripheral cornea, hypopyon, anterior chamber fibrin formation, marked ciliary hyperemia, and whole corneal epithelial defects. As the corneal scraping smear was positive for filamentous fungi and Fusarium species were detected by aqueous humor polymerase chain reaction, anti-fungal therapy was started. Although the initial response to anti-fungal therapy was good, we observed corneal infiltration, worsening hypopyon, and vitreous opacity after tocilizumab infusion. Given that the infection continued to progress despite conservative therapy, we performed penetrating keratoplasty combined with vitrectomy. After removal of the white focus beneath the intraocular lens, a temporary corneal prosthesis was mounted and the dense vitreous opacity was removed. Finally, a frozen donor graft was sutured in place. The corneal infiltration, hypopyon, and vitreous opacity all disappeared after the operation. CONCLUSION: The rapid progression of Fusarium keratitis to endophthalmitis in a patient who was receiving a regular infusion of tocilizumab demonstrates that ocular condition should be closely monitored during systemic tocilizumab administration due to increased risk of infection.

Optimizing correction of coma aberration in keratoconus with a novel soft contact lens.

PURPOSE: To report on a second-generation prototype contact lens (modified lens) with enhanced optics to correct coma aberration and compare its performance with that of the prototype contact lens (conventional lens) used to optimise correction of coma aberration in keratoconus (KC). METHODS: Both lenses were designed as a set of standardised soft contact lenses (SCLs) with asymmetric powers along the posterior surface. The modified lens differs from the conventional lens in that the optical zone is decentred superiorly by 0.7 mm. The on-eye performance was compared between the SCLs and no-lens wearing in terms of manifest refraction, corrected distance visual acuity (CDVA), ocular aberrations, subjective quality of vision, and on-eye lens position relative to the pupil. RESULTS: Thirty-four KC eyes were included. SCLs significantly decreased coma aberration compared to no-lens wear (none, 0.68 ± 0.27 µm; conventional lens, 0.37 ± 0.28 µm; modified lens, 0.19 ± 0.15 µm; P < 0.001), with the reduction in coma aberration being significantly greater with the modified lens than with the conventional lens (P = 0.018). No significant difference in manifest refraction or CDVA was found among the three conditions. Quality of vision was significantly better with the modified lens than with no SCL wear (P < 0.05) but no differences were found between the SCLs. The on-eye optical center position relative to the pupil was closer to the pupil centre using the modified lens than the conventional lens (P < 0.001). CONCLUSION: Optimisation of the location of the optical zone in a standardised asymmetric SCL improves correction of coma aberrations and on-eye optical centration.

Quantitative Analysis of the Association Between Follow-Up Duration and Severity of Limbal Stem Cell Deficiency or Visual Acuity in Aniridia.

Purpose: This study aimed to quantitatively analyze the association between follow-up duration and the severity of limbal stem cell deficiency (LSCD) or visual acuity in patients with aniridia. Methods: A total of 52 eyes of 27 patients with aniridia were enrolled at Osaka University Hospital. Medical records were retrospectively reviewed to obtain information on the severity of LSCD and corrected distance visual acuity (CDVA). LSCD severity was based on a modified severity grading scale. We used an ordered logistic regression model to examine the association between follow-up duration and LSCD severity, and a linear regression model with a generalized linear mixed model for the association between follow-up duration and visual acuity. Results: The mean follow-up duration was 5.2 ± 6.3 years. The mean age at the last follow-up visit was 40.5 ± 18.9 years. The mean CDVA was 1.52 ± 1.09 logMAR. At the last follow-up, 1 examined eye (1.9%) was categorized as stage 0, 7 (13.5%) as Ia, 9 (17.3%) as Ib, 5 (9.6%) as Ic, 2 (3.8%) as IIb, 12 (23.1%) as IIc, and 11 (21.2%) as III. Five eyes (9.6%) were unclassifiable. There was a significant association between follow-up duration and LSCD severity (odds ratio per +1 year, 1.41; P < 0.001). CDVA significantly decreased as follow-up duration increased. Each increase of 1 year in the follow-up duration was associated with a mean difference of +0.021 logMAR (95% confidence interval [CI] 0.01-0.03; P < 0.001). Conclusion: We quantitatively demonstrate that LSCD severity and visual impairment significantly progress as follow-up duration increases.

Efficacy of therapeutic soft contact lens in the management of gelatinous drop-like corneal dystrophy.

BACKGROUND/AIMS: To investigate the efficacy of therapeutic soft contact lenses (SCLs) in gelatinous drop-like corneal dystrophy (GDLD) management. METHODS: This was a retrospective, consecutive, observational case series, including 20 patients (40 eyes) with GDLD treated in Osaka University Hospital within the last 15 years. We tested the effects of therapeutic SCL on clinical features, visual acuity and surgical interventions. Examinations for clinical features and visual acuity were done on patients who had no surgical intervention for 3 years. Scoring and evaluation of changes in three main clinical GDLD features and visual acuity (logMAR units) were performed using Fisher's exact test and Mann-Whitney U test. Surgery-free survival time was compared by Kaplan-Meier analyses in all patients. RESULTS: We found a significantly lower rate of progression in GDLD nodular lesions in patients wearing SCLs compared with those who did not (p=0.0179). No suppressant effects were observed regarding opacity and neovascularisation, and no significant improvements were found in visual acuity (in logMAR values, SCL-on: mean=- 0.036, median=0; SCL-off: mean=0.149, median=+ 0.088; p=0.14). The surgery-free survival time for all 16 SCL-on eyes was 2770 ± 1918 days, significantly longer than that for 22 SCL-off eyes, 1342 ± 1323 days (Kaplan-Meier analysis, p=0.0007), suggesting that therapeutic SCL extends the period until surgical intervention and reduces their necessity in patients with GDLD. CONCLUSION: Wearing therapeutic SCLs in GDLD slows the progression of nodular lesions and decreases the need for surgical interventions.