CONTEXT:Interleukin (IL)-17, a key proinflammatory cytokine, drives inflammation and fibrosis in Graves orbitopathy (GO), and elevated IL-17 and Th17 cells correlate with disease activity and severity. OBJECTIVE:The ORBIT study aimed to evaluate the efficacy and safety of secukinumab, an IL-17A inhibitor, in individuals with active, moderate-to-severe GO. METHODS:A randomized, double-blind, placebo-controlled, parallel-group, multicenter trial was conducted. Adults with active, moderate-to-severe, non-sight-threatening GO randomly (1:1) received secukinumab 300 mg or placebo subcutaneously over a 16-week double-blind treatment period, followed by an additional 16-week open-label treatment phase for proptosis nonresponders. Safety parameters, thyroid-related hormones, and autoantibodies were also assessed. The primary end point was overall response of reduced Clinical Activity Score (CAS) of 2 or more points and a reduction of 2 mm or greater in proptosis from baseline without worsening in the fellow eye at week 16. RESULTS:Twenty-eight adult GO patients with a CAS of 4 or greater were enrolled (secukinumab, n = 14; placebo, n = 14). None in either the secukinumab or placebo group achieved an overall response at weeks 16 and 32, respectively, when all patients received open-label secukinumab. No clinically meaningful changes were observed in ophthalmic symptoms and signs, proptosis, lid aperture, eye muscle motility, CAS, and health-related quality of life either at week 16 or week 32. No meaningful effect on serum levels of thyroid-related hormones and antibodies was observed. Secukinumab was well tolerated, with mostly mild adverse events. Neither treatment-induced study discontinuation nor new safety signals were registered. CONCLUSION:Secukinumab did not show clinical efficacy vs placebo when treating patients with active, moderate-to-severe GO.

Abstract:PURPOSE: This study aimed to assess the effectiveness of tendon elongation using Tutopatch as a secondary intervention for residual infantile esotropia, particularly in patients exhibiting limited abduction, for whom further conventional surgery might not yield successful outcomes. Abstract:METHODS: Design: This retrospective study analysed data from patients with residual deviation angles following previous surgery for infantile esotropia. Participants and intervention: All patients who underwent tendon elongation with Tutopatch between 2009 and 2023 were included in the study, excluding those with other ocular or strabismus diagnoses or those who refused to participate. Setting: The data were collected from patients operated at a single tertiary medical centre. The analysis included pre- and postoperative deviation angles recorded at one day, three months, and twelve months, if available. The dose-effect relationship, defined as the deviation angle in degrees per effective operative distance in millimetres, and overall success rates were determined. Primary and secondary outcome measures: The primary outcome measure was the effect of using Tutopatch on the deviation angle. The secondary measure was the effect on ocular motility. Abstract:RESULTS: Participants and intervention: The study included twenty-nine patients (ten females, 19 males) with a median age of 17 years (range: 5 - 59 years). The median preoperative deviation angle was + 15° (range: + 6° to + 27°), which decreased to - 1.5° (range: - 12° to + 7°) at the three-month follow-up in 22 patients. Subsequent long-term follow-up in ten patients, after a median duration of 34.5 months (range: 13 to 69 months), revealed a median deviation angle of - 1° (range: - 15° to + 15°). Postoperative adduction was slightly reduced, by a median of 5° (range: loss of 30° to gain of 15°). No postoperative complications were observed. Overcorrection necessitated re-operation in five patients, which was uneventful. The median postoperative deviation angle following re-operation was - 1° (range: - 5° to + 8°). Abstract:CONCLUSION: Tendon elongation with Tutopatch is effective in managing significant residual infantile esotropia, particularly in complex cases, with minimal impact on duction limitation. Long-term overcorrection may occur, necessitating re-operation that can be performed without difficulty.

Introduction Glaucoma is one of the most common causes of blindness and affects more than 70 million people worldwide. The disease is characterised by the loss of retinal ganglion cells associated with a progressive optic neuropathy, resulting in an impairment of visual functions, for example, visual field loss. Nowadays, the only modifiable risk factor is the increase in intraocular pressure, and its treatment is to lower this pressure by medication, laser treatment or surgery to avoid disease progression. New methods for preventing and reversing vision loss are thus urgently needed. Several small and two multicentre studies have presented evidence that repetitive transorbital alternating current stimulation (rtACS) can lead to long-lasting visual field improvement. This could open a new and inexpensive therapeutic option for optic atrophy. However, the level of evidence for this method is still fairly rather poor, and further trials are needed. Therefore, this clinical trial aims to prove the effectiveness of rtACS compared with sham stimulation in patients with primary open-angle glaucoma (POAG).Methods and analysis VIRON (Vision Restoration in Optic Neuropathy) is a national, multicentre, prospective, randomised, placebo-controlled, double-blind trial with three arms. The primary objective is to assess the effectiveness of rtACS in patients with POAG compared with sham stimulation. The primary outcome is the change in mean defect (MD) in the visual field immediately after 10 sessions of rtACS (days 9, 16 and 23) compared with the values of initial perimetry (days −21 to –14 and 0) after applying electrical stimulation with a classical montage, compared with sham and electrical stimulation using individualised montage. Secondary outcome measures comprise a long-term effect with changes in MD at 24 weeks after stimulation, and data from the National Eye Institute Visual Function-25 and quality of life (Short Form 36) questionnaires. The target population are patients with glaucomatous optic atrophy and significant glaucomatous visual field defects (MD of 5–22 dB) due to POAG.After randomisation, patients received either classical rtACS (group 1), individual rtACS (group 2) or sham stimulation (group 3) in daily 25 min stimulation sessions in two series of five consecutive days separated by a weekend interval. In group 1, active stimulation will be via the routinely applied montage using two electrodes affixed on the right and left side of the head, next to the eyes, with straightforward fixation. In group 2, the current flow will be individually modelled (MRI-based) to target areas of partial visual field defects by optimising electrode positions in conjunction with an optimised visual fixation direction. Group 3 with sham stimulation will serve as control.The calculated sample size required to achieve a statistical power of 80% for a relevant effect size and allow for dropouts was 300 (100 per group). The trial has already begun with the first patient in July 2023. The planned recruitment period is 24 months with an estimated end of the study in November 2025 (last patient out). An adjusted extension of the study period is planned.Ethics and dissemination VIRON was approved by the Central Ethics Committee of the University Medical Center Göttingen (19 October 2022) and those of the individual participating centres (Bonn: 446/23-EP, Hamburg: 2023-200889-BO-bet, Cologne: 23-1487 and Mainz: 2023-17399-§23b). The study protocol complies with the Declaration of Helsinki, the national medicine device regulation (MDR) laws and the international standards of good clinical practice (GCP).The study protocol (V.5, 24 November 2023) was designed following the Standard Protocol Items: Recommendations for Interventional Trials guidelines and is registered on https://drks.de/search/de/trial/DRKS00029129.As study initiatior the University Medical Center Göttingen (UMG) is responsible for data ownership and data management of the VIRON study. The study data will be published within 6 months of the study being completed. After the publication of the primary results, all data are anonymised and published in an open-access journal to ensure access to the data for third parties.Trial registration number https://drks.de/search/de/trial/DRKS00029129.

Anophthalmia and microphthalmia (AM) are rare ocular developmental disorders in which one or both eyes are absent or abnormally small. Malformations can be limited to the globe, or involve the orbit, such as associated orbital cysts and/or insufficient development of the orbital bones and eyelids. Aetiology of AM is believed to be mainly genetic, with environmental, infectious, nutritional and other exogenous factors also playing a role. Recent advances in genetics have enhanced our understanding of the genetic basis of AM, as well as improving diagnosis and genetic counselling. A comprehensive ophthalmic and paediatric screening is required to detect any other anomalies, and imaging should be performed to assess the orbital content and the presence of an associated cyst. Management requires a multidisciplinary approach including oculoplastic surgeons, ocularists, paediatricians and geneticists. When there is no visual potential, the goal is to achieve the best possible aesthetic outcomes by early socket expansion, which is crucial for minimising the potential impact of AM on facial growth and development. Cosmetic rehabilitation can be achieved with conservative or surgical approaches, including ocular prostheses, socket or orbital expansion, with or without socket and/or eyelid reconstructive surgery. In AM with orbital cyst, the timing of cyst removal is important to avoid distortion of the periorbital tissues.

Background Graves’ orbitopathy (GO) is subject to epidemiological and care-related changes. Aim of the survey was to identify trends in presentation of GO to the European Group On Graves’ Orbitopathy (EUGOGO) tertiary referral centres and initial management over time. Methods Prospective observational multicentre study. All new referrals with diagnosis of GO within September–December 2019 were included. Clinical and demographic characteristics, referral timelines and initial therapeutic decisions were recorded. Data were compared with a similar EUGOGO survey performed in 2012. Results Besides age (mean age: 50.5±13 years vs 47.7±14 years; p 0.007), demographic characteristics of 432 patients studied in 2019 were similar to those in 2012. In 2019, there was a decrease of severe cases (9.8% vs 14.9; p<0.001), but no significant change in proportion of active cases (41.3% vs 36.6%; p 0.217). After first diagnosis of GO, median referral time to an EUGOGO tertiary centre was shorter (2 (0–350) vs 6 (0–552) months; p<0.001) in 2019. At the time of first visit, more patients were already on antithyroid medications (80.2% vs 45.0%; p<0.001) or selenium (22.3% vs 3.0%; p<0.001). In 2019, the initial management plans for GO were similar to 2012, except for lid surgery (2.4% vs 13.9%; p<0.001) and prescription of selenium (28.5% vs 21.0%; p 0.027). Conclusion GO patients are referred to tertiary EUGOGO centres in a less severe stage of the disease than before. We speculate that this might be linked to a broader awareness of the disease and faster and adequate delivered treatment.

Since the last and at the same time first statement of the German ophthalmological societies on the possibilities of reducing myopia progression in childhood and adolescence, many new details and aspects have emerged in clinical research. This second statement updates the previous document and specifies the recommendations on visual and reading behavior as well as on pharmacological and optical therapy options, which have been both refined and newly developed in the meantime.

Multiple sulfatase deficiency (MSD) is an extremely rare autosomal recessively inherited disease with a prevalence of 1:500.000 caused by mutations on the sulfatase-modifying-Factor 1 gene (SUMF1). MSD is most specifically characterised by a combination of developmentally retarded psychomotoric functions, neurodegeneration that entails the loss of many already acquired abilities, and by ichthyosis. Other symptoms include those associated with mucopolysaccharidosis, i.e., facial dysmorphy, dwarfism, and hepatosplenomegaly. In 50–75% of all MSD-affected patients, functional or structural ocular damage is likely. MSD seldom affects the anterior segment of the eye. The main pathology these patients present is a highly conspicuous tapetoretinal degeneration, similar to severe Retinitis pigmentosa, that leads to blindness at an early age. An initially five-year-old boy with MSD, genetically verified at his first examination in our opthalmology department (SUMF1 mutations c.776A>T, p.Asn259Ile; c.797A>T, p.Pro266Leu; c.836A>T, p.Ala279Val), and a 4, 5 year regular follow-up are described. The patient had some visual potential (“tunnel view”), which deteriorated dramatically after his fifth birthday. We observed no evidence of worsening retinal involvement in this patient in spite of his progressively worsening clinical symptoms, extending to total blindness/no light perception. OCT revealed that the outer retinal layers containing photoreceptors were diseased; the ellipsoid zone was only partially discernible and the outer nuclear layer appeared to be thinned out. The inner nuclear layer, ganglion cell layer, and retinal nerve fibre layer were indistinguishable. These anomalies are indicative of a severe pathology within the retina’s inner layers. Characteristic anomalies in the fundus should stimulate clinicians to suspect a case of MSD in their differential diagnosis, and thus to order thorough genetic and paediatric diagnostics.

In this work, we provide a detailed characterization of a rare complication—subconjunctival cyst formation after strabismus surgery—in a large German cohort. We conducted a retrospective analysis of 822 consecutive patients who underwent strabismus surgery between 2015 and 2022. The patients received comprehensive eye and orthoptic examinations preoperatively, at 1 day, and at 3 months postoperatively. Cysts were analyzed with slit-lamp examination, anterior segment optical coherence tomography (AS-OCT), and histopathological subsumption. Nineteen cases of postoperative cysts were observed (2.3

Zusammenfassung Die optische Kohärenztomografie (OCT) wird bei der Diagnostik retinaler und glaukomatöser Erkrankungen routinemäßig eingesetzt. Seitdem eine so hohe Auflösung möglich ist, dass die einzelnen Netzhautschichten darstellbar und auch segmentierbar sind, hielt die OCT auch Einzug in die Neuroophthalmologie. Dieser Beitrag zeigt aktuelle und zukünftige Einsatzmöglichkeiten in der Neuroophthalmologie und vermittelt Kenntnisse über mögliche Tücken.

One of the greatest challenges for ocularists is prosthetic fitting in children, especially in children with congenital anomalies such as clinical anophthalmia or functionless (blind) microphthalmia. The most frequent reason for prosthetic fitting in children is a condition following enucleation for retinoblastoma, followed by trauma and congenital pathologies. The standard treatment after enucleation or evisceration begins intraoperatively with the selection of an suitable implant and the use of a conformer at the end of the operation to shape the prosthetic cavity. An initial prosthesis can be fitted 4 weeks postoperatively, with a final fitting taking place 3 months later. If iatrogenic scarring or scarring due to an infection of the prosthetic cavity occurs, the approach of the ocularist must be appropriately adapted with the use of modified prosthesis shapes and shorter treatment intervals. Surgical options include scar excision and oral mucosa or amniotic membrane transplantation. Congenital anomalies require the shortest treatment intervals and even more so for anophthalmia than for microphthalmia. The strategy is characterized by simultaneous stimulation of the soft tissue of the ocular adnexa as well as the bony orbit. As self-inflating hydrogel expanders are no longer available, conservative prosthetic treatment is the only option. Close cooperation between child/parent, ocularist and ophthalmic plastic surgeon is the best prerequisite for a good long-term treatment outcome.

Eine der größten okularistischen Herausforderungen stellt die prothetische Versorgung von Kindern dar, insbesondere gilt das für Kinder mit angeborenen Anomalien wie klinischem Anophthalmus oder funktionslosem (blinder) Mikrophthalmus. Die häufigste Ursache für eine Prothesenanpassung im Kindesalter ist ein Zustand nach Enukleation wegen eines Retinoblastoms, gefolgt von Trauma und kongenitalen Pathologien. Die Regelversorgung nach Enukleation oder Eviszeration beginnt intraoperativ mit der Auswahl eines adäquaten Implantates und dem Einsatz eines Konformers am Ende der Operation zur Ausformung der Prothesenhöhle. Eine erste Prothese kann meist 4 Wochen postoperativ angepasst werden, die endgültige Versorgung erfolgt 3 Monate später. Kommt es iatrogen oder nach Infektion zu Vernarbungen der Prothesenhöhle, muss das okularistische Vorgehen mit Nutzung modifizierter Prothesenformen und kürzeren Behandlungsintervallen daran adaptiert sein. Operative Optionen umfassen die Narbenexzision, Mundschleimhaut- oder Amniontransplantation. Kongenitale Anomalien verlangen die kürzesten Behandlungsintervalle, beim Anophthalmus noch einmal mehr als beim Mikrophthalmus. Die Strategie ist durch simultane Stimulation des Weichteilgewebes der okulären Adnexe als auch der knöchernen Orbita gekennzeichnet. Nachdem selbstquellende Hydrogelexpander aktuell nicht mehr verfügbar sind, bleibt eine konservative Prothesenbehandlung die einzige Option. Eine enge Kooperation zwischen Kind/Eltern, Okularist und Okuloplastiker ist die beste Voraussetzung für ein langfristig gutes Behandlungsergebnis.

Optical coherence tomography (OCT) has become the most important innovation in ophthalmology over the last 30 years and is used routinely, especially in the diagnosis of retinal and glaucomatous diseases. It is fast, non-invasive and reproducible. Since the procedures can offer such a high resolution that the individual retinal layers can be visualised and segmented, this examination technique has also found its way into neuroophthalmology. Especially the peripapillary nerve fibre layer (RNFL) and the ganglion cell layer (GCL) provide valuable diagnostic and prognostic information in cases of visual pathway disease and morphologically unexplained visual disorders. OCT is helpful in determining the cause of optic disc swelling and EDI-OCT can reliably detect buried, non-calcified drusen. This article is intended to provide the reader with an overview of current and future applications of OCT in neuroophthalmology and knowledge of possible pitfalls.

INTRODUCTION:Myopia is a major cause of degenerative eye disease and increases the risk of secondary visual impairment. Mitigating its progression therefore has great potential of clinically relevant benefit as shown by using highly diluted atropine eye drops in children of Asian origin. However, limited evidence is available regarding the efficacy and safety of low-dose atropine therapy in non-Asian populations. Hence, the Low-dose AtropIne for Myopia Control in Children (AIM) study will test the efficacy and safety of 0.02% atropine vs placebo in a German population. METHODS AND ANALYSIS:AIM is a national, multicentre, prospective, randomised, placebo-controlled, double-blind trial with two parallel arms. The primary objective is to assess the efficacy of atropine 0.02% eyedrops for myopia control in children of Caucasian origin. The primary outcome is the change in cycloplegic refraction after 1 year of treatment (D/year). Secondary and tertiary outcome measures comprise the change in axial length (mm/year) in children treated with 0.02% atropine compared with placebo, the myopic progression of participants treated with 0.01% compared with 0.02% atropine (D/year and mm/year), and the safety profile of both 0.02% and 0.01% atropine. Furthermore, the myopic progression 1 year after cessation of therapy with 0.02% atropine will be evaluated. Inclusion criteria are an age of 8-12 years and myopia of -1 D to -6 D with an estimated annual myopia progression of ≥0.5 D. After randomisation, patients will receive either atropine 0.02% (arm A) or placebo eye drops (arm B) in the first year of treatment. In the second year, they will continue to receive atropine 0.02% (arm A) or switch to atropine 0.01% (arm B). In the third year, they will switch to placebo (arm A) or continue with atropine 0.01% (arm B). To achieve a statistical power of 80%, the calculated sample size is 300. The trial has started in October 2021 with a planned recruitment period of 18 months. ETHICS AND DISSEMINATION:AIM has been approved by the Central Ethics Committee of the University Medical Center Freiburg (21-1106), local ethics committees of each participating centre and the German Federal Institute for Drugs and Medical Devices (61-3910-4044659). It complies with the Declaration of Helsinki, local laws and ICH-GCP. Results and underlying data from this trial will be disseminated through peer-reviewed publications and conference presentations. TRIAL REGISTRATION NUMBER:NCT03865160.