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Purpose Successful utilization of anatomical templates in the evaluation of diffusion-weighted neuroimaging-studies requires accurate registration of intra-individual datasets. We investigated the feasibility of structural MRI image registration onto single-shot and read-out segmented echo-planar diffusion-weighted imaging variants for use in tractographic samplings of the visual system, in particular the optic nerve. Methods Slab volumes of the optic nerve pathway from thirteen volunteers were acquired and preprocessed. Three neuroradiologists marked landmarks (ROIs, regions of interest) on two diffusion-weighted and one structural dataset. Structural ROIs were respectively registered (6/12 degrees of freedom, DOF) onto single-shot (ss-EPI) and readout-segmented (rs-EPI) volumes. All six ROI/FOD (fibre orientation distribution) combinations underwent a targeted tractography task (MRtrix3: iFOD2). Results Inter-rater reliability for ROI-placement was highest in VIBE images (0.66 to 0.95, mean 0.85) and lower in both ss-EPI (mean 0.77) and rs-EPI (0.46 to 0.84, mean 0.64). Sufficient FOD generation in the optic nerve was successful in 12/26 of all cases for ss-EPI volumes (46.2% of cases) and in 18/26 for rs-EPI volumes (69.2%). Spatial shift of VIBE-drawn ROI-coordinates after 6-DOF registration was highest for ss-EPI-targets (medians: 1.15 to 1.4 mm; rs-EPI 0.82 to 0.93 mm), whereas 12-DOF registration caused less spatial shifts (ss-EPI: 0.64 to 1.03 mm; rs-EPI: 0.58 mm to 0.79 mm). Tractography results revealed no significant differences between ss-EPI and rs-EPI sequences on cases with mutual generation of sufficient FODs (n=10). Conclusion Structurally-placed ROIs (highest inter-rater reliability) with 6-DOF registration onto rs-EPI targets (highest FOD-generation rate) seems most suitable for white matter template generation.

ZusammenfassungGlaukomerkrankungen im Kindesalter sind selten. Da ohne Behandlung in der Regel die Erblindung eintritt, ist eine umgehende Diagnosestellung und Therapie von höchster Bedeutung. Die Heterogenität der Erkrankung erfordert eine genaue Kenntnis der möglichen Ursachen und deren individuelle Behandlung. Dieser Artikel soll einen Überblick über die kindlichen Glaukome vermitteln und die wichtigsten Aspekte der Therapie aufzeigen.

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.

Trabeculectomy (TE) remains one of the most frequently performed glaucoma procedures. This surgery enables sufficient reduction of intraocular pressure (IOP) and freedom from using eye drops; however, complication management and time-consuming postoperative care make many ophthalmic surgeons reluctant to include TE in their treatment regimen.This review presents the value of TE compared to other forms of filtering and nonfiltering glaucoma surgery.The use of TE is still the most effective method for lowering IOP and in comparison to other forms of glaucoma surgery in most cases enables freedom from eye drops with a subsequent high quality of life. Postoperative complications occur more frequently, but usually heal spontaneously or can be treated adequately and safely by surgery; however, TE requires intensive postoperative care, which should not be neglected. Repeated surgery due to insufficient IOP reduction is less frequent compared to other forms of glaucoma surgery.HINTERGRUND: Die Trabekulektomie (TE) gehört weiterhin zu den am häufigsten durchgeführten Glaukomeingriffen. Diese Operationstechnik ermöglicht eine suffiziente Drucksenkung und Tropfenfreiheit. Jedoch führen das Komplikationsmanagement sowie die zeitintensive Nachbehandlung dazu, dass viele Ophthalmochirurgen der TE zurückhaltend gegenüberstehen.Die vorliegende Übersichtsarbeit präsentiert den Stellenwert der Trabekulektomie im Vergleich zu anderen filtrierenden und nicht filtrierenden Glaukomoperationen.Die TE ist weiterhin die effektivste Methode zur Senkung des Augeninnendrucks und ermöglicht im Vergleich zu anderen Glaukomoperationen in den meisten Fällen eine Tropfenfreiheit mit konsekutiv hoher Lebensqualität. Postoperative Komplikationen treten zwar häufiger auf, heilen aber meistens spontan ab oder können operativ gut und sicher versorgt werden. Jedoch benötigt die TE eine nicht zu vernachlässigende intensive Nachbehandlung. Erneute Operationen wegen unzureichender Augeninnendrucksenkung sind seltener im Vergleich zu anderen Glaukomoperationen.

Trabeculectomy represents the glaucoma surgical procedure that achieves the greatest reduction in intraocular pressure and enables a low pressure regulation especially in normal pressure glaucoma. For the beginner the operation is challenging due to potential intraoperative and postoperative difficulties. With this in mind this article explains how to learn to perform a trabeculectomy and explains the various steps from assessing the indications via training, performing the surgery independently and follow-up care. The article points out the challenges during the procedure and the possible solutions and explains the individual aftercare using the Mainz treatment scheme as an example. In this way, the risk of serious complications can be reduced to a minimum and in the vast majority of patients a sufficient regulation of intraocular pressure can be achieved without the need for eye drops.Die Trabekulektomie stellt die glaukomchirurgische Maßnahme dar, die die größte Drucksenkung erzielt und insbesondere bei Normaldruckglaukom eine niedrige Druckregulation ermöglicht. Für den Anfänger stellt die Operation aufgrund möglicher intraoperativer und postoperativer Schwierigkeiten eine Herausforderung dar. Vor diesem Hintergrund erläutert dieser Beitrag das Vorgehen beim Erlernen einer Trabekulektomie und erklärt die verschiedenen Schritte von Indikationsstellung über das Anlernen und die selbstständige Durchführung der Operation bis hin zur Nachsorge. Der Beitrag weist auf Herausforderungen bei der Operation und deren Lösungsmöglichkeiten hin und erklärt die individuelle Nachsorge am Beispiel des Mainzer Therapieschemas. Hierdurch kann das Risiko für schwerwiegende Komplikationen auf ein Minimum gesenkt und bei den allermeisten Patienten eine ausreichende Tensioregulation ohne Augentropfennotwendigkeit erzielt werden.

Open-angle glaucomas are a group of chronic progressive optic nerve neuropathies with a gonioscopic open anterior chamber angle. They are one of the main causes of visual impairment and blindness in industrialized countries. The aim of this article is to discuss and evaluate the epidemiology and risk factors for the development of open-angle glaucoma and to present the screening procedure for open-angle glaucoma according to the recently published S2e guidelines of the Association of the Scientific Medical Societies in Germany (AWMF).

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.

Measuring and controlling intraocular pressure (IOP) provide the foundation for glaucoma treatment. Self-tonometry has been proposed as an alternative to measure IOP throughout the entire day better. The novel EYEMATE-SC sensor (Implandata) is implanted in the suprachoroidal space to enable contactless continual IOP monitoring. The aim of the present study was to investigate the 1-year safety, performance, and accuracy of the EYEMATE-SC in patients with primary open-angle glaucoma undergoing simultaneous nonpenetrating glaucoma surgery (NPGS).Prospective, multicenter, open-label, single-arm, interventional clinical trial.Twenty-four eyes of 24 patients with primary open-angle glaucoma who were due to undergo NPGS (canaloplasty or deep sclerectomy).An EYEMATE-SC sensor was implanted during NPGS. Goldmann applanation tonometry (GAT) measurements were compared with the sensors' IOP measurements at all postoperative visits through 12 months.Device position and adverse events.Fifteen eyes underwent canaloplasty, and 9 underwent deep sclerectomy. Successful implantation of the sensor was achieved in all eyes with no reported intraoperative difficulties. Through the 12-month follow-up, no device migration, dislocation, or serious device-related complications were recorded. A total of 536 EYEMATE-SC measurements were pairwise included in the IOP agreement analysis. The overall mean difference between GAT and EYEMATE-SC measurements was 0.8 mmHg (95% confidence interval [CI] of the limits of agreement [LoA], -5.1 to 6.7 mmHg). The agreement gradually improved, and from 3 months after surgery until the end of the follow-up, the mean difference was -0.2 mmHg (95% CI of LoA, -4.6 to 4.2 mmHg) over a total of 264 EYEMATE-SC measurements, and 100% of measurements were within ±5 mmHg of GAT.The EYEMATE-SC sensor was safe and well tolerated through 12 months. Moreover, it allowed accurate, continuous IOP monitoring.

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.

This study investigated whether prematurity and associated factors or prenatal growth restriction have long-term effects on the peripapillary retinal nerve fiber layer (pRNFL) in adulthood.Retrospective cohort study.The Gutenberg Prematurity Eye Study (GPES) is a retrospective cohort study with a prospective ophthalmologic examination in Germany. Selected individuals born term and preterm (age 18-52 years) were examined with spectral-domain optical coherence tomography (SD-OCT) in adulthood, and perinatal medical charts were reviewed. The pRNFL thickness was measured using SD-OCT. Univariate and multivariable linear regression analyses were conducted to investigate associations between pRNFL and gestational age (GA; categorical), birth weight percentile (categorical), retinopathy of prematurity (ROP) occurrence, and treatment and other perinatal parameters with adjustment for age, sex, and spherical equivalent.In total, 766 eyes of 406 preterm and full-term individuals were included (mean age 28.4 ± 8.6 years, 228 females). After adjustment for age, sex, and spherical equivalent, global pRNFL thinning was associated with moderate (GA = 33-36 wk, β = -4.68, P.001), very (GA = 29-32 wk, β = -5.72, P.001), and extreme (GA ≤ 28 wk, β = -8.69, P.001) prematurity but not with low birth weight percentile (25th percentile, P = .9) and ROP occurrence (P = .9) in multivariable analysis. ROP treatment was associated with increased pRNFL in the temporal sector (P = .002). Maternal smoking during pregnancy showed an association with pRNFL thinning (P = .07).Our data indicate that the more preterm individuals are born the more pRNFL thinning occurs, whereas prenatal growth restriction and postnatal occurrence of ROP show less effects on pRNFL thickness. Furthermore, individuals with severe ROP with treatment but not lower ROP stages without treatment showed an increased temporal pRNFL thickness.

Prcis Treatment of leakage with ocular hypotony after trabeculectomy with mitomycin C (MMC) can be safely achieved through conjunctival patch alone or combined with donor scleral graft in cases of melted underlying sclera. Purpose To report outcomes of 2 surgical approaches for treating ocular hypotony in eyes with blebs with late-onset leakage after standard trabeculectomy with MMC. Methods Thirty consecutive cases with bleb leakage and hypotony underwent bleb revision surgery between 2009 and 2014 by the same surgeon (J.W.) at the Department of Ophthalmology of the Mainz University Medical Center, Germany. In 18 patients, an autologous conjunctival patch graft was applied. In 12 patients, the underlying sclera was found melted and an additional scleral donor graft was sutured in place. The authors analyzed intraocular pressure, visual acuity, and optical coherence tomography of the macula preoperatively at 1 day, 1 week, 4 weeks, and 6 months after surgery. Results The mean IOP was 6.2±3.5 mm Hg preoperatively and 21.7±16.4 mm Hg at 1 day, 13.7±6.7 at 1 week, 13.1±5.1 mm Hg at 4 weeks, and 12.1±4.7 mm Hg at 6 months after surgery. Visual acuity (logMar) increased from 0.57±0.49 preoperatively to 0.49±0.40 at 6 months. Optical coherence tomography showed flattening of macular folds that were present before treatment. No serious adverse event was reported. Conclusions This revision technique with conjunctival patch and/or additional donor scleral graft is an effective and safe method for treating late bleb leakage and hypotony maculopathy after trabeculectomy with MMC.

Offenwinkelglaukome sind eine Gruppe chronisch progredienter Optikusneuropathien mit gonioskopisch offenem Kammerwinkel. Sie stellen eine der Hauptursachen fur Sehbehinderung und Blindheit in den Industrielandern dar. Im Rahmen dieses Beitrags sollen Epidemiologie und Risikofaktoren fur das Auftreten des Offenwinkelglaukoms diskutiert und bewertet werden sowie das Vorgehen bei der Fruherkennung eines Offenwinkelglaukoms gemas der kurzlich erschienen S2e-Leitlinie der Arbeitsgemeinschaft der Wissenschaftlichen Fachgesellschaften (AWMF) vorgestellt werden.

The primary therapy of all forms of primary and secondary congenital glaucoma is surgical. In the last few decades, the options for customized surgical approach to different types of childhood glaucoma have significantly developed. In particular, circumferential trabeculotomy has shown some superiority over conventional trabeculotomy and is now also used to treat secondary congenital glaucoma. Goniotomy is still a valid alternative. Tube implants in childhood glaucoma still need improvements.

Zusammenfassung Hintergrund Bei der Behandlung von Glaukompatienten sind die Hauptziele die Bewahrung der Sehfähigkeit und Aufrechterhaltung einer möglichst hohen Lebensqualität bei volkswirtschaftlich akzeptablen Kosten. Daher ist es wichtig, jeden einzelnen Patienten sorgfältig zu evaluieren, um einen individuellen Behandlungsansatz zu erarbeiten. Material/Methoden Basierend auf den aktuellen Erkenntnissen, werden in der Übersicht die Vor- und Nachteile der medikamentösen Glaukomtherapie sowie der gängigen glaukomchirurgischen Methoden zusammengefasst. Die verschiedenen Wirkansätze der neuen minimal-invasiven Verfahren werden erläutert, die derzeit in Deutschland am häufigsten genutzten Verfahren vorgestellt sowie Empfehlungen für Vor- und Nachsorge gegeben. Ergebnisse und Diskussion Seit einigen Jahren stehen außer verschiedenen medikamentösen Tropftherapien und den klassischen operativen Verfahren, auch neue minimal-invasive Therapiealternativen zur Verfügung. Letztere eröffnen die Möglichkeit einer früheren chirurgischen Intervention, besonders für Patienten, die bereits initial oder im Laufe der Therapie für einen medikamentösen Ansatz ungeeignet erscheinen.

To evaluate the role of intraocular pressure (IOP) fluctuations and other factors on conversion of ocular hypertension to open-angle glaucoma (OAG) within a retrospective, longitudinal cohort study. The study population included patients with ocular hypertension defined by IOP > 21 mmHg with normal appearing optic discs and no visual field defect. IOP fluctuation, mean and maximum were examined in 61 eyes over a follow-up period of 36 months (standard deviation (SD) 24). All patients underwent at least two 48-h IOP profiles including night-time IOP measurements in the supine position, visual field examinations, Heidelberg retina tomograph analyses (HRT) and optic disc photographs. Regression analyses were performed to demonstrate the impact of IOP parameters, myopia, sex, cup/disc ratio and visual field results on conversion to glaucoma. While IOP fluctuation and mean did not impact conversion, myopia proved to be a risk factor (HR 14.4; 95% CI: [3.9–53.0]; p ≤ 0.001). Over an average of three years, 6/61 converted to OAG. The study yielded a mean long-term IOP over all available pressure profiles of 18.1 mmHg (SD 3.2) and an IOP fluctuation of 1.9 mmHg (SD 1.1) within a mostly treated cohort. Conversion-free five-year rate was 59.8%. The amount of fluctuation we measured in our study sample did not result in the development of glaucoma in treated ocular hypertension patients. Myopic subjects with ocular hypertension are at a higher risk for glaucoma conversion than non-myopic ocular hypertensive subjects are.

ObjectiveTo evaluate the long-term outcomes of trabeculectomy (TE) surgery in a large cohort with a minimum follow-up of 3 years.DesignRetrospective cohort study.SettingUniversity Eye Hospital, Germany.ParticipantsThree hundred and seventy-nine patients with open-angle glaucoma underwent TE with mitomycin C (MMC) between January 2013 and February 2017 with a minimal follow-up of 3 years. Eligible patients were identified via an electronic surgical case register.InterventionsAll patients had undergone TE with MMC following a set surgical protocol. To assess the influence of cataract surgery following TE, eyes which underwent cataract surgery at least 6 months after TE were matched 1:3 by sex and age to eyes who did not undergo cataract surgery during the follow-up period.Main outcome measuresPrimary outcome was the proportion of surgical success based on intraocular pressure (IOP), surgical complications, the need for revision surgery, loss of light perception and the need for additional pressure-lowering medication.ResultsThe mean follow-up time was 6 (±0.8, IQR: 5.4–6.5) years. Seventy-three per cent of eyes achieved qualified surgical success at the last follow-up (IOP≥5 mm Hg and ≤18 mm Hg, without surgical complications or complete loss of vision) but necessitated additional medical therapy, complete surgical success with no additional medical therapy was achieved in 69% of eyes. There was no significant difference in the success probability between eyes that had undergone cataract surgery after TE and those that had not (p=0.45).ConclusionsThe results demonstrate a high and stable success rate of TE after a mean follow-up time of approximately 6 years, that is, not affected by later cataract surgery.

This study aimed to investigate associations of prematurity and associated factors with optic disc morphology in adulthood as long-term effects.Retrospective cohort study.The Gutenberg Prematurity Eye Study (GPES) is a retrospective cohort study with a prospective ophthalmologic examination of adults (age 18-52 years) in Germany. In every participant, photography of optic discs was performed with a nonmydriatic fundus camera, and optic disc measurements were done manually. The vertical cup-to-disc ratio (VCDR), optic disc area, and torted and tilted discs were assessed and exploratively compared between individuals with retinopathy of prematurity (ROP) with treatment, an ROP group without treatment and groups of individuals of different gestational ages (GAs) without ROP (GA ≤ 28 weeks, GA 29-32 weeks, GA 33-36 weeks, and GA ≥ 37 weeks [control group]).The present analysis included 743 eyes of 393 individuals born preterm and full-term (aged 28.4 ± 8.6 years, 223 females). The VCDR was significantly larger in subjects with a GA ≤28 weeks without ROP compared to the full-term control group (GA ≥37 weeks) (P = .002). Subjects with ROP without treatment also had a larger VCDR (P = .001), whereas those with ROP treatment showed a smaller VCDR than the full-term control group (P = .02). In addition, individuals with ROP treatment were more likely to have a torted disc than the full-term control group (P = .006).The present study provides evidence that individuals born extremely preterm have increased VCDR in adulthood. Furthermore, these results indicate that fetal origins affect optic disc morphology until adulthood, which might predispose the affected individual to degenerative optic nerve head diseases or being incorrectly diagnosed to glaucoma.

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.

Purpose To assess surgical success and the post-operative development of intraocular pressure between XEN45® gelstent, Preserflo® MicroShunt and trabeculectomy with mitomycin C. Methods Data from 105 eyes from 105 patients of matched cases with refractory open-angle glaucoma, who underwent surgery between January 2019, and August 2020, were evaluated. Patients underwent either stand-alone XEN gelstent insertion with Mitomycin C, stand-alone Preserflo with Mitomycin C or trabeculectomy with Mitomycin C. The primary outcome was the proportion of complete surgical success at 6 months post-operatively (i.e. intraocular pressure between 5mmHg and 18mmHg, no revision surgery, no loss of light perception and no post-operative pharmaceutical antiglaucomatous treatment). The reduction of intraocular pressure after 6 months, the classes of antiglaucomatous medication used post-operatively, best-corrected visual acuity, spherical refractive errors and astigmatism were assessed as secondary outcomes. Results We included 35 eyes in each group. After 6-month follow-up, complete success was 73.5% [95%-CI: 57.9%-89.2%] in the trabeculectomy group, 51.4% [95%-CI: 34.0%-68.8%] in the XEN group and 74.2% [95%-CI: 57.9%-90.5%] in the Preserflo group (p = 0.08). Regarding secondary outcomes, the reduction of intraocular pressure was 12.1 ± 7.9 mmHg in the trabeculectomy group and was thereby 5.8 [95%-CI: 2.2-9.6] mmHg greater compared with the XEN group (p Conclusions No statistically significant differences were found between trabeculectomy, XEN45® gelstent implantation and Preserflo® MicroShunt implantation regarding surgical success after 6 months. Yet reduction in intraocular pressure was significantly higher in the trabeculectomy group. However, all three interventions resulted in sufficiently low post-operative intraocular pressure and may therefore be considered individually for glaucoma treatment.

Congenital glaucoma disease leads to blindness if left untreated. It is estimated that 1 child in 10,000 in Germany is born with congenital glaucoma. In adults the disease develops slowly and insidiously, whereas congenital glaucoma leads to a rapid growth of the eye (buphthalmos) mostly directly after birth due to a very high intraocular pressure. The cause is dysgenesis of the iridocorneal angle. Successful operative treatment of congenital glaucoma is possible in the majority of cases (approximately 80-90%). The treatment of childhood glaucoma is primarily surgical. Since 1960 classical probe trabeculotomy has been the most commonly used procedure, which has the advantage that unlike goniotomy, it can also be performed in cases with clouding of the cornea. In this surgery the canal of Schlemm is probed and the inner wall as well as the trabecular meshwork is opened inwardly over approximately 120°. In recent years the 360° trabeculotomy procedure has become established, in which an illuminated catheter filled with viscoelastic material is used (as with conventional canaloplasty) to dilate the canal of Schlemm. The trabecular meshwork is then inwardly opened by pulling on the ends of the catheter. The results of this method are explained in this article.

Background Tube implants can lead to long-term decompensation of the cornea after decades, in particular in complicated childhood glaucoma, because of a variety of causes including contact between the tube tip and the corneal endothelium. The augmentation of a XEN implant with a Baerveldt (250) tube implant, introduced 2016 by Mermoud et al for refractory glaucoma in adult patients, may reduce this risk in children. In our retrospective study, we report on the XEN-augmented Baerveldt (250) tube implant in children. Patients and methods Ten consecutive patients with refractory childhood glaucoma who underwent XEN-augmented Baerveldt implantation by a single surgeon (F.G.) between January 2016 and August 2018 were retrospectively analyzed. In this technique, a XEN tube was inserted under a standard scleral flap from outside into the anterior chamber and its outer end was connected to the Baerveldt (250) tube. The follow-up consisted of regular anterior and posterior segment examination with intraocular pressure (IOP) measurements using a hand-held slit lamp and a Perkins applanation tonometer and/or rebound tonometer (Icare). Results Ten cases (6 female individuals, 4 male individuals, median age 6 years) were operated using a XEN-augmented Baerveldt implant [aphakic glaucoma, primary congenital glaucoma, secondary congenital glaucoma (Peters anomaly), ectropion uveae, and Stickler syndrome were 4, 3, 1, 1, and 1]. Before surgery (XEN-augmented Baerveldt implantation), median IOP was 31 mm Hg. At the last postoperative follow-up, the mean IOP was 18.5 mm Hg with a median follow-up period of 13 months (range, 3 to 21 mo). No shallow or flat anterior chamber was observed. In all cases, this technique could be performed without intraoperative complications. In 3 cases, however, a surgical revision was necessary. There was no prolonged postoperative hypotony or short-term corneal alteration. By using an S-shaped loop the original Baerveldt tube length could be preserved for potential future use. In a total of 6 out of 10 cases, the procedure was a complete success (IOP, ≤20 mm Hg without additional medication). Conclusions XEN-augmented Baerveldt implantation was used for refractory pediatric glaucoma in which conventional surgery had already failed. A new additional modification using an S-shaped loop was introduced. Short-term IOP control seems promising, but long-term results are still pending and the long-term efficacy and safety must still be proven before this technique can be generally recommended.

ZusammenfassungViele klinische Studien belegen, dass der funktionelle Ausgang der Glaukomchirurgie, insbesondere der Filtrationschirurgie, durch eine topische antientzündliche Vor- und Nachbehandlung signifikant beeinflusst werden kann. Die topischen Steroide erweisen sich sowohl in der Vor- als auch in der Nachbehandlung nach Trabekulektomie als günstig, wobei die Dauer der postoperativen Behandlung vom individuellen Befund abhängig gemacht werden soll. Eine systemische Steroidtherapie oder Immunmodulation ist beim uveitischen Sekundärglaukom für den Erfolg der Glaukomchirurgie signifikant von Bedeutung, bei anderen Glaukomformen ist ein Nutzen der systemischen Therapie nicht belegt. Eine zusätzliche topische Therapie mit nicht steroidalen Antiphlogistika ist bei einer kombinierten Katarakt-Glaukom-Chirurgie zu erwägen. Eine topische antientzündliche Therapie nach selektiver Lasertrabekuloplastik ist nicht notwendig, bei Argonlasertrabekuloplastik und Zyklophotokoagulation wirkt diese sich schmerzlindernd aus. Die intravitreale Gabe von Steroiden im Rahmen der Glaukomchirurgie muss aufgrund der Induktion von Augendruckspitzen immer kritisch abgewogen werden.

PReCIS:: The closing limbal suture after trabeculectomy with a fornix-based conjunctival flap plays no critical role in the development of corneal astigmatism and intraocular pressure (IOP). A standard removal is not recommended. Purpose To investigate the effect of removal of the conjunctival suture after trabeculectomy with fornix-based conjunctival flap on corneal astigmatism, visual acuity, and IOP. Methods Eighty-seven cases of trabeculectomy with mitomycin C with a fornix-based conjunctival flap performed in the eyes of 82 patients (5 patients underwent bilateral trabeculectomy) were enrolled in a prospective randomized study. All surgeries were conducted by the same surgeon (J.W.) in the Ophthalmology Department of the University Medical Center of Mainz, Germany. All eyes received a corneal-conjunctival, continuous, mattress, interlocked suture for closing the conjunctiva at the limbus. After randomization, in 46 cases the suture was removed 6 weeks postoperatively; in 41 patients, the suture was left in place. All patients were examined preoperatively, and at 6 weeks, 3 months, 6 months, and 12 months after surgery. Astigmatism was measured using objective refraction and corneal topography, IOP and visual acuity were also assessed. Results were compared using a Wilcoxon test or Mann-Whitney U test for single time-points. Results During follow-up, no significant differences between the 2 study groups regarding refractive or topographic values were found. Patients in the suture removal group had a significantly higher visual acuity than controls at 3 months, 6 months, and 1 year after surgery. IOP was similar in both groups throughout the study. Conclusions Removal of the conjunctival suture in trabeculectomy with a fornix-based conjunctival flap leads to a faster rehabilitation of visual acuity but does not significantly affect corneal astigmatism or IOP.

Purpose To evaluate long-term results of glaucoma surgery in newborn and infants with glaucoma. Methods Seventy-nine eyes of 52 children (age: 3 weeks-15.3 years) with primary congenital or secondary glaucoma treated between 2015 and 2017 were included. The median follow-up time was 3.9 years. Conventional probe trabeculotomy, 360° catheter-assisted trabeculotomy, filtering and cyclodestructive surgery were compared. Strict criteria for surgical success were applied: Complete surgical success (IOP below target IOP, no further surgery) and incomplete surgical success (additional surgery allowed) were analyzed, and IOP at baseline and last follow-up was compared. Results Intraocular pressure (IOP) was significantly reduced in primary congenital (preoperative IOP: 27.8 ± 7.5 mmHg vs. postoperative IOP: 14.2 ± 4.5 mmHg) and secondary glaucoma (preoperative IOP: 29.2 ± 9.1 mmHg vs. postoperative IOP: 16.6 ± 4.7 mmHg). 90% of all eyes reached target IOP with or without medication allowing for additional surgeries. As first surgery, 360° catheter-assisted trabeculotomy had a tendency to higher surgical success than other surgical approaches, while cyclodestructive procedures had lowest. Conclusions We found very promising surgical results in our childhood glaucoma patient group. Surgical success in both congenital and secondary glaucoma was high.

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.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.251GT, 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).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.

Purpose This study assesses short-term intraocular pressure (IOP) change in the fellow eye of glaucoma patients after mitomycin C–augmented trabeculectomy, filtering canaloplasty, or PreserFlo™ microshunt implantation in the treated eye. Materials and methods Retrospective chart review of 235 glaucoma patients (235 eyes) was performed. Patients underwent initial trabeculectomy (187 patients), filtering canaloplasty (25 patients), or PreserFlo™ microshunt implantation (23 patients) in one eye, while the fellow eye was naïve to any previous glaucoma surgery. IOP was evaluated before and on the 1st and 2nd days and at 1 week after surgery. Main outcome measure was IOP change in the fellow eye. Secondary outcomes were proportion of clinically significant IOP elevation in the fellow eye and evaluation of potential risk factors associated with postoperative IOP fluctuation. Results IOP in the fellow eye at 1 week after trabeculectomy was statistically significantly lower than preoperatively (p p , respectively. Conclusions This study shows an IOP-lowering effect in the fellow eye of glaucoma patients after trabeculectomy. Significant IOP rise might occur in the fellow eye of some glaucoma patients after different types of glaucoma surgery.

Background Glaucomatous eyes often show strong intraocular pressure (IOP) fluctuations and individual measurements at different time points are necessary for personalized therapy. To survey IOP variations 48-hours diurnal and nocturnal IOP measurements were performed on two consecutive days. Aims of this study were to investigate the short-term repeatability of 48-hours measurements within one patient’s IOP profile and long-term repeatability between two separate IOP profiles of the same patient. Methods A retrospective cohort study was performed evaluating data of 90 glaucoma patients in a German university medical center between 2006 and 2013. All patients underwent two separate diurnal IOP profiles of 48 h. IOP was measured at 8 am, 2 pm, 6 pm, 9 pm using Goldmann applanation tonometry and at 12 midnight using Perkins tonometry in supine position on two consecutive days. Intraclass correlation coefficients (ICC) were calculated to evaluate agreement for the same time points (each time point agreement) and for consecutive measurements within the IOP profiles (between time point agreement). ICC ≤ 0.4 was defined as poor agreement, 0.4–0.75 as moderate and ≥ 0.75 as excellent. Differences between time points were investigated by Bland Altman plots. Results Each time point measurements of profile 1 showed moderate to excellent agreement (ICCs 0.62–0.93). There was a moderate to excellent agreement for measurements between time points of profile 1 (ICCs day one 0.57–0.86, day two 0.71–0.90). Profile 2 was performed at a median interval of 12.0 months (quartiles 11.0 to 21.0). Each time point agreements within profile 2 showed ICCs from 0.23 to 0.81. It showed moderate to excellent agreement for changes between time points (ICCs 0.53–0.94). Day two demonstrated ICCs from 0.74 to 0.88. Long term IOP repeatability (over both pressure profiles) showed moderate to good agreement (ICCs 0.39–0.67). Conclusions Short and long-term agreement of IOP measurements evaluated by diurnal IOP profiles is moderate to good. Due to mostly moderate agreements, which we believe represent IOP fluctuations, we conclude that it is necessary to perform 48-hours IOP profiles to gain a better overview of the individual IOP fluctuations.

The main goals of glaucoma treatment are to preserve the visual function and maintain as high a quality of life as possible at a cost acceptable to society. Therefore, it is crucial to carefully observe each individual patient in order to determine an individual and personalized treatment approach.This article summarizes the advantages and disadvantages of medicinal glaucoma treatment as well as traditional methods of glaucoma surgery, based on the current state of knowledge. The article explains the various mechanisms of action of new minimally invasive procedures, introduces the methods mostly commonly used in Germany and gives recommendations for preoperative care and postoperative follow-up.In addition to the plethora of medicinal glaucoma treatments and classical surgical procedures, new minimally invasive treatment alternatives have become available in the past few years. The latter are an option for an earlier surgical intervention, especially in naïve or previously treated patients who appear to be unsuitable for medicinal treatment.HINTERGRUND: Bei der Behandlung von Glaukompatienten sind die Hauptziele die Bewahrung der Sehfähigkeit und Aufrechterhaltung einer möglichst hohen Lebensqualität bei volkswirtschaftlich akzeptablen Kosten. Daher ist es wichtig, jeden einzelnen Patienten sorgfältig zu evaluieren, um einen individuellen Behandlungsansatz zu erarbeiten.Basierend auf den aktuellen Erkenntnissen, werden in der Übersicht die Vor- und Nachteile der medikamentösen Glaukomtherapie sowie der gängigen glaukomchirurgischen Methoden zusammengefasst. Die verschiedenen Wirkansätze der neuen minimal-invasiven Verfahren werden erläutert, die derzeit in Deutschland am häufigsten genutzten Verfahren vorgestellt sowie Empfehlungen für Vor- und Nachsorge gegeben.Seit einigen Jahren stehen außer verschiedenen medikamentösen Tropftherapien und den klassischen operativen Verfahren, auch neue minimal-invasive Therapiealternativen zur Verfügung. Letztere eröffnen die Möglichkeit einer früheren chirurgischen Intervention, besonders für Patienten, die bereits initial oder im Laufe der Therapie für einen medikamentösen Ansatz ungeeignet erscheinen.

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.

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.

Purpose The purpose of this study was to investigate the association between intraocular pressure (IOP) and ocular geometry. Methods The Gutenberg Health Study is a population-based cohort study in Mainz, Germany. Study participants underwent a comprehensive ophthalmologic examination including noncontact tonometry, objective refraction, optical biometry, and Scheimpflug imaging of the anterior segment at the first 5-year follow-up examination (in 2012–2017). Multivariable linear regression analysis was carried out to determine associations of IOP and geometric parameter of the human phakic eye, namely central corneal thickness (CCT), corneal curvature, anterior chamber depth (ACD), lens thickness, and axial length. In addition, the relationship of IOP and the anterior chamber angle (ACA) width was analyzed. Results There were 6640 participants with phakia (age 57.3 ± 10.2 years, 49.1% women) that were included in this cross-sectional analysis. Mean IOP was 14.8 ± 2.9 mm Hg in the right eyes and 14.9 ± 2.9 mm Hg in the left eyes. IOP increased with higher CCT, greater posterior segment length, higher age (all P < 0.001), thicker lens (P = 0.003), and female sex (P = 0.05), whereas the ACD was not associated with higher IOP. The IOP increased with a narrower ACA in univariable analysis (P < 0.001), but not in adjusted analysis in subjects with an open angle. Conclusions IOP values are related to ocular geometry, as shown in this population-based study on Caucasian subjects. Thus, knowledge of the architecture of the eye is an important factor when measuring IOP. Longitudinal evaluation will analyze whether some of these parameters are also risk factors for the development of glaucoma.

Purpose Using a bleb-grading system clinically facilitates long-term follow-up of patients with previous glaucoma surgery. Clinical evaluation of these patients can be challenging for untrained ophthalmologists. Morphological bleb configuration might influence planning of follow-up visits in glaucoma patients due to different and individual prognosis after trabeculectomy. In this study, we compared the MaBAGS (Mainz Bleb Appearance Grading System), a classification system for filtering blebs with other classification systems (MBGS/Moorfields Bleb Grading System, IBAGS/Indiana Bleb Appearance Grading Scale) in reference to usability and reliability and compare it to grading by bleb photographs. Methods Forty-two eyes of 31 patients after trabeculectomy were included. Three observers, two senior and one junior observer, graded all blebs using MaBAGS, MBGS and IBAGS during slit-lamp examination. Bleb photographs were reviewed at least 4 weeks after clinical examination. Statistical analysis was performed to determine agreement between the observers using intraclass correlation coefficients. Results With MaBAGS, excellent and good levels of agreement were found for vascularity indices, Seidel test and transparency. Parameters for area and height yielded moderate agreement, while indices for conjunctival mobility and microcysts failed to show satisfying levels of agreement. Using MBGS resulted in excellent and good interobserver consistency for parameters regarding subconjunctival blood, Seidel test, and central and peripheral vascularity. Height and nonbleb vascularity reached moderate levels of agreement. Agreement for area parameters was low. With IBAGS, good levels of agreement were found for height and vascularity, and moderate for extent. In all grading systems, consistency was considerably better between the two experienced observers compared to the inexperienced grader. Conclusions MaBAGS shows good reproducibility. Using such a grading system improves precision of the description of a highly variable clinical finding. The reliability of grading by slit-lamp examination exceeds that of grading on photographs.

Purpose To determine feasibility, efficacy, and safety of ab externo 360-degree trabeculotomy with illuminated microcatheter for congenital glaucoma. Patients and methods The postoperative results of 36 eyes in 23 consecutive patients who underwent 360-degree trabeculotomy for primary congenital glaucoma (PCG) or secondary congenital glaucoma using an illuminated microcatheter were retrospectively analyzed. Success criteria were defined as intraocular pressure (IOP) ≤18 mm Hg without (complete success) and with medication (qualified success). Results In all previously nonoperated eyes with PCG (group 1), the Schlemm's canal was identified and circumferentially cannulated for 360-degree trabeculotomy. In group 2 comprising of operated eyes with PCG and eyes with secondary congenital glaucoma, the performance of 360-degree trabeculotomy failed in 4 eyes because of the Schlemm's canal occlusion or high tissue resistance of the trabecular meshwork. Mean preoperative IOP was 28.6±5 and 29.6±9 mm Hg with 7 of 20 eyes and 7 of 9 eyes receiving IOP-lowering medication in group 1 and group 2, respectively. Mean postoperative IOP was reduced to 13±2.7 and 20.2±7.1 mm Hg after a mean follow-up of 15.3 and 12.7 months with 4 of 20 eyes and 5 of 9 eyes receiving medication in group 1 and group 2, respectively. Four eyes underwent further surgery. Complete success was achieved in 16 and 3 eyes, whereas qualified success was achieved in 20 and 4 eyes from group 1 and group 2, respectively. In all eyes, a mild to moderate postoperative hyphema was observed. Conclusions Ab externo catheter-assisted 360-degree trabeculotomy controls IOP in a majority of patients with congenital glaucoma after a single operation. Moderate blood reflux in the anterior chamber is considered as a common postoperative finding. We did not observe hypotony or other severe complications in our series. In 1 patient, we experienced catheter misdirection in an eye that had previously undergone trabeculotomy.

To evaluate the distribution of peripapillary retinal nerve fiber layer thickness as measured by optical coherence tomography and its associations with ocular parameters in a population-based setting. In the population-based Gutenberg Health Study, 1974 subjects from the Mainz-Bingen region were scheduled for ophthalmologic examinations, including visual acuity, refraction, tonometry, biometry, and measurements of peripapillary retinal nerve fiber layer thickness (pRNFL) by optical coherence tomography (SD-OCT, Spectralis®, Heidelberg Engineering, Germany). The associated factors in the global and sectoral pRNFL data were analyzed using multivariable linear mixed models related to age, sex, and ocular parameters. Advanced age and longer axial length were associated with a thinner pRNFL, whereas pRNFL thickness was independent of sex, intraocular pressure (IOP), and central corneal thickness (CCT). Spherical equivalent was positively associated with pRNFL thickness, except in the temporal pRNFL sector. In this large, population-based study, pRNFL thickness decreased with age. Longer eyes had thinner pRNFL thickness. Sex, IOP, and CCT showed no significant association with pRNFL thickness. These data may improve the interpretation of pRNFL data.

We report a case of a rare complication after trabeculotomy combined with a small trabeculectomy with mitomycin C in a young patient with juvenile glaucoma. The patient underwent uneventful surgery. However, postoperatively, he experienced a long-lasting hypotony with the need of 2 revision surgeries and 2 short episodes of high-intraocular pressure. He developed a fixed dilated pupil over time.

Glaucoma leads to blindness but there are diagnostic and therapeutic developments that aid ophthalmologists in the improved mangement of the disease compared to the past. Known risk factors such as age, myopia, dark pigmented skin, genetics, and elevated intraocular pressure play an important role for the individual patient's prognosis, and many yet unknown or not sufficiently investigated risk factors come along. The structured examination of the optic nerve head is crucial for the clincial diagnosis by the ophthalmologist. At least a photo documentation - better imaging of the optic nerve head and its retinal nerve fibers - should be performed in newly diagnosed suspect glaucoma or ocular hypertension. Visual field testing is just as important and it is recommended to perform 6 visual fields in the first two years after diagnosis. Treatment principles are evolving and minimally invasive surgery techniques are upcoming. However, trabeculectomy with mitomycin C is still superior regarding long-term results compared to microstents and minimal shunt procedures.