Spark ImageWise 48

A Case Of Macular Telangiectasia With Vitreomacular Traction 

Dr. Shashank Somani, Dr. Sarang Lambat, Dr. Prabhat Nangia, Dr. Vinay Nangia 
Suraj Eye Institute, 559, New Colony, Sadar, Nagpur- 440001.

Case Description
A female, 70 years of age, came with the chief complaints of diminution of vision in both eyes (BE) since 2 years and distortion of vision in left eye since a month. She had a history of Diabetes mellitus and systemic hypertension since 15 years. On examination, her best corrected visual acuity (BCVA) was 6/12p, N8 in BE. Anterior segment examination showed pseudophakia in BE. Intraocular pressure (IOP) on Goldmann applanation tonometry (GAT) was 19 mmHg in BE

FigFigure 1 and 2 shows colour fundus photograph of right eye and left eye centered at the macula with presence of pigment patch (blue arrow) suggestive of RPE hyperplasia temporal to fovea in BE and multiple superficial crystals surrounding the pigment patch (red arrow), with presence of sheen suggestive of ERM (black arrow) superiorly in BE. 
Figure 3 – SD-OCT horizontal line scan passing through fovea of right eye, it shows a hyper reflective membrane on the surface of retina suggestive of ERM (white arrow). There is discontinuity in the inner layers of retina with small paracentral foveal cyst like spaces (yellow arrow) in the inner nuclear layer, dipping of the retinal layers temporal to fovea with loss of external limiting membrane (ELM), ellipsoid zone (EZ) and interdigitation zone (IDZ) (yellow Asterisk). 
Figure 4 – SD-OCT horizontal line scan passing through fovea of left eye showing thin hyper reflective membrane attached to the fovea (red arrows), with distortion of the normal foveal contour, causing development of a pseudocyst like space suggestive of an impending lamellar hole (green arrow) with vitreomacular traction. There is a collection of hyper reflective foci (blue arrow) which correlates with the hyper pigmented patch on colour fundus photo with loss of ELM, EZ and IDZ and retinal pigment epithelium (RPE) (yellow arrow). Choroidal shadowing (pink arrow) of the hyperreflective spot is seen. 

A diagnosis of both eye Macular telangiectasia (Type II) with grade 2 vitreomacular traction in the left eye was made. The Patient was advised to undergo pars plana vitrectomy (PPV) with ILM peeling with SF6 gas injection in the left eye to avoid progression of the traction and subsequent development of macular hole. The patient underwent surgery on 07/10/20.

Figure 5 – SD-OCT horizontal line scan passing through fovea of left eye done 1 month post operatively. It shows a better contour of fovea without any traction with resolution of the foveal cyst. The hyper reflective focus (Blue arrow) and the choroidal shadowing (pink arrow) is also seen. 

Discussion 

Macular telangiectasia is characterised by telangiectatic vessels in the juxtafoveolar region of one or both eyes. Gass divided macular Telangiectasia into three groups based upon the phenotype. Type I is typically a unilateral disease characterised by parafoveal dilation of capillaries, microaneurysms, leakage, and lipid deposition. Type II is the most common form of macular Telangiectasia and typically presents with bilateral juxtafoveal telangiectasias with minimal exudate. Type III is extremely rare and is characterised by occlusive telangiectasia.

The pathogenesis of macular Telangiectasia type II is unclear, but it has been postulated that Muller cell dysfunction in macular Telangiectasia type II results in endothelial degeneration, which may lead to retinal capillary proliferation and telangiectasia. The superficial crystals seen on colour fundus photo in patients with macular Telangiectasia type II are thought to represent footplates of degenerated Muller cells. In addition, it has been speculated that the spaces seen on OCT in macular Telangiectasia type II represent tissue loss from retinal degeneration, specifically due to the dysfunction or loss of Muller cells, rather than fluid filled cystic spaces.Muller cells convert glucose into lactate which they supply to the highly energy-demanding photoreceptors. In retinal disease, Muller cells may play a role in protection of photoreceptors through the release of neurotrophic factors.

Idiopathic macular holes are thought to develop from retinal cavitation due to vitreomacular traction. By contrast, in MacTel Type 2, macular holes are postulated to occur from retinal cavitation due to Muller cell degeneration. 

In our patient simultaneous presence of  retinal cavitation and vitreomacular traction features as seen in Fig 4, indicated a high risk of development of macular hole. The patient underwent PPV with ILM peeling and gas injection for the left eye in order to relieve the traction and prevent further loss of the vision due to formation of macular hole. Classical asymmetry of the disease was seen in our patient and the disease was found to be more severe in left eye as contrary to the findings in the MacTel Project Report Number 8 where the researchers found that the disease was severe in right eye of the patients. Occurrence of full thickness macular hole is known in the patients having macular Telangiectasia and perhaps removal of VMT and restoration of foveal contour have reduced the risk of development of macular hole in our patient. We were able to achieve satisfactory anatomical results following the surgery (Fig 5) and the patient was advised a regular follow up.

ReadWise:

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Dr. Sarang Lambat
MS, FRF
Consultant
Vitreoretinal services
Suraj Eye Institute
Nagpur
Email – education@surajeye.org

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