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Background/Objectives : This study aims to compare the effectiveness of traditional rigid probe trabeculotomy and 360-degree catheter trabeculotomy in treating childhood glaucoma, underlining the necessity of early surgical intervention. Methods: This retrospective cohort study, conducted at the University Eye Hospital Mainz, Germany, included 109 patients under 18 years with childhood glaucoma who underwent rigid probe trabeculotomy or 360-degree catheter trabeculotomy between January 2015 and February 2021. Results: A total of 151 eyes from 109 patients were included. The average IOP decreased significantly in both groups, with a greater reduction seen in the 360-degree catheter trabeculotomy group (mean reduction: 10.1 ± 8.7 mmHg; p < 0.001). In the rigid probe trabeculotomy group, the IOP reduction was 8.1 ± 9.0 mmHg ( p < 0.001). The need for revision surgeries was lower in the 360-degree catheter trabeculotomy group. Conclusions: Both trabeculotomy techniques effectively reduced the intraocular pressure in childhood glaucoma. The 360-degree catheter trabeculotomy group demonstrated fewer revision surgeries compared to the rigid probe trabeculotomy group. However, there was no statistically significant difference in the IOP reduction between the groups. These findings indicate that while both methods are effective in managing the IOP in childhood glaucoma, the 360-degree catheter trabeculotomy may provide more favorable long-term results.

Prcis: Bleb revision procedures for hypotony maculopathy (HM) following glaucoma filtering surgery show promising outcomes, including notable improvements in visual acuity and IOP., Purpose: This study assesses morphologic characteristics using SD-OCT in patients with HM following glaucoma filtering surgery and evaluates the results of its treatment., Materials and Methods: A retrospective analysis of all HM patients between January 2019 and March 2023. Inclusion criteria consisted of both preoperative and postrevision SD-OCT images of the macula and the presence of HM as observed on OCT images preoperatively. HM was graded according to its appearance in OCT both prerevision and postrevision surgery. Changes in visual acuity and IOP were assessed., Results: A total of 45 eyes of 45 patients were included. In all, 21 eyes had HM limited to retinal pigment epithelium (RPE), 18 eyes had involvement of RPE and photoreceptor layers, and 6 eyes had additional intraretinal or subretinal edema. After revision surgery with IOP elevation, 64% of eyes had complete HM regression with no HM signs in OCT imaging. Overall, 80% of patients achieved at least 1 grade improvement in HM. Preoperative visual acuity increased from 0.7±0.4 (logMAR) to 0.4±0.4 at 2 weeks postoperatively, over the course of an increase of IOP from 3.5±1.8 to 17.1±10.6 mm Hg at day 1. Eyes with complete HM regression had higher IOP at day 1 compared with those without improvement ( P =0.04). The median time between HM onset and revision was 10.0 days for those with complete regression and 27 days for those without improvement ( P =0.04)., Conclusions: Bleb revision procedures for HM following glaucoma filtering surgery show promising outcomes, including notable improvements in visual acuity and IOP. The timing of revision surgery appears to influence the outcome. In our study, earlier intervention was associated with better results. Even delayed surgeries can lead to an improvement, although complete morphologic restoration may not be achieved in advanced grades of HM., Competing Interests: Disclosure: The authors declare no conflict of interest., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Topic: To identify patient-reported outcome measures (PROMs) that have been used in children and adolescents with glaucoma and to evaluate their methodologic quality., Clinical Relevance: Childhood glaucoma impairs vision and quality of life (QoL) throughout all stages of life. Thus, a PROM needs to cover many different age groups and topics. Various instruments have been used to evaluate patient-reported outcomes (PROs) in patients with childhood glaucoma, however, it is unclear which PROM has the highest methodologic quality and complies best with the needs of patients with childhood glaucoma., Methods: A systematic literature review was performed searching MEDLINE (PubMed), the Cochrane Library, Web of Science, and PsycINFO (EBSCO). We included peer-reviewed full-text articles of the past 10 years in English, German, or Spanish language that reported PROMs in children with glaucoma. The study selection and methodologic quality assessment of the identified PROMs was performed by 2 independent reviewers using a 7-point checklist. The content was mapped onto the World Health Organization International Classification of Functioning, Disability and Health. The systematic review was prospectively registered in PROSPERO (ID CRD42022353936)., Results: The search strategy retrieved 3295 matches. A total of 2901 studies were screened, and 11 relevant articles were identified using 10 different instruments. The instruments addressed functional visual ability, vision-related QoL, health-related QoL, and life satisfaction. Six instruments were applicable for the use in children. Seven of the questionnaires received the highest number of positive ratings (5/7). None of the instruments considered the views of patients with childhood glaucoma during their development., Conclusion: This systematic review provides a descriptive catalog of vision-specific and generic health PRO instruments that have been used in childhood glaucoma cohorts. An instrument specifically developed for childhood glaucoma is lacking which might result in missing important factors, such as permanent treatment with eye drops, repeated surgeries, and heritability of the disease, when investigating the QoL in children with glaucoma., Financial Disclosure(s): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article., (Copyright © 2024 American Academy of Ophthalmology. All rights reserved.)

Background : The aim of this study was to evaluate the short-term efficacy and safety of the Paul Glaucoma Implant (PGI) in pediatric eyes diagnosed with glaucoma following congenital cataract surgery (GFCS). Methods : A retrospective, single-center, descriptive study was conducted on consecutive children diagnosed with GFCS who underwent PGI implantation between July 2022 and November 2023 at the University Medical Center Mainz. The primary outcome measure was the reduction in IOP at the last follow-up visit. Results : Ten eyes of nine children were included in the study. The mean follow-up time was 7.70 ± 4.22 months (4.68-10.72 months). At the end of the study follow-up, the mean (95% CI) reduction in IOP was -14.8 ± 8.73 mmHg (-8.56 to -21.04 mmHg, p < 0.001). At the last follow-up, 30.0% (3/10) of patients achieved an IOP (intraocular pressure) of ≥6 and ≤21 mmHg with a reduction in IOP of ≥25% without treatment, while 90.0% (9/10) achieved this target IOP regardless of glaucoma medication treatment. The mean number of antiglaucoma medications was significantly reduced from 3.50 (IQR = 1) to 2.0 (IQR = 2, p = 0.01), and the visual acuity logMAR improved from 1.26 ± 0.62 to 1.03 ± 0.48 ( p = 0.04). Only one eye experienced numerical hypotony (4 mmHg) without choroidal detachment or anterior chamber shallowing within the first 24 h. No other adverse events were observed during the follow-up period. Conclusions : PGI implantation significantly lowered IOP and the number of antiglaucoma eye drops with a favorable safety profile in children diagnosed with GFCS, thereby achieving a high rate of qualified surgical success in the short term.

Background: Glaucoma is not a rare entity but because very few symptoms occur and visual field defects are frequently first recognized at a late stage, a large proportion of glaucoma diseases remain undetected. The aim of this study was to identify the proportion of undiagnosed glaucoma in German population-based cohort studies and to contextualize them in the context of the literature., Material and Methods: The prevalence of glaucoma in the Gutenberg Health Study (GHS) and the age-related investigations on health of the University of Regensburg (AugUR) was evaluated based on visual field examinations and optic disc color photography according to the ISGEO criteria. Furthermore, the self-reported glaucoma diagnoses were collected and the proportion of undiagnosed glaucoma was determined., Results: The proportion of undiagnosed glaucoma was 55% in the GHS, and 53% in the AugUR study. The results correlate with results from previous studies from other countries in which the proportion of unrecognized glaucoma ranged from 33% to 78%. In the GHS and the AugUR study the proportion of undiagnosed glaucoma was higher in younger age groups and in women., Discussion: Roughly every second case of glaucoma is undetected. As the symptoms are often nonspecific or take a long time to appear, there is a risk of advanced glaucomatous visual field defects or blindness due to a lack of glaucoma awareness. Studies have shown that a systematic screening can halve this risk., (© 2023. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

This article provides an overview of real-world outcomes in glaucoma surgical procedures. While randomized clinical trials provide valuable insights, they do not fully reflect real-world clinical practice. Real-world studies enable the evaluation of outcomes in uncontrolled settings and play a crucial role in counselling and decision-making for glaucoma treatment. By examining real-world data the article aims to identify rare adverse events that may go unnoticed in controlled clinical trials. The focus is on assessing the effectiveness and safety of glaucoma surgical procedures beyond the controlled trial setting., (© 2023. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Purpose: To investigate the association of commonly used systemic medications with glaucoma and intraocular pressure (IOP) in the European population., Design: Meta-analysis of 11 population-based cohort studies of the European Eye Epidemiology Consortium., Participants: The glaucoma analyses included 143 240 participants and the IOP analyses included 47 177 participants., Methods: We examined associations of 4 categories of systemic medications-antihypertensive medications (β-blockers, diuretics, calcium channel blockers [CCBs], α-agonists, angiotensin-converting enzyme inhibitors, and angiotensin II receptor blockers), lipid-lowering medications, antidepressants, and antidiabetic medications-with glaucoma prevalence and IOP. Glaucoma ascertainment and IOP measurement method were according to individual study protocols. Results of multivariable regression analyses of each study were pooled using random effects meta-analyses. Associations with antidiabetic medications were examined in participants with diabetes only., Main Outcome Measures: Glaucoma prevalence and IOP., Results: In the meta-analyses of our maximally adjusted multivariable models, use of CCBs was associated with a higher prevalence of glaucoma (odds ratio [OR], 1.23; 95% confidence interval [CI], 1.08 to 1.39). This association was stronger for monotherapy of CCBs with direct cardiac effects (OR, 1.96; 95% CI, 1.23 to 3.12). No other antihypertensive medications, lipid-lowering medications, antidepressants, or antidiabetic medications were associated with glaucoma. Use of systemic β-blockers was associated with a lower IOP (β coefficient, -0.33 mmHg; 95% CI, -0.57 to -0.08 mmHg). Monotherapy of both selective systemic β-blockers (β coefficient, -0.45 mmHg; 95% CI -0.74 to -0.16 mmHg) and nonselective systemic β-blockers (β coefficient, -0.54 mmHg; 95% CI, -0.94 to -0.15 mmHg) was associated with lower IOP. A suggestive association was found between use of high-ceiling diuretics and lower IOP (β coefficient, -0.30 mmHg; 95% CI, -0.47 to -0.14 mmHg) but not when used as monotherapy. No other antihypertensive medications, lipid-lowering medications, antidepressants, or antidiabetic medications were associated with IOP., Conclusions: We identified a potentially harmful association between use of CCBs and glaucoma prevalence. Additionally, we observed and quantified the association of lower IOP with systemic β-blocker use. Both findings potentially are important, given that patients with glaucoma frequently use systemic antihypertensive medications. Determining causality of the CCB association should be a research priority., Financial Disclosure(s): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article., (Copyright © 2023 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Purpose: To assess long-time results of primary surgical treatment in children with glaucoma after congenital cataract surgery., Methods: A retrospective study of 37 eyes from 35 children with glaucoma after congenital cataract surgery, who underwent surgery between 2011 and 2021 at the Childhood Glaucoma Center, University Medical Center Mainz, Germany. Only children, who received a primary glaucoma surgery in our clinic within the given time (n = 25) and had at least one-year follow-up (n = 21), were included in the further analysis. The mean follow-up time was 40.4±35.1 months. The primary outcome was the mean reduction in IOP (in mmHg) from baseline to follow-up visits after the surgery, measured with Perkins tonometry., Results: 8 patients (38%) were treated with probe trabeculotomy (probe TO), 6 (29%) with 360° catheter-assisted trabeculotomy (360° TO) and 7 (33%) with cyclodestructive procedures. IOP was significantly reduced after probe TO and 360° TO after 2 years, from 26.9 mmHg to 17.4 mmHg (p<0.01) and 25.2 mmHg to 14.1 mmHg (p<0.02), respectively. There was no significant IOP reduction after cyclodestructive procedures after 2 years. Both, probe TO and 360° TO led descriptively to eye drops reduction after 2 years, from 2.0 to 0.7 and 3.2 to 1.1. The reduction was not significant., Conclusions: In glaucoma after congenital cataract surgery, both trabeculotomy techniques, lead to good reduction of IOP after 2 years. There is a need for a prospective study with comparison to the use of glaucoma drainage implants., Competing Interests: The authors have declared that no competing interests exist., (Copyright: © 2023 Strzalkowska et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.)

Glaucoma in infancy and childhood is a rare disease. An immediate diagnosis and treatment are absolutely necessary to prevent blindness of affected children. Childhood glaucoma is characterized by a heterogeneous phenotype: besides primary congenital glaucoma, secondary types often exist and the individualized treatment requires an experienced interdisciplinary team. The pathogenesis is not always discernible and genetic alterations sometimes cause the disease. A surgical procedure is usually necessary to lower the intraocular pressure. Refractive and orthoptic care are equally important to avoid amblyopia. This article gives an overview of childhood glaucoma and outlines the most important diagnostic and therapeutic aspects., (© 2023. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Background: To evaluate the long-term efficacy and safety of modified canaloplasty versus trabeculectomy in open-angle glaucoma., Methods: In total, 210 subjects with open-angle glaucoma were included. 70 were treated with Mitomycin C-augmented modified canaloplasty with enhanced subconjunctival filtration and 140 with Mitomycin C-augmented trabeculectomy. Cases were matched 1:2 by sex and age., Results: In canaloplasty and trabeculectomy groups, 61.4% and 57.9% of participants were female. Mean age was 60.0 ± 13.9 and 63.0 ± 12.2 years, median follow-up time was 4.6 [IQR 4.3, 5.05] years and 5.8 [IQR 5.4, 6.3]. Strict success was achieved in 20.0% and 56.4%, complete success in 24.3% and 66.4%, and qualified success in 34.3% and 73.6% (each p < 0.001). Kaplan-Meier survival analysis showed a better survival probability for trabeculectomy than for canaloplasty ( p < 0.001) and Cox regression analysis revealed an HR of 6.03 (95%-CI 3.66, 9.93, p < 0.001) after canaloplasty. Trabeculectomy showed superiority in terms of IOP decrease (9.2 ± 7.9 mmHg vs. 13.7 ± 10.4 mmHg, p = 0.002), use of AGM (50.0% vs. 10.7%, p < 0.001), and the number of revision surgeries (41.4% vs. 21.4%, p = 0.004). Occurrence of complications was similar in both groups (14.5% vs. 7.5%, p = 0.19)., Conclusions: Trabeculectomy showed superiority in efficacy and equality in safety compared to modified canaloplasty.

Background/aims: To examine the 5-year change in refractive error in phakic eyes and its risk factors in the general population., Methods: The Gutenberg Health Study (GHS) is a population-based cohort study including 15 010 participants from Germany aged 35-74 years at baseline examination (2007-2012). After 5 years, a follow-up examination was carried out (83% participation). 5-year change of spherical equivalent (SE) was computed as difference between follow-up and baseline objective refraction. Linear and logistic regression analysis were conducted analysing potential risk factors. Only phakic eyes at follow-up examination were included., Results: Right eyes of 10 175 subjects were included. An age-related shift of refractive error was identified, namely -0.12 D for age 35-44 years, 0.25 D for age 45-54 years, 0.25 D for age 55-64 years and 0.12 D for age 65-74 years during the 5-year follow-up. Smokers had a hyperopic shift (OR=1.31; p<0.001), while baseline SE (OR=0.89 per dioptre; p<0.001) and female sex (OR=1.49; p<0.001) were linked with a myopic shift. Education, occupation and other cardiovascular parameters were not associated with change in refractive error., Conclusions: The GHS demonstrates a parabolic shift in refractive error with a myopic shift at age 35-44 years, followed by a hyperopic shift at age 45-64 years which decreases at higher age. Smoking is associated with a hyperopic shift whereas female sex and myopic baseline SE is associated with a myopic shift. Educational level and occupation were not linked to a change in refractive error at age 35-74 years., Competing Interests: Competing interests: NP receives financial support and grants from Novartis, Ivantis, Santen, Thea, Boehringer Ingelheim Deutschland GmbH & Co. KG, Alcon, and Sanoculis. AKS receives research support from Allergan, Bayer Vital, Novartis, PlusOptix, and Heidelberg Engineering., (© 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.)

Background: To investigate whether vaccination against SARS-CoV-2 is associated with the onset of retinal vascular occlusive disease (RVOD)., Methods: In this multicentre study, data from patients with central and branch retinal vein occlusion (CRVO and BRVO), central and branch retinal artery occlusion (CRAO and BRAO), and anterior ischaemic optic neuropathy (AION) were retrospectively collected during a 2-month index period (1 June-31 July 2021) according to a defined protocol. The relation to any previous vaccination was documented for the consecutive case series. Numbers of RVOD and COVID-19 vaccination were investigated in a case-by-case analysis. A case-control study using age- and sex-matched controls from the general population (study participants from the Gutenberg Health Study) and an adjusted conditional logistic regression analysis was conducted., Results: Four hundred and twenty-one subjects presenting during the index period (61 days) were enrolled: one hundred and twenty-one patients with CRVO, seventy-five with BRVO, fifty-six with CRAO, sixty-five with BRAO, and one hundred and four with AION. Three hundred and thirty-two (78.9%) patients had been vaccinated before the onset of RVOD. The vaccines given were BNT162b2/BioNTech/Pfizer ( n = 221), followed by ChadOx1/AstraZeneca ( n = 57), mRNA-1273/Moderna ( n = 21), and Ad26.COV2.S/Johnson & Johnson ( n = 11; unknown n = 22). Our case-control analysis integrating population-based data from the GHS yielded no evidence of an increased risk after COVID-19 vaccination (OR = 0.93; 95% CI: 0.60-1.45, p = 0.75) in connection with a vaccination within a 4-week window., Conclusions: To date, there has been no evidence of any association between SARS-CoV-2 vaccination and a higher RVOD risk.

Childhood glaucoma is a rare disease. Since a lacking therapy normally leads to blindness an immediate diagnosis and appropriate treatment are crucial. The heterogeneity of childhood glaucoma requires a comprehensive knowledge of possible underlying pathomechanisms of primary and secondary childhood glaucoma types. Several types of childhood glaucoma are accompanied with ocular or systemic syndromes and hereditary causes are frequent. Thus, an interdisciplinary team of ophthalmologists, orthoptists, pediatricians, human geneticists, and anesthesiologists is vital for a successful supply. Most cases rely on a dysgenesis of trabecular meshwork with impaired outflow. Thus, usually a surgical approach is indicated. To handle a surgery in a buphthalmic eye an experienced hand is required. Special attention should be paid on the correct prescription of topical antiglaucomatous therapy in children since they are more prone to develop side effects. This article aims to provide an overview about the different childhood glaucoma types and to point out the most important aspects of their individual treatment strategies., Competing Interests: Erklärung zu finanziellen Interessen Forschungsförderung erhalten: ja, von einer anderen Institution (Pharma- oder Medizintechnikfirma usw.); Honorar/geldwerten Vorteil für Referententätigkeit erhalten: nein; Bezahlter Berater/interner Schulungsreferent/Gehaltsempfänger: nein; Patent/Geschäftsanteile/Aktien (Autor/Partner, Ehepartner, Kinder) an im Bereich der Medizin aktiven Firma: nein; Patent/Geschäftsanteile/Aktien (Autor/Partner, Ehepartner, Kinder) an zu Sponsoren dieser Fortbildung bzw. durch die Fortbildung in ihren Geschäftsinteressen berührten Firma: nein. Erklärung zu nichtfinanziellen Interessen Dr. med. Julia V. Stingl wird unterstützt durch das Clinical Research Fellowship der Mainz Research School for Translational Biomedicine (TransMed)., (Thieme. All rights reserved.)

Objective: We aimed to compare intraocular pressure (IOP) measurements using iCare® PRO rebound tonometry (iCare) and Perkins applanation tonometry (Perkins) in childhood glaucoma subjects and healthy children and the influence of anaesthesia depth, age and corneal thickness. Material: Prospective clinical, case-control study of children who underwent an ophthalmologic examination under general anaesthesia according to our protocol. Children were 45.45 ± 29.76 months old (mean ± SD (standard deviation)). Of all children, 54.05% were female. IOP was taken three times (T1−T3), according to duration and the depth of anaesthesia. The order of measurement alternated, starting with iCare. Agreement between the device measurements was evaluated using Bland−Altman analysis. Results: 53 glaucoma subjects and 22 healthy controls. Glaucoma subjects: IOP measured with iCare was at T1: 27.2 (18.1−33.8), T2: 21.6 (14.8−30.6), T3: 20.4 mmHg (14.5−27.0) and Perkins 17.5 (12.0−23.0), 15.5 (10.5−20.5), 15.0 mmHg (10.5−21.0) (median ± IQR (interquartile range)). Healthy controls: IOP with iCare: T1: 13.3 (11.1−17.0), T2: 10.6 (8.1−12.4), T3: 9.6 mmHg (7.7−11.7) and Perkins 10.3 (8.0−12.0), 7.0 (5.5−10.5), 7.0 mmHg (5.5−8.5) (median ± IQR). The median IOP was statistically significantly higher with iCare than with Perkins (p < 0.001) in both groups. The mean difference (iCare and Perkins) was 6.0 ± 6.1 mmHg for T1−T3, 7.3 at T1, 6.0 at T2, 4.9 mmHg at T3. Conclusion: The IOP was the highest in glaucoma subjects and healthy children at T1 (under sedation), independently of the measurement method. iCare always leads to higher IOP compared to Perkins in glaucoma and healthy subjects, regardless of the duration of anesthesia.

Background: Spectral domain optical coherence tomography (SD-OCT) is a widely applied non-invasive technique for evaluating optic nerve head parameters. The aim of this study was to evaluate the impact of biometric parameters such as the spherical equivalent (SE) and the anterior corneal curvature (ACC) on the peripapillary retinal nerve fiber layer (pRNFL), Bruch's membrane opening (BMO), and the minimum rim width (MRW) measurements performed by spectral domain optical coherence tomography (SD-OCT) in glaucomatous and healthy eyes., Methods: In this cross-sectional, case-control prospective pilot study, the glaucoma group consisted of 50 patients with previously diagnosed and treated glaucoma and one healthy group of 50 subjects. Two consecutive examinations of pRNFL, BMO, and MRW with SD-OCT for every patient were performed without ACC and objective refraction (imaging 1) and with them (imaging 2)., Results: The interclass correlation coefficient (ICC) reflected high agreement between imaging 1 and imaging 2 in both groups. The ICC in the glaucoma and healthy groups for pRNFL (0.99 vs. 0.98), BMO (0.95 vs. 0.97), and MRW (1.0 vs. 1.0) was comparable., Conclusions: Our preliminary data from a small number of eyes showed that the measurements of pRNFL, MRW, and BMO reflected high agreement between both imaging techniques with ACC and objective refraction and without these parameters in subjects with a refractive error up to ± 6.0 diopters. Further studies with participants with higher refractive error are necessary to evaluate the impact of biometric parameters such as SE and ACC on measurements with SD-OCT., (© 2022. The Author(s).)

Childhood glaucoma is a heterogeneous disease and can be associated with various genetic alterations. The aim of this study was to report first results of the phenotype-genotype relationship in a German childhood glaucoma cohort. Forty-nine eyes of 29 children diagnosed with childhood glaucoma were prospectively included in the registry. Besides medical history, non-genetic risk factor anamnesis and examination results, genetic examination report was obtained (23 cases). DNA from peripheral blood or buccal swab was used for molecular genetic analysis using a specific glaucoma gene panel. Primary endpoint was the distribution of causative genetic mutations and associated disorders. Median age was 1.8 (IQR 0.6; 3.8) years, 64% participants were female. Secondary childhood glaucoma (55%) was more common than primary childhood glaucoma (41%). In 14%, parental consanguinity was indicated. A mutation was found in all these cases, which makes consanguinity an important risk factor for genetic causes in childhood glaucoma. CYP1B1 (30%) and TEK (10%) mutations were found in primary childhood glaucoma patients. In secondary childhood glaucoma cases, alterations in CYP1B1 (25%), SOX11 (13%), FOXC1 (13%), GJA8 (13%) and LTBP2 (13%) were detected. Congenital cataract was associated with variants in FYCO1 and CRYBB3 (25% each), and one case of primary megalocornea with a CHRDL1 aberration. Novel variants of causative genetic mutations were found. Distribution of childhood glaucoma types and causative genes was comparable to previous investigated cohorts. This is the first prospective study using standardized forms to determine phenotypes and non-genetic factors in childhood glaucoma with the aim to evaluate their association with genotypes in childhood glaucoma.

To quantify the results of childhood glaucoma treatment over time in a cohort of children with different types of childhood glaucoma. A retrospective cohort study of consecutive cases involving children with primary congenital glaucoma, primary juvenile, and secondary juvenile glaucoma at the Childhood Glaucoma Center, University Medical Center Mainz, Germany from 1995 to 2015 was conducted. The main outcome measure was the long-term development of intraocular pressure. Further parameters such as surgical success, refraction, corneal diameter, axial length, and surgical procedure in children with different types of childhood glaucoma were evaluated. Surgical success was defined as IOP < 21 mmHg in eyes without a need for further intervention for pressure reduction. A total of 93 glaucomatous eyes of 61 childhood glaucoma patients with a mean age of 3.7 ± 5.1 years were included. The overall mean intraocular pressure at first visit was 32.8 ± 10.2 mmHg and decreased to 15.5 ± 7.3 mmHg at the last visit. In the median follow-up time of 78.2 months, 271 surgical interventions were performed (130 of these were cyclophotocoagulations). Many (61.9%) of the eyes that underwent surgery achieved complete surgical success without additional medication. Qualified surgical success (with or without additional medication) was reached by 84.5% of the eyes.

Background: Central retinal vein occlusion is a variable disease pattern. Preliminary stages of a complete occlusion of the central vein, wich are subsumed under the term venous stasis retinopathy, may occur as transient blurred vision and with subtle alterations of the fundus. Course and prognosis are benign, visual acuity usually recovers. By now, venous stasis retinopathy in children due to Valsalva maneuver has not been described in literature yet., Case Presentation: We present an impressive case of venous stasis retinopathy in a 10-year-old boy with ocular hypertension and megalocornea due to increased intraocular pressure provoked by Valsalva maneuver. Main symptom was transient blurred vision in the left eye. The intraocular pressure was 28 mmHg, fundus exam revealed tortuous veins and a flame shaped hemorrhage at 7 o'clock. Total recovery under topical antiglaucomatous therapy could be observed after 1 month., Conclusions: Acute increase in intraocular pressure, provoked by Valsalva maneuver is a risk factor for venous stasis retinopathy. Further general and vascular risk factors should be ruled out by extensive examination. Children with ocular hypertension might be at higher risk for impending vein occlusion as shown in this case.