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The swinging flashlight test is a standard diagnostic procedure to detect relative afferent pupillary defects. The advantages of the test lie within its objectivity and minimal effort. However, its value depends on its correct execution and interpretation. This questionnaire-based survey investigates whether this is given among German speaking ophthalmologists.A multiple-choice questionnaire with 14 questions on the use of the swinging flashlight test was designed. It was presented to German speaking ophthalmology specialists (primary data) and orthoptists (secondary data) on specialist conferences or by telephone interviews.249 ophthalmologists and 76 orthoptists participated in the survey. Only 2% of ophthalmologists answered all 14 questions correctly. On average 66% (range 29 - 100%) of the questions were answered correctly by the ophthalmologists. The question with the lowest result had a rate of 19%, the question with the highest result was correctly answered by 95%. The orthoptists achieved similar results.The rate of correct answers appears disturbingly low. The swinging flashlight test being a basic tool and an obligatory test in a number of guidelines should rather be performed nearly error-free. In light of the high error rates, misdiagnoses and treatment errors must be feared. Better training seems necessary.Der Swinging-Flashlight-Test ist eine ophthalmologische Standarduntersuchung zur Ermittlung eines relativen afferenten Pupillendefekts (RAPD). Die Vorzüge des Tests liegen in seinem minimalen technischen Aufwand und seiner Objektivität. Sein Nutzen hängt jedoch von der korrekten Durchführung und richtigen Interpretation ab. Inwieweit dies gegeben ist, untersuchte die vorliegende Befragungsstudie unter deutschsprachigen Ophthalmologen.Es wurde ein Multiple-Choice-Fragebogen mit 14 Fragen zur Anwendung des Swinging-Flashlight-Tests entwickelt. Dieser wurde deutschsprachigen Augenfachärzten (Hauptauswertung) und Orthoptistinnen (Nebenauswertung) auf Kongressen vorgelegt oder mittels Telefoninterview abgefragt.249 Augenfachärzte und 76 Orthoptistinnen nahmen an der Befragung teil. Nur 2% der Fachärzte beantworteten alle 14 Fragen richtig. Durchschnittlich wurden 66% (Spanne 29 – 100%) der Fragen richtig beantwortet. Die Frage mit dem schlechtesten Ergebnis wurde von 19%, die mit dem besten Ergebnis von 95% der Teilnehmer richtig beantwortet. Die Ergebnisse der Teilgruppe der Orthoptistinnen stimmten annähernd mit denen der Fachärzte überein.Die Rate an Richtigantworten erscheint beunruhigend niedrig. Für eine klinische Untersuchungsmethode, die als augenärztliches Basishandwerkszeug gilt und in ophthalmologischen Leitlinien als obligate Untersuchung geführt wird, wäre eine nahezu fehlerfreie Anwendung zu fordern. Angesichts der hohen Fehlerrate sind Fehldiagnosen und -behandlungen zu befürchten. Verbesserungen der Weiter- und Fortbildung sind hier unabdingbar.

Potential sources of error in dosage planning in strabismus surgery are (a) prismatic side-effects of spectacle lenses when measuring the preoperative angle with the alternating prism cover test and (b) a potential influence of eye ball axial length on dose response. As both errors take effect in opposite directions, many strabismus surgeons set aside their consideration. This study investigates whether considering both factors for dosage planning yields better operative results. In this prospective, randomised, double-blind, interventional pilot study, we included patients scheduled for purely horizontal strabismus surgery and determined each patient’s surgical dose (total amount of recession/plication) either with (study group) or without (control) consideration of the two factors. The deviation of the resulting angle from the target angle 3 months postoperatively was the primary endpoint. One hundred one patients were included, 51 of which in the intervention group and 50 in the control group. The primary endpoint showed a median deviation from the target of 3.0° in the intervention group and 4.8° in the control group. We observed a group difference of 1.8° in favour of the intervention group (p = 0.053). Subgroup analysis showed a difference between groups of 2.2° for esotropic patients and of 5.1° for patients with hyperopia > + 2 D. Taking prismatic side-effects of spectacle lenses and eye ball length into account when calculating strabismus surgery doses showed a trend towards more accurate results. Esotropic patients and patients with hyperopia > + 2 D seemed to benefit most. International Clinical Trials Registry Platform: DRKS00011121

The incidence of chronic keratoconjunctivitis, which potentially causes long-term loss of visual acuity due to corneal opacity, is considerably less common in children than in adults. It is therefore in danger of being overlooked. In children the appropriate treatment is therefore often introduced too late, or to an insufficient extent. In this article we would like to raise awareness about the diagnosis of chronic keratoconjunctivitis in children, and to present an effective treatment plan for severe stages of the disease. There are two forms of chronic keratoconjunctivitis that occur most frequently in children: hyperergic blepharokeratoconjunctivitis (hBKC) and vernal keratoconjunctivitis (VKC). With hBKC, the patient often has a history of recurring hordeolum and also presents with blepharitis; it is characterized by the marked presence of corneal neovascularization in the lower circumference of the cornea. VKC is typically characterized by changes under the upper eyelid, with marked changes to the superior limbus. If there is a risk of complications involving the cornea, or in the presence of such complications, a consistent long-term topical immunosuppressive and anti-inflammatory treatment is required. Both of these properties are combined in the active ingredient cyclosporine A. Other advantages of topical CSA treatment are its steroid-sparing effect and the long-term reduction of exacerbations. Parents need to be informed about the chronic nature of these two diseases and their tendency to recur; because of these characteristics, treatment, in most cases, should be envisaged for at least one year in order to effectively disrupt the complex immunologic processes. This safeguards the child's visual development and prevents amblyopia caused by scarring and astigmatism. We hope that the data presented will lower the barriers related to prescribing CSA for topical eye application in children.Bei Kindern wird das Auftreten einer chronischen Keratokonjunktivitis, mit dem Potenzial einer dauerhaften Sehminderung durch Hornhauttrübungen, deutlich seltener erwartet als bei Erwachsenen. Folglich wird die notwendige Behandlung oft zu spät oder nur unzureichend eingeleitet. Mit diesem Artikel möchten wir auf die Diagnose der chronischen Keratokonjunktivitis bei Kindern aufmerksam machen und ein wirksames Behandlungskonzept für die schweren Verlaufsformen vorstellen. Zwei Formen der chronischen Keratokonjunktivitis treten im Kindesalter am häufigsten auf: die hypererge Blepharokeratokonjunktivitis (hBKK) und die vernale Keratokonjunktivitis (VKK). Bei der hBKK finden sich rezidivierende Hordeola in der Vorgeschichte und eine Blepharitis; die Hornhautvaskularisationen sind betont in der unteren Hornhautzirkumferenz zu finden. Die VKK zeigt typische Veränderungen unter dem Oberlid und Limbusveränderungen superior betont. Sofern Hornhautkomplikationen bestehen oder drohen, ist eine konsequente und langfristige immunsuppressive und entzündungshemmende Lokaltherapie geboten. Diese beiden Eigenschaften vereint der Wirkstoff Ciclosporin A (CSA). Vorteilhaft an der topischen Gabe von CSA sind zudem der steroidsparende Effekt und die langfristige Reduktion von Exazerbationen. Die Eltern sind über die Chronizität und Rezidivneigung beider Erkrankungen aufzuklären, daher sollte die Therapie primär für mindestens 1 Jahr geplant werden, um die komplexen immunologischen Prozesse wirksam zu durchbrechen. Dies sichert die Sehentwicklung und verhindert narben- und astigmatismusbedingte Amblyopien im Kindesalter. Wir hoffen, dass die dargelegte Datenlage die Hemmschwelle für die Verordnung von CSA auf der kindlichen Augenoberfläche sinken lässt.

Purpose: To present the case of a 9-year-old child with bilateral posterior subcapsular cataract developed through steroid treatment for acute lymphoblastic leukemia. Cataract surgery with trifocal intraocular lens implantation was performed in both eyes. Observations: Uncorrected distance visual acuity increased from +0.3 and + 0.4 logMAR preoperatively to 0.00 and + 0.04 logMAR after surgery. Binocular uncorrected values for intermediate and near visual acuity were −0.04 logMAR and 0.02 logMAR after surgery, respectively. The patient did not report side effects like halos or glare and was able to participate in his daily activities (school and sports) without spectacles. Conclusions and Importance: This report represents the first description of a bilateral implantation of trifocal intraocular lenses in a pediatric cataract case with restoration of visual function in far, intermediate and near distance. Trifocal intraocular lenses to compensate for the loss of accommodation can be an option in selected cases of children with cataract. Keywords: Pediatric cataract surgery, Trifocal IOL, Leukemia, Steroid-induced cataract

Summary Background The human cytokine erythropoietin conveys neuroprotection in animal models but has shown ambiguous results in phase 2 clinical trials in patients with optic neuritis. We assessed the safety and efficacy of erythropoietin in patients with optic neuritis as a clinically isolated syndrome in a multicentre, prospective, randomised clinical trial. Methods This randomised, placebo-controlled, double-blind phase 3 trial, conducted at 12 tertiary referral centres in Germany, included participants aged 18–50 years, within 10 days of onset of unilateral optic neuritis, with visual acuity of 0·5 or less, and without a previous diagnosis of multiple sclerosis. Participants were randomly assigned (1:1) to receive either 33 000 IU erythropoietin or placebo intravenously for 3 days as an adjunct to high-dose intravenous methylprednisolone (1000 mg per day). Block randomisation was performed by the trial statistician using an SAS code that generated randomly varying block sizes, stratified by study site and distributed using sealed envelopes. All trial participants and all study staff were masked to treatment assignment, except the trial pharmacist. The first primary outcome was atrophy of the peripapillary retinal nerve fibre layer (pRNFL), measured by optic coherence tomography (OCT) as the difference in pRNFL thickness between the affected eye at week 26 and the unaffected eye at baseline. The second primary outcome was low contrast letter acuity at week 26, measured as the 2·5% Sloan chart score of the affected eye. Analysis was performed in the full analysis set of all randomised participants for whom treatment was started and at least one follow-up OCT measurement was available. Safety was analysed in all patients who received at least one dose of the trial medication. This trial is registered at ClinicalTrials.gov, NCT01962571. Findings 108 participants were enrolled between Nov 25, 2014, and Oct 9, 2017, of whom 55 were assigned to erythropoietin and 53 to placebo. Five patients were excluded from the primary analysis due to not receiving the allocated medication, withdrawn consent, revised diagnosis, or loss to follow-up, yielding a full analysis set of 52 patients in the erythropoietin group and 51 in the placebo group. Mean pRNFL atrophy was 15·93 μm (SD 14·91) in the erythropoietin group and 14·65 μm (15·60) in the placebo group (adjusted mean treatment difference 1·02 μm; 95% CI −5·51 to 7·55; p=0·76). Mean low contrast letter acuity scores were 49·60 (21·31) in the erythropoietin group and 49·06 (21·93) in the placebo group (adjusted mean treatment difference −4·03; −13·06 to 5·01). Adverse events occurred in 43 (81%) participants in the erythropoietin group and in 42 (81%) in the placebo group. The most common adverse event was headache, occuring in 15 (28%) patients in the erythropoietin group and 13 (25%) patients in the placebo group. Serious adverse events occurred in eight (15%) participants in the erythropoietin and in four (8%) in the placebo group. One patient (2%) in the erythropoietin group developed a venous sinus thrombosis, which was treated with anticoagulants and resolved without sequelae. Interpretation Erythropoietin as an adjunct to corticosteroids conveyed neither functional nor structural neuroprotection in the visual pathways after optic neuritis. Future research could focus on modified erythropoietin administration, assess its efficacy independent of corticosteroids, and investigate whether it affects the conversion of optic neuritis to multiple sclerosis. Funding German Federal Ministry of Education and Research (BMBF).

Optic neuritis is a special challenge to the ophthalmologist. It is a relatively frequent condition but difficult to seize morphologically. It has neurological implications and is subject matter of recent trials.Selective literature search including the authors' professional experience.Practical aids for the ophthalmological management of optic neuritis are derived from the best available evidence and the recent literature is discussed.The present paper provides evidence-based recommendations for a safe handling of optic neuritis as well as information on current issues.Die Optikusneuritis stellt eine besondere Herausforderung für den Augenarzt dar. Sie kommt vergleichsweise häufig vor, kann aber morphologisch nicht gut festgemacht werden. Sie hat neurologische Implikationen und ist Gegenstand neuer Studien.Selektive Literaturrecherche unter Berücksichtigung eigener Erfahrungen.Aus der besten verfügbaren Evidenz erarbeitet die vorliegende Arbeit praktische Hilfen zum augenärztlichen Management der Optikusneuritis und diskutiert die aktuelle Literatur.Der vorliegende Artikel stellt evidenzbasierte Empfehlungen zum sicheren Umgang mit der Optikusneuritis zur Verfügung und informiert über aktuelle Fragestellungen.

Background: The human cytokine erythropoietin (EPO) conveys neuroprotection in preclinical models and has shown ambiguous results in phase 2 clinical trials in optic neuritis. We assessed the efficacy and safety of EPO in optic neuritis as a clinically isolated syndrome in a sufficiently powered RCT. Methods: This randomized, placebo-controlled, double-blind phase 3 trial conducted at 12 German tertiary care centres included participants aged 18-50 years with an acute presentation of unilateral optic neuritis within 10 days, visual acuity ≤ 0·5 and without previously known multiple sclerosis. Participants were randomly assigned (1:1) to receive either 33.000 IU erythropoietin or placebo intravenously for three days as an adjunct to high-dose methylprednisolone (1000 mg/day). Block Randomisation was performed with randomly varying block sizes, stratified by study site and distributed using sealed envelopes. All trial participants and all study staff were masked from treatment assignment with the exception of the trial pharmacist. The first primary outcome was the amount of retinal nerve fibre layer (RNFL) atrophy, measured by optic coherence tomography (OCT) as the difference in RNFL thickness between the affected eye at week 26 and the unaffected eye at baseline. The second primary outcome was low contrast visual acuity at week 26, measured as the 2·5% Sloan chart score of the affected eye. Analysis was performed in a set of all randomised participants for whom treatment was started and at least one follow-up OCT measurement was available. Safety was analysed in all patients who received at least one dose of the trial medication. Findings: The study enrolled 108 participants between November 25 th , 2014 and October 9 th , 2017, 55 of which were assigned to EPO and 53 to placebo. Five patients were excluded from the primary analysis due to screening failure, revised diagnosis or loss to follow-up, yielding a full analysis set of 52 EPO and 51 placebo recipients. The adjusted mean treatment difference of EPO versus placebo was 1·02 µm of RNFL atrophy (95% CI -5·51 to 7·55; p=0·76; unadjusted 15·9 vs 14·7 µm) and -4·03 (-13·6 to 5·01; p=0·38) on the letter score for low contrast visual acuity (unadjusted 49·06 vs 49·60). One patient in the EPO group developed a sinus venous thrombosis which was treated with anticoagulants and resolved without sequelae. Interpretation: EPO conveyed neither functional nor structural neuroprotection in the visual pathways after optic neuritis. Trial Registration: This trial is registered with ClinicalTrials.gov (NCT01962571). Funding Statement: German Ministry of Health and Science (BMBF), Clinical trials programme. Declaration of Interests: WL reports grants from the German Federal Ministry for Education and Research (BMBF) and the German Research Foundation (DFG). He received speakers’ honoraria from and worked on advisory boards for Alcon, Allergan, Santhera, Boehringer Ingelheim, and Merz Pharma. SK has received a doctoral fellowship with funding from the University of Freiburg, Faculty of Medicine and the Else Kroner-Fresenius foundation. GI has received funding from Novartis and Janssen Pharmaceutica. BG and FB have nothing to report. MV has worked on an advisory board for Roche Pharma. SH has received funding from the DFG, the German Federal Institute for Sport Science and the German Ophthalmological Society. PA reports reports grants, personal fees, and non-financial support from Allergan, Biogen, Celgene, Ipsen, Merck, Merz Pharmaceuticals, Novartis, Roche, and Teva outside the submitted work. JU has received a doctoral fellowship with funding from the Ministry of Science, Research and Arts Baden-Wuerttemberg as part of the HAW-Prom program. She received a speakers’ honorary from AMO Ireland (affiliated to Johnson & Johnson Vision) and is co-inventor of patents/patent applications with the numbers 10 2017 126 741, WO 2020/089284 A1 and 20 174 551.0. MW is a managing partner of Blickshift GmbH and coinventor of patent applications WO 2020/089284 A1 and 20 174 551.0. MH reports grants from Bristol-Myers Squibb and speaker’s honoraria from Amgen, Baxter Deutschland, CSL Behring, Biotest Pharma, Fresenius Kabi, Leo Pharma, Merck Serono, Novartis Pharma, Pfizer, Roche Pharma, and Sun Pharmaceuticals Industries. SW has served as a consultant for Bayer, Novartis, Chengdu Kanghong Biotech, Zeiss, and Roche and has received grant support from Zeiss and Heidelberg Engineering. RD was funded by the BMBF, the DFG, and the Hertie Foundation. Ethics Approval Statement: The study protocol was approved by the institutional review boards and ethics committees of each participating site under the conduct of the ethics committee of the University of Freiburg, Germany.

Acute liver failure (ALF) in infancy and childhood is a life-threatening emergency and in about 50 % the etiology remains unknown. Recently biallelic mutations in NBAS were identified as a new molecular cause of ALF with onset in infancy, leading to recurrent acute liver failure (RALF). The phenotype and medical history of 14 individuals with NBAS deficiency was studied in detail and functional studies were performed on patients’ fibroblasts. The phenotypic spectrum of NBAS deficiency ranges from isolated RALF to a multisystemic disease with short stature, skeletal dysplasia, immunological abnormalities, optic atrophy, and normal motor and cognitive development resembling SOPH syndrome. Liver crises are triggered by febrile infections; they become less frequent with age but are not restricted to childhood. Complete recovery is typical, but ALF crises can be fatal. Antipyretic therapy and induction of anabolism including glucose and parenteral lipids effectively ameliorates the course of liver crises. Patients’ fibroblasts showed an increased sensitivity to high temperature at protein and functional level and a disturbed tethering of vesicles, pointing at a defect of intracellular transport between the endoplasmic reticulum and Golgi. Mutations in NBAS cause a complex disease with a wide clinical spectrum ranging from isolated RALF to a multisystemic phenotype. Thermal susceptibility of the syntaxin 18 complex is the basis of fever dependency of ALF episodes. NBAS deficiency is the first disease related to a primary defect of retrograde transport. Identification of NBAS deficiency allows optimized therapy of liver crises and even prevention of further episodes.

INTRODUCTION: Optic neuritis leads to degeneration of retinal ganglion cells whose axons form the optic nerve. The standard treatment is a methylprednisolone pulse therapy. This treatment slightly shortens the time of recovery but does not prevent neurodegeneration and persistent visual impairment. In a phase II trial performed in preparation of this study, we have shown that erythropoietin protects global retinal nerve fibre layer thickness (RNFLT-G) in acute optic neuritis; however, the preparatory trial was not powered to show effects on visual function. METHODS AND ANALYSIS: Treatment of Optic Neuritis with Erythropoietin (TONE) is a national, randomised, double-blind, placebo-controlled, multicentre trial with two parallel arms. The primary objective is to determine the efficacy of erythropoietin compared to placebo given add-on to methylprednisolone as assessed by measurements of RNFLT-G and low-contrast visual acuity in the affected eye 6 months after randomisation. Inclusion criteria are a first episode of optic neuritis with decreased visual acuity to ≤0.5 (decimal system) and an onset of symptoms within 10 days prior to inclusion. The most important exclusion criteria are history of optic neuritis or multiple sclerosis or any ocular disease (affected or non-affected eye), significant hyperopia, myopia or astigmatism, elevated blood pressure, thrombotic events or malignancy. After randomisation, patients either receive 33 000 international units human recombinant erythropoietin intravenously for 3 consecutive days or placebo (0.9% saline) administered intravenously. With an estimated power of 80%, the calculated sample size is 100 patients. The trial started in September 2014 with a planned recruitment period of 30 months. ETHICS AND DISSEMINATION: TONE has been approved by the Central Ethics Commission in Freiburg (194/14) and the German Federal Institute for Drugs and Medical Devices (61-3910-4039831). It complies with the Declaration of Helsinki, local laws and ICH-GCP. TRIAL REGISTRATION NUMBER: NCT01962571. peerReviewed

Although contrast vision is not routinely tested, it is important: for instance, it predicts traffic incidents better than visual acuity. Mesopic contrast sensitivity (CS) testing approximates low-lighting conditions but entails dark adaptation, which can disrupt clinical routine. In receptor-specific diseases, a dissociation of photopic and mesopic sensitivity would be expected, but can photopic CS act as a surrogate measure for mesopic CS, at least for screening purposes? Photopic and mesopic contrast sensitivities were studied in three groups: 47 normal subjects, 23 subjects with glaucoma, and three subjects with cataract. Twenty-eight of the normal subjects were additionally tested with artificial blur. Photopic contrast sensitivity was assessed with both the Freiburg Acuity and Contrast Test (FrACT) and the Mars Letter Contrast Sensitivity Charts. Mesopic contrast sensitivity, without and with glare, was measured with the Mesoptometer IIb. Coefficients of repeatability and limits of agreement were calculated for all tests. Test–retest limits of agreement were ± 0.17 logCS for Mars, ± 0.21 logCS for FrACT, and ±0.20 logCS / ± 0.14 logCS for Mesoptometer IIb without and with glare, respectively. In terms of inter-test comparison, Mars and FrACT largely agreed, except for ceiling effects in the Mars test. While mesopic and photopic contrast sensitivities correlate significantly (r = 0.51, p

The primary objective of this bi-center explorative pilot study was the quantitative assessment of visual field defects and retinal nerve fiber layer thickness (RNFT) over 6 months in patients with acute non-arteritic anterior ischemic optic neuropathy (NAION), in order to elucidate the natural course of NAION and provide a reference dataset for future treatment studies.16 patients (age 41-80 years, nine males, seven females) suffering from acute NAION and presenting within 7 days after onset of symptoms were included in this study. The following examinations were carried out at the initial visit (month 0) and at months 2, 4 and 6: entire (90°) visual field examination with automated static white-on-white perimetry, quantified by mean defect (MD); peripapillary retinal nerve fiber layer thickness (RNFT) measurement with spectral domain optical coherence tomography (SD-OCT); assessment of distant best correct visual acuity (D-BCVA) and a quantification of the relative afferent pupillary defect (RAPD) using the swinging flashlight test with neutral density filters. Perimetric Mean Defect (MD) and RNFT values were each compared between the consecutive visits using the non-parametric Friedman test.The initial MD was 6.2 dB (IQR 5.0-7.4) without significant changes further on. RNFT was 183 μm (IQR 148-252) initially, decreased significantly at month 2 (78 μm (IQR 71-93) and further at month 4 (64 μm (IQR 58-74) and 6 (61 μm (IQR 52-81), Friedman test, p 0.001). Initially, RNFT was above normal limits (due to swelling) in 15/16 patients; at month 2 it was below normal limits in 13/16 patients, at month 4 in 12/13 patients and at month 6 in 9/10 patients. 7/16 patients exhibited segmental swelling of the optic disc, whereas the entire circumference of the optic disc showed RNFL thickening in 9/16 patients.Functional deficits were present directly after onset of NAION and did not change relevantly further on. Morphological changes comprise severe swelling after onset of NAION, which rapidly turns into atrophy. Already after 2 months more than 80 % of the patients showed a RNFT below normal limits. Progressive RNFL thinning between month 2 and month 4 suggests ongoing atrophy, whereas a stable morphologic end point is reached after month 4.