PMC7803128_01
Case 1. Fundus image (a, b) and FA (c, d) show features consistent with MacTel Type 2 in both eyes. Preoperative OCT at initial presentation shows foveal thinning with outer neurosensory atrophy OD (e) and a full-thickness macular hole (FTMH) OS (f). The right eye developed a FTMH at a later visit (g). Later, postoperative OCT of each eye shows complete hole closure in each eye (h, i). Postop OCT-A photos in the right eye show areas of capillary dropout in the superficial bed (j) and in the left eye show telangiectasias in the superficial capillary bed temporal to the fovea with areas of capillary dropout (k).
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fig1
On fundus examination, there was a loss of the foveal reflex in the right eye and a FTMH in the left eye (Figures 1(a) and 1(b)). FTMH with an associated epiretinal membrane (ERM) in the left eye (Figure 1(f)). Fluorescein angiography was significant for mild leakage temporal to the fovea in both eyes with a window defect corresponding to the macular hole in the left eye (Figure 1(d)). At the 2-week postoperative visit, closure of the macular hole was confirmed by OCT (Figure 1(h)). The vision had dropped to 20/80, and OCT confirmed the presence of a FTMH (Figure 1(g)). The hole remained closed on her most recent follow-up visit 26 months after surgery with the BCVA stable at 20/25 (Figure 1(i)). A postoperative OCT-A photo of the right eye shows areas of capillary dropout in the superficial bed (Figure 1(j)) and in the left eye shows telangiectasias in the superficial capillary bed temporal to the fovea with areas of capillary dropout (Figure 1(k)); these are persistent vascular anomalies associated with MacTel Type 2 that have been described in the literature.
PMC7803128_02
Case 2. Fundus image (a, b) and FA (c, d) show loss of the foveal reflex with hyperfluorescence temporal to the fovea in both eyes. On presentation, OCT shows a FTMH in the right eye with intraretinal cysts and loss of the outer foveal layers in the left eye (e, f). Postoperative OCT of the right eye shows a closed macular hole (g). OCT of the left eye demonstrates a stable lamellar hole (f, h). Postop OCT-A shows right-angled venules and FAZ irregularity as well as significant telangiectasias of the deep vascular network with reduced vascular density (i-l). Postoperative FA shows persistent hyperfluorescence temporal to the fovea (m, n).
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Fundus examination revealed a macular hole in the right eye with pigment clumps temporal to the hole and a lamellar hole in the left macula with intraretinal crystals (Figures 2(a) and 2(b)). OCT confirmed the presence of a full-thickness macular hole in the right eye and a lamellar hole in the left (Figures 2(e) and 2(f)). FA showed leakage temporal to the fovea in both eyes with a window defect in the fovea of the right eye (Figures 2(c) and 2(d)). Twenty-one months after PPV, the macular hole in the right eye remained closed (Figure 2(g)) and BCVA was 20/20-1. Postoperative OCT-A demonstrated remodeling of the deep capillary plexus characteristic of MacTel Type 2 (Figures 2(k) and 2(l)).
PMC7803128_03
Case 3. Fundus image (a, b) and FA (c, d) show features typical of MacTel Type 2 with leakage temporal to the fovea in both eyes. On presentation, OCT (e, f) shows a FTMH in the left eye and some intraretinal cysts in the right eye. Postoperatively, OCT of the left eye (h) shows a closed macular hole. OCT-A of both eyes (i-l) demonstrates telangiectatic vessels, right-angled venules, and enlarged FAZ, with capillary bed closure temporal to the fovea. Postoperative FA of both eyes shows persistent leakage temporally (m, n).
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FTMH in the left eye (Figures 3(a) and 3(b)). FA revealed faint leakage temporal to the fovea in each eye, consistent with the diagnosis of MacTel Type 2 (Figures 3(c) and 3(d)). On the 5th postoperative day, the hole was sealed by OCT (Figure 3(g)). Postoperative OCT-A showed persistent features typical of MacTel Type 2 (Figures 3(i)-3(l)). Repeat FA showed significant leakage temporal to the fovea in the right eye and minimal leakage in the left eye (Figure 3(m)).
PMC7803128_04
Case 4. Fundus image (a, b) and FA (c, d) show findings consistent with MacTel Type 2. On presentation, OCT confirms a FTMH in the right eye (e). Postoperative OCT (g) shows the hole still being present. OCT-A (i, j) of the superficial layer shows telangiectatic vessels and vascular invasion of the FAZ, especially in the right eye (i).
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Fundus examination of the right eye revealed a blunted foveal reflex while the left eye showed pigmentary changes centrally and temporal scarring of the retinal pigment epithelium, without any evidence of diabetic retinopathy (Figures 4(a) and 4(b)). FA revealed bilateral foveal leakage characteristic of MacTel Type 2 (Figures 4(c) and 4(d)). In the right eye, OCT confirmed the clinically apparent FTMH (Figure 4(e)). In the left eye, OCT demonstrated hyperreflective deposits consistent with the macular scarring that was apparent on fundus examination (Figure 4(f)). The hole failed to close as shown on the postoperative OCT (Figure 4(g)), and her BCVA at the 22-month visit was stable at 20/60 in the right eye.