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    Collaborative Longitudinal Evaluation of Keratoconus (CLEK) Study: methods and findings to date. Wagner H,Barr J T,Zadnik K Contact lens & anterior eye : the journal of the British Contact Lens Association PURPOSE:To describe baseline and longitudinal findings of the Collaborative Longitudinal Evaluation of Keratoconus (CLEK) Study. METHODS:The CLEK Study is an 8-year, multi-center, natural history study of 1209 patients with keratoconus who were examined annually for 8 years. Its goals are to prospectively characterize changes in vision, corneal curvature, corneal status, and vision-specific quality of life. RESULTS:CLEK Study subjects had a mean age at baseline of 39.3+/-10.9 years. At study entry, 65% of the patients wore rigid contact lenses, and 14% reported a family history of the disease. Subjects exhibited a 7-year decrease in high- (2.03 letters) and low- (4.06 letters) contrast, best-corrected visual acuity, with 19% demonstrating decreases of 10 or more letters in high-contrast, best-corrected acuity and 31% of subjects demonstrating decreases of 10 or more letters in low-contrast, best-corrected acuity in at least one eye. Subjects exhibited an average 8-year increase in corneal curvature of 1.60D in the flat corneal meridian, with 24% demonstrating increases of 3.00D or more. The 8-year incidence of corneal scarring was 20%, with younger age, corneal staining, steeper baseline corneal curvature, contact lens wear, and poorer low-contrast visual acuity predictive of corneal scarring. Data from the National Eye Institute Visual Function Questionnaire suggest that the effect of keratoconus on vision-specific quality of life is disproportionate to its low prevalence and clinical severity. CONCLUSION:Although we report measures of disease severity and visual function across the CLEK sample, clinicians can begin to envisage the course of keratoconus in individual patients by determining whether factors predictive of disease progression are present in those patients. 10.1016/j.clae.2007.03.001
    The Prevalence and Risk Factors for Keratoconus: A Systematic Review and Meta-Analysis. Hashemi Hassan,Heydarian Samira,Hooshmand Elham,Saatchi Mohammad,Yekta Abbasali,Aghamirsalim Mohamadreza,Valadkhan Mehrnaz,Mortazavi Mehdi,Hashemi Alireza,Khabazkhoob Mehdi Cornea PURPOSE:This study was conducted to determine the prevalence and risk factors for keratoconus worldwide. METHODS:In this meta-analysis, using a structured search strategy from 2 sources, 4 electronic databases (PubMed, Web of Science, Google Scholar, and Scopus) and the reference lists of the selected articles were searched from inception to June 2018 with no restrictions and filters. The outcome of the study was the prevalence of keratoconus and its risk factors, including eye rubbing, family history of keratoconus, atopy, allergy, asthma, eczema, diabetes type I and type II, and sex. RESULTS:In this study, 3996 articles were retrieved, of which 29 were analyzed. These 29 articles included 7,158,241 participants from 15 countries. The prevalence of keratoconus in the whole population was 1.38 per 1000 population [95% confidence interval (CI): 1.14-1.62 per 1000]. The prevalence of keratoconus was 20.6 per 1000 (95% CI: 11.68-28.44 per 1000) in men and 18.33 per 1000 (95% CI: 8.66-28.00 per 1000) in women in studies reporting sex. The odds ratio of eye rubbing, family history of keratoconus, allergy, asthma, and eczema was 3.09 (95% CI: 2.17-4.00), 6.42 (95% CI: 2.59-10.24), 1.42 (95% CI: 1.06-1.79), 1.94 (95% CI: 1.30-2.58), and 2.95 (95% CI: 1.30-4.59), respectively. CONCLUSIONS:The results of this study, as the most comprehensive meta-analysis of keratoconus prevalence and risk factors, showed that keratoconus had a low prevalence in the world and eye rubbing, family history of keratoconus, allergy, asthma, and eczema were the most important risk factors for keratoconus according to the available evidence. 10.1097/ICO.0000000000002150
    First Experience With the ICD 16.5 Mini-Scleral Lens for Optic and Therapeutic Purposes. Suarez Cyrielle,Madariaga Virginie,Lepage Benoît,Malecaze Marie,Fournié Pierre,Soler Vincent,Galiacy Stéphane,Mély René,Cassagne Myriam,Malecaze François Eye & contact lens OBJECTIVES:To evaluate the success rate, efficacy, and safety of the ICD 16.5 mini-scleral gas permeable (GP) contact lens. METHODS:This prospective study included referred consecutive patients with irregular corneas and severe ocular surface disease (OSD) in treatment failure. All patients were fitted with the ICD 16.5 mini-scleral GP lens. Even though we had some limited experience with scleral lenses, it was our first experience with the ICD 16.5 mini-scleral GP lens. Efficacy was assessed by comparing best-corrected visual acuity (BCVA) with the mini-scleral lens to baseline BCVA. A subjective visual functioning questionnaire (comfort score, visual quality score, handling rating, and wearing time) was administered in a face-to-face structured interview. RESULTS:Thirty-nine eyes of 23 patients with a mean age of 43±16 years were included. Fitting indications were keratoconus (46%), post-penetrating keratoplasty (21%), other irregular astigmatism (15%), and severe OSD (18%). Twenty-five eyes (64%) were successfully fitted with an 18-month follow-up. The mini-scleral GP lens BCVA was 0.16 logarithm of the minimum angle of resolution (logMAR; 20/25) versus a baseline BCVA of 0.44 logMAR (20/63; P<0.001). Comfort and visual quality scores were 8.5/10 and 7.5/10, respectively. No complications were detected in 96% of the eyes (95% confidence interval, 76.1%-99.4%). One eye experienced corneal graft swelling. CONCLUSIONS:The present findings suggest that the ICD 16.5 mini-scleral GP lens is an effective and safe alternative for managing challenging corneas in a therapeutic impasse. 10.1097/ICL.0000000000000293
    Trends in Corneal Transplantation from 2001 to 2016 in Germany: A Report of the DOG-Section Cornea and its Keratoplasty Registry. Flockerzi Elias,Maier Philip,Böhringer Daniel,Reinshagen Helga,Kruse Friedrich,Cursiefen Claus,Reinhard Thomas,Geerling Gerd,Torun Necip,Seitz Berthold, American journal of ophthalmology PURPOSE:The purpose of this retrospective panel study was to provide an overview of absolute numbers and of trends in the types of and indications for corneal transplantation in Germany from 2001 to 2016. METHODS:A questionnaire about absolute numbers, types of transplantation, and indications was sent to 111 ophthalmologic departments in Germany, out of which 94 (85%) provided their data. RESULTS:Since the year 2001, the number of corneal transplantations has increased by 1.5-fold, from 4730 penetrating keratoplasties (PKPs) in 2001 to 7325 penetrating and lamellar keratoplasties in 2016. The shift from penetrating to lamellar procedures began in 2006. In 2014, lamellar procedures (231 [4%] anterior and 2883 [49%] posterior lamellar keratoplasties) surpassed PKPs (2721, 47%) for the first time. Main indications for keratoplasty in Germany (2016) are Fuchs endothelial corneal dystrophy (46%), pseudophakic corneal decompensation (bullous keratopathy, 13%), repeated keratoplasty after graft failure (11%), keratoconus (8%), and corneal scarring (6%; others: 16%). The number of Descemet membrane endothelial keratoplasties (DMEKs) was 12 times higher (3850, 53%) than Descemet stripping automated endothelial keratoplasties (DSAEKs, 319, 4.4%) in 2016. The proportion of deep anterior lamellar keratoplasties (DALKs) never exceeded 6% (269 in 2011). CONCLUSIONS:The number of keratoplasties in Germany has increased from 2001 to 2016. Since 2014, posterior lamellar keratoplasties have surpassed PKPs. There was a constant increase of DMEKs, with a 12-fold higher number compared to DSAEKs in 2016. The shorter recovery time after DMEK seems to contribute to the trend toward earlier operative intervention in corneal endothelial diseases. 10.1016/j.ajo.2018.01.018
    Objective and subjective evaluation of the performance of medical contact lenses fitted using a contact lens selection algorithm. Visser Esther-Simone,Wisse Robert P L,Soeters Nienke,Imhof Saskia M,Van der Lelij Allegonda Contact lens & anterior eye : the journal of the British Contact Lens Association PURPOSE:To evaluate the performance of medical contact lenses (CLs) for a wide range of clinical indications. DESIGN:Prospective cross-sectional study. METHODS:A total of 281 eyes were evaluated in 281 consecutive patients (≥18 years of age; CL use ≥3 months) who visited the contact lens service in a tertiary academic clinic for a scheduled follow-up visit. The main outcome measured were clinical indications for CL wear; CL type; change in corrected distance visual acuity (CDVA) with CL use; CL wearing duration; CL wearing time; subjective performance measured using a visual analog scale (VAS) questionnaire (score range: 0-100); and effectiveness of the lens-selection algorithm. RESULTS:Wearing CLs significantly improved CDVA compared to wearing spectacles (median change: -0.15 logMAR, range: 1.00 to -2.10; P<.001). Daily-wear CLs were worn by 77% of patients for a median of 15h/day (range: 5-18h/day), median 7 days/week (range: 1-7 days/week). High subjective scores were measured, with similar results obtained between the scleral lens and soft lens groups. The medical CL fitting was found to be generally effective (the overall satisfaction rating was ≥70 for 81% of patients). CONCLUSIONS:Fitting CLs based on the lens-selection algorithm yielded positive clinical results, including improved visual acuity, satisfactory wearing time, and high overall subjective performance. Moreover, subjective performance was similar between users of scleral lenses and users of soft lenses. These results underscore the importance of prescribing scleral lenses and the need for tertiary eye clinics to offer patients a variety of CL types. 10.1016/j.clae.2016.02.006
    Impact of corneal cross-linking combined with photorefractive keratectomy on blurring strength. Labiris Georgios,Sideroudi Haris,Angelonias Dimitris,Georgantzoglou Kimonas,Kozobolis Vassilios P Clinical ophthalmology (Auckland, N.Z.) PURPOSE:The aim of this study was to evaluate the impact of corneal cross-linking combined with photorefractive keratectomy (PRK) on blurring strength. METHODS:A total of 63 patients with keratoconus were recruited for this study, and two study groups were formed according to the therapeutic intervention: corneal collagen cross-linking (CxL) group (33 patients) received corneal cross-linking according to the Dresden protocol, while the rest additionally received topography-guided photorefractive keratectomy (tCxL). The impact of surgical procedure on blurring strength was assessed by power vector analysis. Potential association between blurring strength and vision-specific quality of life was assessed using the National Eye Institute Visual Function Questionnaire (NEI-VFQ) 25 instrument. RESULTS:Blurring strength presented excellent correlation with NEI-VFQ scores both preoperatively and postoperatively (all P<0.01). Both groups demonstrated nonsignificant changes in best-corrected visual acuity; however, only the tCxL group had significant reduction in blurring strength (13.48+10.86 [preoperative], 4.26+7.99 [postoperative], P=0.042). CONCLUSION:Only the combined treatment (tCxL) resulted in significant reduction in blurring strength. Moreover, the excellent correlation of blurring strength with NEI-VFQ scores indicates its reliability as an index of self-reported quality of life in keratoconus, since it seems to address the nonsignificant changes in best-corrected visual acuity following CxL treatments that are conceived as subjective improvement by the patient. 10.2147/OPTH.S100770
    New pinhole sulcus implant for the correction of irregular corneal astigmatism. Trindade Claudio C,Trindade Bruno C,Trindade Fernando C,Werner Liliana,Osher Robert,Santhiago Marcony R Journal of cataract and refractive surgery PURPOSE:To evaluate the effect on visual acuity of the implantation of a new intraocular pinhole device (Xtrafocus) in cases of irregular corneal astigmatism with significant visual impairment. SETTING:University of São Paulo, São Paulo, Brazil. DESIGN:Prospective case series. METHODS:Pseudophakic eyes of patients with irregular corneal astigmatism were treated with the pinhole device. The causes of irregular corneal astigmatism were keratoconus, post radial keratotomy (RK), post-penetrating keratoplasty (PKP), and traumatic corneal laceration. The device was implanted in the ciliary sulcus in a piggyback configuration to minimize the effect of corneal aberrations. Preoperative and postoperative visual parameters were compared. The main outcome variables were manifest refraction, uncorrected and corrected distance and near visual acuities, subjective patient satisfaction, and intraoperative and postoperative adverse events and complications. RESULTS:Twenty-one patients (ages 35 to 85 years) were included. There was statistically significant improvement in uncorrected and corrected (CDVA) distance visual acuities. The median CDVA improved from 20/200 (range 20/800 to 20/60) preoperatively to 20/50 (range 20/200 to 20/20) in the first month postoperatively and remained stable over the following months. Manifest refraction remained unchanged, while a subjective visual performance questionnaire revealed perception of improvement in all the tested working distances. No major complication was observed. One case presented with decentration of the device, which required an additional surgical intervention. CONCLUSIONS:The intraocular pinhole device performed well in patients with irregular astigmatism caused by keratoconus, RK, PKP, and traumatic corneal laceration. There was marked improvement in visual function, with high patient satisfaction. 10.1016/j.jcrs.2017.09.014
    Corneal staining patterns in vernal keratoconjunctivitis: the new VKC-CLEK scoring scale. Leonardi Andrea,Lazzarini Daniela,La Gloria Valerio Alvise,Scalora Tania,Fregona Iva The British journal of ophthalmology AIM:To propose a new scoring system in the assessment of ocular surface epithelial damage in vernal keratoconjunctivitis (VKC). METHODS:25 consecutive patients with VKC (50 eyes) were evaluated using the Quality of Life in children with VKC (QUICK) questionnaire and objective clinical measures: fluorescein and lissamine green staining and cornea confocal microscopy (Heidelberg Retina Tomography 3). Oxford, Van Bljsterweld and a new system, the VKC-Collaborative Longitudinal Evaluation of Keratoconus study (CLEK) (VKC-CLEK) scores, were used to evaluate the epithelial damage after staining. RESULTS:Mean Oxford and VKC-CLEK scores were significantly different after fluorescein staining (P<0.001), but significantly correlated (P<0.001; r=0.649). The same data were obtained comparing Van Bljsterweld and VKC-CLEK after lissamine green staining (P<0.001; r=0.760). In patient with limbal VKC, a statistically significant difference was found comparing new VKC-CLEK scores and Oxford or Van Bljsterweld scores (P<0.001), but not in tarsal VKC. A statistically superior concordance was found between QUICK and VKC-CLEK scores compared with standard staining scores values (P<0.001). CONCLUSIONS:Oxford and Van Bijsterveld scores are not adequate for the evaluation of the epithelial damage in patients with limbal VKC because the staining patterns considered for these tests do not correspond to the staining patterns in patients with VKC. We propose a new scoring system, VKC-CLEK, to better evaluate both limbal and tarsal epithelial damage in patients with VKC. 10.1136/bjophthalmol-2017-311171
    Corneal Complications And Visual Impairment In Vernal Keratoconjunctivitis Patients. Arif Abdus Salam,Aaqil Bushra,Siddiqui Afsheen,Nazneen Zainab,Farooq Umer Journal of Ayub Medical College, Abbottabad : JAMC BACKGROUND:Vernal kerato-conjunctivitis (VKC) is an infrequent but serious form of allergic conjunctivitis common in warm and humid areas where air is rich in allergens. It affects both eyes asymmetrically. Although VKC is a self-limiting disease but visions affecting corneal complications influence the quality of life in school children. The aim of this study was to list the corneal complications due to this condition and to find out the extent of visual impairment among VKC patients. METHODS:This cross-sectional study was conducted in the department of Ophthalmology, Benazir Bhutto Shaheed Hospital on 290 eyes of diagnosed cases of VKC. The diagnosis of VKC was made on the basis of history and examination. Visual acuity was recorded using Snellen's notation and visual impairment was classified according to World Health Organization classification for visual disabilities. RESULTS:The mean age of presentation was 10.83±6.13 years. There were 207 (71.4%) males and 83 (28.6%) females. Corneal scarring was observed in 59 (20.3%) eyes. Keratoconus was found to be in 17 (5.9%) eyes. Shield ulcer was detected in 09 (3.1%) eyes while 07 (2.4%) eyes had corneal neovascularization. Majority of the patients with visual loss had corneal scarring and the complication that led to severe visual loss in most of the eyes was Keratoconus. CONCLUSIONS:Vernal kerato-conjunctivitis in the presence of corneal complications is a sight threatening disease and can lead to severe visual impairment.
    Keratoconus: tissue engineering and biomaterials. Karamichos Dimitrios,Hjortdal Jesper Journal of functional biomaterials Keratoconus (KC) is a bilateral, asymmetric, corneal disorder that is characterized by progressive thinning, steepening, and potential scarring. The prevalence of KC is stated to be 1 in 2000 persons worldwide; however, numbers vary depending on size of the study and regions. KC appears more often in South Asian, Eastern Mediterranean, and North African populations. The cause remains unknown, although a variety of factors have been considered. Genetics, cellular, and mechanical changes have all been reported; however, most of these studies have proven inconclusive. Clearly, the major problem here, like with any other ocular disease, is quality of life and the threat of vision loss. While most KC cases progress until the third or fourth decade, it varies between individuals. Patients may experience periods of several months with significant changes followed by months or years of no change, followed by another period of rapid changes. Despite the major advancements, it is still uncertain how to treat KC at early stages and prevent vision impairment. There are currently limited tissue engineering techniques and/or "smart" biomaterials that can help arrest the progression of KC. This review will focus on current treatments and how biomaterials may hold promise for the future. 10.3390/jfb5030111
    ClearKone-Synergeyes or rigid gas-permeable contact lens in keratoconic patients: a clinical decision. Hashemi Hassan,Shaygan Nasim,Asgari Sedigheh,Rezvan Farhad,Asgari Soheila Eye & contact lens OBJECTIVE:To compare the best-corrected visual acuity (BCVA), vision-related quality of life (VRQoL), comfort, foreign body sensation in the eye, tolerance, and handling of ClearKone-Synergeyes hybrid contact lenses and rigid gas-permeable (RGP) lenses in patients with keratoconus. METHODS:This comparative case series was conducted between 2011 and 2012 on 40 keratoconic patients (20 in each group). The BCVA of each patient was evaluated on the same day when the lens was prescribed. The National Eye Institute Visual Functioning Questionnaire (NEI-VFQ 25) questionnaire was assessed in addition to other subjective criteria 2 months after the lens prescription. RESULTS:The BCVA did not show a significant difference between the two lenses. Of the subjective criteria, the scores of the domains of general vision (P=0.008), ocular pain (P<0.001), distance activity (P=0.008), mental health (P<0.001), role difficulty (P<0.001), dependency (P=0.016), driving (P=0.067), total score of the NEI-VFQ 25 questionnaire (P<0.001), and comfort (P<0.001) were significantly higher in the ClearKone-Synergeyes group when compared with the RGP group. Foreign body sensation was statistically higher with RGP lens versus the ClearKone-Synergeyes lens (P=0.013). Regarding tolerance, the preference of the ClearKone-Synergeyes lens over the RGP lens was borderline (P=0.085). CONCLUSION:Although the BCVA did not differ significantly between the two lenses and both corrected vision to the same extent, satisfaction and VRQoL was better in keratoconic patients who used the ClearKone-Synergeyes lens in comparison with the RGP lens. 10.1097/ICL.0000000000000016
    Corneal haze and visual outcome after collagen crosslinking for keratoconus: A comparison between total epithelium off and partial epithelial removal methods. Razmjoo Hasan,Rahimi Behrooz,Kharraji Mona,Koosha Nima,Peyman Alireza Advanced biomedical research BACKGROUND:Keratoconus is an asymmetric, bilateral, progressive noninflammatory ectasia of the cornea that affects approximately 1 in 2000 of the general population. This may cause a significant negative impact on quality of life. Corneal collagen crosslinking (CXL) is one of the recently introduced methods that have been used to decrease the progression of keratoconus, in particular, as well as other corneal-thinning processes. MATERIALS AND METHODS:A total of 44 keratoconic eyes of 22 patients were enrolled in this randomized prospective study, after obtaining informed consent. In the first group, the corneal epithelium were totally removed and in the second group, the central 3 mm of epithelium was kept intact and partial removal was performed. After collagen crosslinking in both groups, comprehensive ophthalmologic examination was performed on all patients before and 6 months after the surgery. This article is registered at www.clinicaltrial.gov with registration number NCT01809977. RESULTS:The difference between the two groups was not statistically significant regarding postoperative corneal haziness, refraction, and visual acuity (P > 0.05). However, comparison of pre- and postoperative parameters within each group revealed that total removal of the cornea has resulted in significant improvement of K-max (P value: 0.01) and Q-value (P value: 0.009); while eyes in partial removal group had better improvement of corrected vision (P value: 0.006). Both methods had significant and similar increase in optical corneal density (P < 0.0001). CONCLUSION:In our study, keeping the central corneal epithelium intact was not beneficial for decreasing corneal haziness, however, this method caused better improvement in corrected vision. Total epithelium off technique resulted in better improvement of K-max and Q-value. 10.4103/2277-9175.145677
    Vision-Related Quality of Life Before and After Deep Anterior Lamellar Keratoplasty. Yildiz Elvin,Toklu Meltem,Turan Vural Ece Eye & contact lens OBJECTIVES:To determine changes in the vision-related quality of life in patients undergoing deep anterior lamellar keratoplasty (DALK) by using the 25-item National Eye Institute Visual Function Questionnaire (NEI VFQ-25). METHODS:Thirty-five patients who were scheduled for DALK between March 1, 2013, and March 1, 2014 were asked to complete the NEI VFQ-25. NEI VFQ-25 was administered again at 6 months and at 1 year postoperatively. Data on patients' age, sex, preoperative diagnosis, preoperative and postoperative best-corrected visual acuity, and postoperative astigmatism were recorded. RESULTS:Successful DALK with the Anwar big bubble technique was achieved in 23 of 35 (65.7%) patients. The indications for surgery were keratoconus in 15 patients (62.2%), stromal corneal dystrophies in 4 (17%), and corneal scar in 4 (17%). The mean preoperative NEI VFQ-25 composite score (55.2±19.7) improved significantly (76.9±11.6) at 6 months after DALK and continued to improve (84.3±6.6) at 1 year postoperatively (Friedman test, P=0.001). All NEI VFQ-25 subscale item scores increased significantly after surgery. The patients' age was significantly correlated with the NEI VFQ-25 subscale score of mental health at 6 month and at 1 year postoperatively (r=0.92, P=0.008 and r=0.94, P=0.005, respectively). There was a negative relationship between postoperative astigmatism at 1 year and NEI VFQ-25 ocular pain, social functioning, peripheral vision, and mental health subscale scores (r=-0.76, P=-0.07; r=-0.53, P=0.2; r=-0.53, P=0.27; r=-0.80, P=0.05). CONCLUSION:Vision-related quality of life improved significantly after DALK and continued to improve after suture removal. 10.1097/ICL.0000000000000359
    Measurement of visual function among patients undergoing corneal transplantation using the VF-14 index in Morocco. Belghmaidi S,Adarmouch L,Baali M,Sebbani M,Hajji I,Amine M,Moutaouakil A Journal francais d'ophtalmologie OBJECTIVE:To validate the Moroccan version of the VF-14 in candidates for keratoplasty and to assess their quality of life using this tool before and after transplantation. METHODS:This is a longitudinal prospective study that assessed 57 patients who underwent penetrating keratoplasty, recruited in the ophthalmology service in Marrakech over 5 years. The original VF-14 questionnaire was adapted into Moroccan dialect. The questionnaire was administered before and 2 years after transplantation. The VF-12 questionnaire was also used, deleting the last two items. Data analysis was performed using SPSS 16.0 software. Comparisons of VF-14 scores before and after transplantation used the Wilcoxon test for paired samples. RESULTS:In total, 57 patients participated in the study. The most frequent indication for surgery was keratoconus (44%). All patients underwent penetrating keratoplasty. Chronbach's alpha value was 0.989 for VF-14 and 0.990 for VF-12. The two scores were negatively correlated with logMAR visual acuity. The strongest correlation was found with VA in the fellow eye. The average best-corrected visual acuity of the eye scheduled for keratoplasty was 1.1±0.16 logMAR. The average of VF-14 was 53±3. After keratoplasty, the average best-corrected visual acuity of operated eye was 0.34±0.31. The average postoperative astigmatism was 3 D. After keratoplasty, an increase in VF-14 score was observed from 53.5 to 81.92 (P<0.001). In postoperative follow-up, graft rejection was noted in two patients and maculopathy in a single patient. DISCUSSION:Graft transparency, absence of complications, minimal astigmatism, and good visual acuity have long been indicators of a successful corneal transplant. However, this does not provide information on visual quality and its impact on everyday life. It is in this sense that the VF-14 was adapted into several languages, as reliable, valid and sensitive as the original Anglo-American version, to assess objectively and subjectively the quality of life of patients after keratoplasty. CONCLUSION:With advances in techniques and availability of better materials, surgical success in performing keratoplasty is increasing. At the same time, vision-related quality of life of corneal graft recipients deserves more attention from ophthalmologists. 10.1016/j.jfo.2016.07.018
    Interaction between topographic/tomographic parameters and dry eye disease in keratoconus patients. Zemova Elena,Eppig Timo,Seitz Berthold,Toropygin Sergey,Arnold Stefan,Langenbucher Achim,Gräber Stefan,Szentmáry Nóra Current eye research PURPOSE:To determine the interaction between corneal topographic and tomographic parameters and dry eye syndrome (DES) in keratoconus (KC) patients. METHODS:Seventy-seven eyes of 49 patients with KC (age 34.4 ± 11.6 years) were enrolled in this study. In these 77 eyes we recorded surface regularity index (SRI), surface asymmetry index (SAI) and Klyce/Maeda KC index (KCI) using the Topographic Modeling System (TMS-5, Tomey, Tennenlohe, Germany), Index of Surface Variance (ISV), Index of Vertical Asymmetry (IVA), KC Index (KI), Center KC Index (CKI), Index of Height Asymmetry (IHA) and Index of Height Decentration (IHD) using Pentacam (Pentacam HR, Oculus, Germany). Patients were subdivided into mild (grade 1-2) and severe stage (grade 3-4) KC groups according to Pentacam grading. To analyse tear film parameters we assessed in 77 KC eyes McMonnies questionnaire, Schirmer test and break-up time and in 26 eyes (10 eyes with mild KC, 16 eyes with severe KC) high-speed videokeratoscopy (during interblinking interval) using a novel commercially not available system (Tear Inspect). With Tear Inspect the analysed tear film parameters were (1) time of first irregularities of Placido rings and (2) time of eyelid closure. Patients were also subdivided into McMonnies questionnaire positive and negative groups. RESULTS:We did not find significant difference between patients with mild and severe KC in any of the examined tear film parameters (p > 0.66). There was no significant difference in SRI, SAI, KCI, ISV, IVA, KI, CKI, IHA and IHD in McMonnies test positive and negative KC patients (p > 0.07). There was no correlation between SRI, SAI, KCI, ISV, IVA, KI, CKI, IHA and IHD and any of the examined tear film parameters (without high-speed videokeratoscopy) neither in 77 KC patients nor in mild or severe KC eyes (r < 0.3). CONCLUSIONS:There is no interaction between DES and topographic/tomographic changes in KC-patients. 10.3109/02713683.2013.798667
    Hydro cone lens visual performance and impact on quality of life in irregular corneas. Ozek Dilay,Kemer Ozlem Evren,Bayraktar Neslihan Contact lens & anterior eye : the journal of the British Contact Lens Association The aim of this study is to evaluate the visual performance (efficiency) of HydroCone (Toris K) soft silicon hydrogel lenses in patients with irregular corneas. 10.1016/j.clae.2016.04.003
    Rigid gas-permeable contact lens related life quality in keratoconic patients with different grades of severity. Wu Ye,Tan Qi,Zhang Wenqiu,Wang Jianglan,Yang Bi,Ma Wei,Wang Xue,Liu Longqian Clinical & experimental optometry PURPOSE:The aim was to compare the impact of rigid gas-permeable (RGP) contact lenses on vision-related quality of life (VR-QOL) in keratoconic patients with different grades of severity. METHODS:This comparative study was conducted from December 2012 to September 2013 on 46 patients with bilateral keratoconus. Patients were divided into three groups according to the average of the steep keratometry (K) readings in the two eyes of each patient. Main outcome measures included binocular visual acuity (VA), lens wearing time, the 25-item National Eye Institute Visual Function Questionnaire (NEI-VFQ-25), foreign body (FB) sensation, comfort and overall satisfaction. RESULTS:Patients with severe keratoconus showed significantly reduced wearing time compared with the other two groups (4.8 ± 2.5 hours per day). Regarding the subjective criteria, there was no significant difference on NEI-VFQ-25 scores, foreign body sensation, comfort and overall satisfaction between mild and moderate keratoconus groups but scores in the group with severe keratoconus were significantly lower than the other two groups. Binocular VA strongly correlated with NEI-VFQ-25 scores; however, NEI-VFQ-25 scores had no significant correlations with different disease severities. CONCLUSIONS:Appropriate correction with RGP lenses contributes to good VR-QOL for keratoconic patients; however, as the disease progresses to a steep keratometric value of more than 52 dioptres (6.50 mm), RGP lenses did not guarantee a relatively good VR-QOL. Other lens options with new designs might bring better life quality for these patients with severe keratoconus. 10.1111/cxo.12237
    Surgical Options for the Refractive Correction of Keratoconus: Myth or Reality. Fernández-Vega-Cueto L,Romano V,Zaldivar R,Gordillo C H,Aiello F,Madrid-Costa D,Alfonso J F Journal of ophthalmology Keratoconus provides a decrease of quality of life to the patients who suffer from it. The treatment used as well as the method to correct the refractive error of these patients may influence on the impact of the disease on their quality of life. The purpose of this review is to describe the evidence about the conservative surgical treatment for keratoconus aiming to therapeutic and refractive effect. The visual rehabilitation for keratoconic corneas requires addressing three concerns: halting the ectatic process, improving corneal shape, and minimizing the residual refractive error. Cross-linking can halt the disease progression, intrastromal corneal ring segments can improve the corneal shape and hence the visual quality and reduce the refractive error, PRK can correct mild-moderate refractive error, and intraocular lenses can correct from low to high refractive error associated with keratoconus. Any of these surgical options can be performed alone or combined with the other techniques depending on what the case requires. Although it could be considered that the surgical option for the refracto-therapeutic treatment of the keratoconus is a reality, controlled, randomized studies with larger cohorts and longer follow-up periods are needed to determine which refractive procedure and/or sequence are most suitable for each case. 10.1155/2017/7589816
    Rose-K versus Soper contact lens in keratoconus: a randomized comparative trial. Gupta Raghav,Sinha Rajesh,Singh Pooja,Sharma Namrata,Tandon Radhika,Titiyal Jeewan S Middle East African journal of ophthalmology PURPOSE:To perform a comparative evaluation of the efficacy and acceptability of Rose-K and Soper contact lenses in Keratoconus. SETTING:Dr. Rajendra Prasad Center for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi. MATERIALS AND METHODS:A randomized comparative clinical trial was performed in keratoconic eyes fitted with Rose-K (Rose-K group) and Soper (Soper group) contact lenses. Patients data were evaluated for best spectacle corrected visual acuity, best contact lens corrected visual acuity (BCLCVA), corneal topography, glare acuity, contrast sensitivity, tear function tests and specular microscopy. Patients were also asked to complete a self-reported comfort questionnaire at each visit. RESULTS:Sixty eyes were randomized to the Rose-K and Soper groups. The two groups were comparable in all the baseline parameters. There was a statistically significant improvement in BCLCVA in both groups at 3 months (P < 0.01, both groups). The difference between in BCLCVA in both groups was not statistically significant. In both groups, there was a significant improvement in the comfort score at 3 months compared to baseline (P < 0.05, both group). The Rose-K group had statistically significantly better scores at 1 and 3 months compared with the Soper group (P = 0.006 and P < 0.001 respectively). Both groups were associated with a significant (P < 0.01), but comparable improvement in glare acuity at 3 months. There was a significant improvement in contrast sensitivity at 3 months in both groups (P < 0.01); the Rose-K group was significantly better than the Soper group at 1 and 3 months (P = 0.001 and 0.002 respectively). The mean number of trial lenses required for fitting Rose-K lens (2.00 ± 0.59) was significantly lower than the Soper lens (3.43 ± 0.82; P < 0.001). CONCLUSION:Both the contact lens designs provide an equal improvement in visual acuity in patients with Keratoconus. However, Rose-K contact lens provides greater comfort, better quality of vision and requires less chair time compared with the Soper lens and hence may possibly have a greater acceptability. 10.4103/0974-9233.124095
    Nonsurgical Procedures for Keratoconus Management. Rico-Del-Viejo L,Garcia-Montero M,Hernández-Verdejo J L,García-Lázaro S,Gómez-Sanz F J,Lorente-Velázquez A Journal of ophthalmology OBJECTIVES:To describe the past 20 years' correction modalities for keratoconus and their visual outcomes and possible complications. METHODS:A review of the published literature related to the visual outcomes and possible complications in the context of keratoconus management using nonsurgical procedures for the last 20 years (glasses and contact lenses) was performed. Original articles that reported the outcome of any correction modalities of keratoconus management were reviewed. RESULTS:The most nonsurgical procedure used on keratoconus management is the contact lens fitting. Soft contact lenses and soft toric contact lenses, rigid gas-permeable contact lenses, piggyback contact lens system, hybrid contact lenses, and scleral and corneoscleral contact lenses form the contemporary range of available lens types for keratoconus management with contact lenses. All of them try to restore the vision, improve the quality of life, and delay surgical procedures in patients with this disease. Complications are derived from the intolerance of using contact lens, and the use of each depends on keratoconus severity. CONCLUSIONS:In the context of nonsurgical procedures, the use of contact lenses for the management of keratoconic patients represents a good alternative to restore vision and improve the quality of live in this population. 10.1155/2017/9707650
    Pain Management in Corneal Collagen Crosslinking for Keratoconus: A Comparative Case Series. Serna-Ojeda Juan Carlos,Santana-Cruz Omar,Quiroz-Casian Natalia,González-Mendoza Edgar,Mercado-Orozco José Luis,Navas Alejandro,Lichtinger Alejandro,Graue-Hernandez Enrique O Journal of ocular pharmacology and therapeutics : the official journal of the Association for Ocular Pharmacology and Therapeutics To compare management of postoperative pain after corneal collagen crosslinking (CXL) with oral gabapentin or ketorolac. Prospective interventional comparative case series in a single center. Patients undergoing epithelium-off (epi-off) or epithelium-on (epi-on) techniques performed by a single surgeon for progressive keratoconus were enrolled and randomly assigned to the ketorolac (10-mg tablets every 8 h) or the gabapentin (300-mg capsules every 8 h) group and instructed to take the medication for the first 3 postoperative days. Using a numeric scale of pain, scores were assessed for current pain (at the time of applying the questionnaire), and average pain over the preceding 24 h. Eye symptoms and systemic adverse events related to oral medication were also assessed. Thirty-seven patients were included, with 22 (10 epi-on and 12 epi-off) assigned to the ketorolac group and 15 (7 epi-on and 8 epi-off) to the gabapentin group. No statistically significant differences were noted on the pain scale between groups at any point of the study, in the median pain scores of patients at the time of applying the questionnaire, nor in the severity of pain during the 24-h period before the assessment. Also, no differences were found among groups for the eye symptoms and the systemic adverse events. The median regression analysis showed no effect of the type of surgery or gender in the severity of pain. Both oral ketorolac and oral gabapentin can be used with similar results for pain and symptomatic control after epi-on or epi-off CXL procedures. 10.1089/jop.2019.0021
    Prevalence of Keratoconus Among a Palestinian Tertiary Student Population. Shehadeh Mohammad M,Diakonis Vasilios F,Jalil Sara A,Younis Rania,Qadoumi Jamal,Al-Labadi Liana The open ophthalmology journal PURPOSE:To screen for keratoconus and potential associated risk factors in a tertiary student population sample. POPULATION AND METHODS:This cross sectional study included 1234 students attending An-Najah National University (Nablus, West Bank, Palestine), that were randomly selected from a total of 20,000 university students. 634 (51.3%) student participants responded by completing a self-administered questionnaire and were assessed by means of corneal topography. Following initial evaluation, participants were referred for Pentacam evaluation if they demonstrated either a mean keratometry of more than 45 diopters, corneal astigmatism of more than 2 diopters and/or if asymmetric topographic patterns were present. Pentacam images were analyzed by an experienced ophthalmologist based on a number of indices and the participants were classified as normal, keratoconus suspects, and keratoconus patients. RESULTS:A total of 620 participants (mean age, 20.1±1.6 years) were included in this study, 379 (61.1%) were females and 241 (38.9%) were males. Nine subjects were diagnosed with keratoconus, demonstrating a prevalence of 1.5%. 52 (8.4%) participants showed at least one abnormal pentacam index, and were considered as KC suspects. CONCLUSION:Keratoconus is a prevalent disease among the tertiary Palestinian student population. This may be related to a combination of genetic and environmental factors. The results of this study signal the need for public health outreach and intervention for keratoconus. 10.2174/1874364101509010172
    Efficacy of topical microemulsion of fatty acids of the ω-3 series on the sub-epithelial corneal nerves regeneration after epithelium-off corneal collagen cross-linking for keratoconus. Cagini Carlo,Messina Marco,Torroni Giovanni,Riccitelli Francesca,Mariniello Marco,Dua Harminder S International ophthalmology PURPOSE:To evaluate efficacy of a microemulsion of fatty acids of the ω-3 series on the regeneration of the sub-epithelial corneal nerve plexus in patients with keratoconus after epi-off cross-linking. METHODS:In this prospective study, we recruited 40 patients, 18 females, mean age 28 years (range 22-37), who were randomly divided in two groups. Group A, 20 patients, after cross-linking were treated with a microemulsion of fatty acids of the ω-3 series. Group B were treated with hyaluronic acid (0.15%)-based tear substitute. Nerve tortuosity, reflectivity and density were examined with in vivo confocal microscopy. Ocular surface disease index (OSDI) questionnaire at the preoperative and at each follow-up visit (1, 3 and 6 months) after treatment was completed. RESULTS:No significant difference between the two groups was noted at 1 month in terms of nerve density and OSDI. A statistically significant difference between the two groups was detected at 3 months in terms of nerve fibers density (6 ± 0.82 in Group A and 1 ± 0.51 in Group B, P = 0.0001). Reflectivity and tortuosity of the fibers did not show significant differences between the two groups at any time point. At 1 month, OSDI average value in group A and in group B was 31.5 ± 1.94 and 30 ± 1.96, at 3 months 13 ± 1.71 and 28 ± 1.83, and at 6 months 10.5 ± 1.87 and 9.0 ± 1.81, respectively. CONCLUSION:The use of a microemulsion of fatty acids appears to ensure a faster regeneration of nerve fibers in patients undergoing epi-off cross-linking. 10.1007/s10792-019-01170-0
    Assessing Scleral Contact Lens Satisfaction in a Keratoconus Population. Bergmanson Jan P G,Walker Maria K,Johnson Leah A Optometry and vision science : official publication of the American Academy of Optometry PURPOSE:To assess perceived comfort and related experiences of adapted keratoconic scleral contact lens (17-18.2 mm) wearers with a history of wearing other contact lens modalities and to compare these subjective clinical effects with previous experiences. METHODS:Twenty-four keratocones were recruited, signed an informed consent approved by the University Internal Review Board, and were asked to complete a survey and a dry eye questionnaire. RESULTS:Previous lens wearing experience included soft, piggy back, and hybrid lenses but predominantly was corneal gas permeable. An overwhelming majority strongly preferred the comfort and vision with the scleral contact lenses. This cohort of keratoconus patients were on average dry eye suspects according to a dry eye questionnaire but contained individuals with either normal or significantly dry eye scores. However, patients stated that they experienced less dryness with their scleral lenses, but slightly more than half of these patients experienced, at least occasionally, midday fogging. CONCLUSIONS:The scleral contact lenses are extremely well accepted by keratoconic patients because of comfort and vision these devices provide. For many patients, they offer further relief from dryness symptoms. However, midday fogging remains a limitation for many wearers. 10.1097/OPX.0000000000000882
    Association between the prevalence of obstructive sleep apnoea and the severity of keratoconus. Naderan Mohammad,Rezagholizadeh Farzaneh,Zolfaghari Masoome,Naderan Morteza,Rajabi Mohammad Taher,Kamaleddin Mohammad Amin The British journal of ophthalmology AIM:The purpose of this study was to determine the association between prevalence of obstructive sleep apnoea (OSA) in patients with keratoconus (KC) and the severity of KC. METHODS:Six-hundred and sixteen patients with KC and 616 patients without KC in the control group were enrolled in this prospective case-control study. Both groups were matched by age, gender, and body mass index (BMI). The Berlin Questionnaire was administered in both groups. Keratometric and topographic measurements of the KC eyes were recorded. RESULTS:Seventy-six (12.3%) and 40 (6.5%) patients were identified as high risk for developing OSA in KC and control groups, respectively (p<0.001). Family history of OSA and BMI were the risk factors for OSA in the KC group, while in the control group the only risk factor for OSA was the patient's gender. Patients with KC with a high risk of OSA had a significantly higher mean K, flat K, steep K (p<0.05), and a thinner corneal thickness (p<0.05). The severity of KC decreased in both OSA groups except for the grade 4 of high risk group which was the second most frequent group after grade 1 (p=0.005). CONCLUSIONS:Our study revealed that patients with KC are at increased risk of developing OSA, and patients with KC who are at higher risk of developing OSA may have more severe KC. 10.1136/bjophthalmol-2015-306665
    Psychological Aspects and Depression in Patients with Symptomatic Keratoconus. Moschos Marilita M,Gouliopoulos Nikolaos S,Kalogeropoulos Chris,Androudi Sofia,Kitsos George,Ladas Dimitrios,Tsatsos Michael,Chatziralli Irini Journal of ophthalmology PURPOSE:To assess the psychological status of keratoconus sufferers and to determine the relationship between depression and visual impairment in this group of patients. METHODS:Fifty-six patients with keratoconus and forty-seven age- and gender-matched healthy control subjects were retroprospectively analyzed. Every participant underwent a complete ophthalmological examination. Keratoconus diagnosis was confirmed with corneal topography and tomography. Zung Depression Inventory Questionnaire and Patient Health Questionnaire-9 (PHQ-9) were completed by everyone. RESULTS:Visual acuity (logMAR 0.53 ±0.30 versus 0.11 ± 0.16), PHQ-9 score (10.20 ± 4.00 versus 5.40 ± 5.01), and Zung score (46.52 ± 8.70 versus 38.53 ± 8.41) showed a statistically significant difference between keratoconus patients and healthy controls ( < 0.001 for all). Worse visual acuity was strongly correlated with older individuals (rho = 0.339, =0.011) and higher PHQ-9 (rho = 0.765, < 0.001) and Zung score (rho = 0.672, < 0.001). CONCLUSION:Depressive disorders appear to be directly associated with keratoconus, both in frequency and intensity. Worse visual acuity and older age could be identified as predictive factors for their emotional status. Moreover, the disease itself could be recognized as an independent risk factor for depression development, underlying the need for close monitoring and supportive management. To the best of our knowledge, our study is the first in the literature to elaborate the association between keratoconus and depression, by assessing two different questionnaires simultaneously. 10.1155/2018/7314308
    Keratoconus Progression in Patients With Allergy and Elevated Surface Matrix Metalloproteinase 9 Point-of-Care Test. Mazzotta Cosimo,Traversi Claudio,Mellace Pierfrancesco,Bagaglia Simone A,Zuccarini Silvio,Mencucci Rita,Jacob Soosan Eye & contact lens PURPOSE:To assess keratoconus (KC) progression in patients with allergies who also tested positive to surface matrix metalloproteinase 9 (MMP-9) point-of-care test. METHODS:Prospective comparative study including 100 stage I-II keratoconic patients, mean age 16.7±4.6 years. All patients underwent an anamnestic questionnaire for concomitant allergic diseases and were screened with the MMP-9 point-of-care test. Patients were divided into two groups: patients KC with allergies (KC AL) and patients KC without allergies (KC NAL). Severity of allergy was established by papillary subtarsal response grade and KC progression assessed by Scheimpflug corneal tomography, corrected distance visual acuity (CDVA) measurement in a 12-month follow-up. RESULTS:The KC AL group included 52 patients and the KC NAL group 48. In the KC AL group, 42/52 of patients (81%) were positive to MMP-9 point-of-care test versus two positive patients in the KC NAL group (4%). The KC AL group data showed a statistically significant decrease of average CDVA, from 0.155±0.11 to 0.301±0.2 logarithm of the minimum angle of resolution (P<0.005) at 12 months; Kmax value increased significantly, from 50.2 D±2.7 to 55.2 D±1.9 on average. The KC NAL group revealed a slight KC progression without statistically significant changes. Pearson correlation test showed a high correlation between Kmax worsening and severity of PSR in the KC AL group. CONCLUSIONS:The study demonstrated a statistically significant progression of KC in patients with concomitant allergies, positive to MMP-9 point-of-care test versus negative. A high correlation between severity of allergy and KC progression was documented. 10.1097/ICL.0000000000000432
    Acute corneal hydrops in keratoconus: a national prospective study of incidence and management. Barsam A,Petrushkin H,Brennan N,Bunce C,Xing W,Foot B,Tuft S Eye (London, England) PurposeTo determine incidence and management of acute corneal hydrops in the UK.MethodsWe used the BOSU report card system to survey cases of acute corneal hydrops in patients with keratoconus that occurred in the UK between November 2009 and December 2010. Ophthalmologists who reported a case were sent an initial questionnaire, with a follow-up questionnaire after 6 months. We collected information on the demographics, complications, changes in visual acuity, and management. The 2011 National Census was used as a source for population and ethnicity in the UK.ResultsThere were 73 incident cases of acute corneal hydrops, with a response to the initial questionnaire for 64 (88%) patients and follow-up data at 6 months for 57 (78%) patients. For the 64 confirmed cases the median (interquartile range) age of onset was 31.9 (23.2, 41.3) years and 48 (75%) of the cases occurred in males. A total of 42 (66%) patients were white, 14 (22%) were South Asian, and 7 (11%) were black. The proportion of South Asian and black patients with acute corneal hydrops was significantly higher than in the general population (P<0.001). The minimum estimated annual incidence of acute corneal hydrops in patients with keratoconus was estimated to be 1.43 (1.10, 1.83) per 1000. At 6 months following acute corneal hydrops a decision to proceed with keratoplasty had been made for 12 (20.3%) patients.ConclusionsThis is the first population-based estimate of the incidence of acute corneal hydrops in keratoconus. 10.1038/eye.2014.333
    Risk factors for keratoconus in Israel: a case-control study. Gordon-Shaag Ariela,Millodot Michel,Kaiserman Igor,Sela Tzahi,Barnett Itzhaki Guy,Zerbib Yaffa,Matityahu Efrat,Shkedi Shira,Miroshnichenko Svetlana,Shneor Einat Ophthalmic & physiological optics : the journal of the British College of Ophthalmic Opticians (Optometrists) PURPOSE:Keratoconus (KC) is a progressive corneal thinning disorder with an uncertain aetiology. Environmental and genetic factors, including consanguinity, eye rubbing and possibly sun exposure, play a role in the aetiology of KC. Here we test for risk factors for KC in an Israeli population with particular emphasis on sun exposure. METHODS:This case-control study included KC patients who were diagnosed at Care Laser Medical Group, a refractive surgery clinic with branches throughout Israel. The control group included age, sex and ethnicity matched individuals who were randomly selected from patients presenting at the clinic for refractive surgery, but without KC. Study subjects were asked to fill out a self-administered questionnaire that included demographic and geographic details, questions on ocular and general health and sun exposure. Conditional logistic regression was used to analyse univariable and multivariable data to identify risk factors for KC. RESULTS:Seventy-three KC patients and 146 controls participated in the study. Univariable analyses demonstrated that eye rubbing [odds ratio (OR) = 3.76], positive family history of KC (OR = 6.10) and parents' education (<12 years, OR = 0.27, 0.23 for father's and mother's education respectively) were significant risk factors for KC. Univariable analyses of sun exposure behaviour during teenage years proved equivocal with some behaviours emerging as protective for KC (wearing a hat outdoors, OR = 3.13) or as risk factors (spending time in the shade, OR = 0.45), while others showed no association [limiting time in the sun (p = 0.51), and wearing sunglasses (p = 0.20)]. Most of the factors that were significant in the univariable analyses, also emerged as statistically significant in the multivariable model (OR = 3.37, 9.68, 0.35, 5.51 for eye rubbing, family history, parental education, wearing a hat outdoors, with the exception of spending time in the shade (p = 0.88). CONCLUSIONS:Eye rubbing, parents' education (as a measure of socio-economic status) and having family members with KC emerged as significant risk factors for KC. The role of sun exposure in KC remains equivocal and warrants further research. 10.1111/opo.12237
    Intrastromal corneal ring segments for treating keratoconus. Zadnik Karla,Money Sarah,Lindsley Kristina The Cochrane database of systematic reviews BACKGROUND:Keratoconus is a degenerative condition of the cornea that profoundly affects vision and vision-specific quality of life. The axial cornea thins and protrudes, resulting in irregularity and, eventually, scarring of the cornea. There are multiple options available for treating keratoconus. Intrastromal corneal ring segments are small, crescent-shaped plastic rings that are placed in the deep, peripheral corneal stroma in order to flatten the cornea. They are made of polymethylmethacrylate (PMMA). The procedure does not involve corneal tissue nor does it invade the central optical zone. Intrastromal corneal ring segments are approved for use when contact lenses or spectacles are no longer adequate. OBJECTIVES:To evaluate the effectiveness and safety of intrastromal corneal ring segments as a treatment for keratoconus. SEARCH METHODS:We searched the Cochrane Central Register of Controlled Trials (CENTRAL) (which contains the Cochrane Eyes and Vision Trials Register) (2018, Issue 1); Ovid MEDLINE; Embase.com; PubMed; Latin American and Caribbean Health Sciences Literature Database (LILACS); ClinicalTrials.gov and the World Health Organization (WHO) International Clinical Trials Registry Platform (ICTRP). We did not implement any date or language restrictions in the electronic search for trials. We last searched the electronic databases on 25 January 2018. SELECTION CRITERIA:Two review authors independently assessed records from the electronic searches to identify randomized controlled trials (RCTs). Disagreements were resolved by discussion. DATA COLLECTION AND ANALYSIS:We planned for two authors to independently review full-text reports, using standard methodological procedures expected by Cochrane. MAIN RESULTS:We found no RCTs comparing intrastromal corneal ring segments with spectacles or contact lenses. AUTHORS' CONCLUSIONS:In the absence of eligible RCTs to review, no conclusions can be drawn. 10.1002/14651858.CD011150.pub2
    Corneal collagen cross-linking for treating keratoconus. Sykakis Evripidis,Karim Rushmia,Evans Jennifer R,Bunce Catey,Amissah-Arthur Kwesi N,Patwary Showrob,McDonnell Peter J,Hamada Samer The Cochrane database of systematic reviews BACKGROUND:Keratoconus is a condition of the eye that affects approximately 1 in 2000 people. The disease leads to a gradual increase in corneal curvature and decrease in visual acuity with consequent impact on quality of life. Collagen cross-linking (CXL) with ultraviolet A (UVA) light and riboflavin (vitamin B2) is a relatively new treatment that has been reported to slow or halt the progression of the disease in its early stages. OBJECTIVES:The objective of this review was to assess whether there is evidence that CXL is an effective and safe treatment for halting the progression of keratoconus compared to no treatment. SEARCH METHODS:We searched the Cochrane Central Register of Controlled Trials (CENTRAL; 2014, Issue 7), Ovid MEDLINE, Ovid MEDLINE In-Process and Other Non-Indexed Citations, Ovid MEDLINE Daily, Ovid OLDMEDLINE (January 1946 to August 2014), EMBASE (January 1980 to August 2014), Latin American and Caribbean Health Sciences Literature Database (LILACS) (1982 to August 2014), Cumulative Index to Nursing and Allied Health Literature (CINAHL) (1982 to August 2014), OpenGrey (System for Information on Grey Literature in Europe) (www.opengrey.eu/), the metaRegister of Controlled Trials (mRCT) (www.controlled-trials.com), ClinicalTrials.gov (www.clinicaltrials.gov) and the World Health Organisation International Clinical Trials Registry Platform (ICTRP) (www.who.int/ictrp/search/en). We used no date or language restrictions in the electronic searches for trials. We last searched the electronic databases on 28 August 2014. SELECTION CRITERIA:We included randomised controlled trials (RCTs) where CXL with UVA light and riboflavin was used to treat people with keratoconus and was compared to no treatment. DATA COLLECTION AND ANALYSIS:Two review authors independently screened the search results, assessed trial quality, and extracted data using standard methodological procedures expected by Cochrane. Our primary outcomes were two indicators of progression at 12 months: increase in maximum keratometry of 1.5 dioptres (D) or more and deterioration in uncorrected visual acuity of more than 0.2 logMAR. MAIN RESULTS:We included three RCTs conducted in Australia, the United Kingdom, and the United States that enrolled a total of 225 eyes and analysed 219 eyes. The total number of people enrolled was not clear in two of the studies. Only adults were enrolled into these studies. Out of the eyes analysed, 119 had CXL (all using the epithelium-off technique) and 100 served as controls. One of these studies only reported comparative data on review outcomes. All three studies were at high risk for performance bias (lack of masking), detection bias (only one trial attempted to mask outcome assessment), and attrition bias (incomplete follow-up). It was not possible to pool data due to differences in measuring and reporting outcomes. We identified a further three unpublished trials that potentially had enrolled a total of 195 participants.There was limited evidence on the risk of progression. Analysis of the first few participants followed up to one year in one study suggested that eyes given CXL were less likely to have an increase in maximum keratometry of 1.5 D or more at 12 months compared to eyes given no treatment, but the confidence intervals (CI) were wide and compatible with no effect or more progression in the CXL group (risk ratio (RR) 0.12, 95% CI 0.01 to 2.00, 19 eyes). The same study reported the number of eyes with an increase of 2 D or more at 36 months in the whole cohort with a RR of 0.03 favouring CXL (95% CI 0.00 to 0.43, 94 eyes). Another study reported "progression" at 18 months using a different definition; people receiving CXL were less likely to progress, but again the effect was uncertain (RR 0.14, 95% CI 0.01 to 2.61, 44 eyes). We judged this to be very low-quality evidence due to the risk of bias of included studies, imprecision, indirectness and publication bias but noted that the size of the potential effect was large.On average, treated eyes had a less steep cornea (approximately 2 D less steep) (mean difference (MD) -1.92, 95% CI -2.54 to -1.30, 94 eyes, 1 RCT, very low-quality evidence) and better uncorrected visual acuity (approximately 2 lines or 10 letters better) (MD -0.20, 95% CI -0.31 to -0.09, 94 eyes, 1 RCT, very low-quality evidence) at 12 months. None of the studies reported loss of 0.2 logMAR acuity. The data on corneal thickness were inconsistent. There were no data available on quality of life or costs. Adverse effects were not uncommon but mostly transient and of low clinical significance. In one trial, 3 out of 12 participants treated with CXL had an adverse effect including corneal oedema, anterior chamber inflammation, and recurrent corneal erosions. In one trial at 3 years 3 out of 50 participants experienced adverse events including mild diffuse corneal oedema and paracentral infiltrate, peripheral corneal vascularisation, and subepithelial infiltrates and anterior chamber inflammation. No adverse effects were reported in the control groups. AUTHORS' CONCLUSIONS:The evidence for the use of CXL in the management of keratoconus is limited due the lack of properly conducted RCTs. 10.1002/14651858.CD010621.pub2
    Short-term Effect of Scleral Lens on the Dry Eye Biomarkers in Keratoconus. Carracedo Gonzalo,Blanco Maria Serramito,Martin-Gil Alba,Zicheng Wang,Alvarez Jesús Carballo,Pintor Jesús Optometry and vision science : official publication of the American Academy of Optometry PURPOSE:To evaluate the most important signs of dry eye, such as osmolarity, inflammation, and diadenosine tetraphosphate (Ap(4)A) concentration before and after wearing scleral lenses for 8 h in keratoconus patients. METHODS:A pilot, experimental, short-term study involved 26 keratoconus patients (average age, 36.95 ± 8.95 years). They voluntarily enrolled in the study at the Optometry Clinic of the Faculty of Optics and Optometry in the University Complutense of Madrid. They were divided into two groups: patients with intrastromal corneal ring, the ICRS group, and patients without ICRS, the keratoconus (KC) group. Ocular Surface Disease Index questionnaire, the Schirmer test without anesthesia, tear break-up time, matrix metalloproteinase 9 (MMP-9) concentration, osmolarity, and Ap4A concentration were evaluated before and after wearing a scleral lens for 8 h. RESULTS:The patients wore the scleral lenses from 6 to 9 h, with a mean of 7.59 ± 0.73 h. The mean scleral lens sag for all patients was 4310 ± 166.31 μm, ranging from 4200 μm to 4800 μm. No significant changes in the Schirmer test and tear break-up time were found for either group. Ocular Surface Disease Index scores were statistically lower after wearing scleral lenses for both groups (p < 0.05). A significantly lower osmolarity and a significant rise of MMP-9 concentration after wearing scleral lenses were found in both groups (p < 0.05). Diadenosine tetraphosphate concentration was lower after wearing the scleral lens in the KC group (p < 0.05) but no significant difference was found for the ICRS group (p > 0.05). CONCLUSIONS:Short-term scleral lens wearing improves the symptomatology and some signs of dry eye, such as osmolarity and Ap4A concentration. The increase of MMP-9 concentration could be caused by tear film stagnation and use of preserved saline. 10.1097/OPX.0000000000000788
    Changes in tear biomarker levels in keratoconus after corneal collagen crosslinking. Recalde Jose Ignacio,Duran Juan Antonio,Rodriguez-Agirretxe Iñaki,Soria Javier,Sanchez-Tena Miguel Angel,Pereiro Xandra,Suarez Tatiana,Acera Arantxa Molecular vision Purpose:The purpose of this work was to analyze the expressions of matrix metalloproteinase 9 (MMP-9), calcyclin (S100A6), and cystatin S (CST4) in the tears of keratoconus (KC) patients. The correlations between the expressions of these proteins and the values of various ocular surface parameters were examined after accelerated corneal crosslinking (A-CXL) with pulsed ultraviolet light. Methods:This prospective, observational study enrolled patients with different grades of KC, scheduled to undergo the A-CXL procedure, as well as healthy subjects. Tear samples were analyzed by employing customized antibody microarray assays for MMP-9, S100A6, and CST4 proteins. The keratometry readings at the maximum keratometry (Kmax) and the simulated keratometry (SimK) values were obtained for examining the postoperative evolution of corneal topography. The state of the ocular surface was evaluated using the results of the Ocular Surface Disease Index (OSDI) questionnaire, tear osmolarity (OSM) test, Schirmer test (SCH), Tear Break Up Time (TBUT), tear clearance (CLR), and fluorescein (FLUO) and lissamine green (LG) corneal staining. Results:A total of 18 patients (22 eyes) and 10 healthy subjects were studied. The concentrations of MMP-9 and S100A6 decreased in tears, from 104.5 ± 78.98 ng/ml and 350.20 ± 478.08 ng/ml before the surgery to 48.7 ± 24.20 ng/ml and 55.70 ± 103.62 ng/ml, respectively, after 12 months of follow up. There were no changes in the CST4 concentration after 12 months of follow up (2202.75 ± 2863.70 versus 2139.68 ±2719.89 ng/ml). When the patients were divided into three groups according to the evolutive stage of KC, the trends for the three biomarkers in each group were the same as in the general group. Basal concentrations of MMP-9 and S100A6 from healthy subjects and KC patients were compared. The levels of MMP-9 and S100A6 in tears were (9.8 ± 5.11 and 104.55 ± 78.98 ng/ml, <0.01; and 11.35 ± 3.18 and 350.26 ± 478.06 ng/ml, respectively, <0.01). This was not the case for CST4, which did not exhibit statistically significant differences between the two groups (2261.94 ± 510.65 and 2176.73 ± 2916.27 ng/ml respectively, p=0.07). Conclusions:A-CXL promoted a decrease in the concentrations of MMP-9 and S100A6 in the tear film. This effect may be related to the restoration of corneal homeostasis and the consequent repair of the tissue damage caused by KC. Moreover, the A-CXL treatment did not produce lasting alterations in the ocular surface, and the values of the evaluated clinical parameters did not change significantly.
    Keratoconus in an adult with 22q11.2 deletion syndrome. Saffra Norman,Reinherz Benjamin BMJ case reports 22q11.2 Deletion syndrome is one of the most common microdeletional syndromes, with an incidence of 1:4000 live-births, and potentially affects every organ in the body. More than 180 associated clinical features have been reported and not one phenotypic feature is present in 100% of cases. Ocular manifestations reported based on early childhood examinations include eyelid hooding, strabismus, posterior embryotoxon, retinal vessel tortuosity and refractive errors. Keratoconus has been reported once before in association with 22q11.2 deletion syndrome in a young adult. We report the second case of keratoconus in association with 22q11.2 deletion syndrome. 10.1136/bcr-2014-203737
    An Update on the Safety and Efficacy of Corneal Collagen Cross-Linking in Pediatric Keratoconus. El Rami Hala,Chelala Elias,Dirani Ali,Fadlallah Ali,Fakhoury Henry,Cherfan Carole,Cherfan George,Jarade Elias BioMed research international Keratoconus is a degenerative disease that affects adolescents and young adults and presents with variable thinning and conical deformation of the corneal apex. The resultant irregular astigmatism can progress to levels that can significantly affect everyday activities and overall quality of life. Therefore, stopping the progression of the disease is an essential part in managing patients with keratoconus. Corneal collagen cross-linking is a minimally invasive procedure that stiffens the anterior corneal stroma by creating strong covalent bonds between collagen fibrils. Over the past decade, many studies have proved its safety and efficacy in halting keratoconus progression in adults. This review of the literature highlights the growing trend towards using this treatment in pediatric keratoconic patients. In children, keratoconus tends to be more severe and fast progression is often encountered requiring closer follow-up intervals. Standard cross-linking shows comparable results in children with a good safety-efficacy profile during follow-up periods of up to three years. Further research is needed to standardize and evaluate transepithelial and accelerated cross-linking protocols as these could be of tremendous help in a population where cooperation and compliance are major issues. 10.1155/2015/257927
    PPIP5K2 and PCSK1 are Candidate Genetic Contributors to Familial Keratoconus. Khaled Mariam Lofty,Bykhovskaya Yelena,Gu Chunfang,Liu Alice,Drewry Michelle D,Chen Zhong,Mysona Barbara A,Parker Emily,McNabb Ryan P,Yu Hongfang,Lu Xiaowen,Wang Jing,Li Xiaohui,Al-Muammar Abdulrahman,Rotter Jerome I,Porter Louise F,Estes Amy,Watsky Mitchell A,Smith Sylvia B,Xu Hongyan,Abu-Amero Khaled K,Kuo Anthony,Shears Stephen B,Rabinowitz Yaron S,Liu Yutao Scientific reports Keratoconus (KC) is the most common corneal ectatic disorder affecting >300,000 people in the US. KC normally has its onset in adolescence, progressively worsening through the third to fourth decades of life. KC patients report significant impaired vision-related quality of life. Genetic factors play an important role in KC pathogenesis. To identify novel genes in familial KC patients, we performed whole exome and genome sequencing in a four-generation family. We identified potential variants in the PPIP5K2 and PCSK1 genes. Using in vitro cellular model and in vivo gene-trap mouse model, we found critical evidence to support the role of PPIP5K2 in normal corneal function and KC pathogenesis. The gene-trap mouse showed irregular corneal surfaces and pathological corneal thinning resembling KC. For the first time, we have integrated corneal tomography and pachymetry mapping into characterization of mouse corneal phenotypes which could be widely implemented in basic and translational research for KC diagnosis and therapy in the future. 10.1038/s41598-019-55866-5
    Visual performance of scleral lenses and their impact on quality of life in patients with irregular corneas. Ozek Dilay,Kemer Ozlem Evren,Altiaylik Pinar Arquivos brasileiros de oftalmologia PURPOSE:We aimed to evaluate the visual quality performance of scleral contact lenses in patients with keratoconus, pellucid marginal degeneration, and post-keratoplasty astigmatism, and their impact on quality of life. METHODS:We included 40 patients (58 eyes) with keratoconus, pellucid marginal degeneration, and post-keratoplasty astigmatism who were examined between October 2014 and June 2017 and fitted with scleral contact lenses in this study. Before fitting scleral contact lenses, we noted refraction, uncorrected distance visual acuity, spectacle-corrected distance visual acuity, uncorrected contrast sensitivity, and spectacle-corrected contrast sensitivity. We performed corneal topography on and applied a questionnaire that included the National Eye Institute Visual Functioning Questionnaire to all participants. We recorded corrected contrast sensitivity and corrected distance visual acuity on the third month after fitting scleral contact lenses and requested that subjects repeat the National Eye Institute Visual Functioning Questionnaire. RESULTS:The mean age of patients was 28.12 ± 13.19 years. Mean logMAR uncorrected distance visual acuity, spectacle-corrected distance visual acuity, and corrected distance visual acuity with scleral contact lenses were 0.91 ± 0.21 (0.40-1.80), 0.57 ± 0.12 (0.10-1.80), and 0.16 ± 0.02 (0.00-1.30), respectively. We observed significantly higher corrected distance visual acuity with scleral contact lenses compared with uncorrected distance visual acuity and spectacle-corrected distance visual acuity (p<0.05). Mean uncorrected contrast sensitivity, spectacle-corrected contrast sensitivity and CCS with scleral contact lenses were 0.97 ± 0.12 (0.30-1.65), 1.16 ± 0.51 (0.30-1.80), and 1.51 ± 0.25 (0.90-1.80), respectively. Significantly higher contrast sensitivity levels were recorded with scleral contact lenses compared with those recorded with uncorrected contrast sensitivity and spectacle-corrected contrast sensitivity (p<0.05). We found the National Eye Institute Visual Functioning Questionnaire overall score for patients with scleral contact lens treatment to be significantly higher compared with that for patients with uncorrected sight (p<0.05). CONCLUSION:Scleral contact lenses are an effective alternative visual correction method for keratoconus, pellucid marginal degeneration, and post-keratoplasty astigmatism. A significant increase in visual acuity and contrast sensitivity can be obtained with scleral contact lenses in patients with irregular corneas. 10.5935/0004-2749.20180089
    The influence of rigid gas permeable lens wear on the concentrations of dinucleotides in tears and the effect on dry eye signs and symptoms in keratoconus. Carracedo Gonzalo,González-Méijome José Manuel,Martín-Gil Alba,Carballo Jesús,Pintor Jesús Contact lens & anterior eye : the journal of the British Contact Lens Association PURPOSE:To evaluate the signs and symptoms of dry eye and dinucleotide secretion in tears of keratoconus patients (KC) and the potential effect of rigid gas permeable (RGP) contact lens wear. METHODS:Twenty-three KC patients and forty control subjects were enrolled in this study. Signs of dry eye including tear volume, tear stability and corneal staining along with symptoms were assessed using the McMonnies questionnaire. Tears were collected using Schirmer strips, and dinucleotide concentrations in collected tears measured using high pressure liquid chromatography. Values obtained in KC and controls were compared. The effect of contact lens wear in KC was also assessed. RESULTS:KC eyes showed a significantly lower tear volume compared to controls, shorter tear break up time (TBUT), higher corneal staining and higher McMonnies dry eye questionnaire scores (p<0.05). When compared with non-wearers, KC contact lens wearers showed significantly higher symptoms, lower Schirmer and TBUT values (p<0.05). Concentration of Ap4A (0.695±0.304μM vs. 0.185±0.178μM) and Ap5A (0.132±0.128μM vs. 0.045±0.036μM) were higher in KC compared to controls (p<0.001) and only Ap4A was statistically higher in RGP wearers compared to non-wearers (0.794±0.478μM vs. 0.417±0.313μM) (p<0.05). CONCLUSION:Signs and symptoms of dry eye as well as concentrations of Ap4A and Ap5A were markedly increased in KC patients compared to controls. Moreover, Ap4A and symptoms of dry eye were statistically higher in RGP wearers compared to non-wearers. This seems to indicate that factors such as RGP contact lens wear might exacerbate the clinical condition of dry eye. 10.1016/j.clae.2016.04.009
    Keratoconus Natural Progression: A Systematic Review and Meta-analysis of 11 529 Eyes. Ferdi Alex C,Nguyen Vuong,Gore Daniel M,Allan Bruce D,Rozema Jos J,Watson Stephanie L Ophthalmology 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 (K), mean keratometry (K), or maximum keratometry (K), 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 K demonstrated more steepening of K at 12 months. The meta-analysis for K demonstrated a significant increase in K 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 K steepening over 12 months for every 10-year increase in age (P = 0.01). Patients were predicted to have 1 D greater K steepening for every 5 D of greater baseline K (P = 0.003). At 12 months, there was a significant increase in the average K 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 K 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 K. 10.1016/j.ophtha.2019.02.029
    Vision related quality of life in patients with keratoconus. Aydin Kurna Sevda,Altun Ahmet,Gencaga Tugba,Akkaya Sezen,Sengor Tomris Journal of ophthalmology Purpose. The purpose of this study is to evaluate the vision related quality of life in patients with keratoconus by using the National Eye Institute Visual Function Questionnaire-25 (NEI-VFQ-25). Methods. Thirty patients presented with keratoconus (keratoconus group) and 30 healthy patients (control group) were included in this study. Twenty patients were using rigid gas permeable and 10 patients were not using contact lenses in keratoconus group. High and low contrast visual acuity and mean K values of the patients were recorded. Each subject completed the 25-item NEI-VFQ-25. Results. All subscales of NEI-VFQ-25 were lower in the keratoconus patients. The difference was more evident in the subscales of general vision, ocular pain, near vision, vision-specific mental health, vision-specific role difficulties, and peripheral vision (P < 0.05). Overall composite score was 75.2 ± 17.2 in the keratoconus group and 93.2 ± 5.6 in the control group (P = 0.00). Contact lens wearers had higher best corrected visual acuity in comparison with noncontact lens wearers (P = 0.028). Patients with low visual acuity (logMAR > 0.4) in the better eye had lower distance vision, social functioning, mental health, and role difficulties. Meanwhile, patients with low visual acuity (logMAR > 0.4) in the worse eye had lower general health scores (P < 0.05). Conclusions. Vision related quality of life was worse in keratoconus patients. Success in the contact lens usage and maintaining higher visual acuity may improve vision related quality of life. 10.1155/2014/694542
    Global consensus on keratoconus and ectatic diseases. Gomes José A P,Tan Donald,Rapuano Christopher J,Belin Michael W,Ambrósio Renato,Guell José L,Malecaze François,Nishida Kohji,Sangwan Virender S, Cornea BACKGROUND:Despite extensive knowledge regarding the diagnosis and management of keratoconus and ectatic corneal diseases, many controversies still exist. For that reason, there is a need for current guidelines for the diagnosis and management of these conditions. PURPOSE:This project aimed to reach consensus of ophthalmology experts from around the world regarding keratoconus and ectatic diseases, focusing on their definition, concepts, clinical management, and surgical treatments. METHODS:The Delphi method was followed with 3 questionnaire rounds and was complemented with a face-to-face meeting. Thirty-six panelists were involved and allocated to 1 of 3 panels: definition/diagnosis, nonsurgical management, or surgical treatment. The level of agreement considered for consensus was two thirds. RESULTS:Numerous agreements were generated in definitions, methods of diagnosing, and management of keratoconus and other ectatic diseases. Nonsurgical and surgical treatments for these conditions, including the use of corneal cross-linking and corneal transplantations, were presented in a stepwise approach. A flowchart describing a logical management sequence for keratoconus was created. CONCLUSIONS:This project resulted in definitions, statements, and recommendations for the diagnosis and management of keratoconus and other ectatic diseases. It also provides an insight into the current worldwide treatment of these conditions. 10.1097/ICO.0000000000000408
    Cost effectiveness of collagen crosslinking for progressive keratoconus in the UK NHS. Salmon H A,Chalk D,Stein K,Frost N A Eye (London, England) BACKGROUND:Keratoconus is a progressive degenerative corneal disorder of children and young adults that is traditionally managed by refractive error correction, with corneal transplantation reserved for the most severe cases. UVA collagen crosslinking is a novel procedure that aims to prevent disease progression, currently being considered for use in the UK NHS. We assess whether it might be a cost-effective alternative to standard management for patients with progressive keratoconus. METHODS:We constructed a Markov model in which we estimated disease progression from prospective follow-up studies, derived costs derived from the NHS National Tariff, and calculated utilities from linear regression models of visual acuity in the better-seeing eye. We performed deterministic and probabilistic sensitivity analyses to assess the impact of possible variations in the model parameters. RESULTS:Collagen crosslinking is cost effective compared with standard management at an incremental cost of £ 3174 per QALY in the base case. Deterministic sensitivity analysis shows that this could rise above £ 33,263 per QALY if the duration of treatment efficacy is limited to 5 years. Other model parameters are not decision significant. Collagen crosslinking is cost effective in 85% of simulations at a willingness-to-pay threshold of £ 30,000 per QALY. CONCLUSION:UVA collagen crosslinking is very likely to be cost effective, compared with standard management, for the treatment of progressive keratoconus. However, further research to explore its efficacy beyond 5 years is desirable. 10.1038/eye.2015.151
    [Quality of life in patients wearing scleral lenses]. Picot C,Gauthier A-S,Campolmi N,Delbosc B Journal francais d'ophtalmologie OBJECTIVE:To evaluate the contribution of scleral lenses in terms of improving the quality of life in the treatment of astigmatism after penetrating keratoplasty or in keratoconus. METHODS:We conducted an observational retrospective study, evaluating quality of life (QOL) of patients who failed to adapt to RPG lenses, fitted with SPOT(®) scleral lenses between October 2007 and March 2011 in the University Hospital of Besançon Department of Ophthalmology. QOL was assessed before and after scleral lens adaptation with the French version of the National Eye Institute Visual Function Questionnaire 25 (NEI-VFQ 25). RESULTS:We included 47 patients (83 eyes) fitted with scleral lenses on one or both eyes: 56 eyes with keratoconus and 27 post-keratoplasty eyes. The average duration of wearing scleral lenses was 18±10months and the average wearing time was 14±3hours per day. The rate of participation in the survey was 86.5% (41 patients). Visual acuity in the better eye progressed from 0.68±0.46 to 0.15±0.17 logMAR at the 6th month after scleral lens adaptation (P<0.0001). The average scores on the NEI-VFQ 25 questionnaire of patients fitted with scleral lenses for at least 6 months were significantly higher than those without scleral lenses, with a global score of 80.2/100 with, versus 48.1/100 without, scleral lenses (P<0.0001). The global score increased by an average of 32.1±4.6 points (-28, 82) (P<0.0001). Statistical analysis found no significant difference in global score between patients in the keratoconus and keratoplasty groups (P>0.05). Scleral lenses showed a significant improvement in quality of life for patients who had failed or are intolerant to conventional rigid gas permeable contact lenses. In our two main optical indications, keratoconus and keratoplasty, they represent an alternative or a step prior to surgery. 10.1016/j.jfo.2014.10.018
    The impact of keratoconus treatment with the Athens Protocol (partial topography-guided photorefractive keratectomy combined with higher-fluence corneal collagen cross-linking) on quality of life: a long-term study. Clinical ophthalmology (Auckland, N.Z.) PURPOSE:The aim of this study was to evaluate the health-related quality of life (HRQoL) before and after the management of keratoconus (KCN) with Combined Topography-Guided Transepithelial partial photorefractive keratectomy (PRK) and corneal Cross-Linking: the Athens Protocol. PATIENTS AND METHODS:Thirty-four consecutive patients treated for progressive KCN were selected for retrospective telephone interview and individual questionnaire submission. A disease-specific HRQoL questionnaire was administered before and after the Athens Protocol intervention, using the following administrative questionnaires: National Eye Institute Visual Function Questionnaire (NEI VFQ) 25/39 and IVI-28. The disease grade was ranked according to the Amsler-Krumeich classification (KCN severity). Reliability of collected data was evaluated with Cronbach's α test. RESULTS:Patients' age ranged from 15 to 51 years at the time of the operation. Mean time before the operation during which the questionnaires were administered was 52±6 months. General visual acuity, psychological well-being, and driving ability showed significant improvement. Younger keratoconic patients tend to have more enhanced psychological status and to cope better with difficulties in everyday activities (prior to operation) than older patients. CONCLUSION:The Athens Protocol treatment has been proven to be beneficial to patients in a large number of aspects. 10.2147/OPTH.S188519
    Current optometric practices and attitudes in keratoconus patient management. Ortiz-Toquero Sara,Martin Raul Contact lens & anterior eye : the journal of the British Contact Lens Association PURPOSE:To compare the current optometric practices and attitudes in the management of keratoconus patients in the UK and Spain. METHODS:An online survey (adapted to optometric practices) was distributed via a newsletter emailed by various professional organizations in the UK and Spain. RESULTS:Four hundred and sixty-four practitioners (126 in the UK; 338 in Spain) who prescribed gas permeable GP contact lenses (CLs) more than once per month (54.8% of UK practitioners and 28.1% of practitioners in Spain; p<0.01) responded to the questionnaire. A combination of multiple factors is considered necessary in the keratoconus detection (79.4% in the UK, 75% in Spain; p=0.68), and the use of classification criteria is considered relevant (67.5% in the UK, 70.7% in Spain; p=0.49). There is a high consensus on the consideration that GP CL fitting is more difficult in keratoconus (79.4% in the UK, 80.5% in Spain; p=0.79) requiring more diagnostic lenses (3.2±1.4 and 3.4±1.2 in the UK and Spain, respectively; p=0.72) than are necessary for healthy eyes. Using corneal topography is uncommon from both countries (38.1% in the UK, 59.8% in Spain; p<0.01), with a similar ophthalmologist referral pattern (at initial diagnosis, 50% in both the UK and Spain; p=1.00). Few cases of co-management with ophthalmologists were noted (no co-management reported by 60.3% in the UK and 72.8% in Spain, p=0.01). CONCLUSION:This study provides initial observations and evidence regarding keratoconus management by optometrists in the UK and Spain and shows similarity in the professional practices and attitudes of practitioners in these two countries. 10.1016/j.clae.2017.03.005
    Contact lens impact on quality of life in keratoconus patients: rigid gas permeable versus soft silicone-hydrogel keratoconus lenses. Yildiz Elvin Hatice,Erdurmus Mesut,Elibol Emine Savran,Acar Banu,Vural Ece Turan International journal of ophthalmology AIM:To determine the impact of rigid gas permeable (RGP) and silicone-hydrogel keratoconus lenses on the quality of life (QoL) in keratoconus (KCN) patients using the self-reported results from the Contact Lens Impact on Quality of Life (CLIQ) Questionnaire. METHODS:From January 2013 to April 2013, 27 consecutive KCN patients who wore RGP contact lenses (conflexair100 UV KE Zeiss-Wöhlk) or soft silicone-hydrogel contact lenses (SHCLs) for KCN (KeraSoft IC- Bausch&Lomb or Hydrocone Toris K-Swiss lens) completed the CLIQ questionnaire. RESULTS:The mean age of 27 patients was 29.6±8.0y. Fifteen patients were RGP user. The groups were comparable with respect to the mean patient age, sex, and mean K values (P=0.1, P=0.8 and P=0.1, respectively). The mean CLIQperson measure was 42.8±5.5 in RGP group and 39.6±5.5 in SHCLs for KCN group (P=0.06). CLIQperson measure was positively correlated with steep K value (r=0.301, P=0.04). When eyes were stratified by visual acuity with contact lenses, the mean CLIQperson measure was 42.01±5.6 in eyes with a visual acuity of 20/20-20/25 (n=44) and 38.4±5.26 in eyes with a visual acuity of 20/32 or less (n=10; P=0.097). CONCLUSION:RGP lenses and SHCLs for KCN have similar impact on QoL. 10.3980/j.issn.2222-3959.2015.05.38
    Pilot Study of Corneal Sensitivity and Its Association in Keratoconus. Mandathara Preeji S,Stapleton Fiona J,Kokkinakis Jim,Willcox Mark D P Cornea PURPOSE:To evaluate corneal sensitivity and its association with other clinical parameters in keratoconus. METHODS:Twenty-four subjects with keratoconus aged between 18 and 65 years were recruited in this cross-sectional study. Ocular symptoms, corneal topography, tear variables such as tear osmolarity, volume and lower tear meniscus height, ocular surface staining, central sensitivity threshold (CST), and corneal subepithelial nerve mapping were obtained. Association between central CST and other clinical variables was examined using the Spearman correlation coefficient. Partial correlation was performed to control for effects of confounding factors. RESULTS:Data from the most severe eye of each subject were included in analyses. Based on the maximum simulated keratometry (Kmax) reading, subjects were graded as having mild (N = 11; K max ≤ 52 D) or severe (N = 13; K max > 52 D) keratoconus. Central corneal sensitivity was lower (ie, increased CST) in the severe keratoconus group compared with that in the mild keratoconus group (median, interquartile range: 1.09; 0.60-19.66 vs. 0.51; 0.39-1.51 g/mm, P = 0.035). In bivariate correlations, reduced corneal sensitivity in keratoconus was associated with age (ρ = 0.42, P = 0.040), disease duration (ρ = 0.49, P = 0.015) and severity (ρ = 0.44; P = 0.032), lower central nerve fiber density (ρ = -0.68, P = 0.014), contact lens wear (ρ = 0.44; P = 0.025), and contact lens tolerance (ρ = 0.46; P = 0.033). After adjusting for contact lens wear, reduced corneal sensitivity was negatively associated with ocular symptoms (ρ = -0.426, P = 0.048) and pain sensitivity (ρ = -0.423, P = 0.045) and positively associated with corneal staining (ρ = 0.52, P = 0.011). CONCLUSIONS:Altered corneal sensitivity in keratoconus affected ocular symptoms and ocular surface health, which may have significant impact on the success of management options for keratoconus. 10.1097/ICO.0000000000001077
    Risk factors and association with severity of keratoconus: the Australian study of Keratoconus. Sahebjada Srujana,Chan Elsie,Xie Jing,Snibson Grant R,Daniell Mark,Baird Paul N International ophthalmology SIGNIFICANCE:Our results show that asthmatic patients tend to have more severe KC and thus close monitoring for disease progression would be advised, and appropriate treatment strategies may be actioned stabilise the condition that may reduce the need for future corneal transplantation. PURPOSE:To explore a wide range of risk factors associated with the severity of keratoconus (KC). METHODS:A cross-sectional study of KC patients was undertaken in Melbourne, Australia. A questionnaire addressing age, gender, educational background, ocular and medical history, smoking and alcohol consumption, and physical examination comprising anthropometric measurements was collected; eye examination was undertaken. The associations between a range of risk factors and the severity of KC were determined using univariate and multivariable linear regression analyses. RESULTS:A total of 260 KC subjects were included in this study. Mean age of subject was 35.5 (SD = 14.8) years and the majority of the subjects were European 171 (68.2%). Initial univariate regression analysis identified the following risk factors at the p < 0.1 level with KC: higher body mass index, smoking cigarettes, diabetes, rheumatoid arthritis and asthma were associated with increased severity of KC, whereas eczema was associated with less severe KC. Following multivariable regression analysis, only asthma remained as a significant risk factor associated with 2.2 diopters (D) steeper average mean keratometry compared to KC subjects having no asthma [p = 0.03; β = 2.18; 95% confidence intervals: 1.22, 4.14]. CONCLUSION:Our study describes the comprehensive assessment of all the known risk factors in a large KC cohort recruited in Australia. Our study has reported asthma as the only risk factor found to be significantly associated with the severity of KC. The results of this study allow us to better understand the aetiology of KC and such knowledge could be useful in instigate systemic management of patients to slow or prevent KC. 10.1007/s10792-020-01644-6
    Vernal keratoconjunctivitis and keratoconus. Wajnsztajn Denise,Solomon Abraham Current opinion in allergy and clinical immunology PURPOSE OF REVIEW:Vernal keratoconjunctivitis (VKC) is a severe allergic inflammatory disease affecting the conjunctiva in children and young adults. Keratoconus (KC) is a progressive corneal disease characterized by thinning of the corneal stroma, increased and asymmetric corneal curvature, with a potential for significant visual deterioration and is one of the most common corneal complications of VKC. We aimed to review the association of these two diseases, with focus on the mechanisms, prevalence, natural history and treatment strategies of KC associated with VKC. RECENT FINDINGS:KC is a common complication of VKC. KC prevalence can be as high as 26.8% among VKC patients, whereas abnormal corneal topography may appear in up to 71% of them. It is more severe and progresses faster in the setting of VKC (P < 0.05), with remarkable visual deterioration and with an increased need for keratoplasty. Crosslinking treatment and corneal transplantation appear to be as effective for KC patients with VKC as compared to the patients without VKC. However, postoperative complications are higher in patients with VKC and demand close monitoring, tight control of local inflammation and prompt awareness with consequent restrain of eye rubbing. SUMMARY:Patients with VKC should be closely monitored for KC. Prompt recognition of VKC and KC allows tight control of KC pathogenesis mechanisms, timely management of KC progression and preservation of vision and quality of life of young patients. 10.1097/ACI.0000000000000765
    Dry Eye Symptoms in Individuals With Keratoconus Wearing Contact Lenses. Shorter Ellen,Harthan Jennifer,Nau Amy,Fogt Jennifer,Cao Dingcai,Schornack Muriel,Nau Cherie Eye & contact lens OBJECTIVES:To assess dry eye symptoms associated with different contact lens modalities in patients with keratoconus using a dry eye questionnaire. METHODS:An online survey was distributed by the National Keratoconus Foundation. The survey asked participants to report demographic characteristics, current optical correction, age at the time of diagnosis of keratoconus, and contact lens history. The 12-item Ocular Surface Disease Index (OSDI) questionnaire was also completed. Data from participants wearing the same contact lens modality bilaterally were analyzed. RESULTS:The survey was completed by 197 individuals wearing the same contact lens modality bilaterally. The average age of participants at the time of the survey was 47.2±14.8 years (range: 15-87 years), and the average age at which keratoconus was diagnosed was 26.1±9.9 years (range: 8-55 years). The mean overall OSDI score of all participants was 40.2±22.8 (range: 0-100). There was no difference in the mean OSDI scores based on current contact lens modality type (F=1.79; n=187; P=0.13). Based on an OSDI score of 33 or higher, 90% of participants reported symptoms indicative of dry eye disease. Scleral lens wearers reported less discomfort on the individual items related to windy and low-humidity conditions. CONCLUSIONS:Individuals with keratoconus, irrespective of contact lens modality, report a high incidence of dry eye symptoms. 10.1097/ICL.0000000000000802
    Impact of keratoconus, cross-linking and cross-linking combined with photorefractive keratectomy on self-reported quality of life. Labiris Georgios,Giarmoukakis Athanassios,Sideroudi Haris,Gkika Maria,Fanariotis Michael,Kozobolis Vassilios Cornea PURPOSE:To assess the effect of stage 1 keratoconus (KC) and of the 2 prevalent KC therapeutic options: (1) corneal collagen cross-linking (CXL) and (2) CXL combined with topography-guided photorefractive keratectomy (t-PRK) on self-reported quality of life (QOL) by means of the 25-item National Eye Institute Visual Function Questionnaire (NEI-VFQ 25). METHODS:Thirty-two patients with bilateral KC stage 1 (KC group) and best-corrected visual acuity of 20/20 in both eyes were prospectively recruited. Of them, 19 KC patients underwent typical CXL treatment (CXL group), whereas 13 underwent CXL combined with t-PRK treatment (tCXL group). Only the eye with the most advanced condition received the treatment. Subjects responded to the NEI-VFQ 25 1 day before the treatment and 1 year postoperatively. Thirty-nine age-matched contact lens users formed the control group. NEI-VFQ 25 subscale and total scores were associated with spherical equivalent, mean keratometry, and a series of Scheimpflug imaging-derived corneal variance indexes by means of the Spearman correlation coefficient and multivariate linear regression analysis. RESULTS:Preoperatively, significant differences were detected between the KC group and the control group in VFQ 25 total and all subscale scores (P < 0.05), except "general health," "color vision," and "peripheral vision" domains. According to the multivariate linear regression analysis, the index of height decentration was the most significant predictor of VFQ 25 total score (b = 0.943; P = 0.016). Postoperatively, significant differences were detected in "mental health" and "dependency" VFQ 25 domains for both the CXL and tCXL groups (P = 0.05). Furthermore, the tCXL group demonstrated significant differences in the "near activities" (P = 0.04), "role limitations" (P = 0.02), and "driving" (P < 0.01) subscale scores. CONCLUSIONS:Our results suggest that KC exerts a significant impact on KC patients' QOL, even in its early stages with normal best-spectacle-corrected visual acuity. Moreover, both CXL and CXL combined with t-PRK seem to exert a beneficial impact on self-reported QOL, suggesting that they should be applied as soon as possible. 10.1097/ICO.0b013e31823cbe85
    [Visual functions and quality of life in patients with keratoconus]. Cesneková T,Skorkovská K,Petrová S,Cermáková S Ceska a slovenska oftalmologie : casopis Ceske oftalmologicke spolecnosti a Slovenske oftalmologicke spolecnosti PURPOSE:To evaluate quality of life and visual functions in patients with keratoconus wearing rigid gas permeable (RGP) contact lenses. METHODS:Keratometry and objective refraction, uncorrected visual acuity, visual acuity with RGP contact lens, high order aberrations and letter contrast sensitivity before and after RGP application were measured in 22 keratoconus patients. Vision-related quality of life was examined by the National Eye Institute Visual Function Questionnaire (NEI-VFQ 25) RESULTS:The average spherical equivalent in the study group was -7.28 (+/- 5.53), the average keratometry was 50.04 D (+/- 4.24) and the average corneal astigmatism -3,44 Dcyl (+/- 2,72). The uncorrected visual acuity was 0.20 (+/- 0.18), the corrected visual acuity 0.78 (+/- 0.22). The improvement of visual acuity after RGP fitting was statistically significant (p < 0.001). The RGP fitting didn't cause significant changes in high order aberrations and in contrast sensitivity. The NEI-VFQ overall score was 79.2. Keratoconus was associated with lower scores in these categories: dependency, mental health and ocular pain. CONCLUSION:Keratoconus is a disease which may have a marked impact on the quality of life beacuse it affects young adults in their active years. The use of RGP correction can significantly improve visual acuity but, at least in our study group, did not lead to significant improvement in contrast sensitivity and corneal aberrations.
    Prevalence of Keratoconus Based on Scheimpflug Imaging: The Raine Study. Chan Elsie,Chong Elaine W,Lingham Gareth,Stevenson Louis J,Sanfilippo Paul G,Hewitt Alex W,Mackey David A,Yazar Seyhan Ophthalmology PURPOSE:To describe the prevalence and systemic associations of keratoconus in young adults in Perth, Western Australia. DESIGN:Cross-sectional study. PARTICIPANTS:One thousand two hundred fifty-nine participants 20 years of age. METHODS:The Raine Study is a multigenerational, longitudinal cohort study based in Perth, Western Australia. This study represents a cross-sectional analysis of the birth cohort on returning for a 20-year follow-up. Participants underwent a detailed ophthalmic examination, including visual acuity assessment and Scheimpflug imaging using the Pentacam (Oculus, Wetzlar, Germany), and completed a health questionnaire. Keratoconus was defined as a Belin/Ambrόsio enhanced ectasia display score of 2.6 or more in either eye based on Pentacam imaging. MAIN OUTCOME MEASURES:Prevalence of keratoconus in this cohort. RESULTS:Of the 1259 participants, 50.8% were women and 85.7% were White. Fifteen participants had keratoconus in at least 1 eye, giving a prevalence of 1.2% (95% confidence interval, 0.7%-1.9%), or 1 in 84. A significant difference was found in best-corrected visual acuity (0.01 logarithm of the minimum angle of resolution vs. -0.05 logarithm of the minimum angle of resolution; P = 0.007), cylinder (1.25 diopters [D] vs. 0.25 D cylinder; P < 0.001) and spherical equivalent (-1.42 D vs. -0.50 D sphere; P = 0.02) on objective refraction, mean keratometry of the steep meridian (45.19 D vs. 43.76 D; P < 0.001), and mean corneal thickness at the thinnest point (475 μm vs. 536 μm; P < 0.001) between those with and without keratoconus. Keratoconus was associated with regular cigarette smoking (38.5% vs. 14.6%; P = 0.04), but showed no association with gender, race, body mass index, use of spectacles or contact lenses, history of allergic eye disease, or pregnancy. CONCLUSIONS:The prevalence of keratoconus in this Australian population-based study of 20-year-old adults was 1.2% (95% confidence interval, 0.7%-1.9%), or 1 in 84, which is one of the highest reported in the world. This has important implications for screening individuals at a younger age so that treatment can be initiated before disease progression. 10.1016/j.ophtha.2020.08.020
    The Save Sight Keratoconus Registry - Optometry Module: an opportunity to use real-world data to advance eye care. Kandel Himal,Downie Laura E,Watson Stephanie L Clinical & experimental optometry There is increasing focus on the value of real-world clinical registry data in multiple medical disciplines, including ophthalmology. However, disease-focused clinical registries that engage optometrists are rare. This paper introduces the Optometry Module of the Save Sight Keratoconus Registry (SSKR) and highlights the potential advantages it can offer to optometrists for improving their quality of patient care and for engaging in research. Optometrists are primary eye care providers and have a major role in providing clinical care to people with keratoconus. The SSKR system has been developed to collects high-quality information on essential clinical parameters including patient-reported outcomes (i.e., quality of life data). The real-world data from the Optometry Module of the SSKR can be analysed to obtain insights into contemporary optometry keratoconus practice, and be used to identify opportunities for improving clinical care. Optometrists' engagement with the registry supports reflective clinical practice through real-time benchmarking. Optometrists can use the registry system to track patient outcomes, and it provides a framework for educating patients about their keratoconus journey. The system also captures details relating to patient adverse events, with subsequent data analysis enabling risk factors for such events to be identified. In summary, the Optometry Module of the SSKR captures real-world clinical evidence that has the potential to inform practice improvement, facilitate safety surveillance and enable outcomes research in keratoconus, all with the ultimate intent of enhancing care for people living with keratoconus. 10.1080/08164622.2021.1924626
    Patient-related barriers to Rigid Gas Permeable (RGP) lens wear among keratoconus patients in Jordan. Bakkar May M,Haddad Mera F,Qadire Mohammad Al Contact lens & anterior eye : the journal of the British Contact Lens Association PURPOSE:To assess potential patient-related barriers to rigid gas permeable (RGP) lens wear for vision rehabilitation in a sample of keratoconus (KC) patients in Jordan. METHODS:Barriers for RGP contact lens wear among KC patients were discussed and specified by a focus group which consisted of five optometrists who are involved in contact lens practice. The focus group addressed potential barriers for RGP lens wear from the patient's perspective. The identified barriers were then used to design the final questionnaire used in the study. The questionnaire consisted of 8 questions which ask the participant to rate, within a 5-points scale (1: strongly disagree to 5: strongly agree), how each suggested item is considered a barrier for RGP lens wear. RESULTS:204 KC patients (86 females, 118 males) were involved in a semi-structured interview to answer the questionnaire. The average age of participants was 30.85 (±8.74) years old and ranged from 18 to 62 years. The average age of diagnosis of KC (±standard deviation) is 21.96 (±7.22) years old with a range of 12-52 years. All participants reported that they had never tried RGP lenses for their vision rehabilitation; however, other management options were reported such as glasses, keratoplasty, collagen cross linking and Intacs. The mean score of total barriers to RGP wear was high at 3.8 (±0.4) (out of 5). The major identified barriers were: lack of awareness of the benefits of RGP lenses, fear of possible lens complications, lack of medical insurance and high cost of the lens. CONCLUSIONS:The study reveals that several barriers were identified as prevalent. Consequently, there is an imperative need to adopt better planning strategies for management protocol of KC in order to assure best eye care service for KC patients in Jordan. 10.1016/j.clae.2017.12.007
    Prospective two-year study of clinical outcomes following epithelium-off pulsed versus continuous accelerated corneal crosslinking for keratoconus. Ziaei Mohammed,Gokul Akilesh,Vellara Hans,Meyer Jay,Patel Dipika,McGhee Charles N J Clinical & experimental ophthalmology IMPORTANCE:Keratoconus is a debilitating condition with a disproportionately high impact on health resources and vision-specific quality of life. BACKGROUND:This study aimed to compare 2-year outcomes of epithelium-off pulsed (p-ACXL) and epithelium-off continuous (c-ACXL) accelerated corneal crosslinking in progressive keratoconus. DESIGN:Prospective, interventional case series. PARTICIPANTS:Eighty eyes of 80 patients were included. METHODS:The visual, refractive and tomographic results of the two crosslinking protocols were compared. MAIN OUTCOME MEASURES:Uncorrected distance visual acuity, corrected distance visual acuity (CDVA), manifest refraction spherical equivalent (MRSE) and maximum keratometry (K ) on corneal tomography assessment. RESULTS:The mean patient age was 22.51 ± 6.12 years (SD) and 22.08 ± 5.72 years in the p-ACXL and c-ACXL groups, respectively. The mean CDVA significantly improved from 0.30 ± 0.16 logMAR at baseline to 0.23 ± 0.17 logMAR at 24 months (P = .04) in the p-ACXL group and from 0.36 ± 0.22 logMAR to 0.26 ± 0.27 logMAR (P = .02) in the c-ACXL group. The mean induced change in MRSE (+1.79 ± 2.30 D vs +0.27 ± 3.19 D, P = .04) and K (-1.75 ± 1.80 D vs -0.39 ± 1.95 D, P = .04) were superior in the c-ACXL group compared to the p-ACXL group at 24 months. No complications were encountered. CONCLUSIONS AND RELEVANCE:In this prospective study, both p-ACXL and c-ACXL treatments were safe methods to halt the progression of keratoconus within a follow-up period of 24 months. c-ACXL appeared to offer superior refractive and tomographic outcomes when compared to p-ACXL but this did not translate into better visual outcomes. 10.1111/ceo.13567
    Effect of Corneal Cross-linking versus Standard Care on Keratoconus Progression in Young Patients: The KERALINK Randomized Controlled Trial. Larkin Daniel F P,Chowdhury Kashfia,Burr Jennifer M,Raynor Mathew,Edwards Matthew,Tuft Stephen J,Bunce Catey,Caverly Emilia,Doré Caroline, Ophthalmology PURPOSE:To examine the efficacy and safety of corneal cross-linking (CXL) for stabilization of progressive keratoconus. DESIGN:Observer-masked, randomized, controlled, parallel-group superiority trial. PARTICIPANTS:Sixty participants 10 to 16 years of age with progressive keratoconus, one eye of each deemed the study eye. METHODS:The study eye was randomized to either CXL plus standard care or standard care alone, with spectacle or contact lens correction as necessary for vision. MAIN OUTCOME MEASURES:The primary outcome was steep keratometry (K2) in the study eye as a measure of the steepness of the cornea at 18 months. Secondary outcomes included keratoconus progression defined as a 1.5-diopter (D) increase in K2, visual acuity, keratoconus apex corneal thickness, and quality of life. RESULTS:Of 60 participants, 30 were randomized to CXL and standard care groups. Of these, 30 patients in the CXL group and 28 patients in the standard care group were analyzed. Mean K2 in the study eye 18 months after randomization was 49.7 D (standard deviation [SD], 3.8 D) in the CXL group and 53.4 D (SD, 5.8 D) in the standard care group. The adjusted mean difference in K2 in the study eye was -3.0 D (95% confidence interval [CI], -4.9 to -1.1 D; P = 0.002), favoring CXL. Adjusted differences between groups in uncorrected and corrected vision favored eyes receiving CXL: -0.31 logarithm of the minimum angle of resolution (logMAR; 95% CI, -0.50 to -0.11 logMAR; P = 0.002) and -0.51 logMAR (95% CI, -1.37 to 0.35 logMAR; P = 0.002). Keratoconus progression in the study eye occurred in 2 patients (7%) randomized to CXL compared with 12 patients (43%) randomized to standard care. The unadjusted odds ratio suggests that on average, patients in the CXL arm had 90% (odds ratio, 0.1; 95% CI, 0.02-0.48; P = 0.004) lower odds of experiencing progression compared with those receiving standard care. CONCLUSIONS:CXL arrests progression of keratoconus in the majority of young patients. CXL should be considered as a first-line treatment in progressive disease. If the arrest of keratoconus progression induced by CXL is sustained in longer follow-up, particular benefit may be derived from avoiding a later requirement for contact lens wear or corneal transplantation. 10.1016/j.ophtha.2021.04.019
    Asymmetry in keratoconus and vision-related quality of life. Jones-Jordan Lisa A,Walline Jeffrey J,Sinnott Loraine T,Kymes Steven M,Zadnik Karla Cornea PURPOSE:To examine the relationship of increased ocular asymmetry over time to vision-related quality of life in keratoconus. METHODS:The subjects were from the Collaborative Longitudinal Evaluation of Keratoconus Study and had complete data on a least 1 scale of the National Eye Institute Visual Function Questionnaire and examination data at baseline and at least 1 follow-up visit. Three measures of disease asymmetry [visual acuity (VA), corneal curvature, and refractive error] and better eye status were assessed. Multilevel models were fit to the data. RESULTS:The analyses were completed using the data from 961 subjects. Six scales on the National Eye Institute Visual Function Questionnaire had adequate variability to the model (distance activity, driving, mental health, near activity, ocular pain, and role difficulties). Refractive error changes were not associated with statistically significant quality-of-life differences. Except for ocular pain, statistically significant, but not clinically meaningful, differences were found for VA changes and corneal curvature changes. For a 0.1-unit logarithm of the minimum angle of resolution of VA change, the quality-of-life scales decreased between 0.20 and 0.99 units. For a 1.00-diopter steepening of corneal curvature, these decreases were on the order of 0.20 to 0.59 units. Changes related to asymmetry were small as well; decreases were on the order of 0.20 to 0.38 units. CONCLUSIONS:Increasing ocular asymmetry and decreases in VA and corneal steepening in the better eye were associated with decreasing vision-related quality of life, although the magnitudes of the changes were not clinically meaningful. Of these 2 disease status indicators, the vision in the better eye had greater effect on the vision-related quality of life. 10.1097/ICO.0b013e31825697c4
    Corneal cross-linking versus conventional management for keratoconus: a lifetime economic model. Lindstrom Richard L,Berdahl John P,Donnenfeld Eric D,Thompson Vance,Kratochvil David,Wong Chiang,Falvey Heather,Lytle Grace,Botteman Marc F,Carter John A Journal of medical economics AIMS:To assess the cost-effectiveness of corneal collagen cross-linking (CXL) versus no CXL for keratoconus in the United States (US). METHODS:A discrete-event microsimulation was developed to assess the cost-effectiveness of corneal cross-linking (CXL, Photrexa + KXL combination product) versus no CXL for patients with keratoconus. The lifetime model was conducted from a US payor perspective. The source for CXL efficacy and safety data was a 12-month randomized, open-label, sham-controlled, multi-center, pivotal trial comparing CXL versus no CXL. Other inputs were sourced from the literature. The primary outcome was the incremental cost per quality-adjusted life year gained. Costs (2019 USD) and effects were discounted 3% annually. The impacts of underlying uncertainty were evaluated by scenario, univariate, and probabilistic analyses. RESULTS:Starting at a mean baseline age of 31 years and considering a mixed population consisting of 80% slow-progressors and 20% fast-progressors, the CXL group was 25.9% less likely to undergo penetrating keratoplasty (PK) and spent 27.9 fewer years in advanced disease stages. CXL was dominant with lower total direct medical costs (-$8,677; $30,994 versus $39,671) and more QALYs (1.88; 21.80 versus 19.93) compared to no CXL. Considering the impact of reduced productivity loss in an exploratory scenario, CXL was associated with a lifetime cost-savings of $43,759 per patient. CXL was cost-effective within 2 years and cost-saving within 4.5 years. LIMITATIONS:Limitations include those that are common to similar pharmacoeconomic models that rely on disparate sources for inputs and extrapolation on short-term outcomes to a long-term analytical horizon. CONCLUSIONS:Keratoconus is a progressive and life-altering disease with substantial clinical, economic, and humanistic consequences. The economic value of cross-linking is maximized when applied earlier in the disease process and/or younger age, and extends to improved work productivity, out-of-pocket costs, and quality of life. 10.1080/13696998.2020.1851556
    [Standardizing the clinical diagnosis and treatment of keratoconus in China]. Shi W Y,Gao H,Li Y [Zhonghua yan ke za zhi] Chinese journal of ophthalmology Keratoconus, which can lead to blindness, is an eye disease characterized by thinning and protrusion of the cornea. Keratoconus often begins during teenage with an incidence rate of about 1∶2 000. As no consensus on the diagnosis and treatment of keratoconus has never been reached in China, many doctors have not acquired a systemic knowledge of the diagnosis and treatment methods, thus various misunderstandings and problems in the clinical diagnosis and treatment of keratoconus still need our attention. By explaining the necessity and principles in the diagnosis and treatment of keratoconus, this article points out that clinicians should make efforts to comprehensively master the methods of clinical diagnosis and treatment of keratoconus and then provide patients with the most suitable treatment according to their different conditions, which are of great importance for the patients to maintain better visual acuity and quality of life for a long period. . 10.3760/cma.j.issn.0412-4081.2019.06.001
    Clinical guidelines for the management of keratoconus patients with gas permeable contact lenses based on expert consensus and available evidence. Ortiz-Toquero Sara,Rodriguez Guadalupe,Martin Raul Current opinion in ophthalmology PURPOSE OF REVIEW:Fitting rigid gas permeable contact lenses (RGP CLs) in keratoconic patients is the most common visual rehabilitation option to improve patients' quality of life, but require long patient and practitioner chair time. The purpose of this review is to provide evidence-based clinical practice guidelines (CPG) in the management of patients with keratoconus with RGP CLs. RECENT FINDINGS:An extensive literature review from 1990 to 2017 identified 354 potentially relevant publications. Fifty-two articles were reviewed and included in the CPG. An international expert panel of eight contact lens practitioners, with vast experience in keratoconus management reviewed and appraised the CPG following the Appraisal of Guidelines for Research and Evaluation II consortium requirements. The developed CPG clearly outlines a strategy for the successful fitting of RGP CLs in patients with keratoconus. This includes how to calculate parameters of the first diagnostic lens, criteria for assessing good fit and a standardized schedule of wear time and follow-up appointments. SUMMARY:The current evidence and consensus-based CPG helps guide clinicians in a successful strategy for fitting RGP CLs in patients with keratoconus. 10.1097/ICU.0000000000000728
    Impact of Collagen Cross-linking on Psychological Distress and Vision and Health-Related Quality of Life in Patients With Keratoconus. Cingu Abdullah Kursat,Bez Yasin,Cinar Yasin,Turkcu Fatih Mehmet,Yildirim Adnan,Sahin Alparslan,Tas Cuma,Sir Aytekin Eye & contact lens OBJECTIVE:To evaluate the changes in psychological distress level and quality of life (QoL) scores of keratoconus (KC) patients 1 year after corneal collagen cross-linking (CXL). METHODS:Observational cross-sectional study. Thirty-three, consecutive progressive KC patients who received CXL treatment were enrolled in the study. All patients were evaluated before and 1 year after CXL ophthalmologically and psychologically. Main outcome measures were the visual, refractive, and topographic changes and the Beck Depression Inventory, State-Trait Anxiety Inventory (STAI), Short Form-36 (SF-36), and The National Eye Institute Visual Function Questionnaire (NEI-VFQ-25). RESULTS:There were significant improvements in the keratometric readings at flat axis, steep axis, the mean keratometric reading, the corneal astigmatism (Kast), and the maximum keratometric reading. State-Trait Anxiety Inventory-II scores, QoL dimensions, such as physical role difficulty, general health, mental health, ocular pain, near activities, distance activities, peripheral vision, and mental health scores, and the composite score of NEI-VFQ-25, were higher after surgery. There was a positive correlation between the changes observed in Kast and mental health; a negative correlation between the changes in Kast and STAI-II, and a negative correlation between the changes in mental health and STAI-II. Change in STAI-II has significantly predicted the improvement observed in general health. CONCLUSIONS:Our results suggested lower trait anxiety and better vision-related and health-related QoL in KC patients 1 year after successful CXL treatment. Better QoL in these patients seems to be related not only with the visual and refractive results but also improvement observed in trait anxiety. 10.1097/ICL.0000000000000129
    Assessment of the impact of keratoconus on vision-related quality of life. Gothwal Vijaya K,Reddy Shailaja P,Fathima Asma,Bharani Seelam,Sumalini Rebecca,Bagga Deepak K,Sudharman Preeji M Investigative ophthalmology & visual science PURPOSE:We determined if the Impact of Vision Impairment (IVI) is a valid questionnaire to measure the vision-related quality of life (VRQoL) in keratoconus patients, and investigated if the VRQoL varied with disease severity in this sample. METHODS:WE RECRUITED 160 CONSECUTIVE BILATERAL KERATOCONUS PATIENTS (MEAN AGE 23.3 YEARS, 63% MALE) FROM AN INDIAN TERTIARY EYE CARE CENTER. PARTICIPANTS UNDERWENT A CLINICAL EXAMINATION AND COMPLETED THE IVI. PARTICIPANTS WERE DIVIDED INTO THREE GROUPS BASED ON THE AVERAGE OF THE STEEP KERATOMETRY (K) READINGS: mild (average Sim K < 45 diopters [D]), moderate (average Sim K 45-52 D), and severe (average Sim K > 52 D). Rasch analysis was used to validate the IVI and the VRQoL scores thus obtained were compared across the disease groups. RESULTS:THE MAJORITY (63%) OF PATIENTS HAD SEVERE, WHILE THE REMAINDER (37%) HAD MODERATE KERATOCONUS. RASCH ANALYSIS DEMONSTRATED THE VALIDITY OF THE IVI TO ASSESS VRQOL THROUGH TWO SUBSCALES: vision-specific functioning (VF) and emotional well-being (EWB). There was no significant difference in VF (mean change -0.16, P = 0.55) and EWB scores (mean change -0.32, P = 0.23) between moderate and severe keratoconus groups. CONCLUSIONS:The revised IVI subscales have interval-level measurement properties, which support their suitability to measure VRQoL in this keratoconus sample. Patients with moderate or severe keratoconus had similar, but higher VRQoL scores as assessed by the revised IVI subscales, indicating lack of impact of the disease on their VRQoL. However, this does not exclude the possibility of finding an impact in other populations. 10.1167/iovs.12-10783
    Both Subjective Emotional Distress and Visual Handicap Correlate with Belin ABCD Classification in the Worse Eye as Measured with the "Keratoconus End-Points Assessment Questionnaire" (KEPAQ). Clinical ophthalmology (Auckland, N.Z.) BACKGROUND:Keratoconus is a disease characterized by progressive corneal distortion and quality of vision. So far, no study using disease-specific scales has evaluated whether different stages of the disease correlate with higher quality of life (QoL) compromise. METHODS:A total of 114 patients with a confirmed diagnosis of Keratoconus were included in this retrospective study. All patients underwent a clinical and a Pentacam evaluation. They were also administered the "Keratoconus End-Points Assessment Questionnaire" (KEPAQ). Belin ABCD criteria were used for Keratoconus classification. "Better eye" was defined as the eye with the lowest maximum keratometry value. Spearman Rank Order Correlation was used to determine the correlation between the different Belin criteria and the KEPAQ scores in both subscales. RESULTS:Mean age was 28.13 ± 11.57 years, with 39.47% of patients being male. Mean score for the KEPAQ-E was 2.33 ± 3.40 Logit, while for the KEPAQ-F, it was 1.85 ± 3.61 Logit. Criteria A (anterior elevation), B (posterior elevation) and D (visual acuity) in the worse eye correlated significantly with a greater decrease in QoL ( < 0.05 for all correlations). No correlation could be found regarding the better eye. CONCLUSION:A greater corneal distortion in the worse eye, as determined by Belin ABCD, is associated with a greater decrease in patient's QoL. Surgical improvement of the worse eye should probably be performed before surgery of the better eye, as it may provide a better response regarding the quality of life improvement. 10.2147/OPTH.S261785
    Transepithelial versus epithelium-off corneal crosslinking for progressive keratoconus. The Cochrane database of systematic reviews BACKGROUND:Keratoconus is the most common corneal dystrophy. It can cause loss of uncorrected and best-corrected visual acuity through ectasia (thinning) of the central or paracentral cornea, irregular corneal scarring, or corneal perforation. Disease onset usually occurs in the second to fourth decade of life, periods of peak educational attainment or career development. The condition is lifelong and sight-threatening. Corneal collagen crosslinking (CXL) using ultraviolet A (UVA) light applied to the cornea is the only treatment that has been shown to slow progression of disease. The original, more widely known technique involves application of UVA light to de-epithelialized cornea, to which a photosensitizer (riboflavin) is added topically throughout the irradiation process. Transepithelial CXL is a recently advocated alternative to the standard CXL procedure, in that the epithelium is kept intact during CXL. Retention of the epithelium offers the putative advantages of faster healing, less patient discomfort, faster visual rehabilitation, and less risk of corneal haze. OBJECTIVES:To assess the short- and long-term effectiveness and safety of transepithelial CXL compared with epithelium-off CXL for progressive keratoconus. SEARCH METHODS:To identify potentially eligible studies, we searched the Cochrane Central Register of Controlled Trials (CENTRAL) (which contains the Cochrane Eyes and Vision Trials Register) (2020, Issue 1); Ovid MEDLINE; Embase.com; PubMed; Latin American and Caribbean Health Sciences Literature database (LILACS); ClinicalTrials.gov; and World Health Organization (WHO) International Clinical Trials Registry Platform (ICTRP). We did not impose any date or language restrictions. We last searched the electronic databases on 15 January 2020. SELECTION CRITERIA:We included randomized controlled trials (RCTs) in which transepithelial CXL had been compared with epithelium-off CXL in participants with progressive keratoconus. DATA COLLECTION AND ANALYSIS:We used standard Cochrane methodology. MAIN RESULTS:We included 13 studies with 723 eyes of 578 participants enrolled; 13 to 119 participants were enrolled per study. Seven studies were conducted in Europe, three in the Middle East, and one each in India, Russia, and Turkey. Seven studies were parallel-group RCTs, one study was an RCT with a paired-eyes design, and five studies were RCTs in which both eyes of some or all participants were assigned to the same intervention. Eleven studies compared transepithelial CXL with epithelium-off CXL in participants with progressive keratoconus. There was no evidence of an important difference between intervention groups in maximum keratometry (denoted 'maximum K' or 'Kmax'; also known as steepest keratometry measurement) at 12 months or later (mean difference (MD) 0.99 diopters (D), 95% CI -0.11 to 2.09; 5 studies; 177 eyes; I = 41%; very low certainty evidence). Few studies described other outcomes of interest. The evidence is very uncertain that epithelium-off CXL may have a small (data from two studies were not pooled due to considerable heterogeneity (I = 92%)) or no effect on stabilization of progressive keratoconus compared with transepithelial CXL; comparison of the estimated proportions of eyes with decreases or increases of 2 or more diopters in maximum K at 12 months from one study with 61 eyes was RR 0.32 (95% CI 0.09 to 1.12) and RR (non-event) 0.86 (95% CI 0.74 to 1.00), respectively (very low certainty). We did not estimate an overall effect on corrected-distance visual acuity (CDVA) because substantial heterogeneity was detected (I = 70%). No study evaluated CDVA gain or loss of 10 or more letters on a logarithm of the minimum angle of resolution (logMAR) chart. Transepithelial CXL may result in little to no difference in CDVA at 12 months or beyond. Four studies reported that either no adverse events or no serious adverse events had been observed. Another study noted no change in endothelial cell count after either procedure. Moderate certainty evidence from 4 studies (221 eyes) found that epithelium-off CXL resulted in a slight increase in corneal haze or scarring when compared to transepithelial CXL (RR (non-event) 1.07, 95% CI 1.01 to 1.14). Three studies, one of which had three arms, compared outcomes among participants assigned to transepithelial CXL using iontophoresis versus those assigned to epithelium-off CXL. No conclusive evidence was found for either keratometry or visual acuity outcomes at 12 months or later after surgery. Low certainty evidence suggests that transepithelial CXL using iontophoresis results in no difference in logMAR CDVA (MD 0.00 letter, 95% CI -0.04 to 0.04; 2 studies; 51 eyes). Only one study examined gain or loss of 10 or more logMAR letters. In terms of adverse events, one case of subepithelial infiltrate was reported after transepithelial CXL with iontophoresis, whereas two cases of faint corneal scars and four cases of permanent haze were observed after epithelium-off CXL. Vogt's striae were found in one eye after each intervention. The certainty of the evidence was low or very low for the outcomes in this comparison due to imprecision of estimates for all outcomes and risk of bias in the studies from which data have been reported. AUTHORS' CONCLUSIONS:Because of lack of precision, frequent indeterminate risk of bias due to inadequate reporting, and inconsistency in outcomes measured and reported among studies in this systematic review, it remains unknown whether transepithelial CXL, or any other approach, may confer an advantage over epithelium-off CXL for patients with progressive keratoconus with respect to further progression of keratoconus, visual acuity outcomes, and patient-reported outcomes (PROs). Arrest of the progression of keratoconus should be the primary outcome of interest in future trials of CXL, particularly when comparing the effectiveness of different approaches to CXL. Furthermore, methods of assessing and defining progressive keratoconus should be standardized. Trials with longer follow-up are required in order to assure that outcomes are measured after corneal wound-healing and stabilization of keratoconus. In addition, perioperative, intraoperative, and postoperative care should be standardized to permit meaningful comparisons of CXL methods. Methods to increase penetration of riboflavin through intact epithelium as well as delivery of increased dose of UVA may be needed to improve outcomes. PROs should be measured and reported. The visual significance of adverse outcomes, such as corneal haze, should be assessed and correlated with other outcomes, including PROs. 10.1002/14651858.CD013512.pub2
    Comparison of Contact Lens Corrected Quality of Vision and Life of Keratoconus and Myopic Patients. Lee Sunghoon,Jung Gangwook,Lee Hyung Keun Korean journal of ophthalmology : KJO PURPOSE:To compare and analyze changes in vision quality, subjective symptoms, and psycho-social satisfaction in keratoconus and myopic patients following the wearing of contact lenses. METHODS:This study enrolled 25 keratoconus and 25 myopic patients with corrected vision over 0.8 according to the Snellen chart due to treatment with contact lenses. Patients were surveyed prior to the wearing of contact lenses, and again after three months of contact lens usage with a questionnaire about quality of vision and life. The changes in visual function, visual symptoms, and psycho-social well-being before and after contact lens usage were analyzed. RESULTS:The keratoconus patients' overall degree of satisfaction was higher than the overall degree of satisfaction of myopic patients, and the motivation for contact lens usage and purpose of contact lens treatment were different in the two groups. Keratoconus patients experienced greater changes in satisfaction, particularly in satisfaction during night activities, short-distance work, and the reading of fine print. Furthermore, they experienced fewer dry eye symptoms but greater foreign body sensations than patients with myopia following treatment with contact lenses. No statistically significant differences in social role functions existed between the two groups. Keratoconus patients had a lower expectation of visual acuity recovery before treatment with contact lenses (Mann-Whitney U-test, p = 0.049) compared to myopic patients, and more anxiety about vision loss following treatment (Wilcoxon signed-rank test, p = 0.018) compared to their level of anxiety about vision loss before treatment with contact lenses. CONCLUSIONS:Although the same treatment was applied, keratoconus and myopic patients experienced different types of discomfort and areas of improvement in contact lens corrected vision. Therefore, not only corrected vision, but also subsequent improvement and discomfort outcomes should be considered by patients when choosing contact lens treatment. For patients with mild keratoconus, contact lens treatment may be an efficacious first treatment modality. 10.3341/kjo.2016.0107
    Pediatric keratoconus - Current perspectives and clinical challenges. Anitha Venugopal,Vanathi Murugesan,Raghavan Anita,Rajaraman Revathi,Ravindran Meenakshi,Tandon Radhika Indian journal of ophthalmology Keratoconus is an ectatic corneal disease characterized by progressive stromal thinning, irregular astigmatism, and defective vision. It can be unilateral or bilateral with asymmetric presentation. It starts at puberty and either progresses rapidly to an advanced stage of the disease or stops in case of delayed onset and slow progression. Pediatric keratoconus is more aggressive than in adults and the management protocols differ because of various rationales such as accelerated progression, advanced stage of disease at the time of diagnosis and co-morbidities. It poses a burden to the society as it affects the quality of life, social, and educational development in children. Hence early diagnosis, recognition of progression, and timely intervention with collagen crosslinking is imperative to arrest the worsening. Association with systemic syndromes and ocular comorbidities can be of concern in pediatric keratoconus. Severe ocular allergy when associated hastens progress and complicates timely intervention of crosslinking treatment and compliance to contact lens wear. Keratoplasty in pediatric keratoconus has good outcomes but can encounter frequent suture-related concerns. This article discusses the epidemiology, etiopathogenesis, clinical challenges, and current perspectives of management of pediatric keratoconus. 10.4103/ijo.IJO_1263_20
    Translation and validation of the "Questionnaire for research on keratoconus results" (KORQ) in the Colombian population. Balparda K,Galarreta-Mira D,Vanegas-Ramírez C M Archivos de la Sociedad Espanola de Oftalmologia OBJECTIVE:It is proposed to carry out a process of translation and adaptation of the "Questionnaire for Research on Keratoconus Results" (KORQ) into Spanish and its validation in a Spanish-speaking population, such as Colombia. This is intended to provide a tool for measuring and monitoring the commitment to quality of life of patients with this corneal ectasia. MATERIAL AND METHODS:A prospective analytical study was carried out, in which 200 subjects diagnosed with keratoconus completed a translated version of the KORQ questionnaire. The internal validity of the questionnaire was evaluated using Cronbach's Alpha, while its construction was evaluated by an Exploratory Factor Analysis, including a Parallel Horn Analysis for the selection of the number of underlying factors. Subsequently, 50 patients were re-selected to complete the questionnaire one week later. The repeatability of the questionnaire was measured by the Spearman test. RESULTS:The KORQ questionnaire in its Spanish version showed some internal validity in both sections. Both sections of the questionnaire showed an essentially unidimensional behaviour. The test-retest repeatability of the test was excellent (Spearman rho> 0.95). CONCLUSIONS:The translated version of the KORQ is valid in Colombian population. 10.1016/j.oftal.2020.05.037
    Impact of keratoconus in the better eye and the worse eye on vision-related quality of life. Sahebjada Srujana,Fenwick Eva K,Xie Jing,Snibson Grant R,Daniell Mark D,Baird Paul N Investigative ophthalmology & visual science PURPOSE:We assessed the impact of keratoconus disease indicators in the better eye and worse eye on quality of life (QoL) using the Vision and Quality of Life Index (VisQoL) multi-attribute utility instrument (MAUI). METHODS:?tlsb -.01w?>Patients with keratoconus completed the six-item VisQoL utility measure. Visual acuity was assessed using a logMAR chart, and corneal thickness and the keratometric values were measured by using Scheimpflug imaging (Pentacam). Four indicators of keratoconus disease status were considered in this study, namely best corrected visual acuity (BCVA), average front corneal curvature (Front Km), thinnest corneal location (TCL), and spherical equivalent refractive error (SE). As keratoconus is an asymmetric condition, we considered the disease parameters separately for the better eye and the worse eye. The association between the four keratoconus indicators and VisQoL utilities was assessed using multivariate linear regression. RESULTS:A total of 170 patients with keratoconus completed the VisQoL. Patients' median age was 33 (IQR = 18) years (range, 14-75 years) and 58% (n = 99) were males. The median VisQoL utility value was 0.60 (IQR, 0.46; range, 0.02-0.99). The VisQoL utilities reduced with increasing age (ρ = -0.18, P = 0.02) and were higher for males (median = 0.65, IQR = 0.49) than females (median = 0.51, IQR = 0.40). In univariate analyses, BCVA in the better and worse eye, and Front Km and TCL in the worse eye were associated with lower VisQol utilities. However, after adjusting for relevant covariates, only BCVA in the better eye remained associated significantly with reduced VisQoL utilities (β = -0.20, P = 0.018). CONCLUSIONS:Using a vision-specific MAUI, our study demonstrated substantial disutility relating to keratoconus. Worse vision in the better eye (but not the worse eye) was associated independently with a reduction in VisQoL utilities, suggesting that considering VisQoL utilities based on vision in the better eye is an important estimate of the impact of keratoconus from the patients' perspective. Treatment and rehabilitation interventions to retard the progression of vision impairment in the better eye resulting from keratoconus would be most efficacious at an early stage to improve QoL outcomes for patients with this disease. 10.1167/iovs.13-12929
    Development and Validation of the "Keratoconus End-Points Assessment Questionnaire" (KEPAQ), a Disease-Specific Instrument for Evaluating Subjective Emotional Distress and Visual Function Through Rasch Analysis. Clinical ophthalmology (Auckland, N.Z.) BACKGROUND:Keratoconus is a disease characterized by progressive distortion of the corneal anatomy, coupled with a decrease in vision. Assessing quality of life (QoL) in keratoconus is essential. So far, no instrument in the world has been designed to evaluate both visual function and emotional distress in this population. The purpose of the following study is to develop and validate the "Keratoconus End-Points Assessment Questionnaire" (KEPAQ) in a population of ectatic patients, the very first disease-specific scale to measure emotional latent traits in keratoconus. METHODS:A last generation, Rasch analysis method was used for scale validation. First, a number of focus groups were carried out to create a pool of potential items. Then, a series of processes (such as "Content Validity Index") was carried out to develop a prior, 20-question version of the KEPAQ. Then, a study including 150 keratoconus patients was performed, followed by a careful Rasch analysis to validate and optimize both sub-scales (Emotional Compromise, KEPAQ-E, and Functional Compromise, KEPAQ-F). RESULTS:Initially, 86 items were considered as potential elements. After test optimization, 20 items were retained. A total of 150 patients with a confirmed diagnosis of keratoconus were included for the Rasch analysis. The mean age was 29.84 ± 9.96 years. In 150 patients, 12.6% had a history of keratoplasty, 46.0% had corneal rings, and 31.3% had crosslinking. For both sub-scales, misfitting items were removed until no misfitting was determined by repetitive Rasch runs. For the final version of the KEPAQ-E sub-scale, variance explained by the model was 62.4% with a dimensional scale. Person Separation Index and Person Number of Strata were 2.43 and 3.57, respectively. For the final version of the KEPAQ-F sub-scale, variance explained by the model was 61.3% with a unidimensional scale. Person Separation Index and Person Number of Strata were 3.19 and 4.59, respectively. Both sub-scales showed excellent Person Reliability. CONCLUSION:The KEPAQ is a robust scale, developed and validated through the latest theoretical models. It shows excellent psychometric properties, which render it extremely useful for both clinical and research use. To date, the KEPAQ is the only disease-specific scale worldwide to evaluate both functional and emotional compromise in keratoconus patients. 10.2147/OPTH.S254370
    Refractive approaches to visual rehabilitation in patients with keratoconus. Kim Kyeong Hwan,Mian Shahzad I Current opinion in ophthalmology PURPOSE OF REVIEW:As keratoconus is a chronic disease affecting young people, vision-related quality of life is often significantly impaired in patients with this disease. However, successful management of keratoconus, including visual rehabilitation strategies, can improve quality of life in these patients. This review will describe clinical approaches that improve vision-related quality of life in patients with stable keratoconus. RECENT FINDINGS:Several types of contact lenses including scleral lenses have been used successfully to manage keratoconus. Eyes with severe keratoconus, even those in which fitting with other types of lenses is challenging, can be successfully fitted with scleral lenses. Although laser ablative procedures, such as photorefractive keratectomy (PRK) have been traditionally contraindicated in patients diagnosed with or suspected of having keratoconus, PRK has been attempted to partially correct refractive errors in keratoconus. Although phototherapeutic keratectomy and radial keratotomy have been reported to be used in eyes with keratoconus, effectiveness and safety results have varied. Implantation of phakic intraocular lenses and intraocular lenses, including toric intraocular lenses, which primarily correct regular astigmatism, with cataract extraction or refractive lens exchange can improve vision-related quality of life in patients with keratoconus by significantly reducing cylinder while improving uncorrected visual acuity. SUMMARY:Appropriate selection and application of treatment options based on consideration of multiple factors will help patients with keratoconus, improving their vision-related quality of life and delaying or avoiding keratoplasty. 10.1097/ICU.0000000000000675
    Psychometric properties of the NEI-RQL-42 questionnaire in keratoconus. McAlinden Colm,Khadka Jyoti,de Freitas Santos Paranhos Juliane,Schor Paulo,Pesudovs Konrad Investigative ophthalmology & visual science PURPOSE:To assess the psychometric properties of the National Eye Institute Refractive Error Quality of Life (NEI-RQL-42) questionnaire in keratoconus and compare these findings to patients with refractive error correction alone. METHODS:The Portuguese version of the NEI-RQL-42 Quality of Life questionnaire was completed by 44 patients who had keratoconus before and after implantation of intracorneal ring segments. Rasch analysis was used to assess the use of response categories, success in measuring a single trait per subscale (unidimensionality), ability to discriminate person ability (precision), and targeting of questions to person quality of life (QoL). RESULTS:Rasch analysis was performed for the questionnaire subscales using stacked preoperative and postoperative data. Three subscales (Symptoms, Dependence on correction, and Suboptimal correction) contained response categories that were not used as intended. Six subscales contained misfitting items indicating multidimensionality. Eleven subscales exhibited inadequate measurement precision. Only the Near vision subscale demonstrated adequate precision with a person separation greater than 2.0. Targeting of items to person QoL was adequate in 11 of the 12 subscales with a mean item location of less than 1 logit. CONCLUSIONS:Only one NEI-RQL-42 subscale (Near vision) performed adequately in keratoconus. Targeting was better in patients with keratoconus than in patients with refractive error correction alone, but 11 of the 12 subscales remain manifestly inadequate. Better instruments exist for measuring patient-reported outcomes in keratoconus. 10.1167/iovs.12-9969
    Development of a Keratoconus-Specific Questionnaire Using Rasch Analysis. Khadka Jyoti,Schoneveld Paul G,Pesudovs Konrad Optometry and vision science : official publication of the American Academy of Optometry PURPOSE:To develop and validate a keratoconus-specific quality of life (QoL) questionnaire: the Keratoconus Outcomes Research Questionnaire (KORQ). METHODS:The study was carried out in three phases. Phase I: content identifications: items were identified based on an extensive literature review, open-ended patient mail survey, and expert consultations. Each item was scored on a visual analog scale (VAS). Phase II: pilot testing using Rasch analysis. Phase III: testing psychometric properties of the final version of the KORQ. RESULTS:Phase I identified 44 items across 3 different content areas: activity limitation (26), symptoms (20), and convenience (8). The 44-item KORQ was self-administered to 158 people with keratoconus. The 44-item KORQ was multidimensional. Unidimensionality was restored by separating items across three content areas (subscales) as identified in phase I. The activity limitation and symptoms subscales demonstrated adequate measurement precision, but convenience (precision, 1.01) did not. Hence, the convenience subscale was discarded. Rasch analysis revealed that the VAS was disordered. The ordering of the VAS was restored by collapsing categories into 4. An iterative Rasch analysis guided item-removal resulted into a 29-item KORQ (18-item activity limitation and 11-item symptoms). The VAS was replaced by a discrete 4-option labeled categorical rating scale, and it was self-administered by 169 people with keratoconus. Both the subscales demonstrated good psychometric properties. The KORQ scores strongly correlated with visual acuity and contrast sensitivity demonstrating its construct validity. CONCLUSIONS:The 29-item KORQ was a psychometrically robust and valid instrument to assess the impact of keratoconus on activity limitation and symptoms. 10.1097/OPX.0000000000001035
    Psychiatric morbidity of patients with keratoconus: A cross-sectional study. Yildiz Mesut,Turhan Semra Akkaya,Yargı Berru,Ergün Serhat,Örnek Erdem,Baz Fatih,Toker Ayşe Ebru Journal of psychosomatic research BACKGROUND:This study aimed to determine the prevalence of psychiatric disorders in patients with keratoconus and the effect of clinical parameters and psychiatric morbidity on quality of life in this patient group. MATERIALS AND METHODS:This cross-sectional study enrolled 94 patients with keratoconus. All patients underwent a complete ophthalmic and psychiatric examination and completed the The National Eye Institute Refractive Error Quality of Life Instrument-42 (NEI-RQL-42), Beck Depression Inventory (BDI) and Beck Anxiety Inventory (BAI) questionnaires. The current diagnosis of psychiatric disorders was determined using the Structured Clinical Interview for DSM-IV (SCID). The impact of disease severity (binocular BCVA ≥0.4 logMAR, steep K reading ≥52, and Amsler-Krumeich grades) on vision-related quality of life was also analyzed. RESULTS:The patients' mean age was 23.9 ± 4.8 (range, 18-40) years. Of the 94 participants 35 (37.2%) had a psychiatric diagnosis, 13 (13.8%) had moderate-severe depression and 20 (21.2%) had moderate-severe anxiety according to the BDI and BAI, respectively. The probability of having a psychiatric disorder was higher if the keratoconus was more severe. Patients with a psychiatric diagnosis scored lower on physical functioning, role limitations due to emotional problems, energy/fatigue; emotional well-being, social functioning and pain subscales of the Short Form-36 (SF-36). Having a SCID-1 psychiatric diagnosis and the presence of a psychiatric disorder did not significantly affect NEI-RQL-42 questionnaire scores. CONCLUSIONS:There was high psychiatric morbidity among patients with keratoconus. Having a psychiatric disorder was associated with lower QoL as measured using the SF-36. 10.1016/j.jpsychores.2021.110384
    Vision related quality of life of patients with keratoconus after keratoplasty at a Tertiary Eye Hospital in Saudi Arabia. European journal of ophthalmology PURPOSE:To assess the vision-related quality of life (VQoL) for patients with advanced keratoconus (KC) after primary keratoplasty. METHODS:This was a cross-sectional study, undertaken at the King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia, in December 2015. Adults (aged 22-75 years) were interviewed via telephone with regard to their VQoL, on the average, 5 years after undergoing primary keratoplasty for advanced KC. VQoL was measured using the Visual Functioning Index (VF-14, Arabic version). The relationship between VQoL and different demographic and clinical factors was evaluated using linear regression. RESULTS:Ninety-five adults participated in the study (54.7 % males; mean age at the interview = 32.05 ± 7.18 years). Eighty-seven (91.6%) of them had undergone PKP. The overall mean VF-14 score was 86.19% (SD: 12.91). VF-14 score was significantly lower for individuals with higher log average K (beta coefficient, -28.32; 95% confidence interval [CI], -55.59 to -1.04;  = 0.042) and hazy graft (beta coefficient, -12.76; 95% CI, -21.16 to -4.36;  = 0.003) adjusting for the effect of keratoplasty technique and BCVA in the better eye at the last follow up. CONCLUSION:This study suggests that most patients with advanced keratoconus had satisfactory VQoL after primary keratoplasty. Certain subgroups may benefit less substantially than others and would need a particular attention. 10.1177/11206721211020648
    Economic impact of keratoconus using a health expenditure questionnaire: A patient perspective. Chan Elsie,Baird Paul N,Vogrin Sara,Sundararajan Vijaya,Daniell Mark D,Sahebjada Srujana Clinical & experimental ophthalmology IMPORTANCE:This is the first study to estimate the lifetime costs associated with keratoconus based on a questionnaire completed by patients and highlights the significant economic burden of the disease. As keratoconus affects individuals from a young age, the study highlights keratoconus as a public health concern. BACKGROUND:Keratoconus is a disorder characterized by corneal steepening and thinning, leading to reduced visual acuity. To date, there have been no studies evaluating the economic costs of keratoconus from a patient's perspective. DESIGN:A randomized cross-sectional study undertaken in Australia where keratoconus subjects were recruited from public and private ophthalmology and optometry clinics. PARTICIPANTS:A total of 100 participants completed the questionnaire: median age was 31 years and 57% were males. METHODS:A keratoconus health expenditure questionnaire was designed to assess direct and indirect expenditures for each individual. MAIN OUTCOME MEASURES:Total direct and indirect costs associated with the condition were calculated along with the estimated lifetime per capita costs. RESULTS:The total cost related to direct and indirect care was estimated to be AUD 3365. By applying our cost data to keratoconus prevalence data for the Australian population, the total cost is estimated to be approximately AUD 44.7 million per year in Australia. CONCLUSIONS AND RELEVANCE:Our results show that the costs associated with the diagnosis and management of keratoconus represent a significant cost to patients. An understanding of this is important not only to individuals and their families, but also health care providers, health insurers and the wider health system. 10.1111/ceo.13704
    The influence of the refractive correction on the vision-related quality of life in keratoconus patients. Ortiz-Toquero Sara,Perez Sofia,Rodriguez Guadalupe,de Juan Victoria,Mayo-Iscar Agustin,Martin Raul Quality of life research : an international journal of quality of life aspects of treatment, care and rehabilitation PURPOSE:The aim of this study was to assess the impact of refractive correction [spectacles vs rigid gas-permeable contact lenses (RGP CLs)] on the vision-related quality of life (VR-QoL) obtained with the standardized questionnaire, NEI-VFQ-25, in keratoconus patients compared with healthy myopic subjects. METHODS:The Spanish version of NEI-VFQ-25 was administered two consecutive times to 25 keratoconus patients (RGP CL wearers) and 25 healthy myopic subjects (RGP and soft CL wearers). The first time was to assess the VR-QoL for spectacle wearing, such as those for refractive correction, and the second time was for CL wearing. RESULTS:Keratoconus patients showed a lower VR-QoL impairment (P < 0.01) than healthy subjects in the total and all subscale score of NEI-VFQ-25 related to wearing spectacles. With CL correction, keratoconus patients showed a VR-QoL improvement with statistically significant differences (P < 0.04) in only four subscales, including distance activities, mental health, color vision and peripheral vision, compared with healthy subjects. In the keratoconus group, compared to spectacle use, CL wear improved the VR-QoL score (P = 0.01) and all subscales except for ocular pain (P < 0.01) and mental health (P = 0.25). CONCLUSIONS:The use of the NEI-VFQ-25 to explore the difference in the VR-QoL between healthy subjects and patients with keratoconus provides further evidence of improved VR-QoL with RGP CL wear compared with spectacles in keratoconus patients. RGP CL management in keratoconus patients could minimize the impact of the disease on the patient's well-being. 10.1007/s11136-015-1117-1
    Vision-Related Quality of Life in Keratoconus: A Save Sight Keratoconus Registry Study. Tan Jeremy C K,Nguyen Vuong,Fenwick Eva,Ferdi Alex,Dinh Amanda,Watson Stephanie L Cornea PURPOSE:To assess vision-related quality of life using the Impact of Vision Impairment Questionnaire (IVI) in patients with keratoconus enrolled in the Save Sight Keratoconus Registry. METHODS:In this cross-sectional study, data on 107 keratoconic patients were collected through a prospectively designed web-based registry from a quaternary referral eye hospital and 2 corneal subspecialty practices. Vision-related quality of life was evaluated using the IVI. Rasch analysis was used to transform the IVI responses into interval-level measures comprising reading, mobility, and emotional well-being subscales. Associations between best-corrected visual acuity (BCVA), maximum simulated keratometry (Kmax), steep keratometry (K2), and pachymetry for each eye and IVI subscale scores were evaluated with univariate (Pearson correlations) and multivariable regression adjusted for age and gender. RESULTS:Of the 107 patients, 37 (34.5%), 41 (38.0%) and 29 (26.9%) had mild, moderate, and severe keratoconus, respectively. On uni- and multivariable analysis, BCVA in the better eye had the strongest association with reading [r = 0.51; 95% confidence interval (CI), 0.35-0.64, P = 0.004] and mobility (r = 0.55; 95% CI, 0.41-0.67, P < 0.001) subscale scores. BCVA in the better and worse eye, both had the joint strongest associations with emotional scores on univariate analysis, but only the latter was significant on multivariable analysis (r = 0.37; 95% CI, 0.20-0.53, P < 0.001). K2 and Kmax in the better eye also displayed significant associations with reading and mobility scores. CONCLUSIONS:In patients with keratoconus, BCVA in the better eye had the strongest correlation with reading and mobility scores, whereas BCVA in the worse eye was significantly correlated with emotional scores. 10.1097/ICO.0000000000001899
    The Keratoconus Outcome Research Questionnaire: A Cross-Cultural Validation Study of the Danish Version. Bak-Nielsen Sashia,Groenborg Therese K,Ivarsen Anders,Hjortdal Jesper Cornea PURPOSE:To evaluate the psychometric properties of the Danish Keratoconus Outcome Research Questionnaire (KORQ). METHODS:Phase 1: Rasch analysis assessed the original Danish KORQ. Phase 2: Reengineering of the Danish KORQ to optimize the psychometric properties and functioning of the KORQ. RESULTS:The KORQ was self-administered by 195 patients. Phase 1 displayed trouble with local dependency and poor item fit. In phase 2, local dependency was addressed, and acceptable item fit was obtained for both subscales by collapsing item pairs into superitems (A6+A7, A9+A17, and S6+S9) and deleting 2 items (S4 and S2). After these corrections, the inter-item range was 0.28 to 0.72 for activity limitation scale (AL-S) and 0.14 to 0.54 for symptoms scale (S-S). Ordered thresholds were present, except in items A11, S5, and S10. No differential item functioning was present. Person separation reliability was 0.93 for AL-S and 0.81 for S-S. The person-item maps showed that the target was slightly above the study population. The maximal corneal curvature was the clinical measure of keratoconus with the highest correlation of 0.33 for AL-S and 0.24 for S-S. Evaluating individual questions showed that patients had the most trouble seeing at distance, driving at night, and wearing rigid gas-permeable lenses, whereas the least troublesome areas were walking up/downstairs, doing household tasks, avoiding objects on their path, and doing their job. CONCLUSIONS:The revised Danish KORQ largely fulfilled the assumptions of the Rasch model and displayed satisfactory psychometric properties. The need to revise the Danish KORQ highlights that cross-cultural validation is of key importance when working with patient-reported outcomes. 10.1097/ICO.0000000000002354
    Psychometric Properties of the Keratoconus Outcomes Research Questionnaire: A Save Sight Keratoconus Registry Study. Kandel Himal,Pesudovs Konrad,Ferdi Alex,Mills Richard,Chen Jern Yee,Watson Adam,Poon Alexander,Downie Laura E,Watson Stephanie L Cornea PURPOSE:The aim of this study was to evaluate the psychometric properties of the Keratoconus Outcomes Research Questionnaire (KORQ) in patients enrolled in the Save Sight Keratoconus Registry. METHODS:A cross-sectional study was conducted utilizing prospectively collected web-based registry data. The psychometric properties of the KORQ were assessed using both classical test theory and Rasch analysis. Andrich group rating scale variant of the Rasch analysis was conducted using Winsteps software, Version 3.92.1. RESULTS:The KORQ was completed by 189 patients with keratoconus (men, 67.7%; white, 69.8%; median age 29 years; better eye median values: visual acuity, 75 LogMAR letters; Kmax, 51.3 D; K2, 46.5 D; thinnest pachymetry, 485 μm). Cronbach's α for the "Activity Limitation" and "Symptoms" scales were 0.95 and 0.91, respectively, with both scales free from floor or ceiling effects. On Rasch analysis, the category thresholds were ordered and well-spaced for both scales. The Activity Limitation scale had excellent psychometric properties including person separation index (3.6), unidimensionality (variance explained, 65.4%), fit statistics (<1.3 MnSq), and measurement range (3.6 logits). Similarly, the Symptoms scale had satisfactory psychometric properties including person separation index (2.5), unidimensionality (variance explained, 54.3%), fit statistics (<1.30 MnSq except for 1 item), and measurement range (2.0 logits). Both scales were well targeted to the population and free of differential item functioning. CONCLUSIONS:The KORQ is a psychometrically robust patient-reported outcome measure for evaluating quality of life parameters in keratoconus. It enables routine collection and monitoring of meaningful patient-reported outcome data in clinical settings, including registries. 10.1097/ICO.0000000000002169
    Vision-related quality of life and dependency in French keratoconus patients: Impact study. Saunier Valentine,Mercier Audrey-Elodie,Gaboriau Thibaut,Malet Florence,Colin Joseph,Fournié Pierre,Malecaze François,Touboul David Journal of cataract and refractive surgery PURPOSE:To assess the quality of life in French keratoconus patients. SETTING:Fifty-seven Keratoconus National Reference Centers across France. DESIGN:Prospective case series. METHODS:Patients completed the National Eye Institute Visual Functioning Questionnaire-25 (NEI VFQ-25) and a French validated questionnaire on disability and dependency from February to June 2012 when they came for an ophthalmic examination at 57 participating centers across France. An ocular examination including refraction, corneal topography, pachymetry, and slitlamp biomicroscopy was performed. The composite or global NEI VFQ-25 score and the proportion of patients who were dependent (defined by the difficulties with activities of daily living) because of keratoconus were the main evaluation criteria in this study. RESULTS:The study comprised 550 keratoconus patients. Women, corrected distance visual acuity worse than 20/40, steep keratometry higher than 52.0 diopters, history of surgery (corneal transplant, intrastromal corneal ring segments, or corneal crosslinking), and more severe keratoconus according to the Amsler-Krumeich classification were associated with an increasingly negative impact on quality of life (overall scores are significantly lower). Moreover, 4.9% of participants reported having changed their jobs because of keratoconus and 7.8% received keratoconus-related disability. Sixty-nine (12.5%) patients reported having difficulties with activities of daily living and are considered dependent. CONCLUSION:Keratoconus was associated with a significant reduction in quality of life but it did not result in social exclusion. 10.1016/j.jcrs.2017.08.024
    Quality of life in stable and progressive 'early-stage' keratoconus patients. Steinberg Johannes,Bußmann Nils,Frings Andreas,Katz Toam,Druchkiv Vasyl,Linke Stephan J Acta ophthalmologica PURPOSE:To analyse the vision-related quality of life (vr-QoL) in stable and progressive keratoconus (KC) patients with a still good visual acuity. METHODS:Combined prospective/cross-sectional study design. The Refractive Status and Vision Profile (RSVP) and the National Eye Institute Visual Functioning - 25 (NEI-25) questionnaire were used in 16 emmetropic, 32 myopic and 56 KC patients, whereby KC patients with a stable (n = 26) and patients with a progressive stage (n = 30) and some of them before and after corneal cross-linking (CXL; n = 10) were included. All patients had a DCVA in at least one eye of ≥0.7 (decimal chart). RESULTS:The analyses revealed a minor decline of the vr-QoL from emmetropes to myopes to early KC patients with a stable disease. Nonetheless, sociological subscales (i.e. 'social functioning', 'role difficulties' and 'dependency') did not display statistically significant differences comparing these groups. In progressive KC, we could demonstrate a statistically significant decline also of these sociological subscales, which did not improve after CXL. CONCLUSION:Due to a still high and almost unaffected vr-QoL in early KC patients and the distinct decline after progression without rehabilitation after CXL, a reconsideration of current strategies to perform CXL only after a progression is diagnosed should be re-evaluated. 10.1111/aos.14564
    Mini-Scleral Lenses Improve Vision-Related Quality of Life in Keratoconus. Kreps Elke O,Pesudovs Konrad,Claerhout Ilse,Koppen Carina Cornea 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. 10.1097/ICO.0000000000002518
    Sequence of events leading to diagnosis of keratoconus and its impact on quality of life. Indian journal of ophthalmology PURPOSE:Early diagnosis of keratoconus (KCN) and corneal collagen cross-linking can ensure that best-corrected visual acuity is preserved. We report the sequence of events leading to the diagnosis of KCN, as well as its impact on quality of life. METHODS:This survey-based study included patients diagnosed with KCN for the first time at our center. Their corneal tomography was analyzed, and they were provided with a proforma and the NEI-VFQ-25 questionnaire and were asked to answer the given set of questions. RESULTS:The study included 328 eyes of 164 patients. At the time of diagnosis, 112 (68.3%) patients were not aware of a disease called "keratoconus." VKC was present in 56 patients, and 92 patients were not aware of the need to avoid eye rubbing. In total, 101 patients gave a history of sleeping more often on the side with worse KCN. The preferred primary point of contact was an optometrist for 45.1% of patients; 51.2% of patients reported never having visited an ophthalmologist. Sixty-four (39%) patients were advised a screening test to rule out KCN before presenting to our center; 42 (71.8%) of these patients did not get it done. Vision-targeted score showed a significant negative correlation with grade of KCN (r value: -0.471) and positive correlation (r value: 0.534) with LogMAR vision. CONCLUSION:KCN is a disease of the young and severely affects the quality of life. Improving awareness of the general public, ensuring timely referral by optometrists, and keeping a high index of suspicion is emphasized. 10.4103/ijo.IJO_399_21
    Both sub-scales of the "Keratoconus End-Points Assessment Questionnaire" (KEPAQ) are unidimensional and reliable. Balparda Kepa,Herrera-Chalarca Tatiana,Torres-Soto Sneider Alexander,Silva-Quintero Laura Andrea Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie BACKGROUND:Measuring quality of life in keratoconus is important and demands for well-constructed instruments and scales. To date, the Keratoconus End-Points Assessment Questionnaire (KEPAQ) is the only disease-specific scale to measure both functional and emotional compromise due to disease. Nevertheless, not much information exists regarding whether both sub-scales of the test show unidimensionality, a necessary condition in well-functioning instruments. METHODS:A sample of patients with a confirmed diagnosis of keratoconus were administered the full version of the KEPAQ. A Rasch analysis and principal component analysis were performed. RESULTS:A total of 249 patients with keratoconus were included. Their average age was 29.19 ± 10.91 years, and 51.40% were male. Mean score for the KEPAQ-E was 2.51 ± 3.29 logit while it was 2.26 ± 3.70 logit for the KEPAQ-F. For both sub-scales, Kaiser Criterion, Scree Plot Criterion, and Variability Criterion demonstrated unidimensionality. Reliability as measured by Cronbach's alpha was 0.85 for the KEPAQ-E and 0.87 for the KEPAQ-F. CONCLUSIONS:The KEPAQ is a robust, well-designed disease-specific questionnaire that shows unidimensionality. It can be reliably used to measure quality of life in keratoconus patients. 10.1007/s00417-020-04802-9
    Both subscales of the Keratoconus End-Points Assessment Questionnaire have excellent test-retest reliability. Balparda Kepa,Galarreta David J,Vanegas-Ramirez Claudia M,Silva-Quintero Laura A,Botero-Giraldo Mariana,Maya-Naranjo Maria I,Pizarro-Marín Paula A Indian journal of ophthalmology Purpose:The keratoconus end-points assessment questionnaire (KEPAQ) is a disease-specific scale designed to evaluate the quality of life in keratoconus patients and provides the measurement of both functional and emotional compromise in keratoconus. It was previously developed, tested, and validated and now we want to evaluate the test-retest reliability of the KEPAQ, in an effort to contribute evidence on its internal consistency and capability of measuring clinical state with minimal inference of random chance. Methods:This is a prospective analytical study, designed to evaluate the test-retest reliability of the KEPAQ through the repeated application of the questionnaire to a group of clinically stable individuals. A number of patients with a confirmed diagnosis of keratoconus underwent double application of the KEPAQ, seven days apart. Mean KEPAQ score was obtained through Rasch analysis, while test-retest reliability was evaluated through Spearman rank-order correlation and intraclass correlation coefficient. Rasch analysis was performed in JMetrik version 4.1.1 (Psychomeasurement Systems LLC; Charlottesville, VA, USA) in a MacBook Air computer running macOS Catalina version 10.15.2 (Apple Inc.; Cupertino, CA, USA). Results:A total of 100 patients were included. For KEPAQ-E, Spearman correlation was R = 0.963 while ICC was 0.981 (95% confidence interval 0.972-0.987). For KEPAQ-F, Spearman correlation was R = 0.921 while ICC was 0.952 (95% confidence interval 0.929-0.968). Conclusion:The KEPAQ is a robust, well-developed, extremely reliable scale which can be confidently used for clinical and research endeavors. 10.4103/ijo.IJO_3569_20
    [Comparative assessment of the quality of life of keratoconus patients before and after intrastromal keratoplasty]. Sheludchenko V M,Osipyan G A,Arestova O N,Dzhalili R A,Khraystin Kh Vestnik oftalmologii Surgical treatment of keratectasia, in addition to its cessation, is aimed at increasing the acuity and quality of vision. This can also significantly affect patient's quality of life. The criteria for assessing the quality of life does not always consider the balance between quantitative indicators and subjective perception, which can depend on the psychological aspect of self-esteem. Purpose - to perform comparative assessment of the quality of life of patients with keratoconus (KK) before and after intrastromal keratoplasty by a newly developed method using an original questionnaire. MATERIAL AND METHODS:The study included 20 patients (14 men and 6 women, with mean age of 33 years, from 21 to 45 years old; 20 eyes) diagnosed with degree II-IV KK, with non-corrected visual acuity of 0.3 or lower. The original questionnaire with three blocks of questions about subjective assessment of the severity of discomfort associated with the disease, expectations of treatment results and its effectiveness was used to study the quality of life of KK patients who underwent surgery. The evaluation method used a five-point grading scale. RESULTS:Despite the inability to objectively assess the result of treatment, patients can generally note the trend for improvement. Especially significant are the increase of visual acuity, improvement of general health, feeling of comfort and more optimistic view of one's future. Comparison of the expectations and treatment results has shown that some therapeutic effects are overestimated by the patients, while some others are underestimated. A priori, they overvalue the effects of treatment (actions associated with visual tasks - reading, writing), but at the same time undervalue the characteristics of overall personal and emotional state (except for spatial safety and comfort), i.e. the characteristics that determine the quality of life. CONCLUSION:The aims of writing a valid and reliable questionnaire have been achieved partially, and its further development requires comparison of the obtained results with psychodiagnostic data, which would reveal the nature of psychological factors that form the quality of life perception in patients with various vision disorders. 10.17116/oftalma202113705140
    Quality-of-Life Improvement After Scleral Lens Fitting in Patients With Keratoconus. Baudin Florian,Chemaly Alicia,Arnould Louis,Barrénéchea Elsa,Lestable Lolita,Bron Alain M,Creuzot-Garcher Catherine Eye & contact lens OBJECTIVES:To assess the quality-of-life improvement after scleral lens fitting in patients with keratoconus. METHODS:We conducted a prospective observational study of all consecutive patients with keratoconus starting scleral lens fitting in 2019, from January 2 to December 27, at the Dijon University Hospital, France. Quality of life was assessed before and after wearing scleral lenses for 3 months through the National Eye Institute Visual Function Questionnaire 25 (NEI-VFQ 25) questionnaire. We investigated factors potentially influencing quality of life: final visual acuity, keratoconus stage, and maximum keratometry reading. RESULTS:In total, 41 eyes of 24 patients were successfully fitted with scleral lenses, 92.9% were 18 mm in diameter and 7.1% were 17 mm. The visual acuity improved by -0.4±0.3 logarithm of the minimum angle of resolution (P<0.001) after scleral lens fitting. Visual gain increased with keratometry and keratoconus severity (P<0.001; P<0.01). The NEI-VFQ 25 score increased by 19.5±19.1 points on average (P<0.001). No association was found between the factors under study and improvement in quality of life. CONCLUSION:Our study confirms the functional improvement in patients with keratoconus fitted with scleral lenses, especially in patients with more advanced disease. Moreover, it suggests a major role of these lenses in improving quality of life, independently of the severity of the disease and the visual gain. 10.1097/ICL.0000000000000821
    Evaluation of vision-related quality of life in keratoconus patients, and associated impact of keratoconus severity indicators. Panthier Christophe,Moran Sarah,Bourges Jean Louis Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie PURPOSE:To evaluate vision-related quality of life in keratoconus patients and associated impact of keratoconus severity indicators using the National Eye Institute Visual Function Questionnaire (NEI-VFQ-25). METHODS:This was a prospective cross-sectional study carried out from November 1, 2014, to April 30, 2015, in the corneal service of the Hôtel-Dieu hospital, Paris, France. A hundred and one keratoconus patients were consecutively enrolled. Participants completed a French-validated version of the National Eye Institute Visual Function Questionnaire (NEI-VFQ-25). Manifest refraction, maximum keratometry value, and corneal thinnest point were recorded. Associations between clinical and demographic factors, previous medical or surgical treatment, and NEI-VFQ-25 scale scores were evaluated. RESULTS:Vision-related quality of life was no better in patients managed with rigid gas permeable contact lens, collagen cross-linking, or intracorneal ring segment implantation, compared with untreated patients. In advanced keratoconus (stages II, III, and IV of Amsler-Krumeich classification), rigid gas permeable contact lens wearers had better general vision but more ocular pain. Distance-corrected visual acuity worse than 20/40, mean refractive cylinder > 2.5 diopters, and corneal thinnest point < 460 μm in the better eye were associated with a lower vision-related quality of life. In multivariate analysis, only the uncorrected and distance-corrected visual acuity of the better eye remained significantly correlated with vision-related quality of life. CONCLUSIONS:Low distance-corrected visual acuity in the better eye was the strongest predictor of low vision-related quality of life. 10.1007/s00417-020-04680-1
    The Italian version of the Keratoconus Outcomes Research Questionnaire (KORQ): Translation and validation of psychometric properties. European journal of ophthalmology PURPOSE:To develop and validate the Italian version of the Keratoconus Outcomes Research Questionnaire (KORQ). METHODS:Cross-sectional validation study. Keratoconus patients with routine appointments at the outpatient clinic were consecutively enrolled in conjunction with a sample of non-keratoconus controls for comparison. The Italian translation from the original English version of the KORQ was administered to the patients together with the Italian version of the 25-item version of the National Eye Institute Visual Function Questionnaire (NEI VFQ-25). The reliability and validity of the Italian KORQ were assessed using standardized internationally accepted methods for cultural adaptation and validation of health-related quality of life measures. RESULTS:One hundred patients were deemed eligible and completed the questionnaires. The Cronbach's alpha coefficient for internal consistency ranged from 0.71 to 0.956 across the subscales. Spearman correlation coefficient and intraclass correlation coefficient of 0.98 showed excellent test-retest reliability. The control group had better scores on every subscale. Statistically significant correlations were found between the KORQ and analogous domain of the NEI VFQ-25 and with disease severity indicators, such as visual acuity, maximum keratometry, and steepest keratometry, thus demonstrating good construct and concurrent validity. CONCLUSION:The Italian version of the KORQ exhibited excellent internal consistency, test-retest reliability, validity, discriminatory power, and psychometric properties comparable with those of the original English version, and thus may be adopted as a powerful vision-targeted quality of life assessment tool for Italian keratoconus patients. 10.1177/11206721211028044
    Measurement of Quality of Life in Keratoconus. Kandel Himal,Pesudovs Konrad,Watson Stephanie L Cornea PURPOSE:To identify and assess the quality of questionnaires used to measure quality of life in keratoconus and guide selection of the most appropriate questionnaire for evaluating the impact of keratoconus. METHODS:A literature search was carried out in Scopus, Web of Science, PubMed, MEDLINE, Cochrane, and PsycINFO databases. Articles that described a questionnaire to measure quality of life in keratoconus were included. Information on psychometric properties and validity was extracted and analyzed based on a set of quality criteria. Finally, the impact of keratoconus and its management methods on quality of life was reviewed. RESULTS:The search yielded 331 publications, of which 45 articles describing 18 (12 ophthalmic including 2 keratoconus-specific and 6 generic) questionnaires were reviewed. Most of the articles (40, 88.9%) described ophthalmic questionnaires not specific to keratoconus. The National Eye Institute Visual Function Questionnaire was the most frequently used questionnaire (n = 26). Only 4 articles provided information on psychometric properties. The Keratoconus Outcomes Research Questionnaire, the only validated keratoconus-specific questionnaire, had the most superior psychometric properties. However, it consists of items on only 2 domains of quality of life (activity limitation and symptoms). Overall, keratoconus management methods (spectacles, contact lenses, and cross-linking in early stages, corneal transplantation in late stages) improved quality of life. The quality of life scores were associated with clinical measures including visual acuity, corneal topography, pachymetry, and keratoconus severity. CONCLUSIONS:There is a need for a comprehensive and high-quality patient-reported outcome measure in keratoconus. A questionnaire should be chosen based on the purpose and the quality of the questionnaire. This review guides selection of an appropriate questionnaire. 10.1097/ICO.0000000000002170
    Vision-Related Quality of Life in Iranian Patients With Keratoconus: National Eye Institute Vision Function Questionnaire-25. Mahdaviazad Hamideh,Bamdad Shahram,Roustaei Narges,Mohaghegh Sahar Eye & contact lens BACKGROUND:Use of patient-reported outcomes to evaluate the impact of ocular conditions on quality of life (QoL) is rising. Hence, the aims of this study were to determine the impact of keratoconus on QoL, and assess the National Eye Institute of the United States Vision Function Questionnaire (NEI-VFQ) performance in an Iranian population. METHODS:From November 2016 to June 2017, patients with keratoconus definitive diagnosis for more than one year who were being routinely followed at a cornea clinic were recruited. The NEI-VFQ-25 was administered during a face-to-face interview. Ocular examinations comprised best-corrected visual acuity (BCVA) was logged monocularly and binocularly, anterior segment biomicroscopy, refraction, and corneal topography. Keratoconus severity was graded based on steep keratometric (K) reading values. A group of 30 age- and sex-matched subjects with other ocular diseases (except keratoconus) were selected randomly from the cornea clinic. P<0.05 was considered as significant. RESULTS:The mean age of participants was 28.7±7.6 years. The lowest NEI-VFQ subscale scores were related to ocular pain, general vision, mental health, and role difficulty. Almost all NEI-VFQ scores in patients were statistically significant less than mean score of the control group. Composite and all subscales NEI-VFQ scores were lower significantly among patients with higher disease duration (≥5 years). Among clinical data, patients with BCVA ≥0.5 in the better eye had significantly lower NEI-VFQ composite score. Severe keratoconus patients (steep K reading ≥52) had lower NEI-VFQ scores in mental health and dependency subscales (P<0.05). The overall and subscales Cronbach α was above 0.7. CONCLUSION:Iranian patients with keratoconus had physical, emotional, and social impairment in QoL. The NEI-VFQ-25 might be applicable in further studies. 10.1097/ICL.0000000000000492
    Quality of life in keratoconus: evaluation with Keratoconus Outcomes Research Questionnaire (KORQ). Pinto Roberto Damian Pacheco,Abe Ricardo Yuji,Gomes Flávia Cid,Barbisan Paulo Rodolfo Tagliari,Martini Alexandre Fattah,de Almeida Borges Daniel,Fernandes Arthur Gustavo,Arieta Carlos Eduardo Leite,Alves Monica Scientific reports To assess the quality of life of keratoconus patients using the Keratoconus Outcomes Research Questionnaire (KORQ), translated and validated in Portuguese language. The KORQ is the only validated keratoconus specific questionnaire and has a high rating for its psychometric properties. This cross-sectional study enrolled 100 keratoconus patients from a tertiary referral eye hospital between April 2018 and June 2019. Associations between age, sex, allergic conjunctivitis, keratoconus stage, best-corrected visual acuity (BCVA), maximum simulated keratometry (Kmax), steep keratometry (K2), pachymetry, treatments performed, hydrops, and KORQ scores were evaluated using univariate (Wilcoxon test and the Kruskal Wallis test) and multivariate linear regression with stepwise backward modeling. Lower KORQ scores are associated with better quality of life, whereas, higher scores are associated with greater impairment of functional activities and symptoms. Among the 100 patients, mild, moderate, and severe keratoconus were observed in 15%, 46% and 39% of participants, respectively. Univariate analysis showed lower function scores values, with male sex (p < 0.05) and both functional and symptom scores were significantly associated with BCVA < 0.3 (LogMAR) (p < 0.05). Multivariate analysis indicated significantly lower functional scores in individuals with BCVA < 0.3 (LogMAR) (p < 0.001) and those with a history of crosslinking treatment (p = 0.022), while symptom scores were only significantly associated with only BCVA < 0.3 (LogMAR) (p < 0.001). In patients with keratoconus, BCVA in the better eye and history of crosslinkig were factors associated with better quality of life scores using the KORQ. 10.1038/s41598-021-92346-1