Bristol Eye Hospital

Introduction:Cerebral visual impairment (CVI) is an umbrella term that describes a broad range of brain related visual problems and it is now the most common cause of visual impairment (VI) in children in the developed world. Early assessment, management and support are essential to minimise the risk of lifelong negative consequences. Currently there are no internationally agreed clinical guidelines for the investigation and diagnosis of CVI, with variation in existing models of care resulting in potential service health inequities for this population.Objective:The objective of this scoping review is to identify and describe the components of existing service delivery models of care in relation to the detection, care, and management of children with CVI.Methods:We will search multiple databases to include MEDLINE, CINAHL, Embase, APA PsycINFO, and The Cochrane Library for English language publications from OECD countries. Supplementary searches will also be conducted to locate grey literature. We will include any study that describes or assesses service delivery for children (0–17 years) with CVI in health and social care, and education settings.Stakeholder engagement:We consulted with a broad range of stakeholders including subject experts and patient and public representatives during the planning and will continue during the conduct, reporting and dissemination of this scoping review.

Abstract Thyroid eye disease (TED) is an autoimmune disorder of the orbit, usually occurring in association with autoimmune Graves’ disease. Both adaptive and innate immune systems are involved. The exact triggers for the disease remain elusive, but genetic susceptibility and environmental factors are both implicated in dysregulation of self-tolerance mechanisms. Humoral and cellular autoimmunity leads to activation of orbital fibroblasts, which are the main effector cells that produce the pathological manifestations of TED. At a molecular level, circulating antibodies against the thyroid stimulating hormone receptor bind to the receptor, triggering a signalling cascade in which co-signalling with the insulin-like growth factor 1 receptor also occurs. Improved understanding of these pathways is providing targets for biologic drugs against TED. The mechanisms by which the immune system changes translate into the clinical pathology of TED are complex. The anatomy of the orbit contributes to the pathophysiology, with the ‘cone theory’ of TED describing how distribution of mechanical pressure within the closed bony confines of the orbit is instrumental in the manifestation of the disease. Oxidative stress also plays a role. Improved understanding of the role of the microbiome in autoimmune disease may offer some explanation of why particular individuals with otherwise similar genetics and environmental exposures may or may not manifest TED, but research into this is in its early phases.

Abstract People affected by thyroid eye disease can suffer a significant negative psychosocial impact, which may be compounded by effects of associated Graves’ disease. TED can cause psychological distress via changes in appearance, changes in visual function, stress related to diagnostic delays and healthcare processes, treatment side effects, and general exhaustion related to chronic illness. This can lead to low mood and depression, reduced self-confidence and social engagement, and difficulties with employment and relationships. Healthcare professionals may be able to mitigate psychosocial impact to some extent by compassionate listening and provision of clear information, directing patients to relevant resources for self-help and peer-support, and onward referral where further professional help is needed. Effective treatment of TED can improve subjective functional and appearance related quality of life measures. Ability to work is commonly affected in moderate to severe TED, and provision of information for employers may help them to provide appropriate modifications in the workplace. Use of multidisciplinary TED clinics may help to reduce the number of working days lost to healthcare appointments and support patients in remaining in work. For patients experiencing ongoing psychological effects, particularly appearance related concerns, therapies based on cognitive behavioural techniques may be helpful, including self-directed and online programs which are cheap to deliver.

BackgroundJuvenile idiopathic arthritis (JIA)-associated uveitis and chronic anterior uveitis in children may result in permanent sight loss. Currently, the only licensed and approved treatment for JIA-uveitis is adalimumab. However, even in patients where adalimumab may be initially effective, therapeutic response may subside for example, due to neutralising drug antibodies. Further treatment options are necessary to prevent sight loss in children with uveitis. Interleukin 17 is elevated in uveitis. Inhibition of interleukin 17 ameliorates inflammation in mouse models of uveitis. Secukinumab, an antibody which neutralizes interleukin 17 A, has been shown to be partially effective in adult uveitis. The objective of the Bayesian consensus meeting was to quantify prior expert opinion about the potential utility of secukinumab in treatment of uveitis in JIA.MethodsNine international experts in paediatric rheumatology, paediatric ophthalmology and/or paediatric uveitis took part in a structured Bayesian prior elicitation meeting.ResultsThe final consensus was that adalimumab is expected to yield a higher response rate than secukinumab (mean 0.67 vs. 0.55). The uncertainty in the response rate on secukinumab is somewhat larger than for adalimumab. The equivalent sample size for the prior distribution of adalimumab is 15.7 and 13.1 for secukinumab. The decisions based on the combined evidence would still be driven by the trial data, yet substantial enhancement of the power of the study can be expected by adding information from the equivalent of almost 30 patients.ConclusionsThe Bayesian analysis adds substantial enhancement of the power of the study and supports a head-to-head trial of adalimumab and secukinumab for JIA-associated uveitis and chronic anterior uveitis.Trial registrationISRCTN 12,427,150 Registration date 14/02/2023. EudraCT 2022-003068-26 Registration date 07/09/2022.

PurposeTo investigate the impact of central serous chorioretinopathy on sleep and mood in patients with acute and chronic central serous chorioretinopathy.MethodsThis cross-sectional study compared sleep and mood differences between central serous chorioretinopathy and control patients recruited from Ophthalmology clinics at the John Radcliffe Hospital, Oxford between 2012 and 2020. Data including visual acuity, type of central serous chorioretinopathy (acute or chronic; aCSC/cCSC), sex, and chronotype were obtained. Sleep quality was measured using the Pittsburgh Sleep Quality Index (PSQI); the Hospital Anxiety and Depression Scale (HADS) was used to evaluate anxiety (HADS-A) and depression (HADS-D).ResultsA total of 247 age matched controls and 109 patients with central serous chorioretinopathy participated. There were no significant differences in PSQI or HADs (P > 0.05) between the two groups. Females exhibited significantly higher PSQI scores than males both for control and central serous chorioretinopathy groups (P < 0.05). Within the central serous chorioretinopathy group, 88 (81%) had chronic central serous chorioretinopathy and 21 (19%) had acute central serous chorioretinopathy, and an increase in daytime dysfunction was seen in the acute phenotype compared to chronic (P = 0.018).ConclusionIn our study, no significant differences in sleep quality or mood scores were identified in central serous chorioretinopathy patients, when compared to controls. Worsened sleep for females was present when compared to males, both in central serous chorioretinopathy and control groups. Within central serous chorioretinopathy groups, worsened daytime function was observed in acute versus chronic – a larger study would help distinguish the effect of chronicity on sleep.

IntroductionPapilloedema can be the first sign of life-threatening disease, for example, brain tumours. Due to the potential seriousness of this clinical sign, the detection of papilloedema would normally prompt urgent hospital referral for further investigation. The problem is that many benign structural variations of optic nerve anatomy can be mistaken for papilloedema, so-called pseudopapilloedema. The consequence is that many people are referred to hospital because they are incorrectly identified to have papilloedema when they don’t. As a result, hospital referrals of people with suspected papilloedema in England have increased sharply, leading to increased demand for overstretched hospital services and potentially longer waiting times for hospital appointments for those who do have papilloedema.The work programme is aimed at the development of guidelines and educational materials that will help support health professionals to correctly identify people with papilloedema. This article describes the protocol for gathering evidence of current referral practices and pathways for people suspected to have papilloedema in England and the development of guidelines based on this evidence and extensive engagement with community- and hospital-based healthcare professionals, patients, and the public.Methods and analysisBoth qualitative and quantitative data will be collected from Freedom of Information requests to Integrated Care Boards across England about how they organise their community and hospital services for people with suspected papilloedema, with and without headache. Surveys and qualitative interviews of relevant community and hospital healthcare professionals based in England will collect data on how and when people with papilloedema and pseudopapilloedema with or without headache are currently identified and referred to hospital, if needed. This information will be used to inform a Delphi process with the aim of reaching consensus among health professional experts, commissioners and patients on what the most evidence-based and safe diagnostic and referral practices should be for people with suspected papilloedema. The tailored guidelines will be written for healthcare professionals and patients. We will create a range of educational materials and a website designed for health professionals and patients to support the national roll-out and implementation of DIPP study guidelines.Ethics and disseminationEthical approval was granted by the University of Bristol Faculty of Health Sciences Ethics committee (FREC reference: 12457) and Health Research Authority (IRAS no.: 320395). Results of the study will be published on our DIPP study website and disseminated to our stakeholder groups through peer-reviewed journal publications and conference presentations.

We present a case of a macular horseshoe tear that demonstrated signs of progression over time that was therefore deemed sight threatening. There are no reports that provide a definitive means to treat such a rare pathology. Spectral domain optical coherence tomography (SD-OCT) scans were used to monitor subretinal fluid at each follow up. Once confirmation of the accumulation of subretinal fluid was made, vitrectomy surgery including amputation of the horseshoe tear flap, short acting gas tamponade and face down positioning were utilized. Macular horseshoe tears can be treated with a vitrectomy approach akin to retinal detachment repair without the need for retinopexy.

We report an atypical case of combined acute syphilitic necrotizing retinitis and a contiguous acute syphilitic posterior placoid chorioretinitis in an HIV-negative, immunocompetent patient. Observational case report. A 56-year-old man presented with a one-week history of pain and blurred vision in the left eye. He also complained of left-sided hearing loss for several months. Ocular examination demonstrated a unilateral panuveitis with a yellowish placoid macular lesion involving the outer retina contiguous with an ovoid area of full-thickness retinitis extending temporally. Vitreal polymerase chain reaction analysis for HSV, CMV, VZV, and T. gondii were negative, but syphilis serology was reported as positive. An MRI of the head revealed bilateral enhancement along the facial nerves, more marked on the left side, consistent with syphilitic involvement. He was treated for neurosyphilis with a 14-day course of systemic procaine penicillin and oral probenecid. Oral prednisolone (1 mg/kg/day) was commenced 24 hours before initiating antibiotics to prevent Jarisch-Herxheimer reaction and treat his panuveitis. To our knowledge, this is the first report of combined syphilitic necrotizing retinitis and acute syphilitic posterior placoid chorioretinitis occurring in continuity in the same eye. This case highlights the diversity of possible presentations of ocular syphilis, even in HIV-negative immunocompetent patients.

Background: Childhood uveitis is a rare inflammatory eye disease which is typically chronic, relapsing-remitting in nature, with an uncertain aetiology (idiopathic). Visual loss occurs due to structural damage caused by uncontrolled inflammation. Understanding of the determinants of long term outcome is lacking, including the predictors of therapeutic response or how to define disease control. Aims: To describe disease natural history and outcomes amongst a nationally representative group of children with non-infectious uveitis, describe the impact of disease course on quality of life for both child and family, and identify determinants of adverse visual, structural and developmental outcomes. Methods: UNICORNS is a prospective longitudinal multicentre cohort study of children newly diagnosed with uveitis about whom a core minimum clinical dataset will be collected systematically. Participants and their families will also complete patient-reported outcome measures annually from recruitment. The association of patient (child- and treatment- dependent) characteristics with outcome will be investigated using logistic and ordinal regression models which incorporate adjustment for within-child correspondence between eyes for those with bilateral disease and repeated outcomes measurement. Discussion: Through this population based, prospective longitudinal study of childhood uveitis, we will describe the characteristics of childhood onset disease. Early (1-2 years following diagnosis) outcomes will be described in the first instance, and through the creation of a national inception cohort, longer term studies will be enabled of outcome for affected children and families.