Characteristics of multimodal fundus imaging in patients with idiopathic retinal vasculitis, aneurysms and neuroretinitis syndrome_Chinese Journal of Ocular Fundus Diseases

Authors：

Jia Wei ,  Wang Haiyan , Zhang Lei , Wang Ru , Fang Qi , Lu Yina

Shaanxi Eye Hospital, Xi'an People's Hospital (Xi'an Fourth Hospital), Xi'an Key Laboratory of Digital Medical Technology of Ophthalmologic Imaging, Xi'an 710004, China;

Corresponding author：

Wang Haiyan, Email: whyeye@126.com

Keywords：

Retinal vasculitis; Retinitis; Retinal aneurysm; Neuroretinitis; Syndrome

DOI：

10.3760/cma.j.cn511434-20240823-00323

Video：

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Objective To observe and analyze the multimodal imaging characteristics of fundus in patients with idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) syndrome. Methods A retrospective study. From June 2015 to March 2024, 6 patients (11 eyes) diagnosed with IRVAN syndrome in Shaanxi Eye Hospital were included in the study. All patients underwent examinations including best-corrected visual acuity (BCVA), color fundus photography, fluorescein fundus angiography (FFA), optical coherence tomography (OCT) and OCT angiography (OCTA). At the same time, FFA combined with indocyanine green angiography (ICGA) was performed in 6 eyes. Follow-up ranged from 2 to 23 months. Multimodal imaging features were analyzed retrospectively. The number of retinal aneurysms detected by FFA, ICGA, and OCTA was compared by using the Wilcoxon signed-rank test. Results In 11 eyes of 6 cases, a total of 1 male (2 eyes) and 5 females (9 eyes) with the mean age of (31.67±12.91) years were included in this cohort. Color fundus photography showed clear optic disc boundaries in 5 eyes, optic disc aneurysms in 8 eyes, retinal aneurysms in 4 eyes; exudation in 9 eyes, localized around aneurysms. On OCT, vitreous high reflective dots and epiretinal membrane on optic disc in all 11 eyes, and macular epiretinal membrane in 3 eyes were revealed. FFA showed optic disc aneurysms and retinal aneurysms in 9 eyes, late optic disc hyperfluorescence in 11 eyes; local arterial leakage in 3 eyes, local venous leakage in 8 eyes, non-perfusion area in all 11 eyes, and retina neovascularization in 3 eyes. Optic disc aneurysms and retinal aneurysms in 5 eyes (total 18 aneurysms) on ICGA were shown compared with optic disc aneurysms in 4 eyes and retinal aneurysms in 5 eyes (total 13 aneurysms) on simultaneous FFA. OCTA revealed neovascularization on the optic disc in 2 eyes, optic disc aneurysmsin 8 eyes, retinal aneurysms in 1 eye (total 2 aneurysms); while on simultaneous FFA, optic disc aneurysms in 8 eyes and retinal aneurysms in 3 eyes (total 5 aneurysms) with no optic disc were displayed. During OCTA follow-up, new aneurysms appeared at the bifurcation of arteries with an increasing angle between them and non-perfusion area enlargement on FFA. Compared with FFA and ICGA, OCTA in detecting the number of aneurysms had no statistics significance (Z=−1.342, −1.342; P＞0.05). Conclusion Multimodal imaging can demonstrate characteristics of IRVAN syndrome, ICGA provides superior visualization of optic disc and retinal aneurysms, while OCTA confirms optic disc neovascularization and enlargement of artery angles at arterial bifurcations.

1.	Chang TS, Aylward GW, Davis JL, et al. Idiopathic retinal vasculitis, aneurysms, and neuro-retinitis[J]. Ophthalmology, 1995, 102(7): 1089-1097. DOI: 10.1016/s0161-6420(95)30907-4.
2.	Chawla R, Kumar A, Ravani R, et al. Multimodal imaging questions etiology of idiopathic retinal vasculitis, aneurysms and neuroretinitis syndrome (IRVAN syndrome)[J]. Med Hypotheses, 2018, 111: 12-14. DOI: 10.1016/j.mehy.2017.10.031.
3.	Tripathy K. Pathogenesis of idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) or 'idiopathic retinal arteriolar aneurysms (IRAA)' with macular star[J]. Med Hypotheses, 2018, 112: 65-66. DOI: 10.1016/j.mehy.2018.01.016.
4.	Khairallah M, Khochtali S, Ksiaa I. Is there a true neuroretinitis in idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome?[J]. Ocul Immunol Inflamm, 2022, 30(4): 845-847. DOI: 10.1080/09273948.2020.1836234.
5.	Massicotte E, Hassanaly S, Bélair ML, et al. Long-term outcomes in a series of idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome[J]. Can J Ophthalmol, 2018, 53(5): 435-440. DOI: 10.1016/j.jcjo.2017.12.015.
6.	Bajgai P, Katoch D, Dogra MR, et al. Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome: clinical perspectives[J]. Clin Ophthalmol, 2017, 11: 1805-1817. DOI: 10.2147/OPTH.S128506.
7.	Samuel MA, Equi RA, Chang TS, et al. Idiopathic retinitis, vasculitis, aneurysms, and neuroretinitis (IRVAN): new observations and a proposed staging system[J]. Ophthalmology, 2007, 114(8): 1526-1529. DOI: 10.1016/j.ophtha.2006.11.014.
8.	Govindaraj I, Kuriakose A, Rajendran A. Enlarging aneurysm with paracentral acute middle maculopathy in idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN)-a case report[J]. Eur J Ophthalmol, 2024, 34(1): 70-74. DOI: 10.1177/11206721231174259.
9.	Sashihara H, Hayashi H, Oshima K. Regression of retinal arterial aneurysms in a case of idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN)[J]. Retina, 1999, 19(3): 250-251. DOI: 10.1097/00006982-199903000-00014.
10.	Zhang Y, Chang T, Ge G, et al. Extensive dynamics of aneurysms and long-term prognosis in irvan syndrome: a case series[J]. Ocul Immunol Inflamm, 2022, 30(3): 623-627. DOI: 10.1080/09273948.2020.1821902.
11.	王光璐, 卢宁, 王明扬. IRVAN综合征的临床特征和治疗[J]. 眼科研究, 2006, 24(2): 191-194. DOI: 10.3760/cma.j.issn.2095-0160.2006.02.021.Wang GL, Lu N, Wang MY. Clincal feature and treatment of IRVAN syndrome[J]. Chin Ophthal Res, 2006, 24(2): 191-194. DOI: 10.3760/cma.j.issn.2095-0160.2006.02.021.
12.	苗娟, 张娟美, 张迎秋, 等. 特发性视网膜血管炎、视网膜动脉瘤、视神经视网膜炎综合征11例[J], 中华眼底病杂志, 2017, 33(1): 68-69. DOI: 10.3760/cma.j.issn.1005-1015.2017.01.018.Miao J, Zhang JM, Zhang YQ, et al. Idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome 11 cases[J]. Chin J Ocul Fundus Dis, 2017, 33(1): 68-69. DOI: 10.3760/cma.j.issn.1005-1015.2017.01.018.
13.	Sigler EJ, Grosso A. Idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome[J/OL]. Retina, 2016, 36(3): e16-e17[2016-03-01]. https://pubmed.ncbi.nlm.nih.gov/26441263/. DOI: 10.1097/IAE.0000000000000769.
14.	Ouederni M, Sassi H, Chelly Z, et al. Optical coherence tomography angiography in idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) syndrome: a case report[J]. Eur J Ophthalmol, 2022, 32(1): 144-148. DOI: 10.1177/1120672120957593.
15.	……[M]//黎晓新. 视网膜血管性疾病. 北京: 人民卫生出版社, 2017: 5(此处应该是所引章节的页码, 而不只是所引观点的所在页码).M]//Li XX. Retinal vascular diseases. Beijing: People's Medical Publishing House, 2017: 5.
16.	Ali Khan H, Ali Khan Q, Shahzad MA, et al. Comprehensive overview of IRVAN syndrome: a structured review of case reports and case series[J/OL]. Ther Adv Ophthalmol, 2022, 14: 25158414211070880[2021-01-21]. https://pubmed.ncbi.nlm.nih.gov/35282003/. DOI: 10.1177/25158414211070880.
17.	Krishnan R, Shah P, Thomas D. Subacute idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) in a child and review of paediatric cases of IRVAN revealing preserved capillary perfusion as a more common feature[J]. Eye (Lond), 2015, 29(1): 145-147. DOI: 10.1038/eye.2014.213.
18.	Liu XC, Zhang MN, Chen B, et al. A new perspective for analyzing clinical characteristics of idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome[J]. Int Ophthalmol, 2019, 39(7): 1475-1482. DOI: 10.1007/s10792-018-0962-7.
19.	Abdelhakim A, Rasool N. Neuroretinitis: a review[J]. Curr Opin Ophthalmol, 2018, 29(6): 514-519. DOI: 10.1097/ICU.0000000000000527.
20.	Balaratnasingam C, Yannuzzi LA, Franchina M, et al. Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (irvan) in a patient without irvan history[J]. JAMA Ophthalmol, 2019, 137(2): 230-232. DOI: 10.1001/jamaophthalmol.2018.5247.
21.	严密, 廖菊生, 张美霞, 等. 特发性视网膜血管炎、视网膜动脉瘤、视神经视网膜炎综合征临床观察[J], 中华眼底病杂志, 2007, 23(3): 180-183. DOI: 10.3760/j.issn:1005-1015.2007.03.008.Yan M, Liao JS, Zhang MX, et al. Three cases with idiopathic retinaI vascuIitis, aneurysms, and neuroretinitis syndrome[J]. Chin J Ocul Fundus Dis, 2007, 23(3): 180-183. DOI: 10.3760/j.issn:1005-1015.2007.03.008.

1. Chang TS, Aylward GW, Davis JL, et al. Idiopathic retinal vasculitis, aneurysms, and neuro-retinitis[J]. Ophthalmology, 1995, 102(7): 1089-1097. DOI: 10.1016/s0161-6420(95)30907-4.
2. Chawla R, Kumar A, Ravani R, et al. Multimodal imaging questions etiology of idiopathic retinal vasculitis, aneurysms and neuroretinitis syndrome (IRVAN syndrome)[J]. Med Hypotheses, 2018, 111: 12-14. DOI: 10.1016/j.mehy.2017.10.031.
3. Tripathy K. Pathogenesis of idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) or 'idiopathic retinal arteriolar aneurysms (IRAA)' with macular star[J]. Med Hypotheses, 2018, 112: 65-66. DOI: 10.1016/j.mehy.2018.01.016.
4. Khairallah M, Khochtali S, Ksiaa I. Is there a true neuroretinitis in idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome?[J]. Ocul Immunol Inflamm, 2022, 30(4): 845-847. DOI: 10.1080/09273948.2020.1836234.
5. Massicotte E, Hassanaly S, Bélair ML, et al. Long-term outcomes in a series of idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome[J]. Can J Ophthalmol, 2018, 53(5): 435-440. DOI: 10.1016/j.jcjo.2017.12.015.
6. Bajgai P, Katoch D, Dogra MR, et al. Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome: clinical perspectives[J]. Clin Ophthalmol, 2017, 11: 1805-1817. DOI: 10.2147/OPTH.S128506.
7. Samuel MA, Equi RA, Chang TS, et al. Idiopathic retinitis, vasculitis, aneurysms, and neuroretinitis (IRVAN): new observations and a proposed staging system[J]. Ophthalmology, 2007, 114(8): 1526-1529. DOI: 10.1016/j.ophtha.2006.11.014.
8. Govindaraj I, Kuriakose A, Rajendran A. Enlarging aneurysm with paracentral acute middle maculopathy in idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN)-a case report[J]. Eur J Ophthalmol, 2024, 34(1): 70-74. DOI: 10.1177/11206721231174259.
9. Sashihara H, Hayashi H, Oshima K. Regression of retinal arterial aneurysms in a case of idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN)[J]. Retina, 1999, 19(3): 250-251. DOI: 10.1097/00006982-199903000-00014.
10. Zhang Y, Chang T, Ge G, et al. Extensive dynamics of aneurysms and long-term prognosis in irvan syndrome: a case series[J]. Ocul Immunol Inflamm, 2022, 30(3): 623-627. DOI: 10.1080/09273948.2020.1821902.
11. 王光璐, 卢宁, 王明扬. IRVAN综合征的临床特征和治疗[J]. 眼科研究, 2006, 24(2): 191-194. DOI: 10.3760/cma.j.issn.2095-0160.2006.02.021.Wang GL, Lu N, Wang MY. Clincal feature and treatment of IRVAN syndrome[J]. Chin Ophthal Res, 2006, 24(2): 191-194. DOI: 10.3760/cma.j.issn.2095-0160.2006.02.021.
12. 苗娟, 张娟美, 张迎秋, 等. 特发性视网膜血管炎、视网膜动脉瘤、视神经视网膜炎综合征11例[J], 中华眼底病杂志, 2017, 33(1): 68-69. DOI: 10.3760/cma.j.issn.1005-1015.2017.01.018.Miao J, Zhang JM, Zhang YQ, et al. Idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome 11 cases[J]. Chin J Ocul Fundus Dis, 2017, 33(1): 68-69. DOI: 10.3760/cma.j.issn.1005-1015.2017.01.018.
13. Sigler EJ, Grosso A. Idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome[J/OL]. Retina, 2016, 36(3): e16-e17[2016-03-01]. https://pubmed.ncbi.nlm.nih.gov/26441263/. DOI: 10.1097/IAE.0000000000000769.
14. Ouederni M, Sassi H, Chelly Z, et al. Optical coherence tomography angiography in idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) syndrome: a case report[J]. Eur J Ophthalmol, 2022, 32(1): 144-148. DOI: 10.1177/1120672120957593.
15. ……[M]//黎晓新. 视网膜血管性疾病. 北京: 人民卫生出版社, 2017: 5(此处应该是所引章节的页码, 而不只是所引观点的所在页码).M]//Li XX. Retinal vascular diseases. Beijing: People's Medical Publishing House, 2017: 5.
16. Ali Khan H, Ali Khan Q, Shahzad MA, et al. Comprehensive overview of IRVAN syndrome: a structured review of case reports and case series[J/OL]. Ther Adv Ophthalmol, 2022, 14: 25158414211070880[2021-01-21]. https://pubmed.ncbi.nlm.nih.gov/35282003/. DOI: 10.1177/25158414211070880.
17. Krishnan R, Shah P, Thomas D. Subacute idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) in a child and review of paediatric cases of IRVAN revealing preserved capillary perfusion as a more common feature[J]. Eye (Lond), 2015, 29(1): 145-147. DOI: 10.1038/eye.2014.213.
18. Liu XC, Zhang MN, Chen B, et al. A new perspective for analyzing clinical characteristics of idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome[J]. Int Ophthalmol, 2019, 39(7): 1475-1482. DOI: 10.1007/s10792-018-0962-7.
19. Abdelhakim A, Rasool N. Neuroretinitis: a review[J]. Curr Opin Ophthalmol, 2018, 29(6): 514-519. DOI: 10.1097/ICU.0000000000000527.
20. Balaratnasingam C, Yannuzzi LA, Franchina M, et al. Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (irvan) in a patient without irvan history[J]. JAMA Ophthalmol, 2019, 137(2): 230-232. DOI: 10.1001/jamaophthalmol.2018.5247.
21. 严密, 廖菊生, 张美霞, 等. 特发性视网膜血管炎、视网膜动脉瘤、视神经视网膜炎综合征临床观察[J], 中华眼底病杂志, 2007, 23(3): 180-183. DOI: 10.3760/j.issn:1005-1015.2007.03.008.Yan M, Liao JS, Zhang MX, et al. Three cases with idiopathic retinaI vascuIitis, aneurysms, and neuroretinitis syndrome[J]. Chin J Ocul Fundus Dis, 2007, 23(3): 180-183. DOI: 10.3760/j.issn:1005-1015.2007.03.008.

Chinese Journal of Ocular Fundus Diseases

Latest ArticlesCharacteristics of multimodal fundus imaging in patients with idiopathic retinal vasculitis, aneurysms and neuroretinitis syndrome

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