Diagnostic and therapeutic challenges in acute retinal necrosis; an update.

Acute retinal necrosis (ARN) is a rare but severe ophthalmic pathology defined by panuveitis, retinal necrosis, and high rates of retinal detachment. ARN may lead to poor visual outcomes even if promptly diagnosed and treated. ARN may present with a wide spectrum of clinical findings compatible with panuveitis including anterior uveitis, scleritis, vitritis, necrotizing retinitis, occlusive vasculitis, and optic disc edema. The American Uveitis Society introduced clinical criteria in 1994 for the diagnosis of ARN, while more recent criteria have been proposed by the Standardization of Uveitis Nomenclature (SUN) Working Group and the Japanese ARN Study Group. Multimodal imaging is a valuable tool in evaluating patients with ARN, particularly in unusual cases, while utilizing retinal imaging and applying AI algorithms in these areas of clinical research could be highly beneficial. Over the last few years, significant progress has been made in achieving timely diagnosis and treatment. The precise identification of the viral cause in suspected ARN cases has been greatly enhanced by the advancements in PCR techniques and flow cytometry used for intraocular fluids. systemic (intravenous or oral) antivirals with adjunctive intravitreal antiviral therapy are recommended as first-line therapy to reduce disease severity, the risk of vision loss, and retinal detachment incidence. Although aciclovir was the first existing antiviral agent, at present many clinicians prefer high-dose valaciclovir orally or intravenous aciclovir combined with intravitreal foscarnet. Despite significant progress in diagnosing and treating ARN, further research is needed to improve visual outcomes in this challenging clinical condition.

Diagnostic and therapeutic considerations in patients with bilateral diffuse uveal melanocytic proliferation.

PURPOSE: This review aims to summarize the current knowledge concerning the clinical features, diagnostic work-up, and therapeutic approach of bilateral diffuse uveal melanocytic proliferation (BDUMP). METHODS: A meticulous literature search was performed in the PubMed database. A supplementary search was made in Google Scholar to complete the collected items. Our search strategy utilized the following keywords: "bilateral diffuse uveal melanocytic proliferation", "BDUMP", and "Paraneoplastic Syndrome". Articles were considered based on their relevance, with the search spanning publications up to 2023. Studies were excluded if they did not contribute pertinent information or lacked methodological rigor. A critical appraisal of included studies was conducted, assessing study design, sample size, methodology, and potential bias, ensuring a thorough and transparent review process. RESULTS: BDUMP is a rare and potentially sight-threatening condition characterized by the bilateral proliferation of melanocytes within the uvea. BDUMP is typically observed in middle-aged or elderly individuals and is often associated with an underlying malignancy, most commonly of gastrointestinal origin. BDUMP is frequently misdiagnosed as a benign nevus or choroidal metastasis, leading to delayed diagnosis and treatment. The ophthalmic symptoms and signs typically precede the diagnosis of a systemic malignancy, emphasizing the crucial role of ophthalmologists in the recognition of BDUMP. Several diagnostic modalities can aid in the diagnosis of BDUMP, including ophthalmic examination, imaging studies such as optical coherence tomography, fluorescein angiography, and indocyanine green angiography, and biopsy of the uveal tissue. Treatment of BDUMP is directed towards the underlying malignancy and may include chemotherapy, radiotherapy, or surgical resection. Additionally, strict monitoring with regular follow-ups may contribute to the detection of new lesions and the reduction in the size of existing ones. CONCLUSIONS: BDUMP can be considered a potential biomarker in the management of malignancies, especially when the primary underlying tumor has not been detected. Further research is needed to better understand the pathogenesis of BDUMP and its association with malignancy.

Central retinal artery occlusion after cardioversion of atrial fibrillation.

Central retinal artery occlusion (CRAO) is a vascular ophthalmic emergency. Often caused by a sudden interruption of blood flow to the eye, with profound and painless vision loss, resulting in irreversible cell damage. An impacted embolus at the narrowest part of the central retinal artery is the most common cause. Cardioversion is a medical procedure used to restore a normal heart rhythm in individuals with atrial fibrillation (AF). In some cases, cardioversion can lead to thromboembolic complications. If an embolus reaches the central retinal artery, it can block the blood flow to the retina, resulting in CRAO and subsequent vision loss.

Asymptomatic retinal dysfunction in a patient with alpha-methylacyl-CoA racemase deficiency.

PURPOSE: To present a case of a young female patient with asymptomatic retinal dysfunction associated with alpha-methylacyl-CoA (AMACR) racemase deficiency. METHODS: Retrospective analysis of the medical notes of a single patient. Detailed slit-lamp examination was completed by Optos colour fundus photography and enhanced depth imaging optical coherence tomography (EDI-OCT). Genetic testing was conducted to establish the diagnosis, and the patient was also referred to the Department of Neurology for further assessment. RESULTS: Dilated fundoscopy and ophthalmic imaging revealed bilateral retinal pigment epithelium abnormalities that could be associated with a genetic retinal disorder. Indeed, genetic testing showed that this lady was homozygous for AMACR (OMIM 604489; Gene ID 23600) variant NM 014324.6: c.154T>C; p.(Ser52Pro). She had no detectable neurological deficit. CONCLUSION: AMACR deficiency is a rare genetic condition that can potentially contribute to retinal dystrophy through various mechanisms. Additionally, it may lead to a wide spectrum of systemic signs and symptoms. Interestingly, in contrast to other reported studies, our patient was completely asymptomatic, with no evidence of systemic disorders.

Punctate inner choroidopathy: A review of the current diagnostic and therapeutic approaches.

Punctate inner choroidopathy (PIC) is an uncommon idiopathic inflammatory condition characterized by multifocal chorioretinopathy that primarily affects young adults, with a predilection for myopic females. Clinically, it manifests as small, yellowish-white lesions in the inner choroid and outer retina, often associated with inflammatory changes. Accurate diagnosis remains a challenge due to its resemblance to other posterior uveitic entities, necessitating an astute clinical eye and advanced imaging techniques for differentiation. Multimodal imaging plays a crucial role by offering valuable insights, as it enables the visualization of various abnormalities related to uveitis. The pathogenesis of PIC is still a subject of debate, with a complex interplay of genetic, immunological, and environmental factors proposed. Managing PIC presents multiple challenges for clinicians. Firstly, variable disease severity within and among patients requires diverse treatments, from observation to aggressive immunosuppression and/or anti-VEGF therapy. Secondly, treatment must distinguish between primary causes of vision loss. New or worsening PIC lesions suggest active inflammation, while new neovascular membranes may indicate secondary neovascular processes. Thirdly, deciding on maintenance therapy is complex, balancing PIC prognosis variability against immunosuppression risks. Some patients have long periods of inactivity and remission, while others face sudden, vision-threatening episodes during quiescent phases. Through a systematic review of the literature, this paper sheds light on the current understanding of PIC, its challenges, and the prospects for future research. By synthesizing existing knowledge, it aims to aid clinicians in accurate diagnosis and guide treatment decisions for improved visual outcomes in individuals affected by PIC.

An Unusual Manifestation of Brucella-Associated Uveitis in a Young Male Patient.

This is a unique case of retinoschisis as an ocular manifestation of brucellosis. A 38-year-old male presented with recurrent episodes of bilateral eye redness, predominately in his left eye. His visual acuity was not affected, and he did not report any other symptoms. On slit lamp examination, binocular Koeppe nodules of the iris and cells in the left anterior chamber were observed. Fundoscopy followed by meticulous multimodal imaging confirmed left inferior retinoschisis. The patient was diagnosed with panuveitis, and a series of laboratory examinations revealed positive anti-IgM Brucella antibodies. Ocular brucellosis can cause variable, atypical, and serious presentations, hence, early diagnosis is paramount to avoid complications.

Cytokines in Immune-mediated "Non-infectious" Uveitis.

Uveitis is a significant cause of ocular morbidity and accounts for approximately 5 - 10% of visual impairments worldwide, particularly among the working-age population. Infections are the cause of ~ 50% cases of uveitis, but it has been suggested that infection might also be implicated in the pathogenesis of immune-mediated "non-infectious" uveitis. There is growing evidence that cytokines (i.e., interleukins, interferons, etc.) are key mediators of immune-mediated "non-infectious" uveitis. For example, activation of the interleukin-23/interleukin-17 signalling pathway is involved in immune-mediated "non-infectious" uveitis. Studies in animal models have been important in investigating the role of cytokines in uveitis. Recent studies of clinical samples from patients with uveitis have allowed the measurement of a considerable array of cytokines even from very small sample volumes (e.g., aqueous and vitreous humour). The identification of complex patterns of cytokines may contribute to a better understanding of their potential pathogenetic role in uveitis as well as to an improved diagnostic and therapeutic approach to treat these potentially blinding pathologies. This review provides further insights into the putative pathobiological role of cytokines in immune-mediated "non-infectious" uveitis.

The large Hellenic Study of Uveitis: epidemiology, etiologic factors and classification.

PURPOSE: To analyse the demography, etiology, and classification of uveitis at a tertiary academic referral center. METHODS: An observational study was conducted on the archives of uveitic patients at the Ocular Inflammation Service of the Department of Ophthalmology at the University Hospital of Ioannina (Greece) from 1991 to 2020. This study aimed to investigate the epidemiological profile of patients, including their demographics and the main etiologic factors of uveitis. RESULTS: Out of 6191 cases with uveitis, 1925 were infectious, 4125 were non-infectious, and an overall of 141 masquerade syndromes were recorded. Among these cases, 5950 patients were adults, with a slight female predominance, while 241 were children (< 18 years old). Interestingly, 24.2% of cases (1500 patients) were associated with 4 specific microorganisms. Herpetic uveitis (HSV-1 and VZV/HZV) was the most common cause of infectious uveitis (14.87%), followed by toxoplasmosis (6.6%) and tuberculosis (2.74%). In 49.2% of non-infectious uveitis cases, no systematic correlation was found. The most frequent causes of non-infectious uveitis included sarcoidosis, white dot syndromes, ankylosing spondylitis, lens-induced uveitis, Adamantiades-Behçet disease, and idiopathic juvenile arthritis. Infectious uveitis was more common in the rural population, whereas non-infectious uveitis was more frequently recorded in the urban population CONCLUSIONS: Although our study was conducted on a predominantly white Caucasian population, it also reflects the effect of increasing immigration, improvements of diagnostic techniques, changes in referral patterns, and various actual changes in disease incidence.

Central Serous Chorioretinopathy: An Update on the Current State of Management.

Central serous chorioretinopathy (CSCR) is a relatively common retinal disorder that leads to central vision impairment, often with a high recurrence rate. The exact etiology and pathogenetic mechanisms have not been fully elucidated but are likely to be associated with hyperpermeability of the choroidal capillaries and failure of the retinal pigment epithelium (RPE), leading to serous detachment of the neurosensory retina. Multimodal imaging plays a critical role in the diagnostic approach and monitoring of CSCR. Fortunately, the natural course of the disease is usually self-limiting, with spontaneous resolution and total fluid reabsorption. However, some patients may exhibit recurrences or persistent subretinal fluid (chronic CSCR), leading to progressive and irreversible RPE atrophy or photoreceptor damage. Thus, to prevent permanent visual loss, individualized treatment should be considered. Recent developments in the diagnostic and therapeutic approach have contributed to better outcomes in patients with CSCR. More studies are required to improve our understanding of epidemiology, pathogenesis, diagnosis, and treatment, with a significant impact on the management of this challenging clinical entity. The purpose of this review is to summarize the current knowledge about the clinical features, diagnostic workup, and therapeutic approach of CSCR.

Unexplained visual loss in retinal detachment repair: comparing gas, silicone oil and heavy silicone oil by multivariable regression.

PURPOSE: To measure the proportion of unexplained and all causes of visual loss following primary rhegmatogenous-retinal-detachment (RRD) repair, comparing gas tamponade (SF6, C2F6, C3F8), silicone oil (SO, 1000cs and 5000cs) and heavy silicone oil (Densiron). METHODS: Retrospective, continuous, comparative study from 01/1/2017-31/5/2021. All primary RRDs were included after successful removal of SO and Densiron. Primary failures were excluded. Visual loss was defined as reduction of ≥0.30 logMAR units. Multivariable binary-logistic and linear regression models to compare tamponade, and all cases of unexplained visual loss and logMAR gain were performed. Covariates included age, ocular co-morbidities, pre-op vision, macula-status, high-myopia, giant-retinal-tear (GRT), perfluorocarbon-use, combined buckle/PPV, PVR-C, retinectomy, tamponade agent and post-operative lens status. RESULTS: Of 1,012 primary RRDs, we found an incidence of unexplained visual loss in 15/1012 (1.5%, SF6:1/341[0.3%], C2F6:4/338[1.2%], C3F8:2/239[0.8%], Densiron:0/33[0.0%], SO-1000cs:5/43[11.6%] and SO-5000cs:3/18[16.7%]), and visual loss of all causes in 57/1012 (5.6%, SF6:13/341[3.8%], C2F6:14/338[4.1%], C3F8:15/239[6.3%], Densiron:2/33[6.1%], SO-1000cs:9/43[20.9%] and SO-5000cs:4/18[22.2%]). On multivariable binary-logistic regression, we report that macula-on RRD (Odds-Ratio[OR]5.7,95% Confidence-interval[CI]1.2-28.2, p=0.032), GRT (OR35.0,CI 2.0-617.3, p=0.015), combined buckle/PPV (OR37.7,CI 2.0-711.4, p=0.015), SO1000cs (OR86.6,CI 5.6-1,348.0), p=0.001) and 5000cs (OR37.2,CI 1.3-1,101.5, p=0.036) (Reference-tamponade:SF6) were associated with unexplained visual loss. Duration of oil tamponade was not linked to increase in unexplained visual loss (p=0.569). CONCLUSIONS: Correlation between SO in detachment repairs and unexplained visual loss has been established, however incidence with HSO has not been compared to other agents. This study demonstrates that although SO was linked with risk-adjusted increased unexplained visual loss relative to gas tamponade, no such association was found for Densiron, on multivariable analysis.

The Large Hellenic Study of Uveitis: Diagnostic and Therapeutic Algorithms, Complications, and Final Outcome.

PURPOSE: The purpose of this study is to present the diagnostic and therapeutic algorithms, complications, and final outcome in the management of uveitic patients at a tertiary academic referral center. DESIGN: Observational study. METHODS: Analysis of the archives of 6191 uveitic patients at the Ocular Inflammation Service of the Department of Ophthalmology of the University Hospital of Ioannina in Greece from 1991 to 2020. RESULTS: During the 30 years of the study, the diagnostic ability climbed from 45.43% (1991-1995) to 73.4% (2016-2020). This improvement was linked to several factors including the increase in the number of diagnostic paracenteses for the analysis of intraocular fluids, the range and quality of laboratory blood tests, the multimodal ophthalmic imaging, the proper use of nonophthalmic imaging, and the multidisciplinary approach. The degree of uveitis-related complications was related to the severity and cause of inflammation, the recurrence rate, inappropriate treatment, and the prolonged or initially inactive inflammation. The 3 most common complications included cataract, macular edema, and glaucoma. Apart from the modern treatments and surgical techniques, the 3-month preoperative control of inflammation played a critical role in the surgical outcomes. The percentage of patients with a successful outcome increased from 72% (2001-2005) to 90.50% (2016-2020). The center's experience, prompt referral, patient's compliance, and regular follow-ups are associated with a better outcome. The analysis of the results allowed the development of diagnostic and therapeutic algorithms. CONCLUSIONS: Developing diagnostic and therapeutic algorithms allows for the efficient management of uveitis, leading to better visual outcome and therefore a better quality of life.

Neovascular Glaucoma: An Update.

Neovascular glaucoma (NVG) is a severe type of secondary glaucoma with devastating complications and generally poor visual prognosis. NVG is defined by the development of pathological neovessels over the iris and the iridocorneal angle that can block the outflow of aqueous humor, causing elevation of intraocular pressure (IOP). The pathogenesis of NVG is, in most cases, associated with ischemia of the posterior segment, which is most frequently associated with proliferative diabetic retinopathy or central retinal vein occlusion. The advanced stages of NVG are by iris and angle neovascularization, angle, and extremely high IOP, accompanied by ocular pain and poor vision. The therapeutic approach of NVG is based on the reduction of retinal ischemia by panretinal photocoagulation. Intravitreal anti-VEGF administration can contribute to the regression of neovascularization, and topical and systemic medications may be necessary for IOP control. However, if medical treatment with these agents is not enough, surgical procedures may be required to lower IOP and prevent glaucomatous optic neuropathy. Early and prompt diagnosis, with identification of the underlying etiology, can improve IOP control and final visual outcome. The aim of this study is to review current knowledge of the pathogenesis and management of NVG.

Experience gained during vitreoretinal fellowships in the United Kingdom.

BACKGROUND: There are 16 vitreoretinal (VR) fellowships listed on the British and Eire Association of Vitreoretinal Surgeons (BEAVRS) website offering places to 23 applicants, however, this list is not exhaustive. The purpose of this survey was to evaluate surgical volume, training, and experience of VR fellows in the UK. METHODS: An anonymous survey was disseminated online to current and past VR fellows who are members of BEAVRS. Participants were asked about their surgical experience and confidence, before and during their fellowship, in performing a variety of procedures. Participants were also asked about their academic achievements and their career prospects. RESULTS: All 26 respondents felt that their fellowship met their surgical needs and would recommend it to others. Upon completion, 92% felt prepared to work as a consultant. Following fellowship completion, the median (IQR) number of procedures performed were: phacoemulsification: 91 (51-131), pars-plana vitrectomy (PPV): 351 (226-451), simple-retinal detachment (RD): 176 (126-226), complex-RD: 31 (16-51), scleral buckle (SB): 16 (80-26), membrane-peels: 76 (41-88), intraocular-foreign body (IOFB) removal: 3 (3-3), indirect laser: 51 (11-91), scleral-fixated intraocular-lens (sfIOL): 3 (3-8), removal-of-dropped-nucleus (RODN): 16 (8-26), diabetic membrane delaminations: 16 (8-16); with an increase of confidence in performing all VR procedures (p < 0.001). Participants completed 2 (1-2) presentations and 2 (0-3) papers with no difference in academic performance between those with/without postgraduate qualifications (p = 0.409). CONCLUSIONS: Overall, fellowships in the UK are of a high quality and prepare the fellow adequately for progression into a consultant post. They help increase surgical confidence and provide opportunities to complete academic work. Fellowships without VR on-call commitments can be improved by incorporating on-call duties. Finally, COVID-19 impacted exposure to elective cases.

"Comparing outcomes of advanced nurse practitioners to ophthalmologists performing posterior YAG capsulotomy, a six-year study of 6308 eyes".

PURPOSE: To primarily report the baseline characteristics and visual acuity (VA) outcomes of advanced nurse practitioners (ANP) compared to ophthalmologists following YAG posterior capsulotomy (YAGPC). We secondarily looked to characterise the risk factors that lead to a repeated YAGPC. METHOD: Retrospective consecutive case series of 6,308 eyes attending the Birmingham and Midlands Eye Centre. RESULTS: ANPs performed 33.1% of YAGPC compared to 66.9% ophthalmologists. Compared to ophthalmologists, ANPs performed YAGPC in lower proportion of patients with ocular co-morbidities (p < 0.001) and had lower proportion of patients requiring further YAGPC compared to ophthalmologists (p < 0.001). Median pre, post-operative and LogMAR gain in VA of 0.48 (IQR 0.30-0.78), 0.18 (IQR 0.10-0.40) and 0.30 (0.08-0.48) LogMAR units, respectively. Multivariate regression showed that ANPs had a significantly lower rate of repeat YAGPC compared to ophthalmologists even when adjusting for age, ethnicity, training grade and ocular co-morbidities. No difference in visual outcomes was found between operator grade and ethnicity on multivariate analysis. CONCLUSION: YAGPC leads to excellent visual outcomes. ANPs can deliver safe and effective YAGPC.

Comparing the outcomes of YAG laser anterior capsulotomies performed by an advanced nurse practitioner to ophthalmologists in the management of anterior capsular contraction syndrome.

BACKGROUND: Anterior capsular contraction syndrome (ACCS) describes the progressive fibrotic phimosis of the anterior capsular bag that usually occurs a few months after cataract surgery. YAG laser anterior capsulotomy (YAGAC) is the most common treatment option due to the low-risk profile of this intervention. PURPOSE: In this series, we evaluated the outcomes of an advanced nurse practitioner (ANP) in conducting this laser intervention, comparing the results with those of ophthalmologists. METHODOLOGY: Our study represents a single-centre, retrospective, continuous case series of 108 eyes that underwent YAGAC due to ACCS between January 2017 and July 2020 at the Birmingham and Midland Eye Centre, the second largest tertiary referral centre in the United Kingdom. RESULTS: The groups treated by ANPs and ophthalmologists were similar in respect to age, gender, and laterality of the laser procedure. Eyes treated by ophthalmologists had significantly more ocular comorbidities (p < .001), the most common of which was glaucoma. Although the complication rate was higher in the ophthalmologist group, it did not reach statistical significance. However, there was a trend toward significance in the retreatment rate, with 8.6% (7/81) of eyes lasered by ophthalmologists requiring further YAGAC, and no repeat procedure was needed in the ANP group. CONCLUSIONS: YAGAC leads to good visual outcomes and a low complication rate in both ophthalmologist and ANP groups. IMPLICATIONS: Advanced nurse practitioners can deliver results that are comparable with those of experienced ophthalmologists.

A novel case of Raoultella planticola - associated acute dacryocystitis.

This study aims to report an acute dacryocystitis associated with Raoultella planticola infection. The patient's medical records and laboratory were thoroughly assessed. A thorough literature search was performed in PubMed database. An additional search was made in Google Scholar to complete the collected items. This is the first recorded case of acute dacryocystitis attributed to Raoultella planticola infection. A limited number of studies has related this pathogen with conjunctivitis. Acute dacryocystitis on rare occasions can also be caused by atypical organisms. Swabs must be obtained and patients must be started on empirical treatment while waiting for the laboratory results.

Ocular toxoplasmosis: a review of the current diagnostic and therapeutic approaches.

PURPOSE: This review aims to summarize the current knowledge concerning the clinical features, diagnostic work-up and therapeutic approach of ocular toxoplasmosis focusing mainly on the postnatally acquired form of the disease. METHODS: A meticulous literature search was performed in the PubMed database. A supplementary search was made in Google Scholar to complete the collected items. RESULTS: Ocular toxoplasmosis is one of the most frequent infectious etiologies of posterior uveitis. It typically presents with retinochoroiditis. Setting an accurate diagnosis depends to a considerable degree on detecting characteristic clinical characteristics. In addition to the evaluation of clinical features, the diagnosis of toxoplasmosis relies at a large degree on serologic testing. The detection of the parasite DNA in the aqueous or vitreous humor can provide evidence for a definitive diagnosis. The current mainstay for the treatment, if necessary, is the use of oral antibiotic with systemic corticosteroids. Recent evidence suggests other therapeutic approaches, such as intravitreal antibiotics can be used. CONCLUSION: Recent developments in the diagnostic and therapeutic approach have contributed to preventing or limiting vision loss of patients suffering from ocular toxoplasmosis. Further studies are required to provide a better understanding of epidemiology, pathogenesis, diagnosis, and treatment with a significant impact on the management of this challenging clinical entity.