Abstract
PurposeTo evaluate retinal vascular changes on optical coherence tomography angiography (OCTA) in patients with macular telangiectasia type 2 (MacTel 2) and to assess their correlation with visual acuity.MethodsTwenty-six patients (52 eyes) with MacTel 2 and 20 age-matched controls (40 eyes) were included. Fundus examinations, including fundus autofluorescence, swept-source optical coherence tomography, and OCTA, were performed. Differences in the vascular density in the fovea and parafovea, the area of the foveal avascular zone, and the diameter of the ellipsoid zone defect of the two groups were analyzed.ResultsThe foveal vascular density of the superficial capillary plexus was significantly lower in the MacTel 2 group than in the control group (p = 0.027). The vascular density in the entire deep capillary plexus was also significantly less in the MacTel 2 group than in the control group (all p < 0.05). The mean diameter of ellipsoid-zone disruption on OCT in the MacTel 2 group was 634.6 ± 104.3 𝜇m. The foveal avascular zone areas of the superficial and deep capillary plexuses were significantly enlarged in the MacTel 2 group compared to those in the control group (0.45 ± 0.12 mm2 vs. 0.27 ± 0.08 mm2, p < 0.001; 0.56 ± 0.15 mm2 vs. 0.40 ± 0.14 mm2, p = 0.001). In addition, the enlarged foveal avascular zone of the superficial and deep plexus was negatively correlated with best corrected visual acuity (logMAR) in MacTel 2 patients (p = 0.013, r = -0.642 and p = 0.042, and r = -0.511, respectively).ConclusionsRetinal vascular density changes occur in the superficial fovea and in the entire deep capillary plexus of patients with MacTel 2. The enlarged foveal avascular zone areas of the superficial and deep plexuses were prominent in the MacTel 2 group, and this enlargement correlates with worsened visual acuity.
Highlights
The foveal avascular zone areas of the superficial and deep capillary plexuses were significantly enlarged in the MacTel 2 group compared to those in the control group (0.45 ± 0.12 mm2 vs. 0.27 ± 0.08 mm2, p < 0.001; 0.56 ± 0.15 mm2 vs. 0.40 ± 0.14 mm2, p = 0.001)
Retinal vascular density changes occur in the superficial fovea and in the entire deep capillary plexus of patients with MacTel 2
Retinal microvascular changes in macular telangiectasia type 2 superficial and deep plexuses were prominent in the MacTel 2 group, and this enlargement correlates with worsened visual acuity
Summary
Macular telangiectasia type 2 (MacTel 2) is a neurodegenerative disease related to Muller cell dysfunction and vascular alterations in the macular capillary network [1, 2]. Patients with this condition have loss of macular transparency surrounding the fovea, crystalline deposits in the inner retina, right-angled vessels, and intraretinal cavities [3, 4]. These typical changes occur along with thinning of the central retina and focal loss of the ellipsoid zone (EZ). It leads to central EZ loss and foveal atrophy.[6, 8] The presence of EZ loss on OCT is an important factor for the prognosis of patients with MacTel 2 [9]
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