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 Table of Contents  
CASE REPORT
Year : 2023  |  Volume : 3  |  Issue : 1  |  Page : 92-96

Macular deep capillary nonperfusion in idiopathic retinal vasculitis, aneurysm, and neuroretinitis syndrome


Department of Ophthalmology, The Second Hospital of Jilin University, Changchun, China

Date of Submission16-Apr-2022
Date of Acceptance16-Aug-2022
Date of Web Publication20-Jan-2023

Correspondence Address:
Jia Li
The Second Hospital of Jilin University, Jilin Province, Changchun
China
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_941_22

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  Abstract 


Here, we report a rare case of a 47-year-old man presenting with idiopathic retinal vasculitis, aneurysm, and neuroretinitis (IRVAN) syndrome combined with primary open-angle glaucoma (POAG). He was treated with retinal photocoagulation combined with intraocular pressure (IOP)-lowering therapy. At the 1-year follow-up, the patient's vision had not decreased and the IOP was well controlled. The abnormality of this case is the nonperfusion of macular deep capillary. Many studies support that POAG has no effects on the ischemia of deep capillary plexus. This case suggests that ischemia in the deep capillary plexus should be considered in IRVAN syndrome.

Keywords: IRVAN syndrome, ischemia, macular


How to cite this article:
Wang Y, Li Y, Zhang W, Li J. Macular deep capillary nonperfusion in idiopathic retinal vasculitis, aneurysm, and neuroretinitis syndrome. Indian J Ophthalmol Case Rep 2023;3:92-6

How to cite this URL:
Wang Y, Li Y, Zhang W, Li J. Macular deep capillary nonperfusion in idiopathic retinal vasculitis, aneurysm, and neuroretinitis syndrome. Indian J Ophthalmol Case Rep [serial online] 2023 [cited 2023 Feb 1];3:92-6. Available from: https://www.ijoreports.in/text.asp?2023/3/1/92/368228



Idiopathic retinal vasculitis, aneurysm, and neuroretinitis (IRVAN) syndrome was first reported by Dr. Kincaid and Dr. Shatz in 1983.[1] In 2007, Samuel et al.[2] divided IRVAN syndrome into five stages: 1. aortic aneurysm, exudation, neuritis, and retinal vasculitis; 2. capillary nonperfusion; 3. posterior pole neovascularization or vitreous hemorrhage; 4. neovascularization in the anterior segment of the eye; and 5. neovascular glaucoma. At present, retinal photocoagulation in the area of capillary nonperfusion is an effective treatment for stage 2 IRVAN syndrome.[3] Ischemia is an important risk factor for vision loss in IRVAN syndrome.[4],[5] Peripheral capillary ischemia has been widely reported in IRVAN syndrome, but central retinal capillary perfusion is rarely restricted. Ischemia in the macular deep capillary plexus of IRVAN syndrome has been reported by Zina et al.,[6] who considered branch retinal artery occlusion (BRAO) as the main cause of the ischemia. Here, we supply another case to confirm that capillary nonperfusion in IRVAN syndrome could involve fovea, especially in macular deep capillary.


  Case Report Top


In June 2007, a 47-year-old man presented to the Second Hospital of Jilin University complaining of blurred vision in both eyes. The patient had no history of hypertension, diabetes, or family history. Fundus fluorescein angiography (FFA) showed periretinal capillary nonperfusion and retinal artery expansion in both eyes [Figure 1]b and [Figure 1]e. He was diagnosed with stage 2 IRVAN syndrome combined with primary open-angle glaucoma (POAG) and was treated with retinal photocoagulation combined with intraocular pressure (IOP)-lowering drugs. At the 2-month follow-up, FFA showed crescent-shaped hyperreflective lesions in the macular area [Figure 1]c and [Figure 1]f.
Figure 1: Laser spots are visible around the retina of both eyes. The branch of the retinal blood vessel shows tumor-like expansion (yellow arrow). Crescent-shaped hyperreflective lesions (red circle) can be seen in the macular area

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At the 3-month follow-up, the patient's IOP was not yet well controlled. On ophthalmic examination, the best-corrected visual acuity was 1.0 and the IOP was 28 mmHg in the right eye and 27 mmHg in the left eye. Visual field examination revealed a tubular visual field in both eyes. The optic cup was deeply indented (right: C/D = 0.8; left: C/D = 0.7). Optic disk optical coherence tomography angiography (OCTA) [Figure 2] showed that the blood flow density next to the optic nerves in both eyes was significantly reduced. Macular OCTA [Figure 3] showed that the blood flow density in the fovea and parafovea was lower in the deep capillary plexus. The macular ischemia of the right eye was grade 1 and of the left eye was grade 2. The IOP was controlled at 10–16 mmHg after trabeculectomy. At the 1-year follow-up, the patient's vision had not decreased and the IOP was well controlled. Timeline can be seen in [Figure 4].
Figure 2: Optic disk OCTA shows that the blood flow density next to the optic nerve in both eyes is significantly reduced. OCTA = optical coherence tomography angiography

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Figure 3: Macular OCTA in the right eye shows a significant decrease in blood flow density in the fovea and parafovea (yellow arrow). The red box shows a significant decrease in the density of deep capillaries. Microcyst-like low-density areas (red arrows) can be seen in the inner nuclear layer of the macula. OCTA = optical coherence tomography angiography

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Figure 4: A 47-year-old man diagnosed and surgically treated for stage 2 IRVAN syndrome combined with POAG. IRVAN = idiopathic retinal vasculitis, aneurysm, and neuroretinitis, POAG = primary open-angle glaucoma

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  Discussion Top


This patient had typical manifestations of IRVAN syndrome, with tumor-like expansion of the retinal arteries, macular exudation, and a loss of capillary perfusion around the retina [Figure 1]. FFA is used to observe perfusion of the retina and distribution of aneurysms in IRVAN syndrome. Here, we suggest that OCTA can be used to assist in observing the blood perfusion of the cross section of retina, especially the deep capillaries. In this case, OCTA showed that the blood flow density of the deep retinal capillaries in the macular area was significantly reduced [Figure 3].

The potential factors of macular capillary nonperfusion included the following: 1. an elevated IOP caused by POAG and 2. idiopathic retinal vasculitis. Unexpectedly, the information collected and shown in [Table 1] suggests that the POAG-induced IOP elevation had an impact on the superficial capillary plexus in the macular area, whereas the deep capillary plexus has a high tolerance to IOP fluctuations.[7] The results reported by Zhao et al.[8] suggest that for an IOP between 40 and 60 mmHg, the macular deep capillary plexus is significantly resistant to IOP elevation. Above all, ischemia in the macular deep capillary plexus is not caused by POAG.
Table 1: Microvascular macular changes in glaucoma

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What causes the retinal capillary nonperfusion of IRVAN syndrome? Many scholars support the hypothesis that “a developmental or congenital macroaneurysm” leads to “vascular flow abnormalities” resulting in retinal capillary nonperfusion.[15] Liu et al. reported that the size of retinal nonperfusion is relevant to the number of aneurysms.[16] BRAO may be another important reason for the ischemia. Zina et al.[6] and Zacharia et al.[5] found that BRAO is the important reason for capillary nonperfusion in macular and peripheral retina, respectively. Tripathy[15] thought that capillary nonperfusion is extensive and may also involve fovea, causing macular ischemia. This case could confirm his view. What is more, ischemia in the deep capillary plexus of IRVAN syndrome has also been reported by Zina et al.[6] Ischemia in the retinal deep capillaries may be the main reason for vision loss in IRVAN patients. Due to the lack of OCTA examination in most of the reported cases, we cannot conclude whether deep retinal capillary ischemia is a common condition or is related to the disease severity. More case observation is required to solve this problem.


  Conclusion Top


In conclusion, POAG has no effects on the ischemia of macular deep capillary. Macular deep capillary nonperfusion should not be ignored in IRVAN syndrome.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Kincaid J, Schatz H, Bilateral retinal arteritis with multiple aneurysmal dilatations. Retina 1983;3:171-8.  Back to cited text no. 1
    
2.
Samuel MA, Equi RA, Chang TS, Mieler W, Jampol LM, Hay D, et al. Idiopathic retinitis, vasculitis, aneurysms, and neuroretinitis (IRVAN): New observations and a proposed staging system. Ophthalmology 2007;114:1526-9 e1.  Back to cited text no. 2
    
3.
Wolff B, Azar G, Vasseur V, Sahel JA, Vignal C, Mauget-Faÿsse M. Microcystic changes in the retinal internal nuclear layer associated with optic atrophy: A prospective study. J Ophthalmol 2014;2014:395189.  Back to cited text no. 3
    
4.
MacIver S, Bass SJ, Sherman J. Visual acuity recovery in a case of idiopathic retinal vasculitis aneurysms and neuroretinitis. Optom Vis Sci 2012;89:E356-63.  Back to cited text no. 4
    
5.
Zacharia JA, Chin AT, Rebhun CB, Louzada RN, Adhi M, Cole ED, et al. Idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome presenting with branch retinal artery occlusion. Ophthalmic Surg Lasers Imaging Retina 2017;48:948-51.  Back to cited text no. 5
    
6.
Zina S, Ksiaa I, Abdelhedi C, Ben Amor H, Attia S, Khochtali S, et al. Multimodal imaging in IRVAN syndrome presenting with branch retinal artery occlusion. Eur J Ophthalmol 2020. doi: 10.1177/1120672120965492.  Back to cited text no. 6
    
7.
Tian M, Tappeiner C, Zinkernagel MS, Huf W, Wolf S, Munk MR. Evaluation of vascular changes in intermediate uveitis and retinal vasculitis using swept-source wide-field optical coherence tomography angiography. Br J Ophthalmol 2019;103:1289-95.  Back to cited text no. 7
    
8.
Zhao D, He Z, Wang L, Fortune B, Lim JK, Wong VH, et al. Response of the trilaminar retinal vessel network to intraocular pressure elevation in rat eyes. Invest Ophthalmol Vis Sci 2020;61:2. doi: 10.1167/iovs. 61.2.2.  Back to cited text no. 8
    
9.
Hosari S, Hohberger B, Theelke L, Sari H, Lucio M, Mardin CY. OCT angiography: Measurement of retinal macular microvasculature with spectralis II OCT angiography-reliability and reproducibility. Ophthalmologica 2020;243:75-84.  Back to cited text no. 9
    
10.
Milani P, Urbini LE, Bulone E, Nava U, Visintin D, Cremonesi G, et al. The macular choriocapillaris flow in glaucoma and within-day fluctuations: An optical coherence tomography angiography study. Invest Ophthalmol Vis Sci 2021;62:22. doi: 10.1167/iovs. 62.1.22.  Back to cited text no. 10
    
11.
Takusagawa HL, Liu L, Ma KN, Jia Y, Gao SS, Zhang M, et al. Projection-resolved optical coherence tomography angiography of macular retinal circulation in glaucoma. Ophthalmology 2017;124:1589-99.  Back to cited text no. 11
    
12.
El-Nimri NW, Manalastas PI, Zangwill LM, Proudfoot JA, Bowd C, Hou H, et al. Superficial and deep macula vessel density in healthy, glaucoma suspect, and glaucoma eyes. J Glaucoma 2021;30:e276-84.  Back to cited text no. 12
    
13.
Hohberger B, Lucio M, Schlick S, Wollborn A, Hosari S, Mardin C. OCT-angiography: Regional reduced macula microcirculation in ocular hypertensive and pre-perimetric glaucoma patients. PLoS One 2021;16:e0246469.  Back to cited text no. 13
    
14.
Li F, Lin F, Gao K, Cheng W, Song Y, Liu Y, et al. Association of foveal avascular zone area withstructural and functional progression in glaucoma patients. Br J Ophthalmol 2022;106:1245-51.  Back to cited text no. 14
    
15.
Tripathy K. Pathogenesis of idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) or 'idiopathic retinal arteriolar aneurysms (IRAA)' with macular star. Med Hypotheses 2018;112:65-66.  Back to cited text no. 15
    
16.
Liu XC, Zhang MN, Chen B, Li Y, Huang HB. A new perspective for analyzing clinical characteristics of idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome. Int Ophthalmol 2019;39:1475-82.  Back to cited text no. 16
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]
 
 
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