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Year : 2023  |  Volume : 3  |  Issue : 1  |  Page : 214-215

A case of congenital retinal macro-vessel: An abominable trunk

Department of Ophthalmology, AIIMS, Jodhpur, Rajasthan, India

Date of Submission25-Jul-2022
Date of Decision11-Aug-2022
Date of Acceptance18-Nov-2022
Date of Web Publication20-Jan-2023

Correspondence Address:
Nikhil Agrawal
Room No. 3116 AIIMS Academic Block, AIIMS, Jodhpur, Rajasthan
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_1815_22

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Keywords: Arteriovenous malformation, congenital retinal vein, retinal macrovessel

How to cite this article:
Meena S, Sheeemar A, Agrawal N, Bhatnagar K, Shakrawal J, Tandon M. A case of congenital retinal macro-vessel: An abominable trunk. Indian J Ophthalmol Case Rep 2023;3:214-5

How to cite this URL:
Meena S, Sheeemar A, Agrawal N, Bhatnagar K, Shakrawal J, Tandon M. A case of congenital retinal macro-vessel: An abominable trunk. Indian J Ophthalmol Case Rep [serial online] 2023 [cited 2023 Feb 1];3:214-5. Available from: https://www.ijoreports.in/text.asp?2023/3/1/214/368173

A 16-year-old male patient presented to the eye outpatient department for routine ophthalmic evolution. On examination, his best corrected visual acuity was 20/20 in both eyes, and the anterior segment evaluation was remarkably normal. The fundus of the right eye was within normal limits; however, there was the presence of an abnormally prominent aberrant vessel originating from the disc. The vessel after its origin from the disk, coursed around the macula above the horizontal raphe and distributed into multiple small vessels encircling the fovea [Figure 1]. Furthermore, nicking of the vessels was noted and therefore, the patient was counseled for detailed ophthalmic and systemic evaluation. Fundus fluorescein angiography was done which showed the filling of the aberrant vessel in the early phase associated with laminar flow, which was visible even before the filling of the rest of the venous network [Figure 2]. This was suggestive of its venous origin draining into the central retinal vein. Optical coherence tomography of the macula was normal. The patient was evaluated systemically and an MRI brain was done to rule out venous malformation. Fortunately, the patient was systemically fit, and no signs of arteriovenous malformations were found on radiological imaging.
Figure 1: (a and b) Show colour fundus pictures of the right and left eye with an aberrant large vessel originating from the disc and branching over the superior macula. (c and d) 0 show corresponding red-free images

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Figure 2: (a and b) Show fluorescein angiography of the left eye with laminar flow in the aberrant vessel without any fluorescence in other retinal veins, suggestive of the venous nature of the aberrant vessel. In (b)the aberrant vessel shows uniform fluorescence, whereas laminal flow can be seen in other retinal veins, suggestive of early filling of the aberrant vessel before the rest of the retinal veins and indicative of an arterio-venous communication/shunt with the aberrant retinal vessel

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  Discussion Top

A congenital retinal macrovessel is an abnormal vessel crossing the central macula with distribution both above and below the horizontal raphe.[1] It was first described by Mauthner, and the term was later coined by Brown et al.[1],[2] It has been reported as a type of arteriovenous malformation (stage 1) with vessel course described as crossing the fovea, encircling the fovea, or bypassing the fovea.[3] Usually, it is a vein, and the patient is asymptomatic. Vision loss can reportedly occur from macular hemorrhage, serous macular detachment, cystoid edema, or vessel crossing the fovea.[4],[5] Pichi et al. reported an association of 24% of retinal macrovessel with brain venous malformation.[3] In our patient, the aberrant vessel was a vein as seen angiographically encircling the fovea without causing any vision loss.

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

Brown GC, Donoso LA, Magargal LE, Goldberg RE, Sarin LK. Congenital retinal macrovessels. Arch Ophthalmol 1982;100:1430-6.  Back to cited text no. 1
Mauthner L. Lehrbuch der Ophthalmoscopie. Vienna, Austria: Tendler Co; 1869. p. 249.  Back to cited text no. 2
Pichi F, Freund KB, Ciardella A, Morara M, Abboud EB, Ghazi N, et al. Congenital retinal macrovessel and the association of retinal venous malformations with venous malformations of the brain. JAMA Ophthalmol 2018;136:372-9.  Back to cited text no. 3
de Crecchio G, Pacente L, Alfieri MC, Greco GM. Valsalva retinopathy associated with a congenital retinal macrovessel. Arch Ophthalmol 2000;118:146-7.  Back to cited text no. 4
Ascaso FJ. Spontaneous resolution of central serous chorioretinopathy in a patient with congenital retinal macrovessel. Circulation 2011;124:e904-5.  Back to cited text no. 5


  [Figure 1], [Figure 2]


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