Occlusive Retinal Vasculitis in a Pediatric Patient With Kikuchi-Fujimoto Disease and Sickle Cell Trait.

A 13-year-old Black male patient with a history of Kikuchi-Fujimoto disease (KFD) and sickle cell trait presented with acute painless vision loss and no light perception vision (NLP) in his left eye. The examination was indicative of occlusive retinal vasculitis with near total central retinal artery occlusion (CRAO). He was started on oral steroids with dramatic reperfusion and improvement of the retinal hemorrhages. However, his vision remained at NLP. Oral steroids were tapered, and rituximab infusion was initiated. While ocular involvement is uncommon in KFD, vision-limiting complications, such as occlusive retinal vasculitis, ophthalmic artery occlusion, and CRAO can occur. Early systemic immunosuppression is key in achieving rapid remission. [Ophthalmic Surg Lasers Imaging Retina 2024;55:235-239.].

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.

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.].

A Curious Case of Occlusive Retinal Vasculitis in a Young Individual Associated with COVID-19 Vaccination.

A 23-year-old man presented with the blurring of vision in the left eye for 4 days. Best-corrected visual acuity was 6/6 N6 in both eyes. Examination revealed an unremarkable right eye while the left eye showed occlusive retinal vasculitis with no retinitis, choroiditis, or macular involvement. Fundus fluorescein angiography confirmed the same. History revealed the patient had received 2nd dose of Covishield vaccination 4 weeks before the onset of symptoms. Blood investigations were negative for infectious or any systemic autoimmune disease. Serum homocysteine and serum CMV IgG levels were grossly increased while tests for antiphospholipid syndrome were weakly positive. He responded well to a combination of intravitreal and oral antivirals, oral steroids for vasculitis and tablets Clopilet and Homin. This case is extremely intriguing in terms of the involvement of the adenoviral vector vaccine either as a causative factor or being just a coincidental finding.

Retinal arterial occlusive vasculitis following IgA nephropathy: A case report.

PURPOSE: To discuss diagnosis and management in the case of a patient presenting with bilateral ischemic retinal vasculopathy associated with a previously undiagnosed IgA nephropathy. CASE REPORT: In 2021, a 35-year-old male presented with a sudden onset asymmetric bilateral (OU) visual loss. Best-corrected visual acuity (BCVA) was 20/200 OD, and no light perception (NLP) OS with an associated relative afferent pupillary defect (RAPD). Slit-lamp examination (SLE) confirmed normal anterior segment anatomy OU. Indirect ophthalmoscopy and subsequent fluorescein angiography (FA) confirmed the presence of bilateral arterial attenuation, telangiectatic lesions, associated perivascular sheathing, and capillary leakage. Spectral domain optical coherence tomography (OCT) showed multiple areas of thinning of the inner retina. This constellation of diagnostic findings was highly suggestive of a bilateral ischemic retinal vasculopathy with an inflammatory vasculitis. Based on a high index of suspicion for a systemic etiology, nephrology was consulted, and a diagnosis of IgA nephropathy was confirmed by renal biopsy. Systemic immunomodulatory therapy was initiated. CONCLUSION: Although it is among the most commonly occurring forms of glomerulonephritis leading to renal failure, IgA nephropathy rarely presents with a bilateral retinal vasculopathy. Schölein - Henoch purpura, the other primary disease associated with glomerular IgA deposition, may be indistinguishable from primary IgA nephropathy. A comprehensive retinal examination with multimodal functional and structural ophthalmic diagnostic testing in conjunction with renal biopsy was needed to confirm the diagnosis. It is critical to include primary renal pathology when formulating a differential diagnosis for cases of bilateral retinal vasculitis, particularly in young otherwise healthy patients.

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.

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.

Vancomycin-Associated Hemorrhagic Occlusive Retinal Vasculitis: A Case Series and Systematic Review.

This study describes three unilateral cases of hemorrhagic occlusive retinal vasculitis (HORV) after cataract surgery and a review of the literature until February 2022, including 21 articles reporting HORV cases. Altogether, 61 eyes (41 patients) were included. Twenty patients had bilateral and 21 patients had unilateral HORV. Prophylactic vancomycin was given to all patients. Additional vancomycin use was associated with the worst outcome. The mean time to HORV was 9 days post-cataract surgery. In bilateral cases, the median time between surgeries was 7 days. Visual acuity was < 20/400 in 48%, with no light perception in 20%. Neovascular glaucoma developed in 43%. Central macular thickening or hyperreflectivity of the inner retinal layers on optical coherence tomography was associated with worse outcomes. Corticosteroid treatment, early panretinal laser photocoagulation, or anti-vascular endothelial growth factor therapy, and prophylaxis alternative to vancomycin is recommended. [Ophthalmic Surg Lasers Imaging Retina 2022;53:702-712.].

IRVAN syndrome: A retrospective review of 9 cases from Tunisia.

PURPOSE: To describe clinical features, relevant imaging findings, disease course, and response to treatment in 9 patients (18 eyes) with idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome. METHODS: Retrospective review of the charts of nine patients (18 eyes) diagnosed with IRVAN syndrome at Fattouma Bourguiba University Hospital, Monastir, Tunisia, from January 1, 2011 to January 1, 2022. RESULTS: Nine patients were included with bilateral involvement in all cases. Mean initial best-corrected visual acuity (VA) was 20/32 (range, 20/1600-20/20). Clinical findings at presentation included vitreous cells (10 eyes, 55.6%), peripapillary exudates (12 eyes, 66.7%), partial or complete macular star (11 eyes, 61.1%), and vascular sheathing (11 eyes, 61.1%). Fluorescein angiography showed arteriolar aneurysms (18 eyes, 100%), areas of peripheral capillary non-perfusion (16 eyes, 88.9%), and retinal neovascularization (6 eyes, 33.3%). Optical coherence tomography showed macular edema in 5 eyes (27.8%). Optical coherence tomography angiography of the optic disc demonstrated papillary aneurysms in 4 eyes of 2 patients. Indocyanine green angiography showed retinal arteriolar aneurysmal dilatations in 4 eyes of 2 patients. Ten eyes (55.6%) had stage 2 disease, 6 eyes (33.3%) had stage 3, and 2 eyes (11.1%) had stage 1. Treatment modalities included peripheral photocoagulation (16 eyes, 88.9%), intravitreal bevacizumab (4 eyes, 22.2%), and intravitreal triamcinolone acetonide (1 eye, 5.6%). Mean final best-corrected VA was 20/32 (range, 20/600-20/20). Ocular complications included vitreous hemorrhage in 3 eyes (16.7%), branch retinal artery occlusion in 2 eyes (11.1%) and submacular fibrosis in 3 eyes (16.7%). CONCLUSION: IRVAN syndrome should be highly suspected in patients with peripapillary exudates associated with vascular sheathing and vitreous cells. Multimodal imaging confirms the diagnosis by showing retinal macroaneurysms. Early treatment of macular edema and/or peripheral retinal non-perfusion is mandatory to improve prognosis.

Occlusive retinal vasculitis: novel insights into causes, pathogenesis and treatment.

PURPOSE OF REVIEW: Occlusive retinal vasculitis (ORV) has a large differential diagnosis and varied therapeutic approaches. This review highlights existing and novel causes and treatment options for ORV. RECENT FINDINGS: Mutations in CAPN5, TREX1, and TNFAIP3 have been associated with dominantly inherited forms of ORV. Various intraocular therapeutics have been shown to occasionally cause ORV; the most recent medications associated with ORV are vancomycin and brolucizumab. In cases of ORV linked to Behçet's disease, clinical trials support the use of tumor necrosis factor alpha inhibitors. SUMMARY: Identification of the underlying etiology of ORV is critical to help guide treatment. Treatment in ORV involves both treatment any underlying infection or autoimmune condition, cessation of the any offending causative agent and local treatment of neovascular complications.

Cooperation with rheumatologists on intensive systemic treatment for psoriatic arthritis-related panuveitis with retinal vasculitis: a case report.

BACKGROUND: Patients with psoriatic arthritis (PsA) may develop uveitis, a potentially serious ocular complication. PsA-related uveitis may result in significant morbidity and even vision loss if underdiagnosed or under-treated. We presented a case with long-standing recurrent uveitis and retinal vasculitis successfully managed by fortified systemic immunomodulators for systemic PsA. CASE PRESENTATION: A 47-year-old woman was referred under the impression of acute anterior uveitis in her right eye in recent one month. Ocular examinations showed panuveitis in both eyes with intense vitreous opacity in her right eye. Fundus fluorescence angiography revealed retinal vasculitis in both eyes. Systemic surveys excluded the possibility of infection but showed an elevated inflammation marker. With intensive immunosuppressive treatment, inflammation resolved and the vision improved. CONCLUSION: Our case highlights not only the importance of intensified systemic therapy in treating PsA-related uveitis but the importance of multidisciplinary collaboration. Recurrent uveitis may be an indicator of disease activity prior to other inflammatory markers.

Bilateral Occlusive Retinal Vasculitis in a Patient with Dermatomyositis.

A 48-year-old woman with a history of dermatomyositis (DMS) presented with 2 weeks of worsening myalgias, weakness, and diffuse edema following cessation of her systemic immunosuppression and subsequently developed severe bilateral vision loss consistent with bilateral frosted branch angiitis. Multimodal imaging was performed, and the patient was successfully treated with pulse-dose steroids and intravenous immunoglobulin, as well as intravitreal aflibercept. Ophthalmic involvement of DMS is typically limited to episcleritis, conjunctivitis, and uveitis. We present an uncommon case of bilateral occlusive retinal vasculitis with frosted branch angiitis in a patient with DMS. The significant improvement anatomically and in visual acuity in our patient suggests a role of combined anti-vascular endothelial growth factor and systemic immunosuppression in the management of DMS -related frosted branch angiitis. We suggest that retinal vasculitis should be considered in patients with known DMS and acute vision loss, with prompt referral for ophthalmologic evaluation.

Diagnostic Markers in Ocular Sarcoidosis in A High TB Endemic Population - A Multicentre Study.

AIM: To look at markers to differentiate ocular sarcoidosis from ocular tuberculosis in a high TB endemic population. MATERIALS & METHODS: Retrospective multicenter study involving seven tertiary eyecare centers in India between July 2016 to December 2016. Demographic, clinical and laboratory data were retrieved from respective hospital databases. Group A included biopsy-proven ocular sarcoidosis while group B included ocular tuberculosis. RESULTS: Data from 2726 consecutive patients with uveitis were retrieved from the databases. Group A had 61 cases while group B included 307 cases. The mean age in group A was 43 ± 16.55 years while in group B was 38 ± 13.13 years. Females were more common in group A. Uveitis with low Schirmer test (OR-30, CI-95%), candlewax retinal vasculitis (OR-8.69, CI-95%), hilar lymphadenopathy (OR-15.23, CI-95%), fissural nodules (OR-39.11, CI-95%) had higher odds of having ocular sarcoidosis. CONCLUSION: Presence of dry eye, candlewax retinal vasculitis, hilar lymphadenopathy, and fissural nodules if present in a patient with uveitis, could help differentiate ocular sarcoidosis from ocular tuberculosis in a high TB endemic population.

Subretinal Hemorrhage Complicating Retinal Angiomatous Proliferation in Tubercular Retinal Vasculitis.

PURPOSE: To report a patient with submacular hemorrhage due to retinal angiomatous proliferation (RAP) in tubercular retinal vasculitis (TRV). METHODS: Case report. RESULTS: We report a 33-year-old Asian Indian patient of TRV presenting with capillary non-perfusion areas, submacular hemorrhage and venous loops. The patient presented with sudden onset decrease in vision in the right eye. Multimodal imaging revealed presence of retinal vascular anastomosis and stage 2 RAP. Systemic examination was within normal limits. Laboratory evaluation revealed positive Mantoux and interferon gamma release assay. He underwent right eye intravitreal injection of recombinant tissue plasminogen activator (12.5µg/0.1ml) with 100% sulphur hexafluoride (SF6) tamponade. The patient had successful displacement of the submacular hemorrhage with some improvement in visual acuity. CONCLUSION: This case highlights that rare vascular alterations such as RAP can develop in subjects with ocular tuberculosis.

EFFICACY OF INTRAVITREAL AFLIBERCEPT INJECTIONS IN THE TREATMENT OF IDIOPATHIC RETINAL VASCULITIS, ANEURYSMS, AND NEURORETINITIS SYNDROME.

PURPOSE: To present a case of idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome that was successfully managed with serial intravitreal aflibercept injections. METHODS: Ophthalmic imaging and visual acuity were used to monitor disease state and track treatment methods to determine the most valuable combination of treatment medication and treatment interval. RESULTS: A 28-year-old woman with idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome status after panretinal photocoagulation of both eyes presented with bilateral cystoid macular edema. We demonstrate successful management of retinal cystoid macular edema associated with idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome using serial intravitreal aflibercept injections. CONCLUSION: Intravitreal aflibercept has a useful role in managing the potential retinal complications associated with idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome and provides further insights into treatment of the later stages of this rare disease.