Diagnostic work-up of retinal vasculitis: An algorithmic approach.

Retinal vasculitis has heterogenous etiologies encompassing infections, autoimmune, masquerades and idiopathic. The heterogeneity in the underlying clinical features and aetiologies of retinal vasculitis makes its diagnosis challenging for clinicians and the work up thus becomes quite extensive and many a times several unwarranted investigations are done to look for a possible etiology. Timely and accurate diagnosis is crucial for effective management and vision preservation. The algorithmic approach needs understanding of the phenotype, clinical as well as imaging biomarkers so that only customized investigations are done in order to make the timely diagnosis and initiate specific therapy wherever required. In this review article, we shall present an algorithmic approach that combines clinical assessment, ophthalmic imaging, laboratory investigations, and targeted ancillary tests.

An Update on Noninfectious Retinal Vasculitis.

BACKGROUND: Retinal vasculitis (RV) signifies the inflammation of various retinal vessels. Noninfectious RV differs from infectious RV with regard to its pathogenesis and treatment. It can have varied clinical presentations and may be associated with systemic vasculitic diseases. SUMMARY: Noninfectious RV can be caused due to type-III hypersensitivity reactions, increased expression of intracellular adhesion molecules, and genetic susceptibility. Noninfectious RV is primarily classified on the basis of the type of retinal vessels involved. It can be further classified as an occlusive or nonocclusive. RV can be a major association of systemic diseases like Behcet's disease, sarcoidosis and systemic lupus erythematosus. Newer modalities, like ultra-widefield fundus fluorescein angiography, can help in the management of RV. Effective treatment of noninfectious RV requires anti-inflammatory and immunosuppressive therapy. The patients may require treatment with high-dose corticosteroids and biological agents. Anti-vascular endothelial growth factor injections and laser photocoagulation may be indicated to treat the occlusive disease. Prompt treatment may prevent complications like vitreous hemorrhage, neovascular glaucoma, and tractional retinal detachment. The treatment more often requires a multidisciplinary approach. KEY MESSAGES: This review provides a comprehensive update on the various causes of noninfectious RV, including both systemic and isolated ocular conditions. It also details various complications and management strategies for this condition.

Intermediate Uveitis with Cystoid Macular Edema as Presentation of Retained Caterpillar Hair in the Vitreous Cavity.

PURPOSE: To report an unusual case of retained caterpillar hair in the vitreous cavity presenting as recurrent Intermediate Uveitis with cystoid macular edema. METHOD: Case Report. RESULTS: A 40-year-old male presented to our uveitis clinic with recurrent episodes of redness and diminution of vision in his left eye for 3 years. He was diagnosed and treated elsewhere as a case of recurrent intermediate uveitis and was referred to our center for a second opinion to initiate immunosuppressive therapy. A detailed history revealed that a caterpillar had fallen into his left eye 3 years back, followed by severe irritation, pain, and redness. He received topical antibiotics and corticosteroids, and retained caterpillar hair was removed from the ocular surface. During the last 3 years, the patient had multiple episodes of inflammation despite being treated with oral and topical corticosteroids. At the current visit, a meticulous clinical examination at our centre revealed multiple, migrated, retained caterpillar hair in the vitreous cavity. The patient was diagnosed with Ophthalmia Nodosa (Type V) and managed with surgical intervention (vitreous surgery). The case highlights an unusual presentation of ON and the role of pars plana vitrectomy in its successful management. CONCLUSION: We highlight a rare and unusual presentation of ophthalmia nodosa and its successful management.

Outcomes after giant peripheral retinotomy and anterior flap retinectomy for rhegmatogenous retinal detachments with advanced proliferative vitreoretinopathy using small gauge vitrectomy.

PURPOSE: To analyze the visual and anatomical outcomes for eyes with rhegmatogenous retinal detachment (RRD) and advanced proliferative vitreoretinopathy (PVR) undergoing giant peripheral retinotomy (GPR) using 25-gauge pars plana vitrectomy (PPV). METHODS: In this retrospective multi-center study, patients with RRD with either anteroposterior or circumferential retinal shortening and advanced PVR requiring more than 90-degree GPR with/without relaxing retinotomy were included. Subjects of either gender, any age group, and with complete surgical notes were included. Outcome measures of the study included anatomical success (i.e. complete retinal re-attachment) at 6 months using survival analysis, visual outcomes, and post-operative complications. RESULTS: Forty-one eyes of 41 patients (33 males) with a mean age of 44.9 ± 21.4 years were included. At 6 months follow-up, anatomical success was seen in 29 eyes (70.7%) with a cumulative re-attachment rate of 66% (95% confidence interval = 48 = 79%). All re-detachments occurred at ≤6 months with a peak at 4-6 months (n = 9). Twenty-three eyes (56%) achieved ambulatory vision (5/200) or better. Direct perfluorocarbon liquid-silicone oil exchange was performed in 20 eyes. Intra-operative complications included persistent retinal folds (2 eyes), subretinal air (1 eye), and subretinal bleed (1 eye). Eleven eyes (26.8%) developed secondary glaucoma (2 eyes required a drainage device), and hypotony of ≤6 mmHg was noted in 3 eyes (7.3%). Corneal decompensation was noted in 8 eyes (19.5%), and 3 eyes (7.3%) underwent re-surgery for re-RRD. CONCLUSION: After GPR using small gauge PPV, two-thirds achieve anatomical success, and over half have ambulatory vision, but overall post-operative complications can occur in more than half of the eyes.

Central Retinal Artery Occlusion With Cilioretinal Artery Sparing.

This case report describes a diagnosis of central retinal artery occlusion in a patient in their early 30s who presented with decreased vision in the left eye for 1 week.

Update on coronavirus disease 2019: Ophthalmic Manifestations and Adverse Reactions to Vaccination.

The coronavirus disease 2019 (COVID-19) pandemic caused by the severe acute respiratory syndrome coronavirus 2 was one of the most devastating public health issues in recent decades. The ophthalmology community is as concerned about the COVID-19 pandemic as the global public health community is, as COVID-19 was recognized to affect multiple organs in the human body, including the eyes, early in the course of the outbreak. Ophthalmic manifestations of COVID-19 are highly variable and could range from mild ocular surface abnormalities to potentially sight and life-threatening orbital and neuro-ophthalmic diseases. Furthermore, ophthalmic manifestations may also be the presenting or the only findings in COVID-19 infections. Meanwhile, global vaccination campaigns to attain herd immunity in different populations are the major strategy to mitigate the pandemic. As novel vaccinations against COVID-19 emerged, so were reports on adverse ophthalmic reactions potentially related to such. As the world enters a post-pandemic state where COVID-19 continues to exist and evolve as an endemic globally, the ophthalmology community ought to be aware of and keep abreast of the latest knowledge of ophthalmic associations with COVID-19 and its vaccinations. This review is a summary of the latest literature on the ophthalmic manifestations of COVID-19 and the adverse ophthalmic reactions related to its vaccinations.

Intravitreal immunotherapy in non-infectious uveitis: an update.

INTRODUCTION: In the past several years, there have been numerous advances in pharmacotherapeutics for the management of uveitis and other ocular inflammatory diseases, including newer therapeutic agents and ocular drug delivery systems. One of the most attractive modes of drug delivery is the intravitreal route since it has proven to be safe and efficacious and prevents unwanted systemic adverse events related to the agent. AREAS COVERED: In this review, intravitreal delivery of various pharmacotherapeutic agents for noninfectious uveitis has been described. An extensive review of the literature was performed using specific keywords on the PubMed database to identify clinical studies employing various pharmacotherapeutic agents with intravitreal drug delivery for noninfectious uveitis. The mode of action, safety, efficacy, and tolerability of these drugs have also been elucidated. EXPERT OPINION: Several agents, including biologic response modifier agents, have been found to be safe and efficacious for various indications of uveitis, such as cystoid macular edema, active uveitis, and other conditions such as retinal vasculitis and vitreous haze. The use of intravitreal biological therapies, especially infliximab, has been fraught with potential safety signals such as photoreceptor toxicity. However, pharmacotherapeutic agents such as corticosteroids and anti-vascular endothelial growth factor agents are now widely used in the clinical management of uveitis and its complications.

Retrolental Bullous Retinal Detachment as the Presenting Feature of Vogt-Koyanagi-Harada Disease in an Elderly Indian Female: Diagnostic and Therapeutic Challenge.

PURPOSE: To report an atypical case of Vogt-Koyanagi-Harada (VKH) disease with retrolental bullous retinal detachment (RD). METHODS: A case report. CASE: A 67-year-old Indian woman with bilateral, gradual visual loss presented with both eyes light perception, keratic precipitates, 2+ cells and bullous RD, which was retrolental in right eye (RE). Systemic investigations were unremarkable. She received systemic corticosteroids, and underwent pars plana vitrectomy (PPV) in left eye (LE). Intraoperatively, sunset glow with leopard-spot fundus were suggestive of VKH disease. Immunosuppressive therapy was added. At 2 years, vision was 3/60 in RE and 6/36 in LE. The LE retina reattached immediately following surgery, while RE exudative RD resolved very gradually following corticosteroids. SUMMARY: This report illustrates diagnostic as well as therapeutic challenge in VKH disease presenting with retrolental bullous RD. PPV provided a faster anatomical and functional restoration than systemic corticosteroid therapy alone, which has potential adverse effects in the elderly.

Choriocapillaris Flow Deficit in Tubercular Serpiginous-Like Choroiditis with and without Paradoxical Worsening.

PURPOSE: The aim is to analyze automated quantification of choriocapillaris flow deficit (CCFD) on swept-source (SS)-optical coherence tomography angiography (OCTA) in tubercular serpiginous-like choroiditis (TBSLC). METHODS: In this prospective study, automated CCFD calculations were performed on SS-OCTA and compared with CCFD areas on indocyanine green angiography (ICGA). Patients were divided into two groups based on the occurrence of paradoxical worsening (PW). RESULTS: Twenty-nine eyes (29 subjects; 18 males; mean age: 33±12 years) were included. The mean CCFD at baseline was 34.9 ± 4.3% on OCTA in eyes without PW and 35.4 ± 5.0% on SS-OCTA with PW (p = .77). At 4 and 12 weeks, CCFD on SS-OCTA improved to 30.6 ± 3.9% and 28.0 ± 4.2% (p < .001) without PW, respectively, and increased to 42.9 ± 4.4% and 48.8 ± 4.1% (p < .001) with PW, respectively. The SS-OCTA CCFD correlated well with ICGA (r = 0.48; p < .001). CONCLUSIONS: Automated quantitative serial assessment of CCFD on SS-OCTA can serve as a useful biomarker of disease activity in eyes with TBSLC.

Blau syndrome: Lessons learned in a tertiary care centre at Chandigarh, North India.

Objectives: Blau syndrome (BS) is a rare autoinflammatory disease characterized by arthritis, dermatitis, and granulomatous uveitis in early childhood. The study presents the clinical experience of patients with BS at a tertiary care centre in Chandigarh, North India. Methods: Analysis of the clinical profile of patients of BS with NOD2 gene mutations under follow-up was carried out. Results: Diagnosis of BS was genetically confirmed in 11 patients (10 children and one adult; six male and five female patients) from 10 families. The median age of onset of symptoms was 12 months (range, 4 months-4 years), while the age at diagnosis ranged from 2.3 to 26 years. The classic triad of arthritis, dermatitis, and uveitis was present in 6/11 (54.5%) patients. The frequency of arthritis, dermatitis, and uveitis was 100%, 81.8%, and 72.7%, respectively. The median age at diagnosis of ocular symptoms was 4 years (range, 2-26 years). Family history was noted in six families. Renal involvement was observed in two children. All patients in our cohort had the R334W variant in NOD2 gene. An asymptomatic carrier sibling with R334W mutation was identified in one family. Methotrexate was used as a first-line agent in all children. Adalimumab, which was commenced in five patients with uveitis, resulted in significant improvement in four patients. The total follow-up duration of the present cohort is 1,063.8 patient-months. Conclusions: The possibility of BS should always be considered in patients with arthritis and early ocular involvement. Uveitis is often progressive and refractory to currently available therapies. Systemic involvement appears to remain a significant cause of morbidity and mortality.

Nontubercular Mycobacteria Associated Uveitis in HIV Positive Patients.

PURPOSE: To report clinical features and outcomes of nontuberculous mycobacteria (NTM) presenting as uveitis in HIV positive patients. MATERIALS AND METHODS: Retrospective study of HIV positive patients who were diagnosed as uveitis due to NTM. RESULTS: Six eyes of four HIV positive patients with NTM were studied. Average age at presentation was 35.5 years (range 30-38). With specific PCR primers, Mycobacterium avium was detected in three patients (75%) and Mycobacterium fortuitum in one patient (25%). Culture was positive in two cases. Two eyes (33.33%) each had endophthalmitis and necrotizing retinitis like picture, one eye (16.66%) each had chorioretinitis and frosted branch angitis like. Visual acuity improved in two eyes (33.33%), worsened in three eyes (50%), and remained unchanged in one eye (16.6%). CONCLUSIONS: NTM infection is a unique entity in immunosuppressed with poor visual outcome. PCR forms a useful tool for rapid diagnosis and timely initiation of specific anti-tuberculosis therapy.

Recent advances in wide field and ultrawide field optical coherence tomography angiography in retinochoroidal pathologies.

Introduction: With the advent of wide field (WF) and ultra-wide field (UWF) imaging in color fundus photography and fluorescein angiography (FA), the importance of an expanded field-of-view (FOV) has been illustrated for early detection of several chorioretinal disorders. The need to bring similar utility to optical coherence tomography angiography (OCTA) is imperative.Areas covered: For this review, we explored all research items with WF or UWF on Pubmed Central and Google Scholar. Software-based methods included algorithms for motion correction, artifact reduction, image pre-processing, post-processing and montage protocols while hardware methods included swept-source OCTA, faster scanning sources, hardware-based motion tracking and image registration, scanning sources with faster speeds and lower resolution. For various disorders such as diabetic retinopathy, uveitis, vascular disorders, among others, the increased FOV showed demonstrably increased detection rates and diagnostic yield.Expert opinion: Increased FOV in OCTA imaging is a meaningful and impactful step in ophthalmic imaging. Overcoming the hardware-based limitations can potentially yield higher FOV without loss of important details. In addition to improvements in the hardware and available equipment, better software tools and image processing algorithms may greatly enhance the utility of UWF OCTA in care of patients with various retinochoroidal diseases.

Ocular syphilis in patients with human immunodeficiency virus/acquired immunodeficiency syndrome in the era of highly active antiretroviral therapy.

PURPOSE: Re-emergent ocular syphilis in patients with Human immunodeficiency virus (HIV) co-infection has both diagnostic and management difficulties because of the overlapping risk factors. The clinical manifestations described in non-HIV may not be the same in patients with HIV coinfection. Immune recovery uveitis (IRU) may also alter the course of the disease causing recurrences. We studied the clinical features in correlation with CD4 counts, systemic immune status, sexual preferences and management outcomes in HIV/AIDS patients with ocular syphilis in the highly active antiretroviral treatment (HAART) era from a high endemic HIV population like India. METHODS: Retrospective analysis of all patients with ocular syphilis and HIV/AIDS seen between 2016 and 2019 was done. RESULTS: A total of 33 patients (56 eyes) with a CD4 count range of 42-612 cells/cu.mm were included. Ocular syphilis was found to be higher in individuals with high risk behavior such as men who have sex with men (MSMs) (45%). Panuveitis was the commonest manifestation (53.57%) and was even the presenting feature of HIV and syphilis in many patients. Significant vitritis, usually uncommon in HIV/AIDS immunocompromised patients was noted even with low CD4 counts in patients with ocular syphilis. Significant correlation was noted between ocular presentation and CD4 counts (P < 0.05). CONCLUSION: Ocular syphilis presents differently in patients with HIV/AIDS. Diffuse retinitis is seen commonly in low counts (<100 cells/cu.mm). Classical placoid chorioretinitis lesions usually described in non-HIV individuals is uncommon in HIV patients and is seen in higher CD4 counts ( >400 cells/cu.mm). Ocular manifestations can be an indicator of the immune status of the patient. Not all patients with ocular manifestations have associated features of systemic syphilis. Ocular manifestations can be the first presentation of HIV/AIDS. Although, there is good response to systemic penicillin and HAART, recurrences and immune recovery uveitis (IRU) can also occur.

Posterior hypopyon in fungal endogenous endophthalmitis secondary to presumably contaminated dextrose infusion.

PURPOSE: To describe the presentation, clinical course and management of a patient with posterior hypopyon secondary to atypical (fungal) endogenous endophthalmitis. OBSERVATIONS: A 55-year-old Asian Indian female presented with decreased vision in the left eye (OS). The best-corrected visual acuity was 20/20 in the right eye (OD) and counting fingers (CF) in the left eye (OS) at the time of initial presentation. Slit-lamp examination revealed 1+ cells and 1+ flare in the anterior chamber of OS. Clinical examination and imaging assessment with fundus photography revealed vitritis, a posterior hypopyon and retinal exudates. The patient had an episode of fever one month before presentation for which an intravenous dextrose infusion was administered. After carefully evaluating the patient, ocular images, detailed history and necessary laboratory tests, a working diagnosis of endogenous endophthalmitis was reached. Empirical treatment with topical and systemic antibiotics, and topical cycloplegics and steroids was initiated. Therapeutic and diagnostic pars plana vitrectomy (PPV) was subsequently performed; microbiology and cytology analyses revealed evidence of fungal elements. Therefore, systemic anti-fungal treatment was initiated; the patient demonstrated significant clinical improvement with good visual outcome. CONCLUSION AND IMPORTANCE: Posterior hypopyon in endophthalmitis is a rarely observed entity and is typically obscured due to dense vitritis. Such clinical manifestation may suggest a possible infectious etiology as described in this case.

Familial Nanophthalmos Presenting with Spontaneous Uveal Effusion Syndrome.

Purpose: To report two unique cases of spontaneous uveal effusion syndrome (UES) from the same family.Methods: The patients included father (54 years old) and a daughter (23 years old). The father presented 7 years ago with bilateral exudative retinal detachment, nanophthalmic eyes (axial length 16.11 mm and 16.43 mm in right and left eyes, respectively) with hypermetropia, and a thickened sclera on MRI. The daughter presented with left eye peripheral exudative annular retinal detachment with bilateral nanophthalmos (axial length 20.7 mm and 20.33 mm, in right and left eyes, respectively), with MRI showing ciliochoroidal detachment, suprachoroidal effusion and mild thickening of the sclera in left eye.Results: The father underwent subscleral sclerectomy in the left eye that led to resolution of submacular fluid. Scleral biopsy confirmed the deposition of glycosaminoglycans on Alcian blue stain. The daughter was kept under observation, because of absence of any macular subretinal fluid, and counselled about the familial nature of the disease and possible need for future intervention.Conclusion: This is the first report of familial nanophthalmos presenting with spontaneous UES.

Optic Disc Neovascularization in Tubercular Serpiginous-Like Choroiditis.

Optic disc neovascularization in uveitis is uncommon, and has been associated with chronic inflammations like Behcet's disease, Vogt-Koyanagi-Harada disease, sarcoidosis, etc. To our knowledge, optic disc neovascularization has not been reported in tubercular serpiginous-like choroiditis. We report three such cases.

Fungal retinitis following influenza virus type A (H1N1) infection.

A 43-year-old male presented with left eye foveal retinits causing an acute visual loss following influenza virus type A infection (H1N1 infection or Swine flu). Considering viral (influenza) etiology, a prompt treatment with oral corticosteroids was started. But an initial poor response prompted an immediate diagnostic vitrectomy, which revealed Candida albicans. The retinitis healed with scar formation following anti-fungal therapy. This case highlights that even in the setting of an acute retinitis in an immunocompetent patient with recent history of viral systemic illness, a high index of suspicion of a fungal (rather than viral) infection should be kept in mind.

Clinical profile of uveitis patients developing central serous chorioretinopathy: An experience at a tertiary eye care center in India.

PURPOSE: To evaluate clinical profile of patients with uveitis who developed central serous chorioretinopathy (CSC). METHODS: Retrospective case series of consecutive patients of uveitis with CSC managed at a tertiary eye care center in India between 1994 and 2014. The data about clinical features, investigations, treatment, and outcomes were obtained from their medical records. RESULTS: A total of 31 eyes of 26 patients with uveitis with a diagnosis of CSC between June 1994 and May 2014 were included in the study. The mean age of presentation was 42.8 ± 9.2 years, and 88.4% of the patients were male. CSC was bilateral in 19.2% of the patients, and in 38.4% patients uveitis was because of infectious etiology. CSC developed in 23 (88.5%) patients when they were on oral corticosteroid. The most common cause of uveitis in our study was choroiditis (48.4%), followed by retinal vasculitis (12.9%). The mean time for resolution of CSC was relatively less in patients with uveitis because of infectious etiology. In 10% eyes vision remained the same and deterioration of vision was noted in 19% eyes. Best corrected visual acuity of the patients at the time of presentation with CSC was 0.56 ± 0.34 and after the resolution of CSC was 0.48 ± 0.5 (P < 0.0005). CONCLUSION: Patients with choroidal inflammations are more prone to develop CSC compared with other subtypes of uveitis. Management of CSC in uveitis can be challenging.