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Purpose: Treatment with glucocorticoids following paediatric cataract surgery is crucial to prevent inflammation, but may lead to secondary glaucoma, and hypothalamic-pituitary-adrenal axis suppression. We wish to compare glaucoma outcomes following high-dose and low-dose glucocorticoid treatment after paediatric cataract surgery., Methods: This cohort study included Danish children undergoing cataract surgery before 10 years of age, receiving either a low-dose or high-dose postoperative glucocorticoid treatment. Case identification and collection of a standardized dataset were retrospective, from 1 January 2010 to 31 December 2016, and prospective thereafter, until 31 December 2021. High-dose treatment included 0.5-1.0 mg subconjunctival depot dexamethasone or methylprednisolone, followed by 6-8 drops of dexamethasone for 1 week, tapered by one drop weekly. Low-dose treatment included 6 drops for 3 days, followed by 3 drops for 18 days. Sustained (>3 months) ocular hypertension or glaucoma was compared between the two groups., Results: Overall, 267 children (388 eyes) were included in the study. Ninety-five children (133 eyes) had received high-dose treatment and had a median follow-up time of 89 months (IQR: 57.2-107.4), while 173 children (255 eyes) had received the low-dose treatment and had a median follow-up time of 40.5 months (IQR: 22.9-60.4). Survival curves showed a lower risk of glaucoma in the low-dose group for children with axial lengths ≥18 mm., Conclusion: Low-dose glucocorticoid treatment was associated with a lower risk of glaucoma in children with axial lengths ≥18 mm. The same effect was not observed in children with shorter eyes. High-dose glucocorticoid should be limited in children undergoing cataract surgery., (© 2024 The Author(s). Acta Ophthalmologica published by John Wiley & Sons Ltd on behalf of Acta Ophthalmologica Scandinavica Foundation.)

Purpose: To investigate visual development and long-term complications after cataract surgery in childhood., Methods: This cross-sectional study included patients from a family with Marner's hereditary cataracts who had cataract surgery before 18 years of age. The study was conducted from 1 January 2022 until 31 December 2022. The patients contributed to their medical files and participated in an updated ophthalmologic examination., Results: We included 52 patients (101 eyes, 34 females). The median age at cataract surgery was 7 years (IQR: 5-10) and the age at examination was 40 years (IQR: 21-54). Primary and secondary intraocular lens implantation had been performed in 47.5% (25 patients, 48 eyes) and 16.8% (10 patients, 17 eyes). Visual acuity was ≤0.3 logMAR in 77% (78 eyes), and <0.5 logMAR in 8% (8 eyes). Glaucoma was present in 17% (9 patients, 12 eyes), ocular hypertension in 6% (3 patients, 4 eyes), and 10% (5 patients, 5 eyes) had prior retinal detachment. Mild visual field loss (2 < mean defect (MD) ≤ 6 dB) was found in 62% (63 eyes) and moderate to advanced visual field loss (MD > 6 dB) in 24% (24 eyes). Thirty-five patients (67%) held a driver's licence, and three were not allowed to drive due to low visual function. All patients were employed., Conclusion: After cataract surgery in childhood, many patients achieve normal visual acuity, but mild visual field loss is common. Long-term follow-up is important due to the high risk of glaucoma., (© 2024 The Author(s). Acta Ophthalmologica published by John Wiley & Sons Ltd on behalf of Acta Ophthalmologica Scandinavica Foundation.)

Purpose: To determine vision-related quality of life (VR-QoL) and functional and structural parameters associated with VR-QoL in patients with glaucoma before and 12 months after trabeculectomy., Methods: Fifty-eight patients undergoing trabeculectomy were included. Participants completed the 25-item National Eye Institute Visual Function Questionnaire (VFQ-25) prior to and 12 months after trabeculectomy. Functional (visual acuity and visual fields) and structural (ganglion cell layer volume) parameters were evaluated for their association with VR-QoL using multiple linear regression of VFQ-25 subscale scores. Intraocular pressure and the number of glaucoma medications were also included in the analyses., Results: The VFQ-25 composite score did not change after trabeculectomy (before: 74.9; 12 months: 74.0; p = 0.512). The subscale mental health had a significantly higher score 12 months after trabeculectomy (before: 65.6; 12 months: 71.4; p = 0.017). The VFQ-25 scores for general health (before: 68.5; 12 months: 62.5; p = 0.009) and role difficulties (before: 78.9; 12 months: 53.7; p < 0.001) were significantly lower 12 months after trabeculectomy. No functional or structural parameters were associated with VFQ-25 composite score., Conclusion: Overall, VR-QoL in glaucoma patients was similar before and after trabeculectomy, reflecting the procedure's stabilizing effect on both objective and subjective visual function. The absence of correlations between VR-QoL and clinical parameters emphasizes the multifaceted nature of VR-QoL and highlights the limitations of depending solely on objective clinical metrics to evaluate patients' subjective experiences. Using both objective measures and VR-QoL, clinicians can better understand the challenges patients face due to glaucoma and trabeculectomy, potentially leading to better solutions., (© 2024 The Authors. Acta Ophthalmologica published by John Wiley & Sons Ltd on behalf of Acta Ophthalmologica Scandinavica Foundation.)

Purpose: To examine the long-term risk of glaucoma after cataract surgery in childhood., Methods: This study took place from January 2022 until December 2022 and included patients from a large family with hereditary childhood cataract who had cataract surgery before 18 years of age. Patients underwent an ophthalmologic examination to determine the presence of glaucoma or ocular hypertension (OHT). Patients who did not want to participate in the examination could contribute with a medical journal from their treating ophthalmologist. The risk of long-term glaucoma was determined using survival analysis, and risk factors were assessed using a Cox proportional hazards regression model., Results: We included 68 patients (133 eyes) with a median age at cataract surgery of 7 years (IQR: 5-10). The median follow-up time after cataract surgery to glaucoma/OHT or the latest ophthalmologic examination was 35 years (IQR: 15-48). Twelve patients (18 eyes) had glaucoma, and five patients (eight eyes) had OHT, resulting in 15 patients with glaucoma/OHT. The long-term risk of glaucoma/OHT diagnosed in adulthood was 47.7% (CI: 21.8-70.9) at the age of 70 years of patients who were free of glaucoma before their 18th year. We could not confirm or dismiss an association between glaucoma/OHT and sex, age at surgery, number of ocular interventions before 18 years of age or glaucoma after cataract surgery in a first-degree relative., Conclusion: Cataract surgery in childhood is associated with a high risk of late-onset glaucoma. Regular lifelong follow-up is important to ensure early diagnosis and prevent extensive vision loss., (© 2024 The Authors. Acta Ophthalmologica published by John Wiley & Sons Ltd on behalf of Acta Ophthalmologica Scandinavica Foundation.)

Background: Stickler syndrome (STL) is a collagenopathy caused by pathogenic variants in collagen-coding genes, mainly COL2A1 or COL11A1 associated with Stickler syndrome type 1 (STL1) or type 2 (STL2), respectively. Affected individuals manifest ocular, auditory, articular, and craniofacial findings in varying degrees. Previous literature and case reports describe high variability in clinical findings for patients with STL. With this case report, we broaden the clinical spectrum of the phenotype., Materials and Methods: Case report on two members of a family (mother and son) including clinical examination and genetic testing using targeted trio whole exome sequencing (trio-WES)., Results: A boy and his mother presented with microphthalmia, congenital cataract, ptosis, and moderate-to-severe sensorineural hearing loss. Trio-WES found a novel heterozygote missense variant, c.4526A>G; p(Gln1509Arg) in COL11A1 in both affected individuals., Conclusions: We report a previously undescribed phenotype associated with a COL11A1-variant in a mother and son, expanding the spectrum for phenotype-genotype correlation in STL2, presenting with microphthalmia, congenital cataract, and ptosis not normally associated with Stickler syndrome.

Purpose: Evaluation of long-term functional and structural outcomes in patients with primary congenital glaucoma (PCG) based on visual acuity (VA), visual field (VF) using standard automated perimetry, and peripapillary retinal nerve fibre layer thickness (pRNFL)., Methods: We retrospectively reviewed medical records of all patients diagnosed with PCG in Denmark from 1977 to 2016. Severe vision loss was defined as VA <6/60 and/or VF >20 decibels (dB). Prognostic factors were evaluated in a correlation matrix., Results: The median age of the 94 patients (153 PCG eyes) was 12 years (IQR 9-16). In PCG eyes 62% had VA ≥6/18 but 22% had <6/60. VA in the better seeing eye was ≥6/18 in 90% and <6/60 in 5%. VF was measured in 59 PCG eyes and the median mean defect was 5.1 dB (IQR 2.1-9.6) with 52% better than 6 dB and 9% worse than 20 dB. Generalized pRNFL was reduced below the age-expected 1st percentile in 29% of the 58 PCG eyes where pRNFL was measured. Poor VA, poor VF and reduced pRNFL were all correlated (p = 0.0001). More surgeries (p < 0.0001) and longer diagnostic delay (p = 0.004) were associated with poorer vision and to a lesser degree with poor VF pRNFL., Conclusion: In Denmark, most patients with bilateral PCG retain VA ≥6/18 in the better seeing eye. Poor VA was associated with poor VF. Longer diagnostic delay and more surgeries were associated with a poorer prognosis., (© 2023 The Authors. Acta Ophthalmologica published by John Wiley & Sons Ltd on behalf of Acta Ophthalmologica Scandinavica Foundation.)

This prospective randomized controlled trial aimed to compare the efficacy and safety of topical preservative-free diclofenac (DICLO) to dexamethasone (DEX) eyedrops, and their combination (DEX+DICLO) after trabeculectomy. Sixty-nine patients with medically uncontrolled glaucoma were randomized to receive topical postoperative treatment with DICLO ( n = 23), DEX ( n = 23), or a combination of DEX and DICLO ( n = 23). The primary outcome was the intraocular pressure (IOP) 12 months postoperatively. Secondary outcomes included surgical success, failure, visual field, and visual acuity from baseline to 12 months postoperatively. IOP reached the lowest point one day after trabeculectomy. At 12 months, IOP was 10.0 mmHg (95% CI, 8.4-11.6 mmHg) for DICLO, 10.9 mmHg (95% CI, 9.4-12.3 mmHg) for DEX, and 11.2 mmHg (95% CI, 9.1-13.3 mmHg) for DEX+DICLO. There were no significant differences in IOP, surgical success, failure, visual field, or visual acuity between the DICLO, DEX, or DEX+DICLO groups. We found that topical diclofenac was not statistically different from topical dexamethasone in controlling IOP 12 months after trabeculectomy. Combining diclofenac and dexamethasone offered no added IOP control compared to dexamethasone alone.

Objectives: Childhood cataract is a chronic condition that may interfere with the child's learning capacities. We aimed to investigate whether childhood cataract influences academic development by comparing school performance in reading and mathematics in children with cataract to a matched control group., Design: Nationwide registry-based cohort study., Settings: Two surgical centres that perform all treatments for childhood cataract in Denmark., Participants: Children born between 2000 and 2009 diagnosed with cataract before 10 years of age (n=275) and an age-matched and sex-matched control group (n=2473)., Main Outcome Measures: School performance was assessed as test scores in national tests performed at regular intervals from grade 2 to grade 8 in reading and mathematics. Analyses were corrected for birth origin, child somatic and mental disorder and parental socioeconomic status and mental disorders., Results: Of 275 children, 85 (30.9%) were operated for bilateral cataract, 79 (28.7%) unilateral cataract and 111 (40,4%) were not operated. We found that children with cataract have lower participation rate in the tests (62.5%) compared with the control cohort (77.2%) (p value=0.0001). After adjusting the pooled analyses for birth origin, somatic and mental disease in the child and parental socioeconomic status and mental disorders, we found that the children with cataract scored significantly lower in mathematics compared with those without cataract (mean difference=-4.78, 95% CI: -8.18 to -1.38, p value=0.006), whereas no difference was found regarding scores in reading (p=0.576). The lower score in mathematics was driven by children who had been operated for bilateral cataract (p-value=0.004)., Conclusion: Children with cataract without somatic or neurodevelopmental comorbidities or psychosocial adversities seem to do well in school, whereas children operated for bilateral cataract have higher frequencies of difficulties in mathematical tasks., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Introduction: The number of people living with visual impairment is increasing. Visual impairment causes loss in quality of life and reduce self-care abilities. The burden of disease is heavy for people experiencing visual impairment and their relatives. The severity and progression of age-related eye diseases are dependent on the time of detection and treatment options, making timely access to healthcare critical in reducing visual impairment. General practice plays a key role in public health by managing preventive healthcare, diagnostics and treatment of chronic conditions. General practitioners (GPs) coordinate services from other healthcare professionals. More involvement of the primary sector could potentially be valuable in detecting visual impairment., Methods: We apply the Medical Research Council framework for complex interventions to develop a primary care intervention with the GP as a key actor, aimed at identifying and coordinating care for patients with low vision. The development process will engage patients, relatives and relevant health professional stakeholders. We will pilot test the feasibility of the intervention in a real-world general practice setting. The intervention model will be developed through a participatory approach using qualitative and creative methods such as graphical facilitation. We aim to explore the potentials and limitations of general practice in relation to detection of preventable vision loss., Ethics and Dissemination: Ethics approval is obtained from local authority and the study meets the requirements from the Declaration of Helsinki. Dissemination is undertaken through research papers and to the broader public through podcasts and patient organisations., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Introduction: To compare the effect of three different anti-inflammatory regimens consisting of preservative-free dexamethasone (DEX), diclofenac (DICLO) eye drops, and their combination (DEX + DICLO) following trabeculectomy on early postoperative inflammation., Methods: A prospective randomized controlled trial. Sixty-nine patients undergoing trabeculectomy were randomized to receive either postoperative treatment with topical DEX (n = 23), topical DICLO (n = 23), or a combination of topical DEX and topical DICLO (n = 23) after trabeculectomy. The primary outcome was the anterior chamber flare measurement in the first 3 months postoperatively. Secondary outcomes included intraocular pressure, central corneal thickness, conjunctival injection, and number of cells in the anterior chamber from baseline to 3 months postoperatively., Results: Anterior chamber flare reached a maximum 1 day after trabeculectomy with an increase of 55% (95% CI 37-73%) for DEX, 64% (95% CI 47-82%) for DICLO, and 57% (95% CI 39-75%) for DEX + DICLO and returned to near pre-operative values 6 weeks after surgery. There were no significant differences in anterior chamber flare [effect size for DICLO: 0.16 (95% CI - 4.3 to 4.6), effect size for DEX + DICLO: 0.09 (95% CI - 4.1 to 4.3)], intraocular pressure, central corneal thickness, conjunctival injection, or number of cells in the anterior chamber between DEX, DICLO, or DEX + DICLO groups., Conclusion: We found that topical diclofenac was not statistically different from topical dexamethasone in controlling early postoperative inflammation after trabeculectomy, while combining diclofenac and dexamethasone offered no added anti-inflammatory control compared to dexamethasone alone., Trial Registration: www., Clinicaltrials: gov (NCT04054830)., (© 2023. The Author(s).)

Purpose: To examine the prevalence and risk factors for hypothalamus-pituitary-adrenal axis suppression (HPA axis suppression) in infants receiving glucocorticoid (GC) eye drops after ocular surgery., Methods: This was a clinical observational cohort study. Children under the age of two receiving GC eye drops after cataract or glaucoma surgery between 1 January 2017 and 31 December 2021 were included at one centre. Medical history and results of the adrenocorticotropic hormone (ACTH) stimulation tests were obtained through patient charts., Results: Forty-nine infants were included in the study. Ten out of 22 patients (45.5%) tested during treatment and two out of 27 patients (7.4%) tested after treatment cessation were diagnosed with HPA axis suppression. The duration of HPA axis suppression extended beyond 3 months in 8 out of 12 patients. Logistic regression showed that infants with HPA axis suppression had received a higher GC dose/body weight/day before the first ACTH test (p < 0.001). There was a 79% (95% CI:1.28;2.50) increase in the odds of having HPA axis suppression for a 0.01 mg GC increase/kg/day corresponding to an additional daily eye drop for an infant weighing 5 kg. There was an association between HPA axis suppression and number of days from surgery to test (p = 0.003), age at surgery (p = 0.035) and cumulated GC dose (p = 0.005). Three infants with HPA axis suppression had affected growth and one had Cushing-like features, but there were no cases of Addisonian crisis., Conclusion: Infants are at risk of having hypothalamus-pituitary-adrenal axis suppression if they receive a high daily glucocorticoid dose per weight by topical ocular administration. Infants receiving glucocorticoids after ocular surgery should be monitored clinically or by ACTH testing., (© 2022 The Authors. Acta Ophthalmologica published by John Wiley & Sons Ltd on behalf of Acta Ophthalmologica Scandinavica Foundation.)

This prospective randomized controlled trial aimed to compare changes in intraocular pressure in three different anti-inflammatory regimens following trabeculectomy. Sixty-nine patients were randomized to receive either postoperative prophylaxis with topical preservative-free dexamethasone (DEX), diclofenac (DICLO), or their combination (DEX+DICLO). Our main outcome measure was an intraocular pressure (IOP) change of a minimum 4 mmHg following the withdrawal of anti-inflammatory prophylaxis 9 weeks after trabeculectomy. We found that the IOP decreased ≥ 4 mmHg in 18.6% of eyes after cessation of the topical steroid DEX (n = 3/22) and DEX+DICLO (n = 5/21), whereas a decrease in IOP was not observed in the DICLO group. In conclusion, IOP decreased in nearly 1/5 of patients after cessation of topical steroidal anti-inflammatory prophylaxis after trabeculectomy. This points toward a steroid-induced increase in IOP even after trabeculectomy. Thus, increased postoperative IOP may be related to steroid use, and the success or failure of a trabeculectomy cannot be fully evaluated before anti-inflammatory prophylaxis with steroids is stopped or changed to non-steroidal eye drops.

Purpose: To describe the causes of ectopia lentis (EL) and the outcomes after surgery in a Danish population., Setting: The Eye Clinic Rigshospitalet and Kennedy Center in Copenhagen., Design: Retrospective cohort study., Methods: Medical records of patients with nontraumatic EL born after 1980 and seen at the Eye Clinic Rigshospitalet and Kennedy Center from 1983 to 2019 were reviewed. Clinical information regarding family history, comorbidities, genetic workup, ophthalmological examinations, and surgical history was retrieved., Results: 72 patients (38 males), of whom 68 had bilateral EL (94.4%) were identified. Marfan syndrome (MFS) was found in 34 (47.2%) and biallelic variants in ADAMTSL4 in 4 (5.6%). Surgery was performed in 38 (52.8%) patients, 66 eyes, with a median age at the time of first eye surgery of 8.4 years (range 0.8 to 39.0 years) and a follow-up of 2.3 years (range 0 to 25.7 years). Intraocular lenses were implanted in 9 (23.7%) (11 eyes). Corrected distance visual acuity improved from 0.7 to 0.2 logMAR (median) in right eyes and from 0.7 to 0.3 logMAR in left eyes postoperatively. 21 patients (56.8%), 42 eyes, did not experience any surgery-related complications. 3 patients (3 eyes) experienced a perioperative tear in the posterior capsule. Temporary postoperative ocular hypertension was reported in 3 patients (7.9%) (3 eyes), and 2 patients (5.4%) (2 eyes) developed persistent ocular hypertension. There were no cases of postoperative retinal detachment., Conclusions: The main reason for EL was MFS. Surgery improved visual acuity, and postoperative ocular hypertension was the most common complication, whereas retinal detachment was not observed., (Copyright © 2022 Published by Wolters Kluwer on behalf of ASCRS and ESCRS.)

Purpose: To investigate the association between visual function and ultrastructural characteristics of the retina and optic disc in children operated for cataract and factors influencing this relationship., Methods: We included 56 children aged 7-18 years who had been operated for bilateral or unilateral cataract. Three-dimensional swept source OCT scans of the macula and the optic disc were obtained using the Triton OCT (Topcon, Corporation, Tokyo, Japan). Best corrected distance visual acuity was measured using the HOTV 3 Meter visual chart. Contrast vision was evaluated using the Freiburg Visual Acuity Contrast Test (FrACT). Healthy fellow eyes of the unilateral group were used as control eyes., Results: We did not find any associations between visual acuity or contrast sensitivity and structural retinal parameters. Shorter axial length, the presence of glaucoma and pseudophakia (opposed to aphakia) were all significant predictors of greater mean macular thickness in a multiple regression analysis, (p = 0.039). Two children had foveal hypoplasia, and one child had a right eye with a thin, disorganized retina., Conclusions: Children with pseudophakia have a thicker macula but this does not seem to be related to visual function. Obtaining high quality OCT scans of the macula and optic disc in severely visually impaired children with glaucoma and/or nystagmus is challenging. We found structural macular changes which contributed to poor vision but most eyes with very reduced vision were difficult to image, and the prevalence of structural changes may be underestimated., (© 2021 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Background: To report long-term biometric and refractive outcomes in a group of Danish children after surgery for childhood cataract., Methods: Children between 7 and 18 years who had undergone uni- or bilateral cataract surgery at the Department of Ophthalmology, Rigshospitalet, Denmark, were examined in this cross-sectional study. Swept source optical coherence tomography (OCT) based optical biometry (IOLmaster 700) and anterior tomography (Pentacam) was performed. Healthy fellow eyes from those with unilateral cataract were used as controls., Results: We included 56 children in the study with a median age at surgery of 43.8 months (1.6-137.6). The amount of higher order aberrations was significantly increased in operated eyes (median root mean square 0.461 μm [range 0.264-1.484]) compared with non-operated eyes (median root mean square 0.337 μm [range 0.162-0.498], p < 0.001). Younger age at surgery was positively associated with more higher order aberrations at follow-up (p < 0.001), but we found no significant associations between the amount of higher order aberrations and visual acuity or contrast vision. Longer axial length was associated to glaucoma while shorter axial length was associated to strabismus (p < 0.001)., Conclusions: Eyes operated for childhood cataract have higher order aberrations compared with non-operated eyes. Higher order aberrations are complex refractive errors that cannot be corrected by normal lenses and may contribute to poor visual outcomes for the children. We found an association between young age at surgery and higher order aberrations., (© 2022 The Authors. Clinical & Experimental Ophthalmology published by John Wiley & Sons Australia, Ltd on behalf of Royal Australian and New Zealand College of Ophthalmologists.)

Purpose: To examine the incidence of mental disorders in children with cataract compared with children without cataract., Design: Nationwide cohort study based on entries in comprehensive national databases., Methods: The incidence of mental disorders in children born between 2000 and 2017 diagnosed with cataract before 10 years of age (n = 485) was compared with sex- and age-matched controls (n = 4358). Analyses were corrected to somatic disease in the child and parental socioeconomic status and psychiatric morbidity. The study was conducted as 2 university hospitals in Denmark managing children 6 years of age our younger with cataract., Results: The incidence of mental disorders was nearly doubled in children with cataract compared with controls (odds ratio [OR], 1.83; 95% CI, 1.28-3.63). The risk of anxiety disorders was quadrupled (OR, 4.10; 95% CI, 1.90-8.84) and the risk of developmental delay was doubled (OR, 2.66; 95% CI, 1.45-4.90). The risk of mental disorders was significantly higher in children diagnosed with cataract in the first 3 years of life compared with controls (OR, 2.36; 95% CI, 1.53-3.64), whereas those diagnosed with cataract later in childhood did not have an increased risk (OR, 1.24; 95% CI, 0.66-2.30)., Conclusions: The risk of mental disorders, in particular anxiety and neurodevelopmental delay, is markedly increased in children with cataract and even more so in those diagnosed within the first 3 years of life. Psychiatric screening instruments may be integrated in the management of these children., (Copyright © 2021 The Authors. Published by Elsevier Inc. All rights reserved.)

Purpose: To investigate the socio-economic status of families affected by childhood cataract and to assess how the socio-economic status is affected by cataract diagnosis., Materials and Methods: Children born between 2000 and 2017, seen between the age 0 and 10 years in the same period at Rigshospitalet or Aarhus University Hospital for cataract (N = 485), were included and compared to a matched children group without cataract (N = 4358). Socio-economic status was evaluated by the parents' income, employment, education, marital status and family structure., Results: Parents of children with cataract were more likely to have a low yearly income (OR = 1.60, 95% CI (1.12-2.27)), be out of work (OR = 1.74, 95% CI (1.34-2.26)) and have basic education as the highest attained education (OR = 1.64, 95% CI (1.27-2.13)) prior to diagnosis. This social gradient was not affected by the diagnosis. In addition, a higher number of children with cataract lived in multi-family residencies (13.8% versus 8% in group of children without cataract) and they had a greater number of siblings (6.2% had ≥4 siblings versus 2.1% in group of children without cataract)., Conclusion: Families affected by childhood cataract have a lower socio-economic status and educational background even before cataract is diagnosed but the diagnosis does not aggravate the differences between these families and the background population. The lower socio-economic status and parental educational background should be taken into consideration in the management of these families., (© 2021 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Purpose: To systematically review the literature on the treatment of vernal keratoconjunctivitis (VKC) in children and young adults and conduct comparative efficacy analysis on clinical signs and symptoms using network meta-analyses., Methods: We systematically searched the databases PubMed/MEDLINE, EMBASE, Cochrane Central and Web of Science on 21 October 2019 for randomized controlled trials (RCT). Studies considered had patients with VKC < 20 years of age randomized into either intervention (any medical intervention) or comparator (active treatment, placebo treatment or non-treatment control), where pre-defined outcomes (data from ≥2 weeks and as close as possible to 2 months) of symptoms (itching, tearing, photophobia and foreign body sensation) and signs (hyperaemia, punctate keratitis, Horner-Trantas dots and macropapillae) were reported. Risk of bias within studies was evaluated using the Cochrane risk of bias tool. Comparisons were made using network meta-analyses., Results: We identified 39 studies with data on 2046 individuals. Twenty-three studies were eligible for quantitative analyses. None were systemic therapy. Temporal trend analysis showed that an initial focus on topical mast cell stabilizers turned to a focus on calcineurin inhibitors and a more diverse variety of pharmacological strategies. Studies varied in population, treatment duration and quality. The quantitative analysis revealed that efficacy of different therapies differed substantially across important clinical signs and symptoms, but there was a general trend of superior efficacy when using topical corticosteroids with stronger efficacy of the more potent corticosteroids., Conclusion: We provide an overview of RCTs comparing the efficacy of treatments for VKC in children and young adults, which we find differs across symptoms and signs. Overall, we saw a general trend of superior efficacy with topical corticosteroids. However, our findings highlight the need for better studies, consensus on core outcomes and potential for individualized therapy., (© 2021 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Purpose : Bilateral childhood cataracts can be caused by a metabolic disease, constitute a part of a syndrome, run in families, be sporadic or iatrogenic. The amount of work-up needed to establish a cause is discussed and the aim of the present study was to evaluate causes of bilateral childhood cataract. Methods : Chart review of 211 Danish children with bilateral cataracts. Information on work-up was retrieved with special focus on general health, metabolic screening, evaluation for congenital infections and genetic testing. Results : Cataract was seen in combination with systemic disease in 40.8%, 29.4% had hereditary cataracts, 27.0% had isolated cataract, in 1.4% it was associated with ocular malformations and 1.4% had been born prematurely without any other sequelae than the cataract. A genetic cause could be demonstrated in 74 children. Conclusion : Systemic comorbidities are very common in children with cataract and are not always known prior to the diagnosis of cataract. Genetic evaluation, especially targeted analyses, provided a molecular genetic diagnosis in a large proportion of those tested but it also failed to provide a molecular genetic diagnosis in some patients with a family history suggesting autosomal dominant inheritance. Most importantly, in some patients, genetic work-up provided a diagnosis in patients where it had therapeutic consequences and where the systemic disease would have caused irreversible damage, had it not been treated timely. Given the high prevalence of systemic disease, it seems advisable to co-manage children with bilateral cataracts with a pediatrician and to include genetic evaluation as part of the work-up.

Purpose: To systematically review the results of comparative studies of modern cataract surgery in pediatric uveitis with or without intraocular lens (IOL) implantation and to perform comparative meta-analyses to compare visual acuity outcomes and complication rates., Methods: On 12 November 2020, we systematically searched the Cochrane Central, PubMed/MEDLINE, EMBASE, ClinicalTrials.gov, and all affiliated databases of the Web of Science. Two authors independently reviewed studies and extracted data. Studies were reviewed qualitatively in text and quantitatively with meta-analyses. Outcome measures were preoperative and postoperative best-corrected visual acuity (BCVA), inflammation control, and rates of postoperative complications., Results: Ten studies of 288 eyes were eligible for review of which the majority were eyes with juvenile idiopathic arthritis-associated uveitis. Summary estimates revealed that the BCVA was better in pseudophakic eyes vs. aphakic eyes (1-year postoperative: -0.23 logMAR, 95% CI: -0.43 to -0.03 logMAR, P =0.027; 5-year postoperative: -0.35 logMAR, 95% CI: -0.51 to -0.18 logMAR, P =0.000036). Pseudophakic eyes had more visual axis opacification (OR 6.76, 95% CI: 2.73 to 16.8, P =0.000036) and less hypotony (OR 0.19, 95% CI: 0.04 to 0.95, P =0.044)., Conclusions: In modern era cataract surgery on eyes with pediatric uveitis with IOL implantation leads to satisfactory and superior visual outcomes and no differences in complication rates apart from an increased prevalence of visual axis opacification and a decreased prevalence of hypotony when compared to aphakia. However, limitations of the retrospective design and the presence of selection bias necessitate a careful interpretation., Competing Interests: The authors declare that they have no conflicts of interest., (Copyright © 2021 Diana Chabané Schmidt et al.)

Purpose: To evaluate the surgical success after trabeculotomy for primary congenital glaucoma (PCG) in Denmark in the last 40 years., Methods: Retrospective review of medical files on children who all underwent trabeculotomy as the first surgical intervention between January 1rst, 1977 and December 31, 2016. Information on diagnosis and surgical procedures were extracted. Primary outcome was surgical success was defined as intraocular pressure (IOP) < 21 mmHg without medications one year after surgery. Secondary outcome was the number of reoperations needed., Results: Out of 118 children with PCG, 96 children (144 eyes) had an ab externo trabeculotomy as primary IOP lowering procedure opening Schlemm's channel 4 clock hours into the anterior chamber. Complete surgical success at one year was achieved in 73% (106 of 144 eyes). On the long term, IOP could be controlled by one surgical procedure in 2/3 of children, eight eyes required >5 surgical procedures to control IOP. In 12.1% of children the second glaucoma procedure was performed >5 years after the initial trabeculotomy. There was a tendency towards greater risk of needing a second procedure in patients < 3 months of age at primary trabeculotomy (hazard ratio, HR, 2.01, 95% CI 0.96-4.22) and in boys (HR 2.02, 95% CI 0.97-4.18) and a lower risk of requiring a third surgery if the second surgery was trabeculectomy with MMC., Conclusion: Dedicated follow-up of patients with PCG is essential as the disease may continue to progress even after years of quiescence and some children need multiple surgeries to control IOP. Additional glaucoma procedures are required in 1/3 of children and boys and younger patients seem to be at greater risk., (© 2020 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Purpose: To evaluate the efficacy of vitrectomy combined with hyaloido-zonula-iridectomy from an anterior or a posterior approach in patients with treatment-resistant aqueous misdirection (chronic aqueous misdirection) by systematically reviewing existing literature in combination with presentation of a case series., Methods: A systematic literature review was performed in PubMed, EMBASE and Cochrane Library databases using search terms: malignant glaucoma, ciliary block, ciliolenticular block and aqueous misdirection. A consecutive series of three pseudophakic patients (5 eyes) diagnosed with chronic aqueous misdirection after cataract surgery is presented., Results: A literature search identified 31 articles describing treatment of chronic aqueous misdirection with vitrectomy and a hyaloido-zonula-iridectomy. Studies, where patients were treated with a complete vitrectomy from pars plana in combination with a hyaloido-zonula-iridectomy, reported low relapse rates. Studies describing a surgical approach with vitrectomy performed from the anterior chamber, followed by a hyaloido-zonula-iridectomy, also reported low relapse rates except for one reporting relapse in nearly half of the patients. In our case series, a complete vitrectomy combined with a hyaloido-zonula-iridectomy resolved the chronic aqueous misdirection in all five eyes after one procedure except one eye where the hyaloido-zonula-iridectomy was repeated due to an insufficient opening. Some of the eyes still needed antiglaucomatous treatment due to chronic angle closure., Conclusion: In treatment-resistant malignant glaucoma, vitrectomy combined with a hyaloido-zonula-iridectomy should be considered performed to ensure communication between the anterior chamber and the vitreous cavity. If the condition has been unresolved for a long time, extensive synechiae of the angle may decrease the success rate due to chronic angle closure., (© 2020 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Glaucoma is a widespread sight-threatening condition often only recognized when very pronounced. It is initially characterized by peripheral visual field losses, while advanced stages also affect the central vision. Some of these patients may experience visual hallucinations, the Charles Bonnet syndrome (CBS). In this systematic review and meta-analysis, we provide an overview of the literature dealing with the prevalence of CBS in patients with glaucoma. We searched the databases PubMed/MEDLINE, Embase, Web of Science, the Cochrane Central and PsycInfo on 22 March 2020. Eight studies (n = 827 patients) were identified and included for a qualitative and quantitative analysis. No studies included a representative sample of patients with only glaucoma. In patients with glaucoma in different stages and with ocular comorbidities, prevalence of CBS was 2.8% (CI95%: 0.7-6.1%). Among patients with glaucoma where all had bilateral low visual acuity, prevalence of CBS was 13.5% (CI95%: 8.4-19.6%). In patients with glaucoma who visited vision rehabilitation clinics, presumably due to an extensive vision impairment, prevalence of CBS was 20.1% (CI95%: 16.8-23.6%). Risk factors of CBS besides low vision were high age, female gender, reduced contrast sensitivity and not living alone. Taken together, we find that CBS may not be rare in patients with advanced glaucoma with and without ocular comorbidities. However, limitations of the current literature should be highlighted and careful approach towards conclusions is important. More studies are needed to better understand the prevalence and risk factors among different populations of patients with glaucoma., (© 2020 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Purpose: Cataract surgery on eyes with shallow anterior chambers may be demanding. Glycerol intake prior to surgery has been a well-known method in an effort to increase anterior chamber depth. It is used since it is thought that glycerol as an osmotic agent causes the vitreous body to shrink, pulling back the iris and thereby deepening the anterior chamber - making the surgery easier. Our controlled clinical trial tests this hypothesis and investigates the effect of glycerol on anterior chamber depth (ACD), intraocular pressure (IOP), corneal thickness (CCT), pupil diameter change after viscodilation (PD), operating time and perioperative complications., Methods: We performed a controlled clinical trial. All patients underwent cataract surgery on both eyes with at least 7 days apart. Preoperatively the patient was given glycerol orally when the right eye was operated - when the left eye was operated, nothing was given. In this way, each patient was serving as its own control. Measurements of ACD, IOP and CCT were performed before and after glycerol intake, pupillary diameter was measured before and after viscoelastics during the operation, and operating time and surgical complications were noted., Results: The study included 22 patients with bilateral cataract and anterior chambers depth <2.5 mm. Glycerol caused the anterior chamber to increase by 0.022 mm (p < 0.05), and IOP was lowered by 5.1 mmHg compared to the control group (p < 0.05). However, exposure to glycerol showed no effect on CCT, pupillary dilation of viscoelastics, operating time or surgical complications., Conclusion: Glycerol increases anterior chamber depth and lowers intraocular pressure significantly. These changes had no significant impact on operating time nor on the complication rate, suggesting that these changes are too subtle to have a clinical impact on the cataract procedure., (© 2020 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

There is no consensus on the surgical management of coexisting cataract in patients who undergo glaucoma surgery. In this study, we systematically reviewed the literature to compare the efficacy and safety of phacotrabeculectomy and trabeculectomy either alone or followed by later phacoemulsification. We systematically searched the literature databases PubMed/MEDLINE, EMBASE, and the Cochrane Central. Eligible studies were comparative trials of eyes with glaucoma that underwent either phacotrabeculectomy or trabeculectomy with or without later phacoemulsification. Our primary outcome measure was intraocular pressure (IOP) control closest to 12 months. Secondary outcome measures were efficacy closest to 12 months in terms of visual acuity, visual field, prevalence of complications, needling or revision, number of antiglaucomatous medications, and surgical success. We identified 25 studies with a total of 4,749 eyes. The IOP did not differ significantly between those who underwent phacotrabeculectomy versus trabeculectomy with (MD: 0.63, CI95%: -0.32, 1.59, p =0.19) or without later phacoemulsification (MD: -0.52, CI95%: -1.45, 0.40, p =0.27). However, phacotrabeculectomy was associated with lower risk of complications (RR: 0.80, CI95%: 0.67, 0.95, p =0.01) and better visual acuity corresponding to a 1.4-line difference (MD: -0.14, CI95%: -0.27, -0.95, p =0.03) compared to trabeculectomy. Other secondary outcome measures did not differ significantly (visual field, needling or revision, number of antiglaucomatous medications, and surgical success). In conclusion, postoperative IOP is comparable, and the number of complications is lower when phacotrabeculectomy is compared to trabeculectomy with or without later phacoemulsification in patients with coexisting glaucoma and cataract. However, our study also reveals that the level of evidence is low, and randomized clinical trials are warranted., Competing Interests: The authors declare that there are no conflicts of interest regarding the publication of this paper., (Copyright © 2021 Afrouz Ahmadzadeh et al.)

Importance: Red reflex testing is a simple and inexpensive method implemented in many countries as an important part of infant screening for ocular pathologies., Objectives: To review the literature on the diagnostic accuracy of the red reflex test in infant screening for ocular pathologies and to perform meta-analyses to provide summary estimates., Data Sources: The following literature databases were searched for English-language, peer-reviewed literature, published until April 19, 2020: Cochrane Central, PubMed/MEDLINE, Embase, Web of Science Core Collection, BIOSIS Previews, Current Contents Connect, Data Citation Index, Derwent Innovations Index, KCI-Korean Journal Database, Russian Science Citation Index, SciELO Citation Index, CINAHL (Cumulative Index to Nursing and Allied Health Literature), and ClinicalTrials.gov., Study Selection: Eligibility criteria were defined according to population (studies of consecutively screened infants), exposure (red reflex or Brückner test as the index test), comparator (any ophthalmological examination), and study type (any study with diagnostic test accuracy data)., Data Extraction and Synthesis: The Preferred Reporting Items for Systematic Reviews and Meta-Analysis of Diagnostic Test Accuracy Studies (PRISMA-DTA) guidelines were followed. Data were extracted independently by 2 authors. For summary estimates of diagnostic test accuracy, the hierarchical summary receiver operating characteristics curve was used. Prevalence of ocular pathologies was introduced for a prevalence meta-analysis, which was then used in calculations of diagnostic accuracy of the red reflex test when applied in infant screening., Main Outcomes and Measures: True-positive, false-positive, true-negative, and false-negative findings; sensitivity; specificity; and positive and negative predictive values., Results: In this meta-analysis, 8713 unique infants from 5 unique studies were eligible for qualitative and quantitative review. All studies used the red reflex test without pupillary dilation and were compared with a reference test performed with pupillary dilation. For any ocular pathology, an estimated sensitivity of 7.5% (95% CI, 7.4%-7.5%) and specificity of 97.5% (95% CI, 97.5%-97.5%) was found. Focusing on ocular pathologies that required a medical or surgical intervention, sensitivity improved to 17.5% (95% CI, 0.8%-84.8%) and specificity remained high at 97.6% (95% CI, 87.7%-99.6%)., Conclusions and Relevance: These findings suggest that an abnormal red reflex finding most likely reflects an underlying ocular pathology. However, a normal red reflex finding during screening does not exclude ocular disease.

Purpose: Favourable outcome after glaucoma surgery depends on proper control of the inflammatory response. Failing filtration bleb and consequently increased intraocular pressure is an important cause of continuous visual field deterioration after uncomplicated glaucoma surgery. The present systematic review and meta-analysis aimed to compare the effects of employing either Mitomycin C (MMC), 5-Fluoruracil (5-FU) or no anti-metabolite in needling revision of trabeculectomies., Methods: We performed a systematic literature search in PubMed, Cochrane and EMBASE to identify randomized and non-randomized trials published between year 2000 and March 2019 comparing the efficacy of needling filtering blebs when using no anti-fibrotic agent, MMC or 5-FU in patients with glaucoma. Efficacy was defined as intraocular pressure at 12 months or latest follow-up, rate of complications, rates of success and the number of re-needling cases. Comparisons were made using network meta-analyses., Results: We identified one randomized trial and five retrospective trials. Twelve months after needling revision of trabeculectomy, no significant difference was observed when comparing the effect of the use of MMC with 5-FU or without any use of anti-metabolite on intraocular pressure, complication rates, qualified success, complete success or number of re-needling cases., Conclusion: We found no significant difference in the efficacy of using either MMC, 5-FU or no anti-metabolite in needling revision of trabeculectomies. The number of studies meeting our inclusion criteria was limited. Considering the design of studies, only one was randomized leading to an overall low quality of evidence on the subject matter. Further research is warranted., (© 2020 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Purpose: To describe long-term objective and subjective visual outcomes in a group of Danish children after cataract surgery., Methods: Follow-up examination of 56 children aged 7-18 years who had undergone uni- or bilateral cataract surgery. Subjective visual function was assessed using the Cardiff Visual Ability Questionnaire for Children (CVAQC) and compared to objective visual acuity for distance and near, contrast vision and stereopsis., Results: Better visual acuity on the better seeing and contrast vision on worst seeing eye were significant predictors of increased subjective visual function in a multivariate analysis, p = 0.024. Children in the unilateral group had a significantly better CVAQC ratio compared to children in the bilateral group, median of 0.88 (range 0.50-1.00) versus 0.80 (range 0.55-0.98), p = 0.027. Reading small print, playing ball games and seeing friends in the playground were the most difficult CVAQC items in the unilateral group and reading small print, seeing the board in the classroom and seeing friends in the playground were the most difficult in the bilateral group., Conclusion: Children with unilateral disease often have a healthy eye to support the operated eye, why they overall have better subjective visual function. Many of the most difficult visual tasks were related to academic activities which might hamper future academical capabilities., (© 2020 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Purpose: To perform an epidemiological survey of all patients in Denmark diagnosed with primary congenital glaucoma (PCG) from 1977 to 2016., Methods: A retrospective, nationwide study based on a review of medical files of all children with PCG born in Denmark from 1977 to 2016. PCG was defined as glaucoma due to isolated angle dysgenesis affecting children from birth and including late onset/late recognized PCG, excluding glaucoma associated with other congenital abnormalities. Incidence and risk factors of PCG including gender, consanguinity, family history, ethnicity, comorbidity and prematurity were evaluated and stratified by decade (1977-1986, 1987-1996, 1997-2006 and 2007-2016). Age at first symptoms, age at diagnosis, age at time of first surgery and possible diagnostic delay were assessed., Results: Of 118 patients were identified, annual incidence of PCG was 4.8 per 100 000 live born. 62.3% of cases were bilateral. The relative risk (RR) of PCG was significantly higher in boys (62.7% of patients, RR 1.60 (95% CI 1.10-2.32)). 13.6% had comorbidity. Median age at time of first symptoms was 75 days, median age at time of diagnosis was 150 days and median diagnostic delay was 75 days with no significant difference throughout the decades. Unilateral cases presented symptoms and were diagnosed significantly later than bilateral cases., Conclusion: We provide unique nation-based information on the incidence of PCG from a single country covering 40 years. Male gender was a risk factor. Diagnostic delay was unchanged throughout the 4 decades and a high percentage of comorbidity was revealed. Introducing a standardized paediatric screening of all PCG children should be considered., (© 2019 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Purpose: The intrinsically photosensitive retinal ganglion cells (ipRGCs) and sleep quality are impaired in patients with primary open-angle glaucoma (POAG). In this study, we investigated whether ipRGCs and sleep quality were also impaired in patients with normal tension glaucoma (NTG)., Methods: We performed pupillometry and sleep quality assessment in 15 patients with NTG and 17 healthy age-matched controls. Pupillometry protocol consisted of monocular stimulation with high illuminance (100 lux) red (633 nm, 300 cd/m 2 or 15.23 log quanta/cm 2 /s) and blue light (463 nm, 332 cd/m 2 or 15.27 log quanta/cm 2 /s) and binocular pupil measurements. Prior to light stimulation, patients were dark-adapted for 5 min. The late postillumination pupillary response (PIPR L ate ) to blue light was used as marker of ipRGC activity. Sleep quality was assessed by Pittsburgh Sleep Quality Index (PSQI) questionnaire., Results: The PIPR L ate to blue light was significantly reduced in patients with NTG compared to healthy subjects (p < 0.001), indicating impairment of the melanopsin-mediated pupillary pathway. There was no significant difference in the response elicited by red light (p = 0.6). Baseline pupil diameter and pupillary constriction amplitude to both red and blue light were reduced in patients with NTG (p < 0.05). The global score in PSQI was not significantly different between healthy controls and patients with NTG, indicating normal sleep quality (p = 0.6). Furthermore, we found no correlation between sleep parameters and pupillary light reflex parameters., Conclusion: Patients with NTG exhibited reduced ipRGC activity compared to healthy subjects, while no differences were observed in sleep quality., (© 2019 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Background: Children with cataract require frequent monitoring to detect complications, adjust refractive correction and treat amblyopia. This is time consuming for the families. The aim of the study was to evaluate how often children with cataract are seen as outpatients or under general anaesthesia during the first 7 years of life., Methods: We performed a retrospective chart review of all children with congenital and childhood cataract born between 2000 primo and 2017 seen at our institution. The cumulated number of outpatient visits and examinations and/or surgeries in general anaesthesia was extracted for age 1, 3, 5 and 7 years., Results: Children who had cataract surgery were seen significantly more often than children without surgery. During the first year of life, children with bilateral surgery had a median of nine outpatient visits, children with unilateral cataract had 11 and children without surgery had five outpatient visits. At 7 years of age, half of the children operated bilaterally before 1 year of age had undergone at least five procedures/examinations in general anaesthesia versus 1/4 of those with unilateral surgery and none of those without surgery. Children were seen less frequently with advancing age., Conclusion: The management, treatment and follow-up of children with cataract are demanding, requiring frequent hospital visits and repeated examinations and/or surgical procedures in general anaesthesia over many years, but mainly during the first year of life. Surgical patients are more complex and require closer follow-up. This message is important to convey to the parents at the onset of the disease., (© 2019 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Purpose: To investigate the correlation between performance on a virtual reality simulator and real-life cataract surgical performance., Setting: Nine ophthalmology departments in Denmark and Copenhagen Academy for Medical Education and Simulation, Copenhagen, Denmark., Design: Prospective multicenter study., Methods: Cataract surgeons with different experience levels were included. The participants performed 3 consecutive video-recorded phacoemulsification surgeries that were rated by masked raters using the Objective Structured Assessment of Cataract Surgical Skills (OSACSS) scoring system. Thereafter, the participants performed a previously validated test on an Eyesi virtual reality simulator. Primary outcomes were the mean OSACSS score from all 3 surgeries and the simulator score from the participants' first repetition of the performance test., Result: Nineteen surgeons participated. There was a statistically significant correlation between the simulator performance score and the mean OSACSS score across all experience levels, with a Pearson correlation of 0.65 (P = .003, R 2  = 0.42)., Conclusion: Simulator performance was significantly correlated with real-life cataract surgical performance., (Copyright © 2019 ASCRS and ESCRS. All rights reserved.)

The purpose of this systematic review was to evaluate the literature regarding prophylactic treatment of intraocular pressure (IOP) elevation after uncomplicated cataract surgery to provide an evidence-based guideline for cataract surgeons. The relevant literature was identified in EMBASE and PubMed. The risk of bias was assessed according to the 'Cochrane Handbook for Systematic Reviews of Interventions' and the ROBINS-I tool. The Grading of Recommendation, Assessment, Development and Evaluation (GRADE) criteria were used to rate the quality of evidence, and relevant data were systematically extracted to evaluate the pressure-lowering effect of the active substances. The primary outcomes for this systematic review were the absolute and relative pressure-lowering effect of the different drugs after 3-8 hr and 1 day after surgery. In total, 23 randomized controlled trials and one nonrandomized controlled study consisting of 45 treatment arms with 14 different active substances were included in the qualitative synthesis. According to the GRADE criteria, nine trials were graded as 'high' quality of evidence, 12 trials as 'moderate', while three trials were given the grade 'low' quality of evidence. The primary outcomes showed most consistency between the trials, which studied the effect of timolol, and presented a relative effect from 18.6% to 29.6% at 3-8 hr and 9.8% to 23.6% at day 1. This systematic review indicates that timolol, latanoprost and travoprost alone or medications containing timolol as an additive active substance, such as dorzolamide + timolol, brinzolamide + timolol and brimonidine + timolol, are characterized by a good relative IOP-lowering effect, which can be gained by a single dose postoperatively., (© 2019 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Surgical outcome after trabeculectomy depends on maintaining a functional bleb which requires a delicate balance between incomplete wound healing to prevent fibrosis and enough wound healing to prevent hyperfiltration. Thus, controlling the inflammatory response is essential. We conducted a systematic review and meta-analysis to compare the effectiveness of different formulations of steroids (topical, systemic and depot) and nonsteroidal anti-inflammatory drugs (NSAIDs) in achieving long-term pressure control with fewer antiglaucomatous medications, preserving visual acuity and visual fields while considering surgical and postoperative complications. We used the PubMed, EMBASE and Cochrane Libraries to identify randomized controlled trials (RCTs) comparing the effect of different formulations of steroids (topical, systemic or depot) to NSAIDs. Data on prespecified outcomes from eligible references were entered into a meta-analysis using the Review Manager 5.3 program. We identified seven RCTs with a total of 342 included patients. Topical steroids were superior to placebo in controlling intraocular pressure (IOP) and reducing the risk of complications. Supplementing topical steroids with a perioperative steroid depot (subtenon or intracamerally) or systemic steroids in the early postoperative period did not provide better outcome or a change in the risk of complications. Addition of topical NSAIDs to topical steroids in patients undergoing trabeculectomy did not change the outcome or risk profile. For patients undergoing phacotrabeculectomy and topical NSAID, there was a nonsignificant trend towards better intermediate IOP control and a reduced need for antiglaucomatous medications compared to topical steroids but there was no difference in effect for patients undergoing trabeculectomy. In conclusion, there is a low level of evidence to support the clinician in deciding which postoperative regime provides a more favourable outcome because of inconsistency in the reported outcomes between studies and a low number of patients for each comparable intervention and outcome. It does seem that topical steroids are better than no anti-inflammatory treatment after glaucoma surgery, but further research is recommended., (© 2018 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Purpose: Craniopharyngioma often causes visual loss due to the close relation to the anterior visual pathways. This study investigates the incidence and predictors of visual outcomes in patients with craniopharyngioma., Methods: Data from sixty-six patients who underwent surgery for craniopharyngioma from 2009 to 2013 in Denmark were reviewed. Primary outcomes were visual acuity (VA) and visual field (VF) defects from pre-and postoperative visits. Secondary outcomes were optic nerve atrophy (OA) and papilledema., Results: Fifty-eight patients were included. The VA of the patients 1-year after surgery improved by -0.16 log(MAR) (95%CI: -0.30 to -0.02; p = 0.0266). Visual field (VF) defects worsened in 17 eyes (30%), remained stable in 21 eyes (37%) and improved in 19 eyes (33%). The presence of papilledema and the absence of OA were significantly correlated with an improvement in VA postoperatively (p = 0.011 and p = 0.011, respectively). Patients undergoing surgery within a week or less after their first ophthalmological examination had a significant improvement in VA (-0.36; 95%CI: -0.62 to -0.09; p = 0.0099). Patients undergoing surgery using a subfrontal approach also showed improvement in VA (p = 0.048). Tumour recurrence had a significantly worse VA outcome (p = 0.0074)., Conclusion: Patients show a slight improvement in VA 1-year after operation for craniopharyngioma. The presence of papilledema and early surgical intervention is associated with a significant improvement in VA. Early involvement of a dedicated ophthalmologist is recommended to secure an early detection of a visual decline and potential tumour recurrence., (© 2017 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Exfoliation syndrome (XFS) is the most common known risk factor for secondary glaucoma and a major cause of blindness worldwide. Variants in two genes, LOXL1 and CACNA1A, have previously been associated with XFS. To further elucidate the genetic basis of XFS, we collected a global sample of XFS cases to refine the association at LOXL1, which previously showed inconsistent results across populations, and to identify new variants associated with XFS. We identified a rare protective allele at LOXL1 (p.Phe407, odds ratio (OR) = 25, P = 2.9 × 10 -14 ) through deep resequencing of XFS cases and controls from nine countries. A genome-wide association study (GWAS) of XFS cases and controls from 24 countries followed by replication in 18 countries identified seven genome-wide significant loci (P < 5 × 10 -8 ). We identified association signals at 13q12 (POMP), 11q23.3 (TMEM136), 6p21 (AGPAT1), 3p24 (RBMS3) and 5q23 (near SEMA6A). These findings provide biological insights into the pathology of XFS and highlight a potential role for naturally occurring rare LOXL1 variants in disease biology.

Purpose: To investigate the effect of virtual reality proficiency-based training on actual cataract surgery performance. The secondary purpose of the study was to define which surgeons benefit from virtual reality training., Design: Multicenter masked clinical trial., Participants: Eighteen cataract surgeons with different levels of experience., Methods: Cataract surgical training on a virtual reality simulator (EyeSi) until a proficiency-based test was passed., Main Outcome Measures: Technical performance in the operating room (OR) assessed by 3 independent, masked raters using a previously validated task-specific assessment tool for cataract surgery (Objective Structured Assessment of Cataract Surgical Skill). Three surgeries before and 3 surgeries after the virtual reality training were video-recorded, anonymized, and presented to the raters in random order., Results: Novices (non-independently operating surgeons) and surgeons having performed fewer than 75 independent cataract surgeries showed significant improvements in the OR-32% and 38%, respectively-after virtual reality training (P = 0.008 and P = 0.018). More experienced cataract surgeons did not benefit from simulator training. The reliability of the assessments was high with a generalizability coefficient of 0.92 and 0.86 before and after the virtual reality training, respectively., Conclusions: Clinically relevant cataract surgical skills can be improved by proficiency-based training on a virtual reality simulator. Novices as well as surgeons with an intermediate level of experience showed improvement in OR performance score., (Copyright © 2017 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Purpose: To compare the anterior lamina cribrosa (LC) surface position in patients with idiopathic intracranial hypertension (IIH), primary open-angle glaucoma (high-tension glaucoma [HTG] and normal-tension glaucoma [NTG]), and healthy controls using enhanced depth imaging spectral-domain optical coherence tomography (EDI-OCT)., Methods: This was a retrospective, cross-sectional observational study of 11 eyes with IIH, 20 eyes with HTG, 20 eyes with NTG, and 37 control eyes. Serial horizontal B-scans of the optic nerve head were obtained using EDI-OCT. The LC depth, defined as the distance from the Bruch membrane opening plane to the anterior LC surface, was manually measured on selected B-scans covering the central three-quarters of the optic nerve head in each eye., Results: Mean LC depth in patients with IIH (325.2 ± 92.1 µm) was significantly (p&lt;0.01) decreased compared to control subjects (387.8 ± 53.9 µm). In HTG, the mean LC depth (493.0 ± 115.2 µm) was significantly increased compared to NTG (376.6 ± 27.1 µm, p&lt;0.05) and control subjects (332.7 ± 53.1 µm, p&lt;0.001). The trans-LC pressure difference (TLPD) (intraocular pressure - cerebrospinal fluid pressure) was positively correlated with the LC depth (r = 0.96, p&lt;0.001) and inversely correlated with visual field perimetric mean deviation in patients with IIH (r = -0.88, p&lt;0.001)., Conclusions: The LC is positioned anteriorly in patients with IIH and posteriorly in glaucomatous eyes compared to normal controls. The positional changes of the LC could be a result of the TLPD between the intraocular and intracranial pressure compartments.

Purpose of the Study: Primary congenital glaucoma (PCG OMIM 231300) can be caused by pathogenic sequence variations in cytochrome P450, subfamily 1, polypeptide 1 (CYP1B1). The purpose of this study was to investigate the contribution of sequence variations in CYP1B1 in a cohort of individuals with PCG residing in Denmark., Methods: The study included 37 unrelated individuals with PCG. Individuals were investigated for CYP1B1 mutations by Sanger sequencing of polymerase chain reaction products using BigDye terminators and capillary electrophoresis., Results: A total of 12 mutations were identified and 5 of these were novel. Six were missense mutations; 4 were truncating mutations (2 nonsense and 2 frameshift); 1 was an in-frame deletion and 1 was an in-frame duplication. Mutations in CYP1B1 could fully explain the PCG phenotype in 7 individuals (18%). Five individuals were compound heterozygous or presumed compound heterozygous, 1 was homozygous and 1 was apparently homozygous. Three individuals were heterozygous for sequence variations in CYP1B1 thought to be pathogenic-one of these was p.(Tyr81Asn). Several known sequence variations with presumably no functional effect were found in the cohort., Conclusions: In this study, we identified 12 CYP1B1 mutations, 5 of which were novel. The frequency of CYP1B1 mutations in this cohort was comparable with other populations. We also detected an individual heterozygous for p.(Tyr81Asn) mutation, previously suggested to cause autosomal dominant primary open-angle glaucoma.

Purpose: For the first time to compare the 1-year success rates of trabeculectomy and the new clear-cornea filtering procedure, intrastromal diathermal keratostomy (IDK)., Methods: Prospective clinical observational study including 99 consecutively operated eyes (69 patients) with well-established primary open-angle glaucoma referred for filtering operation. We compared the change in intraocular pressure (IOP), number of anti-glaucomatous medication, complications and reoperations after 1 year. Seventy-four eyes were operated with trabeculectomy, and 25 with IDK. Complete success was defined as IOP ≤ 18 mmHg, IOP lowered ≥ 30%, no medication and no reoperation. Qualified success was defined as IOP ≤ 18 mmHg, and IOP lowered ≥ 30% with or without medication or reoperation., Results: Intraocular pressure pre- and postoperatively in the trabeculectomy group was 25.3 and 14.1 mmHg versus 23.8 and 15.8 mmHg in the IDK group. The number of medications pre- and postoperatively in the trabeculectomy group was 3.1 and 0.5 versus 3.2 and 1.3 in the IDK group. Forty-four (59%) of the eyes in the trabeculectomy group met the complete success criteria compared with 5 (20%) in the IDK group (p < 0.01). Sixty-eight (92%) in the trabeculectomy group and 16 (64%) in the IDK fulfilled the qualified success criteria (p < 0.01)., Conclusion: Both the complete and qualified 1-year success rates of IDK are significantly lower compared with the success rates of trabeculectomy. The IDK does not reduce the IOP as much as the procedure of trabeculectomy, and it carries a higher frequency of reoperations and more medication postoperatively. Thus, our prospective data do not support the procedure of IDK as 'the gold standard' for filtering surgery in patients with open-angle glaucoma., (© 2011 The Authors. Acta Ophthalmologica © 2011 Acta Ophthalmologica Scandinavica Foundation.)

Purpose: To investigate whether normal tension glaucoma (NTG) is associated with increased risk of developing dementia/Alzheimer disease (AD)., Methods: A total of 69 patients with NTG were identified in the case note files in the Glaucoma Clinic, University Hospital of Copenhagen (Rigshospitalet), Denmark from 1980 to 2001. The rate of a subsequent diagnosis of dementia as recorded in nationwide health care registers from 1980 to 2005 was estimated and compared with the rate for the general population [standard mortality rate (SMR) = observed number of cases of dementia/expected number of cases of dementia]., Results: The 69 patients had a median age of 64 years (quartiles 57 and 73) at the time of the diagnosis, and the average follow-up period was 12.7 years. The SMR(dementia) was 0.20 (95% CI 0.01-1.11). No patient developed AD., Conclusion: Normal tension glaucoma was not associated with an increased risk of developing dementia/AD., (© 2011 The Authors. Acta Ophthalmologica © 2011 Acta Ophthalmologica Scandinavica Foundation.)

Axenfeld-Rieger syndrome (ARS) is a rare autosomal dominant disorder, which encompasses a range of congential malformations affecting the anterior segment of the eye. ARS shows genetic heterogeneity and mutations of the two genes, PITX2 and FOXC1, are known to be associated with the pathogenesis. There are several excellent reviews dealing with the complexity of the phenotype and genotype of ARS. In this study, we will attempt to give a brief review of the clinical features and the relevant diagnostic approaches, together with a detailed review of published PITX2 and FOXC1 mutations.

Purpose: To study the effect of dorzolamide on the preretinal oxygen tension (RPO(2)) in retinal areas affected by experimental branch retinal vein occlusion (BRVO) in pigs., Methods: Experimental BRVO was induced by diathermy close to the optic disc. RPO(2) was measured with an oxygen-sensitive electrode 0.5 mm above the BRVO-affected area, which was compared to the retinal areas not affected by BRVO. In one group of five pigs, RPO(2) was measured at baseline, 1 and 3 hours after BRVO, and after intravenous injection of 500 mg dorzolamide. In a second group of five pigs, RPO(2) was measured 1 week after the BRVO, both before and after intravenous injection of 500 mg dorzolamide., Results: The average baseline RPO(2) was 2.64 +/- 0.09 kPa (mean +/- SD). In the BRVO-affected areas, RPO(2) decreased significantly (by 0.67 +/- 0.29 and 0.94 +/- 0.13 kPa) at 1 hour and 3 hours after BRVO induction. In the non-BRVO areas RPO(2) increased significantly (by 0.51 +/- 0.14 kPa) 1 hour after BRVO induction, but subsequently decreased and reached baseline 3 hours after BRVO induction. One week after BRVO induction, RPO(2) was 0.67 +/- 0.29 kPa lower in affected areas when compared with the non-BRVO areas. In the BRVO-affected areas, dorzolamide increased RPO(2) significantly (by 0.36 +/- 0.21 kPa at 3 to 4 hours and by 0.67 +/- 0.40 kPa) 1 week after BRVO induction., Conclusions: Retinal hypoxia induced by experimental BRVO remained significant 1 week after BRVO. Dorzolamide increased retinal oxygen tension in the BRVO-affected areas both at 4 hours and 1 week after experimental BRVO in pigs.