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Purpose To evaluate the retinal microvascular density using optical coherence tomography angiography (OCTA) in patients with systemic lupus erythematosus (SLE) treated with hydroxychloroquine (HCQ). Methods Nineteen eyes of 19 patients with SLE (study group) without HCQ retinopathy and 19 eyes of 19 healthy subjects (control group) were included in this study. The study group was divided into patients using HCQ for > 5 years (high-risk group) and < 5 years (low-risk group). The VD data of the 3 × 3 mm OCT angiogram of the superficial capillary plexus (SCP) and the choriocapillaris (VD-CC), the foveal avascular zone (FAZ) area and the central retinal thickness (CRT) were extracted and analyzed. Results VD in the en face SCP was significantly reduced in the high-risk group and the low-risk group compared with that in the control group (p < 0.001, p = 0.001) and in the high-risk group compared with the low-risk group (p = 0.007). Correlation analysis between the cumulative dose of HCQ and the VD of the study group revealed a negative correlation, but no statistical significance (p = 0.074). However, a significant positive correlation was observed for the low-risk group (p = 0.035). In patients with SLE, VD-CC was lower (p = 0.042) and the FAZ area larger (p = 0.019). CRT showed no difference between the groups (p = 0.183). Conclusion In this study, SLE patients showed a reduced VD in both groups. In patients treated with HCQ < 5 years, HCQ might have a protective effect on retinal microvasculature. Analysis of retinal microvascular density using OCTA could be useful in the diagnosis and monitoring of vascular alteration in patients with SLE.

Background/AimsTo analyse the distribution of macular ganglion cell layer thickness (GCLT) in patients with foveal hypoplasia (FH) with or without albinism to obtain new insights into visual pathway anomalies in albinos.MethodsPatients with FH who presented at our institution between 2013 and 2018 were retrospectively drawn for analysis. Mean GCLT was calculated after automated segmentation of spectral domain-optical coherence tomography (SD-OCT) scans. Patients with FH due to albinism (n = 13, termed ‘albinism FH’) or other kinds (n = 10, termed ‘non-albinism FH’) were compared with control subjects (n = 15). The areas: fovea (central), parafovea (nasal I, temporal I) and perifovea (nasal II, temporal II) along the horizontal meridian were of particular interest. Primary endpoints of this study were the ratios (GCLT-I- and GCLT-II-Quotient) between the GCLT measured in the temporal I or II and nasal I or II areas.ResultsThere was a significant difference between the GCLT-I-Quotient of healthy controls and albinism FH (p<0.001), as well as between non-albinism FH and albinism FH (p = 0.004). GCLT-II-Quotient showed significant differences between healthy controls and albinism FH (p<0.001) and between non-albinism FH and albinism FH (p = 0.006). The best measure for distinguishing between non-albinism FH and albinism FH was the calculation of GCLT-II-Quotient (area temporal II divided by area nasal II), indicating albinism at a cut-off of <0.7169. The estimated specificity and sensitivity for this cut-off were 84.6% and 100.0%, respectively. The estimated area under the curve (AUC) was 0.892 [95%CI: 0.743–1.000, p = 0.002].ConclusionMacular GCLT-distribution showed a characteristic temporal to central shift in patients with FH due to albinism. Calculation of the GCLT-II-Quotient at a cut-off of <0.7169 presents a new diagnostic criterion for identification of ocular albinism.

PurposeTo evaluate the effect of topical mydriatic eye drops on optical coherence tomography angiography (OCTA) parameters in patients with age-related macular degeneration (AMD).Methods27 eyes of 27 patients suffering from AMD were included in this cross-sectional study. Patients with ≥-4.5 diopters spherical equivalent, corneal opacities or dense cataract preventing high-quality imaging were excluded. Whole-en-face scans of the superficial capillary plexus (SCP) and deep capillary plexus (DCP) in the central 3x3mm foveal region as well as whole-en-face and peripapillary scans of the radial peripapillary capillaries (RPC) were generated using OCTA (AngioVue®, Optovue). Imaging was first conducted with patients’ eyes in miosis, then in mydriasis after instillation of a dilating eye drop (0.5% tropicamide, 2.5% phenylephrine-HCl). Main outcome measures were flow density (FD), foveal avascular zone (FAZ), signal strength index (SSI) and motion artifact score (MAS).ResultsOur results reveal that in AMD patients there is no significant difference between FD measurements taken in miosis and those taken in mydriasis around the SCP (p = 0.198), DCP (p = 0.458), RPC whole-en-face (p = 0.275) and RPC peripapillary (p = 0.503). Measurements taken in these two states appear to be equivalent for assessment of FD (90%CI within ± 0.05). No significant difference was found either in the area of the FAZ (p = 0.338) or in the SSI (p = 0.371) before and after the instillation of tropicamide/phenylephrine. MAS was significantly lower after the application of mydriatic eye drops (p = 0.003).ConclusionsOur findings reveal that neither measurements of FD nor measurements of the FAZ area changed significantly in AMD patients after the application of tropicamide/phenylephrine. Since MAS improved significantly in dilation, mydriatic examination is recommended. Nevertheless, a comparison of OCTA metrics from images taken with different pupil states (miosis versus mydriasis) is valid for clinical trials.

The WW-and-C2-domain-containing (WWC) protein family is involved in the regulation of cell differentiation, cell proliferation, and organ growth control. As upstream components of the Hippo signaling pathway, WWC proteins activate the Large tumor suppressor (LATS) kinase that in turn phosphorylates Yes-associated protein (YAP) and its paralog Transcriptional coactivator-with-PDZ-binding motif (TAZ) preventing their nuclear import and transcriptional activity. Inhibition of WWC expression leads to downregulation of the Hippo pathway, increased expression of YAP/TAZ target genes and enhanced organ growth. In mice, a ubiquitous Wwc1 knockout (KO) induces a mild neurological phenotype with no impact on embryogenesis or organ growth. In contrast, we could show here that ubiquitous deletion of Wwc2 in mice leads to early embryonic lethality. Wwc2 KO embryos display growth retardation, a disturbed placenta development, impaired vascularization, and finally embryonic death. A whole-transcriptome analysis of embryos lacking Wwc2 revealed a massive deregulation of gene expression with impact on cell fate determination, cell metabolism, and angiogenesis. Consequently, a perinatal, endothelial-specific Wwc2 KO in mice led to disturbed vessel formation and vascular hypersprouting in the retina. In summary, our data elucidate a novel role for Wwc2 as a key regulator in early embryonic development and sprouting angiogenesis in mice.

Background/AimDacryocystorhinostomy (DCR) remains the gold standard therapy for nasolacrimal duct obstruction (NLDO), but is invasive and does not maintain the physiology of the lacrimal pathway. With transcanlicular microdrill dacryoplasty (MDP), there is a minimally invasive alternative surgical approach. This study aimed to present this modern lacrimal duct surgery technique and to evaluate its long-term success rate in a large study population.MethodsThe medical records of 1010 patients with acquired NLDO were retrospectively reviewed. Adult patients who had undergone transcanalicular MDP were included. The evaluation included the following parameters: age, gender, success rate, complication rate, obstruction grade and patient satisfaction. Long-term results regarding patient satisfaction and success rate were evaluated by a telephone survey. Only a complete resolution of symptoms was defined as success.Results793 eyes of 576 patients after transcanalicular MDP could be included in the study. The mean follow-up time was 8.7±0.9 years. Initial surgical success rate was 84.0%. At the time of the follow-up, 57.5% (n=229) still had full resolution of symptoms. The mean patient satisfaction with the procedure was 6.9±3.2 out of 10 points. Heavy bleeding occurred in two cases only (0.25%).ConclusionThis is the first study to show the success rate of microendoscopic lacrimal duct surgery after such a long follow-up period and in such a large study population. Transcanalicular MDP is a minimally invasive technique with a very low complication rate and can be used as an alternative procedure before performing more invasive lacrimal duct surgery such as DCR.

Zusammenfassung Hintergrund Ziel der Studie war die Analyse des Stellenwertes von „eLearning“ im Lern- und Fortbildungsverhalten von Augenärzten in Deutschland sowie die Bewertung der Akzeptanz einer neuen „eLearning“-Anwendersoftware (App). Material und Methoden Ophthalmologische Weiterbildungsassistenten und Fachärzte wurden im Rahmen von Fortbildungsveranstaltungen mittels eines Fragebogens nach ihren Fortbildungsaktivitäten befragt. Des Weiteren erfolgte nach Vorstellung und Anwendung einer „eLearning-App“ eine strukturierte Bewertung. Ergebnisse Es nahmen 149 Augenärzte an der Befragung teil. Während der überwiegende Teil der Kollegen (74,3 %) analoge Fachzeitschriften wöchentlich oder monatlich zur Weiterbildung nutzt, verwenden 45,9 % der Augenärzte digitale Printmedien (Bücher, Zeitschriften, Artikel) sowie 46,5 % Fachbücher in gedruckter Form. Lediglich 35 % der Befragten bilden sich über Online-Fortbildungsplattformen, z. B. digitale Kurse (CME-Kurse) oder Portale zum Abrufen aufgezeichneter Vorträge, fort. Die Nutzung der angebotenen „eLearning-App“ ging insgesamt mit einer positiven Akzeptanz einher; 91,7 % der befragten Kollegen würden diese Form der interaktiven Lernvermittlung weiterempfehlen. Diskussion Trotz fortschreitender Digitalisierung in allen Lebensbereichen nimmt „eLearning“ als Lernmedium in der ophthalmologischen Fort- und Weiterbildung weiterhin einen geringen Stellenwert ein. Interessanterweise ergab die Bewertung der App-Nutzer eine hohe Benutzerakzeptanz, unabhängig von Alter oder Arbeitsbereich.

The WWC protein family is an upstream regulator of the Hippo signalling pathway that is involved in many cellular processes. We examined the effect of an endothelium-specific WWC1 and/or WWC2 knock-out on ocular angiogenesis. Knock-outs were induced in C57BL/6 mice at the age of one day (P1) and evaluated at P6 (postnatal mice) or induced at the age of five weeks and evaluated at three months of age (adult mice). We analysed morphology of retinal vasculature in retinal flat mounts. In addition, in vivo imaging and functional testing by electroretinography were performed in adult mice. Adult WWC1/2 double knock-out mice differed neither functionally nor morphologically from the control group. In contrast, the retinas of the postnatal WWC knock-out mice showed a hyperproliferative phenotype with significantly enlarged areas of sprouting angiogenesis and a higher number of tip cells. The branching and end points in the peripheral plexus were significantly increased compared to the control group. The deletion of the WWC2 gene was decisive for these effects; while knocking out WWC1 showed no significant differences. The results hint strongly that WWC2 is an essential regulator of ocular angiogenesis in mice. As an activator of the Hippo signalling pathway, it prevents excessive proliferation during physiological angiogenesis. In adult animals, WWC proteins do not seem to be important for the maintenance of the mature vascular plexus.