About OJO | Search | Ahead of print | Current Issue | Archives | Author Instructions | Reviewer Guidelines | Online submissionLogin 
Oman Journal of Ophthalmology Oman Journal of Ophthalmology
  Editorial Board | Subscribe | Advertise | Contact
https://www.omanophthalmicsociety.org/ Users Online: 71  Wide layoutNarrow layoutFull screen layout Home Print this page  Email this page Small font size Default font size Increase font size

 Table of Contents    
Year : 2014  |  Volume : 7  |  Issue : 2  |  Page : 96-97  

Branch retinal artery occlusion associated with congenital retinal macrovessel

1 Guru Nanak Eye Centre, Maulana Azad Medical College, New Delhi, India
2 Department of Ophthalmology, University College of Medical Sciences, New Delhi, India

Date of Web Publication19-Jul-2014

Correspondence Address:
Neha Goel
9/2 Punjabi Bagh Ext, New Delhi - 110 026
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0974-620X.137172

Rights and Permissions

A congenital retinal macrovessel (CRM) is a large retinal vessel, usually a vein, which traverses through the central macula and has large tributaries extending on both sides of the horizontal raphe. In the majority of cases, CRM have no effect on visual acuity, although in rare cases, macular hemorrhage, foveolar cysts, serous macular detachment, and the presence of the anomalous vessel in the foveola can affect vision. We describe a case of CRM with decreased vision secondary to a branch retinal artery occlusion (BRAO). To the best of our knowledge, this association has not been reported previously.

Keywords: Congenital retinal macrovessel, aberrant retinal vessels, branch retinal artery occlusion, optical coherence tomography, macular thickness, retinal nerve fiber layer thickness

How to cite this article:
Goel N, Kumar V, Seth A, Ghosh B. Branch retinal artery occlusion associated with congenital retinal macrovessel. Oman J Ophthalmol 2014;7:96-7

How to cite this URL:
Goel N, Kumar V, Seth A, Ghosh B. Branch retinal artery occlusion associated with congenital retinal macrovessel. Oman J Ophthalmol [serial online] 2014 [cited 2023 Mar 31];7:96-7. Available from: https://www.ojoonline.org/text.asp?2014/7/2/96/137172

   Introduction Top

Congenital retinal macrovessels (CRM) are rare, aberrantly large branches of retinal arteries or veins that typically cross the horizontal raphe to either supply or drain the macula. 1 Reduction of vision in the involved eye is rare and has been attributed to serous macular detachment, hemorrhage, foveal cyst, or to the mere presence of the aberrant vessel in the foveal area. We present a case of CRM with decreased vision due to co-existent branch retinal artery occlusion (BRAO) and ensuing hemimacular atrophy.

   Case Report Top

A 45-year-old healthy female presented with decreased vision in her left eye noticed during a routine ophthalmic evaluation. On examination, the best corrected visual acuity (BCVA) was 20/20 in the right eye and 20/80 in the left eye. Anterior segments of both eyes were normal. Fundus examination of the left eye revealed a large aberrant tributary of a dilated tortuous superotemporal vein that branched inferiorly and crossed the horizontal raphe with several tributaries reaching up to the fovea. The inferotemporal artery as well as vein was attenuated [Figure 1]a. The right fundus was normal. The cup:disc ratio was 0.3 in both eyes.
Figure 1: (a) Color fundus photograph of the left eye showing a large aberrant branch of a dilated superotemporal vein reaching up to the fovea (congenital retinal macrovessel). The inferotemporal vessels are attenuated. (b) Fluorescein angiogram showing multiple arteriovenous communications around the CRM. Horizontal (c) and vertical (d) SD-OCT scans through the macula showing marked thinning of the macula inferiorly especially involving the inner retinal layers. A macular thickness map (e) confirms that the superior quadrant is unaffected

Click here to view

Fluorescein angiography (FA) revealed, in addition, early filling and delayed emptying of the aberrant vein, altered perifoveal capillary network, and foveal avascular zone. Though anastomoses were seen between its branches and that of the superotemporal vein, there were no abnormal leakages or capillary nonperfusion areas [Figure 1]b.

Both eyes of the patient were studied using RTVue spectral domain (SD)-OCT (Optovue Inc., Fremont, California, USA). The left macula showed perifoveal and parafoveal thinning in the inferior quadrant, especially of the inner retinal layers [Figure 1]c-e. Also, the retinal nerve fiber layer (RNFL) thickness in the left eye was significantly reduced in the inferotemporal quadrant [Figure 2]a. Standard automated perimetry using 30-2 visual field threshold testing with a size III stimulus (Humphrey Field Analyser II, Humphrey Instruments Inc., San Leandro, CA, USA) revealed a corresponding field defect in the superonasal quadrant [Figure 2]b. The left eye was unremarkable with all values within normal range.
Figure 2: (a) Retinal nerve fiber layer (RNFL) thickness map showing significant reduction in the inferotemporal quadrant. (b) Standard automated perimetry using 30-2 visual field threshold testing showing a corresponding field defect in the superonasal quadrant

Click here to view

A detailed review of the patient's systemic history revealed no abnormality. Complete laboratory testing including hemogram, coagulation profile, erythryocyte sedimentation rate (ESR), and lipid profile were within normal limits. Echocardiography and carotid Doppler examination were unremarkable. The patient was advised to follow up regularly.

   Comment Top

CRMs are typically associated with normal visual acuity and are mostly detected on routine examination. The anomalous vessel is remarkably stable and complications only occur exceptionally as described in literature. [1] Archer et al. have classified retinal arteriovenous anastomoses in three groups depending on the caliber of the communicating vessels, the presence of a capillary plexus bridging these vessels, and the grade of visual impairment. [2] This case likely belongs to group 2 of this classification, which includes single or multiple direct arteriovenous communication without capillary bed, as seen on FA.

In our case, SD-OCT demonstrated segmental (inferior) macular thinning notably of the inner retinal layers along with reduction in the corresponding peripapillary RNFL, consistent with an ischemic insult to this part of the retina (an inferior BRAO). [3] To the best of our knowledge, this is the first reported case of decreased vision due to BRAO associated with a CRM. It has been suggested that decompensation of CRMs can cause a relative ischemia of the macular area due to direct arteriovenous communications which results in chronic, stable visual impairment. [4] This may have been the cause in our patient, with diminution of vision noticed later incidentally.

   References Top

1.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
2.Archer DB, Deutman A, Ernest JT, Krill AE. Arteriovenous communications in the retina. Am J Ophthalmol 1973;75:224-41.  Back to cited text no. 2
3.Leung CK, Tham CC, Mohammed S, Li EY, Leung KS, Chan WM, et al. In vivo measurements of macular and nerve fibre layer thickness in retinal arterial occlusion. Eye (Lond) 2007;21:1464-8.  Back to cited text no. 3
4.Chronister CL, Nyman NN, Meccariello AF. Congenital retinal macrovessel. Optom Vis Sci 1991;68:747-9.  Back to cited text no. 4


  [Figure 1], [Figure 2]

This article has been cited by
1 Macrovasos congénitos retinianos: presentación de un caso
B. Vidal-Villegas, C. Llorente La-Orden, C. Calvo González, M.P. Villegas-Pérez, E. Santos Bueso
Archivos de la Sociedad Española de Oftalmología. 2021; 96(9): 492
[Pubmed] | [DOI]
2 Congenital retinal macrovessels: Case presentation
B. Vidal-Villegas, C. Llorente La-Orden, C. Calvo González, M.P. Villegas-Pérez, E. Santos Bueso
Archivos de la Sociedad Española de Oftalmología (English Edition). 2021; 96(9): 492
[Pubmed] | [DOI]
Chiara Preziosa, Paolo Milani, Paola Ciasca, Fulvio Bergamini, Giovanni Staurenghi, Marco Pellegrini
RETINAL Cases & Brief Reports. 2021; 15(5): 605
[Pubmed] | [DOI]
4 Multimodal imaging of aberrant macular microvessel crossing the foveal avascular zone in two young adults
Xianming Jiang, Cong Zheng, Fangfang Du, Shibei Ai
BMC Ophthalmology. 2020; 20(1)
[Pubmed] | [DOI]
5 Multimodal imaging in a case of a congenital retinal macrovessel associated with a retinal cavernous hemangioma: a case report
Walid Zbiba, Issam Eddine Elleuch, Sana Sayadi, Meriem Laadheri
BMC Ophthalmology. 2020; 20(1)
[Pubmed] | [DOI]
6 Anomalous retinal artery associated with branch retinal artery occlusion and neovascular glaucoma: A case report
Wan-Ju Yang, Yan-Ning Yang, Ming-Gao Cai, Yi-Qiao Xing
World Journal of Clinical Cases. 2020; 8(5): 980
[Pubmed] | [DOI]
7 Congenital retinal macrovessels
Pratyusha Ganne, NageshaC Krishnappa
Indian Journal of Ophthalmology. 2018; 66(1): 129
[Pubmed] | [DOI]
8 Congenital retinal macrovessel in a patient with rhegmatogenous retinal detachment
Vinod Kumar, Neha Goel
Indian Journal of Ophthalmology. 2018; 66(12): 1860
[Pubmed] | [DOI]


    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

  In this article
   Case Report
    Article Figures

 Article Access Statistics
    PDF Downloaded225    
    Comments [Add]    
    Cited by others 8    

Recommend this journal