Abstract
ObjectivesTo examine the topographical correlation between ellipsoid zone loss and telangiectasia in the deep capillary plexus in patients with macular telangiectasia type 2 (MacTel).Methods38 eyes (20 subjects) diagnosed with MacTel were imaged with OCTA between March 2016 and June 2019 in this single center, cross-sectional observational study. The en face OCTA and OCT were evaluated for areas of deep capillary plexus telangiectasia and ellipsoid zone loss, respectively, and their outlines were superimposed to study their overlap (mm2). The primary outcome was percentage of overlap and its relationship to MacTel stage. Secondary outcomes included the relationship between neovascularization and hyperreflective foci as well as correlations between ellipsoid zone loss, deep capillary plexus telangiectasia and visual acuity.ResultsIn nonproliferative MacTel stage, ellipsoid zone loss was localized to margins of telangiectatic areas (mean overlap = 15.2%). In proliferative stages, ellipsoid zone loss showed a higher degree of overlap with telangiectatic areas (mean overlap = 62.8%). Overlap increased with advancing MacTel stages, with an overall average of 45.3%. Overlap correlated highly with ellipsoid zone loss (r = 0.831; p<0.0001). Telangiectasia was present in all 38 eyes (range: 0.08mm2–0.99mm2), while ellipsoid zone loss was absent in 6 (range: 0.00–3.32mm2). Visual acuity correlated most strongly with ellipsoid zone loss (r = 0.569; p = 0.0002), followed by overlap (r = 0.544; p = 0.0004), and finally, telangiectasia (r = 0.404; p<0.0118). Presence of hyperreflective foci on OCT correlated with the presence and intraretinal location of neovascularization.ConclusionsEllipsoid zone loss occurs at the margins of deep capillary plexus telangiectasia in nonproliferative MacTel, with progressively increasing overlap as MacTel advances, peaking in proliferative disease. Deep capillary plexus telangiectasia and its overlap with ellipsoid zone loss are two promising markers of nonproliferative MacTel, while hyper-reflective foci are markers for proliferative MacTel.
Highlights
Idiopathic macular telangiectasia type 2 (MacTel) is a rare, acquired macular disease that leads to progressive vision impairment
In nonproliferative MacTel stage, ellipsoid zone loss was localized to margins of telangiectatic areas
Telangiectasia was present in all 38 eyes, while ellipsoid zone loss was absent in 6
Summary
Idiopathic macular telangiectasia type 2 (MacTel) is a rare, acquired macular disease that leads to progressive vision impairment. The DCP is of particular interest in MacTel as it harbors the majority of vascular changes[3], is poorly perfused (as marked by lower parafoveal vascular density[8, 9]), and may show evidence of MacTel before the superficial capillary plexus (SCP).[10]
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