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Abstract Objective The aim of this prospective pilot study is to establish an initial database to register patients diagnosed with different types of childhood glaucoma and the set-up of a national registry for childhood glaucoma (ReCG) in Germany. 28 children with different types of diagnosed childhood glaucoma, who were admitted and treated at the Childhood Glaucoma Center of the University Medical Center Mainz, Germany were included. Main outcome measures were the type of childhood glaucoma, mean intraocular pressure (IOP) and genetic data of the patients. Results The documents and questionnaires for each individual included: informed consent form of the parents, medical history form of the child, patient’s gestational history questionnaire and general anesthesia examination form. Primary congenital and secondary childhood glaucoma were revealed in 11 (39%) and 17 (61%) patients, respectively. The mean IOP measured with Perkins tonometer in all patients under general anesthesia at the time of inclusion was 17.5 ± 11.8 mmHg in the right and 17 ± 8.9 mmHg in the left eyes. In 33% of children with glaucoma mutations in the CYP1B1, FOXC1, LTBP2 and TEK genes were found. The development of specific questionnaires for childhood glaucoma provides detailed baseline data to establish a ReCG in Germany for the first time.

Abstract Background Severe congenital ophthalmological malformations and glaucoma might be an important occasional feature in patients with Coffin-Siris syndrome (CSS), especially Coffin-Siris syndrome 9 (CSS9, OMIM #615866) caused by SOX11 mutation. Recently, primary (open-angle) glaucoma was described in two children with the most common form of Coffin-Siris syndrome, CSS1 (OMIM #135900) by ARID1B (AT-rich interaction domain-containing protein 1B) gene mutation. In this article, we present the first report of glaucoma with Coffin-Siris syndrome 9 as well as the first report of secondary glaucoma with any form of Coffin-Siris syndrome. These findings indicate that secondary glaucoma is an occasional finding in patients with Coffin-Siris syndrome. Case presentation A child with secondary childhood glaucoma and additional ocular manifestations was evaluated and treated at the childhood glaucoma centre in Mainz, Germany. Examination under general anaesthesia revealed ocular anterior segment dysgenesis (ASD) (Peters type iridocorneal dysgenesis) in combination with congenital limbal stem cell deficiency (LSCD), aniridia, and cataract. The patient also had multiple other congenital anomalies and severe developmental delay. To explain his combination of anomalies, molecular genetic analysis from peripheral blood was performed in late 2018 and early 2019. Following normal findings with a panel diagnostic of 18 genes associated with congenital glaucoma, whole exome sequencing was performed and revealed a novel likely pathogenic heterozygous variant c.251G>T, p.(Gly84Val) in the SOX11 gene (SRY-related HMG-box gene 11). The variant had occurred de novo. Thus, the multiple congenital anomalies and developmental delay of the patient represented Coffin-Siris syndrome 9 (CSS9, OMIM #615866). Conclusions When eye diseases occur in combination with other systemic features, genetic analysis can be seminal. Results indicate that glaucoma is an occasional feature of patients with Coffin-Siris syndrome. As early treatment may improve the visual outcome of patients with glaucoma, we suggest that patients with Coffin-Siris syndrome should receive specific ophthalmological screening.

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.

Introduction Neonates and young infants with diagnosed or highly suspected glaucoma require an examination under anaesthesia to achieve accurate intraocular pressure (IOP) measurements, since crying or squinting of the eyes may increase IOP and lead to falsely high values. IOP considerably depends on perioperative variables such as haemodynamic factors, anaesthetics, depth of anaesthesia and airway management. The aim of this paper is to report the design and baseline characteristics of EyeBIS, which is a study to develop a standardised anaesthetic protocol for the measurement of IOP under anaesthesia in childhood glaucoma, by investigating the link between the magnitude of IOP and depth of anaesthesia.Methods and analysis This is a single-centre, prospective cohort study in 100 children with diagnosed or highly suspected glaucoma all undergoing ophthalmological examination under general anaesthesia. 20 children, who undergo general anaesthesia for other reasons, are included as controls. The primary outcome measure is the establishment of a standardised anaesthetic protocol for IOP measurement in childhood glaucoma by assessing the relationship between IOP and depth of anaesthesia (calculated as an electroencephalography variable, the bispectral index), with special emphasis on airway management and haemodynamic parameters. The dependence of IOP under anaesthesia on airway management and haemodynamic parameters will be described, using a mixed linear model. Restricting the model to patients with healthy eyes will allow to determine a 95% reference region, in which 95% of the measurement values of patients with healthy eyes can be expected.Ethics and dissemination The study has been approved by the local ethics committee of the Medical Association of Rhineland-Palatine (Ethik-Kommisssion der Landesaerztekammer Rheinland-Pfalz), Germany (approval number: 2019-14207). This work will be disseminated by publication of peer-reviewed manuscripts, presentation in abstract form at national and international scientific meetings and data sharing with other investigators.Trial registration number ClinicalTrials.gov Registry (NCT03972852).

Background: To compare two-year results of a filtering trabeculotomy (FTO) to conventional trabeculectomy (TE) in open-angle glaucoma by exact matching. Methods: 110 patients received an FTO and 86 a TE. FTO avoided the need for an iridectomy due to a preserved trabeculo-descemet window anterior to the scleral flap. TE employed a trabecular block excision and iridectomy. Mitomycin C was used in both. FTO and TE were exact matched by baseline intraocular pressure (IOP) and the number of glaucoma medications. Complete and qualified success (IOP ≤18 mmHg and IOP reduction ≥ 30%, with or without medication) were primary endpoints. IOP, visual acuity (BCVA), complications and intervention were secondary endpoints. Results: 44 FTO were exact matched to 44 TE. The IOP baseline in both groups was 22.5±4.7 mmHg on 3±0.9 medications. At 24 months, complete success was reached by 59% in FTO and 66% in TE, and qualified success by 59% in FTO and 71% in TE. In FTO, IOP was reduced to 12.4±4.3 mmHg at 12 months and 13.1±4.1 mmHg at 24 months. In TE, IOP was 11.3±2.2 mmHg at 12 months and 12.0±3.5 mmHg at 24 months. Medications could be reduced at 24 months to 0.6±1.3 in FTO and 0.2±0.5 in TE. There were no significant differences between the two groups in IOP, medications, complications or interventions at any point. Conclusion: Modifying aqueous flow through a limited trabeculotomy in FTO yielded clinical outcomes similar to traditional TE but allowed to avoid an iridectomy.

Background: To compare two-year results of a filtering trabeculotomy (FTO) to conventional trabeculectomy (TE) in open-angle glaucoma by exact matching. Methods: 110 patients received an FTO and 86 a TE. FTO avoided the need for an iridectomy due to a preserved trabeculo-descemet window anterior to the scleral flap. TE employed a trabecular block excision and iridectomy. Mitomycin C was used in both. FTO and TE were exact matched by baseline intraocular pressure (IOP) and the number of glaucoma medications. Complete and qualified success (IOP ≤18 mmHg and IOP reduction ≥ 30%, with or without medication) were primary endpoints. IOP, visual acuity (BCVA), complications and intervention were secondary endpoints. Results: 44 FTO were exact matched to 44 TE. The IOP baseline in both groups was 22.5±4.7 mmHg on 3±0.9 medications. At 24 months, complete success was reached by 59% in FTO and 66% in TE, and qualified success by 59% in FTO and 71% in TE. In FTO, IOP was reduced to 12.4±4.3 mmHg at 12 months and 13.1±4.1 mmHg at 24 months. In TE, IOP was 11.3±2.2 mmHg at 12 months and 12.0±3.5 mmHg at 24 months. Medications could be reduced at 24 months to 0.6±1.3 in FTO and 0.2±0.5 in TE. There were no significant differences between the two groups in IOP, medications, complications or interventions at any point. Conclusion: Modifying aqueous flow through a limited trabeculotomy in FTO yielded clinical outcomes similar to traditional TE but allowed to avoid an iridectomy.

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Primary open-angle glaucoma (POAG) is a leading cause of blindness due to chronic degeneration of retinal ganglion cells and their optic nerve axons. It is associated with disturbed regulation of intraocular pressure, elevated intraocular levels of TGF-β2, aberrant extracellular matrix (ECM) deposition and increased outflow resistance in the trabecular meshwork (TM). The mechanisms underlying these changes are not fully understood. Cell-matrix interactions have a decisive role in TM maintenance and it has been suggested that TGF-β-induced inhibition of matrix metalloproteases may drive aberrant ECM deposition in POAG. Invadopodia and podosomes (invadosomes) are distinct sites of cell-matrix interaction and localized matrix-metalloprotease (MMP) activity. Here, we report on the effects of TGF-β2 on invadosomes in human trabecular meshwork cells. Human TM (HTM) cells were derived from donor tissue and pretreated with vehicle or TGF-β2 (2 ng/ml) for 3d. Invadosomes were studied in ECM degradation assays, protein expression and MMP-2 activity were assessed by western blot and zymography and ECM protein transcription was detected by RT-qPCR. HTM cells spontaneously formed podosomes and invadopodia as detected by colocalization of Grb2 or Nck1 to sites of gelatinolysis. Pretreatment with TGF-β2 enhanced invadosomal proteolysis and zymographic MMP-2 activity as well as MMP-2, TIMP-2 and PAI-1 levels in HTM cell culture supernatants. Rho-kinase inhibition by H1152 blocked the effects of TGF-β2. Concomitant transcription of fibronectin and collagens-1, -4 and -6 was increased by TGF-β2 and fibrillar fibronectin deposits were observed in areas of invadosomal ECM remodelling. In contrast to a current hypothesis, our data indicate that TGF-β2 induces an active ECM remodelling process in TM cells, characterized by concurrent increases in localized ECM digestion and ECM expression, rather than a mere buildup of material due to a lack of degradation. Invadosomal cell adhesion and signaling may thus have a role in POAG pathophysiology.

Abstract Purpose  To evaluate existing international IT-based ophthalmological medical data projects, and to define a glaucoma data set based on existing international standards of medical and ophthalmological documentation. To develop the technical environment for easy data mining and data exchange in different countries in Europe. Methods  Existing clinical and IT-based projects for documentation of medical data in general medicine and ophthalmology were analyzed to create new data sets for medical documentation in glaucoma patients. Different types of data transfer methods were evaluated to find the best method of data exchange between ophthalmologists in different European countries. Results  Data sets from existing IT projects showed a wide variability in specifications, use of codes, terms and graphical data (perimetry, optic nerve analysis etc.) in glaucoma patients. New standardized digital datasets for glaucoma patients were defined, based on existing standards, which can be used by general ophthalmologists for follow-up examinations and for glaucoma specialists to perform teleconsultation, also across country borders. Datasets are available in different languages. Different types of data exchange methods using secure medical data transfer by internet, USB stick and smartcard were tested for different countries with regard to legal acceptance, practicability and technical realization (e.g. compatibility with EMR systems). Conclusion  By creating new standardized glaucoma specific cross-national datasets, it is now possible to develop an electronic glaucoma patient record system for data storage and transfer based on internet, smartcard or USB stick. The digital data can be used for referrals and for teleconsultation of glaucoma specialists in order to optimize glaucoma treatment. This should lead to an increase of quality in glaucoma care, and prevent expenses in health care costs by unnecessary repeated examinations. [ABSTRACT FROM AUTHOR]

AbstractPurpose:Deep sclerectomy is a non-penetrating filtering procedure that is not generally accepted, as tissue dissection is difficult and varying success rates have been reported. The purpose of the present study was to compare the use of CO2, excimer and erbium:YAG lasers in dissection of the deep corneoscleral lamella. Methods:In enucleated porcine eyes a superficial lamellar scleral flap of 5 × 5 mm was surgically dissected. The deep lamella was removed using a pulsed erbium:YAG, a CO2or an excimer laser (10 eyes/group). All eyes were analysed histologically and 3 in each group by scanning electron microscopy (SEM). Results:It is feasible to ablate the deep corneoscleral lamella with the CO2, excimer and erbium:YAG lasers without perforating the anterior chamber. The following histology and SEM showed a smoother surface after dissection with the CO2and excimer lasers compared to the erbium:YAG laser. There was no thermal damage after excimer laser treatment, compared to a damage zone of 10–30 μm using the erbium laser and one of 70–100 μm with the CO2laser. Conclusions:Excimer,erbium:YAG and CO2lasers allow the microsurgical dissection of the deep lamella. The excimer and CO2lasers achieve a more regular and smoother tissue surface. The excimer laser has the advantage to dissect without thermal tissue damage.Copyright © 2008 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

AbstractPurpose??The aim of this study is to assess the functional results and morphological parameters in children surgically treated for glaucoma.Methods??Data from 43 patients and 68 eyes who were operated in our department between 1990 and 2002 were collected. This retrospective trial included primary congenital glaucoma (n=36), and secondary glaucoma (n=7) in Rieger-Axenfeld syndrome and Sturge Weber syndrome. Intraocular pressure (IOP), axial length of the eyeball, visual acuity, refractive errors and orthoptic status were analysed.Results??The age of patients at the first surgery was 6.0?5.3 months (range 0.7 to 28.0 months). The mean period of follow-up was 57.3?36.8 months (6.0?161.0).The mean number of surgical procedures performed on one eye was 2.5?2.4 procedures (1?11). The mean IOP before the first surgery was 31.0?7.9?mmHg (17.5?52.0), and was 15.0?3.9?mmHg (7.0?28.0) at the last visit. 49 eyes (72.1%) did not need any further medical treatment after the last surgical procedure. The IOP was 18?mmHg or lower without medication in 29 eyes (42.6%) after just one surgical procedure (21 trabeculotomy, 8 combined trabeculotomy/trabeculectomy with or without mitomycin-C). At the first examination, the mean axial length of the eyeball was 22.6?1.8?mm (the mean normal value at this age is 20.3?0.7?mm), and was 24.4?2.0?mm at the last visit (the mean normal value at this age is 22.2?0.6?mm). The best corrected visual acuity at the last visit was 0.25?4.6 lines; the normal range of visual acuity at this age is from 0.4?4.0 lines to 0.8?3.0 lines. Visual acuity was 0.32 or more in 53.0% of the eyes. Visual acuity was lower than 0.1 in only 15.2% of the eyes. Myopia was present in 57.4% of the eyes with a mean spherical equivalent of ?6.1?3.9 dioptres. 15 patients (34.9%) developed strabismus. 22 patients (51.2%) were treated with part-time occlusion. Binocular function as assessed with the Lang-1 test was positive in 17 of 30 patients (56.7%).Conclusions??Although a good long-term IOP-control can often be achieved in childhood glaucoma, the visual acuity remains below the normal range in most cases despite close orthoptic follow-up. [ABSTRACT FROM AUTHOR]

Purpose To evaluate the long-term outcome of trabeculectomy under the conditions of intensified postoperative care (IPC) compared with conventional follow-up. Methods One hundred and seventy-seven consecutive primary trabeculectomies (168 patients) with a median follow-up of 4.5 years were retrospectively analyzed at the University Eye Hospital, Würzburg. Seventy-three trabeculectomies (70 patients) were followed by the surgeon under the conditions of IPC (group 1), and 104 trabeculectomies (98 patients) were followed by other ophthalmologists without IPC (group 2). The following measures to control wound healing were predominantly used in group 1: (1) increase in topical steroid administration if corkscrew vessels were present; (2) repeated injections of 5-fluorouracil (5-FU) at the beginning of bleb scarring; and/or (3) needling plus 5-FU administration if an encapsulated bleb developed. Postoperative intraocular pressure (IOP) was defined as “successful” when it did not exceed 21 mmHg and was at least 20% lower than the treated preoperative IOP. Stable visual acuity was defined as ±1 line. Results The success rates of IOP control were about 95% in both groups. In the IPC group 45 of 73 eyes fulfilled all success criteria without additional anti-glaucoma medication (61.6%), whereas in conventional follow-up only 33 of 104 eyes fulfilled all success criteria without medication (31.7%). Conclusions This study demonstrates that a much higher proportion of filtering blebs reach target IOP without medication if measures of IPC are consistently used by a glaucoma specialist when necessary. [ABSTRACT FROM AUTHOR]

BackgroundThe purpose of this study was to evaluate the influence of previous argon laser trabeculoplasty (ALT) on transforming growth factor-beta 2 (TGF-beta 2) concentration of the aqueous humor and its influence on bleb scarring after trabeculectomy.MethodsFifty-one patients with primary open-angle glaucoma (POAG) and 29 patients with exfoliation (XFS) glaucoma were recruited for this prospective study before undergoing trabeculectomy. Sixty to 200 µl of aqueous humor were analyzed for total and biologically active TGF-beta 2 concentrations (R and D Systems). TGF-beta 2 levels and a standardized bleb assessment were compared between the ALT- and non-ALT-treated groups.ResultsPOAG eyes without ALT showed significantly higher total TGF-beta 2 levels (2,317.7±1,041.1 pg/ml) than eyes with previous ALT (1,621.6±899.6 pg/ml; P=0.026). No significant difference was found for active TGF-beta 2 levels (ALT: 238.1±119.0 pg/ml; no ALT: 220.1±96.9 pg/ml; P=0.585). In XFS patients ALT did not alter total TGF-beta 2 levels (ALT: 1,524.9±624.9 pg/ml, no ALT: 1,220±499.1 pg/ml; P=0.20), but active TGF-beta 2 was significantly higher in the ALT-treated (237.0±99.7 pg/ml) than in the non-ALT-treated (140.0±95.3 pg/ml, P=0.028) group. Bleb grading revealed no statistical difference between the ALT- and non-ALT-treated groups in POAG ( P=0.545, Fisher's exact test), whereas XFS patients with ALT were at increased risk for scarring compared to non-ALT-treated patients ( P=0.053).ConclusionsALT appears to increase the risk of scarring in XFS patients because of increased levels of activated TGF-beta 2. [ABSTRACT FROM AUTHOR]