Oman Journal of Ophthalmology

CASE REPORT
Year
: 2022  |  Volume : 15  |  Issue : 3  |  Page : 393--396

Intraretinal and subretinal fluid in exudative nonproliferative macular telangiectasia type 2


George J Manayath, Rohan Suresh Ninan, Shishir Verghese, Venkatapathy Narendran 
 Department of Retina and Vitreous, Aravind Eye Hospital and Postgraduate Institute of Ophthalmology, Coimbatore, Tamil Nadu, India

Correspondence Address:
Shishir Verghese
Department of Retina and Vitreous, Aravind Eye Hospital and Post Graduate Institute of Ophthalmology, Coimbatore, Civil Aerodrome Post, Peelamedu, Coimbatore - 641 014, Tamil Nadu
India

Abstract

A 62-year-old man presented with complaints of decreased vision in BE. His best-corrected visual acuity (BCVA) was 6/12 in both eyes (BE). Based on his fundus examination and imaging findings, he was diagnosed with BE Macular Telangiectasia type 2 (MacTel 2) and was advised of regular follow-up. Over the course of follow-up, he developed further reduction in vision in the right eye (RE) to 6/24. Spectral-domain optical coherence tomography (SD-OCT) showed the presence of a subfoveal detachment (SFD) with intraretinal cystoid edema. He was diagnosed to have an exudative variant of preproliferative MacTel 2 and underwent three intravitreal injections of bevacizumab in the RE following which there was an improvement in BCVA to 6/12 along with the complete resolution of SFD and IRF on SD-OCT. This report describes a rare case of exudative nonproliferative macular telangiectasia type 2 presenting with the presence of intraretinal fluid.



How to cite this article:
Manayath GJ, Ninan RS, Verghese S, Narendran V. Intraretinal and subretinal fluid in exudative nonproliferative macular telangiectasia type 2.Oman J Ophthalmol 2022;15:393-396


How to cite this URL:
Manayath GJ, Ninan RS, Verghese S, Narendran V. Intraretinal and subretinal fluid in exudative nonproliferative macular telangiectasia type 2. Oman J Ophthalmol [serial online] 2022 [cited 2023 Feb 8 ];15:393-396
Available from: https://www.ojoonline.org/text.asp?2022/15/3/393/360418


Full Text



 Introduction



Idiopathic macular telangiectasia type 2 (MacTel 2) is a bilateral retinal disease of uncertain etiology presenting during the fifth and sixth decades of life. Prevalence of MacTel 2 may be as high as 0.1% in individuals above the age of 40 years.[1] A slow decrease in visual acuity and metamorphopsia form the most common symptoms with distinctive changes to the macular capillary network as well as neurosensory atrophy seen on multimodal imaging. MacTel 2 has been divided into two stages, a nonproliferative stage and a proliferative stage based on clinical, therapeutic, and prognostic relevance. The nonproliferative stage is further divided into atrophic and exudative subtypes. The atrophic stage is characterized by foveal atrophy which contributes to reduced vision. The exudative stage may be characterized by the presence of subfoveal detachment (SFD) which leads to visual decline.[1] The treatment with anti-vascular endothelial growth factors (anti-VEGF) for the exudative nonproliferative stage of MacTel 2 has been described in literature. We describe a case of exudative nonproliferative MacTel 2 where intraretinal fluid (IRF) developed along with SFD during follow-up and its response to anti-VEGF therapy.

 Case Report



A 62-year-old man, presented to us with complaints of progressive diminution of vision in both eyes (BE) for the past 6 months. He had no systemic illnesses. His best-corrected visual acuity (BCVA) was 6/12 in BE. On examination, his anterior segment showed pseudophakia in BE. Fundus examination of BE showed a dull gray lesion at the fovea with telangiectatic vessels. In addition, there was asteroid hyalosis in the right eye (RE) [Figure 1]a and [Figure 1]b. Fundus autofluorescence of the RE showed a pinpoint hyperautofluorescence at the fovea and the left eye (LE) revealed a moderate foveal hyperautofluorescence [Figure 1]c and [Figure 1]d. Spectral-domain optical coherence tomography (SD-OCT) of BE revealed inner retinal cavitation with preservation of the internal limiting membrane and mild foveal thinning [Figure 1]e and [Figure 1]f. Based on the multimodal imaging findings, he was diagnosed with BE MacTel 2 and was advised of regular follow-up.{Figure 1}

On his review after a year, he presented with complaints of decreased vision in the RE. On examination, his BCVA was 6/24 in the RE and 6/9 in the LE. Fundus examination in BE revealed a subtle grayish discoloration of the perifoveal retina with telangectatic vessels in BE and additionally, in the RE, there was the presence of shallow subretinal fluid at the fovea. SD-OCT revealed a subfoveal dome-shaped elevation suggestive of SFD along with hyporeflective cystoid spaces in the inner and outer retinal layers suggestive of IRF [Figure 2]a and [Figure 2]b. An OCT angiography (OCTA) was done which showed a minimal increase in space between vessels in the superficial and the deep vascular network in BE [Figure 2]c and [Figure 2]d. A diagnosis of RE exudative nonproliferative MacTel 2 was confirmed and the patient underwent intravitreal anti-VEGF injection of bevacizumab in the RE. On follow-up after a month his BVCA in RE was 6/24 with SD-OCT showing resolution of intraretinal hyporeflective cystoid spaces and mild reduction in SFD [Figure 3]a. Following two more intravitreal injections of bevacizumab his BCVA improved to 6/12 with SD-OCT showing complete resolution of SFD, which did not recur till the last follow-up visit after 1 year [Figure 3]b and [Figure 3]c.{Figure 2}{Figure 3}

A patient consent was acquired for the publication of this report and images.

 Discussion



Mac Tel 2 is an acquired neurodegenerative macular disease, most commonly bilateral, with a female preponderance.[1],[2] The pathogenesis of MacTel 2 involves degenerative changes mainly affecting the Muller cells. Retinal capillary endothelial cell degeneration probably triggers the vascular changes, leading to the outward proliferation of deep capillaries into photoreceptors.[1]

Nonproliferative MacTel 2 consist of an atrophic form and an exudative form. Increased vascular permeability of anomalous deep retinal capillaries results in low-grade intraretinal exudation which manifests as a mild perifoveal thickening in the early stage but later prominent degenerative changes resulting in retinal thinning. The formation of an intraretinal angiomatous complex due to the outward proliferation of deep capillaries eventually gives rise to subretinal neovascularization (SRNV).[1],[3] The source of fluid in nonproliferative MacTel 2 has not been clearly described angiographically. However, various authors have suggested that an intraretinal neovascular or ectatic complex is formed by anomalous capillaries growing deep into the photoreceptor layer.[3],[4] Excessive exudation from this complex leads to SFD before any hemorrhagic complications occur. Therefore, this exudative subset may be considered preproliferative stage of MacTel 2. Though SFD has previously been reported as an OCT subtype in nonproliferative exudative MacTel 2, intraretinal cystoid edema has never been reported. Our case showed intraretinal cystoid spaces in association with SFD and its relatively earlier resolution compared to SFD, with anti-VEGF, suggests that the intraretinal exudation also may rarely present as a subtype of exudative manifestation of MacTel 2. The closest differential considered for this intraretinal cystoid spaces associated with SFD in MacTel 2, was diabetic macular edema associated with MacTel 2. However, the patient was not a diabetic by history or blood investigations.

Eyes with MacTel 2 can develop SRNV. These cases present with the rapid decrease in vision, and is usually associated with subretinal hemorrhage, hard exudates, and scarring. In eyes with SRNV, there may be increased thickness and reflectivity of the outer retinal layer and the presence of subretinal fluid. OCTA was done to evaluate carefully for the presence of intraretinal neovascularization or SRNV, which revealed only ectatic deep capillary plexus and the absence of neovascularization. This suggests the deep capillary plexus ectasia as the source of intraretinal and subretinal exudation in this preproliferative subset of MacTel 2.

Various treatment modalities described in literature include photodynamic therapy, intravitreal triamcinolone acetonide, and intravitreal anti-VGEF agents have been described in the exudative variant of nonproliferative MacTel 2.[3],[4]

The role of anti-VEGF showing a good anatomical response (resolution of subretinal fluid) in nonproliferative MacTel 2 has been well documented in various studies.[5],[6] The visual outcomes have been contrasted in different reports. Mehta et al., in their series, did not find any visual improvement despite a good anatomical response to anti-VEGF therapy.[5] However, Manayath et al., in their comparative study, showed visual improvement in 87.5% of eyes treated with anti-VEGF as compared to an observation group, where visual worsening was seen in 62.5%. They postulated that visual loss in MacTel 2 has been attributed to irreversible atrophic changes in the outer retinal layers which may be accelerated by the presence of persistent long-standing SFD. They recommended prompt treatment with anti-VEGF to bring about immediate foveal reattachment and thereby delay foveal atrophy.[6] Similar to their study, our case despite the presence of intraretinal cystoid changes along with SFD, responded promptly to anti-VEGF therapy with the disappearance of intraretinal cystoid spaces preceding the resolution of SFD and requiring only three injections over a long-term follow-up of 1 year. To summarize, this is the first report to document the presence of intraretinal cystoid edema along with SFD in the exudative nonproliferative subset of MacTel 2 and its complete resolution with anti-VEGF over a long-term follow-up.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understand that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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