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Purpose To evaluate the outcome of Descemet Membrane Endothelial Keratoplasty (DMEK) in eyes with pre-existing glaucoma. Design In this retrospective, observational case series we included data of 150 consecutive DMEKs in eyes with pre-existing glaucoma of 150 patients after excluding data of the second treated eye of each patient and of re-DMEKs during follow-up. Cumulative incidences of IOP elevation (IOP > 21 mmHg or ≥ 10 mmHg increase in IOP from preoperative value), post-DMEK glaucoma (need of an additional intervention due to worsening of the IOP), graft rejection, and graft failure rate were analyzed using Kaplan–Meier survival analysis. COX regression analysis was used to evaluate independent risk factors. Results The 36-month cumulative incidence of IOP elevation was 53.5% [95 CI 43.5–63.5%] and of post-DMEK glaucoma 36.3% [95 CI 26.3–46.3%]. Graft rejection occurred with a 36-month cumulative incidence of 9.2% [CI 95% 2.3–16.1]. None of the analyzed risk factors increased the risk for the development of graft rejection. The 36-month cumulative incidence of graft failure was 16.6% [CI 95% 8.4–24.8]. Independent risk factors for graft failure were the indication for DMEK “status after graft failure” (n = 16) compared to Fuchs’ dystrophy (n = 74) (p = 0.045, HR 8.511 [CI 95% 1.054–68.756]) and pre-existing filtrating surgery via glaucoma drainage device (GDD) (n = 10) compared to no surgery/iridectomy (n = 109) (p = 0.014, HR 6.273 [CI 95% 1.456–27.031]). Conclusion The risks of postoperative complications (IOP elevation, post-DMEK glaucoma, graft rejection, and graft failure) in patients with pre-existing glaucoma are high. In particular, pre-existing filtrating surgery via GDD implantation—but not trabeculectomy—and DMEK after graft failure increase the risk of graft failure.

ZusammenfassungInfektionen mit Toxoplasma gondii, Treponema pallidum und Mycobacterium tuberculosis sind nach wie vor die wichtigsten infektiösen Ursachen einer posterioren Uveitis. Epidemiologie, klinisches Bild, Diagnostik und Therapie dieser Krankheitsbilder werden dargestellt.

BACKGROUND/AIMS: Intravitreal rituximab is an off-label treatment option for primary vitreoretinal lymphoma (PVRL). The objective of this study was to monitor the therapeutic response and safety profile of intravitreal rituximab in a cohort of PVRL patients. METHODS: In this retrospective, uncontrolled, open label, multicentre study, 20 eyes from 15 consecutive patients diagnosed with PRVL received at least one intravitreal injection of 1mg in 0.1ml rituximab. Biodata of the PVRL patients was recorded as well as visual acuity and vitreous haze score immediately before rituximab intravitreal injection and at follow-up examinations. Intravitreal rituximab safety data was also recorded. Additional rituximab injections were made during control visits on a pro re nata (PRN) regime using increased vitreous haze to indicate recurrence. RESULTS: There was significant vitreous haze reduction (p=0.0002) followed by significant improvement of visual acuity (mean best visual acuity before therapy 0.57 logMAR, after therapy 0.20 logMAR (p=0.0228) during the follow-up time up to 4 years. Only mild ocular side effects were reported. Median follow-up time was 565 days (range, 7-1253 days). CONCLUSION: Intravitreal rituximab therapy shows promising PVRL regression without any severe side effects. Although our clinical data support rituximab as intravitreal therapy in PVRL disease, further study is warranted.

Introduction To evaluate the long-term effect on intraocular pressure (IOP) and glaucoma medication of selective laser trabeculoplasty (SLT) compared to minimally invasive glaucoma surgery (MIGS) in primary open-angle glaucoma (POAG) and its potential in clinical practice. Methods A total of 342 consecutive patients (stand-alone procedures) were included. One hundred and five patients underwent SLT treatment (360° SLT, 95–105 spots, Trabeculas SLT ARCLaser, Nürnberg, DE), 107 patients had an ab interno-derived trabeculotomy (Trabectome®, NeoMedix, Tustin, USA), and 130 patients received iStent inject® implantation (2 implants-Glaukos, CA, USA). IOP and glaucoma therapy were evaluated preoperatively, 1 day, 6 weeks, 3 months, 6 months, and 1, 2, and 3 years postoperatively. Statistical analysis was performed using a regression model and propensity matching score (reduced cohort number) using SPSS v20.0. Kaplan–Meier analysis was included using the following six criteria: criterion A (IOP ≤ 21 mmHg with or without medication, qualified success), criterion B (IOP ≤ 18 mmHg with or without medication, qualified success), criterion C (IOP ≤ 21 mmHg without medication, complete success), criterion D (IOP ≤ 18 mmHg without medication, complete success), criterion E (IOP ≤ 21 mmHg and IOP reduction > 20% after therapy), and criterion F (IOP ≤ 18 mmHg and IOP reduction > 20% after therapy). Results In the matched cohort, the SLT cohort showed an IOP reduction of 31.2% from 19.9 ± 2.3 to 13.7 ± 2.7 mmHg (p

Purpose The study aims to evaluate changes in contrast sensitivity (CS) during therapy with intravitreal vascular endothelial growth factor (VEGF) inhibitors in patients with neovascular age-related macular degeneration (nAMD) and diabetic macular edema (DME). Methods Prospective, uncontrolled, multicenter study on patients with neovascular AMD or DME who underwent intravitreal injection therapy with Ranibizumab, Aflibercept, or Bevacizumab was conducted. Best corrected visual acuity (BCVA) and CS measured by Mars Letter Contrast Sensitivity Test (MLCS) and Freiburg Visual Acuity and Contrast Test (FrACT) in logCS were evaluated before 3 consecutive VEGF inhibitor injections, which followed the pro renata regimen in treatment-naïve and pretreated eyes with a maximum of 9 injections. Correlation of MLCS and FrACT was calculated by the Spearman’s rank correlation coefficient. Results Eighty eyes of 74 patients (mean age 72.7; SD ± 9.96) were included. BCVA improved significantly from 0.44 (SD ± 0.21) logMAR to 0.38 (SD ± 0.23) logMAR by 0.06 (SD ± 0.14) logMAR values (p p p = 0.035). A positive correlation between MLCS and FrACT was found (r = 0.389; p Conclusions Intravitreal injection therapy with VEGF inhibitors led to an improvement of BCVA and CS measured by MLCS and FrACT. MLCS was superior and more sensitive compared to FrACT and even BCVA to evaluate CS in elderly patients with macular pathology.

The aim of this study was to evaluate whether XEN® implantation is a reasonable and safe method to lower the intraocular pressure (IOP) and amount of medication for adult primary open-angle glaucoma (POAG) over a 3-year period. The influence of the type of anesthesia, previous glaucoma surgery, and postoperative interventions on the outcome were examined.In this retrospective study, 96 eyes were included. XEN® implantation was performed as sole procedure under general (n = 86) or local anesthesia (n = 10). IOP and number of glaucoma medication were assessed preoperatively: day 1, week 6, month 3, 6, 12, 24, and 36. Further outcome parameters were Kaplan-Meier success rates, secondary intervention, and complication rates.IOP decreased from 20.7 ± 5.1 to 12.8 ± 2.5 mmHg at the 36-month follow-up (p 0.001) and glaucoma therapy was reduced from 3.3 ± 0.8 to 1.2 ± 1.6 (36 months, p 0.001). Transient postoperative hypotony was documented in 26 eyes (27.1%). General anesthesia resulted in a significant improvement of the survival rate compared to local anesthesia (77% vs. 50%, p = 0.044). Prior iStent inject®, Trabectome®, or SLT laser had no significant impact, such as filter bleb revision. The number of postoperative needlings had a significantly negative influence (p = 0.012).XEN® implantation effectively and significantly lowers the IOP and number of glaucoma therapy in POAG in the 36-month follow-up with a favorable profile of side effects and few complications. In case of IOP, general anesthesia has a significant positive influence on the survival rate, whereas prior SLT or MIGS does not have significant impact.

AimsTo investigate the retinal vascular structure and capillary anomalies of affected and fellow eyes of patients with unilateral Coats’ disease using multimodal imaging.MethodsClinical investigation of both eyes of each patient with diagnosed Coats’ disease using ultra-widefield (UWF) fundus imaging, including UWF fluorescein angiography (UWFFA), spectral domain optical coherence tomography (OCT) and optical coherence tomography angiography (OCT-A).ResultsWe analysed 38 eyes of 19 patients with unilateral Coats’ disease and found that all fellow eyes (19/19; 100%) revealed vascular alterations, detected by UWFFA, predominantly located in the temporal periphery. Thereby, 89% of the fellow eyes (17/19) presented capillary bed abnormalities, that did not exceed the capillary level; 58% (11/19) presented tortuous abnormalities and 26% (5/19) presented microaneurysmatic abnormalities, exceeding the capillary level. If primarily affected eyes presented central Coats’ specific vascular abnormalities, fellow eyes revealed tortuous vascular abnormalities twice as often (78% (7/9) vs 40% (4/10); P=0.096). In primarily affected eyes, a tendency towards larger foveal avascular zones was revealed, compared to fellow eyes (0.28±0.16 mm2 vs 0.20±0.10 mm2; P=0.123).ConclusionThe use of modern multimodal imaging allows the detection of even subtle vascular changes in fellow eyes of patients with Coats’ disease. Coats’ disease appears to be a bilateral ocular disease with a predominant manifestation in one eye of the affected patients.

ZUSAMMENFASSUNGDie Uveitis im Kindesalter weist ein hohes Erblindungsrisiko auf. Bei einer nicht infektiösen Uveitis intermedia und posterior sind lokal applizierte Steroid-Augentropfen nicht ausreichend wirksam, sodass bei Visusverschlechterung und Komplikationen eine systemische Therapie erfolgen muss. Diese entspricht im Wesentlichen dem Therapiealgorithmus der JIA-Assoziierten Uveitis anterior wie in der S2k-Leitlinie 045–012 zur Diagnostik und antientzündlichen Therapie der Uveitis bei juveniler idiopathischer Arthritis mit Stand 01/2018 beschrieben. Bei pädiatrischen Patienten jedoch, die nur an einem Auge ohne Systemerkrankung leiden, wäre eine intravitreale Therapie wünschenswert, um Nebenwirkungen einer systemischen Therapie zu vermeiden. Intravitreal verabreichte Steroide weisen zwar eine sehr gute Wirksamkeit auf. Leider besteht jedoch das Risiko für ein Steroid-induziertes Glaukom sowie eine Steroid-induzierte Katarakt, sodass intravitreal verabreichte Steroide laut S2k-Leitlinie nur als Rescue-Therapie genutzt werden sollten.

Die White-dot-Syndrome umfassen eine Gruppe von Erkrankungen, die durch gelb-weisliche oder grauliche Herde in der auseren Netzhaut, dem retinalen Pigmentepithel und der Aderhaut, charakterisiert sind. Sowohl klinisch als auch wissenschaftlich betrachtet stellen sie eine diagnostische und therapeutische Herausforderung dar. Zu den White-dot-Syndromen gehoren primare inflammatorische Choriokapillaropathien wie die akute posteriore multifokale plakoide Pigmentepitheliopathie (APMPPE)/akute multifokale ischamische Choriokapillaropathie (AMIC), das „multiple evanescent white-dot-syndrome“ (MEWDS)/„acute idiopathic blind spot enlargement syndrome“ (AIBSE), die multifokale Choroiditis (MFC), die punktformige innere Choroidopathie (PIC), die serpiginose Choroiditis (SC), „acute zonal occult outer retinopathy“ (AZOOR) und die akute makulare Neuroretinopathie (AMN). Zu den primar stromalen Choroiditiden gehort die Birdshot-Retinochoroidopathie (BSRC). Die Pathogenese dieser Erkrankungen ist weitestgehend unbekannt. Immunologische Reaktionen auf vorangegangene virale Infekte bei genetischer Disposition scheinen ein gemeinsamer Nenner zu sein.

The aim of this study is to assess whether the ab interno canaloplasty is a reasonable minimally invasive method to lower significantly the IOP level and number of antiglaucomatous medication over a certain period of time in adult primary open angle glaucoma (POAG). In this retrospective cohort outcome study, 36 eyes of 28 POAG patients (mean age 74.8 ± 9.3 years) with an IOP above target pressure were included. Ab interno canaloplasty (ABiC) was performed in all subjects (MEyeTech GmbH, Alsdorf, Germany) as sole procedure in pseudophakic eyes (n = 20) or in combination with cataract surgery in phakic eyes (n = 16). The intraocular pressure (IOP) and the number of glaucoma medication were assessed preoperatively, day 1, week 6, month 3, month 6, and month 12. IOP decreased from 19.8 ± 4.1 to 13.8 ± 3 mmHg at 12 months follow-up (n = 21, p

Background: Knowledge about artifacts in optical coherence tomography angiography (OCTA) is important to avoid misinterpretations. An overview of possible artifacts in posterior uveitis provides important information for interpretations. Methods: In this monocentric prospective study, OCTA images from a total of 102 eyes of 54 patients with posterior uveitis, and an age-matched control group including 34 healthy subjects (67 eyes), were evaluated (day 0, month 3, month 6). We assigned different artifacts to distinct layers. Various types of artifacts were examined in different retinal layers. The χ2 test for the comparison between the control and uveitis group and Cochran’s Q test for the longitudinal comparison within the uveitis group were used. Results: A total of 2238 images were evaluated; 1836 from uveitis patients and 402 from healthy subjects. A total of 2193 artifacts were revealed. Projection (812 [36.3%]), segmentation (579 [25.9%]), shadowing (404 [18.1%]), and blink artifacts (297 [13.3%]) were the most common artifact types. The uveitis group displayed significantly more segmentation artifacts and projection artifacts (p < 0.001). No segmentation artifacts were documented in healthy subjects. The consecutive examinations within the uveitis group revealed the same artifact types without significance (p > 0.1). Conclusions: The uveitis patients showed more segmentation and projection artifacts than the control group. Within the uveitis group, artifacts remained longitudinally constant in terms of artifact type and pattern. The artifacts therefore appear to be reproducible on an individual level.

Purpose To compare the blood flow situation in primary open-angle glaucoma (POAG) and pseudoexfoliation glaucoma (PXG) using optical coherence tomography angiography (OCTA). Methods In this prospective study a total of 26 POAG and 23 PXG eyes were included. All patients underwent a complete ophthalmological examination including standard automated perimetry, stereoscopic photographs of the optic disc, peripapillary retinal nerve fibre layer analysis and examination of vascular parameters of the optic nerve head (ONH), the peripapillary region and macula using OCTA. In addition to the vascular parameters recorded by the device, the vascular images were graphically evaluated using Image J. All recorded vascular parameters were compared between both groups and correlated to structural and functional parameters. Results The mean superficial perifoveal plexus perfusion density (PD) was significantly lower in PXG eyes than compared to POAG eyes using OCTA (32.57% ± 3.57% vs. 34.92% ± 2.11%, p = 0.007). The mean PD parameters for the superficial peripapillary plexus (40.98% ± 3.04% vs. 42.09% ± 2.29%, p = 0.152) as well as the size of the foveal avascular zone (FAZ) (0.23 mm2 ± 0.1 mm2 vs. 0.23 mm2 ± 0.09 mm2) did not differ between both groups. Additional graphic evaluation using Image J showed no significant difference for superficial perifoveal plexus PD (32.97% ± 1.11% vs. 33.35% ± 0.95%, p = 0.194) and peripapillary plexus PD (46.65% ± 0.83% vs. 46.95% ± 0.5%, p = 0.127) between the groups. Retinal nerve fibre layer (RNFL) thickness correlated significantly with peripapillary plexus PD for both OCTA data and Image J data (p p = 0.032). Conclusion The severity of the glaucoma seems to be crucial for peripapillary and macular perfusion densities, and not the form of glaucoma. An additional graphic evaluation is a possible step that could be implemented to improve the comparability of OCTA scans and to optimize the possibility of quantitative perfusion analysis in the case of deviating quality criteria.

PURPOSE Intraocular pressure (IOP) elevation occurs regularly after Descemet membrane endothelial keratoplasty (DMEK). This study evaluated the long-term incidence of IOP elevation and post-DMEK glaucoma after DMEK. METHODS A total of 463 consecutive DMEKs in 352 patients performed between September 2011 and September 2014 at the Universitatsmedizin-Charite Berlin were reviewed. Data of the second treated eye of each patient and of re-DMEKs during the follow-up were excluded. The 12- and 36-month incidence of IOP elevation and post-DMEK glaucoma was analyzed using the Kaplan-Meier survival analysis. IOP elevation was defined as IOP ≥22 mm Hg or ≥10 mm Hg from preoperative baseline. COX regression analysis was used to evaluate the risk factors for IOP elevation and the development of a post-DMEK glaucoma. RESULTS The 12-month incidence of IOP elevation was 15.9% [95 confidence interval (CI), 12.0%-19.8%] and that of post-DMEK glaucoma was 3.9% (95 CI, 1.7%-6.1%); the 36-month incidence was 18.8% (95 CI, 14.5%-23.1%) and that of post-DMEK glaucoma was 6.6% (95 CI, 3.7%-9.5%). The most frequent cause was steroid-induced IOP elevation with a 12-month incidence of 11.7% (95 CI, 8.2%-15.2%) and a 36-month incidence of 12.9% (95 CI, 9.2%-16.6%), respectively. The incidence of postoperative pupillary block IOP elevation was 7.5% (95 CI, 4.8%-10.2%). A preexisting glaucoma increased the risk of IOP elevation [P < 0.001, hazard ratio (HR) 3.331; 95% CI, 1.919-5.782] and the development of a post-DMEK glaucoma (P < 0.001, HR 6.633; 95% CI, 2.556-17.215). The preoperative diagnosis also influenced the risk of IOP elevation [Fuchs corneal dystrophy (FED) vs. bullous keratopathy; P = 0.012, HR 2.354; 95% CI, 1.203-4.608] and post-DMEK glaucoma (FED vs. graft failure; P = 0.01, HR 4.412; 95% CI, 1.419-13.723, FED vs. bullous keratopathy; P = 0.09, HR 2.679; 95% CI, 0.858-8.358). CONCLUSIONS Incidence of IOP elevation is high in the first 12 months after DMEK, especially steroid-induced IOP elevation. Steroid-induced IOP elevation could be treated effectively by tapering down the steroid medication or changing the steroid drug. A preexisting glaucoma and the preoperative diagnosis increase the risk for an IOP elevation and a post-DMEK glaucoma.

ZusammenfassungBei vaskulären Erkrankungen der Netzhautperipherie ist eine Bildgebung, die einen umfassenden Einblick in die periphere Netzhaut ermöglicht und diese reproduzierbar abbilden kann, von hoher klinischer Relevanz. Die Weitwinkelangiografie ist in der Differenzialdiagnose peripherer vaskulärer Netzhauterkrankungen insbesondere zur Therapieentscheidung und Verlaufsbeurteilung wertvoll. Da Erkrankungen wie Morbus Coats und die familiär exsudative Vitreoretinopathie meist innerhalb der 1. oder Anfang der 2. Lebensdekade diagnostiziert werden, ist bei den meisten Patienten mit unzureichender Compliance bei Fundusaufnahmen zu rechnen. Gerade hier ermöglicht die Ultraweitwinkelbildgebung eine deutlich kürzere Aufnahmezeit im Vergleich zu Kompositaufnahmen. Dennoch können auch die aktuellsten Systeme nicht die gesamte Netzhaut vollständig abbilden. Der Bildausschnitt muss daher gezielt durch Blickrichtungen zum betroffenen Areal gelenkt werden und dem Fotografen die klinische Fragestellung stets zu Beginn der Aufnahmen bewusst sein. Daher steht nach wie vor eine gründliche Funduskopie in Mydriasis durch den erfahrenen Augenarzt an erster Stelle.

ZusammenfassungDie PEHCR (peripher exsudative hämorrhagische Chorioretinopathie) ist eine Erkrankung, die sich klinisch besonders durch subretinale Blutungen, retinale Exsudate, retinale Pigmentepithelabhebungen (RPE-Abhebungen), exsudative Ablatio und Sub-RPE-Blutungen manifestiert. Die PEHCR-Läsion ist oft durch ihr polypoidales Muster charakterisiert, welches den Polypen der PCV (polypoidale Choriovaskulopathie) sehr ähnelt. Die Diagnosestellung erfolgt am besten mit einer Weitwinkel-ICGA (ICGA: Indocyaningrünangiografie). Bei ca. der Hälfte der Patienten werden am betroffenen Auge oder am Partnerauge makuläre Veränderungen in Form von Drusen bis hin zur exsudativen AMD (altersabhängigen Makuladegeneration) detektiert. Da es direkt zur PEHCR nur sehr wenig Literatur gibt, werden in dieser Arbeit zudem die im Rahmen der AMD beschriebenen peripheren Veränderungen, die mit Weitwinkelbildgebung untersucht wurden, diskutiert.

To assess the long-term outcome of Trabectome surgery in the treatment of primary open angle glaucoma (POAG) and pseudoexfoliative glaucoma (PEX): 3-year results. Trabectome surgery (NeoMedix, Tustin, CA, USA) was performed in 268 POAG patients (women 57.46%, men 42.54%, age 72.35 ± 9.63 years) and 98 PEX glaucoma patients (women 58.16%, men 41.84%, age 73.42 ± 8.54 years), and uncontrolled intraocular pressure (IOP). Parameters were examined preoperatively, 1 day, 6 weeks, 3, 6, 12, 24 and 36 months post surgery. Kaplan-Meier analysis was performed using Criteria A (IOP ≤ 21 mmHg or ≥20% reduction from preoperative IOP), Criteria B (IOP ≤ 18 mmHg or ≥20% IOP reduction), Criteria C (IOP ≤ 21 mmHg, with or without medication) and D (IOP ≤ 18 mmHg, with or without medication). Complete success was defined as IOP ≤ 21 mmHg (Criteria E) and IOP ≤ 18 mmHg without medication (Criteria F). IOP was reduced from 19.10 ± 4.11 mmHg to 14.27 ± 2.93 mmHg (p

The white dot syndromes include a group of diseases which are characterized by multiple yellowish-white foci in the outer retina, retinal pigment epithelium, and choroid. For clinicians and researchers alike they present significant diagnostic and therapeutic challenges. White dot syndromes include primary inflammatory choriocapillaropathies, such as acute posterior multifocal placoid pigment epitheliopathy (APMPPE)/acute multifocal ischemic choriocapillaropathy (AMIC), multiple evanescent white dot syndrome (MEWDS)/acute idiopathic blind spot enlargement (AIBSE), multifocal choroiditis (MFC), punctate inner choroidopathy (PIC), serpiginous choroiditis (SC), acute zonal occult outer retinopathy (AZOOR), and acute macular neuroretinopathy (AMN). Among the primary stromal choroiditis is birdshot retinochoroidopathy (BSRC); however, the pathogenesis of these disorders is largely unknown. Immunological reactions to previous viral infections with a genetic disposition seem to be a common denominator.

Die okulare Hypotonie ist eine Folge einer fehlenden Produktion oder eines Verlusts von Kammerwasser. Intraokulare Entzundungen, Medikamente oder eine proliferative Vitreoretinopathie (PVR) mit Uberwachsung des Ziliarkorpers konnen zu einer Sekretionsminderung des Kammerwassers fuhren. Bei der Hypotonie durch Kammerwasserverlust unterscheidet man externe Grunde – wie Z. n. (fistulierender) Chirurgie oder Traumata – von internen Ursachen, wie Zyklodialysespalten oder Netzhautablosungen. In diesem Ubersichtsartikel wird die kausale Therapie der okularen Hypotonie aus der Sicht der Netzhaut- und Glaskorperchirurgie diskutiert: Ziliarkorperrefixation, Entfernung von Ziliarkorpermembranen, PVR-Chirurgie, die Wahl der Tamponade sowie Moglichkeiten und Grenzen des Irisdiaphragmas im Vergleich zu pharmakologischen Ansatzen.

Investigation of vascular diseases of the peripheral retina requires imaging procedures that allow a comprehensive view of the periphery, as well as reproducible pictures. In particular, ultra-wide field fluorescence angiography facilitates diagnosis, therapeutic decisions and follow-up examinations. While vasculopathies such as Coats disease and familiar exudative vitreoretinopathy are diagnosed within the first and second decade of life, patients' compliance during fundus imaging is typically reduced within this age range. Compared to the repeated imaging procedures for composite formation, ultra-wide field imaging has significantly reduced recording time. Nevertheless, current imaging systems are not able to map the entire retina in scaled proportions. Therefore, the imaging frame must be guided by patients' gaze onto the affected retinal area. Moreover, the medical photographer must be aware of the clinical setting and the region of interest. Hence, previous detailed funduscopy by trained ophthalmologists will remain indispensable.Bei vaskulären Erkrankungen der Netzhautperipherie ist eine Bildgebung, die einen umfassenden Einblick in die periphere Netzhaut ermöglicht und diese reproduzierbar abbilden kann, von hoher klinischer Relevanz. Die Weitwinkelangiografie ist in der Differenzialdiagnose peripherer vaskulärer Netzhauterkrankungen insbesondere zur Therapieentscheidung und Verlaufsbeurteilung wertvoll. Da Erkrankungen wie Morbus Coats und die familiär exsudative Vitreoretinopathie meist innerhalb der 1. oder Anfang der 2. Lebensdekade diagnostiziert werden, ist bei den meisten Patienten mit unzureichender Compliance bei Fundusaufnahmen zu rechnen. Gerade hier ermöglicht die Ultraweitwinkelbildgebung eine deutlich kürzere Aufnahmezeit im Vergleich zu Kompositaufnahmen. Dennoch können auch die aktuellsten Systeme nicht die gesamte Netzhaut vollständig abbilden. Der Bildausschnitt muss daher gezielt durch Blickrichtungen zum betroffenen Areal gelenkt werden und dem Fotografen die klinische Fragestellung stets zu Beginn der Aufnahmen bewusst sein. Daher steht nach wie vor eine gründliche Funduskopie in Mydriasis durch den erfahrenen Augenarzt an erster Stelle.

PEHCR (peripheral exudative haemorrhagic chorioretinopathy) is a disease manifested clinically, particularly by subretinal bleeding, retinal exudates, retinal pigment epithelium detachments (RPE detachments), exudative retinal detachment and sub-RPE bleeding. The PEHCR lesion is often characterized by its polypoidal pattern, which is very similar to PCV (polypoidal choroidal vasculopathy) polyps. Diagnosis is best made with a wide-field ICGA (indocyanine green angiography). In approximately half of patients, macular changes in the form of drusen, up to exudative AMD (age-related macular degeneration), are detected in the affected eye or partner eye. Since there is very little literature directly available on PEHCR, this work also discusses the peripheral changes described in the context of AMD that were investigated with wide-field imaging.Die PEHCR (peripher exsudative hämorrhagische Chorioretinopathie) ist eine Erkrankung, die sich klinisch besonders durch subretinale Blutungen, retinale Exsudate, retinale Pigmentepithelabhebungen (RPE-Abhebungen), exsudative Ablatio und Sub-RPE-Blutungen manifestiert. Die PEHCR-Läsion ist oft durch ihr polypoidales Muster charakterisiert, welches den Polypen der PCV (polypoidale Choriovaskulopathie) sehr ähnelt. Die Diagnosestellung erfolgt am besten mit einer Weitwinkel-ICGA (ICGA: Indocyaningrünangiografie). Bei ca. der Hälfte der Patienten werden am betroffenen Auge oder am Partnerauge makuläre Veränderungen in Form von Drusen bis hin zur exsudativen AMD (altersabhängigen Makuladegeneration) detektiert. Da es direkt zur PEHCR nur sehr wenig Literatur gibt, werden in dieser Arbeit zudem die im Rahmen der AMD beschriebenen peripheren Veränderungen, die mit Weitwinkelbildgebung untersucht wurden, diskutiert.

PurposeTo report the effectiveness of treatment with antivascular endothelial growth factor (VEGF)-inhibitor and/or immunosuppressants in punctate inner choroidopathy (PIC) using standard imaging modalities and optical coherence tomography angiography (OCTA) over a time period of 16 months.MethodsIn this prospective, unmasked, single-centre study, 23 individuals with PIC underwent imaging with spectral domain OCT, fluorescein angiography, indocyanine green angiography and OCTA. Two groups were formed based on systemic treatment. In case of choroidal neovascularisation (CNV) activity, intravitreal anti-VEGF injections were carried out in both groups.ResultsGroup I included 12 patients (24 eyes) with 18 affected eyes (75%) who did not receive any systemic therapy at baseline. Group II contained 11 patients (22 eyes) who started systemic immunosuppressive therapy on average 2 years before baseline. All eyes with recurrence of CNV or residual fluid (group I: seven eyes; group II: six eyes) received anti-VEGF agents. Group I showed a significant reduction of CNV size (p=0.0078), as well as a decrease of fluid retention (p=0.0078) on OCTA after anti-VEGF injection. Group II did not demonstrate any significant reduction of CNV size, vessel shape or fluid retention post injection. But overall, fluid accumulation was significantly lower in group II (median=0.03 mm2) than in group I (median=0.32 mm2) (p=0.0028).ConclusionImmunosuppressants in addition to anti-VEGF agents showed a significant reduction of fluid accumulation, that is, reduced disease activity. We conclude that there is a benefit and effectiveness of immunosuppressants to control inflammatory secondary CNV in PIC.

Purpose: To analyze the preferred practice respective diagnosis, treatment, and complications in patients with acute retinal necrosis in Germany.Methods: The uveitis-section of the German Ophthalmologic Society developed a questionnaire with 12 questions concerning patients with acute retinal necrosis seen in the 5 years up to August 2009.Results: In total, 35 eye hospitals answered the questionnaire and reported 213 patients with acute retinal necrosis. Diagnosis was made clinically in 86%. Anterior chamber tap, vitreous biopsy, diagnostic vitrectomy, and serology were performed for confirmation. Therapy was started with acyclovir in all institutions, and continued with ganciclovir, foscarnet and brivudine in some cases. Intravitreal injections were performed in 46%. Additional oral steroids were given in 80%. A following oral antiviral treatment was performed in 94%.Conclusions: Relevant variations were seen in diagnosis and treatment practices. The survey outlines the need for a unique diagnost...

Hintergrund Beim primaren vitreoretinalen Lymphom (PVRL), einem seltenen hamatopoetischen okularen Tumor, handelt es sich meist um ein groszelliges diffuses B-Zell-Lymphom. Das PVRL, das fruher auch als primares intraokulares Lymphom (PIOL) bezeichnet wurde, ist eine Unterart des primaren ZNS-Lymphoms (PZNSL).

To evaluate changes in macular sensitivity as measured via microperimetry (MP) in patients with macular edema (ME) secondary to retinal vein occlusion (RVO) after dexamethasone implantation (DEX implantation, Ozurdex), in comparison to distance visual acuity, reading ability, and spectral domain optical coherence tomography (SD-OCT).Twenty-three patients with ME secondary to RVO were treated with a DEX implantation in this prospective, observational case study. Patients were controlled at baseline and then monthly with microperimetry and assessment of distance visual acuity, reading ability, and SD- OCT. Side effects of the DEX implant were monitored by measuring the intraocular pressure (IOP) and lens grading with Lens Opacities Classification System (LOCS) charts.The 23 patients with RVO included 16 patients with branch retinal vein occlusion (BRVO) and 7 patients with central retinal vein occlusion (CRVO). Improvement in distance visual acuity, reading ability, and in central 8-points microperimetry showed statistical significance from month 1 to 3, whereas the 40-points microperimetry improved with statistical significance from month 1 to 2. The reduction of central retinal thickness measured with SD-OCT remained statistically significant until month 4. Subgroup analysis of patients with BRVO and CRVO confirmed the best test results observed at month two after DEX implantation.The highest macular sensitivity was measured two months after DEX implantation. This corresponds to the best test results for distance visual acuity, reading ability, and SD-OCT observed two months after treatment.

Background To evaluate the efficacy and safety of dexamethasone (DEX) implants in paediatric patients with noninfectious intermediate or posterior uveitis. Methods Prospective single center exploratory case series. Children and adolescents, 6 to 17 years old, with a vitreous haze score of ≥1.5+ or cystoid macular edema (CME) of >300 μm were enrolled. Vitreous haze score at month 2 was chosen as primary endpoint. Best corrected visual acuity (BCVA), central retinal thickness (CRT) and concomitant medication at month 6 were defined as secondary endpoints. Intraocular pressure (IOP) and cataract formation were determined as safety endpoints. Results Three out of 6 eligible patients participated in the case series. At month 2, vitreous haze was reduced from a score of 1.5+ to 0.5+ and 0 and BCVA improved by ≥3 lines, ≥4 lines and ≥2 lines of Early Treatment of Diabetic Retinopathy (ETDRS)-letters, respectively. Visual acuity gain was accompanied by a CRT reduction of −186 μm and −83 μm in the first and third patient, in whom CME was the indication for DEX implantation. A reduction of concomitant medication was achieved in 1 patient. IOP increase was seen in all 3 patients, but could be treated sufficiently with primarily IOP lowering medications and without need for glaucoma surgery. Cataract progression did not occur. Conclusions DEX implants led to an improvement in all endpoints, especially BCVA. This study confirms that IOP rises may also occur in the paediatric population and should be monitored and treated appropriately. Trial registration European Union Drug Regulating Authorities Clinical Trials (EudraCT)- nr: 2013–000541-39.

Purpose: To assess the correlation between the disc damage likelihood scale (DDLS) objectively measured by a nonmydriatic fundus camera, confocal laser scanning ophthalmoscopy (HRT3), and spectral-domain optical coherence tomography (SD-OCT) in uveitic glaucoma. Methods: A total of 59 patients with uveitic glaucoma (21 female, 38 male; mean age 56.8 ± 18.7 years) were included in this prospective cross-sectional study. All patients were measured by the Kowa Nonmyd WX 3D camera (2D/3D nonmydriatic retinal camera, Kowa Company), the HRT3 (Heidelberg Engineering), and SD-OCT (Carl Zeiss Meditec) by one examiner on the same day. All 3 devices graded the optic disc topography. Statistical data were calculated using SPSS (v 20.0, SPSS). Results: In patients showing borderline results in one of the modalities (n = 45), the DDLS showed a significant correlation with the retinal nerve fiber layer (p = 0.016), while Moorfields regression analysis (p = 0.550) and glaucoma probability score (p = 0.629) did not correlate significantly. The highest predictive power was demonstrated by the objectively measured DDLS (area under the receiver operating characteristic curve 0.445-0.588), compared to R. Burk (0.149-0.375) and F.S. Mikelberg (0.033-0.450) coefficients considering HRT and optical coherence tomography. Conclusions: In this study cohort, the objective DDLS showed the highest predictive power and thus is a reliable tool in diagnosing uveitic glaucoma. These 3 devices cannot be used interchangeably. As diagnosis and follow-ups are challenging in uveitis patients, the stereophotography is additionally a valuable tool.

To evaluate the therapeutic outcome for dexamethasone implant (DEX) or intravitreal ranibizumab (IVR) injections over 6 months in patients with macular edema due to branch or central retinal vein occlusion (BRVO, CRVO), in a real-life setting. A total of 107 patients with BRVO or CRVO were included into this retrospective single-center observational study. Patients were treated with monotherapy consisting of DEX or three monthly IVR injections following a pro re nata regimen (PRN). Best-corrected visual acuity (BCVA), central retinal thickness (CRT) and intraocular pressure (IOP) were compared between the two therapy groups after 1, 3 and 6 months. BRVO patients treated with DEX achieved a statistically significant gain in BCVA measured in logMAR after 1 month (mean gain, 95% CI: 0.21, 0.08–0.34, p = 0.001), 3 months (0.16, 0.03–0.28, p = 0.012) and 6 months (0.19, 0.07–0.32, p = 0.002), whereas patients treated with IVR showed a statistically significant BCVA gain in month 3 (mean improvement, 95% CI: 0.13, 0.01–0.26, p = 0.039) and month 6 (0.16, 0.03–0.29, p = 0.018). BCVA in CRVO patients with DEX worsened slightly at month 6 (mean worsening, 95% CI: −0.08, −0.24 to 0.08, p = 0.305), while IVR treated-patients achieved a statistically significant BCVA gain at 3 months (mean improvement, 95% CI: 0.14, 0.02–0.25, p = 0.021). Both therapies were accompanied by statistically significant CRT reductions of 150 to 200 μm (median). Adverse events reported were predictable and limited. In a clinical setting, comparable improvement in BCVA and CRT were observed after DEX and IVR injections for treatment of BRVO. CRVO patients showed greater benefit with IVR.

To report the effectiveness of repeated intravitreal dexamethasone (DEX) inserts in noninfectious uveitis patients.Prospective, single-center, interventional clinical trial between February 2010 and March 2015.Patients with noninfectious uveitis with cystoid macular edema and/or vitreitis.Patients were treated with a 700-μg intravitreal DEX insert (Ozurdex; Allergan, Inc., Irvine, CA). Follow-up visits were scheduled 1, 3, and 6 months after injection. Best-corrected visual acuity (BCVA), central retinal thickness (CRT), vitreous haze (VH) score, intraocular pressure (IOP), and adverse events were recorded.Primary outcome was the reduction of CRT. Secondary outcome was the improvement in BCVA and reduction of VH.In total, 109 eyes of 76 patients received 298 DEX inserts. Fifty-two patients were women (68%). The mean age of all participants was 57 years (range, 24-88 years). More than 3 DEX inserts were injected into 44% of eyes. Mean number of injections were 1.54±0.5 (standard deviation [SD]), 1.98±0.84, and 2.46±1.1 over 12, 18, and 24 months, respectively. Central retinal thickness decreased significantly (P0.001) from 465 μm at baseline to 318, 342, and 388 μm after 1, 3, and 6 months, respectively. Similar trends were seen in eyes receiving a second, third, and fourth DEX insert. Patients with idiopathic uveitis and sarcoidosis benefited well from DEX inserts. The greatest overall benefit was achieved in patients with no systemic treatment and patients receiving antimetabolites and cyclosporin A. A significant VH score reduction was documented in 44% of eyes after 1 month. A gain of more than 3 lines in BCVA was recorded in 31% to 37%, 26% to 39%, and 8% to 32% of eyes after 1, 3, and 6 months, respectively. A transient rise in mean IOP after 1 month (P0.001) and after 3 months (P = 0.001) was seen.The repeated longer-term administration of DEX inserts in noninfectious uveitis patients, either alone or in combination with other therapies, led to improved CRT, BCVA, and VH. Underlying diseases and concomitant systemic therapy seem to have an impact on overall treatment benefit. Ocular complications were reversible and were managed by local treatment, with exception of cataract formation.

To evaluate the efficacy of mineralocorticoid-receptor antagonists in comparison to acetazolamide and observation in the treatment of central serous chorioretinopathy.Retrospective, interventional cohort study on 93 patients with acute or chronic central serous chorioretinopathy (37 patients: acetazolamide group, 20 patients: mineralocorticoid-receptor antagonist group, 8 patients: observation group, and 27 patients with a therapy switch between both medications). Main outcome measures were the change in best-corrected visual acuity, subretinal fluid (SRF) volume, central retinal prominence, and highest retinal prominence (HRP) at 12 weeks.HRP and SRF volume improved with statistical significance (P ≤ 0.05) after mineralocorticoid-receptor antagonist (P = 0.0000003 for the prominence, P = 0.008 for the volume) and acetazolamide (P 0.0000001 for the prominence, P = 0.0000007 for the volume) treatment. HRP and SRF volume also improved after observation, but without statistical significance (P = 0.08 for the prominence, P = 0.72 for the volume). Corresponding visual acuity improved significantly in acetazolamide (P = 0.002) and mineralocorticoid-receptor antagonist (P = 0.03) treated patients. Interestingly, HRP and SRF volume in acetazolamide nonresponsive patients improved after switch to mineralocorticoid-receptor antagonists, whereas no benefit was seen in patients switching vice versa.Both medical treatments are effective first-line treatment options for central serous chorioretinopathy. In patients who are nonresponsive to acetazolamide, therapy switch to mineralocorticoid-receptor antagonists could be beneficial.

Unter einer posterioren Uveitis werden fokale, multifokale und diffuse Lasionen einer Choroiditis, Chorioretinitis, Retinochoroiditis, Retinitis und Neuroretinitis subsumiert. Posteriore Uveitiden umfassen ein breites Spektrum von infektiosen und nichtinfektiosen autoimmunologischen Entzundungen. Die posteriore Uveitis manifestiert sich am haufigsten jedoch endogen. Im 1. Teil dieser Publikation wurden Epidemiologie, Atiologie, Symptomatik, diagnostisches Vorgehen und Grundkonzepte der Therapie dargestellt ( Heiligenhaus et al. 2014 ). In diesem 2. Teil werden die in unserer Bevolkerung typischen infektiosen und nichtinfektiosen Formen der posterioren Uveitis beschrieben.

Purpose: To investigate the efficacy and safety of a single dexamethasone intravitreal implant (Ozurdex®, 700 µg). Methods: In this prospective noncomparative case series, 84 patients (54 females) received a dexamethasone intravitreal implant. At weeks 4, 12 and 24 after the injection, vitreous haze, macular thickness and best corrected visual acuity (BCVA) were assessed and adverse events reported. Results: Clearance of vitreous haze could be achieved after 4 weeks in 61% of all eyes (p < 0.001) and remained significant until week 24 (p < 0.001). This was paralleled by a reduction of central retinal thickness after 4 (p < 0.001), 12 (p < 0.001) and 24 weeks (p < 0.006). Significant and fast improvement of BCVA was already achieved after 4 weeks (p < 0.001) but vanished by week 24. Intraocular pressure reached ≥35 mm Hg in 3 eyes and was significantly more frequent in intermediate uveitis compared to posterior uveitis (p < 0.016). Conclusions: The dexamethasone implant is effective in controlling intraocular posterior segment inflammation and reduces central retinal thickness fast and effectively.

Das Behcet-Syndrom (auch Morbus Behcet oder Admantiades-Behcet-Erkrankung) ist eine chronische Vaskulitis. Die Erkrankung ist durch Exazerbation und Remission einer Vielzahl von Symptomen und Organmanifestationen charakterisiert. Die Befunde variieren von milden mukokutanen Lasionen bis zu schweren, visusbedrohenden intraokularen Entzundungen. Es kann daruber hinaus Gelenke, alle Gefasbahnen und das zentrale Nervensystem betreffen. Aufgrund hoher Morbiditat und erheblicher Mortalitat ist eine adaquate Therapie von groser Bedeutung. Cyclosporin A ist das bisher in Deutschland einzig zugelassene Therapeutikum zur Behandlung der Augenbeteiligung. Neuro- und Nephrotoxizitat schranken den Einsatz allerdings ein. Bei Patienten mit schweren Uveitisverlaufen haben sich „Biologika“ als therapeutischer „Durchbruch“ erwiesen. Interferon-α hat sich als sehr wirksam bei intraokularer Entzundung gezeigt. Monoklonale Antikorper gegen TNF-α und Interleukin-1 sind ebenfalls in klinischen Studien effektiv gewesen und sind in einigen Landern fur diese Indikation bereits zugelassen. Dieser Beitrag fasst den aktuellen Wissenstand zusammen und unterstreicht die bedeutende Rolle des Augenarztes in der Therapie des Behcet-Syndroms.

Title: Morphological diagnostics in paediatric glaucoma Background: Morphological parameters vary in paediatric glaucoma with a minor knowledge on standard values available compared to an adult cohort. Accordingly, we designed a study to assess the objectively measured disc damage likelihood scale (DDLS) by KOWA stereophotography as a novel parameter in comparison to standard glaucoma diagnostics, HRT 3 and OCT. Methods and Findings: Prospective study of 47 eyes (n=47) categorized into three groups; 14 normal optic discs (11 female f, 3 male m, 12.7 ± 6.1years), 15 glaucoma suspects (3f, 12m, 11.7 ± 3.1years) and 18 paediatric glaucoma (10f, 8m, 11.2 ± 2.5years). Stereometric measurements of the optic disc by KOWA camera, Moorfields regression analysis (MRA) and glaucoma probability score (GPS) of the HRT3 and retinal nerve fibre layer (RNFL) of the OCT were analysed. Results were split in 2 groups: Diagnosis 1 was defined as “outside normal limits”, Diagnosis 2 as “borderline or outside normal limits”. Referring to Diagnosis 1 and 2, DDLS did not show a significant correlation with MRA, GPS and RNFL in glaucoma suspects, healthy children and paediatric glaucoma. In paediatric glaucoma the correlation of the GPS compared to KOWA rim/disc ratio was significant in all six quadrants; supernasal (r=-0.794 p=0.006), nasal (r=-0.845 p=0.002), inferonasal (r=-0.659 p=0.038), infertemporal (r=-0.636 p=0.048), temporal (r=-0.656 p=0.039) and superotemporal segment (r=-0.755 p=0.012). Regarding the area under the curve (Receiving operating characteristics ROC) the highest predictive power was demonstrated by DDLS (0.669-0.833) compared to R.Burk (0.142-0.595) and F.S.Mikelberg (0.050-0.619) coefficients considering all devices. Conclusions: The DDLS showed the highest predictive power for glaucomatous damage compared to glaucoma coefficients of the established devices. Additionally, the gold standard of optic nerve head assessment still includes the examination of the fundus photography. As stereo photography is an easy and timesaving instrument, it might be an additive tool in paediatric glaucoma.

Purpose: To describe retinal vascular changes in Birdshot Retinochoroiditis (BSRC) with multimodal imaging techniques and functional values. Methods: In this single-center study, 64 eyes of 32 subjects with BSRC were classified according to disease activity and duration and underwent imaging with spectral domain optical coherence tomography, fluorescein angiography, indocyanine green angiography, fundus autofluorescence, and optical coherence tomography angiography (OCTA). Results: Mean age of the patients was 60 years (range, 38–74). OCTA revealed capillary loops (58%), telangiectatic vessels (44%), increased intercapillary spaces (52%), altered vascular architecture (53%), and rarefication of C-scans (63%) in retinal layers. Increased rarefications of C-scans (p = 0.0056; p = 0.0046) and altered vascular architecture (p = 0.0120; p = 0.0243) in superficial and deep capillary layers were significantly correlated with disease activity. Conclusion: OCTA adds new insights in a multimodal imaging approach of retinal vascular layer visualization in BSRC and may contribute to existing methods for diagnosing severity and potentially progression of the disease.

Purpose: To investigate whether vascular differentiation marker von Willebrand factor (vWf) and proliferation marker KI67 expression correlate with MUC4 localization around stromal tumor vascularization in human conjunctival malignant melanoma (CMM). Materials and methods: For the purposes of this study, we analyzed samples from human CMMs (n = 4), conjunctival compound nevi (n = 7), and samples from healthy conjunctiva (n = 7) for MUC1, 4, and 16 by immunohistochemistry. To test CMM vessel association of MUC4, we investigated the co-localization of MUC4 with vWf or KI67 in human CMM specimens (n = 10) by immunohistochemistry. Also, we investigated the MUC4 localization around vessels of healthy conjunctiva (n = 10). Results: The immunohistochemical analysis demonstrated membrane-associated mucin expression in epithelia of CMM, nevi and healthy conjunctiva, whereas only MUC4 was localized perivascular in CMM tissue in this preliminary analysis. Co-staining analysis with vWf and KI67 demonstrated MUC4 localization around stromal vessels in human CMM specimens. In contrast, no MUC4 localization has been seen around healthy conjunctiva stroma vessels. Conclusions: MUC4 was detected around vWf/KI67-positive CMM stromal vascular tissue, but not around healthy conjunctival stroma vessels. Therefore, we assume that MUC4 might play a role in tumor cell migration toward vessels inducing metastasis.

Background Patients with initially low visual acuity were excluded from the therapy approval studies for retinal vein occlusion. But up to 28 % of patients presenting with central retinal vein occlusion have a baseline BCVA of less than 34 ETDRS letters (0.1). The purpose of our study was to assess visual acuity and central retinal thickness in patients suffering from central retinal vein occlusion and low visual acuity (

Purpose: To assess the efficacy and tolerability of intravitreal dexamethasone 0.7 mg sustained-release insert (Ozurdex®) in patients with sclerouveitis and recurrent cystoid macula edema (CMO) refractory to treatment.Methods: Interventional retrospective case series of five patients receiving 13 intravitreal dexamethasone inserts.Results: Three of five patients presented with an associated systemic disorder, whereas two patients had idiopathic sclerouveitis. All patients received immunosuppressive therapy. The CRT mean (SD) decreased in all eyes from 428 μm (137) (baseline) to 327 μm (149) (1 month), 342 μm (155) (3 months), 297 μm (99) (6 months) and reduced scleral inflammation. No morphologic adverse changes were noted, in particular, no scleral melting or necrosis occurred.Conclusions: Intravitreal dexamethasone may be an effective and safe therapeutic option in sclerouveitis with otherwise treatment-resistant CMO. It resolves not only CMO, but also provides a reduction of scleral inflammatio...

Hintergrund: Bei der Behandlung der Chorioretinopathia centralis serosa (CRCS) gibt es derzeit verschiedene Therapiestrategien. Die Laserkoagulation von, in der Fluoreszenzangiographie sichtbaren Quellpunkten ist eine Möglichkeit der Behandlung, in der Regel bei Patienten, bei denen sich weder [zum vollständigen Text gelangen Sie über die oben angegebene URL], 29. Jahrestagung der Retinologischen Gesellschaft