Idiopathic Retinal Vasculitis, Aneurysms, And Neuroretinitis Research Articles

AN ATYPICAL RETINAL VASCULITIS, ANEURYSMS AND NEURORETINITIS IRVAN: RARE CASE REPORT AND MANAGEMENT

Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome is a rare and progressive disorder that predominantly affects both the eyes of young female individuals and can threaten visual function. Peripheral ischemia and macular exudation are regular ndings in patients. The treatment options include panretinal photocoagulation (PRP), systemic immunosuppression,and intravitreal antiangiogenic and corticosteroid therapy. Here we are presenting a case of old chronic IRVAN presenting as recurrent epiretinal membrane. Patient was underwent ERM removal and Dexamethasone implant to prevent recurrence in this case.

IRVAN syndrome: A retrospective review of 9 cases from Tunisia

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. 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. 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%). 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.

Comprehensive overview of IRVAN syndrome: a structured review of Case Reports and Case Series.

Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome is a rare spectrum of retinal vasculitis, aneurysms, and neuroretinitis affecting young individuals in their third decade. Most of our current knowledge is based on case reports, case series, and a handful of collaborative studies. There is much diversity in treatment approaches and outcomes in the reported literature. We have aggregated published case reports and case series into quantitative and narrative synthesis to draw evidence-based conclusions toward clinical features, atypical and rare findings, systemic associations, disease course, and treatment outcomes. The analysis suggested the disease mostly affects young individuals with a female predilection. Anterior chamber and vitreous inflammation are common than previously believed. The most prevalent pattern of retinal vasculitis in IRVAN eyes is mixed vasculitis, followed by arteritis and phlebitis. Most eyes at the time of presentation have capillary nonperfusion and require treatment. Most eyes retain good visual acuity; however, treatment is required to maintain visual function. Intravitreal antivascular endothelial growth factors administered as an adjunct to retinal laser photocoagulation are more likely to improve visual outcomes. Besides, we have discussed the different hypotheses on the etiopathogenesis of the disease and stronger evidence suggests an inflammatory origin of the disease.

Idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN): a case report and outcomes of 1.5-year observation

ENWEndNote BIBJabRef, Mendeley RISPapers, Reference Manager, RefWorks, Zotero AMA Świerczyńska M, Sedlak L, Wąs P, Nowak M, Dorecka M, Mrukwa-Kominek E. Idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN): a case report and outcomes of 1.5-year observation. Klinika Oczna / Acta Ophthalmologica Polonica. 2022. doi:10.5114/ko.2022.118406. APA Świerczyńska, M., Sedlak, L., Wąs, P., Nowak, M., Dorecka, M., & Mrukwa-Kominek, E. (2022). Idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN): a case report and outcomes of 1.5-year observation. Klinika Oczna / Acta Ophthalmologica Polonica. https://doi.org/10.5114/ko.2022.118406 Chicago Świerczyńska, Marta, Lech Sedlak, Paweł Wąs, Marta Nowak, Mariola Dorecka, and Ewa Mrukwa-Kominek. 2022. "Idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN): a case report and outcomes of 1.5-year observation". Klinika Oczna / Acta Ophthalmologica Polonica. doi:10.5114/ko.2022.118406. Harvard Świerczyńska, M., Sedlak, L., Wąs, P., Nowak, M., Dorecka, M., and Mrukwa-Kominek, E. (2022). Idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN): a case report and outcomes of 1.5-year observation. Klinika Oczna / Acta Ophthalmologica Polonica. https://doi.org/10.5114/ko.2022.118406 MLA Świerczyńska, Marta et al. "Idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN): a case report and outcomes of 1.5-year observation." Klinika Oczna / Acta Ophthalmologica Polonica, 2022. doi:10.5114/ko.2022.118406. Vancouver Świerczyńska M, Sedlak L, Wąs P, Nowak M, Dorecka M, Mrukwa-Kominek E. Idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN): a case report and outcomes of 1.5-year observation. Klinika Oczna / Acta Ophthalmologica Polonica. 2022. doi:10.5114/ko.2022.118406.

A Late Presentation of Unilateral Idiopathic Retinal Vasculitis, Aneurysms, and Neuroretinitis (IRVAN) Syndrome

Although uncommon, idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome can lead to vision threatening complications if not diagnosed and treated appropriately. This case report describes an atypical presentation of unilateral IRVAN syndrome, which resulted in significant visual loss in an elderly male. CE Notification: This article is available as a COPE accredited CE course. You may take this course for 1-hour credit. Read the article and take the qualifying test to earn your credit. Click here to Enroll (https://www.crojournal.com/a-late-presentation-of-unilateral-idiopathic-retinal-vasculitis-aneurysms-and-neuroretinitis-irvan-syndrome)

Multimodal imaging in IRVAN syndrome presenting with Branch Retinal Artery Occlusion.

To describe multimodal imaging findings in a patient with idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome presenting with branch retinal artery occlusion (BRAO). A 33-year-old woman presented with acute BRAO in the right eye. A diagnosis of underlying IRVAN syndrome was made based on the presence of arteriolar aneurysms on the optic disc and along major arterioles and faint retinal hard exudates in both eyes. Eight months later, best-corrected visual acuity was 20/25 in the right eye and 20/20 in the left eye. The hard exudates had increased, and there were extensive areas of peripheral retinal capillary nonperfusion without new vessels. Optical coherence tomography (OCT) showed a localized retinal thinning corresponding to the prior BRAO. Fundus autofluorescence showed nodulo-linear periarterial hypoautofluorescence. OCT angiography (OCTA) showed localized ischemic changes, mainly involving the deep capillary plexus, corresponding to the area of resolved BRAO. It also clearly delineated the optic disc aneurysms. The patient received bilateral scatter laser photocoagulation directed to areas of peripheral capillary nonperfusion. Over a 6-month follow-up period, visual acuity remained unchanged, and there was no evidence of disease progression. Multimodal imaging, including fundus autofluorescence, OCT, and OCTA can provide additional valuable information in the evaluation of IRVAN syndrome complicated with BRAO.

Is There a True Neuroretinitis in Idiopathic Retinal Vasculitis, Aneurysms, and Neuroretinitis (IRVAN) Syndrome ?

ABSTRACT Purpose : To clarify whether a true neuroretinitis is part of idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome.Methods : Critical literature review.Results : Neuroretinitis has been considered as a prominent feature of IRVAN syndrome. It has been described as late diffuse optic disc staining on fluorescein angiography. However, patients with IRVAN syndrome usually do not present with visual function alterations consistent with optic neuropathy or neuroretinitis. The typical stellate exudative maculopathy seen in IRVAN syndrome is primarily caused by leakage from retinal arteriolar aneurysms at or near the optic disc, and it could be misinterpreted as a feature of neuroretinitis.Conclusion : There is no clinical evidence of true neuroretinitis at any of the disease stages in patients with IRVAN syndrome. The acronym « IRVARE » (Idiopathic Retinal Vasculitis, Aneurysms, and Retinal Exudates) is proposed to better characterize this syndrome.

A new perspective for analyzing clinical characteristics of idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome.

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. Six patients with IRVAN syndrome (1 male and 5 females, age 5-38years) 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. 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. In IRVAN syndrome, the number and location of retinal aneurysms correlate with the size of retinal non-perfusion area and type of neovascularization.

Long-term outcomes in a series of idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome

ObjectiveIdiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome is a rare entity with a potentially poor visual prognosis. Our objective is to review the clinical presentation and long-term outcomes of patients with IRVAN syndrome. DesignThis is a retrospective case series. MethodsWe reviewed the charts of all the patients diagnosed with IRVAN syndrome at our tertiary care centre from 2002 to 2015. ResultsWe included the long-term clinical outcomes of 7 eyes (5 patients) diagnosed with IRVAN syndrome. After a mean follow-up of 84.9 months, best-corrected visual acuity was 20/40 or better in the majority of eyes (70%). Four (57.1%) patients had systemic conditions, namely, multiple sclerosis, ischemic stroke, and positive antiphospholipid titres. All eyes were treated with laser photocoagulation. Four (40%) eyes received adjunctive intravitreal bevacizumab injections. ConclusionIRVAN is an important diagnosis for clinicians to recognize. When treated in a timely manner, long-term visual outcomes can be favourable.

Multimodal imaging questions etiology of idiopathic retinal vasculitis, aneurysms and neuroretinitis syndrome (IRVAN syndrome)

Idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) syndrome is a rare entity of unknown etiology for which many hypotheses have been proposed with inflammation being the most commonly accepted hypothesis. We report cases of a 9 year old girl and a 22 year old male patient with diagnosis of IRVAN syndrome. The conclusions drawn from the clinical examination and multimodal imaging including optical coherence tomography angiography of the patients are discussed. Our conclusions and interpretation point towards IRVAN being a developmental vascular anomaly rather than a consequence of inflammation. It is thus proposed to be renamed as Idiopathic retinal arteriolar aneurysm syndrome (IRAA).

Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome: clinical perspectives.

Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome typically affects young, healthy individuals. Despite the dramatic fundus appearance seen in this syndrome, these patients are usually asymptomatic. The syndrome includes peculiar vascular abnormalities in the form of multiple aneurysmal dilatations seen along retinal arterioles and optic nerve-head arterioles, which are best appreciated on fluorescein angiography. Neuroretinitis and retinal vasculitis are seen in all patients, and manifested by staining of the optic nerve head and diffuse leakage from vessels, mainly arterioles, on fluorescein angiography. The devastating vision-threatening outcomes of this syndrome include exudative retinopathy and extensive peripheral retinal nonperfusion areas, which can eventually lead to neovascularization. This review summarizes current knowledge on the variable clinical aspects of this disease, highlighting diagnostic and treatment strategies.

Vanishing retinal arterial aneurysms with anti-tubercular treatment in a patient presenting with idiopathic retinal vasculitis, aneurysms, and neuroretinitis.

BackgroundIdiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome presents with characteristic clinical manifestations such as aneurysms at arteriolar bifurcations and optic nerve and retinal vascular inflammation. Regression of such features on treatment with anti-tubercular therapy (ATT) combined with corticosteroids has not been reported in literature.FindingsA 30-year-old female with sudden painless decreased vision in the left eye was referred with a diagnosis of presumed tuberculous retinal vasculitis and a positive tuberculin skin test. Based on the clinical and angiographic features of the right eye, a diagnosis of IRVAN syndrome was made. In the left eye, the patient had vitreous hemorrhage for which pars plana vitrectomy was performed. The vitreous sample was positive for Mycobacterium tuberculosis using multiplex polymerase chain reaction, and the patient was started on standard four-drug ATT and oral corticosteroids. At 6-month follow-up, vanishing of retinal arterial aneurysms was observed.ConclusionsThe pathogenesis of IRVAN syndrome is uncertain. One of the postulates is that the features of arterial aneurysms and other retinal vascular alterations occur secondary to acquired inflammatory reaction. We hypothesize that IRVAN syndrome may be a morphological diagnosis possibly associated with various entities, one of which could be ocular tuberculosis. It may be prudent to rule out intraocular tuberculosis in cases labeled as IRVAN syndrome in an endemic population.

Combination Anti-VEGF and Corticosteroid Therapy for Idiopathic Retinal Vasculitis, Aneurysms, and Neuroretinitis Syndrome

Vision loss associated with the idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome most commonly occurs from macular edema or complications related to neovascularization. The authors present a case of advanced IRVAN associated with a massive exudative response characterized by peripheral retinal telangiectasias, exudative retinal detachment, and macular edema with lipid maculopathy. The patient was managed successfully with visual acuity from hand motion to 20/150 using a combination of local corticosteroids, intravitreal bevacizumab, panretinal photocoagulation, and eventually pars plana vitrectomy for progressive vitreomacular traction. VEGF- and non-VEGF-mediated mechanisms appear to be involved in the pathogenesis of IRVAN given the efficacy of combination therapy. [ophthalmic surg lasers imaging retina. 2013;44:599-602.].

Imaging in the Diagnosis and Management of Idiopathic Retinal Vasculitis, Aneurysms, and Neuroretinitis (IRVAN)

Imaging in the Diagnosis and Management of Idiopathic Retinal Vasculitis, Aneurysms, and Neuroretinitis (IRVAN)

Spontaneous branch artery occlusion in idiopathic retinitis, vasculitis, aneurysms, and neuroretinitis syndrome despite panretinal laser photocoagulation of widespread retina nonperfusion

Editor, I diopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome is an exceptional form of arteries disease. The diagnosis is based on three major criteria (retinal vasculitis, aneurysmal dilatations at arterial bifurcations, and neuroretinitis) and three minor criteria (peripheral capillary nonperfusion, retinal neovascularization, and macular exsudation) (Chang et al. 1995). In most cases, visual loss is because of peripheral neovascular retinopathy with vitreous haemorrhage secondary to peripheral nonperfusion or to exsudative maculopathy with retro-foveal exsudate. One case of branch artery occlusion has been reported after direct laser of the aneurysm. To our knowledge, spontaneous occlusion of first-order artery has not been described. Here, we report a spontaneous branch artery occlusion that occurred after prior peripheral laser photocoagulation as recommended before or shortly after the development of neovascularization. We present a case report describing a young patient affected with idiopathic retinitis, vasculitis, aneurysms, and neuroretinitis syndrome (IRVAN) who developed spontaneously branch artery occlusion after the photocoagulation of the no-perfuse peripheral retinal surface. A 29-year-old woman presented at our department for scotoma and decreased vision in her left eye (LE) (20 ⁄ 50). She presented with a history of sudden visual decrease in the LE 4 days ago. She had no known systemic illnesses. Infectious, autoinflammatory, and vascular causes were eliminated. Preliminary aetiological investigations were negatives. The patient signed a comprehensive consent form, according to Good Clinical Practice guidelines and French legislation, before proceeding any examinations or treatments. Slit-lamp biomicroscopy did not reveal any abnormality. On fundus examination and photographs of both eyes, we observed an extensive peripapillary exudation, arterial narrowing, and aneurysmal dilatations along the arteries. In the LE, the inferotemporal area showed a yellow-white ischemic retina (Fig. 1A). The spectral domain optical coherence tomography (SD-OCT) (SD-OCT, Cirrus; Carl Zeiss Meditec, Dublin, CA, USA) confirmed the presence of exudation translated as focal hyperreflectivity in the plexiform outer layer with any modification of the retinal pigment epithelium (RPE) or cystoid macular oedema (Fig. 1B). Fluorescein angiography of the LE illustrated the macroaneurysmal dilatations along the arterial tree leakage. The left inferotemporal arterial flow stopped after a macroaneurysm and delayed filling of the corresponding vein (Fig. 1C). A retrograde arterial staining was observed along the FA sequence (Fig. 1D). Photocoagulation peripheral retinal scars were also seen in the both eyes. After discussing treatment options, both antiaggregants and steroids were introduced. Two months later, arterial flow was partially restored without extensive photocoagulation on fluorescein angiography (Fig. 2A early stage FA, Fig. 2B late stage FA), but the patient had no visual acuity improve-

Spontaneous Resolution of Macular Exudation with Good Visual Recovery in Idiopathic Retinal Vasculitis, Aneurysms, and Neuroretinitis (IRVAN): A Case Report

A 14-year-old boy presented with decreased visual acuity in both eyes of three-months duration. Fundus examination revealed macular exudation in both eyes with aneurysmal changes at the arteriolar bifurcations. Fluorescein angiography confirmed the presence of numerous aneurysmal dilatations of the retinal arterioles and extensive peripheral capillary nonperfusion areas. A provisional diagnosis of idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) was made. Scatter photocoagulation was advised in view of the extensive capillary nonperfusion areas. The patient was then lost to follow-up. He presented a year later with bilateral nonresolving vitreous hemorrhage, more severe in the right eye. He underwent right pars plana vitrectomy. Intraoperatively, the fundus features were similar to those at the initial presentation, but with the absence of any macular exudation. IRVAN represents an evolving clinical condition, with ill-defined clinical aspects, particularly with respect to management. In view of the spontaneous absorption of the macular exudates, we would like to suggest a cautious approach to applying photocoagulation of the retinal aneurysms.

Extensive dynamics in location, shape, and size of aneurysms in a patient with idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome

PurposeTo describe an unusual case of idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome with rapid dynamics in the number and appearance of the aneurysms. DesignObservational case report. MethodsClinical and angiographic data of the patient were reviewed. ResultsIn the course of only 6 months, preexisting retinal aneurysms resolved while new ones appeared. Changes were observed in the shape and size of preexisting lesions. The resolution of lesions in eyes previously untreated by laser is reported for the first time. ConclusionsVascular lesions in IRVAN syndrome may show an unusually rapid turnover. The resolution of aneurysms is a part of the natural course of the disease and may occur without previous retinal laser photocoagulation.