Enlarging aneurysm with paracentral acute middle maculopathy in idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) - a case report.

INTRODUCTION: Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome is a rare clinical entity affecting young healthy individuals. Treatment primarily involves pan retinal photocoagulation (PRP) to capillary non perfusion areas. Intravitreal anti-VEGF or steroids are given in the presence of macula edema. Oral steroids do not alter the course of the disease. Arterial occlusions have been reported in IRVAN. METHODS: Retrospective case review. RESULT: A twenty seven year old male presented to us with mild blurring of vision for one week. His BCVA was OU 20/20. Anterior segment examination was normal. Fundus examination showed bilateral disc aneurysm with OS arterial aneurysm along the inferior arcade. Fundhus fluorescein angiography and OCT angiography were confirmatory of the disc and retinal aneurysm. Capillary non perfusion (CNP) areas were noted in the periphery. Two days later he presented with paracentral scotoma in his left eye which was confirmed by Amsler chart. Fundus, OCT and OCTA were confirmatory of Paracentral Acute Middle Maculopathy (PAMM). The retinal aneurysm had increased in size from 333 micron diameter to 566 micron diameter. Panretinal photocoagulation to the CNP areas was done and intravitreal antiVEGF was given. At 6 months follow up, retinal aneurysm had disappeared. DISCUSSION: Our case describes a unique event with sudden increase in size of the aneurysm leading to acute blockage in the deep capillary plexus thus being the first report of PAMM in IRVAN. The patient was treated with PRP and intravitreal anti-VEGF for the enlarging aneurysm which reduced in size within a week.

Toxic posterior segment syndrome with retinal vasculitis likely caused by intraocular cotton fiber after vitreoretinal surgery - a case report.

BACKGROUND: Intraocular inflammation is common after anterior or posterior segment surgery. They typically manifest either as non-infectious inflammation of the anterior or posterior segment, known as toxic anterior or posterior segment syndrome (TPSS), or as sterile or infective endophthalmitis. In this report, we describe a rare case of TPSS following vitreoretinal surgery, presenting as hemorrhagic retinal vasculitis. CASE PRESENTATION: A 58-year-old male diagnosed with a left eye acute rhegmatogenous retinal detachment underwent an uneventful primary pars plana vitrectomy with silicone oil endotamponade on the same day of presentation. At presentation, there were no signs of intraocular inflammation, and his visual acuity in the affected eye was 20/200. RESULTS: The retina was well-attached with silicone oil in place on the first post-operative day. Along the inferior retinal periphery, a hemorrhagic occlusive vasculitis was observed. Clinical examination revealed retained intraocular cotton fiber along the inferotemporal quadrant over the retinal surface. In addition to the standard post-operative medications, a course of systemic steroids (40 mg per day of Prednisolone tablets) was started. At the end of the first post-operative week, clinical signs of hemorrhagic retinal vasculitis were beginning to resolve, and by the end of the fourth post-operative week, they had completely resolved. CONCLUSION: This report describes an unusual diagnosis of TPSS after vitreoretinal surgery, most likely due to the presence of an intraocular cotton fiber. This excessive inflammation of the posterior segment usually responds to a course of topical and systemic steroids.

Rare case report: a 26-year-old man with Eales' disease.

Purpose: To report the case of a 26-year-old male with bilateral Eales' disease that led to total blindness in the left eye and legal blindness in the right eye in a short time. Methods: A total clinical systemic examination, computed tomography, magnetic resonance imaging, genetic testing, and optical coherence tomography were performed in the reported case. Results: The eye condition was managed by scatter laser treatment, Anti-VEGF injections, anterior chamber paracentesis and trabeculectomy. Non-steroidal eye drops, as well as prostaglandin analogues, beta-blockers, and carbonic anhydrase inhibitors, have been used as local treatment. Systemic treatment included an intravenous methylprednisolone course, oral corticosteroids, azathioprine, mycophenolate mofetil and a total amount of 12 Anti-VEGF injections. Conclusion: Despite the aggressive treatment with oral steroids, immunosuppressants, and anti-VEGF injections, there were many exacerbations, and remission was not achieved. As a result, aggressive neovascular glaucoma developed, which led to total blindness in the left eye and legal blindness in the right eye. Abbreviations: HLA = human leukocyte antigens, Anti-VEGF = vascular endothelial growth factor inhibitors, BCVA = best corrected visual acuity, FA = fundus angiography, HBsAg = hepatitis B surface antigen, Anti-HCV = hepatitis C antibodies, TPHA = Treponema Pallidum hemagglutination assay, PCR = polymerase chain reaction, HSV = Herpes simplex virus, VZV = Varicella zoster virus, CMV = cytomegalovirus, IOP = intraocular pressure.

Central retinal artery occlusion as a presenting symptom in Eales' disease: a case report.

BACKGROUND: Eales' disease is an idiopathic peripheral retinal vasculopathy characterized by retinal phlebitis, ischemia, retinal neovascularization, and recurrent vitreous hemorrhages. But CRAO is an unusual presentation. CASE PRESENTATION: A 27-year-old healthy female nurse of Indian descent presented with sudden vision loss in her right eye upon awakening. Central retinal artery occlusion (CRAO), combined with mild central retinal vein occlusion (CRVO), was diagnosed. During the second of three consecutive sessions of hyperbaric oxygen treatments, her vision rapidly improved. One week later, she developed peripheral phlebitis in the same eye. Infectious, inflammatory, and hematologic etiologies were excluded. The systemic evaluation was normal except for a positive Mantoux tuberculin skin test. Following systemic steroidal treatment, she experienced gradual improvement of her vasculitis. Two weeks later, mild retinal phlebitis appeared in her left eye. Eales' disease was diagnosed after the exclusion of other diseases. CONCLUSION: This is an unusual Eales' disease case, which presented as combined CRAO with mild CRVO. The association of CRAO and Eales' disease is reported here for the first time, to our best knowledge.

FROSTED BRANCH ANGIITIS ASSOCIATED WITH MIXED CONNECTIVE TISSUE DISEASE.

PURPOSE: To present a case of frosted branch angiitis associated with an exacerbation of mixed connective tissue disease (MCTD). METHODS: Single case report. RESULTS: A 31-year-old woman presented with a flare of her long-standing MCTD after a change in her immunosuppressive medications. She developed blurred vision and floaters first in the left eye but eventually in both eyes. Fundoscopy showed patchy perivascular sheathing of tertiary branch venules surrounded by retinal hemorrhages characterized as frosted branch angiitis. The patient's MCTD symptoms and retinal vasculitis improved with continued immunosuppressive therapy. At 1-month follow-up, her visual acuity had improved to 20/20 bilaterally with complete resolution on fundoscopy. CONCLUSION: To the authors' knowledge, this is the first report of frosted branch angiitis seen in association with MCTD. The improvement in the patient's visual acuity and fundoscopic findings in this case supports the role of immunosuppressive therapy to treat secondary frosted branch angiitis associated with an autoimmune condition such as MCTD. However, it is recommended that a comprehensive medical workup is performed to exclude an infective cause, particularly in immunocompromised patients.

Subretinal Abscess Complicated by Post-infectious Retinal Vasculitis Following Strabismus Surgery.

An 8-year-old girl presented with a subretinal abscess after strabismus surgery. This was treated successfully with medial rectus suture removal, pars plana vitrectomy, intravitreal antibiotics, and intravenous antibiotics. Recovery was complicated by acute post-infectious retinal vasculitis after tapering high-dose corticosteroids, requiring an extended corticosteroid regimen over 2 months until resolution. [J Pediatr Ophthalmol Strabismus. 2023;60(3):e26-e30.].

RETINAL VASCULITIS SEVERITY ASSESSMENT: Intraobserver and Interobserver Reliability of a New Scheme for Grading Wide-Field Fluorescein Angiograms in Retinal Vasculitis.

PURPOSE: Wide-field fluorescein angiography is commonly used to assess retinal vasculitis (RV), which manifests as vascular leakage and occlusion. Currently, there is no standard grading scheme for RV severity. The authors propose a novel RV grading scheme and assess its reliability and reproducibility. METHODS: A grading scheme was developed to assess both leakage and occlusion in RV. Wide-field fluorescein angiography images from 50 patients with RV were graded by four graders, and one grader graded them twice. An intraclass correlation coefficient (ICC) was used to determine intraobserver-interobserver reliability. Generalized linear models were calculated to associate the scoring with visual acuity. RESULTS: Repeated grading by the same grader showed good intraobserver reliability for both leakage (ICC = 0.85, 95% CI 0.78-0.89) and occlusion (ICC = 0.82, 95% CI 0.75-0.88) scores. Interobserver reliability among four independent graders showed good agreement for both leakage (ICC = 0.66, 95% CI 0.49-0.77) and occlusion (ICC = 0.75, 95% CI 0.68-0.81) scores. An increasing leakage score was significantly associated with worse concurrent visual acuity (generalized linear models, ß = 0.090, P < 0.01) and at 1-year follow-up (generalized linear models, ß = 0.063, P < 0.01). CONCLUSION: The proposed grading scheme for RV has good to excellent intraobserver and interobserver reliability across a range of graders. The leakage score related to present and future visual acuity.

Retinal Vasculitis Following COVID-19 Vaccination: Causation or Coincidence?

BACKGROUND: With the popularity of coronavirus disease 2019 (COVID-19) vaccination, some rare ocular adverse events have gradually been reported. This report shows a rare case of retinal vasculitis following COVID-19 vaccination. DESCRIPTION: A 37-year-old male complained of unilateral severe vision loss 13 days after the first dose of an inactivated COVID-19 vaccine. The initial eye examination showed gray-white edema in the macula, and scattered patchy gray-white lesions in the peripheral retina, accompanied by peripheral retinal small vessel occlusion. After glucocorticoid treatment, the gray-white lesions gradually subsided. However, due to the initial severe damage in the fundus, macular and peripheral retinal lesions were significantly atrophied, and the vision recovery was poor. CONCLUSION: The ocular adverse event in this report may be related to the inactivated COVID-19 vaccination, however, it is difficult to rule out the accidental rare adverse events after a large number of vaccinations.

Rescue Treatment with Infliximab for a Bilateral, Severe, Sight Threatening Frosted Branch Angiitis Associated with Concomitant Acute Onset of Presumed Dermatomyositis.

PURPOSE: To report a severe bilateral frosted branch angiitis associated with acute onset of systemic dermatomyositis. METHOD: History and clinical examination, laboratory evaluation, fundus' and skin's color pictures, fluorescein angiography (FA), optical coherence tomography (OCT). RESULTS: A 32-year-old female was referred for a bilateral frosted branch angiitis and vitreitis, with skin rash, muscle pain, weakness, severe headache, compatible with dermatomyositis. After an initial improvement following an aggressive treatment by systemic steroids and mycophenolate mofetil, she was switched to intravenous infliximab (RemicadeTM, Janssen Biotech, Inc. USA) due to lack of efficacy. Nine months later, her visual acuity was improved up to 20/20 in OU, with both retinal vasculitis and vitreitis being solved. CONCLUSION: Dermatomyositis is a severe multiorgan disease which might severely involve eye structures. Anti-TNF-Alpha agents, particularly Infliximab, might offer a rapid control and long-term remission.

A Comparative Study between Occlusive and Non-occlusive Retinal Vasculitis: Data from a Referral Center in Tunisia, North Africa.

PURPOSE: To compare clinical profile and visual outcomes of occlusive versus non-occlusive retinal vasculitis (RV). METHODS: A retrospective comparative study. RESULTS: 284 patients were enrolled, including 124 patients with occlusive RV (ORV) and 160 patients with non-occlusive RV (NORV). Patients with ORV were older (p ≤ 10-3), predominantly male (p ≤10-3), with less bilateral involvement (31.5% vs 53,4%; p ≤ 10-3). Infectious RV was more frequently diagnosed in the ORV group than in the NORV group (48.8% vs 32.9%, p = .006). Behçet disease and ocular tuberculosis were the leading causes of ORV. Idiopathic RV, Behçet disease, and sarcoidosis were the most common causes of NORV. Independent predictive factors of poor visual outcome were worse baseline visual acuity in both groups (p = .006 and p ≤ 10-3, respectively), and retinal hemorrhages (p = .048) and optic atrophy (p = .040) in the ORV group. CONCLUSION: Occlusive and non-occlusive RV have distinctive clinical and etiological profile.

Effects of Subcutaneous Repository Corticotropin Gel Injection on Regulatory T Cell Population in Noninfectious Retinal Vasculitis.

AIM: To evaluate the effect of repository corticotropin injection (RCI) on regulatory T cell population in patients with noninfectious retinal vasculitis. PATIENTS AND METHODS: Patients with active noninfectious retinal vasculitis were included in a prospective nonrandomized open-label study. RESULTS: Eighteen patients (33 eyes) were included in the study. Eleven (61.1%) patients [20 (60.6%) eyes] and 7 (38.9%) patients [13 (33.3%) eyes] were in the responsive and non-responsive groups, respectively. We did not find any statistically significant difference within the PPP-R group, within the PPP-NR group, or between these two groups in regard to regulatory T cell population. No significant systemic or ocular complications were found. CONCLUSION: RCI may be a complementary treatment in patients with non-infectious retinal vasculitis with or without uveitis. This study did not demonstrate an increase in regulatory T cell population in patients with noninfectious retinal vasculitis.

Idiopathic Retinal Vasculitis, Aneurysms and Neuroretinitis: Clinical Improvement with Infliximab.

PURPOSE: Idiopathic retinal vasculitis, aneurysms and neuroretinitis is a rare vision-threatening condition. If untreated vision loss occurs due to complications of progressive retinal ischaemia including retinal neovascularisation, neovascular glaucoma and retinal exudation. Despite the proposed underlying inflammatory aetiology this condition demonstrates poor response to corticosteroid treatment. The aim was to describe two paediatric cases of idiopathic retinal vasculitis, aneurysms and neuroretinitis treated with infliximab. METHODS: Two case reports. RESULTS: Infliximab treatment led to resolution of aneurysmal dilatations and retinal vasculitis, and reversal of some retinal capillary non-perfusion. CONCLUSION: Early infliximab treatment should be considered in cases of idiopathic retinal vasculitis, aneurysms and neuroretinitis.

A Case of Idiopathic Retinitis Vasculitis Aneurysms and Neuroretinitis (IRVAN) Treated with Adalimumab.

PURPOSE: To report a case of IRVAN in a 13-year-old girl responding well to Adalimumab and Azathioprine. RESULTS: A 13-year-old girl presented to us with central scotoma for a duration of 10 months. She was treated earlier with oral steroids with poor response. Fundus examination revealed features of IRVAN. She was treated with intravitreal dexamethasone implant in both eyes with oral Mycophenolate Mofetil (MMF) with transient response to it. So she was switched over to subcutaneous Adalimumab 40 mg once in 2 weeks and oral Azathioprine 50 mg BD. The disease activity was well controlled with the current regime. CONCLUSION: Though various treatment modalities have been described in literature for the treatment of IRVAN. This is the first case of IRVAN to be treated with Adalimumab along with Azathioprine to be reported.

MULTIMODAL IMAGING OF ACUTE MULTIFOCAL HEMORRHAGIC RETINAL VASCULITIS: A CASE REPORT.

PURPOSE: To report a case of acute multifocal hemorrhagic retinal vasculitis and demonstrate the multimodal imaging. METHODS: Interventional case report. RESULTS: A 54-year-old woman without significant past medical history complained of redness and blurred vision in both eyes. Her visual acuity was counting fingers and 20/60. Mild conjunctival injection, anterior chamber cells, and vitreous haze were noted. Fundus showed multifocal intraretinal hemorrhages. Fluorescein angiography revealed vasculitic process with intraretinal hemorrhage blocking defects and retinal ischemic changes in both eyes. Anterior chamber tap fluid polymerase chain reaction for varicella zoster virus, herpes simplex virus I/II, cytomegalovirus, and Epstein-Barr virus was unremarkable. Rheumatology was consulted and systemic vasculitis was ruled out. Her vision improved to 20/50 and 20/20 after pulse methylprednisolone therapy, oral methotrexate, and prednisolone treatment. CONCLUSION: Acute multifocal hemorrhagic retinal vasculitis can occur in an immunocompetent patient. Multimodal Imaging is useful in the diagnosis and follow-up. Patients could benefit from early and aggressive immunosuppressive therapy.