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Year : 2022  |  Volume : 2  |  Issue : 2  |  Page : 589-591

Optical coherence tomography angiography in type III retinal astrocytic hamartoma

Department of Vitreo-Retina, Disha Eye Hospital, Kolkata, West Bengal, India

Date of Submission05-Sep-2021
Date of Acceptance21-Oct-2021
Date of Web Publication13-Apr-2022

Correspondence Address:
Kshitiz Kumar
Department of Vitreo-Retina, Disha Eye Hospital, Barrackpore, 88 (63A) Ghoshpara Road, Kolkata - 700 120, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_2316_21

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Keywords: Intratumoral calcification, optical coherence tomography angiography, optically empty spaces, retinal astrocytic hamartoma, ultra-widefield fundus imaging

How to cite this article:
Kumar K, Sinha TK, Bhattacharya D. Optical coherence tomography angiography in type III retinal astrocytic hamartoma. Indian J Ophthalmol Case Rep 2022;2:589-91

How to cite this URL:
Kumar K, Sinha TK, Bhattacharya D. Optical coherence tomography angiography in type III retinal astrocytic hamartoma. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 May 18];2:589-91. Available from: https://www.ijoreports.in/text.asp?2022/2/2/589/342943

A 26-year-old male had BCVA of plano 20/20 in the right eye (RE) and −2.75 Dsph/−2.0 Dcyl × 20° 20/20 in the left eye (LE). Fundus evaluation revealed no abnormality in RE, normal disc, and macula in LE with a “mulberry-like, partially calcified, avascular looking lesion along an inferotemporal arcade about 2 disc diameter in size. There was a prominent stalk-like vitreous condensation bridging the optic disc and the superior aspect of the lesion mimicking a sheathed vessel (pseudo-vessel). Ultra-widefield fundus imaging (UWFI) and angiography (UWFA) were captured using the Optos 200Tx (Optos, Dunfermlike, UK) [Figure 1]. UWFA revealed multiple autofluorescence spots in the pre-arterial phase within the lesion suggestive of calcification, persistent autofluorescence with capillaries filling the lesion from temporal half during the arterial phase with confirmation of the pseudo-vessel to be vitreous condensation, capillary plexus, and draining venules along the inferonasal end of the lesion during the arteriovenous phase and late phase angiogram showing minimal leakage with staining [Figure 1]b,[Figure 1]c,[Figure 1]d,[Figure 1]e. RAH was imaged using the Solix full-range OCT (Optovue Inc, Freemont CA, USA), a new ultrahigh-speed SD-OCTA device [Figure 2] and [Figure 3]. 6.40 × 6.40 (mm) EnFace image revealed hyper-bright areas in the inferior and nasal half and hypo-bright areas in the superior and temporal half of the lesion [Figure 2]a. Corresponding structural OCT confirmed a heterogeneous lesion arising from the nerve fiber layer and protruding into the vitreous cavity with shadowing of the outer layers and choroid. RAH measured 1290 μm in thickness and 3230 μm along its widest dimension. Vitreous changes in the form of thickening and adhesion were present overlying the lesion [Figure 2]b. Furthermore, 3 × 3 (mm) SLO image and the corresponding OCT images showed three prominent optically empty spaces [OESs; classic clear, moth-eaten areas] along the cross-lines interspersed with homogenous areas [Figure 2]c,[Figure 2]d,[Figure 2]e. The largest OES measured 474 μm. OES corresponded with the autofluorescent spots and were concentrated in the bright areas of EnFace image suggestive of calcification. Thus, the lesion was only partially calcified. The 6.40 × 6.40 (mm) EnFace superficial vascular complex (SVC) slab revealed perilesional capillary network superotemporally abutting the lesion and dense intratumoral capillary plexus in the nasal and inferior half over the calcified areas of the lesion [Figure 3]a and [Figure 3]b. However, the deep vascular complex (DVC) slab revealed largely avascular regions deep within the tumor [Figure 3]d and [Figure 3]e. The corresponding grid vessel density (%) of the superficial and deep EnFace slab showed relatively higher vessel concentration in the superficial layers of the tumor [Figure 3]c and [Figure 3]f. Compared to conventional fluorescein angiography (UWFA), OCTA showed more intratumoral vascularity of the RAH. Overall, the vascular characteristics of benign clinically avascular type III RAH got established.
Figure 1: UWFI of left eye. (a) UWF color photographs showing glistening mulberry-like type III RAH with stalk of vitreous condensation. (b and c) Autofluoresceing intralesional calcification is visible during the pre-arterial and arterial phase of UWFA with feeder vessels. (d) Arteriovenous phase UWFA shows the feeder arteriole, capillary plexus, and the draining venule. (e) Late-phase UWFA shows minimal leakage and tissue staining of the tumor

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Figure 2: OCTA images. (a) 6.40 × 6.40 (mm) EnFace OCT image showing a hyper bright region in inferior and nasal half and hypo bright areas in superior and temporal half of the tumor. (b) Corresponding structural OCT shows lesion arising from the nerve fiber layer, optically empty spaces (OES), posterior shadowing, protrudence into the vitreous cavity with overlying vitreous condensation. (c-e) 3 × 3 (mm) SLO image with corresponding OCT images showing “moth-eaten” appearance with OES more concentrated in the inferior and nasal half corresponding with the hyper bright regions of EnFace image

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Figure 3: OCTA images. EnFace SVC slab (a and b) images showing peri-lesional and intratumoral capillary plexus with corresponding grid vessel density (c). EnFace DVC slab (d and e) images showing largely avascular deeper layers of the tumor with corresponding grid vessel density (f)

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  Discussion Top

Nearly two-thirds of patients with classic RAH are not associated systemically with features of tuberous sclerosis complex (TSC).[1] As per the recent categorization,[2] this case was of a typical RAH type III found incidentally on routine examination. The study patient did not have subependymal giant cell astrocytoma (SEGA) on thorough evaluation, which has a strong relationship with type III RAH in TSC.[2]

Optical coherence tomography angiography (OCTA) features of intratumoral microvasculature in type I, II, and IV RAH have been reported recently;[3],[4] however, OCTA findings in type III RAH has not been reported before.

OES could represent intralesional calcification in calcified tumors and intraretinal cavitation in non-calcified tumors.[1] In a recent study, type III RAH showed “moth-eaten” OES representing intratumoral calcification on SD-OCT in 100% of cases, whereas clinically evident calcification was seen in only 70% of cases.[1] This case of type III RAH studied on multimodal imaging had mulberry appearance with evidence of partial calcification on FAF, OCTA SLO image showed moth-eaten texture, and EnFace image showed a mix of hyper and hypo-bright areas corresponding with regions of calcification and non-calcification. Location and concentration OES in this lesion matched with regions of calcification.

UWFA and OCTA of this type III RAH highlighted vasculatures, which were clinically not evident. This knowledge of tumor vascularity is important as there have been instances of recurrent vitreous hemorrhage in RAH, sometimes due to abnormal vessels and enlargement of the lesion, leading to rupture or necrosis of normal vessels, or due to leakage from inside of the hemorrhage-filled cysts.[5] Thus, the presence of vascular plexus in the superficial layers of the tumor with overlying vitreous condensation makes type III RAH more prone to hemorrhage if there is increased retinal traction because of enlargement or vitreous detachment. This report presents hitherto unreported OCTA features of type III RAH and completes the missing OCTA characteristics of all types of RAH available in the existing literature.

Consent for publication

Written informed consent was obtained from the patient for publication of this case report along with consent to publish the images.

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

Shields CL, Say EA, Fuller T, Arora S, Samara WA, Shields JA. Retinal astrocytic hamartoma arises in nerve fiber layer and shows “moth-eaten”optically empty spaces on optical coherence tomography. Ophthalmology 2016;123:1809-16.  Back to cited text no. 1
Pichi F, Massaro D, Serafino M, Carrai P, Giuliari GP, Shields CL, et al. Retinal astrocytic hamartoma: Optical coherence tomography classification and correlation with tuberous sclerosis complex. Retina 2016;36:1199-208.  Back to cited text no. 2
Despréaux R, Mrejen S, Quentel G, Cohen SY. En face optical coherence tomography (OCT) and OCT angiography findings in retinal astrocytic hamartomas. Retin Cases Brief Rep 2017;11:373-9.  Back to cited text no. 3
Mellen PL, Sioufi K, Shields JA, Shileds CL. Invisible, honeycomb-like, cavitary retinal astrocytic hamartoma. Retin Cases Brief Rep 2020;14:211-4.  Back to cited text no. 4
Soeta M, Arai Y, Takahashi H, Fujino Y, Tanabe T, Inoue Y, et al. Novel preretinal hair pin-like vessel in retinal astrocytic hamartoma with vitreous hemorrhage. Retin Cases Brief Rep 2018;12:212-5.  Back to cited text no. 5


  [Figure 1], [Figure 2], [Figure 3]


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