Abstract
In this cross-sectional observational study, we investigated the relationship between photoreceptor layer disruption and telangiectasia in patients diagnosed with early stage macular telangiectasia type 2 (MacTel). A total of 31 eyes (17 patients) with MacTel were imaged with adaptive optics scanning laser ophthalmoscopy (AOSLO) and optical coherence tomography angiography (OCTA). Confocal AOSLO was used to visualize dark regions of nonwaveguiding outer segments, which we refer to as “photoreceptor lesions”. En-face OCTA images of the deep capillary plexus (DCP) were used in conjunction with confocal AOSLO to evaluate the topographic relationship between areas of capillary telangiectasias and photoreceptor lesions. Among seven eyes with early stage MacTel (stage 0–2 based on OCT), we identified ten photoreceptor lesions, all of which were located within parafoveal quadrants containing DCP telangiectasia on OCTA. Seven of the lesions corresponded to the intact ellipsoid zone on spectral-domain OCT (SD-OCT), and three of these also corresponded to the intact interdigitation zone. This work demonstrates a topographic relationship between AOSLO photoreceptor lesions and DCP telangiectasias, and it also suggests that these lesions with normal SD-OCT appearance may represent areas of photoreceptors at risk for dysfunction. Thus, confocal AOSLO may have a meaningful role in detecting early photoreceptor abnormalities in eyes with MacTel.
Highlights
Macular telangiectasia type 2 (MacTel) is an acquired, bilateral degenerative retinal disease that leads to progressive loss of vision
It is characterized by juxtafoveolar telangiectatic capillaries, right-angled venules, loss of retinal transparency, and ellipsoid zone (EZ) loss on spectral-domain optical coherence tomography (SD-OCT) [1,2,3]
We wondered whether the telangiectasia that is seen in earlier stages of Mactel, before the appearance of EZ loss, could be related to subclinical photoreceptor dysfunction
Summary
Macular telangiectasia type 2 (MacTel) is an acquired, bilateral degenerative retinal disease that leads to progressive loss of vision. Over the last few decades, the basic understanding of the pathophysiology of MacTel has been transformed largely via histopathology studies [4,5,6] and OCT angiography (OCTA) imaging [7,8,9,10] The pathophysiology of this rare entity is thought to be related to complex neurovascular dysfunction—namely the loss of Muller cells leading to photoreceptor dysfunction and reactive vascular changes in the outer retina [4,6]. The results reported demonstrate the utility of AOSLO to better understand the pathophysiology of retinal diseases such as MacTel as well as to identify the earliest signs of photoreceptor abnormalities that might otherwise be missed using standard imaging modalities
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