Definitions for Keratoconus Progression and Their Impact on Clinical Practice.

PURPOSE: There is currently no consensus on which keratoconus need cross-linking nor on how to establish progression. This study assessed the performance of diverse progression criteria and compared them with our clinical knowledge of keratoconus evolution. METHODS: This was a retrospective, longitudinal, observational study. Habitual progression criteria, based on (combinations of) keratometry (K MAX ), front astigmatism (A F ), pachymetry (P MIN ), or ABCD progression display, from 906 keratoconus patients were analyzed. For each criterion and cutoff, we calculated %eyes flagged progressive at some point (R PROG ), individual consistency C IND (%examinations after progression detection still considered progressive), and population consistency C POP (% eyes with CIND >66%). Finally, other monotonic and consistent variables, such as front steep keratometry (K 2F ), mean radius of the back surface (R mB ), and the like, were evaluated for the overall sample and subgroups. RESULTS: Using a single criterion (e.g., ∆K MAX >1D) led to high values of R PROG . When combining two, (K MAX and A F ) led to worse C POP and higher variability than (K MAX and P MIN ); alternative criteria such as (K 2F and R mB ) obtained the best C POP and the lowest variability ( P <0.0001). ABC, as defined by its authors, obtained R PROG of 74.2%. Using wider 95% confidence intervals (95% CIs) and requiring two parameters over 95CI reduced R PROG to 27.9%. CONCLUSION: Previous clinical studies suggest that 20% to 30% of keratoconus cases are progressive. This clinical R PROG value should be considered when defining KC progression to avoid overtreatment. Using combinations of variables or wider margins for ABC brings R PROG closer to these clinical observations while obtaining better population consistency than current definitions.

Influence of Specialty Contact Lens Wear on Posterior Corneal Tomography in Keratoconus Subjects.

OBJECTIVE: To evaluate the effect of specialty contact lens (CL) wear on posterior corneal tomography in keratoconus subjects. METHODS: Patients with keratoconus who were wearing specialty CL were included in this retrospective cohort study. Tomographic parameters were evaluated with Scheimpflug imaging (Pentacam HR) before lens fitting and immediately after removal of CLs worn habitually for a period of several months. Subjects were divided into groups, according to type of lens (corneal, scleral, and hybrid) and keratoconus severity based on Belin/Ambrosio D (BAD-D) score, for further analysis. RESULTS: Thirty-four eyes of 34 subjects diagnosed with keratoconus were included. Mean duration of habitual CL wear was 7.0±0.3 months. For the entire cohort, a small increase in flat keratometric reading at the anterior corneal surface (K1F; P =0.032) and at the posterior surface (K1B; P =0.041) was found. In the corneal CL group (10 eyes; 29.4%), flattening of the anterior corneal curvature was detected (K max ; P =0.015). An increase in K1B value was seen in the scleral CL group (15 eyes; 44.1%) ( P =0.03). Combined topometric indices showed a small but significant difference in the entire cohort ( P <0.05) and in the subgroups of corneal CL wear and of moderate keratoconus (BAD-D score≥7). CONCLUSION: Various types of specialty CLs exert a differential influence on corneal parameters. A small steepening of keratometry at the posterior surface (K1B) was observed in the scleral lens group. Although corneal lens wear flattens the anterior cornea (K max ), it does not significantly alter the posterior corneal surface.

Immunopathophysiology and clinical impact of uveitis in inflammatory rheumatic diseases: An update.

BACKGROUND: Uveitis is one of the most frequent ophthalmologic manifestations in rheumatology. Uveal inflammation can underlie a systemic inflammatory rheumatic disease (SIRD) in approximately 30% of cases with a significant burden on the quality of life since it represents a cause of blindness in up to 20% of cases in Western countries. METHODS: In this review, we provide a comprehensive overview of the pathophysiology of uveitis associated with SIRDs. According to our literature survey on the epidemiology of uveitis among SIRDs, spondyloarthritides, Behçet's disease and sarcoidosis get the major impact. RESULTS: In Behçet's uveitis, the key players are highly polarized Th1 and Th17 lymphocytes, natural killer T cells and γδ T cells. All contribute to a great destructive inflammatory environment with the most serious visual damage resulting from the involvement of the posterior segment of the eye. In contrast, spondyloarthritides-related uveitis derives from a complex interaction between genetic background and extra-ocular inflammatory mediators originating from enthesitis, arthritis, psoriatic lesions and microbiome pro-inflammatory alterations. In such conditions, the immune infiltration of CD4+ T cells, Th17 and natural killer cells along with pro-inflammatory cytokines, TNF-α among all, leads to intraocular inflammation. Lastly, granuloma formation represents the primary hallmark lesion in sarcoid uveitis. This suggests a profound link between the innate system that mainly recruits activated macrophages and adaptive system involving by Th1, Th17 and Th17.1 cells. CONCLUSIONS: Awareness among rheumatologists of a potential severe ocular involvement generates new insights into targeted therapeutic approaches and personalized treatments for each patient.

Clinical Outcome of Hybrid Contact Lenses in Keratoconus.

OBJECTIVES: To report the clinical outcome of hybrid contact lens fitting in keratoconus. METHODS: A retrospective chart review was performed of keratoconus patients who had been fitted with hybrid contact lenses in one or both eyes. Patients with a history of previous intraocular surgery (except for corneal crosslinking) and relevant concurrent ocular disease limiting visual outcome were excluded. The outcome data at 6-month follow-up included hybrid lens-corrected visual acuity, wearing time, and lens discontinuation. RESULTS: Fifty-four keratoconus patients (102 eyes) were included. Mean visual acuity with habitual correction before hybrid lens fitting was 0.63±0.29 (decimal Snellen). Eyes were fitted with Eyebrid lenses (LCS Laboratories, Caen, France) and SynergEyes lenses (SynergEyes Inc., Carlsbad, CA). Refits were necessary in 13 eyes, because of poor fitting (76.9%) or suboptimal refraction (23.1%). Visual acuity had significantly improved with hybrid lenses (0.93±0.14; P<0.0001). In 37 eyes (37.8%), hybrid lens wear was discontinued within 6 months after lens fitting. Reported issues were discomfort in 27 eyes (73%), handling difficulties in 14 eyes (37.8%) and poor vision in 1 eye (2.7%). Success rate of lens fitting was significantly correlated to cone morphology (P=0.01). CONCLUSIONS: New generation hybrid lenses are an appropriate, safe therapeutic option for keratoconus patients. Excellent visual outcomes are achieved in successful fittings, yet a dropout rate of 37.8%-mainly occurring in sagging, more peripheral, cones - still limit their clinical success.

Determining the Most Suitable Tomography-Based Parameters to Describe Progression in Keratoconus. The Retrospective Digital Computer Analysis of Keratoconus Evolution Project.

OBJECTIVES: To identify the most suitable parameters to describe keratoconus progression. METHODS: Longitudinal retrospective cohort study. Monotonicity and consistency of over 250 parameters extracted from the Pentacam tomographies (Oculus, Germany) of 743 patients was analyzed. Repeatability was calculated for 69 patients (published elsewhere). The parameters were scored based on their performance for each desired feature and a global ranking was created. RESULTS: Overall, parameters that average a higher number of corneal points performed better than single-point parameters. Zonal keratometries on areas surrounding the maximum curvature point and the steepest front keratometry performed best, followed by front best-fit sphere and mean keratometry of both surfaces. Platform-dependent indices (e.g., Belin-Ambrósio Deviation- or index height decentration-) obtained good scores, but platform-independent LOGIK performed slightly better. Finally, although minimum radius in both surfaces worked competently, minimum pachymetry (PachyMin) performed considerably poorer. CONCLUSIONS: We presented a list of parameters whose behavior was repeatable, monotonic and consistent, features desirable to describe change. The parameters normally used to follow keratoconus progression may not be the most adequate, as evidenced by the poor performance of PachyMin. Although calculated for a specific Scheimpflug device, most of the best-performing parameters are platform-independent variables, and results may be generalized, pending validation.

Corneal features in trastuzumab emtansine treatment: not a rare occurrence.

PURPOSE: Ado-trastuzumab emtansine (T-DM1/Kadcyla®;Genentech) is an antibody-drug conjugate used in the treatment of human epidermal growth factor receptor-2-positive metastasized breast cancer. Few studies report a spectrum of corneal changes in patients treated with this drug. Our aim is to specify the nature and prevalence of corneal features of T-DM1 treatment in order to formulate guidelines as to which findings necessitate systemic treatment cessation or dose reduction. METHODS: We performed a cross-sectional, prospective study in all patients currently treated with T-DM1 or recently stopped in Ghent University Hospital, Belgium. RESULTS: A total of 12 patients completed a full ophthalmic workup. Ten patients were currently using T-DM1, and two patients had recently (< 10 weeks) stopped treatment because of clinical non-response. Twenty eyes of 10 patients currently on T-DM1-treatment all exhibited coarse cystoid lesions to the deep corneal epithelial cells, primarily in the midperipheral area, both biomicroscopically and on confocal microscopy. The two patients who stopped treatment, displayed no corneal epithelial changes. Only three patients reported symptoms which were attributed to other ocular factors, likely not to be related to T-DM1 treatment. CONCLUSIONS: This case series shows that asymptomatic, low-grade corneal epithelial changes are hallmark features in T-DM1-treatment and should not alarm clinicians. These findings are relatively stationary, reversible and thus do not require ocular treatment or cessation of systemic treatment.

Long-Term Visual Outcome of Patients with Blau Syndrome.

PURPOSE: To document the long-term visual outcomes in patients with Blau syndrome. METHODS: A retrospective institutional cohort study was conducted, and 13 patients with genetically confirmed Blau syndrome were included. Demographic and clinical data were collected from standardised medical charts. Baseline was defined as the first detected uveitis and data were recorded onwards at intervals of 1, 3, 5, 10, 15 and 20 years. RESULTS: Anterior uveitis was the most common classification at baseline (57.1%). Among patients with documented uveitis lasting 10 years or more, all of them developed panuveitis. Median logMAR visual acuity at baseline was 0 (range -0.5; 0.7), 0.19 (range 0; 1.5) at year 5, and 0.7 (range 0.1 - no perception of light) at year 20, as recorded in 13, 16, and 10 eyes, respectively. All patients received treatment with topical and oral steroids, and multiple systemic immunosuppressants including biologics. Disease control, defined as having cells <1+ in both eyes and using topical steroid eye drops less than twice daily, was achieved in 14.3% to 37.5% of patients at the different time points. Cataract surgery was performed in 12 eyes of 8 patients, 3 eyes of 3 patients necessitated glaucoma surgery, and 4 eyes of 4 patients required surgery for retinal detachment. CONCLUSION: Uveitis associated with Blau syndrome commonly leads to severe, chronic panuveitis, requiring long-term systemic immunosuppression. Early diagnosis and timely initiation of biologics may prevent significant visual impairment.

Anterior scleral thickness in Marfan syndrome: A quantitative analysis.

PURPOSE: To investigate the anterior scleral thickness (AST) in patients with Marfan syndrome (MFS). METHODS: A prospective, cross-sectional study was conducted at the Department of Ophthalmology, Ghent University Hospital, Ghent, including patients with a genetically confirmed clinical diagnosis of MFS and age-, gender- and axial length-matched controls. Subjects with known corneal, conjunctival or scleral pathology and a history of ocular surgery, including pars plana vitrectomy, recent contact lens use or high-grade astigmatism were excluded. Subjects underwent non-cycloplegic autorefraction, Scheimpflug-based corneal tomography, axial length measurement and spectral-domain optical coherence tomography (OCT). AST was manually measured at 1 mm (AST1), 2 mm (AST2) and 3 mm (AST3) from the scleral spur, temporally and nasally. RESULTS: A total of 56 subjects (28 subjects in the MFS group and 28 matched subjects in the control group) were included in this study. In patients with MFS, AST was significantly reduced compared to matched controls, both overall and at every analysed measuring point in the nasal and temporal areas (p < 0.001). Central corneal thickness (CCT) and mean keratometry (Kmean) values were significantly lower in patients with MFS (p < 0.05). A positive correlation was found between nasal AST and CCT in patients with MFS. No correlation was found between AST and Kmean or between AST and axial length. In patients with MFS with ectopia lentis, compared to those without, temporal AST3 was significantly lower (p < 0.05). AST was significantly lower in patients with MFS harbouring a variant predicted to cause haploinsufficiency compared to those with a variant expected to lead to a dominant negative effect for both nasal and temporal measurements. CONCLUSION: Based on anterior segment OCT measurements, AST of patients with MFS is significantly lower compared to matched controls.

Ligneous conjunctivitis mimicking preseptal cellulitis in a 3-month-old infant.

BACKGROUND: Ligneous conjunctivitis is a very rare form of pseudomembranous conjunctivitis with few published cases in literature. We aim to describe the ocular findings and treatment in an infant with ligneous conjunctivitis resembling preseptal cellulitis on presentation. MATERIALS AND METHODS: Case report of a 3-month-old girl who was referred to a tertiary centre for ophthalmic assessment due to progressive eyelid oedema with no response to initiated topical and systemic antibiotics. Ethical approval has been achieved from the local ethics committee of the Ghent University Hospital and informed consent has been obtained from the parents of the child. RESULTS: Examination under general anaesthesia showed multiple, wood-like fibrinous pseudomembranes, originating from the conjunctiva, consistent with ligneous conjunctivitis. After careful removal of the coagulated exudate covering the cornea, a central corneal epithelial defect was evident without stromal infiltration. Histopathologic examination confirmed the predominance of fibrin within the pseudomembranes. Plasminogen activity was below the normal range. Genetic analysis did not identify a pathogenic variant in the PLG gene. The corneal epithelium re-epithelialised during the following days and the conjunctival lesions gradually subsided over the ensuing weeks whilst continuing heparin-containing artificial tears. CONCLUSION: A high level of suspicion is warranted in atypical cases of preseptal cellulitis which show no response to antibiotic treatment. Particularly in young children, examination under general anesthesia is warranted to allow diagnosis of rare causes of secondary eyelid oedema. We report an infant with unilateral ligneous conjunctivitis who responded well to topical, commercially-available heparin-containing artificial tears treatment. This approach is an effective and easy first-line treatment option in this condition, particularly in milder phenotypes.

Investigating the Link Between Visual Quality and Vision-Related Quality of Life in Patients With Keratoconus.

PURPOSE: The purpose of the study was to investigate the optical and visual determinants of vision-related quality of life (VR-QoL) in patients with keratoconus. METHODS: A cross-sectional case-control study was conducted at the Department of Ophthalmology, Antwerp University Hospital, Belgium. Patients previously diagnosed with keratoconus and healthy emmetropic or ametropic volunteers were included. Patients younger than 18 years, with ametropia of more than ±10 D, or with a history of corneal surgery or relevant ocular comorbidity limiting visual acuity were excluded. Assessment included autorefraction, high-contrast visual acuity testing, corneal imaging, intraocular straylight analysis, contrast sensitivity, aberrometry, and the National Eye Institute Visual Functioning Questionnaire (NEI VFQ-25). The Rasch-modified visual functioning scale (VFS) and socio-emotional scale were used to quantify VR-QoL. Stepwise linear regression was used to investigate the association between the clinical variables and VR-QoL. RESULTS: Seventy-seven patients with keratoconus (77 pairs of eyes) and 77 age-matched and sex-matched controls were included in the study. The scores on the VFS and the SES were significantly lower in patients with keratoconus compared with controls ( P < 0.001). Higher-order and lower-order aberrations, high-contrast visual acuity, and contrast sensitivity were poorer in patients with keratoconus ( P < 0.001). Bivariate analyses showed that spectacle-corrected high-contrast visual acuity, higher spatial frequency contrast sensitivity, and higher-order aberration metrics were strong predictors of SES ( P < 0.001) and of VFS ( P < 0.001). Higher-order aberration of the worse eye was the strongest predictor for both SES (b = 0.310, P < 0.001) and VFS (b = 0.638, P < 0.001) on stepwise regression. Contrast sensitivity was not included in the stepwise regression because of insufficient data in the keratoconus group (33/77 patients; 42.9%). CONCLUSIONS: Both higher and lower aberration showed a strong correlation with VR-QoL, surpassing high-contrast visual acuity. These findings underline the importance of visual quality measures within keratoconus research and clinical care.

Concurrent Ocular and Cerebral Toxoplasmosis in a Liver Transplant Patient Treated with Anti-CD40 Monoclonal Antibody.

Toxoplasma gondii, an obligate intracellular parasitic protozoon, usually causes a mild, acute infection followed by a latent asymptomatic phase with tissue cysts or a chronic form with recurrent retinochoroiditis. However, immunocompromised patients can cause disseminated disease due to the reactivation of the latent tissue cysts or due to a primary infection. Here, we present a rare case of bilateral ocular toxoplasmosis and concurrent subacute toxoplasma encephalitis in a 70-year-old patient on anti-CD40 treatment following his liver transplant. The diagnosis was confirmed by PCR of anterior chamber fluid and brain biopsy, and no other sites of disseminated disease were detected on PET-CT. The patient has been treated with sulfamethoxazole-trimethoprim 800/160 mg with virtually complete resolution of the neurological and ocular symptoms. Iatrogenic blockade of the CD40 pathway may elicit a particular susceptibility for CNS reactivation of T. gondii.

Defining a therapeutic range for adalimumab serum concentrations in the management of pediatric noninfectious uveitis, a step towards personalized treatment.

BACKGROUND: Adalimumab is currently considered the most efficacious anti-TNFα agent for childhood noninfectious uveitis (NIU). The objective of this study was to define a therapeutic range for adalimumab trough levels in the treatment of childhood NIU. METHODS: A retrospective, observational, pilot study of 36 children with NIU aged < 18 years, treated with adalimumab. Serum adalimumab through levels and adalimumab anti-drug antibodies (ADA) were analysed at least 24 weeks after start adalimumab. RESULTS: Adalimumab trough levels were significantly higher in complete responders 11.8 µg/mL (range 6.9-33.0) compared to partial or non-responders 9,2 µg/mL (range 0-13.6) (p = 0,004). Receiver-operator characteristics analyses with an area under the curve of 0,749 (95% CI, 0,561-0,937) defined 9.6 µg/mL as the lower margin for the therapeutic range. This cut-off corresponds with a sensitivity of 88% and a specificity of 56% (positive predictive value, 85%; negative predictive value, 62.5%). A concentration effect curve defined 13 µg/mL as the upper margin. Approximately one-third (30.5%) of patients had an adalimumab trough concentration exceeding 13 µg/mL. Free ADA were observed in 2 patients (5.5%). CONCLUSIONS: A therapeutic range of adalimumab trough levels of 9.6 to 13 µg/mL, which corresponds with an optimal clinical effect, was identified. Therapeutic drug monitoring may guide the optimisation of treatment efficacy in children with NIU in the treat-to-target era.

Acute syphilitic necrotizing retinitis associated with placoid chorioretinitis in an immunocompetent patient.

PURPOSE: We report an atypical case of combined acute syphilitic necrotizing retinitis and a contiguous acute syphilitic posterior placoid chorioretinitis (ASPPC) in an HIV-negative, immunocompetent patient. METHOD: Observational case report. RESULTS: A 56-year-old male presented with a one-week history of pain and blurred vision in the left eye. He also complained of left-sided hearing loss for several months. Ocular examination demonstrated a unilateral panuveitis with a yellowish placoid macular lesion involving the outer retina contiguous with an ovoid area of full-thickness retinitis extending temporally. Vitreal polymerase chain reaction (PCR) analysis for HSV, CMV, VZV and T. gondii were negative but syphilis serology was reported as positive. An MRI of the head revealed bilateral enhancement along the facial nerves, more marked on the left side, consistent with syphilitic involvement. He was treated as neurosyphilis with a 14-day course of systemic procaine penicillin and oral probenecid. Oral prednisolone (1 mg/kg/day) was commenced 24 hours prior to initiating antibiotics to prevent Jarisch-Herxheimer reaction and treat his panuveitis. CONCLUSION: To our knowledge, this is the first report of combined syphilitic necrotizing retinitis and ASPPC occurring in continuity in the same eye. This case highlights the diversity of possible presentations of ocular syphilis, even in HIV-negative immunocompetent patients.

Influence of Author's Gender on the Peer-Review Process in Vision Science.

PURPOSE: To investigate the gender gap in first/last authors in vision science and whether gender affects manuscript review times. DESIGN: Observational retrospective database study. METHODS: First/last author's gender and country were assigned to 30 438 PubMed records (data derived from Q1-Q2 Ophthalmology journals for 2016-2020). Using mixed models, the influence of First Author Female (FAF) and Last Author Female (LAF) were evaluated on the manuscripts' review timeline. This analysis was performed globally and in predefined subgroups (English names, Asian names, specific topics). Additionally, the gender GAP was explored by country, journal, and research topics. RESULTS: The percentages of FAF/LAF were unevenly distributed by country; in the top 30 ophthalmology journals, FAF accounted for 40.0%±6.7% of the publications whereas LAF accounted for 27.1%±4.9%. Overall, FAF/LAF papers underwent significantly longer times to be reviewed (up to +10 days) and accepted (+5 days). These differences persisted when only English names-easily recognizable worldwide-were considered, but not for Asian names. Delays >1 month to get published were found for FAF in 3 of 4 topics analyzed (eg, amblyopia). CONCLUSIONS: Significant differences were found in both review and acceptance times for FAF or LAF papers. The causes for this are likely multifactorial and could be explained by a combination of gender bias and by women's concerns with being held to higher standards, something that has been previously documented, thereby perhaps delaying the rebuttal to reviewers. Increased awareness of this source of potential bias may assist in the implementation of preventive and corrective measures.

Diagnostic patterns in keratoconus.

PURPOSE: To investigate the current patterns of diagnosis and referral in keratoconus. METHODS: A retrospective chart review was performed of patients who had recently been diagnosed with keratoconus and attended dedicated clinics at Antwerp University Hospital, Belgium and Maria Middelares General Hospital, Ghent, Belgium between June 2013 and February 2018. Exclusion criteria included longstanding keratoconus diagnosis, reduced cognitive capabilities and prior surgical procedures (corneal crosslinking, penetrating keratoplasty or any type of refractive surgery). RESULTS: Three-hundred and ninety-nine patients (722 eyes) were included in this study. The mean age was 24.7 ± 6.5 years and the average maximal keratometry was 51 ± 5.2 D for the better eye and 58.4 ± 9.6 D for the worse eye. Upon diagnosis, 233 eyes (32.2 %) and 51 eyes (7.1 %) had a thinnest pachymetry <450 and <400 µm, respectively. At 6-month follow-up, 58 % of patients had been fitted with specialty contact lenses. During follow-up, 199 eyes (27.6 %) underwent corneal crosslinking. One patient underwent corneal graft surgery of his worse eye due to contact lens intolerance and insufficient visual acuity. CONCLUSION: Despite advances in diagnostic tools, keratoconus is often diagnosed at a relatively late stage. Earlier detection of keratoconus would increase the overall clinical benefit of corneal crosslinking. Further research into screening strategies is required to develop cost-effective screening programs.

Scleral shape and its correlation with corneal parameters in keratoconus.

PURPOSE: To assess the correlation of the scleral shape and corneal tomographic parameters in keratoconus. METHODS: Twenty eyes of 15 keratoconus patients with no previous specialty lens wear or ocular surgery were included in this study. Corneal imaging was obtained with the Pentacam HR and three-dimensional (3D) corneoscleral maps were acquired using the Eye Surface Profiler, ESP. Sagittal height was calculated at the central corneal level (annulus of 0-4 mm radius), peripheral cornea (annulus 4-6 mm radius) and sclera (annulus 6-8 mm radius) using ESP maps and Pentacam HR (exclusively for the central cornea). The flattest and steepest regions of each annulus and the circumferential scleral asymmetry were calculated based on custom-made software. The Pearson correlation coefficient (r) was used to evaluate the correlation between corneal parameters as measured by Pentacam HR and scleral asymmetry. RESULTS: Anterior corneal parameters, such as flattest and steepest keratometry, were found to be correlated with scleral asymmetry in keratoconus (all r>0.5, p < 0.05). In contrast, anterior astigmatism showed poor correlation with the level of scleral irregularity (r=-0.11; p = 0.32). Other disease-specific parameters pertaining to the posterior corneal curvature and corneal thickness were not correlated with scleral asymmetry. The steepest regions of the central cornea, peripheral cornea, and sclera tended to share a common angle (r = 0.92; p < 0.001 for central cornea compared to sclera). CONCLUSION: Anterior corneal parameters measured by corneal imaging are associated with the level of scleral asymmetry and the orientation of the steepest area of the sclera in eyes with keratoconus.

The corneoscleral shape in keratoconus patients with and without specialty lens wear.

PURPOSE: To evaluate differences in corneoscleral shape in keratoconus patients with and without specialty lenses compared to controls. METHODS: A cross-sectional study was performed comparing three groups of keratoconus eyes: 24 lens-naïve keratoconus eyes (17 patients; group 1), 7 eyes with corneal lens wear (7 patients; group 2) and 7 eyes with scleral lens wear (7 patients; group 3). For comparison, 25 eyes of 25 emmetropic participants and 11 eyes of 11 astigmatic participants were included. Corneoscleral topography measurements taken with the Eye Surface Profiler (ESP, Eaglet Eye BV, Houten, Netherlands) were exported and assessed using custom-made software to demarcate the limbal radius, and to calculate sagittal height and corneoscleral asymmetry. RESULTS: In non-lens wearing keratoconus patients, sagittal height was found to be significantly larger than in control eyes, in both the corneal periphery and sclera (paired t-test, pairwise comparisons p < 0.01). The level of peripheral corneal and scleral asymmetry was also significantly higher in non-lens wearing keratoconus eyes compared to controls (t-test, p < 0.01). Both corneal and scleral lens wear resulted in significant changes to the shape of the corneal periphery and sclera. In all 3 groups of keratoconus eyes, asymmetry of the peripheral cornea showed a very strong correlation with scleral asymmetry (R2 = 0.90, 0.86 and 0.85 for groups 1-3, respectively). CONCLUSION: The corneal periphery and sclera have a distinctly different shape in keratoconus eyes compared to controls. Specialty lens wear induces significant regional changes to the shape of the anterior eye in keratoconus eyes.

Scheimpflug-Based Analysis of the Reflectivity of the Cornea in Marfan Syndrome.

Purpose: We sought to investigate corneal reflectivity in Marfan syndrome (MFS) on the basis of Scheimpflug light intensity distribution. Methods: In a retrospective case-control analysis, the left eyes of 40 MFS patients and 40 age- and refraction-matched healthy controls were investigated. Patients with MFS meeting the Ghent II diagnostic criteria and with genetic confirmation of disease were included. Exclusion criteria were the following: coexisting corneal, conjunctival, or scleral pathology; use of medication known to affect corneal transparency; history of ocular surgery; and insufficient data. Scheimpflug tomography images were exported to analyze corneal transparency in different corneal layers and regions. Each corneal image was automatically segmented, after which the corresponding pixel intensities in the defined regions of interest were statistically modeled using a Weibull probability density function from which parameters α (transparency) and ß (homogeneity) were derived. Results: The cornea in MFS showed significantly higher light reflectivity (overall cornea, α = 71 ± 17 arbitrary units (a.u.)) than in the control group (overall cornea, α = 59 ± 15 a.u.) (t test, P = 0.003). The α parameter was significantly higher in MFS eyes in all examined layers and regions (P < 0.05), whereas the ß parameter showed no statistical difference between MFS and controls (P > 0.05). The difference in α did not correlate with ocular biometric properties (corneal thickness and curvature) or ectopia lentis (P > 0.05). Conclusions: The cornea in MFS shows significantly higher reflectivity than healthy controls with similar levels of homogeneity. Translational Relevance: The proposed methodology detects corneal reflectivity changes in MFS not available from regular slit-lamp examination.

Mini-Scleral Lenses Improve Vision-Related Quality of Life in Keratoconus.

PURPOSE: To evaluate the effects of mini-scleral lenses on visual acuity (VA) and visual functioning in patients with keratoconus. METHODS: This prospective, interventional study examined the effects of mini-scleral lenses on VA and visual functioning in patients with keratoconus. Patients younger than 18 years or with a history of refractive surgery or corneal graft surgery were excluded. Patients were fitted with mini-Misa lenses, Senso mini-scleral lenses, or Zenlens mini-scleral lenses. Outcome measures were scleral lens-corrected VA and vision-related quality of life as assessed with the National Eye Institute Visual Functioning Questionnaire (NEI VFQ-39). RESULTS: Eighty-nine eyes of 50 keratoconus patients were included in the study. Median baseline logarithm of the minimal angle of resolution VA with habitual correction was 0.22 (range 0.02-1.04). Mini-scleral lens fitting resulted in a statistically significant visual improvement (median 0; P < 0.0001). At the 6-month follow-up, 11 patients (22%) had abandoned mini-scleral lens wear, primarily because of difficulties with lens handling (7 patients). Of the 39 patients with continued wear, 33 patients (84.6%) wore their lenses for a daily average of 12 hours. NEI-VFQ scoring in these patients showed significantly improved results for both visual functioning and socioemotional scales after scleral lens fitting (P < 0.0001). CONCLUSIONS: Mini-scleral lenses significantly improve VA and visual functioning on NEI-VFQ in patients with keratoconus. Difficulties with lens insertion and removal are the principal reason for lens dropout.