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 Table of Contents  
Year : 2021  |  Volume : 1  |  Issue : 2  |  Page : 320-322

Multimodal imaging in a case of macular vortex vein varix

Department of Vitreo-Retina and Uveitis, Sadguru Netra Chikitsalaya, Chitrakoot, Satna, Madhya Pradesh, India

Date of Submission28-May-2020
Date of Acceptance15-Sep-2020
Date of Web Publication01-Apr-2021

Correspondence Address:
Dr. Gaurav Mohan Kohli
Department of Vitreo-Retina and Uveitis, Sadguru Netra Chikitsalaya, Chitrakoot, Satna - 210 204, Madhya Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_1650_20

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Variceal dilations of vortex vein are not uncommon in eyes with high myopia. However, its occurrence in emmetropic eyes and posterior location has rarely been reported. We report a case of 55-year-old man who presented with a reddish orange sub-retinal lesion at the macula with subtle pulsation and collapse on pressure indentation. The lesion appeared hyerfluorescent on early frames of FFA with persistent silhouette in the late frames. ICGA showed dilated and tortuous vascular complex with hyperfluorescence in the mid phase without any evidence of late leakage. On SD–OCT the lesion appeared as a hypo-reflective sinusoidal space extending from the Hallers layer to the choriocapillary bed with distortion of the overlying RPE. The En-face Angio-OCT imaging was suggestive of a flow void sinusoidal tract with internal septa. The clinical features and collaborative findings on multimodal imaging were suggestive of variceal dilatation of the vortex vein ampulla.

Keywords: Macular vortex vein, Varix, Vortex vein, Vortex vein ampulla

How to cite this article:
Bhatia P, Kohli GM, Shenoy P, Sen A. Multimodal imaging in a case of macular vortex vein varix. Indian J Ophthalmol Case Rep 2021;1:320-2

How to cite this URL:
Bhatia P, Kohli GM, Shenoy P, Sen A. Multimodal imaging in a case of macular vortex vein varix. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Sep 25];1:320-2. Available from: https://www.ijoreports.in/text.asp?2021/1/2/320/312334

Vortex veins are frequently located at the equator and are considered to be one of the consistent landmarks,[1] rarely they can be anomalous and located at the posterior pole.[2] Though uncommon, imaging characteristics of posterior vortex vein have been previously reported in eyes with high myopia and posterior staphyloma.[3],[4] The clinicians need to be familiar with the clinical feature and imaging characteristics of these lesions so as to differentiate these from other pathological conditions of the choroid. Herein we describe the multimodal imaging characteristics with special emphasis on Angio-OCT (OCT-A) features for a case of anomalous sub-macular choroidal macro-vessel/vascular malformation in a middle-aged emmetropic male, diagnosed as vortex vein varix (VVV).

  Case Report Top

A 55-year-old man was referred to the Retina clinic for sub-optimal recovery of vision following phacoemulsification with intraocular lens (IOL) implantation in his left eye (OS). The recorded corrected distance visual acuity in OS was 20/40. The anterior segment examination was unremarkable with a well-centered IOL. Fundus examination (OS) revealed a reddish-orange well-defined sub-retinal lesion in the macular area, with tentacular projections radiating out from the lesion [Figure 1]a. The lesion blanched and showed subtle pulsations on giving gentle pressure over the eyeball. However, no alteration of the lesion was noted on valsalva maneuver. Similar lesion was also identified in the right eye (OD), the finer details of which remained obscured due to cataract [Figure 1]b.
Figure 1: (a) Fundus photograph of left eye showing reddish orange sub-retinal lesion with radiating tentacular projections. (b) Fundus photograph of right eye. (Hazy due to cataract). (c) Normal B-scan

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No identifiable lesion could be discerned on horizontal axial ultrasound B-scan [Figure 1]c. On Spectral Domain-OCT (SD-OCT) Optovue Angiovue ©(Fremont, CA, USA), the lesion appeared as a bean-shaped, linear, hypo reflective, sinusoidal void in the Haller's layer with impingement of the overlying choriocapillaries, creating smooth convex shallow bump of the overlying Retinal Pigment Epithelium (RPE). No intra-retinal changes were appreciated [Figure 2]a. The manually segmented Enface OCT-A image at the level of the choroid showed a lesion with a central body comprising of an ampulla with radially oriented linear tracts, simulating a medusa-head appearance. There was no detectable flow signal within the lesion [Figure 2]b, [Figure 2]c, [Figure 2]d.
Figure 2: (a) EDI OCT showing dilated choroidal veins appearing as a bean shaped sinusoidal dilatation creating a smooth shallow convex bump of the overlying RPE (dotted box). (b) En-face OCTA using manual segmentation at the level of choroid showing a lesion with a central body comprising of an ampulla with radially oriented linear tracts (red arrows) simulating a medusa-head appearance, with no detectable flow signal. (c) SLO image dilated ampulla of the choroidal vessel with radially oriented linear tracts. (d) OCT-A scan showing no internal flow within the dilated choroidal vessel

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On fundus fluorescein angiography (FFA) the area corresponding to the lesion showed mild hyper fluorescence in the arterial phase. The lesion appeared deeper to the retinal arterioles, increasing in intensity in the subsequent phases, with no change in size. In the late phase, the background choroidal fluorescence surrounding the lesion decreased with a persisting silhouette of fluorescence at its center [Figure 3]a, [Figure 3]b, [Figure 3]c. Indocyanine green angiography (ICG) revealed a conspicuous, dilated choroidal vascular array converging collectively on a sinusoid and dilated vessel. The lesion appeared hypercyanescent in the early phase with increase in cynascence due to pooling within the dilated ampulla. The vascular lesion appeared discrete with no evidence of leakage [Figure 3]d, [Figure 3]e, [Figure 3]f.
Figure 3: (a) FFA of left eye showing arterial phase with hypofluorescence in the macular area. (b) Late venous phase showing prominent background choroidal fluorescence with hyperfluorescence at the center of the lesion (arrow). (c) Decreasing intensity of background choroidal fluorescence in the late phase with a persisting silhouette of hyperfluorescence at the center of the lesion. (d and e) Early and mid-phase of ICGA respectively, showing an array of choroidal vessels (*) converging towards a central sinusoidal oval vessel (→) giving a medusa head appearance. (f) Late phase of ICGA showing persistent cynascence due to pooling within the dilated ampulla

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The initial impression of the lesion on cursory examination mimicked that of a mass lesion like amelanotic nevus or choroidal osteoma. Ultrasound B-scan ruled out the presence of a tumoral mass. FFA and ICG aided in recognizing the lesion as being a vascular anomaly. The OCT-A was supportive in studying the flow characteristics and internal structure. A diagnosis of vortex vein varix was made. Being a vascular anomaly no treatment was required and at three months, visual acuity was maintained at 20/40 in OS. Cataract surgery in OD is awaited.

  Discussion Top

Dilatation of the vortex ampulla is commonly referred to as a varix or a varicosity of the vortex vein.[5] Literature on Macular Vortex Vein Varix describes its close association with myopia and posterior staphyloma, with an estimated incidence being 10.6% to 26% in highly myopic eyes.[4],[6] Submacular varix has an infrequent and quaint association with macular vortex veins. In our case, the diagnosis of a vortex vein varix was made based on the clinical findings of blanching of the lesion and subtle pulsation on gentle pressure over the globe. However, we were not able to demonstrate associated gaze-evoked changes. The inability of ultrasound-B scan to successfully identify the vascular choroidal lesion was probably due to the collapse of the varix under the pressure of the ultrasound probe [Figure 1]c.

Consistent with previous reports, the SD-OCT showed a sub-macular hypo reflective sinusoidal void within the choroid with well-demarcated external and internal limits.[3] The sinusoidal void was indicative of the enlarged vessels within the Hallers layer extending through the middle Sattler layer, with observable compression of the middle layer as well as the choriocapillaries. The Enface OCT-A at the site of the lesion showed three hypo reflective voids with internal septa. This sub-RPE hypo reflective space in the center was surrounded by radially oriented linear hypo reflective tracts. The central body was representative of the dilated ampule of the vortex vein while the tracts represented the draining choroidal venular tributaries [Figure 2]b. The choroidal vasculature being a low flow system, was hypo-reflective on OCT-A scans with no observable internal flow [Figure 2]b and [Figure 2]d.

In our case the clinically apparent vascular malformation was seen as a hypo reflective flow void both on en-face as well as cross-sectional OCT-A scan, despite evident flow on FFA and ICG. This supports our diagnosis of a varicial dilatation of the macular vortex vein ampullae, represented by the sinusoidal dilatation with tendency to collapse under digital pressure.[7] Our OCT-A findings for VVV are in contrast to that seen in choroidal macrovessel,[8] wherein there is presence of an observable flow signal within the macrovessel complex.

Despite the initial impression on SD-OCT simulating that of a subfoveal macrovessel as described by Lima et al., and Choudhry et al.[9],[10]; our case of VVV differed from choroidal macrovessel in being an irregular elevation at the macula compared to a linear cord like impression seen in cases with choroidal macrovessel. The choroidal macrovessel represents an arteriolar channel that supplies the choroid, filling up early in the angiographic sequence unlike VVV which represents an anomalous venous dilatation draining the choroid posteriorly. Multimodal imaging helped in differentiating VVV to choroidal macrovessel in our case.

  Conclusion Top

Vortex vein varix has been reported in eyes with myopia, we report its presence in an emmetropic eye along with imaging findings important for discriminating it from macular vortex vein. In addition to the already known OCT findings, our report describes pertinent OCT-A features and highlights its role in differentiating vascular lesions based on their internal flow velocity, which remains informative in studying vascular malformations. OCT-A provides additional information on the internal flow and can mitigate the use of invasive investigations for ominous appearing vascular lesions.

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.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Rutnin U. Fundus appearance in normal eyes. Am J Ophthalmol 1967;64:821-39.  Back to cited text no. 1
Sekimoto M, Hayasaka S, Watanabe M, Setogawa T. Vortex veins in the macula. Ophthalmologica 1988;197:34-5.  Back to cited text no. 2
Kaplan RI, Walsh JB, Rosen RB, Gupta M. Macular vortex vein in a highly myopic eye imaged by optical coherence tomography angiography. Retin Cases Brief Rep 2018;10. doi: 10.1097/ICB.0000000000000808.  Back to cited text no. 3
Moriyama M, Cao K, Ogata S, Ohno-Matsui K. Detection of posterior vortex veins in eyes with pathologic myopia by ultra-widefield indocyanine green angiography. Br J Ophthalmol 2017;101:1179-84.  Back to cited text no. 4
Vahdani K, Kapoor B, Raman VS. Multiple vortex vein ampulla varicosities. BMJ Case Rep. 2010;2010:bcr0820103223. Published 2010 Nov 19. doi:10.1136/bcr.08.2010.3223.  Back to cited text no. 5
Moriyama M, Ohno-Matsui K, Futagami S, Yoshida T, Hayashi K, Shimada N, et al. Morphology and long-term changes of choroidal vascular structure in highly myopic eyes with and without posterior staphyloma. Ophthalmology 2007;114:1755-62.  Back to cited text no. 6
Levy J, Yagev R, Shelef I, Lifshitz T. Varix of the vortex vein ampulla: A small case series. Eur J Ophthalmol 2005;15:424-7.  Back to cited text no. 7
Mori H, Yamada H, Sato Y, Takahashi K. Optical coherence tomographic angiography and ultra-widefield indocyanine green angiography of a choroidal macrovessel. Am J Ophthalmol Case Rep 2020;18:100612.  Back to cited text no. 8
Choudhry N, Rao R. Enhanced depth imaging features of a choroidal macrovessel. Retin Cases Brief Rep 2016;10:18-21.  Back to cited text no. 9
Lima L, Laud K, Chang L, Yannuzzi L. Choroidal macrovessel. Br J Ophthalmol 2010;95:1333-4.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3]


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