Five-year corneal cross-linking outcomes: A Save Sight Keratoconus Registry Study.

BACKGROUND: We aimed to determine the long-term outcomes of epithelium-off cross-linking (CXL) in keratoconus patients. METHODS: An observational registry study from 41 centres across 5 countries was carried out. Primary outcomes included the mean change in visual acuity (VA), Kmax, K2, and thinnest corneal thickness (TCT) at 1-5 years. Secondary outcomes included the percentage of eyes with worsening, stable and improving outcomes. RESULTS: There were 976 eyes of 794 patients with 1-year of complete follow-up, 501 eyes with 2-years, 355 with 3-years, 235 with 4-years and 162 with 5-years. There was a significant improvement in mean VA from baseline by 3.7 logMAR letters (p < 0.001) in year 1, and 6.9 (p < 0.001) in year 5. Mean Kmax decreased by 1.2 dioptres (D; p < 0.01) in year 1. During subsequent years the Kmax flattening appeared sustained but this was not statistically significant. K2 flattened significantly from baseline in year 1 and then remained stable. At 1 year, 4.1% patients were poor responders to CXL in terms of VA, losing ≥15 letters. The proportion of the poor responders remained unchanged: 4.9% at 5-years. The proportion of poor responders in terms of Kmax remained similar: 5.9% steepening by ≥2D at 1-year and 7.5% at 5-years. The proportion of K2 poor responders remained stable with 4.7% steepening by ≥2D at 1-year and 5.8% at 5-years. CONCLUSIONS: Cross-linking is effective at stabilising keratoconus up to 5 years in most patients. However, a small proportion of eyes failed to stabilise and had reduced vision.

Acute disseminated encephalomyelitis with bilateral optic neuritis following ChAdOx1 COVID-19 vaccination.

BACKGROUND: Acute disseminated encephalomyelitis (ADEM) is a rare immune-mediated inflammatory demyelinating disease of the central nervous system. We report a case of ADEM presenting with bilateral optic neuritis temporally associated with the ChAdOx1 vaccine against SARS-COVID19 virus. CASE PRESENTATION: A 36-year-old female presented with bilateral optic neuritis following her first dose of the ChAdOx1 vaccine. Initial MRI Brain showed evidence of demyelination within the subcortical white matter, with no radiological involvement of the optic nerves. Visual evoked potentials were consistent with bilateral optic neuritis which was confirmed radiologically on follow up MRI. She was treated with intravenous steroids with improvement both in symptoms and radiological appearance. A pseudo-relapse occurred which was treated with a further course of intravenous steroids followed by an oral taper. The clinical, radiological and serological results were most consistent with diagnosis of ADEM. CONCLUSIONS: ADEM is an exceedingly rare complication of ChAdOx1 vaccine despite millions of doses. While it is imperative clinicians remain aware of neurological complications of vaccines, the importance of vaccination to control a pandemic should not be undermined.

Comparative Efficacy and Safety of Standard Versus Accelerated Corneal Crosslinking for Keratoconus: 1-Year Outcomes From the Save Sight Keratoconus Registry Study.

PURPOSE: The aim of this study was to compare the efficacy and safety of standard [Ultraviolet (UV) light power: 3 mW/cm2, duration: 30 minutes] versus accelerated (UV power: 9 mW/cm2, duration: 10 minutes) corneal crosslinking (CXL) for stabilizing keratoconus. METHODS: A total of 684 eyes (555 patients; mean age ± SD, 25.0 ± 7.9 years; women, 30.6%) from 24 international sites with epithelium-off CXL for keratoconus had follow-up data at 1-year and met the inclusion criteria. Two hundred sixty-six eyes (228 patients) had undergone standard CXL, and 418 eyes (327 patients) had undergone accelerated CXL. The outcome measures included changes in visual acuity, keratometry, minimum corneal thickness, and frequency of adverse events. The outcomes were compared using mixed-effects regression models adjusted for age, sex, visual acuity, keratometry, pachymetry, doctor, practice, and eye laterality. RESULTS: The adjusted mean changes (95% confidence interval) in outcomes were similar in standard and accelerated CXL in visual acuity [6.5 (2.0, 11.1) versus 5.5 (0.4, 10.6) logMAR letters, respectively], Kmax [-0.9 (-1.4, -0.3) D versus -1.2 (-1.9, -0.4) D, respectively], K2 [-0.4 (-0.9, 0.2) D versus -0.4 (-1.1, 0.3), D respectively], or minimum corneal thickness [-13.3 (-20.3, -6.3) µm versus -16.6 (-24.5, -8.6) µm, respectively] (all P > 0.05). The frequency of adverse events at the 12-month visit was also similar between the CXL groups (standard, 8.3% vs. accelerated, 5.5%; P = 0.21). CONCLUSIONS: This real-world observational study found that both standard and accelerated CXL were similarly safe and effective in stabilizing keratoconus at 1-year postsurgery in the real-world setting. The findings support the adoption of accelerated CXL for time and convenience.

The Impact on Work Patterns of Implementing the Save Sight Keratoconus Registry in the Hospital Setting.

PURPOSE: The adoption of clinical registries has the potential to improve outcomes, while reducing the costs of health care. We sought to evaluate the changes in workflow that occurred with implementation of the Save Sight Keratoconus Registry (SSKR) in corneal clinics. METHODS: A prospective time-motion study and a clinician survey were conducted. The timing of clinic consultations was recorded before and after implementation of the SSKR. The activities were assigned into 3 main categories: 1) direct patient care (eg, talking to, examining), 2) indirect patient care (record keeping), and 3) indirect patient care (reading). RESULTS: Overall, there was no change in average time spent per patient with or without the SSKR; 12.3 (5.3 SD) versus 12.1 (5.3 SD) minutes, respectively, P = 0.84. There was no change in time spent providing direct care with or without the SSKR; 5.4 (2.8 SD) versus 5.9 (2.8 SD) minutes, respectively, P = 0.51. Within direct patient care, there was no impact with or without the SSKR on the time spent examining (1.9 [1.0 SD] vs. 1.9 [1.4 SD] minutes, respectively, P = 0.58) or talking to patients (3.5 [2.3 SD] vs. 4.1 [2.3 SD] minutes, respectively, P = 0.21). Indirect care time was unchanged overall for record keeping (3.2 [2.2 SD] vs. 4.6 [2.9 SD], respectively, P = 0.16) and reading tasks (3.1 [1.8 SD] vs. 2.0 [1.3 SD], respectively, P = 0.09). CONCLUSIONS: The SSKR was implemented into clinical practice without affecting the total consultation time, time spent directly interacting with patients, or use of patient records. Our findings support that registries requiring data entry could be widely adopted into routine clinical practice.

Keratoconus Natural Progression: A Systematic Review and Meta-analysis of 11 529 Eyes.

PURPOSE: We set out to describe the natural history of keratoconus. We included untreated patients, and our key outcome measures were vision, refraction, and corneal curvature. CLINICAL RELEVANCE: Keratoconus affects 86 in 100 000 people, causing visual loss due to increasing irregular corneal astigmatism, and the quality of life declines in patients. Interventions are used to stabilize the disease or improve vision, including corneal cross-linking (CXL) and grafting, but these carry risks. Detailed knowledge of the natural history of keratoconus is fundamental in making informed decisions on when their benefits outweigh these risks. METHODS: We included prospective or retrospective studies of pediatric or adult patients who reported 1 or more of visual acuity, refraction, and corneal curvature measures: steep keratometry (K2), mean keratometry (Kmean), or maximum keratometry (Kmax), thinnest pachymetry, corneal transplantation rates, corneal scarring incidence, and patient-reported outcome measures (PROMs). Databases analyzed included Medline, Embase, CENTRAL, and CINAHL. Searches were carried out until October 2018. Bias assessment was carried out using the Joanna Briggs Institute model of evidence-based healthcare. RESULTS: Our search yielded 3950 publication titles, of which 41 were included in our systematic review and 23 were incorporated into the meta-analysis. Younger patients and those with greater Kmax demonstrated more steepening of Kmax at 12 months. The meta-analysis for Kmax demonstrated a significant increase in Kmax of 0.7 diopters (D) at 12 months (95% confidence interval [CI], 0.31-1.14; P = 0.003). Our meta-regression model predicted that patients had 0.8 D less Kmax steepening over 12 months for every 10-year increase in age (P = 0.01). Patients were predicted to have 1 D greater Kmax steepening for every 5 D of greater baseline Kmax (P = 0.003). At 12 months, there was a significant increase in the average Kmean of 0.4 D (95% CI, 0.18-0.65; P = 0.004). CONCLUSIONS: We report the first systematic review and meta-analysis of keratoconus natural history data including 11 529 eyes. Younger patients and those with Kmax steeper than 55 D at presentation have a significantly greater risk of progression of keratoconus. Closer follow-up and a lower threshold for cross-linking should be adopted in patients younger than 17 years and steeper than 55 D Kmax.