A Systematic Review of the Quality of Clinical Practice Guidelines for Chronic Limb-Threatening Ischemia.

BACKGROUND: To assess the quality of clinical practice guidelines (CPGs) for chronic limb-threatening ischemia (CLTI) using the Appraisal of Guidelines for Research and Evaluation II instrument. METHODS: A systematic review of Medline, Embase, and online CPG databases was carried out. Four CPGs on CLTI were identified: Global Vascular Guidelines (GVG), European Society of Cardiology (ESC), American College of Cardiology, and National Institute for Health and Care Excellence guidelines on lower limb peripheral arterial disease. Two independent appraisers analyzed the 4 CPGs using the Appraisal of Guidelines for Research and Evaluation II instrument. CPGs were ranked across 6 domains with 23 items that ranged from 1 (strongly disagree) to 7 (strongly agree). A scaled domain score was calculated as a percentage of the maximum possible score achievable. A domain score of ≥50% and an overall average domain score of ≥80% reflected a CPG of adequate quality recommended for use. RESULTS: GVG had the highest overall score (82.9%), as an average of all domains, and ESC had the lowest score (50.2%). GVG and National Institute for Health and Care Excellence guidelines had all domains scoring >50%, while American College of Cardiology had 5 and ESC had 3. Two domains, rigor of development and applicability, scored the lowest among the CPGs. There was a lack of detail in describing systematic methods used in the literature review, how guidelines were formulated with minimal bias, and the planned procedure for updating the guidelines. Implications of guideline application and monitoring of outcomes after implementations were not explicitly discussed. CONCLUSIONS: The GVG guideline published in 2019 discussing CLTI is assessed to be of high quality and recommended for use. This review helps to improve clinical decision-making and quality of future CPGs for CLTI.

Measuring Quality of Life in Chronic Limb-threatening Ischemia Patients and Informal Carers: A Scoping Review.

OBJECTIVE: To review quality of life (QOL) instruments for chronic limb-threatening ischemia (CLTI) patients and informal carers, and their use in QOL and cost-utility analysis (CUA) studies. BACKGROUND: CLTI is a global health problem with significant morbidity affecting patients and informal carers. QOL is increasingly measured for holistic outcomes assessment and CUA. However, measurement instruments in CLTI are poorly understood. METHODS: MEDLINE, EMBASE, PsycINFO, CINAHL, COSMIN, PROQOLID, CEA registry, and NHS EED databases were searched for all English language studies up to May 2021. Features of instruments, evidence of measurement property appraisal, and trends in use were assessed. Prospective protocol registration (Open Science Framework: https://doi.org/10.17605/OSF.IO/KNG9U ). RESULTS: A total of 146 studies on QOL instruments (n=43), QOL outcomes (n=97), and CUA (n=9) were included. Four disease-specific QOL instruments are available for lower extremity arterial disease (intermittent claudication or CLTI). VascuQoL-25 and VascuQoL-6 have been used in CLTI. There is no CLTI-specific instrument. Of 14 generic instruments, SF-36, EQ-5D-3L, NHP, and WHOQOL-BREF were most common. Studies reporting partial measurement property appraisal favored VascuQoL-25, VascuQoL-6, and SF-36. Feasibility considerations include mode of administration and responder burden. None of 4 available carer-specific instruments have been used in CLTI. Since 1992, the number of QOL studies has increased considerably, but CUA studies are scarce. Informal carers have not been assessed. CONCLUSIONS: This review provides a comprehensive reference for QOL measurement in CLTI that helps end-users with instrument selection, use, and interpretation. However, a CLTI-specific instrument is needed. There is an opportunity to benefit society through future CUA studies and evaluation of QOL in informal carers.

Quality of Life in Chronic Limb Threatening Ischaemia: Systematic Review and Meta-Analysis.

OBJECTIVE: To assess the comparative effectiveness and temporal changes in quality of life (QoL) outcomes after revascularisation, major lower extremity amputation (MLEA), and conservative management (CM) in chronic limb threatening ischaemia (CLTI). DATA SOURCES: MEDLINE, Embase, PsycINFO, CINAHL, and Web of Science. REVIEW METHODS: A systematic review and meta-analysis were performed on QoL measured by any QoL instrument in adult patients with CLTI after open surgery (OS), endovascular intervention (EVI), MLEA, or CM. Randomised controlled trials and prospective observational studies published in any language between 1 January 1990 and 21 May 2021 were included. There was a pre-specified measurement time point of six months. Random effects meta-analysis was conducted on total scores for each QoL instrument. Certainty of evidence was assessed using the Grading of Recommendations, Assessment, Development and Evaluations approach (PROSPERO registration: CRD42021253953). RESULTS: Fifty-five studies with 8 909 patients were included. There was significant heterogeneity in the methods used to measure QoL, and the study characteristics. In particular, 14 different QoL instruments were used with various combinations of disease specific and generic instruments within each study. A narrative summary is therefore presented. Comparative effectiveness data showed there was reasonable certainty that QoL was similar between OS and EVI at six months. Temporal outcomes suggested small to moderate improvements in QOL six months after OS and EVI compared with baseline. Limited data indicated that QoL can be maintained or slightly improved after MLEA or CM. Treatment effects were overestimated owing to small study effects, selective non-reporting, attrition, and survivorship bias. CONCLUSION: QoL after OS and EVI appears to be similar. Revascularisation may provide modest QoL benefits, while MLEA or CM can maintain QoL. However, certainty of evidence is generally low or very low, and interpretation is hampered by significant heterogeneity. There is a need for a CLTI specific QoL instrument and methodological standardisation in QoL studies.

A Systematic Review of Cost-Utility Analyses in Chronic Limb-Threatening Ischemia.

BACKGROUND: To review and describe the available literature on cost-utility analysis of revascularization and non-revascularization treatment approaches in chronic limb-threatening ischemia. METHODS: A systematic review was performed on cost-utility analysis studies evaluating revascularization (open surgery or endovascular), major lower extremity amputation, or conservative management in adult chronic limb-threatening ischemia patients. Six bibliographic databases and online registries were searched for English language articles up to August 2021. The outcome for cost-utility analysis was quality-adjusted in life years. Procedures were compared using incremental cost-effectiveness ratios which were converted to 2021 United States dollars. Study reporting quality was assessed using the 2022 Consolidated Health Economic Evaluation Reporting Standards statement. The study was registered in International Prospective Register of Systematic Reviews (CRD42021273602). RESULTS: Three trial-based and five model-based studies were included for review. Studies met between 14/28 and 20/28 criteria of the Consolidated Health Economic Evaluation Reporting Standards CHEERS statement. Only one study was written according to standardized reporting guidelines. Most studies evaluated infrainguinal disease, and adopted a health care provider perspective. There was a large variation in the incremental cost-effectiveness ratios presented across studies. Open surgical revascularization (incremental cost-effectiveness ratios: $3,678, $58,828, and $72,937), endovascular revascularization (incremental cost-effectiveness ratios: $52,036, $125,329, and $149,123), and mixed open or endovascular revascularization (incremental cost-effectiveness ratio: $8,094) maybe more cost-effective than conservative management. CONCLUSIONS: The application of cost-utility analyses in chronic limb-threatening ischemia is in its infancy. Revascularization in infrainguinal disease may be favored over major lower extremity amputation or conservative management. However, data is inadequate to support recommendations for a specific treatment. This review identifies short and long-term considerations to address the current state of evidence. Cost-utility analysis is an important tool in healthcare policy and should be encouraged amongst the vascular surgical community.

A Review of Illness Perceptions in Chronic Limb-Threatening Ischemia: Current Knowledge Gaps and a Framework for Future Studies.

BACKGROUND: The aim of this study is to review illness perceptions (IPs) in chronic limb-threatening ischemia (CLTI) patients undergoing revascularization (open surgical or endovascular), major lower extremity amputation, or conservative management. METHODS: MEDLINE, EMBASE, PsycINFO, CINAHL, WOS, and Scopus databases were searched from inception to August 20, 2021 for studies evaluating IP in CLTI according to Leventhal's common sense model (CSM). Since only 1 study was identified, a post hoc secondary literature search of MEDLINE was performed for reviews of IP in cardiovascular disease and diabetes to identify potential learning points for future research. All studies underwent narrative synthesis guided by tabulated data. RESULTS: One study and 7 reviews were included from the primary and secondary literature searches, respectively. Timeline and controllability were the main aspects of IP that predict prosthetic use in CLTI patients, more so at 6 months than 1 month. Other reviews in cardiovascular disease and diabetes identified important targets for future research: (1) factors that affect IP and whether IP can be used as an outcome measure, (2) relationship between IP and clinician-reported and patient-reported outcomes, and (3) methods to educate and change maladaptive IP. The importance of using valid and reliable measures of IP that encompass all components of Leventhal's' CSM was stressed. CONCLUSIONS: Knowledge of IP in CLTI patients is severely limited in contrast to other fields in cardiovascular disease and diabetes. This review helps to close this gap by raising awareness of IP and its importance within the vascular surgical community, and by providing a framework for future studies.

PERIPHERAL RETINAL VASCULITIS: Analysis of 110 Consecutive Cases and a Contemporary Reappraisal of Tubercular Etiology.

PURPOSE: Describe the clinical features and outcomes of patients with peripheral retinal vasculitis (RV) and describe clinical characteristics of presumed tubercular RV in a nonendemic setting. METHODS: Retrospective cohort study of 110 consecutive patients with peripheral RV at a tertiary referral eye care center in the United Kingdom. Retinal vasculitis was defined as RV with vitritis associated with peripheral retinal ischemia. Patients who also had positive Quantiferon Gold in Tube test, positive tuberculin skin test, and/or other evidence of systemic tuberculosis such as biopsy were labeled with presumed tubercular RV. Treatment success was defined as resolution of inflammation, and successful tapering of oral corticosteroids to less than 10 mg/day or topical steroids to less than twice a day. RESULTS: Mean age of the study population was 42.69 ± 14.95 years. Patients were predominantly Asian (49.1%) and Male (67.0%). A total of 73.2% had bilateral involvement. Sixty-nine (62.72%) patients had presumed tubercular RV. A total of 52.8% patients received antitubercular therapy, 65.5% received oral corticosteroids, and 17.3% required steroid-sparing oral immunosuppressive agents. A total of 85.19% of patients with presumed tubercular RV achieved treatment success with concurrent antitubercular therapy as against 75.61% of patients with nontubercular RV. CONCLUSION: This is the largest study of the epidemiology, clinical features, and outcomes of both peripheral RV and presumed tubercular RV to date. Presumed tubercular RV commonly seems to affect young males of Asian descent and had vitreous hemorrhage as common clinical findings and also demonstrated a good treatment outcome with antitubercular therapy.

What does IGRA testing add to the diagnosis of ocular tuberculosis? A Bayesian latent class analysis.

BACKGROUND: To evaluate the contribution made to the diagnostic work-up for patients with suspected ocular tuberculosis (TB) by QuantiFERON-TB Gold In-Tube (QFT) tests using latent class analysis model. METHODS: A single centre retrospective cohort study. A Bayesian latent class model was constructed on the basis of demographics, phenotypes and test results from patients attending a tertiary referral center in the UK. This estimated the probability of ocular TB for each patient in two versions, first with and then without QFT. The estimated probability of ocular TB was compared with treatment failure. RESULTS: From a database of 365 patients with clinical signs suggestive of ocular TB, 267 patients who had QFT and complete data were evaluated. Mean age was 45.0 ± 15.4 years with 141 (52.9%) male and 148 (50.5%) of Asian ethnicity. QFT was positive in 208 (70.1%) patients and ATT was instituted in 145 (49.5%) patients with 100 (34.1%) patients also having concurrent systemic corticosteroid therapy. The best estimate of a QFT level separating TB-positive and TB-negative patients was extremely low. This weak discrimination between TB and non-TB groups was reflected in poor positive and negative predictive values for treatment failure. CONCLUSIONS: The latent class model did not successfully predict treatment failure, despite taking all variables into account. The threshold between TB and non-TB in QFT values was implausibly low and removing QFT from the model made prediction slightly worse. A larger prospective study is required to establish the role of all tests, demographics and phenotypes in diagnosis.

Retinal vessel caliber changes in vasculitis.

BACKGROUND: Retinal vasculitis is a potentially sight-threatening inflammation of the retinal vessels, but little is known about the in vivo vascular changes, which occur in affected eyes. The authors therefore sought to measure vessel caliber in eyes with vasculitis. METHODS: Retrospective case-control study. Vasculitis was confirmed using fluorescein angiography. Vessel calibers were measured using validated semiautomated software. RESULTS: There were 21 eyes from 15 patients with vasculitis and 33 control eyes from 21 control subjects. Most cases were diagnosed with idiopathic vasculitis. All had periphlebitis, and one eye also had arteritis. After adjustment for age and gender, mean arteriolar caliber was 143 µm (95% confidence interval [CI], 134-152) in cases and 158 µm (95% CI, 151-165) in controls (P = 0.01). Venular caliber was similar in cases (229 µm; 95% CI, 215-243) and controls (228 µm; 95% CI, 217-234; P = 0.91), whereas arteriole-to-venule ratio was smaller in cases (0.63; 95% CI, 0.60-0.66) compared with controls (0.70; 95% CI, 0.02-0.11; P = 0.004). CONCLUSION: Retinal vasculitis was associated with narrower arteriolar caliber, whereas venular caliber was similar to controls. This resulted in a smaller arteriole-to-venule ratio in eyes with vasculitis.

Characterization of punctate inner choroidopathy using enhanced depth imaging optical coherence tomography.

PURPOSE: To perform qualitative and quantitative analyses of retinal and choroidal morphology in patients with punctate inner choroidopathy (PIC) using enhanced depth imaging optical coherence tomography (EDI-OCT). DESIGN: Cross-sectional, consecutive series. PARTICIPANTS: A total of 2242 patients attending 2 tertiary referral uveitis clinics at Moorfields Eye Hospital were screened; 46 patients with PIC diagnosis were identified, and 35 eyes (35 patients) had clinically inactive PIC had EDI-OCT images that met the inclusion criteria. METHODS: Punctate inner choroidopathy lesions were qualitatively assessed for retinal features, such as (1) focal elevation of the retinal pigment epithelium (RPE), (2) focal atrophy of the outer retina/RPE, and (3) presence of sub-RPE hyperreflective deposits and choroidal features: (a) presence of focal hyperreflective dots in the inner choroid and (b) focal thinning of the choroid adjacent to PIC lesions. Quantitative analyses of the retina, choroid, and choroidal sublayers were performed, and associations with clinical and demographic data were examined. MAIN OUTCOME MEASURES: Prevalence of each lesion pattern and thickness of retinal and choroidal layers. RESULTS: A total of 90 discrete PIC lesions were captured; 46.6% of PIC lesions consisted of focal atrophy of the outer retina and RPE; 34.4% consisted of sub-RPE hyperreflective deposits; and 18.8% consisted of localized RPE elevation with underlying hyporeflective space. Focal hyperreflective dots were seen in the inner choroid of 68.5% of patients, with 17.1% of eyes presenting focal choroidal thinning underlying PIC lesions. By excluding high myopes, patients with "atypical" PIC had reduced retinal thickness compared with patients with "typical" PIC (246.65±30.2 vs. 270.05±24.6 µm; P = 0.04), and greater disease duration was associated with decreases in retinal thickness (r = -0.53; P = 0.01). A significant correlation was observed between best-corrected visual acuity and foveal retinal thickness (r = -0.40; P = 0.03). CONCLUSIONS: In a large series of patients with clinically inactive PIC, one fifth of the lesions analyzed revealed RPE elevation with underlying hyporeflective space, described before as a sign of activity and suggesting subclinical inflammation. Retinal thickness seems to be associated with disease type and duration of disease in non-highly myopic eyes. Improved visualization of the inner choroid using EDI-OCT may allow noninvasive assessment of inflammatory status.

Patient perspectives of quality of life in chronic limb-threatening ischemia: a qualitative study.

BACKGROUND: Quality of life (QOL) is an outcome that matters to patients with chronic limb-threatening ischemia (CLTI). However, we identified the lack of and need for a CLTI-specific QOL instrument. Our group is developing this instrument which requires a deep understanding of patient perspectives of QOL in CLTI. METHODS: Qualitative inquiry with patient and public involvement was performed in accordance with the Standards for Reporting Qualitative Research. Reflexive thematic analysis of semi-structured interviews was conducted within a constructivist-interpretivist research paradigm. Data were organized and managed in NVivo. Techniques to enhance trustworthiness included maintaining an audit trail, member checking, mentoring, and peer-debriefing. Patient and the public were consulted for feedback on codes, themes, and thematic maps. RESULTS: Thirteen participants (median age: 74 years, range: 43-90 years) with a variety of patient demographics were interviewed. Four themes were developed on QOL in CLTI: (i) 'independence as key to life satisfaction', (ii) 'change in identity when continuity is needed', (iii) 'coping with intractable disease', and (iv) 'not wanting to be alone'. Member checking with patient and public involvement confirmed the relevance and centrality of these themes to the lived experiences of patients with CLTI. CONCLUSIONS: The thematic outputs contribute important insights into what QOL truly means to patients with CLTI and what matters for their QOL. The content validity of the new CLTI-specific QOL instrument is improved by giving patients voice. This study highlights the value of qualitative inquiry and patient and public involvement in vascular surgical research.

Mimickers of anterior uveitis, scleritis and misdiagnoses- tips and tricks for the cornea specialist.

BACKGROUND: Anterior uveitis, inflammation of the anterior chamber and related structures, is a cohort of diseases that can present to almost any general or sub-specialty Ophthalmology practice. Its features classically involve anterior chamber cell and flare. Below the surface of these two signs exist a panoply of diagnoses. BODY: The purpose of this review is to provide a general framework for diagnoses of anterior uveitis that are often missed as well as non-uveitic pathologies that often mimic anterior uveitis. Diagnostic deviation in either direction can have vision-threatening and rarely life-threatening consequences for patients. Using a comprehensive literature review we have collected a broad spectrum of etiologies of anterior uveitis that are easily missed and non-uveitic pathologies that can masquerade as anterior uveitis. CONCLUSIONS: We present a focused review on specific misdiagnosed anterior uveitis pathologies and some of the conditions that can masquerade as anterior uveitis and scleritis.

The Infectious Uveitis Treatment Algorithm Network (TITAN) Report 1-global current practice patterns for the management of Herpes Simplex Virus and Varicella Zoster Virus anterior uveitis.

AIMS: To present current expert practice patterns and to formulate a consensus for the management of HSV and VZV AU by uveitis specialists worldwide. METHODS: A two-round online modified Delphi survey with masking of the study team was conducted. Responses were collected from 76 international uveitis experts from 21 countries. Current practices in the diagnosis and treatment of HSV and VZV AU were identified. A working group (The Infectious Uveitis Treatment Algorithm Network [TITAN]) developed data into consensus guidelines. Consensus is defined as a particular response towards a specific question meeting ≥75% of agreement or IQR ≤ 1 when a Likert scale is used. RESULTS: Unilaterality, increased intraocular pressure (IOP), decreased corneal sensation and diffuse or sectoral iris atrophy are quite specific for HSV or VZV AU from consensus opinion. Sectoral iris atrophy is characteristic of HSV AU. Treatment initiation is highly variable, but most experts preferred valacyclovir owing to simpler dosing. Topical corticosteroids and beta-blockers should be used if necessary. Resolution of inflammation and normalisation of IOP are clinical endpoints. CONCLUSIONS: Consensus was reached on several aspects of diagnosis, choice of initial treatment, and treatment endpoints for HSV and VZV AU. Treatment duration and management of recurrences varied between experts.

The Infectious Uveitis Treatment Algorithm Network (TITAN) Report 2-global current practice patterns for the management of Cytomegalovirus anterior uveitis.

AIMS: To present current practice patterns in the diagnosis and management of Cytomegalovirus anterior uveitis (CMV AU) by uveitis experts worldwide. METHODS: A two-round modified Delphi survey with masking of the study team was performed. Based on experience and expertise, 100 international uveitis specialists from 21 countries were invited to participate in the survey. Variation in the diagnostic approaches and preferred management of CMV AU was captured using an online survey platform. RESULTS: Seventy-five experts completed both surveys. Fifty-five of the 75 experts (73.3%) would always perform diagnostic aqueous tap in suspected CMV AU cases. Consensus was achieved for starting topical antiviral treatment (85% of experts). About half of the experts (48%) would only commence systemic antiviral treatment for severe, prolonged, or atypical presentation. The preferred specific route was ganciclovir gel 0.15% for topical treatment (selected by 70% of experts) and oral valganciclovir for systemic treatment (78% of experts). The majority of experts (77%) would commence treatment with topical corticosteroid four times daily for one to two weeks along with antiviral coverage, with subsequent adjustment depending on the clinical response. Prednisolone acetate 1% was the drug of choice (opted by 70% of experts). Long-term maintenance treatment (up to 12 months) can be considered for chronic course of inflammation (88% of experts) and those with at least 2 episodes of CMV AU within a year (75-88% of experts). CONCLUSIONS: Preferred management practices for CMV AU vary widely. Further research is necessary to refine diagnosis and management and provide higher-level evidence.

Choroidal assessment in idiopathic panuveitis using optical coherence tomography.

BACKGROUND: Idiopathic panuveitis is a diagnosis of exclusion that lacks distinguishing features on fluorescein and indocyanine green angiography. Choroidal hypoperfusion or ischaemia has been implicated in panuveitis of different aetiologies. In this study, we use enhanced depth imaging optical coherence tomography (OCT) to examine the choroid and its vasculature in patients with this disease. METHODS: In this retrospective, cross-sectional study, OCT-derived measurements of retinal and choroidal thickness were obtained after manual segmentation using custom software. Choroidal measurements were further subdivided into Haller's large vessel layer (HLVL) and Sattler's medium vessel layer (SMVL), and correlated with clinical parameters. RESULTS: Twenty-one eyes from 21 patients were included. A reduction in hypo-reflective spaces, corresponding to vascular lumens, was observed in HLVL. The mean thickness of both the choroid (233.7 ± 73.3 µm), and HLVL (167.8 ± 53.7 µm), was less than that previously reported for normal eyes. Choroidal thickness expressed as a ratio to retina thickness showed significant correlation to visual acuity (r = 0.58, p = 0.006). This correlation was maintained in the ratio between HLVL and retinal thickness (r = 0.56, p = 0.009), but not in SMVL to retinal thickness (r = 0.352, p = 0.12). CONCLUSIONS: This study reports novel OCT-derived parameters in patients with idiopathic panuveitis. We noted loss of hyporeflectivity in HLVL, and thinning of both HLVL and the choroid as a whole. The observed correlation between visual acuity and the ratio of choroidal to retinal thickness is a strong enhanced depth imaging (EDI)-OCT derived candidate for prospective validation in future studies.

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.

Choroidal Involvement in a Case of Viral Retinitis.

PURPOSE: To describe a challenging case of acute retinal necrosis. METHODS: Clinical data, including medical history, clinical findings and imaging features were retrospectively collected in a 28-year-old female with unilateral active retinitis. RESULTS: The yellowish area of retinitis surrounding a pigmented chorioretinal scar and the full thickness retinal hyper-reflectivity associated with diffuse increase in choroidal demonstrated a challenging case of necrotising retinitis, where baseline clinical and tomographic features were atypical and misleading towards a toxoplasmic aetiology. The detection of virus genome in ocular samples was necessary to achieve a correct diagnosis of acute retinal necrosis. CONCLUSION: Although rare, choroidal involvement may occur in acute retinal necrosis along with the well-known retinal features.

Case of Amaurosis Fugax in the Setting of a Persistent Primitive Hypoglossal Artery Requiring Carotid Endarterectomy with Regional Anesthesia.

BACKGROUND A persistent primitive hypoglossal artery (PPHA) is a rare congenital anomaly leading to persistent carotid-basilar anastomosis. This is a report of an 83-year-old man with a PPHA presenting with amaurosis fugax of the left eye requiring carotid endarterectomy under regional anesthesia. CASE REPORT An 83-year-old man presented with 2 weeks of intermittent self-resolving visual disturbances, followed by an episode of left eye amaurosis fugax. The patient had been referred to the hospital for further investigation of symptoms 1 day following the amaurosis fugax event. Carotid Doppler ultrasound demonstrated a greater than 90% stenosis of the left internal carotid artery. Computed tomography carotid and Circle of Willis angiography confirmed a mixed, ulcerated plaque and revealed a persistent left hypoglossal artery originating from the left internal carotid artery and continuing as the basilar artery. On day 3 of admission, left carotid endarterectomy was performed under conscious sedation and regional anesthesia to permit continuous monitoring of neurological status and avoid the need for intraoperative shunting. "Permissive hypertension" by targeting a systolic blood pressure of 190 to 200 mmHg was sought for the duration of clamp time. There was no deterioration of neurological function during clamping of the carotid vessels. The patient recovered well and was discharged 2 days after surgery, with no residual neurology. CONCLUSIONS This report has presented a rare case of PPHA to highlight awareness of this congenital vascular anomaly when undertaking carotid endarterectomy.

Immunosuppressive therapy for Vogt-Koyanagi-Harada disease: a retrospective study and review of literature.

BACKGROUND: Vogt-Koyanagi-Harada (VKH) disease is an idiopathic autoimmune disease which targets melanin-containing tissues such as the uvea, meninges, ear and skin. This typically presents in the eye with acute findings of granulomatous anterior uveitis, diffuse choroidal thickening, multiple focal areas of sub-retinal fluid and, in severe cases, optic nerve involvement with bullous serous retinal detachment can occur. Early initiation of treatment has been advocated to prevent progression to the chronic stage of the disease, which can result to a sunset glow fundus with devastatingly poor visual outcome. Treatment is usually initiated with corticosteroids followed by an early introduction of immunosuppressive treatment (IMT) to achieve immediate response after disease presentation, although the choice of IMT for VKH can vary. MAIN FINDINGS: We conducted a retrospective case-series to investigate the management trend of treating VKH over a 20-year period. Twenty-six patients were included and we found a shift from steroid monotherapy to combined IMT/low-dose steroid for the management of acute initial-onset of VKH in the last 10 years. Our average time from diagnosis to initiation of IMT was 2.1 months. 81% (21 of 26 patients) of our patients treated with combined IMT/steroid were able to achieve disease stability with significant good visual outcome at 24 months (Median VApre-IMT = 0.3 Logmar vs VApost-IMT = 0.0 Logmar, p = 0.0001). MMF monotherapy was the most common IMT used and it was well-tolerated by our patients. Even so, 50% of our patients who were treated with MMF did not achieve disease control. We then performed a literature review to identify any IMT which could be superior in the treatment of VKH. We also share our experience (where applicable) on the various treatment options found from the literature review. SHORT CONCLUSION: Our study found that patients with VKH who were treated with combined IMT/low-dose steroids achieved significantly better visual improvement at 24 months compared to steroid monotherapy. We frequently chose MMF and this appears to be well tolerated by our patients. Since its introduction, anti-TNF agents are increasingly becoming a popular choice of treatment for VKH as these have been shown to be safe and effective. However, more data is required to provide evidence that anti-TNF agents can be used as first-line treatment and as monotherapy.

Predictive Factors for Magnetic Resonance Imaging Changes Suggestive of Demyelination in Adult Patients with Uveitis Scanned Prior to Commencing Adalimumab Therapy.

PURPOSE: To report the predictive clinical factors for abnormal magnetic resonance imaging (MRI) scans suggestive of demyelination by analysis of MRI's performed for adult non-infectious uveitic patients prior to commencing adalimumab therapy. METHODS: Retrospective case review of 240 patients was conducted in a single tertiary institution between November 2017 and March 2020. Aetiology of underlying disease, clinical characteristics, and MRI outcomes were analysed. RESULTS: The presence of bilateral idiopathic intermediate uveitis (IIU) (p = .0048) and neurological symptoms (p = .028) were highly predictive of an abnormal MRI strongly suggestive of demyelination (MRSSD); 5 out of 64 scans (7.8%) with these clinical characteristics had MRSSD. CONCLUSIONS: Tumor necrosis factor antagonist-induced demyelination is a concern in adalimumab use. We propose an MRI screening protocol to identify those at high risk of demyelination; positive results can be maximised by screening all patients with IIU and those with neurological symptoms.

Demystifying Ocular Syphilis - A Major Review.

Syphilis, caused by the spirochaete, Treponema pallidum, continues to be a public health challenge globally with its rates steadily increasing in the past few years. The disease is transmitted through small breaks in the skin during sexual contact, or via congenital transmission in utero, either across the placenta or by contact with an active genital lesion during delivery. Estimated 5.7-6 million new cases are detected every year worldwide in the 15-49 years age group. An increased incidence has been reported in most populations with particular clusters in special groups like men who have sex with men, female sex workers, and their male clients. Ocular syphilis has a varied presentation and is considered a great mimicker in all cases of uveitis. The laboratory diagnosis of syphilis is predominantly based on serological tests including TPHA and VDRL. Parenteral penicillin is the cornerstone of treatment for all stages of ocular syphilis.