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Purpose: To assess the validity of retinal vasculitis as the preferred diagnostic term for multiple conditions., Design: Perspective., Methods: This Perspective is based on expert opinion and review of literature focused on the current nosology and pathology of retinal vasculitis. Interpretation of the subset of intraocular inflammation named "retinal vasculitis" based on fundamental knowledge of the blood-retinal barrier, the neurovascular unit, and pathological and functional responses to a variety of stimuli is provided. Correlation with multimodal imaging and known mechanisms of immunologically mediated disease are discussed., Results: A search of Medline in early 2024 for the phrase "retinal vasculitis" resulted in 2041 citations encompassing immunologic, genetic, neoplastic, infectious, drug-related, and ischemia-related disorders. Classification schemes and angiographic grading systems are descriptive and do not address pathologic mechanisms adequately, in part due to lack of histologic confirmation. Although optical coherence tomography (OCT) angiography holds promise for better imaging of retinal vascular changes, it does not reveal the key feature of leakage and only partially improves understanding of pathophysiology. Diagnosing catastrophic retinal vascular occlusion after intravitreal injections as a retinal vasculitis is the most recent example of speculative application of the term to complex and rare disorders., Conclusions: Retinal vasculitis is a diagnostic term that is over-used and imprecise. Revised nosology should limit the term to primary inflammation of the retinal vasculature itself that results in opening of the blood-retinal barrier with or without retinal vascular occlusions. Pending new histologic or mechanistic evidence, the provisional term of "retinal vascular inflammation" or "retinal vasculopathy" should be used for leakage or occlusion occurring in the context of intraocular inflammation., Competing Interests: Conflicts of interest No conflict., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: Retinal vasculitis is an inflammatory condition that affects the retinal blood vessels., Summary: It can manifest as an idiopathic disorder or be secondary to various infectious or non-infectious diseases, mimicking syndromes, isolated ocular disorders, or drug-induced reactions. Recognizing its distinctive features is crucial for early diagnosis and accurate treatment. This review aimed to demonstrate the variety of tools available to detect disease activity, assess complications, measure the extent of retinal damage, and guide therapy effectively., Key Message: This review article highlights the use of multimodal imaging in the comprehensive evaluation of retinal vasculitis., (© 2024 S. Karger AG, Basel.)

Purpose: To characterize the clinical features of occlusive retinal vasculitis (ORV)., Design: Retrospective case series., Participants: Forty-two patients with ORV., Methods: A retrospective chart review identified all patients with ORV seen at the University of Colorado uveitis service between January 2013 and April 2020. All included patients demonstrated noninfectious uveitis and evidence of vascular occlusion in the presence of retinal vascular inflammation on widefield fluorescein angiography., Main Outcome Measures: Demographic data, visual acuity, clinical findings, and fluorescein angiography findings., Results: We identified 73 eyes from 42 patients (15 men, 27 women) with ORV. Thirty-one of 42 patients had bilateral disease. Most eyes (54/73) showed mixed arteriolar and venous vasculitis compared with primarily arteriolar (6/73) or venous (15/73) vasculitis. Thirteen of 42 patients had an underlying systemic condition, most commonly granulomatosis with polyangiitis; however, bilaterality was not associated with a systemic condition. Retinal nonperfusion was present equally in zone 2 (28/73) and zone 3 (28/73) compared with zone 1 (16/73). Retinal or iris neovascularization was present in 25 of 73 eyes. Eighteen of 42 patients required more than 1 immunosuppressive medication (average, 1.33) to prevent progressive vascular occlusive disease., Conclusions: Occlusive retinal vasculitis is a heterogeneous entity with significant risk of visual impairment. Systemic disease was more prevalent in this specific cohort compared with cohorts from prior studies of retinal vasculitis., (Copyright © 2021 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Purpose: To introduce a novel method for assessment of retinal vasculitis using swept-source OCT angiography (SS-OCTA)., Design: Retrospective case series., Participants: Patients with retinal vasculitis., Methods: The subjects were identified among the clinic population and imaged with 12 × 12-mm SS-OCTA scans centered on the fovea. A custom retina segmentation superimposed the color retinal thickness map on a modified en face flow scan. Findings from en face flow scans were correlated with localized perivascular retinal thickening on B-scans. Results from SS-OCTA were compared with fluorescein angiography (FA) to examine the proportion of perivascular thickening to retinal vascular leakage or staining., Results: Twenty-one patients with retinal vasculitis underwent same-day FA and SS-OCTA. Visible retinal vascular leakage/staining on FA corresponded to increased perivascular retinal thickness on SS-OCTA in 17 patients. Five patients had a second examination with same-day FA and SS-OCTA after treatment of the vasculitis. Three of those 5 patients showed improved retinal vascular leakage/staining on post-treatment FA and decreased perivascular retinal thickness on SS-OCTA scans., Conclusions: Swept-source OCT angiography detects structural retinal thickening secondary to inflammatory retinal vascular leakage. Further studies are required to confirm whether SS-OCTA may serve as a semiquantitative alternative to FA to diagnose and monitor the response to treatment in patients with retinal vasculitis., (Published by Elsevier Inc.)

Purpose: Pediatric idiopathic uveitis typically shows anterior segment involvement. Whether retinal vasculitis is an important manifestation of this disease remains unknown and was therefore the subject of this study., Methods: This study was performed involving patients with pediatric idiopathic uveitis. Fundus fluorescein angiography was used to assess the presence of retinal vasculitis., Results: A total of 1,867 patients with pediatric uveitis were seen between December 2008 and January 2018, of whom 1,364 had undergone fundus fluorescein angiography examination. Idiopathic uveitis was the most common entity, accounting for 81.2%. Among these patients with idiopathic uveitis, 79.6% had retinal vasculitis in at least one eye. After 1-year treatment with oral prednisone mostly combined with cyclosporine, 76.3% patients in the retinal vasculitis group achieved control of their ocular inflammation, which was significantly lower as compared with 85.1% in those without (P = 0.008). Retinal vasculitis was an independent predictor for a lower probability of inflammation control after 1-year treatment. Visual function (best-corrected visual acuity > 20/25 in the better seeing eye) was worse in the retinal vasculitis group than in the control group after 5 years., Conclusion: Almost 80% of patients with pediatric idiopathic uveitis show manifestations of retinal vasculitis, which is associated with a lower probability of inflammation control resulting in a worse visual prognosis.

Purpose: To report a case of hemorrhagic occlusive retinal vasculitis after cataract surgery., Methods: A 74-year-old woman presented with blurry vision and distorted vision, which started 2 days after an uncomplicated cataract surgery in the left eye. Intracameral vancomycin was injected during the case. The patient reported being treated with systemic vancomycin in the past., Results: The visual acuity was 20/80 in the left eye. She had trace cells in the anterior chamber with no hypopyon and intraocular lens implant within the capsular bag in the left eye. Dilated fundus examination revealed no vitritis, There were large patches of peripheral retinal hemorrhages and retinal ischemia. The patient was diagnosed with hemorrhagic occlusive retinal vasculitis likely secondary to hypersensitivity reaction to intracameral vancomycin. The patient was started on oral prednisone, and the topical difluprednate course was escalated. Within 3 weeks, vision improved to 20/30 in the left eye. She underwent pan retinal photocoagulation targeting the ischemic areas in the periphery., Conclusion: The patient had previous exposure to systemic vancomycin, which may have sensitized her immune system. Later on, the hypersensitivity reaction took place after exposure to intracameral vancomycin during cataract surgery. Our hemorrhagic occlusive retinal vasculitis case had a favorable visual outcome, and recognition of this entity will ensure that vancomycin will not be used for infection prophylaxis in the fellow eye at the time of cataract surgery.

Purpose : To report a novel peripheral finding in patients with Birdshot retinochoroiditis (BRC) using ultra-widefield fluorescein angiography (UWFFA). Methods : UWFFA images from 90 patients diagnosed with BRC were reviewed. All images were evaluated for the presence of peripheral retinal ischemic changes and ischemia-related complications. Findings : Thirty-six eyes of 24 patients showed features of peripheral ischemia, including 18 eyes with peripheral capillary non-perfusion (CNP), 5 eyes with teleangectasias, with or without microaneurysms, and 13 eyes with both teleangectasias and CNP. None of the 36 eyes with peripheral ischemic changes developed retinal neovascularization following the diagnosis of peripheral ischemia. Conclusions : Peripheral ischemia may present in eyes with chronic inflammation as seen in BRC but does not seem to progress to neovascular complications. This is likely to be more common than suspected and has now become evident following the advent of UWFFA. Abbreviations : HLA-A29: Human leukocyte antigen - A29; FFA: Fundus fluorescein angiography; OCT: Optical coherence tomography; ICGA: Indocyanine green angiography; UWF: Ultra-wide field; UWFFA: Ultra-wide field fluorescein angiography; CNP: Capillary non-perfusion.

Background: Idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome is a very rare primary retinal vasculitis. It seems to evolve in stages, where there is initially a florid vasculitis associated with aneurysm formation. Neuroretinitis and macula edema are common features. Subsequently, retinal ischemia ensues, leading to neovascularization. If untreated, further sight-threatening complications occur, including traction retinal detachment and secondary glaucoma., Methods and Results: Here, we describe a patient with early idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome, who was treated with a combination of systemic immunosuppression and localized photocoagulation. There was substantial regression of the aneurysms and improvement of the macular edema. Treatment of the disorder should be based on the clinical stage and complications of the disorder., Conclusions: As shown here, a multidisciplinary approach can be very helpful in managing patients with this severe sight-threatening disorder.

Purpose: To describe a case of retinal occlusive vasculopathy associated with intravitreal administration of rituximab., Methods: A single case report from a tertiary referral center., Results: The patient described in the following case report developed a diffuse retinal occlusive vasculopathy following intravitreal rituximab for intraocular lymphoma. He required panretinal photocoagulation, avastin, and cyclophotocoagulation to control his intraocular pressure from ocular ischemia resulting in neovascular glaucoma., Conclusions: This is the first reported case of occlusive retinal vasculopathy subsequent to intravitreal administration of rituximab. Whereas hypersensitivity reactions to intravenous infusion of rituximab have been noted in the literature, no such reports exist in response to intravitreal therapy.

Purpose: To evaluate the usefulness of fluorescein angiography (FA) to detect occult retinal vasculitis in children with otherwise apparently quiescent intermediate uveitis, posterior uveitis, and panuveitis based on clinical examination alone., Design: Retrospective chart review., Participants: Pediatric uveitis patients evaluated at the Children's Medical Center in Dallas, Texas., Methods: Retrospective chart review of pediatric patients treated with immunosuppressive therapy in the uveitis clinic at the Children's Medical Center, Dallas, Texas, between September 2015 and September 2016. Patients with noninfectious uveitis requiring immunosuppressive therapy, in which posterior segment involvement (intermediate uveitis, posterior uveitis, or panuveitis) was known or suspected, and whose disease apparently was quiescent on clinical examination were included., Main Outcome Measures: The incidence of occult retinal vasculitis detected on FA alone., Results: Fourteen pediatric patients met inclusion criteria. Six patients (43%) demonstrated intermediate uveitis, and 8 patients (57%) demonstrated panuveitis. Eleven patients (79%) were found to show additional evidence of occult retinal vasculitis on FA., Conclusions: Fluorescein angiography can be an important tool in evaluating pediatric uveitis patients with known or suspected posterior involvement for the presence of occult retinal vasculitis. Failure to control occult retinal vasculitis adequately may be a contributing factor to seemingly recalcitrant cases, inability to wean off immunomodulatory therapy, and long-term complications leading to poor prognosis., (Copyright © 2019 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Purpose: We aimed to analyze the clinical characteristics of idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome. Furthermore, we aimed to correlate the number and location of retinal aneurysms with the size of retinal non-perfusion area and neovascularization., Methods: Six patients with IRVAN syndrome (1 male and 5 females, age 5-38 years) were enrolled in this study. Fundus fluorescein angiography (FFA) was used to determine the total number of retinal aneurysms, number of aneurysms within the first branch of the retinal artery, minimum distance between the non-perfusion margin and the optic disc, and the number of retinal aneurysms in each quadrant, as well as the type of neovascularization., Results: The size of the non-perfusion area was positively correlated with the total number of retinal aneurysms, the number of aneurysms within the first branch of the retinal artery, and the number of retinal aneurysms in each quadrant (P < 0.05). During the 5-year follow-up, one patient exhibited a dynamic change in the number and location of retinal aneurysms., Conclusions: In IRVAN syndrome, the number and location of retinal aneurysms correlate with the size of retinal non-perfusion area and type of neovascularization.

Introduction: To report a case of retinal vasculitis associated with cutaneous leukocytoclastic vasculitis., Methods: Retrospective chart review., Results: A 28-year-old man, who initially presented with occlusive retinal vasculitis and vitreous hemorrhage in right eye that resolved with sectoral photocoagulation. Laboratory investigations for tuberculosis, sarcoidosis, syphilis and sickle cell disease were negative. Past history included recent diagnosis of Enterobacter epididymo-orchitis and multiple red nodules on skin of forearm. Fourteen months later, he developed active retinal vasculitis in right eye and recurrent nodules on forearm. Skin biopsy revealed neutrophilic infiltrates in and around dermal vessels with destruction of vessel walls leading to scattered neutrophils, lymphocytes and histiocytes between collagen bundles, suggestive of leukocytoclastic vasculitis. Both skin and ocular lesions resolved with oral corticosteroid and methotrexate therapy and did not recur over a six-year period., Conclusion: We have reported the first case of clinically manifest retinal vasculitis, associated with a common form of cutaneous vasculitis.

Aim : To evaluate patterns of retinal vasculitis with ultra-wide field imaging (UWF) and ascertain the risk factors for retinal neovascularization. Methods : Consecutive patients of retinal vasculitis were included prospectively. Patients with retinal vasculitis secondary to uveitis were excluded. UWF was done for all the patients. Retinal involvement was classified into three zones and area of capillary non-perfusion was stratified into clock hours. Results : Two hundred patients were included, 85% ( n  = 170) were male. Mean age was 28.99 ± 10.56 years. Clinical examination revealed 65% cases ( n  = 130) to be bilateral, while UWF angiography detected 72.5% ( n  = 145) to have bilateral involvement. Retinal neovascularization was present in 47% ( n  = 188).Presence of posterior disease had very high odds ratio for development of retinal neovascularization as compared to cases restricted to retinal periphery (OR = 45.03, CI = 6.10-332.30, p  = < 0.001). Conclusion : UWF imaging is useful in detecting retinal vasculitis, which is otherwise obscure to clinical examination and assessing risk factors for retinal neovascularization.

Competing Interests: There are no conflicts of interest

Frosted branch angiitis (FBA), a rare form of retinal vasculitis presenting as bilateral perivascular sheathing, resembling the appearance of frosted tree branches in winter, was first reported by Ito et al. 1 in 1976, in a young immunocompetent boy. FBA predominantly affects healthy young patients, the youngest reported in an 11-month-old infant 2 and oldest in a 42-year-old patient. 3 Classical symptoms include sudden onset of blurred vision with floaters and photopsiae. Fundus examination shows widespread perivascular translucent sheathing affecting both arterioles and venules, more commonly latter. Fluorescein angiography shows late staining of vessels with no obstruction of blood flow. Electroretinogram shows reduced amplitude and visual fields show generalized constriction. Medline search did not show any case of frosted branch angiitis in a patient with typhoid fever.

Purpose: To study the sheathing of retinal vasculitis in various systemic autoimmune diseases using adaptive optics imaging (AOI)., Methods: Prospective, observational case series with six patients: Behçet disease (n = 1); systemic lupus erythematosus (n = 1); idiopathic retinal vasculitis (n = 2); granulomatosis with polyangiitis (n = 1); and Takayasu aorta arteritis (n = 1). Fundus photograph (FP), fundus fluorescein angiography (FFA) were done in all cases at presentation. Using the Image J software, perivascular sheathing and wall-to-wall diameter of the vessel involved were measured on AOI at time of presentation and on follow-up., Results: AOI was able to pick the pipe-stem sheathing in SLE and IRV(I) and parallel sheathing in rest, which correlated with FP and FFA. Moreover, the decrease and a complete resolution in the sheathing were also noted by AOI on follow-up., Conclusions: AOI can be used as an additional investigative tool for diagnosis and to monitor the disease course during the treatment.

Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) is a rare syndrome affecting the retinal and optic disc vasculature. Diffuse retinal ischemia, macular edema, and neovascularization may lead to bilateral vision loss. The authors report a case of a 36-year-old woman presenting with branch retinal artery occlusion (BRAO) in her right eye who was subsequently diagnosed with IRVAN syndrome. She was treated with panretinal photocoagulation for peripheral retinal ischemia and pars plana vitrectomy for vitreous hemorrhage. She later developed a BRAO in her left eye. This case demonstrates that BRAO may be a presenting feature of IRVAN syndrome. [Ophthalmic Surg Lasers Imaging Retina. 2017;48:948-951.]., (Copyright 2017, SLACK Incorporated.)

PurposeTo quantify the additional information provided by ultra-widefield fluorescein angiography, compared with 7-field standard imaging, in patients with retinal vasculitis (RV).Patients and methodsRetrospective case series of 106 patients.ResultsRetinal vascular pathology was identified by UWF FFA, but not by standard ETDRS 7-field area, in 62 patients (58.5%) and in 79 eyes (43.4%). The pathology included active RV (47 eyes, 25.8% of eyes); retinal ischaemia, or infarction (53 eyes, 29.1%); and retinal neovascularization (7 eyes, 3.8%). A change to management was made in 36 patients (34%). Of these, 21 (20% of all patients undergoing angiography) were made after the identification of retinal vascular pathology, which was found only on UWF FFA, outside the ETDRS area.ConclusionUltra-widefield fluorescein angiography has clear advantages over standard multi-field imaging. It is currently the standard method of assessment for RV in this centre.

Purpose: To investigate the structural optical coherence tomography and related microvascular flow characteristics in eyes with retinal vasculitis., Methods: Regions involved with perivascular infiltration in eyes with retinal vasculitis, but no evidence of large vessel occlusion were evaluated with optical coherence tomography (OCT), OCT angiography, and fluorescein angiography., Results: Ten eyes of 5 patients with a variety of etiologies of retinal vasculitis were evaluated. These patients did not have either cotton wool spots or deeper placoid areas of retinal ischemia. Around large vessels there was perivascular infiltration with leakage and staining seen during fluorescein angiography. Structural OCT showed slight thickening with loss of visualization of normal retinal laminations. OCT angiography showed a lack of flow signal in capillary sized vessels in the same areas. Treatment resulted in a rapid thinning of the affected areas, with the inner and middle layers of the retina becoming thinner than surrounding uninvolved areas. OCT angiography did not show a return of capillary perfusion in these regions. The thickness change in the structural OCT as shown by a heat map had a pattern mimicking the original perivascular infiltration around large retinal vessels., Conclusion: Capillary level perfusion abnormalities can develop in regions adjacent to large vessel inflammatory infiltrate that result in retinal thinning without the development of usual stigmata of acute microvascular flow obstruction such as cotton wool spots. This suggests that retinal damage may occur in retinal vasculitis that would not be recognized without using OCT-based imaging modalities.

Purpose: To determine factors affecting the visual outcome in eyes with retinal vasculitis and the rate of neovascularization relapse in ischemic vasculitis., Design: Retrospective cohort study., Methods: We reviewed 1169 uveitis patients from Moorfields Eye Hospital, London, UK. Retinal vasculitis was observed in 236 eyes (121 ischemic, 115 nonischemic) that were compared with a control group (1022 eyes) with no retinal vasculitis. Ultra-widefield fluorescein angiography images were obtained in 63 eyes with ischemic vasculitis to quantify area of nonperfusion measured as ischemic index., Results: The risk of vision loss was significantly more in the retinal vasculitis compared with the non-vasculitis group (hazard ratio [HR] 1.67, 95% confidence interval [CI] 1.24-2.25, P = .001). Retinal vasculitis had twice the risk of macular edema compared to the non-vasculitis group. Macular ischemia increased the risk of vision loss in vasculitis eyes by 4.4 times. The use of systemic prednisolone in eyes with vasculitis was associated with a reduced risk of vision loss (HR 0.36, 95% CI 0.15-0.82, P = .01). Laser photocoagulation was administered in 75 eyes (62.0%), out of which 29 (38.1%) had new vessel relapse and required additional laser treatment. The median ischemic index was 25.8% (interquartile range 10.2%-46%). Ischemia involving ≥2 quadrants was associated with increased risk of new vessel formation (HR 2.7, 95% CI 1.3-5.5, P = .003)., Conclusions: Retinal vasculitis is associated with an increased risk of vision loss, mainly secondary to macular ischemia, and has a higher risk of macular edema compared to eyes with no vasculitis. Ischemia involving ≥2 quadrants is a risk factor for new vessel formation., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Purpose of Review: Ophthalmologists and rheumatologists frequently have a miscommunication among themselves, and as a result differ in their opinion for patients consulting them with retinal vasculitis. This report seeks to establish a common understanding of the term, retinal vasculitis, and to review recent studies on this diagnosis., Recent Findings: The genetic basis of some rare forms of retinal vascular disease has recently been described. Identified genes include CAPN5, TREX1, and TNFAIP3; Behçet's disease is a systemic illness that is very commonly associated with occlusive retinal vasculitis; retinal imaging, including fluorescein angiography and other newer imaging modalities, has proven crucial to the identification and characterization of retinal vasculitis and its complications; although monoclonal antibodies to interleukin-17A or interleukin-1 beta failed in trials for Behçet's disease, antibodies to TNF-alpha, either infliximab or adalimumab, have demonstrated consistent benefit in managing this disease. Interferon treatment and B-cell depletion therapy via rituximab may be beneficial in certain types of retinal vasculitis., Summary: Retinal vasculitis is an important entity for rheumatologists to understand. Retinal vasculitis associated with Behçet's disease responds to monoclonal antibodies that neutralize TNF, but the many other forms of noninfectious retinal vasculitis may require alternate therapeutic management.

Purpose: To investigate the relationships between peripheral vasculitis, ischemia, and vascular leakage in uveitis using ultra-widefield fluorescein angiography (FA)., Design: Cross-sectional, consecutive case series., Methods: Consecutive ultra-widefield FA images were collected from 82 uveitis patients (82 eyes) in a single center. The extent of peripheral vasculitis, capillary nonperfusion, and vessel leakage were quantified. Parameters included: (1) foveal avascular zone area and macular leakage, (2) peripheral diffuse capillary leakage and ischemia, (3) peripheral vasculitis, and (4) leakage from neovascularization. Central macular thickness measurements were derived with optical coherence tomography. Main outcome measures were correlations between central and peripheral fluorangiographic changes as well as associations between visual function, ultra-widefield FA-derived metrics, and central macular thickness., Results: Although central leakage was associated with peripheral leakage (r = 0.553, P = .001), there was no association between foveal avascular zone size and peripheral ischemia (r = 0.114, P = .324), regardless of the underlying uveitic diagnosis. Peripheral ischemia was, however, correlated to neovascularization-related leakage (r = 0.462, P = .001) and focal vasculitis (r = 0.441, P = .001). Stepwise multiple regression analysis revealed that a poor visual acuity was independently associated with foveal avascular zone size and central macular thickness (R(2)-adjusted = 0.45, P = .001)., Conclusions: We present a large cohort of patients with uveitis imaged with ultra-widefield FA and further describe novel methods for quantification of peripheral vascular pathology, in an attempt to identify visually significant parameters. Although we observed that relationships exist between peripheral vessel leakage, vasculitis, and ischemia, it was only macular ischemia and increased macular thickness that were independently associated with a reduced visual acuity., (Copyright © 2015 Elsevier Inc. All rights reserved.)

The authors present a case report of a 20-year-old patient with an atypical clinical picture of bilateral vasculitis associated with bilateral macular oedema and decrease in visual acuity. On basis of the clinical picture, frosted branch angiitis was diagnosed. The patient was instructed to continue the broad-spectrum antibiotic therapy prescribed by an ophthalmic practitioner of the appropriate catchment area. The visual acuity progressively improved and after application of systemic corticosteroid therapy the patients health state improved completely. According to the authors knowledge, this is the first diagnosed case of frosted branch angiitis in the Slovak Republic.

A 7-year-old girl with IRVAN (idiopathic retinal vasculitis, aneurysms, and neuroretinitis) syndrome was monitored for 9 years. The patient had symmetric multiple aneurysmal dilations, retinal vasculitis, and severe hard exudation in the macula bilaterally. Long-term visual acuity in her untreated right eye was 20/25. In the left eye, treated with laser panretinal photocoagulation and intravitreal vascular endothelial growth factor inhibitor therapy, visual acuity decreased to 20/200 from macular hard exudates and central fibrovascular tissue. Observation is a consideration in IRVAN syndrome if the vision remains good with hard exudation., (Copyright 2014, SLACK Incorporated.)

Purpose: To report on the clinical features and etiology of patients with retinal vasculitis (RV)., Materials and Methods: We reviewed medical records of 47 patients (75 affected eyes) diagnosed with RV. Clinical presentations, ocular complications, associated systemic diseases, and treatment regimens were registered., Results: Etiology of RV included infectious causes in 10/47, (21%) while an association with systemic and/or ocular non-infectious disorders was noted in 22/47 (47%). Eales' disease and Behcet's disease represented the most common clinical entities in non-infectious group while tuberculosis-associated RV was diagnosed in 6/10 (60%) among those with infectious disorders. RV was bilateral in 28/47 (60%) patients. Retinal veins were most commonly affected (72%, 34/47). Involvement of arteries was present in 12/47 (25%) and was associated with viral infections and Behcet's disease. Ocular complications developed in 60/75 (80%) eyes. The most common complications were elevated intraocular pressure and/or glaucoma (33/75, 44%). Retinal detachment, vitreous hemorrhage, and cystoid macular edema developed in similar percentages (15%)., Conclusions: RV in Thailand manifested mostly in male patients, was typically bilateral and involved mostly veins. Involvement of arteries was observed in patients with viral infections and Behcet's disease. Tuberculosis was the most common infectious cause.

Objective: To discuss and illustrate the role of ultra-widefield fluorescein angiography (UWFFA) in the diagnosis and management of peripheral retinal vasculitis., Design: Retrospective case series., Participants: Four consecutive patients in whom UWFFA showed far peripheral vasculitis were included. All patients were seen between May 2011 and May 2012 at the Doheny Eye Institute., Methods: Conventional fluorescein angiogram (FA) images or areas determined by the Early Treatment of Diabetic Retinopathy Study Group protocol for imaging the posterior pole and peripheral retina were superimposed on the UWFFA images. The ability to detect the extent and severity of vasculitis and vascular occlusion using both conventional FA and UWFFA was compared by 2 investigators, and any discrepancies were adjudicated by a third investigator., Results: In none of the cases was the full extent of vasculitis and capillary occlusion visible in the fields normally portrayed by conventional FA. In contrast, capillary nonperfusion and peripheral vasculitis were detectable by UWFFA in all cases. In 2 cases, the posterior extent of vasculitis could have been detected by conventional FA., Conclusions: Detection and depiction of the extent and severity of peripheral vascular changes are enhanced with UWFFA, aiding in the management of vasculitis in the retinal periphery., (Copyright © 2013 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved.)

Hemoglobinopathies are a group of inherited disorders characterized by quantitative or qualitative malformations of hemoglobin (Hb). Some of these diseases present vaso-occlusive phenomena that are responsible for high morbidity in clinical and/or ophthalmologic terms. Diagnosis of hemoglobinopathies is performed exclusively through hemoglobin electrophoresis. From the ophthalmologic perspective, the most important representative of this group of diseases is sickle cell retinopathy, which presents a wide spectrum of fundus manifestations and may even lead to irreversible vision loss if not properly diagnosed and treated. The aim of this review is to present the classification of sickle cell retinopathy and to describe current management and future perspectives for its treatment, taking into consideration the clinical management of these patients.

Purpose: To characterize a unique cytomegalovirus (CMV)-associated retinopathy in patients with limited immune dysfunction., Methods: Retrospective observational case series. CMV was confirmed as the pathogenic agent via polymerase chain reaction analysis of aqueous or vitreous humor samples or via immunohistochemical analysis of retinal biopsy specimens., Results: Five non-HIV patients with granular necrotizing retinitis, vitritis, and severe occlusive vasculopathy were identified. Patient histories all suggested a basis for limited immune dysfunction including advanced age (n = 4), diabetes mellitus (n = 4), and noncytotoxic immunotherapy (n = 3). Diagnosis of CMV retinitis was delayed in all cases and patients received either no antiviral therapy (n = 2) or incorrect antiviral therapy (n = 3) for presumed herpes simplex/varicella zoster-related acute retinal necrosis. Retinitis subsequently regressed in all cases with introduction of systemic ganciclovir/valganciclovir (n = 5) and/or intravitreal foscarnet (n = 2). Four of five patients developed neovascularization because of extensive retinal ischemia., Conclusion: The clinical expression of CMV-associated retinopathy is strongly related to immune status. In patients with limited immune dysfunction, a mixed clinical picture of intraocular inflammation with panretinal occlusive vasculopathy, more characteristic of acute retinal necrosis, and peripheral slowly progressive granular retinitis, more characteristic of classic CMV retinitis, is observed. Recognition of this atypical clinical presentation, which the authors term chronic retinal necrosis, should prompt molecular testing for CMV to determine the appropriate antiviral therapy. Consideration should also be given to prophylactic panretinal photocoagulation in such eyes, given the high risk of neovascular complications.

Background: Serpiginous choroiditis, a chronic disease affecting the choroid and retinal pigment epithelium, is characterized by acute lesions located adjacent to atrophic scars initiated from the peripapillary region. This report describes a chorioretinal disease accompanied with retinal phlebitis finally diagnosed as serpiginous choroiditis by the characteristic extension of the lesions., Case: A 68-year-old man was referred to our hospital with blurred vision in his both eyes. A small amount of cells infiltrating the vitreous, partial vitreous opacity, retinal phlebitis, grayish-white lesions around the optic disc and retinal phlebitis, and cystoid macular edema were observed in the both eyes. Since active lesions were found at the border of the inactive lesions and appeared in an interlocking polygonal pattern that spread out continuously, the condition was diagnosed as serpiginous choroiditis with retinal phlebitis. Systemic administration of predonisolone was initiated, which resolved the retinal phlebitis, and the active lesions were turned to scars. No recurrence has been observed since., Conclusion: Atypical serpiginous choroiditis accompanied with retinal phlebitis responds to systemic steroid administration.

A case of a 14 year-old boy that was admitted complaining of bilateral ocular visual blurring for 2 years is reported. The ophthalmological examination disclosed bilateral mild optic disc hyperemia and swelling, retinal exudation, few retinal hemorrhages, multiple aneurysms, as well as vasculitis. Fluorescein angiography showed extensive peripheral retinal ischemia, dilatations and hyperfluorescence of the vessels walls, and leakage of the optic disc in the late phases in both eyes. This rare case represents an entity characterized by peripheral retinal vascular occlusion, retinal vasculitis, multiple posterior retinal aneurysms, and neuroretinitis (IRVAN). Systemic evaluation and laboratory work-up did not suggest any systemic abnormality. Panretinal laser photocoagulation was performed in both eyes, and the patient was treated with oral prednisone with maintenance of the visual acuity after 1 year of follow-up. Laser treatment should be considered when angiographic evidence of widespread retinal no perfusion is present, and before the development of signs of retinal neovascularization.

To describe the use of intravitreal bevacizumab in idiopathic retinal vasculitis prior to vitrectomy and delamination. A young healthy male presented with idiopathic retinal vasculitis. His condition was refractory to panretinal photocoagulation and systemic steroids. The progression of his condition necessitated vitrectomy and delamination. Intravitreal bevacizumab was used to facilitate the surgical procedure, with effective results. Intravitreal bevacizumab can be an adjunct to vitrectomy in idiopathic retinal vasculitis. However, caution must be taken due to its ability to promote fibrosis and to produce further tractional and/or rhegmatogenous retinal detachment. Further controlled studies are required to understand its benefits in managing complicated idiopathic retinal vasculitis.

Purpose: Idiopathic retinitis, vasculitis, aneurysms, and neuroretinitis (IRVAN) is a rare syndrome that can progress rapidly to severe visual loss mainly due to the development of proliferative retinopathy despite aggressive therapeutic retinal photocoagulation or pars plana vitrectomy. We report a case of IRVAN treated with ranibizumab as an adjunctive treatment of IRVAN., Methods: Interventional case report. A 50-year-old woman was diagnosed with IRVAN. Complete ocular and systemic examination and fundus fluorescein angiography were performed to evaluate the patient. Treatment with 2 monthly intravitreal injections of 0.5 mg ranibizumab was initiated for each eye followed by panretinal photocoagulation (PRP) in the right eye (RE) and pars plana vitrectomy, endolaser, and additional PRP in the left eye (LE)., Results: Immediate regression of optic nerve neovascularization in the RE was noted after ranibizumab treatment. One year after initial presentation, best-corrected visual acuity was 20/20 in the RE and 20/50 in the LE and results of fundus examination have shown complete regression of neovascularization bilaterally., Conclusions: We propose a modified treatment modality for stage 3 IRVAN with the adjunctive use of ranibizumab. Our patient has shown complete regression of posterior segment neovascularization accompanied with significant improvement of her visual acuity. Ranibizumab seems to have encouraging results as an adjunctive treatment of IRVAN.

Purpose: To review the clinical features, disease progression, and effects of treatment on idiopathic retinitis, vasculitis, aneurysms, and neuroretinitis (IRVAN)., Design: Retrospective interventional case series., Participants: Ten patients with IRVAN originally reported in 1995 and 12 additional patients identified since the original series., Intervention: Patients in the series had testing that may have included fluorescein angiography, indocyanine green angiography, and systemic evaluation. Treatments included panretinal laser photocoagulation, cryotherapy, vitrectomy surgery, and injection of periocular or intravitreal steroids., Main Outcome Measures: Initial visual acuity (VA), initial stage at diagnosis, clinical course, surgical intervention, final VA, and complications of disease., Results: A total of 44 eyes of 22 patients were studied; 9 eyes had reached stage 1 or 2 disease at last follow-up, 17 had reached stage 3, and 12 had reached stage 4 or 5. At the time of last follow-up, 14 eyes had maintained 20/20 vision, 15 had between 20/40 and 20/200 vision, and 9 had 20/300 vision or worse. Later stages of retinal ischemia are associated with worse VA. Thirty-two of 38 followed eyes were treated. Twenty-five were treated initially with panretinal laser photocoagulation. The clinical course of each eye after initiation of panretinal laser photocoagulation was evaluated with respect to the final VA and stage of ischemic retinopathy at the initiation of treatment. Panretinal laser photocoagulation was initiated in 3 eyes at stage 2, 16 at stage 3, 5 at stage 4, and 1 at stage 5. Seven eyes underwent grid laser retinal photocoagulation of the macula for macular edema., Conclusions: Idiopathic retinitis, vasculitis, aneurysms, and neuroretinitis is an isolated retinal vascular disease that can progress rapidly to severe vision loss due to ischemic sequelae despite treatment with panretinal laser photocoagulation. Based on our review of the largest cohort of IRVAN patients, early panretinal laser photocoagulation should be considered when angiographic evidence of widespread retinal nonperfusion is present, and before (or shortly after) the development of neovascularization. A functional staging system is proposed to improve treatment paradigms.