Primary Branch Retinal Artery Occlusion in Idiopathic Retinal Vasculitis, Aneurysms, and Neuroretinitis Syndrome Associated With Hyperhomocysteinemia

Parchand, Swapnil; Bhalekar, Swapnil; Ak, Gupta; Singh, Ramandeep

doi:10.1097/icb.0b013e31823c1289

Cited by 8 publications

(13 citation statements)

References 7 publications

(5 reference statements)

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“…Secondary vascular occlusions after krypton-laser photocoagulation of aneurysmal dilatations have been reported, but primary appearance of vascular occlusion is a very rare phenomenon and has been reported in only two cases. 31 Parchand et al 32 reported a patient who on detailed workup for inflammatory and infectious vascular diseases was found to have elevated levels of homocysteine. Similarly, Venkatesh et al 33 found a patient to have combined arteriolar and venular obstruction unrelated to laser photocoagulation.…”

Section: Classification and Clinical Featuresmentioning

confidence: 99%

“…However, direct photocoagulation of leaking macroaneurysms is contraindicated, as it leads to occlusion of blood vessels originating at the site of vessel burn. 32 The precise time to initiate treatment is not clearly known, as we do not wait for neovascularization in these cases. Rouvas et al 59 advocated deferral of PRP when peripheral ischemia is present in fewer than two quadrants of the retina.…”

Section: Treatmentmentioning

confidence: 99%

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

Bajgai¹,

Katoch²,

Dogra³

et al. 2017

OPTH

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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.

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Section: Classification and Clinical Featuresmentioning

confidence: 99%

Section: Treatmentmentioning

confidence: 99%

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

Bajgai¹,

Katoch²,

Dogra³

et al. 2017

OPTH

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“…While a number of studies have suggested a diverse range of treatment options for this condition, the exact pathogenesis of IRVAN syndrome still remains unknown. Associations of IRVAN syndrome with allergic fungal sinusitis [ 5 ], elevated intracranial pressure [ 6 ], hyperhomocysteinemia [ 7 ], and p-ANCA [ 8 ] have been reported in literature. In addition, similarities in the pathogenesis of IRVAN syndrome and presumed tuberculous retinal vasculitis have been suggested [ 9 ].…”

Section: Resultsmentioning

confidence: 99%

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

Singh

Sharma

Agarwal

et al. 2016

J Ophthal Inflamm Infect

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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.

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“…Subsequent fibrovascular proliferation may lead to tractional retinal detachment. 6 Other less frequent retinal associations/coincidental findings include branch retinal artery occlusion, 29 , 34 – 36 branch retinal vein occlusion, 27 , 29 peripheral or midperipheral microvascular occlusions, 13 , 37 , 38 serous neurosensory detachments, 6 , 33 , 39 vitreomacular traction, 28 and rarely retinal degeneration, 18 , 40 optic atrophy, 40 and choroidal neovascularization 41 ( Figure 3 ).…”

Section: Discussionmentioning

confidence: 99%

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

Khan

Shahzad

et al. 2022

Ophthalmol Eye Dis

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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.

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Primary Branch Retinal Artery Occlusion in Idiopathic Retinal Vasculitis, Aneurysms, and Neuroretinitis Syndrome Associated With Hyperhomocysteinemia

Cited by 8 publications

References 7 publications

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

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

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

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

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