Radius-Maumenee syndrome-associated glaucoma, a therapeutic challenge: A case report.

INTRODUCTION: Radius-Maumenee syndrome is a rare cause of open-angle glaucoma, secondary to elevated episcleral venous pressure (EVP) without any orbital or systemic abnormalities. CASE PRESENTATION: We present a case of a male patient in his mid-sixties, who presented with bilateral dilated episcleral vessels, bilateral glaucoma, chorioretinal folds in both maculae and choroidal effusion in his left eye. Our case highlights the differentials that should be considered and the systematic investigations that should be performed. We describe the clinical, optical coherence tomography and angiography findings of this patient and propose a potential pathophysiological mechanism leading to the propensity for perioperative complications. CONCLUSION: Radius-Maumenee syndrome should be a diagnosis of exclusion. Secondary glaucoma can remain refractory to medical treatment and filtering surgery carries the risk intra-operative or post-operative uveal effusions.

A practical approach to uveitis screening in children with juvenile idiopathic arthritis.

BACKGROUND: Juvenile idiopathic arthritis (JIA)-associated uveitis typically presents as a silent chronic anterior uveitis and can lead to blindness. Adherence to current screening guidelines is hampered by complex protocols which rely on the knowledge of specific JIA characteristics. The Multinational Interdisciplinary Working Group for Uveitis in Childhood identified the need to simplify screening to enable local eye care professionals (ECPs), who carry the main burden, to screen children with JIA appropriately and with confidence. METHODS: A consensus meeting took place in January 2023 in Barcelona, Spain, with an expert panel of 10 paediatric rheumatologists and 5 ophthalmologists with expertise in paediatric uveitis. A summary of the current evidence for JIA screening was presented. A nominal group technique was used to reach consensus. RESULTS: The need for a practical but safe approach that allows early uveitis detection was identified by the panel. Three screening recommendations were proposed and approved by the voting members. They represent a standardised approach to JIA screening taking into account the patient's age at the onset of JIA to determine the screening interval until adulthood. CONCLUSION: By removing the need for the knowledge of JIA categories, antinuclear antibody positivity or treatment status, the recommendations can be more easily implemented by local ECP, where limited information is available. It would improve the standard of care on the local level significantly. The proposed protocol is less tailored to the individual than the 'gold standard' ones it references and does not aim to substitute those where they are being used with confidence.

Show don't tell: assessing the impact of co-developed patient information videos in paediatric uveitis.

BACKGROUND/OBJECTIVES: There is a paucity of online educational content targeting children and young people with uveitis. We evaluated the impact of a co-designed patient education video on subjective and objective understanding of childhood uveitis. SUBJECTS/METHODS: Co-designed patient education media were produced in collaboration with the Childhood Uveitis Studies steering group and the Great Ormond Street Hospital Generation R Young People's Advisory Group and narrated by children. Patients managed within the Uveitis service at GOSH were invited to take part in a pre-post survey, undertaken immediately prior to and following viewing of a patient education video. RESULTS: Forty-three patients participated. These were stratified according to age, duration of disease, and treatment type for analysis. Self-rated knowledge improved across all groups (p = 0.001), particularly in those with a new diagnosis of uveitis (Z = -8.124, p < 0.001). Objective knowledge scores improved across all questions, especially in younger children, those with new disease, and those on steroid only treatment (Z = -3.847, p < 0.001, Z = -3.975, p < 0.001, Z = -3.448, p < 0.001; respectively). Most participants reported the videos to be easy to understand and with the right amount of information. All stated that they learned something new. CONCLUSIONS: Patient understanding of disease and treatment is crucial to achieving the best possible outcomes for this chronic, relapsing remitting and potentially blinding disorder. Our findings data shows the potential value of co-designed patient information videos, specifically in our study benefitting younger patients and those recently diagnosed. We suggest that other clinical teams could collaborate fruitfully with patient groups to develop similar videos to target possible misinformation and potentially improve patient outcomes.

Quality assessment of anterior segment OCT images: Development and validation of quality criteria.

BACKGROUND: The utility of medical imaging is dependant on image quality. We aimed to develop and validate quality criteria for ocular anterior segment optical coherence tomography (AS-OCT) images. METHODS: We undertook a cross-sectional study using AS-OCT images from patients aged 6-16. A novel three-level grading system (good, limited or poor) was developed based on the presence of image artefact (categorised as lid, eyelash, cropping, glare, or movement artefact). Three independent experts graded 2825 images, with agreement assessed using confusion matrices and intraclass correlation coefficients (ICC) for each parameter. RESULTS: There was very good inter-grader IQA agreement assessing image quality with ICC 0.85 (95 %CI: 0.84-0.87). The most commonly occurring artefact was eyelash artefact (1008/2825 images, 36 %). Graders labelled 621/2825 (22 %) images as good and 384 (14 %) as poor. There was complete agreement at either end of the confusion matrix with no 'good' images labelled as 'poor' by other graders, and vice versa. Similarly, there was very good agreement when assessing presence of lash (0.96,0.94-0.98), movement (0.97,0.96-0.99), glare (0.82,0.80-0.84) and cropping (0.90,0.88-0.92). CONCLUSIONS: The novel image quality assessment criteria (IQAC) described here have good interobserver agreement overall, and excellent agreement on the differentiation between 'good' and 'poor' quality images. The large proportion of images graded as 'limited' suggests the need for refine this classification, using the specific IQAC features, for which we also report high interobserver agreement. These findings support the future potential for wider clinical and community care implementation of AS-OCT for the diagnosis and monitoring of ocular disease.

How should we approach management of childhood onset chronic anterior uveitis refractory to adalimumab?

INTRODUCTION: The management of refractory juvenile idiopathic associated uveitis (JIAU) or childhood-onset chronic anterior uveitis (CAU) is a challenge. There is no clear consensus or evidence base for to suggest the most appropriate therapy after primary or secondary failure of biweekly adalimumab. In this scenario, most clinicians advocate switching to another anti-tumor necrosis factor alpha inhibitor; however, there are a variety of other disease modifying agents to choose from albeit with a differing levels of evidence. AREAS COVERED: We discuss how to define nonresponse and potential treatment options for patients with JIAU and CAU refractory to biweekly adalimumab. EXPERT OPINION: Uncontrolled CAU and JIAU remain one of the most challenging diseases to manage and can lead to irreversible loss of vision in a third of those affected. Amongst the possible choices, weekly adalimumab, infliximab, tocilizumab and abatacept have more evidence to support their use. JAK inhibitors seem to be a promising option. Golimumab and Rituximab has also been thought to be partially effective in some refractory cases, whereas IL-17, IL-23, and IL-12 inhibition along with apremilast seem not to be a therapeutic option currently. The route of administration should also be considered as there can be significant pros and cons for different children.

Patient-targeted mobile applications in healthcare.

There has been an increase in the number of healthcare-related applications targeted at patients for use on mobile phones. With an increasing proportion of the population using such applications, it is important to understand the associated limitations, safety concerns and challenges of legalisation. This article explores the impact of these applications on frontline care and patient wellbeing, evaluating the literature surrounding the benefits and challenges of patient-targeted mobile applications in health care and analysing the limitations of existing research. The proclaimed benefits of such applications are not always evidence based. Furthermore, many healthcare applications are created by laypeople and not validated by healthcare authorities, creating a potential to cause patient harm. Further research is needed to identify long-term effects on both healthcare systems and individuals' psychosocial wellbeing. However, research in this field often lacks a universal perspective and may be influenced by underlying financial motives to promote use of the applications.

Ocular Inflammatory Events Following COVID-19 Vaccination in the Paediatric Population: A Multinational Case Series.

BACKGROUND: Ocular inflammatory events following COVID-19 vaccination have been reported in the adult population. METHODS: Multinational case series of patients under the age of 18 diagnosed with ocular inflammatory events within 28 days of COVID-19 vaccination. RESULTS: Twenty individuals were included. The most common event was anterior uveitis (n = 8, 40.0%), followed by intermediate uveitis (7 patients, 35%), panuveitis (4 patients, 20%), and posterior uveitis (1 patient, 5%). The event was noticed in the first week after vaccination in 11 patients (55.0%). Twelve patients (60.0%) had a previous history of intraocular inflammatory event. Patients were managed with topical corticosteroids (n = 19, 95.0%), oral corticosteroids (n = 10, 50.0%), or increased dose of immunosuppressive treatment (n = 6, 30.0%). Thirteen patients (65.0%) had a complete resolution of the ocular event without complications. All patients had a final visual acuity unaffected or less than three lines of loss. CONCLUSION: Ocular inflammatory events may happen in the paediatric population following COVID-19 vaccination. Most events were successfully treated, and all showed a good visual outcome.

The incidence, presenting clinical findings and treatment patterns of Birdshot Retinochoroiditis in a high-prevalence region: findings from Northern Ireland, England and Wales.

BACKGROUND: Birdshot Retinochoroiditis (BSRC) is a rare, chronic posterior uveitis that is strongly associated with HLA-A*29.2 positivity. To date, no robust incidence studies of BSRC have been undertaken. We present the first epidemiological study of BSRC in a high-prevalence region. METHODS: In collaboration with the British Ophthalmological Surveillance Unit, all new cases of BSRC between May 2017 and June 2019 were prospectively collected. Presenting demographics, symptoms, signs and treatment modalities were collected. A follow-up questionnaire twelve months later was also sent. RESULTS: Thirty-seven confirmed cases meeting the reporting criteria were identified. Twenty-three cases had both baseline and follow-up data. The total population incidence of BSRC was 0.035 cases per 100,000 person-years [95% CI 0.025-0.048 cases per 100 000 people]. 97.3% were HLA-A*29 positive. The median age was 46 years, with females making up 78% of patients. There were no significant differences in the latitudinal incidence of BSRC. At presentation, floaters were the most common symptom. Optic disc swelling was the most common sign. Mean presenting visual acuity was independent of symptom duration. Combined systemic corticosteroids and immunomodulatory therapy were the most common treatments at baseline and follow-up. Intravitreal steroids were equally popular at follow-up. CONCLUSIONS: This study provides the first nationwide estimate of the incidence of BSRC in a high-prevalence region. Cases were more common in females, with a broad range of presentation ages. No significant latitudinal effect of incidence was identified. Systemic therapy with steroids and IMT remain the most common treatments.

New and Updated Recommendations for the Treatment of Juvenile Idiopathic Arthritis-Associated Uveitis and Idiopathic Chronic Anterior Uveitis.

OBJECTIVE: The Multinational Interdisciplinary Working Group for Uveitis in Childhood identified the need to update the current guidelines, and the objective here was to produce this document to guide clinicians managing children with juvenile idiopathic arthritis-associated uveitis (JIAU) and idiopathic chronic anterior uveitis (CAU). METHODS: The group analyzed the literature published between December 2014 and June 2020 after a systematic literature review conducted by 2 clinicians. Pediatric rheumatologists were paired with ophthalmologists to review the eligible 37 publications. The search criteria were selected to reflect those used for the 2018 Single Hub and Access point for pediatric Rheumatology in Europe (SHARE) recommendations, in order to provide an update, rather than a replacement for that publication. The summary of the current evidence for each SHARE recommendation was presented to the expert committee. These recommendations were then discussed and revised during a video consensus meeting on January 22, 2021, with 14 voting participants, using a nominal group technique to reach consensus. RESULTS: JIAU treatment was extended to include CAU. Fourteen recommendations regarding treatment of JIAU und CAU with >90% agreement were accepted. CONCLUSION: An update to the previous 2018 SHARE recommendations for the treatment of children with JIAU with the addition of CAU was created using an evidence-based consensus process. This guideline should help support clinicians to care for children and young people with CAU.

Quantitative and qualitative assessment of anterior segment optical coherence tomography capture of disease state in childhood anterior uveitis.

BACKGROUND/AIMS: Anterior segment optical coherence tomography (AS-OCT) assessment of anterior chamber inflammation is an emerging tool. We describe the performance of AS-OCT in a paediatric population. METHODS: A mixed-methods prospective study, using routine clinical assessment as reference standard, and AS-OCT, with Tomey CASIA2 or Heidelberg Spectralis HS1, as index test, with data collected on patient perceptions of imaging. Repeatability, diagnostic indices, responsiveness to clinical change and clinical correlations of imaging-based metrics (image cell count, size, density and brightness) were assessed, with construction of receiver operated characteristic curves. Exploratory thematic analysis of responses from families was undertaken. RESULTS: A total of 90 children (180 eyes) underwent imaging. Bland Altman limits of agreement for CASIA2 repeatability ranged from +17 cells (95% CI 13.6 to 21.1) to -19 cells (95% CI -15.6 to -23.2) and HS1 from +1 (95% CI 0.9 to 1.2) to -1.0 (-1.2 to -0.8) cells. CASIA2 imaging had higher sensitivity of 0.92 (95% CI 0.78 to 0.97) vs HS1 imaging 0.17 (95% CI 0.07 to 0.34), with positive correlation between clinical grade and CASIA2 cell count (coefficient 12.8, p=0.02, 95% CI 2.2 to 23.4). Change in clinical grade at follow-up examinations correlated with change in image based 'cell' count (r2=0.79, p<0.001). Patients reported a potential positive impact of seeing their disease activity. CONCLUSION: Our findings suggest that OCT-based imaging holds the promise of deeper understanding of disease, improved patient experience and more granular monitoring of activity with resultant improved outcomes, but further work is needed to refine acquisition and analysis protocols.

Hypotony in uveitis: an overview of medical and surgical management.

Hypotony is a well-recognised, sight-threatening complication of uveitis. It can also be the final common endpoint for a multitude of disease entities. Multiple mechanisms underlie hypotony, and meticulous clinical history alongside ocular phenotyping is necessary for choosing the best intervention and therapeutic management. In this narrative review, a comprehensive overview of medical and surgical treatment options for the management of non-surgically induced hypotony is provided. Management of ocular hypotony relies on the knowledge of the aetiology and mechanisms involved. An understanding of disease trajectory is vital to properly educate patients. Both anatomical and functional outcomes depend on the underlying pathophysiology and choice of treatment.

Long-term ocular and visual outcomes following symptomatic and asymptomatic congenital CMV infection: a systematic review protocol.

INTRODUCTION: Cytomegalovirus (CMV) is one of the most common congenitally acquired infections worldwide. Visual impairment is a common outcome for symptomatic infants, with long-term ophthalmic surveillance often recommended. However, there are no clear guidelines for ophthalmic surveillance in infants with asymptomatic disease. We aim to conduct a systematic review to establish the overall prevalence and incidence of eye and vision related disorders following congenital CMV infection (cCMV). METHODS AND ANALYSIS: A systematic review and meta-analysis (pending appropriate data for analysis) of cross-sectional and longitudinal studies will be conducted. The PubMed, Embase and CINAHL databases will be searched up to 29 March 2022 without date or language restrictions. Studies will be screened by at least two independent reviewers. Methodological quality of included studies will be assessed using the Joanna Briggs Institute tool. The primary outcome measures will be incidence and/or prevalence of vision impairment or ophthalmic disorders in patients with symptomatic and asymptomatic cCMV infection. A narrative synthesis will be conducted for all included studies. The overall prevalence will be estimated by pooling data using a random-effects model. Heterogeneity between studies will be estimated using Cochran's Q and the I2 statistics. Egger's test will be used to assess for publication bias. ETHICS AND DISSEMINATION: Ethical approval is not required as there is no primary data collection. Study findings will be disseminated at scientific meetings and through publication in peer-reviewed journals. TRIAL REGISTRATION NUMBER: This is not a clinical trial, but the protocol has been registered: CRD42021284678 (PROSPERO).

BTS clinical statement for the diagnosis and management of ocular tuberculosis.

The BTS clinical statement for the diagnosis and management of ocular tuberculosis (TB) draws on the expertise of both TB and and ophthalmic specialists to outline the current understanding of disease pathogenesis, diagnosis and management in adults. Published literature lacks high-quality evidence to inform clinical practice and there is also a paucity of data from animal models to elucidate mechanisms of disease. However, in order to improve and standardise patient care, this statement provides consensus points with the currently available data and agreed best practice.

Ocular Tuberculosis: More than 'Of Mice and Men'.

Tuberculosis (TB), caused by infection with members of the Mycobacterium tuberculosis-complex, is one of the oldest known infectious disease entities, resulting in the death of millions of humans each year. It also results in a substantial degree of morbidity and mortality in animal species. Extrapulmonary TB is well recognized in humans, and the eye is one site that can be affected. Studies seeking to understand ocular TB have often relied on animal models; however, these have their limitations and may not truly reflect what happens in humans. We wish to raise awareness among ophthalmologists and vision scientists of naturally occurring cases of ocular TB in animals, namely cattle and domestic cats, and the possibilities of gaining further understanding of this presentation of TB by adopting a collaborative approach. This will hopefully improve outcomes for both human and animal patients.