Incidence of Leber hereditary optic neuropathy in 2019 in Japan: a second nationwide questionnaire survey.

Background: Leber hereditary optic neuropathy (LHON) is an acute or subacute optic neuropathy that mainly affects young males. The first nationwide epidemiological survey of LHON was conducted in 2014 in Japan, and LHON was officially designated as a rare intractable disease by the Japanese government in 2015. We conducted a second survey of the annual incidence of LHON in 2019, and estimated the total number of patients with LHON in Japan.

Correction: The evaluation of a web-based tool for measuring the uncorrected visual acuity and refractive error in keratoconus eyes: A method comparison study.

[This corrects the article DOI: 10.1371/journal.pone.

The evaluation of a web-based tool for measuring the uncorrected visual acuity and refractive error in keratoconus eyes: A method comparison study.

Purpose: To evaluate the outcome of a web-based digital assessment of visual acuity and refractive error, compared to a conventional supervised assessment, in keratoconus patients with complex refractive errors.

Material And Methods: Keratoconus patients, aged 18 to 40, with a refractive error between -6 and +4 diopters were considered eligible. An uncorrected visual acuity and an assessment of refractive error was taken web-based (index test) and by manifest refraction (reference test) by an optometrist.

Nationwide epidemiological approach to identify associations between keratoconus and immune-mediated diseases.

Background: The aetiology of keratoconus (KC) remains poorly understood. KC has typically been described as a non-inflammatory disorder of the cornea. Nonetheless, there is increasing presumptive evidence for the role of the immune system in the pathogenesis of KC.

Intraoperative Optical Coherence Tomography-Assisted Descemet Membrane Endothelial Keratoplasty: Toward More Efficient, Safer Surgery.

Purpose: To evaluate the clinical value of intraoperative optical coherence tomography (iOCT) and prolonged overpressure in Descemet membrane endothelial keratoplasty for surgical safety, efficiency, and outcome.

Methods: All Descemet membrane endothelial keratoplasties performed by the same surgeon from November 2016 through April 2018 at the University Medical Center Utrecht were included, including 6 months of follow-up. The primary outcome was the prevalence of adverse events, and the secondary outcomes included critical decision-making and surgery time.

Prospective 3-arm study on pain and epithelial healing after corneal crosslinking.

Purpose: To investigate the effect of 3 regimes on pain and wound healing after corneal crosslinking (CXL).

Setting: Tertiary academic referral center, Utrecht, the Netherlands.

Design: Prospective cohort study.

Validation of an Independent Web-Based Tool for Measuring Visual Acuity and Refractive Error (the Manifest versus Online Refractive Evaluation Trial): Prospective Open-Label Noninferiority Clinical Trial.

Background: Digital tools provide a unique opportunity to increase access to eye care. We developed a Web-based test that measures visual acuity and both spherical and cylindrical refractive errors. This test is Conformité Européenne marked and available on the Easee website.

The Independent Effect of Various Cross-Linking Treatment Modalities on Treatment Effectiveness in Keratoconus.

Purpose: To investigate the 1-year outcomes of using various corneal cross-linking (CXL) techniques for treating keratoconus.

Methods: Setting: This is a prospective longitudinal cohort study performed at a tertiary academic medical center.

Patient Population: Six hundred seventy eyes of 461 patients with progressive keratoconus who underwent CXL were followed up for 1 year.

Clinical Evaluation and Validation of the Dutch Crosslinking for Keratoconus Score.

Importance: Defining keratoconus progression is fundamental in clinical decision making because crosslinking treatments are indicated when the disease is considered progressive. Currently, there is no consensus which parameters should be used to define progression.

Objective: To assess and validate a novel clinical scoring system as an easy-to-use assessment tool for crosslinking treatment in patients with keratoconus.

Autorefraction Versus Manifest Refraction in Patients With Keratoconus.

Purpose: To compare visual performance using autorefraction and manifest refraction assessments in patients with keratoconus and investigate whether autorefraction measurements lead to suboptimal visual performance.

Methods: Corrected distance visual acuity (CDVA) was measured in 90 eyes of 61 patients with keratoconus with both autorefraction and manifest refraction, in a random order. Maximum keratometry (Kmax), cone location, and wavefront aberration were determined with Scheimpflug tomography.

Higher order optical aberrations and visual acuity in a randomized controlled trial comparing transepithelial versus epithelium-off corneal crosslinking for progressive keratoconus.

Purpose: The purpose of this study was to compare the effects of transepithelial crosslinking (trans-CXL) versus epithelium-off crosslinking (epi-off CXL) for progressive keratoconus with respect to the development of higher order aberrations (HOAs) and their effects on visual acuity.

Materials And Methods: A total of 61 patients were randomized and examined preoperatively and 1, 3, 6, and 12 months postoperatively in an academic referral center. Total corneal HOAs were compared between the two treatment groups using mixed linear modeling.

Von Helmholtz's ophthalmometer: historical review and experience with one of the last surviving original devices.

Hermann von Helmholtz (1821-1894) was one of the most important scientists of the nineteenth century in optics and ophthalmology. One of his significant contributions in the field of vision sciences was the invention of the ophthalmometer in 1850, which was the precursor of the keratometers still used in clinical practice today. However, this development tends to be little recognized, and to be overshadowed by others of the achievements of this singular scientist.

Cost-Effectiveness Analysis of Corneal Collagen Crosslinking for Progressive Keratoconus.

Purpose: To evaluate the cost effectiveness of corneal collagen crosslinking (CXL) for progressive keratoconus from the healthcare payer's perspective.

Design: A probabilistic Markov-type model using data from published clinical trials and cohort studies.

Participants: Two identical cohorts, each comprising 1000 virtual patients with progressive bilateral keratoconus, were modeled; one cohort underwent CXL and the other cohort received no intervention.

Age-specific Incidence and Prevalence of Keratoconus: A Nationwide Registration Study.

Purpose: To determine the age-specific incidence and prevalence of keratoconus in the modern era of diagnostics.

Design: Epidemiologic study.

Participants: Total of 4.

Nationwide reduction in the number of corneal transplantations for keratoconus following the implementation of cross-linking.

Purpose: Keratoconus is characterized by corneal ectasia and irregular astigmatism, which can lead to diminished vision and corneal scarring. Approximately 10-20% of patients with keratoconus eventually require a corneal transplant. Corneal cross-linking (CXL) is a relatively new treatment that may help prevent the need for corneal transplantation.

Higher-order aberrations 1 year after corneal collagen crosslinking for keratoconus and their independent effect on visual acuity.

Purpose: To evaluate the effect of corneal collagen crosslinking (CXL) in progressive keratoconus patients on higher-order aberrations (HOAs) and the effect of change in HOAs on visual acuity between baseline and 1 year after CXL.

Setting: Tertiary academic referral center, Utrecht, the Netherlands.

Design: Prospective cohort study.

Predictors for treatment outcomes after corneal crosslinking for keratoconus: a validation study.

Previous research suggested that baseline corrected distance visual acuity (CDVA) and maximum keratometry (Kmax) are the predictors for effectiveness of corneal crosslinking (CXL) for keratoconus. The aim of this study was to validate the previously determined predictors in a new treatment cohort. A prospective cohort of 112 eyes in 90 consecutive patients was used to validate the results of 102 eyes in 79 patients from our previous prospective cohort.

Trends in penetrating and anterior lamellar corneal grafting techniques for keratoconus: a national registry study.

Purpose: Keratoconus is a progressive disorder and one of the primary indications for corneal transplantation. Anterior lamellar keratoplasty offers several advantages over other techniques, including endothelial preservation and longer graft survival. In this study, we examined the recent trend of using lamellar techniques for keratoconus at a national level.

Corneal Cross-Linking for Pediatric Keratoconus: Long-Term Results.

Purpose: To assess the efficacy and safety of cross-linking in pediatric patients with keratoconus and to provide a systematic literature overview regarding this subject.

Methods: In this prospective cohort, 54 eyes of 36 pediatric patients with keratoconus underwent standard epithelium-off cross-linking. Follow-up measurements taken up to 5 years after treatment were compared with baseline values.

Transepithelial versus epithelium-off corneal cross-linking for the treatment of progressive keratoconus: a randomized controlled trial.

Purpose: To compare the clinical effects and safety of transepithelial corneal cross-linking (CXL) to epithelium-off (epi-off) CXL in progressive keratoconus.

Design: Randomized clinical trial (noninferiority).

Methods: Patients received either transepithelial CXL with Ricrolin TE (n = 35) or epi-off CXL with isotonic riboflavin (n = 26) in 1 academic treatment center, using a simple unrestricted randomization procedure.

A multivariate analysis and statistical model for predicting visual acuity and keratometry one year after cross-linking for keratoconus.

Purpose: To investigate putative prognostic factors for predicting visual acuity and keratometry 1 year following corneal cross-linking (CXL) for treating keratoconus.

Design: Prospective cohort study.

Methods: This study included all consecutively treated keratoconus patients (102 eyes) in 1 academic treatment center, with minimal 1-year follow-up following CXL.