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：

Export PDF Favorites Scan Get Citation

Abstract Full text Figures/Tables Video References Cited by

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.

Citation： Jia Wei, Wang Haiyan, Zhang Lei, Wang Ru, Fang Qi, Lu Yina. Characteristics of multimodal fundus imaging in patients with idiopathic retinal vasculitis, aneurysms and neuroretinitis syndrome. Chinese Journal of Ocular Fundus Diseases, 2025, 41(5): 373-380. doi: 10.3760/cma.j.cn511434-20240823-00323 Copy

1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.	苗娟, 張娟美, 張迎秋, 等. 特發性視網膜血管炎、視網膜動脈瘤、視神經視網膜炎綜合征11例[J], 中華眼底病雜志, 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.
15.	黎曉新, 廖菊生. 視網膜血管的組織解剖和功能[M]//黎曉新. 視網膜血管性疾病. 北京: 人民衛生出版社, 2017: 5-12.Li XX, Liao JS. The histological anatomy and function of retinal blood vessels[M]//Li XX. Retinal vascular diseases. Beijing: People's Medical Publishing House, 2017: 5-12.
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.
18.
19.
20.
21.	嚴密, 廖菊生, 張美霞, 等. 特發性視網膜血管炎、視網膜動脈瘤、視神經視網膜炎綜合征臨床觀察[J], 中華眼底病雜志, 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.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12. 苗娟, 張娟美, 張迎秋, 等. 特發性視網膜血管炎、視網膜動脈瘤、視神經視網膜炎綜合征11例[J], 中華眼底病雜志, 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.
15. 黎曉新, 廖菊生. 視網膜血管的組織解剖和功能[M]//黎曉新. 視網膜血管性疾病. 北京: 人民衛生出版社, 2017: 5-12.Li XX, Liao JS. The histological anatomy and function of retinal blood vessels[M]//Li XX. Retinal vascular diseases. Beijing: People's Medical Publishing House, 2017: 5-12.
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.
18.
19.
20.
21. 嚴密, 廖菊生, 張美霞, 等. 特發性視網膜血管炎、視網膜動脈瘤、視神經視網膜炎綜合征臨床觀察[J], 中華眼底病雜志, 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

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

Abstract Full text Figures/Tables Video References Cited by

Previous Article

Next Article

Format

Content