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Year : 2021  |  Volume : 1  |  Issue : 1  |  Page : 130-132

Optical coherence tomography angiography of epiretinal neovascularization in a case of macular telangiectasia type 2 and coexistent proliferative diabetic retinopathy

1 Department of Medical Retina, Watany Eye Hospital, Cairo, Egypt
2 Department of Ophthalmology, Ain Shams University, Cairo, and Vice Chairman, Watany Eye Hospital, Cairo, Egypt

Date of Submission27-May-2020
Date of Acceptance01-Sep-2020
Date of Web Publication31-Dec-2020

Correspondence Address:
Dr. Rania G Estawro
Watany Eye Hospital (WEH), Cairo 11775
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_1641_20

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Epiretinal neovascularization (ERN) is a novel optical coherence tomography angiography (OCTA) finding, which has been recently described in association with macular telangiectasia type 2 (MacTel 2). To our knowledge, this is the first documentation of ERN and coexistent proliferative diabetic retinopathy (PDR), which could be clinically confused with diabetic foveal neovascularization. OCTA is a useful tool to evaluate ERN and differentiate it from diabetic foveal neovascularization.

Keywords: Epiretinal neovascularization, macular telangiectasia type 2, optical coherence tomography angiography, proliferative diabetic retinopathy

How to cite this article:
Estawro RG, Hassouna AK. Optical coherence tomography angiography of epiretinal neovascularization in a case of macular telangiectasia type 2 and coexistent proliferative diabetic retinopathy. Indian J Ophthalmol Case Rep 2021;1:130-2

How to cite this URL:
Estawro RG, Hassouna AK. Optical coherence tomography angiography of epiretinal neovascularization in a case of macular telangiectasia type 2 and coexistent proliferative diabetic retinopathy. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Mar 2];1:130-2. Available from: https://www.ijoreports.in/text.asp?2021/1/1/130/305498

Macular telangiectasia type 2 (MacTel 2) is a bilateral neurodegenerative disease, characterized by ectatic intraretinal vascular proliferations within the juxta-foveal area.[1],[2] Optical coherence tomography angiography (OCTA) is a newly introduced imaging tool, which offers insights into MacTel2 microvascular features with depth resolved precision.[3],[4]

Recently, OCTA was used to first describe epiretinal neovascularization (ERN) in association with MacTel 2.[5] Interestingly, “foveal retinal neovascularization” has been also documented in eyes with proliferative diabetic retinopathy (PDR) using OCTA.[6]

This report aimed to confirm the incidence of ERN with coexistent PDR, which could be clinically confused with diabetic foveal neovascularization.

  Case Report Top

Current report adheres to the Tenets of the Declaration of Helsinki, and to the local Ethics Committee of Watany Research Development Center (WRDC) standards. Awritten informed consent for publication of clinical details and images was obtained from the patient.

A 52-year-old female with history of insulin-dependent diabetes mellitus and essential hypertension, presented to our clinic complaining of diminution of vision in both eyes (OU). Her past ocular history was noncontributory, and her best-corrected visual acuity was 20/32 in the right eye (OD) and 20/40 in her left eye (OS). Anterior segment examination was unremarkable, while fundus examination revealed signs of PDR and temporal juxta-foveal retinal pigment epithelium (RPE) alterations OU.

Multicolor imaging, fundus autofluorescence (FAF) and fluorescein angiography (FA) were performed using (HRA; Heidelberg Engineering, Heidelberg, Germany). Optical coherence tomography (OCT) and OCTA imaging were performed using (RTVue XR; Optovue, Fremont, California, USA).

Multicolor imaging showed bilateral PDR changes and temporal juxta-foveal RPE changes [[Figure 1], R1 and L1]. Enface structural OCT showed temporal juxta-foveal RPE pigmentary alterations and retinal vascular changes [[Figure 1], R2 and L2]. FAF showed temporal juxta-foveal RPE changes suggestive of MacTel 2 [[Figure 1], R3 and L3]. FA showed bilateral multiple micro-aneurysms, multiple leaking diabetic neovessels elsewhere (NVEs), and peripheral retinal ischemia, in association with temporal juxta-foveal vascular lesion showing progressive leakage [[Figure 1], R4 and L4]. OCT line-scan of the right eye showed juxta-foveal intraretinal hyper reflectivity with multiple tiny interruptions of internal limiting membrane (ILM), associated with ILM drape and epiretinal proliferation [[Figure 1], R5]. OCT line-scan of the left eye showed juxta-foveal intraretinal hyper reflectivity with foveal cavitation, associated with multiple tiny interruptions of ILM and epiretinal proliferation [[Figure 1], L5].
Figure 1: Multimodal imaging of both fundi. (R1, L1) Multicolor image showed proliferative diabetic retinopathy (PDR). (R2, L2) Enface OCT images showed juxta-foveal retinal pigment epithelium (RPE) alterations. (R3, L3) Fundus autofluorescence images showed juxta-foveal RPE changes. (R4, L4) Fluorescein angiography images showed leaking vascular proliferations. (R5, L5) OCT line scans showed extension of the vascular lesion into the vitreous cavity (yellow arrows)

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Enface OCTA imaging showed bilateral juxta-foveal intraretinal vascular lesion with preretinal extension in both eyes, and B scan with angio-overlay confirmed anastomotic connections between the preretinal and intraretinal telangiectatic vessels passing through ILM [Figure 2]. Vitreoretinal interface (VRI) slab—involving layers between ILM and 40 μ above—revealed extension of a vascular frond into the vitreous cavity OU [[Figure 2], R2-R3 and L2-L3]. Retinal slab—involving layers between ILM and outer plexiform layers (OPL)—confirmed presence of intraretinal telangiectatic vessels [[Figure 2], R4-R5 and L4-L5]. Outer retinal layers (ORL) slab—involving layers between OPL and Bruch's membrane—did not show any abnormal vascular flow [[Figure 2], R6-R7 and L6-L7]. Three dimensional (3D) angio-display of the juxta-foveal vascular lesions were constructed by Optovue engineers for better visualization of the vascular lesion [Figure 3].
Figure 2: Optical coherence tomography angiography (OCTA) scanning of both maculae. (R1, L1) Montage OCTA showed juxta-foveal telangiectatic vessels. (R2-R3 and L2-L3) Vitreoretinal interface slab showed epiretinal neovascularization (yellow arrows), and anastomotic vascular connections passing through internal limiting membrane (ILM). (R4-R5 and L4-L5) Retinal slab showed intraretinal telangiectatic vessels. (R6-R7 and L6-L7) Outer retinal slab showed no abnormal vasculature

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Figure 3: Three-dimensional (3D) angio-visualization of the juxta-foveal vascular lesion, right eye (top) and left eye (bottom)

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The patient received bilateral pan retinal photocoagulation (PRP) treatment. However, she did not come back for follow-up assessment and we did not get the chance to monitor her treatment response.

  Discussion Top

Advancements in retinal imaging technology and segmentation strategies have revolutionized our understanding of different retinal pathologies. OCTA, in particular, has provided an in vivo depth resolved visualization of different retinal vascular disorders and angiographic characterization of both MacTel 2 and DR, with layer specific orientation.[3],[4],[7] In current case, FA enabled a two-dimensional assessment of PDR angiographic changes, with late frames showing bilateral juxta-foveal progressive vascular leakage, mimicking diabetic vitreoretinal proliferations and obscured the underlying pathology. On the other hand, OCTA imaging enabled a three-dimensional visualization of the lesion with the ability to dissect the preretinal component from the intraretinal component.

Thorough examination of OCTA slabs, revealed intraretinal telangiectatic capillaries matching the described criteria of MacTel 2.[4],[5] Besides, VRI slab confirmed an associated epiretinal vascular frond overlying ILM. Interestingly, angio-overlay in corresponding B scan showed anastomotic connections between the preretinal and intraretinal telangiectatic vessels passing through ILM [Figure 2], favoring a single pathology and matching the recent reporting of “ERN” complicating MacTel 2.[5] However, it cannot be ascertained whether the presence of preretinal vascular proliferations should be ascribed to MacTel 2 or to diabetic viteroretinal proliferations without further histopathologic studies

The Müller cell affection in MacTel 2 was confirmed using histopathological correlation with reported focal defects in the ILM and focal loss of the outer limiting membrane within the central macular area.[8] It was postulated that preretinal extension of MacTel 2 was attributed to the interruptions of ILM by abutting intraretinal pigment and/or the baring of the abnormal capillaries of the Superficial Capillary Plexus (SCP) on the retinal surface.[5] In current case, author hypothesizes that diabetic retinal ischemia might have increased vitreous levels of abnormal angiogenetic factors, which stimulated aberrant telangiectatic capillaries to proliferate towards the hypoxic environment of the vitreous cavity, through ILM alterations secondary to Muller's cells disruption.

Unlike previous reporting of ERN features, degenerative intraretinal spaces and ILM drape were observed in current patient. In addition, this is the first report of PDR with coassociated ERN complicating MacTel 2. This raises the need for further study and characterization of this novel finding through histopathology and lager number of cases.

  Conclusion Top

To author's knowledge, this is the first report of ERN complicating MacTel 2 with coassociated PDR. OCTA is a useful tool to evaluate ERN and differentiate it from diabetic foveal neovascularization.


Qienyuan Zhou, Clinical Development, Optovue Inc.

Fremont, California, United States.

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

Dr. Estawro has no financial or proprietary interest in any material or method mentioned.

Dr. Hassouna receives fees for lectures by Alcon Laboratories, Inc., Johnson & Johnson Vision Care, Inc., and Mundipharma GmbH, travel support from Alcon Laboratories, Inc., Bayer, and PhysIOL.

Conflicts of interest

There are no conflicts of interest.

  References Top

Wu L, Evans T, Arevalo JF. Idiopathic macular telangiectasia type 2 (idiopathic juxtafoveolar retinal telangiectasis type 2A, Mac Tel 2). Surv Ophthalmol 2013;58:536-59.  Back to cited text no. 1
Charbel Issa P, Gillies MC, Chew EY, Bird AC, Heeren TFC, Peto T, et al. Macular telangiectasia type 2. Prog Retin Eye Res 2013;34:49-77.  Back to cited text no. 2
Spaide RF, Klancnik JM, Cooney MJ, Yannuzzi LA, Balaratnasingam C, Dansingani KK, et al. Volume-rendering optical coherence tomography angiography of macular telangiectasia type 2. Ophthalmology 2015;122:2261-9.  Back to cited text no. 3
Roisman L, Rosenfeld PJ. Optical coherence tomography angiography of macular telangiectasia type 2. Dev Ophthalmol 2016;56:146-58.  Back to cited text no. 4
Ayachit AG, Reddy LU, Joshi S, Ayachit GS. Epiretinal neovascularization: A novel OCT angiography finding in macular telangiectasia type 2. Ophthalmol Retina 2019;3:516-22.  Back to cited text no. 5
Murakawa S, Hasegawa T, Koizumi H, Maruko I, Iida T. Foveal retinal neovascularization in proliferative diabetic retinopathy: Assessment by optical coherence tomography angiography. Retina Phila Pa 2017;37:e135-7.  Back to cited text no. 6
Hwang TS, Jia Y, Gao SS, Bailey ST, Lauer AK, Flaxel CJ, et al. Optical coherence tomography angiography features of diabetic retinopathy. Retina Phila Pa 2015;35:2371-6.  Back to cited text no. 7
Cherepanoff S, Killingsworth MC, Zhu M, Nolan T, Hunyor AP, Young SH, et al. Ultrastructural and clinical evidence of subretinal debris accumulation in type 2 macular telangiectasia. Br J Ophthalmol. 2012;96:1404-9.  Back to cited text no. 8


  [Figure 1], [Figure 2], [Figure 3]


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